MedWorm: Atresia

Venous malformations of the temporal bone are a common feature in CHARGE syndrome

The Laryngoscope — Tue, 07 Feb 2012 05:00:00 +0100

Conclusions:Temporal bone venous abnormalities are a common feature in CHARGE syndrome. The pattern of venous abnormality suggests that there is a failure of the sigmoid sinus/jugular bulb to fully develop, resulting in persistence of emissary veins. Recognition of these abnormal venous structures during otologic surgery is critical to avoiding potentially catastrophic bleeding. (Source: The Laryngoscope)

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The Effect of Adriamycin Exposure on the Notochord of Mouse Embryos

Birth Defects Research Part B: Developmental and Reproductive Toxicology — Mon, 06 Feb 2012 05:00:00 +0100

The notochord has important structural and signaling properties during vertebrate development with key roles in patterning surrounding tissues, including the foregut. The adriamycin mouse model is an established model of foregut anomalies where exposure of embryos in utero to the drug adriamycin leads to malformations including oesophageal atresia and tracheoesophageal fistula. In addition to foregut abnormalities, treatment also causes branching, displacement, and hypertrophy of the notochord. Here, we explore the hypothesis that the notochord may be a primary target of disruption leading to abnormal patterning of the foregut by examining notochord position and structure in early embryos following adriamycin exposure. Treated (n = 46) and control (n = 30) embryos were examined during the ...

Hyberbaric oxygen increases atresia in normal & steroid induced PCO rat ovaries

Reproductive Biology and Endocrinology — Mon, 06 Feb 2012 05:00:00 +0100

Conclusions: HBOT of six weeks itself, changed ovarian morphology in favor of atresia both in PCO group and control group. This result of aggravated follicular atresia after HBOT on EV induced PCO may be due to long-term exposure in our protocol which with this state seems to be inapplicable in the improvement of PCO morphology. (Source: Reproductive Biology and Endocrinology)

Cholangiocyte cilia are abnormal in syndromic and non-syndromic biliary atresia

Modern Pathology AOP — Fri, 03 Feb 2012 05:00:00 +0100

Authors: Andrew S Chu, Pierre A Russo & Rebecca G Wells (Source: Modern Pathology AOP)

Clinical Characteristics Of Neonates With VACTERL Association

Pediatrics International — Thu, 02 Feb 2012 05:00:00 +0100

AbstractBackground: The VACTERL association (VA) is the nonrandom co‐occurrence of Vertebral anomalies, Anal atresia, Cardiovascular malformations, Tracheo‐esophageal fistula and/or Esophageal atresia, Renal anomalies, and/or Limb‐anomalies, and is referred to the first letters of its components. Studies investigating the clinical characteristics of VA patients and probing of the observed current six component types are limited, and none of them is focused to neonates. Clinical characteristics of our patients diagnosed as having VA in the newborn period is investigated.Methods: We retrospectively reviewed the neonates whose final diagnosis was VACTERL association. Presence of at least 3 components of previously reported 6 anomalies was accepted as VACTERL association. Gender, b...

Familial 4.8 MB deletion on 18q23 associated with growth hormone insufficiency and phenotypic variability

American Journal of Medical Genetics Part A — Thu, 02 Feb 2012 05:00:00 +0100

AbstractThe deletion of the long arm of chromosome 18 causes a contiguous gene deletion syndrome with a highly variable phenotype, usually related to the extent of the deleted region. The most commonly reported clinical features include: decreased growth, microcephaly, facial abnormalities, hypotonia, developmental delay, intellectual disability, congenital aural atresia with hearing impairment and limb anomalies. Here we report on a familial terminal deletion of 18q23 region transmitted from a mother to two daughters, resulting in a remarkable phenotypic variability. The deletion was first detected by conventional cytogenetic analysis in one daughter and subsequently characterized using fluorescence in situ hybridization (FISH) and array‐CGH. FISH analysis using subtelomeric 18p and 18q...

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Maternal Asthma Medication Use and the Risk of Selected Birth Defects

PEDIATRICS — Wed, 01 Feb 2012 05:00:00 +0100

CONCLUSIONS: Positive associations were observed for anorectal atresia, esophageal atresia, and omphalocele and maternal periconceptional asthma medication use, but not for other defects studied. It is possible that observed associations may be chance findings or may be a result of maternal asthma severity and related hypoxia rather than medication use. (Source: PEDIATRICS)

Overexpression of mouse TTF‐2 gene causes cleft palate

Journal of Cellular and Molecular Medicine — Wed, 01 Feb 2012 05:00:00 +0100

AbstractIn humans, mutations of the gene encoding for thyroid transcription factor‐2 (TTF‐2 or FOXE1) result in Bamforth syndrome. Bamforth syndrome is characterized by agenesis, cleft palate, spiky hair, and choanal atresia. TTF‐2 null mice (TTF‐2‐/‐) also exhibit cleft palate, suggesting its involvement in the palatogenesis. However, the molecular pathology and genetic regulation by TTF2 are largely unknown. In the present study, the recombinant expression vector pBROAD3‐TTF‐2 containing the promoter of the mouse ROSA26 gene was created to form the structural gene of mouse TTF‐2 and was microinjected into the male pronuclei of fertilized ova. Sequence analysis confirmed that the TTF‐2 transgenic mouse model was established successfully. The transgenic mice displayed a...

Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child

Pediatric Cardiology — Tue, 31 Jan 2012 16:45:46 +0100

We present an infant who died due to unilateral right-sided pulmonary vein atresia in conjunction with severe contralateral pulmonary vein stenosis who was diagnosed with the assistance of cardiac computed tomography scanning. Content Type Journal ArticleCategory Images in Pediatric CardiologyPages 1-3DOI 10.1007/s00246-012-0178-yAuthors Jeffrey E. Vergales, Division of Pediatric Cardiology, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA, USAShawn C. West, Division of Pediatric Cardiology, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA, USAAndrew W. Hoyer, Division of Pediatric Cardiology, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA, USA Journal Pediatric Cardiol...

Global gene analysis of oocytes from early stages in human folliculogenesis shows high expression of novel genes in reproduction

Molecular Human Reproduction — Fri, 27 Jan 2012 05:00:00 +0100

The pool of primordial follicles in humans is laid down during embryonic development and follicles can remain dormant for prolonged intervals, often decades, until individual follicles resume growth. The mechanisms that induce growth and maturation of primordial follicles are poorly understood but follicles once activated either continue growth or undergo atresia. We have isolated pure populations of oocytes from human primordial, intermediate and primary follicles using laser capture micro-dissection microscopy and evaluated the global gene expression profiles by whole-genome microarray analysis. The array data were confirmed by qPCR for selected genes. A total of 6301 unique genes were identified as significantly expressed representing enriched specific functional categories such as &lsq...

Surgical Results for Functional Univentricular Heart With Total Anomalous Pulmonary Venous Connection Over a 25-Year Experience [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 24 Jan 2012 05:00:00 +0100

Conclusions TAPVC III, IV, and pulmonary atresia are risk factors for early postoperative death. Intensive intervention, including perioperative management and operation, is required in these complex patients. (Source: The Annals of Thoracic Surgery)

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Risk Factor Analysis for Second-Stage Palliation of Single Ventricle Anatomy [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 24 Jan 2012 05:00:00 +0100

Conclusions Second-stage palliation can be performed at low risk for patients with left ventricular dominance, but significant risk remains for patients with left ventricular hypoplasia and unbalanced atrioventricular septal defect. Atrioventricular valve insufficiency is a persistent problem that has not been neutralized by repair strategies. (Source: The Annals of Thoracic Surgery)

Lung hypoplasia in rats with esophageal atresia and tracheo-esophageal fistula.

Pediatric Research — Wed, 18 Jan 2012 05:00:00 +0100

In conclusion, the lungs were hypoplastic in rats with experimental EA-TEF due to defective embryonal airway branching. However, arteriolar wall and respiratory epithelial patterns remained normal. These findings suggest that similarly defective lung development might contribute to chronic respiratory disease in EA-TEF patients.Methods:Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle on E7, E8, and E9. Lungs were recovered at E15, E18, and E2. Lung weight/body weight ratio, total DNA and protein, radial alveolar count, arteriolar wall thickness, lung maturity, and mesenchymal control of airway branching were assessed. E13 lungs were cultured for 72 h and explant airway branching was measured daily. For comparisons, nonparametric tests (*P < 0.05) were used. PM...

Congenital aural atresia: Bone-anchored hearing aid vs. external auditory canal reconstruction

International Journal of Pediatric Otorhinolaryngology — Tue, 17 Jan 2012 21:16:10 +0100

Abstract: Objective: To compare the audiologic outcome and feasibility of bone-anchored hearing aid (BAHA) and external auditory canal reconstruction (EACR) surgeries in pediatric patients presenting a congenital aural atresia (CAA).Methods: A retrospective chart review of 40 patients operated in our tertiary pediatric care center between 2002 and 2010 was performed. 20 patients underwent EACR, whereas another 20 patients were implanted with a BAHA device. Air conduction (AC), bone conduction (BC), pure tone average (PTA) and speech discrimination score (SDS) were compared preoperatively, and hearing gain (HG) postoperatively at 6 and at 12 months at frequencies of 500, 1000, 2000 and 4000Hz. Operative time, complications and associated microtia were documented as well. EACR patients were ...

4-vinylcyclohexene diepoxide: a model chemical for ovotoxicity.

Systems Biology in Reproductive Medicine — Mon, 16 Jan 2012 06:54:23 +0100

Authors: Kappeler CJ, Hoyer PB Abstract The occupational chemical 4-vinylcyclohexene diepoxide (VCD) has been shown to cause selective destruction of ovarian small pre-antral (primordial and primary) follicles in rats and mice by accelerating the natural, apoptotic process of atresia. Chemicals that destroy primordial follicles are of concern to women because exposure can result in premature ovarian failure (early menopause). In`itial studies using in vivo exposure of rats determined that VCD specifically targets primordial and primary (small pre-antral) follicles and that repeated dosing is required. Through a method of isolation of ovarian small follicles, biochemical and molecular studies determined that intracellular pro-apoptotic pathways are activated following VCD dosing in ...

Early Fibrotic Injury in A Rat Model of Bile Duct Ligation: Further Evidence of Intrinsic Matrix Metalloproteinase 8

Journal of Surgical Research — Sat, 14 Jan 2012 22:29:07 +0100

Conclusions: We have demonstrated a rapid but not immediate rise in MMP-8 expression at both a genetic and proteomic level. Interestingly TIMP-1 gene expression does not parallel that of MMP-8, but it is likely that this is due to higher levels of TIMP-1 present at baseline. While an increase in neutrophils is found immediately after injury, MMP-8 levels increase on day 4 of injury, providing further evidence of an intrinsic source of MMP-8. (Source: Journal of Surgical Research)

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Human Cholangiocyte Susceptibility to Infection With Rotavirus - Parallels to the Murine Model of Biliary Atresia

Journal of Surgical Research — Sat, 14 Jan 2012 22:28:58 +0100

(Source: Journal of Surgical Research)

The role of CK7, Ki‐67, CD34 and vimentin in the differentiation between biliary atresia and idiopathic neonatal hepatitis in Egyptian cholestatic neonates

APMIS — Thu, 12 Jan 2012 05:00:00 +0100

This study aimed to investigate the role of immunohistochemical staining of CK7, Ki67, CD34, and vimentin in addition to other clinicopathological features in the differentiation between BA and INH. Cases included 30 infants with BA and 30 infants with INH. The diagnosis was based on clinical, laboratory, and liver biopsy examination. Female gender and elevated serum gamma glutamyle transferase were in favor of BA. Portal tract changes, such as bile ductular proliferation documented by CK7, Ki67 immunostaining and angiogenesis documented by CD34 immunostaining, favored the diagnosis of BA. Copper associated protein was positive in 70% of BA cases, but not detected in INH cases. Parenchymatous changes, such as giant cell transformation and positive iron deposition and Kupffer cell prolifera...

An audit of a community protocol for identifying neonatal liver disease

Archives of Disease in Childhood — Wed, 11 Jan 2012 05:00:00 +0100

Performing a split bilirubin test to identify liver disease in any infant who remains jaundiced beyond 2 weeks of age has been recognised as good clinical practice. The Leeds Community Midwifery Team performed this test, following an agreed protocol, from December 2000. By February 2008, 882 infants had been tested. Three infants were identified as having significant liver disease, including one with biliary atresia. Examining the liver unit database, a further 38 infants with Leeds post codes presented with neonatal liver disease during the study period. Five infants were identified appropriately by the midwives but not reported via the study protocol, 29 were referred from secondary care, (1) by a general practitioner at 9 days of age and (2) who did not become jaundiced before 3 months,...

MR Imaging and CT Evaluation of Congenital Pulmonary Vein Abnormalities in Neonates and Infants [Pediatric Imaging]

Radiographics recent issues — Wed, 11 Jan 2012 05:00:00 +0100

Magnetic resonance (MR) imaging and computed tomography (CT) are increasingly being used in diagnosis and follow-up of congenital pulmonary vein anomalies in neonates and infants. Such anomalies include total or partial anomalous pulmonary venous return, sinus venosus defect, malposition of the septum primum, cor triatriatum, pulmonary vein atresia or stenosis, and abnormal number or course of the pulmonary veins. MR imaging provides a wealth of anatomic and functional data that are valuable in case management and planning intervention. Gadolinium-enhanced MR angiography is the mainstay of anatomic evaluation. Ventricular volumetry with two-dimensional steady-state free-precession sequences and flow analysis with cine phase-contrast imaging provide physiologic information that may be used ...

Generation of a cholangiocyte‐specific cDNA expression library for the identification of B and T cell autoantigens in murine biliary disease

Hepatology Research — Wed, 11 Jan 2012 05:00:00 +0100

Conclusion: A comprehensive mouse cholangiocyte cDNA expression library has been generated and is available for use as a source of multiple cholangiocyte‐specific antigens for immunological studies. The library can be used to screen for specificity of T cell lines or hybridomas. Furthermore, this library has potential uses in SEREX analysis of autoantibody reactivity. The cholangiocyte‐specific cDNA library is a powerful tool for the identification of target antigens in murine inflammatory cholangiopathies and is available as a shared resource. (Source: Hepatology Research)

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The Correlation Between Pre and Postoperative Hearing Level with High Resolution Computed Tomography (HRCT) Findings in Congenital Canal Atresia (CAA) Patients

Indian Journal of Otolaryngology and Head and Neck Surgery — Mon, 09 Jan 2012 19:37:47 +0100

This study was conducted at Center A to evaluate the correlation between pre and postoperative hearing level with HRCT based on a Jahrsdoefer grading system in patients with CAA. All records and HRCT films with CAA from January 1997 until December 2007 at Center A were evaluated. The demographic data, operative records, pre and post operative hearing levels and HRCT findings were analyzed. Hearing level in this study was based on a pure tone average of air-bone gap at 500 Hz, 1 kHz and 2 kHz or hearing level obtained from auditory brainstem response eudiometry. This study was approved by Research Ethics Committee (code number, FF-197-2008). Thirty-two ears were retrospectively evaluated. The postoperative hearing level of 30 dB and less was considered as successfu...

For Children With Rare Disease, Maternal Liver Grafts More Tolerable

Health News from Medical News Today — Thu, 05 Jan 2012 08:00:00 +0100

Results may have important implications for counseling parents on organ donation Children with a rare, life-threatening disease that is the most common cause of neonatal liver failure - biliary atresia - better tolerate liver transplants from their mothers than from their fathers, according to a UCSF-led study. In the study, researchers reviewed all pediatric liver transplants nationwide from 1996 to 2010, and compared the outcomes for patients who received liver grafts from their mothers with those for patients who received livers from their fathers... (Source: Health News from Medical News Today)

Genetic variability of interleukin4 gene in Taiwanese children with biliary atresia.

Cytokine — Thu, 05 Jan 2012 05:00:00 +0100

This study aims to investigate whether polymorphisms of the IL4 gene are associated with susceptibility to BA. Genomic DNA was extracted from whole blood samples of 53 Taiwanese children with BA and 904 ethnically-matched healthy controls. The IL4 -590 C/T, -33 C/T, and 8375 A/G polymorphisms were genotyped using the Pre-Developed TaqMan Allelic Discrimination Assay in a real-time polymerase chain reaction system. No significant difference between children with BA and healthy controls were found when comparing genotype, allele, carrier, and haplotype frequencies of these IL4 gene variants. These results suggest that the tested polymorphisms of IL4 gene are unlikely to contribute significantly to BA susceptibility in Taiwanese children. PMID: 22227092 [PubMed - as supplied by publisher]...

Chromosome 15q24 microdeletion syndrome

Orphanet Journal of Rare Diseases — Wed, 04 Jan 2012 05:00:00 +0100

is a recently described rare microdeletion syndrome that has been reported in 19 individuals. It is characterized by growth retardation, intellectual disability, and distinct facial features including long face with high anterior hairline, hypertelorism, epicanthal folds, downslanting palpebral fissures, sparse and broad medial eyebrows, broad and/or depressed nasal bridge, small mouth, long smooth philtrum, and full lower lip. Other common findings include skeletal and digital abnormalities, genital abnormalities in males, hypotonia, behavior problems, recurrent infections, and eye problems. Other less frequent findings include hearing loss, growth hormone deficiency, hernias, and obesity. Congenital malformations, while rare, can be severe and include structural brain anomalies, cardiov...

Maternal liver grafts more tolerable for children with rare disease

EurekAlert! - Medicine and Health — Tue, 03 Jan 2012 05:00:00 +0100

(University of California - San Francisco) Children with a rare, life-threatening disease that is the most common cause of neonatal liver failure -- biliary atresia -- better tolerate liver transplants from their mothers than from their fathers, according to a UCSF-led study. (Source: EurekAlert! - Medicine and Health)

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Decreased Risk of Graft Failure with Maternal Liver Transplantation in Patients with Biliary Atresia

American Journal of Transplantation — Sun, 01 Jan 2012 05:00:00 +0100

The presence of maternal cells in offspring may promote tolerance to noninherited maternal antigens (NIMAs). Children with biliary atresia (BA) have increased maternal cells in their livers, which may impact tolerance. We hypothesized that patients with BA would have improved outcomes when receiving a maternal liver. We reviewed all pediatric liver transplants recorded in the SRTR database from 1996 to 2010 and compared BA and non‐BA recipients of maternal livers with recipients of paternal livers for the incidences of graft failure and retransplantation. Rejection episodes after parental liver transplantation were examined for patients transplanted at our institution. BA patients receiving a maternal graft had lower rates of graft failure compared to those receiving a paternal graft (3....

Patients with Multiple Sclerosis with Structural Venous Abnormalities on MR Imaging Exhibit an Abnormal Flow Distribution of the Internal Jugular Veins

Journal of Vascular and Interventional Radiology : JVIR — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions: MR imaging provides a noninvasive means to separate stenotic from nonstenotic MS cases. The former group was more prevalent in the present MS population and carried significantly less flow in the IJVs than the latter. (Source: Journal of Vascular and Interventional Radiology : JVIR)

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Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

The authors report on their experience with a modified Spitz procedure using a Collis gastroplasty for the repair of long-gap esophageal atresia (EA). The authors reviewed 5 cases of children with long-gap (5-6 vertebral bodies) EA. The repair was carried out 3 months after permanent monitoring, enteral nutrition via gastrostomy tube, and permanent aspiration of the proximal esophagus. The Collis gastroplasty was performed to create a gastric tube along the lesser gastric curvature using an Endo-GIA linear stapler. The gastric tube can be easily mobilized into the thorax after ligation of the gastric vessels and anastomosed to the proximal esophageal segment. There were no introperative complications. The median postoperative hospital stay was 39 days (range, 30-60 days) with a median fol...

Preduodenal portal vein, malrotation, and high jejunal atresia: a case report

Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

We describe a newborn affected by high jejunal atresia, malrotation, and a complex cardiac anomaly, in whom PDPV was diagnosed at early relaparotomy because of stenosis of the jejunal anastomosis. Occurrence of PDPV with intestinal atresia has not been previously reported in the literature. (Source: Journal of Pediatric Surgery)

Bowhunter's syndrome diagnosed with provocative digital subtraction cerebral angiography.

Baylor University Medical Center Proceedings — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Taylor WB, Vandergriff CL, Opatowsky MJ, Layton KF Abstract Bowhunter's syndrome, also known as rotational occlusion of the vertebral artery, involves posterior circulation ischemia resulting from dynamic compromise of the dominant vertebral artery. This case highlights the importance of provocative digital subtraction angiography in making the diagnosis. A 41-year-old man presented for outpatient neurological evaluation for "lightheadedness" of several years' duration provoked by leftward head rotation. The only abnormality identified on initial magnetic resonance angiography was atresia of the nondominant left vertebral artery. Conventional digital subtraction angiography (DSA) followed by provocative DSA revealed development of a dynamic stenosis of the right vertebral ...

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Nine new twin pairs with esophageal atresia: A review of the literature and performance of a twin study of the disorder

Birth Defects Research Part A: Clinical and Molecular Teratology — Sun, 01 Jan 2012 05:00:00 +0100

CONCLUSIONThe observation of higher concordance rates for MZ compared to DZ twin pairs indicates that genetic factors contribute to isolated EA. Birth Defects Research (Part A) 2012. © 2012 Wiley Periodicals, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Indication of liver transplantation for jaundice-free biliary atresia with portal hypertension.

Annals of Transplantation — Fri, 30 Dec 2011 05:00:00 +0100

Conclusions: Even in jaundice-free BA patients, after supportive treatments for portal hypertension are performed, it is necessary to assess the esophageal and gastrointestinal varices regularly and to also prepare for LT simultaneously because there is a probability of the complication of uncontrollable gastrointestinal bleeding.<br /> PMID: 22210415 [PubMed - in process] (Source: Annals of Transplantation)

Iatrogenic Cushing syndrome due to nasal steroid drops

European Journal of Pediatrics — Wed, 28 Dec 2011 06:52:30 +0100

In conclusion, the serious side effects of the nasal drops should have been explained to the family, and their long-term use should have been refrained. Content Type Journal ArticleCategory Case ReportPages 1-2DOI 10.1007/s00431-011-1645-9Authors Veysel Nijat Baş, Clinics of Pediatric Endocrinology, Dr. Sami Ulus Research and Training Hospital of Women’s and Children’s Health and Diseases, Ankara, TurkeySemra Çetinkaya, Clinics of Pediatric Endocrinology, Dr. Sami Ulus Research and Training Hospital of Women’s and Children’s Health and Diseases, Ankara, TurkeyZehra Aycan, Clinics of Pediatric Endocrinology, Dr. Sami Ulus Research and Training Hospital of Women’s and Children’s Health and Diseases, Ankara, Turkey Journal European Journal of PediatricsOnline ISSN...

Inhibition of Follicular Development Induced by Chronic Unpredictable Stress Is Associated with Growth and Differentiation Factor 9 and Gonadotropin in Mice.

Biology of Reproduction — Wed, 28 Dec 2011 05:00:00 +0100

In this study, the chronic unpredictable mild stress model was used to produce psychosocial stress in mice. The number of different developmental stage of follicles was counted on H&E staining ovarian section. Real-time PCR and Western blotting were used to detect the mRNA and protein level of GDF9 respectively. The results show that chronic unpredictable stress inhibits follicular development, increases follicular atresia and suppresses GDF9 expression. Exogenous gonadotropins treatment partly restores the repressed antral follicular development, but has no effect on the repressed secondary follicular development associated with chronic stress. Treatment with recombinant GDF9 restores secondary follicular development. Co-treatments with GDF9 and gonadotropins restore both secondary an...

Parenteral nutrition supplementation in biliary atresia patients listed for liver transplantation

Liver Transplantation — Fri, 23 Dec 2011 13:27:30 +0100

In conclusion, PN improves the nutritional status of malnourished BA patients awaiting LT, and this is associated with post‐LT outcomes comparable to those of patients not requiring PN. Liver Transpl 18:121–129, 2012. © 2011 AASLD. (Source: Liver Transplantation)

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Pulmonary Atresia With Aortopulmonary and Coronary Artery Collaterals: Precise Depiction by Low-Dose Computed Tomography

Journal of the American College of Cardiology — Thu, 22 Dec 2011 02:25:47 +0100

A 6-year-old female with tetralogy of Fallot whose echocardiogram and cardiac catheterization showed pulmonary atresia, ventricular septal defect, aortopulmonary collaterals, and collaterals from the coronary arteries to the main pulmonary artery. Computed tomography (2 × 64-slice, 0.75-mm reconstruction; Online Video) with prospective electrocardiographic gating showed a large collateral from the right coronary artery ostium and another collateral arising from the left coronary artery ostium supplying a small main pulmonary artery (A and B). There was severe hypoplasia and isolation of the right pulmonary artery before bifurcation into upper and lower lobe branches, which were supplied by multiple aortopulmonary collaterals (A and C). This rare variant of tetralogy of Fallot has been pre...

Growth Factors, Their Receptors, Neuropeptide-Containing Innervation, and Matrix Metalloproteinases in the Proximal and Distal Ends of the Esophagus in Children With Esophageal Atresia.

Medicina (Kaunas) — Wed, 21 Dec 2011 10:12:03 +0100

CONCLUSIONS. A decrease in PGP 9.5-containing neuronal structures in the proximal esophagus supports insufficient innervation of this part of the organ in EA. A decrease in MMP-2 positive cells in the esophageal atresia-affected proximal esophagus indicates also a possible decrease of tissue adaptive and regenerative reactions. Low expression of TGF-β and almost the absence of EGFR in the EA-affected specimens may result in disturbances of cell growth, proliferation, and differentiation, indicating a significant role of these substances in morphopathogenesis of EA. FGFR and VEGF seem not to characterize EA pathogenesis. PMID: 22123559 [PubMed - as supplied by publisher] (Source: Medicina (Kaunas))

Detecting 22q11.2 deletion in Chinese children with conotruncal heart defects and single nucleotide polymorphisms in the haploid TBX1 locus

BMC Medical Genetics - Latest articles — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions: CTDs, especially pulmonary atresia with ventricular septal defect and tetralogy of Fallot, are the most common disorders associated with the 22q11.2 deletion syndrome. Those patients with both CTDs and 22q11.2 deletion generally have a typical or atypical deletion region within the TBX1 gene. Our results indicate that TBX1 genetic variants may be associated with CTDs. (Source: BMC Medical Genetics - Latest articles)

Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collaterals: Neonatal Pulmonary Artery Rehabilitation Without Unifocalization [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival. (Source: The Annals of Thoracic Surgery)

Long‐term maintenance treatment with omeprazole in children with healed erosive oesophagitis: a prospective study

Alimentary Pharmacology and Therapeutics — Sun, 18 Dec 2011 05:00:00 +0100

Conclusions Remission of erosive oesophagitis is maintained with omeprazole treatment for at least 21 months in most children aged 1–16 years, and the drug is well tolerated. To maintain remission, some 60% of patients require more than half the dose required for healing. In children with GERD‐predisposing conditions, GERD is often chronic and relapsing, and requires long‐term management. (Source: Alimentary Pharmacology and Therapeutics)

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Pulmonary Atresia with Ventricular Septal Defect and Multifocal Pulmonary Blood Supply: Does an Intensive Interventional Approach Improve the Outcome?

Congenital Heart Disease — Fri, 16 Dec 2011 05:00:00 +0100

Conclusions. Combining surgical unifocalization procedures with subsequent early and intensive catheter‐based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions. (Source: Congenital Heart Disease)

Hybrid intra-operative pulmonary artery stenting in congenital heart disease

Indian Journal of Thoracic and Cardiovascular Surgery — Wed, 14 Dec 2011 16:40:31 +0100

Conclusion Hybrid PA stenting during surgery for congenital heart disease in cases of complex branch PA stenosis is an alternative to traditional angioplasty. The procedure is safe and effective, and ameliorates right-ventricular dysfunction. Content Type Journal ArticleCategory Original ArticlePages 1-5DOI 10.1007/s12055-011-0127-zAuthors Kouakou Grégoire Ayegnon, Cardiac Pediatric Surgery Unity, Clochville of Tours, FranceEric Bergoend, Cardiac Pediatric Surgery Unity, Clochville of Tours, FranceThierry Bourguignon, Cardiac Pediatric Surgery Unity, Clochville of Tours, FranceRoland Bonnefoy, Pediatric Cardiology Unity, Clochville of Tours, FrancePaul Neville, Cardiac Pediatric Surgery Unity, Clochville of Tours, France Journal Indian Journal of Thoracic and...

Incidence, Diagnosis, and Outcomes of Vocal Fold Immobility After Esophageal Atresia (EA) and/or Tracheoesophageal Fistula (TEF) Repair

European Journal of Pediatric Surgery — Wed, 14 Dec 2011 05:00:00 +0100

In this study, 3% of patients were diagnosed with vocal fold paralysis after esophageal atresia repair. The etiology of vocal fold paralysis in this study is difficult to assess. Preoperative fiberoptic laryngoscopy is recommended to identify children with congenital vocal fold paralysis prior to surgical intervention, especially in those requiring revision surgery.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Practical Imaging Approach to Bowel Obstruction in Neonates: A Review and Update

Seminars in Roentgenology — Tue, 13 Dec 2011 21:25:22 +0100

The newborn with intestinal obstruction can elicit unease in many radiologists and neonatologists. The clinical presentation may be nonspecific, with bilious or nonbilious vomiting, abdominal distension, and often failure to pass meconium evident. The etiology of neonatal intestinal obstruction could lie anywhere from esophagus to anus. Some disorders, such as malrotation with midgut volvulus, are life-threatening and require prompt decision-making, whereas others, such as duodenal atresia, which often have a similar clinical presentation, may be managed semielectively. This is one scenario in which the application of a logical approach, radiology, provides the most definitive information. The overarching goal of this article is to provide a helpful and practical imaging strategy for evalu...

Coronary Artery Ostial Atresia: Diagnosis of Conotruncal Anastomotic Collateral Rings Using CT Angiography

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 12 Dec 2011 05:00:00 +0100

(Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

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IMD/ADM2 Facilitates Oocyte-Cumulus Cell Interactions [Genomics and Proteomics]

Journal of Biological Chemistry — Fri, 09 Dec 2011 05:00:00 +0100

Ovarian folliculogenesis has been studied as a model of hormonal regulation of development and differentiation, cell death, and cell-cell communication. In addition to gonadotropins from the pituitary and follicular paracrine factors, oocyte secreted factors have been shown to play critical roles in the regulation of follicular cell functions. Except for the well characterized BMP family proteins, including GDF9 and BMP15, oocytes are known to secrete oocyte secreted factors that are important for the regulation of cumulus cell survival and the maintenance of tertiary structure of cumulus cell-enclosed oocyte complexes (COCs). Based on genomic screening and studies of COCs cultured in vitro, we showed that intermedin (IMD)/adrenomedullin 2 (ADM2) is a novel oocyte-derived ligand important ...

Fetal Gastroschisis: A Comparison of Second vs. Third-Trimester Bowel Dilatation for Predicting Bowel Atresia and Neonatal Outcomes.

Ultraschall in der Medizin — Fri, 09 Dec 2011 05:00:00 +0100

CONCLUSION: Fetuses with isolated gastroschisis successfully underwent postnatal surgery in most cases (93.2 %), except for one termination, one intrauterine death and 3 cases of neonatal death. A fetal bowel dilatation > 10 mm before 30 + 0 weeks had the highest predictive value for postnatal bowel complications. PMID: 22161619 [PubMed - as supplied by publisher] (Source: Ultraschall in der Medizin)

Fenestration closure in a calcified ventricular septal defect patch [CASE STUDIES]

Asian Cardiovascular and Thoracic Annals — Wed, 07 Dec 2011 05:00:00 +0100

We describe the closure of such a defect in a 6-year-old boy, using a double Dacron patch sandwich. (Source: Asian Cardiovascular and Thoracic Annals)

Abnormal Development of Lung Innervation in Experimental Esophageal Atresia

European Journal of Pediatric Surgery — Wed, 07 Dec 2011 05:00:00 +0100

This study tests the hypothesis in rats with EA-TEF that deficient lung innervation could be one of the causes of respiratory disease.Pregnant rats were treated with either 2 mg/kg i.p. adriamycin or vehicle on E7, E8 and E9. Lungs and tracheas were retrieved on E15, E18 and E21 (term: E22). Innervation was examined by regular (PGP 9.5 and GDNF) and whole-mount (PGP 9.5 and α-actin) immunohistochemistry. PGP 9.5 and GDNF mRNA were measured by real-time, quantitative RT-PCR and the levels of PGP 9.5 protein by immunoblot. Embryonic lung primordia harvested on E13 were cultured for 72 h and airway peristalsis was assessed under an inverted microscope. PGP 9.5 expression was then examined in explants by whole-mount immunohistochemistry and RT-PCR. Values were compared with non-parametric...

Patients With Biliary Atresia Have Elevated Direct/Conjugated Bilirubin Levels Shortly After Birth

PEDIATRICS — Thu, 01 Dec 2011 05:00:00 +0100

CONCLUSIONS: Patients with BA have elevated DB/CB levels shortly after birth. To detect affected infants earlier and improve outcomes, the results suggest two possibilities: (1) screen all newborns for elevated DB/CB levels, rather than just those who appear jaundiced; and then (2) follow all newborns with elevated DB/CB levels, rather than just those with DB:TB ratios >0.2. (Source: PEDIATRICS)

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Early Detection of Biliary Atresia Raises Questions About Etiology and Screening

PEDIATRICS — Thu, 01 Dec 2011 05:00:00 +0100

(Source: PEDIATRICS)

Patients With Biliary Atresia Have Elevated Direct/Conjugated Bilirubin Levels Shortly After Birth

PEDIATRICS — Thu, 01 Dec 2011 05:00:00 +0100

Infants with biliary atresia (BA) have better outcomes if detected and treated early, typically before 8 weeks of age. Making an early diagnosis is difficult, however, because newborns appear healthy and start developing disease at an unknown time. Patients with BA have elevated direct/conjugated bilirubin (DB/CB) levels at birth. BA could be detected earlier if: (1) all newborns have DB/CB levels measured, including those not jaundiced; and (2) all elevated DB/CB levels are followed, independent of total bilirubin measurements. (Read the full article) (Source: PEDIATRICS)

Targeted Proteolysis of Plectin Isoform 1a Accounts for Hemidesmosome Dysfunction in Mice Mimicking the Dominant Skin Blistering Disease EBS-Ogna

PLoS Genetics — Thu, 01 Dec 2011 05:00:00 +0100

by Gernot Walko, Nevena Vukasinovic, Karin Gross, Irmgard Fischer, Sabrina Sibitz, Peter Fuchs, Siegfried Reipert, Ute Jungwirth, Walter Berger, Ulrich Salzer, Oliviero Carugo, Maria J. Castañón, Gerhard Wiche Autosomal recessive mutations in the cytolinker protein plectin account for the multisystem disorders epidermolysis bullosa simplex (EBS) associated with muscular dystrophy (EBS-MD), pyloric atresia (EBS-PA), and congenital myasthenia (EBS-CMS). In contrast, a dominant missense mutation leads to the disease EBS-Ogna, manifesting exclusively as skin fragility. We have exploited this trait to study the molecular basis of hemidesmosome failure in EBS-Ogna and to reveal the contribution of plectin to hemidesmosome homeostasis. We generated EBS-Ogna knock-in mice mimicking the huma...

Obstructive Sleep Apnea in Infants.

Am J Respir Crit Car... — Thu, 01 Dec 2011 05:00:00 +0100

Authors: Katz ES, Mitchell RB, D'Ambrosio CM Abstract Obstructive sleep apnea in infants has a distinctive pathophysiology, natural history, and treatment compared to older children and adults. Infants have both anatomical and physiological predispositions towards airway obstruction and gas exchange abnormalities; including a superiorly-placed larynx, increased chest wall compliance, ventilation-perfusion mismatching, and ventilatory control instability. Congenital abnormalities of the airway, such as laryngomalacia, hemangiomas, pyriform aperature stenosis, choanal atresia and laryngeal webs may also have adverse effects on airway patency. Additional exacerbating factors predisposing infants towards airway collapse include neck flexion, airway secretions, gastro-esophageal reflux,...

Psychosocial and cognitive consequences of major neonatal surgery

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions: To ensure true quality of life after neonatal surgical stress, pediatric surgeons must consider not only physical assessments but also cognitive, emotional, and psychosocial assessments. (Source: Journal of Pediatric Surgery)

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The outcome of conservative treatment for anastomotic leakage after surgical repair of esophageal atresia

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions: Postoperative esophageal AL is effectively treatable by conservative methods in most neonates. The occurrence of AL may aggravate the severity of esophageal stricture but does not affect the incidence of GER, esophageal dysmotility, and tracheomalacia. (Source: Journal of Pediatric Surgery)

Bile duct duplication as a cause of distal bowel gas in neonatal duodenal obstruction

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions: Neonatal DA presenting with distal bowel gas via an anomalous bifurcated bile duct conduit is more common than initially thought and occurs more frequently than duodenal stenosis. These patients might be at risk for cholestasis, possibly owing to duodeno-biliary reflux through the ampulla. (Source: Journal of Pediatric Surgery)

Colon atresia and frontal encephalocele: a rare association

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

We present a case of an infant with colonic atresia and a frontal encephalocele and believe this to be a newly reported association. We review possible pathogenic mechanisms. (Source: Journal of Pediatric Surgery)

Characteristics of Bovine Oocytes with Different Meiotic Competence in Terms of Their Mitochondrial Status and Expression of Nuclear‐Encoded Factors

Reproduction in Domestic Animals — Thu, 01 Dec 2011 05:00:00 +0100

ContentThe present study was designed to characterize bovine oocytes with different meiotic competence and atresia levels in terms of their mitochondrial status. Oocyte subpopulations were recovered either from medium (MF) or small (SF) follicles and categorized as healthy, light‐atretic and mid‐atretic according to oocyte morphology. Mitochondrial activity, morphology and distribution, adenosine triphosphate (ATP) content and expression of mitochondrial transcription factor A (TFAM) and nuclear respiratory factor 1 (NRF1) were assessed before (GV) and after (MII) maturation. The data were related to follicular size regardless of or with regard to oocyte atresia. Regardless of atresia, the MF subpopulation showed a significantly higher mitochondrial activity and frequency of oocytes wi...

Hepatic Arterial Buffer Response after Pediatric Living Donor Liver Transplantation: Report of a Case

Transplantation Proceedings — Thu, 01 Dec 2011 05:00:00 +0100

Conclusion: Because excessive portal pressure after LDLT is reversible, it has been suggested that it may be possible to prevent the progress of hepatic arterial complications if temporary portal pressure modulation can be performed for cases among the high-risk group for hepatic arterial complications. (Source: Transplantation Proceedings)

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Management of Single‐Ventricle Patients With Berlin Heart EXCOR Ventricular Assist Device: Single‐Center Experience

Artificial Organs — Thu, 01 Dec 2011 05:00:00 +0100

This study aims to describe our center's multidisciplinary team management of single‐ventricle patients supported long term with the Berlin Heart EXCOR Pediatric VAD. Patient #1 was a 4‐year‐old with double‐outlet right ventricle with aortic atresia, L‐looped ventricles, and heart block who developed heart failure 1 year after Fontan. She initially required extracorporeal membrane oxygenation support and was transitioned to Berlin Heart systemic VAD. She was supported for 363 days (cardiac intensive care unit [CICU] 335 days, floor 28 days). The postoperative course was complicated by intermittent infection including methicillin‐resistant Staphylococcus aureus, intermittent hepatic and renal insufficiencies, and transient antithrombin, protein C, and protein S deficiencies resu...

Laparoscopic Gastric Pull-Up and Thoracoscopic Esophago-Esophagostomy Combined with Intrathoracic Fundoplication for Long-Gap Pure Esophageal Atresia

Journal of Laparoendoscopic — Wed, 30 Nov 2011 19:58:02 +0100

Journal of Laparoendoscopic & Advanced Surgical Techniques Dec 2011, Vol. 21, No. 10: 973-978. (Source: Journal of Laparoendoscopic)

Association of X‐prolyl aminopeptidase 1 rs17095355 polymorphism with biliary atresia in Thai children

Hepatology Research — Mon, 28 Nov 2011 08:00:31 +0100

Conclusion: The association between XPNPEP1 rs17095355 polymorphism and BA has been demonstrated, particularly with the T allele. We hypothesize that the XPNPEP1 rs17095355 polymorphism confers increased susceptibility to the disease. (Source: Hepatology Research)

Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience [Original articles]

European Journal of Cardio-Thoracic Surgery — Wed, 23 Nov 2011 05:00:00 +0100

Conclusions: A simple three-tiered classification based on RV size may allow initial stratification into biventricular or univentricular repair for patients with normal RV size and severe RV hypoplasia. In patients with moderate RV hypoplasia, the presence of RV-to-coronary-artery connections or a TV Z-score < –2 should caution one against attempting biventricular repair. (Source: European Journal of Cardio-Thoracic Surgery)

Developmental Maxillofacial Anomalies

Seminars in Ultrasound CT and MRI — Tue, 22 Nov 2011 20:40:15 +0100

There is a wide variety of developmental maxillofacial anomalies secondary to defective transformation of the first pair of pharyngeal arches into adult structures. Computed tomography and magnetic resonance imaging are essential to precisely define aberrant maxillofacial anatomy and guide appropriate management. This review article provides an overview of maxillofacial embryogenesis, clinical symptoms, and characteristic imaging features of several anomalies, including choanal atresia, congenital nasal piriform aperture stenosis, dacryocystoceles, nasolacrimal duct stenosis, cleft lip and palate, micrognathia, and midline nasal masses. Finally the major features of common first pharyngeal arch syndromes are discussed. (Source: Seminars in Ultrasound CT and MRI)

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Successful management of a thick transverse vaginal septum with a vesicovaginal fistula by vaginal expansion and surgery

International Urogynecology Journal — Tue, 22 Nov 2011 18:01:42 +0100

We present a case of a thick transverse vaginal septum with a vesicovaginal fistula treated with vaginal expansion using dilators, followed by surgery. A 27-year-old woman was admitted to our hospital with vaginal atresia and cyclical hematuria. Urethroscopy and cystography showed a vesicovaginal fistula between the proximal vagina and bladder. The vaginal septum was 4 cm thick. Over a 6-month period, the distal vagina was expanded with the use of dilators and the septum was sufficiently thinned. Reconstruction using a transvaginal and transabdominal approach created a direct anastomosis between the proximal and distal vagina. No vaginal strictures or contractures in the anastomotic region have been observed during the 12 months following surgery. The combination of expansi...

Cytomegalovirus‐specific T cell reactivity in biliary atresia at the time of diagnosis is associated with deficits in regulatory T cells

Hepatology — Tue, 22 Nov 2011 05:00:00 +0100

Conclusions: Liver T cell responses to CMV were identified in the majority of BA patients at diagnosis, suggesting perinatal CMV infection as a plausible initiator of bile duct damage. Deficiency of Tregs in BA implies decreased inhibition of inflammation and autoreactivity, potentially allowing for exaggerated bile duct injury. (HEPATOLOGY 2011.) (Source: Hepatology)

Exuberant Mucometra Associated with Atresia of the Cervix in a Queen

Reproduction in Domestic Animals — Tue, 22 Nov 2011 05:00:00 +0100

ContentsThis short communication reports the clinical, ultrasonographic and histopathological findings in a cat with atresia of the uterine cervix and mucometra. After 6 months of continuous oestrous behaviour, a remarkable abdominal enlargement was observed in a 14‐year‐old queen. A presumptive diagnosis of mucometra was concluded after the ultrasound evaluation and based on clinical signs and blood analyses. Ovariohysterectomy revealed a notable symmetrical distension (4–5 cm in diameter) of both uterine horns that were filled with fluid (690 ml); microbiological analyses confirmed the aseptic nature of the uterine fluid. Ovarian follicular cysts and cystic subsurface epithelial structures, >1.5 cm in diameter, were present in both ovaries and no corpora lutea were obser...

An audit of anesthetic management and complications of tracheo‐esophageal fistula and esophageal atresia repair

Pediatric Anesthesia — Tue, 22 Nov 2011 00:47:02 +0100

Conclusions: This audit adds to the data already published about incidences of complications and comorbidities associated with TOF/OA repair. Defining anesthetic practice with regard to ventilation and analgesic strategies is important in comparing the adequacy and risk of techniques used. Our audit shows that a range of differing anesthetic techniques are still employed by different anesthetists and institutions and details some of the techniques being used for managing difficult ventilation. (Source: Pediatric Anesthesia)

Direct/Conjugated Bilirubin Predicts Biliary AtresiaDirect/Conjugated Bilirubin Predicts Biliary Atresia

Medscape Today Headlines — Mon, 21 Nov 2011 23:17:13 +0100

Researchers have found evidence that infants with biliary atresia exhibit elevated direct/conjugated bilirubin levels at birth. Medscape Medical News (Source: Medscape Today Headlines)

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Maternal Microchimerism in Hirschsprung’s Disease

American Journal of Perinatology — Mon, 21 Nov 2011 05:00:00 +0100

Amer J PerinatolDOI: 10.1055/s-0031-1295645Hirschsprung’s disease (HD) presents with severe constipation due to absent ganglion cells in the distal rectum. We sought to determine whether maternal chimeric cells are present in aganglionic bowel. We hypothesize that chimeric cells are part of the unfavorable microenvironment that leads to the destruction of enteric neurons in HD. Intestinal biopsies and resections from seven male patients with HD were compared with four male patients with chronic constipation and six with bowel atresia. Fluorescence in situ hybridization was used to identify chimeric cells based on male/female (XX/XY) differences. The location and immunophenotype of chimeric cells were also studied. Chimeric cells were present more often in the small intestine and rectum, ...

The effects of FSH and Activin A on follicle development in vitro.

Reproduction — Mon, 21 Nov 2011 05:00:00 +0100

Authors: Cossigny DA, Findlay JK, Drummond A Abstract Numerous studies have shown roles for activins in gonadal regulation, however little is known about its specific roles in early folliculogenesis. Ovarian follicular growth was investigated in 10-day cultures of day 4 postnatal whole ovaries treated with activin A (ActA; 50ng/ml), with or without FSH (100ng/ml) in vitro. We hypothesized that treatment with ActA + FSH would affect rates of growth and atresia in follicles. None of the treatments effected primordial follicle activation and antral follicles were not observed after 10 days in culture. Primordial follicle numbers from all treatment groups were approximately 20% of those in day 4 fresh ovaries indicating that activation had occurred. In the presence of ActA, preantral f...

Stent implantation into the interatrial septum in patients with univentricular heart and a secondary restriction of interatrial communication.

Kardiologia Polska — Sat, 19 Nov 2011 21:24:02 +0100

Conclusions: Percutaneous stent implantation into the interatrial septum in children with univentricular heart and secondary restriction of interatrial communication is a safe and effective method. Kardiol Pol 2011; 69, 11: 1137-1141. PMID: 22090221 [PubMed - in process] (Source: Kardiologia Polska)

Congenital aural atresia treated with floating mass transducer on the round window: 5 years of imaging experience

La Radiologia Medica — Fri, 18 Nov 2011 06:53:19 +0100

Conclusions HRCT provides accurate information about anatomy and malformations of the middle and inner ear and can thus assist the surgeon in planning the procedure. Content Type Journal ArticleCategory Head and Neck Radiology / Radiologia della Testa e del ColloPages 1-12DOI 10.1007/s11547-011-0751-0Authors M. Barillari, Institute of Radiology, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, ItalyR. Cerini, Institute of Radiology, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, ItalyM. Carner, ENT Department, University of Verona, Verona, ItalyC. Cacciatori, Institute of Radiology, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, ItalyF. Spagnolli, Institute of Radiology, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, ...

Double bubble sign

Emergency Medicine Journal — Fri, 18 Nov 2011 05:00:00 +0100

A 4-day-old full-term male neonate presented with postprandial non-bilious vomiting. Delayed meconium passage had been recorded at 36 h of age. Progressive abdominal distention was noted. A supine radiograph of the abdomen showed marked distention of the stomach and proximal duodenum with double bubble sign (figure 1). Air density was not identified distal to the duodenum. Duodenal obstruction was strongly suspected. Exploratory laparotomy revealed duodenal atresia proximal to the ampulla of Vater and severe dilatation in the first part of the duodenum, which were repaired by duodenoduodenostomy. The double bubble sign is a classic radiographic manifestation of duodenal obstruction, the cause of which could be intrinsic (such as duodenal atresia, duodenal stenosis or duodenal web) or ...

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Efficacy of US-guided Percutaneous Cholecystocholangiography for the Early Exclusion and Type Determination of Biliary Atresia [Pediatric Imaging]

Radiology — Thu, 17 Nov 2011 05:00:00 +0100

Conclusion: PCC is a safe and useful technique for early exclusion when biliary atresia cannot be ruled out after traditional screening tests; in addition, it may be useful for preoperative type determination of biliary atresia. © RSNA, 2011 (Source: Radiology)

Biliary Atresia in Neonates and Infants [Letters to the Editor]

Radiology — Thu, 17 Nov 2011 05:00:00 +0100

(Source: Radiology)

Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience

European Journal of Pediatrics — Mon, 14 Nov 2011 16:53:24 +0100

Conclusions: For patients with TOF-PA, CT could accurately delineate pulmonary arterial morphology with the same level of accuracy as cardiac catheterization. Therefore, CT can be considered a reasonable diagnostic alternative for such patients. Content Type Journal ArticleCategory Original PaperPages 1-8DOI 10.1007/s00431-011-1621-4Authors Ming-Tai Lin, Department of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanJou-Kou Wang, Department of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanYih-Sharng Chen, College of Medicine, National Taiwan University, Taipei, TaiwanWen-Jeng Lee, College of Medicine, National Taiwan University, Taipei, TaiwanHsin-Hui Chiu, Department of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanChun-An Chen, Depa...

Mutational analysis of NOG in esophageal atresia and tracheoesophageal fistula patients

Pediatric Surgery International — Mon, 14 Nov 2011 16:51:22 +0100

Conclusion SNPs in the coding region of the NOG gene are identified infrequently in human cases of EA/TEF. Further investigation of SNPs in the promoter region of NOG is warranted, as is the effect of synonymous SNPs on NOG mRNA stability. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s00383-011-3022-1Authors Andrew J. Murphy, Department of Pediatric Surgery, Vanderbilt University Children’s Hospital, Doctor’s Office Tower, Suite 7102, 2200 Children’s Way, Nashville, TN 37232-9780, USAYina Li, Department of Cell and Developmental Biology, Vanderbilt University Medical Center, Nashville, TN 37232, USAJoshua B. Pietsch, Department of Pediatric Surgery, Vanderbilt University Children’s Hospital, Doctor’s Office Tower, Suite 7102, 220...

Stool color cards hasten referrals for biliary atresia

Modern Medicine — Fri, 11 Nov 2011 05:00:00 +0100

NEW YORK (Reuters Health) - Using stool color cards to screen newborns for biliary atresia increases both physician and parent awareness of the condition, and also leads to earlier treatment, Taiwanese researchers reported online in Pediatrics October 24th. (Source: Modern Medicine)

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Main Cause Of Severe Pediatric Liver Disease Now Has Possible Treatment Target

Health News from Medical News Today — Thu, 10 Nov 2011 08:00:00 +0100

Unexpected discovery of a new molecular signature for a destructive and often lethal pediatric liver disease may lead to a new therapeutic target for the hard-to-treat condition. In a study that included human livers and a mouse model of biliary atresia, researchers report in the November Journal of Clinical Investigation that not all children with biliary atresia share the same disease process. Some patients have a second molecular conductor of disease called Th2 (T helper cell 2) immune system... (Source: Health News from Medical News Today)

Copy number detection in discordant monozygotic twins of Congenital Diaphragmatic Hernia (CDH) and Esophageal Atresia (EA) cohorts

European Journal of Human Genetics — Wed, 09 Nov 2011 05:00:00 +0100

Authors: Danielle Veenma, Erwin Brosens, Elisabeth de Jong, Cees van de Ven, Connie Meeussen, Titia Cohen-Overbeek, Marjan Boter, Hubertus Eussen, Hannie Douben, Dick Tibboel & Annelies de Klein (Source: European Journal of Human Genetics)

Possible treatment target found for main cause of severe liver disease in kids

EurekAlert! - Medicine and Health — Tue, 08 Nov 2011 05:00:00 +0100

(Cincinnati Children's Hospital Medical Center) Unexpected discovery of a new molecular signature for a destructive and often lethal pediatric liver disease may lead to a new therapeutic target for the hard-to-treat condition. Researchers report in the November Journal of Clinical Investigation that not all children with biliary atresia share the same disease process. Some patients have a second molecular conductor of disease called Th2 (T helper cell 2) immune system. (Source: EurekAlert! - Medicine and Health)

Longest survivor of pulmonary atresia with ventricular septal defect: well-developed major aortopulmonary collateral arteries demonstrated by multidetector computed tomography.

Circulation — Tue, 08 Nov 2011 05:00:00 +0100

Authors: Fukui D, Kai H, Takeuchi T, Gondo T, Oba T, Mawatari K, Tonai T, Matsuo Y, Ueda S, Niiyama H, Ueno T, Imaizumi T PMID: 22064959 [PubMed - in process] (Source: Circulation)

A newborn with overlapping features of AEC and EEC syndromes

American Journal of Medical Genetics Part A — Mon, 07 Nov 2011 05:00:00 +0100

AbstractEctrodactyly, ectodermal dysplasia, clefting (EEC) syndrome is the prototype of several p63 conditions, which include ankyloblepharon, ectodermal dysplasia, clefting (AEC) syndrome, limb‐mammary syndrome (LMS), Rapp‐Hodgkin syndrome (RHS), ADULT syndrome, and others. All these disorders include combinations of ectodermal dysplasia, orofacial clefting and limb malformations in variable severity. A newborn patient is presented with diffuse erythematous and desquamating skin lesions and anal atresia. She also had sparse and lightly colored thin hair, deeply set eyes, hypoplastic alae nasi, and a short philtrum. Cleft lip/palate and ankyloblepharon were not present. Complete cutaneous syndactyly was present on both hands in between the third and fourth fingers. Mild ectrodactyly wa...

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Pulmonary artery sling: Current results with cardiopulmonary bypass

The Journal of Thoracic and Cardiovascular Surgery — Fri, 04 Nov 2011 04:00:00 +0100

Conclusions: Pulmonary artery sling is best repaired with median sternotomy, cardiopulmonary bypass, and left pulmonary artery reimplantation. This resulted in uniformly patent left pulmonary arteries in all patients. Preoperative computed tomography imaging, echocardiography, and bronchoscopy are essential for precise operative planning. The frequently associated tracheal stenosis is best repaired with slide tracheoplasty. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Laparoscopic Gastric Pull-Up and Thoracoscopic Esophago-Esophagostomy Combined with Intrathoracic Fundoplication for Long-Gap Pure Esophageal Atresia

Journal of Laparoendoscopic — Fri, 04 Nov 2011 03:14:08 +0100

Journal of Laparoendoscopic & Advanced Surgical Techniques , Vol. 0, No. 0. (Source: Journal of Laparoendoscopic)

Medical Management of Chronic Liver Diseases (CLD) in Children (Part II): Focus on the Complications of CLD, and CLD that Require Special Considerations.

Paediatric Drugs — Thu, 03 Nov 2011 11:05:08 +0100

Authors: El-Shabrawi MH, Kamal NM Abstract Treatment of the causes of many chronic liver diseases (CLDs) may not be possible. In this case, complications must be anticipated, prevented or at least controlled by the best available therapeutic modalities. There are three main goals for the management of portal hypertension: (i) prevention of the first episode of variceal bleeding largely by non-selective β-adrenoceptor antagonists, which is not generally recommended in children; (ii) control of bleeding by using a stepwise approach from the least to most invasive strategies; (iii) and prevention of re-bleeding using bypass operations, with particular enthusiasm for the use of meso-Rex bypass in the pediatric population. Hepatic encephalopathy management also consists of three main a...

Thoracoscopic Repair of Esophageal Atresia and Tracheo-Esophageal Fistula in Neonates: Evolution of a Technique

Journal of Laparoendoscopic — Tue, 01 Nov 2011 18:10:23 +0100

Journal of Laparoendoscopic & Advanced Surgical Techniques , Vol. 0, No. 0. (Source: Journal of Laparoendoscopic)

Stool Color Card Screening for Biliary Atresia

PEDIATRICS — Tue, 01 Nov 2011 04:00:00 +0100

CONCLUSION: Stool color card screening seemed to increase parents' and physicians' awareness of biliary atresia. It also was associated with a decline in the proportion of late referral. Thus, screening might be especially effective in areas with high a proportion of late referral. Improvements in the speed of workup and the operation room should be the focus of education and training in the future. (Source: PEDIATRICS)

mTOR kinase inhibition results in oocyte loss characterized by empty follicles in human ovarian cortical strips cultured in vitro

Fertility and Sterility — Tue, 01 Nov 2011 04:00:00 +0100

Conclusion(s): mTOR inhibition results in a conserved destruction of the oocyte by adjacent granulosa cells (GC) in the absence of increased caspase activity. This model of oocyte loss is not consistent with classic apoptosis/atresia. (Source: Fertility and Sterility)

Role of angiotensin in ovarian follicular development and ovulation in mammals: a review of recent advances.

Reproduction — Tue, 01 Nov 2011 04:00:00 +0100

Authors: Gonçalves PB, Ferreira R, Gasperin B, Oliveira JF Abstract Angiotensin (Ang) II is widely known for its role in the control of systemic blood vessels. Moreover, Ang II acts on the vascular control of ovarian function, corpus luteum formation, and luteolysis. Over the past 10 years, our research group has been studying the new concept of the renin-angiotensin system (RAS) as autocrine/paracrine factor regulating steroidogenesis and promoting different cellular responses in the ovary, beyond vascular function. We have developed and used different in vivo and in vitro experimental models to study the role of RAS in the ovary, and a brief overview of our findings is presented here. It is widely accepted that there are marked species difference in RAS function in follicle deve...

Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis. (Source: The Annals of Thoracic Surgery)

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Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review

Liver International — Tue, 01 Nov 2011 04:00:00 +0100

ConclusionsSome patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension. (Source: Liver International)

Predictors of postnatal outcome in neonates with gastroschisis

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusion: Outcome in our patients was favorable as measured by survival, LOS, and days on PN. Primary predictors of poor outcome were factors related to short bowel syndrome and secondary closure, indicating a need to further improve treatment of short bowel syndrome. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Dysphagia is not unusual after repair of esophageal atresia (EA). The lack of a uniform definition has led to a variance when it comes to reporting the prevalence of dysphagia among patients operated on for EA. The authors' aim was to estimate the occurrence and degree of dysphagia, using a numerical score with its statistical versatility independent of a specific definition. The results were used to find early risk factors within this patient group. The results were also used to see whether a correlation between dysphagia and symptoms of gastroesophageal reflux and quality of life could be found. (Source: Journal of Pediatric Surgery)

Late‐onset achalasia after esophageal atresia repair

Diseases of the Esophagus — Tue, 01 Nov 2011 04:00:00 +0100

SUMMARYThe development of achalasia in a patient with a history of esophageal atresia (EA) is rare. Here, we report a patient who had undergone surgery for EA at birth and presented achalasia at 30 years of age. He was successfully treated with laparoscopic surgery. (Source: Diseases of the Esophagus)

Orthotopic liver transplantation for adults with Alagille syndrome

Clinical Transplantation — Tue, 01 Nov 2011 04:00:00 +0100

Conclusion: The outcome of first LT in A‐AGS is excellent compared with the overall reported adult patient and graft survival. Although A‐AGS were sicker than P‐AGS at transplant, their outcomes were comparable with that of P‐AGS. (Source: Clinical Transplantation)

Balloon dilation for management of choanal atresia and stenosis

International Journal of Pediatric Otorhinolaryngology — Tue, 01 Nov 2011 01:32:14 +0100

Conclusions: This is a novel use of high pressure, non-compliant balloons. We found success in dilating membranous stenoses, post-repair granulation, and scar tissue in revision cases. Balloon dilation is an effective adjuvant tool for use in the repair of choanal atresia and stenosis. (Source: International Journal of Pediatric Otorhinolaryngology)

Fas Expression and Mediated Activation of an Apoptosis Programme in Bovine Follicular Granulosa Cells In Vitro

Reproduction in Domestic Animals — Sat, 29 Oct 2011 04:00:00 +0100

This study will aid in further understanding the mechanism of regulation of Fas on bovine oocyte formation and development. (Source: Reproduction in Domestic Animals)

Reappraising the portoenterostomy procedure according to sound physiologic/anatomic principles enhances postoperative jaundice clearance in biliary atresia

Pediatric Surgery International — Fri, 28 Oct 2011 05:49:13 +0100

Conclusions Jaundice clearance following OKOPE would appear to be better than after EPE. Content Type Journal ArticleCategory Original articlePages 1-5DOI 10.1007/s00383-011-3019-9Authors Hiroki Nakamura, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421 JapanHiroyuki Koga, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421 JapanMomoko Wada, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421 JapanGo Miyano, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunk...

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Twelve-year prevalence of common neonatal congenital malformations in Zhejiang Province, China.

World Journal of Pediatrics : WJP — Thu, 27 Oct 2011 12:50:22 +0100

CONCLUSIONS: The incidences of CHD, intestinal atresia/stenosis, kidney malformations, hypospadias, OFC, and accessory auricle anomaly have increased in the last 12 years, but the incidences of anorectal malformations, polydactyly and syndactyly remain stable. The incidence of hydrocephalus shows a downward trend. PMID: 22015725 [PubMed - in process] (Source: World Journal of Pediatrics : WJP)

Toll-Like Receptors in Secondary Obstructive Cholangiopathy

Diagnostic and Therapeutic Endoscopy — Thu, 27 Oct 2011 11:45:29 +0100

Secondary obstructive cholangiopathy is characterized by intra- or extrahepatic bile tract obstruction. Liver inflammation and structural alterations develop due to progressive bile stagnation. Most frequent etiologies are biliary atresia in children, and hepatolithiasis, postcholecystectomy bile duct injury, and biliary primary cirrhosis in adults, which causes chronic biliary cholangitis. Bile ectasia predisposes to multiple pathogens: viral infections in biliary atresia; Gram-positive and/or Gram-negative bacteria cholangitis found in hepatolithiasis and postcholecystectomy bile duct injury. Transmembrane toll-like receptors (TLRs) are activated by virus, bacteria, fungi, and parasite stimuli. Even though TLR-2 and TLR-4 are the most studied receptors related to liver infectious disease...

Pseudodysplastic regenerative mucosa associated with congenital ileal atresia

Pathology International — Thu, 27 Oct 2011 05:34:47 +0100

A male neonate was clinically diagnosed with congenital intestinal atresia. Surgical operation was performed and the ileum including the atretic portion was resected. Grossly, there was a plaque‐like elevation of mucosa at the proximal side of the ileal atresia. Microscopic examination of this lesion revealed proliferation of severely atypical glands. Although dysplasia was a serious diagnostic concern, we concluded that ischemia due to the intestinal atresia induced this benign pseudodysplastic regenerative mucosa, judging from the pattern of coexisting inflammation and the literature review. (Source: Pathology International)

Roles of Reactive Oxygen Species and Antioxidants in Ovarian Toxicity.

Biology of Reproduction — Thu, 27 Oct 2011 04:00:00 +0100

Authors: Devine PJ, Perreault SD, Luderer U Abstract Proper functioning of the ovary is critical to maintain fertility and overall health, and ovarian function depends on the maintenance and normal development of ovarian follicles. This review presents evidence about the potential impact of oxidative stress on the well-being of primordial, growing and preovulatory follicles, as well as oocytes and early embryos, examining cell types and molecular targets. Limited data from genetically modified mouse models suggest that several antioxidant enzymes, which protect cells from reactive oxygen species (ROS), may play important roles in follicular development and/or survival. Exposures to agents known to cause oxidative stress, such as gamma-irradiation, chemotherapeutic drugs, or polycyc...

Carbimazole: Neonatal choanal atresia, tracheoesophageal fistula and clinodactyly following in utero exposure: case report

Reactions — Wed, 26 Oct 2011 13:05:17 +0100

(Source: Reactions)

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Over-Prescription of Acid-Suppressing Medications in Infants: How It Came About, Why It’s Wrong, and What to Do About It

The Journal of Pediatrics — Mon, 24 Oct 2011 04:00:00 +0100

It has been almost 20 years since proton pump inhibitors (PPIs) were initially shown to be effective, safe, and well-tolerated for the short-term treatment of gastroesophageal reflux disease (GERD) in children over 1 year of age; GERD diagnosed on the basis of symptoms and hard diagnostic evidence of erosive esophagitis seen at endoscopy. In these studies, mostly performed in children 2 to 17 years of age, PPIs were shown to effectively treat symptoms and erosive esophagitis that were refractory to histamine-2-receptor antagonists (H2RA), buffering agents, prokinetics, and in some subjects, antireflux surgery. Subsequent to those studies with omeprazole, other PPIs were found to be similarly effective. Efficacy and safety were also shown for maintenance of remission of chronic, relapsing e...

Aspects of esophageal atresia in a population-based setting: incidence, mortality, and cancer risk

Pediatric Surgery International — Fri, 21 Oct 2011 16:01:36 +0100

Conclusions EA has a stable incidence, the survival has improved substantially during recent decades, and the cancer risk might not be increased. Content Type Journal ArticleCategory Original ArticlePages 1-9DOI 10.1007/s00383-011-3014-1Authors Jenny Oddsberg, Upper Gastrointestinal Research, Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, SwedenYunxia Lu, Upper Gastrointestinal Research, Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, SwedenJesper Lagergren, Upper Gastrointestinal Research, Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, Sweden Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric S...

Is complex gastroschisis predictable by prenatal ultrasound?

BJOG: An International Journal of Obstetrics and Gynaecology — Fri, 21 Oct 2011 04:00:00 +0100

Conclusions Intra‐abdominal bowel dilatation is the only ultrasound marker predictive of complex GS but it is a strong marker. (Source: BJOG: An International Journal of Obstetrics and Gynaecology)

A gallstone in the Roux-en Y limb during pregnancy in a female patient with biliary atresia

Pediatric Surgery International — Thu, 20 Oct 2011 15:52:58 +0100

Abstract A 28-year-old female developed liver dysfunction at the 13th gestation week of her second pregnancy. A postpartum radiological examination revealed a stone in the Roux-en Y (RY) limb. She underwent surgical removal of the gallstone in the RY limb. Bile congestion during pregnancy led to the development of a gallstone. Content Type Journal ArticleCategory Case ReportPages 1-3DOI 10.1007/s00383-011-2998-xAuthors Masayuki Obatake, Department of Pediatric Surgery, Nagasaki University Hospital, 1-7-1 Sakamoto, Nagasaki-shi, Nagasaki, 852-8501 JapanYasuaki Taura, Department of Pediatric Surgery, Nagasaki University Hospital, 1-7-1 Sakamoto, Nagasaki-shi, Nagasaki, 852-8501 JapanKyoko Mochizuki, Department of Pediatric Surgery, Nagasaki University Hospital, 1-7-1 Saka...

Otologic and Audiology Aspects of Microtia Repair

Seminars in Plastic Surgery — Thu, 20 Oct 2011 04:00:00 +0100

Seminars in Plastic Surgery 2011; 25: 273-278DOI: 10.1055/s-0031-1288919ABSTRACTCongenital abnormalities of the outer ear pose a reconstructive challenge for plastic surgeons and otologists. Many patients with microtia of the auricle have concurrent atresia of the external auditory canal. The hearing loss associated with canal atresia can have long-lasting effects if not treated promptly and appropriately. The diagnosis and workup for canal atresia requires an otologic evaluation. Audiologic and radiologic evaluations direct treatment, which varies depending on unilateral or bilateral presence of atresia. Rehabilitation of hearing loss can be performed via hearing aids, bone-anchored conductive devices or canalplasty. Due to the complexity of treatments, communication between the reconstru...

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Th2 signals induce epithelial injury in mice and are compatible with the biliary atresia phenotype

Journal of Clinical Investigation — Wed, 19 Oct 2011 17:53:30 +0100

Biliary atresia (BA) is a destructive cholangiopathy of childhood in which Th1 immunity has been mechanistically linked to the bile duct inflammation and obstruction that culminate in liver injury. Based on reports of decreased Th1 cytokines in some patients and the development of BA in mice lacking CD4+ T cells, we hypothesized that Th1-independent mechanisms can also activate effector cells and induce BA. Here, we tested this hypothesis using Stat1–/– mice, which lack the ability to mount Th1 immune responses. Infection of Stat1–/– mice with rhesus rotavirus type A (RRV) on postnatal day 1 induced a prominent Th2 response, duct epithelial injury and obstruction within 7 days, and atresia shortly thereafter. A high degree of phosphorylation of the Th2 trans...

Glycomic analyses of ovarian follicles during development and atresia.

Matrix Biology — Wed, 19 Oct 2011 04:00:00 +0100

Authors: Hatzirodos N, Nigro J, Irving-Rodgers HF, Vashi AV, Hummitzsch K, Caterson B, Sullivan TR, Rodgers RJ Abstract To examine the detailed composition of glycosaminoglycans during bovine ovarian follicular development and atresia, the specialized stromal theca layers were separated from the stratified epithelial granulosa cells of healthy (n=6) and atretic (n=6) follicles in each of three size ranges: small (3-5mm), medium (6-9mm) and large (10mm or more) (n=29 animals). Fluorophore-assisted carbohydrate electrophoresis analyses (on a per cell basis) and immunohistochemistry (n=14) were undertaken. We identified the major disaccharides in thecal layers and the membrana granulosa as chondroitin sulfate-derived ∆uronic acid with 4-sulfated N-acetylgalactosamine and ∆uronic a...

The utility of muscle sparing axillar skin crease incision for pediatric thoracic surgery

Pediatric Surgery International — Tue, 18 Oct 2011 15:56:03 +0100

Conclusions MSASCI may become the standard approach for the thoracic surgery for small children. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s00383-011-3013-2Authors Tomoaki Taguchi, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanKouji Nagata, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanYoshiaki Kinoshita, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSatoshi Ieiri, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanTatsuro Tajiri, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, F...

Amino acid transport system L activity in developing mouse ovarian follicles

Human Reproduction — Tue, 18 Oct 2011 04:00:00 +0100

CONCLUSIONS These results demonstrate, for the first time, evidence of specific system L amino acid transport in intact, mouse ovarian follicles and profile L-leucine uptake during folliculogenesis. A better understanding of ovarian follicle metabolic pathways is necessary for improved in vitro maturation as well as determining the impact of altered metabolism on fertility. (Source: Human Reproduction)

Angiotensin II Signaling Promotes Follicle Growth and Dominance in Cattle.

Endocrinology — Tue, 18 Oct 2011 04:00:00 +0100

In conclusion, these data suggest that AngII signaling promotes follicle growth in cattle and does so by regulating genes involved in estradiol secretion and granulosa cell proliferation and differentiation. PMID: 22009728 [PubMed - as supplied by publisher] (Source: Endocrinology)

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Endoscopic balloon dilatation of esophageal strictures in children

International Journal of Pediatric Otorhinolaryngology — Sat, 15 Oct 2011 08:22:27 +0100

Conclusions: Endoscopic balloon dilatation is a simple, safe and efficacious treatment of esophageal strictures in children. (Source: International Journal of Pediatric Otorhinolaryngology)

Evidence that severe acute stress and starvation induce rapid atresia of ovarian vitellogenic follicles in Atlantic bluefin tuna, Thunnus thynnus (L.) (Osteichthyes: Scombridae)

Journal of Fish Diseases — Thu, 13 Oct 2011 18:10:28 +0100

AbstractThe effects of different stressors on the atretic degeneration of ovarian vitellogenic follicles, as well as on the ovarian mass, were examined in female Atlantic bluefin tuna, Thunnus thynnus (L.), from the Mediterranean Sea. The stressors taken into consideration were short‐term starvation (up to 14 days), long‐term cage rearing (1 year) and crowding‐induced severe panic frenzy. Wild‐caught individuals were used as a control group. Fish subjected to either severe panic frenzy or starvation exhibited a decrease in gonad mass and had significantly higher intensity of α atresia in the vitellogenic follicles (means: 78% and 58%, respectively; range: 36–100%) than either wild or long‐term caged individuals (means: 32% and 30%, respectively; range: 19–44%). The exten...

Efficacy of US-guided Percutaneous Cholecystocholangiography for the Early Exclusion and Type Determination of Biliary Atresia [Pediatric Imaging]

Continuous Publishing articles — Thu, 13 Oct 2011 04:00:00 +0100

Percutaneous cholecystocholangiography is a safe and accurate technique for early exclusion of biliary atresia in infants with cholestatic liver disease by established testing modalities. (Source: Continuous Publishing articles)

Coronary sinus ostial atresia with persistent left superior vena cava demonstrated on cardiac CT in an infant with a functional single ventricle

Pediatric Radiology — Wed, 12 Oct 2011 16:32:23 +0100

We report an infant with functional single ventricle in whom CSOA and PLSVC were successfully demonstrated on preoperative cardiac CT. Content Type Journal ArticleCategory Case ReportPages 1-3DOI 10.1007/s00247-011-2258-1Authors Cherry Kim, Asan Medical Center, Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, KoreaHyun Woo Goo, Asan Medical Center, Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, KoreaJeong Jin Yu, Department of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South KoreaTae-Jin Yun, Department of Pediatric Cardiac Surgery,...

Identification of Soluble Forms of Vascular Endothelial Growth Factor receptors, sVEGFR‐1 and sVEGFR‐2, in Bovine Dominant Follicles

Reproduction in Domestic Animals — Wed, 12 Oct 2011 04:00:00 +0100

ContentsThe development of dominant follicles requires the parallel growth of a vascular network, regulated by VEGF and its receptors VEGFR‐1 and VEGFR‐2. Here, we demonstrate the presence of mRNA for the soluble forms of VEGFR‐1 and VEGFR‐2 by RT‐PCR and the respective proteins by Western blot, in bovine dominant follicles. The 3′ end of the mRNA coding region and the deduced C‐terminal amino acid sequence of the bovine VEGFR soluble forms were similar to those previously described in human and mice. The relative abundance of sVEGFR‐1 was higher in dominant follicles of day 4, decreasing on day 6 and further on day 9 of the cycle. In contrast, sVEGFR‐2 expression was low on day 4 follicles and increased as the cycle advanced, becoming greater on day 9. The changes of sVE...

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“Parenteral nutrition supplementation in biliary atresia patients listed for liver transplantation”

Liver Transplantation — Mon, 10 Oct 2011 04:00:00 +0100

Conclusions:PN improves nutritional status in malnourished BA patients awaiting LTx, which is associated with post‐LTx outcomes comparable to those not requiring PN. Liver Transpl, 2011. © 2011 AASLD. (Source: Liver Transplantation)

Anesthetic management of congenital tracheoesophageal fistula.

Paediatric Anaesthesia — Sun, 09 Oct 2011 19:19:08 +0100

This article reviews (a) risk factors and preoperative considerations of the patient with tracheoesophageal fistula, (b) anesthetic management, including (i) airway management, (ii) induction of anesthesia and monitoring and (iii) postoperative disposition, (c) considerations for concomitant congenital heart disease, (d) considerations for thoracoscopic repair and (e) long-term outcomes and considerations of the patient with repaired esophageal atresia/tracheoesophageal fistula. PMID: 20723095 [PubMed - in process] (Source: Paediatric Anaesthesia)

Congenital aural atresia surgery: Transmastoid approach, complications and outcomes

European Archives of Oto-Rhino-Laryngology — Sat, 08 Oct 2011 15:44:55 +0100

Abstract Repair of complete congenital aural atresia (CAA) could be a challenging procedure due to complications reported with CAA surgery such as facial nerve palsy, canal stenosis, graft lateralization, sensorineural hearing loss or the difficulty involved in the surgical technique. From 2006 to 2009, we used a one stage-modified transmastoid approach for surgical repair of 33 ears with complete CAA via a non-randomized controlled clinical trial. Some modifications in the technique of mastoidectomy, ossiculoplasty, fascia and skin grafting and meatoplasty have been described. Patients were followed up for 12 months to assess audiometric results and post-operative complications. Changes in air-bone gap and need for revision surgery or hearing aids were assessed at fo...

Gene expression in swine granulosa cells and ovarian tissue during the estrous cycle.

Genetics and Molecular Research — Fri, 07 Oct 2011 19:20:02 +0100

Gene expression in swine granulosa cells and ovarian tissue during the estrous cycle. Genet Mol Res. 2011;10(3):2258-67 Authors: Silva PV, Guimarães SE, Guimarães JD, Neto JB, Lopes PS, do Nascimento CS, de Campos CF, Weller MM, Botelho ME, Faria VR Abstract The components of the insulin-like growth factor (IGF) system appear to be involved in regulation of ovarian follicular growth and atresia in the pig. We investigated the expression pattern of mRNAs for IGF1 (IGF1), its binding proteins (IGFBP1, IGFBP2, IGFBP3, and IGFBP5), and epidermal growth factor in swine follicle cells and ovarian tissue throughout the estrous cycle using the real-time quantitative PCR technique. The results of gene expression were analyzed using linear regression with gene expression as a depen...

Neonatal exposure to single doses of estradiol or testosterone programs ovarian follicular development–modified hypothalamic neurotransmitters and causes polycystic ovary during adulthood in the rat

Fertility and Sterility — Fri, 07 Oct 2011 04:00:00 +0100

Conclusion(s): Neonatal exposure to EV activated the reproductive hypothalamus and permanently modified ovarian follicular development. TP exposure had some similar effects as EV at the hypothalamus, and it modified ovarian development mimicking the effects of EV. This last effect could be through TP conversion to estradiol because DHT, a nonaromatizable androgen, did not modify follicular development. (Source: Fertility and Sterility)

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Pulmonary atresia with intact ventricular septum and diastolic liver expansion

Heart — Thu, 06 Oct 2011 04:00:00 +0100

An 18-year-old woman with pulmonary atresia and intact ventricular septum palliated by classic Glenn anastomosis and right ventricle to left pulmonary artery opening was referred to our tertiary centre for the assessment of severe tricuspid regurgitation. The patient presented with a history of palpitations and tenderness of the right hypochondrium. The liver was enlarged, pulsatile and tender on palpation. Surprisingly, the hepatic expansion was noted to be asynchronous with the radial pulse. On transthoracic echocardiogram and cardiac MRI, the right ventricle was small and heavily hypertrophied (panel A). The right atrium, inferior vena cava and hepatic veins were markedly dilated (panels A and B). There was no significant tricuspid stenosis or regurgitation. During atrial contraction, t...

X-linked inhibitor of apoptosis protein (XIAP) and active caspase-3 expression patterns in antral follicles in the sheep ovary.

Reproduction — Wed, 05 Oct 2011 04:00:00 +0100

Authors: Phillipps HR, Kokay IC, Grattan DR, Hurst PR Abstract X-linked inhibitor of apoptosis protein (XIAP) interacts with caspases to inhibit their activity, thereby providing a potential mechanism for regulation of granulosa apoptosis occurring during follicular atresia. The aims of the study were to determine the presence and localization of XIAP mRNA and protein content in the sheep ovary and compare these expression patterns with active caspase-3 protein in the same antral follicles. Romney ewe estrous cycles (N=25) were synchronized with 2-3 Estrumate injections and ovarian tissue collected during the luteal and follicular phases of the cycle. The presence of XIAP mRNA was confirmed by RT-PCR using laser capture microdissected ovarian cell samples. XIAP mRNA was subsequentl...

A Large Coronary‐Pulmonary Artery Fistula in a Cyanotic Patient Leading to Severe Biventricular Dysfunction and Heart Failure

Echocardiography — Sat, 01 Oct 2011 04:00:00 +0100

We report a 17‐year‐old male with PA with VSD and a large CPAF from the anterior right aortic sinus connecting to the left pulmonary artery, who presented with severe CHF. The left anterior descending and the right coronary artery both arose from the proximal part of the CPAF, possibly leading to extensive coronary steal and biventricular dysfunction (Echocardiography, ****;**:E1‐E6) (Source: Echocardiography)

Indications for neonatal and pediatric distraction

International Journal of Oral and Maxillofacial Surgery — Sat, 01 Oct 2011 04:00:00 +0100

The neonate with upper airway obstruction secondary to micrognathia can be treated with non-operative therapy or surgical intervention. Patients with severe obstructive sleep apnea (OSA) and/or those who have failed alternative measures have historically been managed with a tracheostomy. Although tracheostomy may be life saving, it is associated with high complication rates and can lead to developmental delays. More recently, mandibular distraction osteogenesis (DO) has been used to treat neonates with OSA who have airway obstruction at the tongue base. However, for this treatment to be successful, it is importance that there are no other sites of obstruction (e.g., laryngotracheomalacia, choanal atresia, etc.). In addition, infants with complex syndromes do not consistently have complete ...

Evaluation of esophageal motility and reflux in children treated for congenital diaphragmatic hernia with the use of combined multichannel intraluminal impedance and pH monitoring

Journal of Pediatric Surgery — Sat, 01 Oct 2011 04:00:00 +0100

Conclusions: With the use of pH-multichannel intraluminal impedance, both GER and esophageal motility in patients with congenital malformations can be analyzed. In patients with CDH, impaired motility seems to involve only the distal esophagus. In this group, the specific pattern of reflux is probably caused by the involvement of gastroesophageal junction, without significant intrinsic innervation abnormalities as observed in patients with esophageal atresia. (Source: Journal of Pediatric Surgery)

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Esophageal atresia with distal fistula and long overlapping upper esophageal pouch

Journal of Pediatric Surgery — Sat, 01 Oct 2011 04:00:00 +0100

Esophageal atresia (EA) with tracheoesophageal fistula (TEF) (EA-TEF) is one of the common neonatal anomalies often presenting with excessive salivation, and inability to feed or pass a feeding tube into the stomach . The present report describes an unusual case of EA-TEF with a long upper esophageal pouch that crossed over the lower esophagus allowing the passage of an infant feeding tube (IFT) to a sufficient distance to give the false impression of the tube passed into the stomach. (Source: Journal of Pediatric Surgery)

Pyloric atresia associated with Dieulafoy lesion and gastric dysmotility in a neonate

Journal of Pediatric Surgery — Sat, 01 Oct 2011 04:00:00 +0100

Abstract: In this case study, we report a male infant with pyloric atresia, extreme gastric distension, and a caliber-persistent gastric artery (Dieulafoy lesion) with massive gastric bleeding. After a transverse pyloroplasty and endoscopic hemoclip application to the caliber-persistent gastric artery, very slow gastric emptying developed, which required repeated surgical interventions. Gastroduodenostomy failed to promote gastric emptying. The intraoperative and postmortem histologic examinations of the gastric wall revealed a loss of interstitial cells of Cajal, which possibly explains the extreme motility disorder. (Source: Journal of Pediatric Surgery)

Prosthetic valve implantation with preservation of the entire valvular and subvalvular apparatus of the tricuspid valve in congenitally corrected transposition of the great arteries.

The Heart Surgery Forum — Sat, 01 Oct 2011 04:00:00 +0100

We present a patient with congenitally corrected transposition of the great arteries who underwent surgical replacement of the tricuspid valve with a bioprosthesis and reconstruction of the left ventricular outflow tract with bovine conduit. PMID: 21997658 [PubMed - in process] (Source: The Heart Surgery Forum)

Transaminase on Rotavirus Gastroenteritis

Pediatrics International — Sat, 01 Oct 2011 04:00:00 +0100

Conclusion: From this data, rotavirus infection is occasionally accompanied with hepatitis, but only in a mild form, and does not correlate with neurological complications. High levels of transaminase might reflect high IL‐6. (Source: Pediatrics International)

Long-term results of esophageal atresia: Helsinki experience and review of literature

Pediatric Surgery International — Fri, 30 Sep 2011 05:50:03 +0100

Abstract Esophageal atresia (EA) affects one in 2,840 newborns, and over half have associated anomalies that typically affect the midline. After EA repair in infancy, gastroesophageal reflux (GER) and esophageal dysmotility and respiratory problems are common. Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, six cases of esophageal cancer have been reported in young adults treated for EA. According to our data, the statistical risk for esophageal cancer is not higher than 500-fold that of the general population. However, the overall cancer incidence among adults with repaired EA does not...

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Twenty‐Five–Year survival of children with birth defects in New York State: A population‐based study

Birth Defects Research Part A: Clinical and Molecular Teratology — Fri, 30 Sep 2011 04:00:00 +0100

CONCLUSION: Using the statewide, population‐based birth defects surveillance data in New York State, the survival experience of the study cohort was examined across all survival time periods by individual birth defect of interest. Several risk factors that affect survival were identified. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Decreasing Rate of Biliary Atresia in Taiwan: A Survey, 2004-2009

PEDIATRICS — Fri, 30 Sep 2011 04:00:00 +0100

Biliary atresia (BA) is the leading cause of liver death and liver transplantation in the pediatric age group. The pathogenesis of BA remains unclear, but epidemiological studies may enhance our understanding of the possible causes. Decreasing rates of BA have been found in Taiwan since 2007, which may be related to improvements in general socioeconomic status and possibly the popularity of rotavirus vaccination; this may shed light on possible preventive interventions for BA. (Read the full article) (Source: PEDIATRICS)

Clinical characterization of a newly described neonatal diabetes syndrome caused by RFX6 mutations

American Journal of Medical Genetics Part A — Fri, 30 Sep 2011 04:00:00 +0100

We report on a new case due to a novel homozygous splice site mutation and update on the clinical outcome of a previously reported patient. In addition we review the clinical and molecular features of all RFX6 mutated cases to better characterize the syndrome. Our results suggest that despite the early postnatal fulminant course, patients who survive may expect a relatively favorable prognosis. © 2011 Wiley Periodicals, Inc. (Source: American Journal of Medical Genetics Part A)

The Right-sided Aortic Arch in Children with Oesophageal Atresia and Tracheo-oesophageal Fistula

European Journal of Pediatric Surgery — Thu, 29 Sep 2011 04:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1285906A right-sided aortic arch (RAA) occurs in around 5% of patients with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF). This anatomical variation can complicate the operative management of these patients, as it is often not diagnosed preoperatively but only discovered at thoracotomy, and it remains unproven as to whether a right or left thoracotomy is the best operative approach. This retrospective study aimed to determine the prevalence of RAA in OA/TOF, review the accuracy of preoperative investigations, and investigate the best operative approach, by reviewing the literature and our own patient series.The case notes of all infants with OA/TOF over a 15 year period (1994–2008) were retrospectively analysed to identify those with a...

Side-to-Side Aorto-GoreTex Central Shunt Warrants Central Shunt Patency and Pulmonary Arteries Growth [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 28 Sep 2011 04:00:00 +0100

Conclusions Central shunts can be performed in neonates and children with minimal risk of shunt occlusion. Side-to-side aorto-Gore-Tex anastomosis seems to reliably warrant shunt patency and harmonious growth of pulmonary arteries. (Source: The Annals of Thoracic Surgery)

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Predictors of re‐intervention in neonates with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum

Catheterization and Cardiovascular Interventions — Tue, 27 Sep 2011 04:00:00 +0100

Conclusion:Interventional cardiac catheterization shows favorable outcome for critical PS and PA/IVS patients. Predictors for re‐intervention included the diagnosis of PA/IVS, hospital stay >7.5 days after first intervention and TR1 gradient > 43mmHg. © 2011 Wiley‐Liss, Inc. (Source: Catheterization and Cardiovascular Interventions)

Predictors of reintervention in neonates with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum

Catheterization and Cardiovascular Interventions — Tue, 27 Sep 2011 04:00:00 +0100

Conclusion: Interventional cardiac catheterization shows favorable outcome for patients with critical PS and PA/IVS. Predictors for reintervention included the diagnosis of PA/IVS, hospital stay ≥7.5 days after first intervention and TR1 gradient ≥ 43 mm Hg. © 2011 Wiley Periodicals, Inc. (Source: Catheterization and Cardiovascular Interventions)

New technique of off-pump atrial septostomy for complex congenital cardiac anomalies [Original articles]

European Journal of Cardio-Thoracic Surgery — Mon, 26 Sep 2011 04:00:00 +0100

Conclusions: This technique demonstrates the surgical feasibility of a beating-heart atrial septostomy. It avoids the adverse effects of cardiopulmonary bypass, reduces morbidity, and has no mortality. It is safe, economical, and easily reproducible. To our knowledge, this technique has not been reported in literature so far. (Source: European Journal of Cardio-Thoracic Surgery)

Should we always plan a Fontan completion after a Kawashima procedure? [Original articles]

European Journal of Cardio-Thoracic Surgery — Mon, 26 Sep 2011 04:00:00 +0100

Conclusion: Unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure will ultimately develop pulmonary arteriovenous malformations. Elective non-fenestrated Fontan completion in the years following Kawashima procedure should be recommended. (Source: European Journal of Cardio-Thoracic Surgery)