MedWorm: Atresia

Echocardiographic determinants of successful balloon dilation in pulmonary atresia with intact ventricular septum

European Journal of Echocardiography — Thu, 11 Mar 2010 09:46:28 +0100

Conclusion Successful balloon dilation in membranous type PA–IVS can be predicted by a scoring system using RV/LV diameter ratio and RV/LV length ratio. (Source: European Journal of Echocardiography)

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Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

Pediatric Radiology — Thu, 11 Mar 2010 02:38:04 +0100

Conclusion Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00247-010-1603-0Authors Hye Jin Yoo, Seoul National University College of Medicine and the Institute of Radiation Medicine Department of Radiology 101 Daehangno, Jongno-Gu Seoul 110-744 South KoreaWoo Sun Kim, Seoul National University College of Medicine and the Institute of Radiation Medicine Department of Radiology 101 Daehangno, Jongno-Gu Seoul 110-744 South KoreaJung-Eun Cheon, Seoul National University College of Medicine and the Institute of Radiation Medicine Department of Radiology 101 Daehang...

Live Webcast: Surgery for Esophageal Atresia

What's New on MedlinePlus — Tue, 09 Mar 2010 15:28:02 +0100

Watch a live webcast of surgery for esophageal atresia (a problem in infants) on March 11 at 5:00 p.m. EST. Also, browse through links to prerecorded webcasts of surgical procedures. These are actual procedures performed at medical centers in the United States. (Source: What's New on MedlinePlus)

Thoracoscopic Repair of Esophageal Atresia Through the Right Chest in Neonates with Right-Sided Aortic Arch

Journal of Laparoendoscopic — Tue, 09 Mar 2010 05:02:10 +0100

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

Congenital Left Main Coronary Artery to Main Pulmonary Artery Fistula with Bicuspid Aortic Valve: A Case Report and Review of Literature

Journal of Cardiac Surgery — Mon, 08 Mar 2010 00:00:00 +0100

We report on a 68-year-old man with congenital left main to pulmonary artery fistula associated with bicuspid aortic valve and moderate aortic stenoses, who underwent successful aortic valve replacement with ligation of CAF and also review the natural history, pathophysiology, and management of CAF. (J Card Surg ****;**:**-**) (Source: Journal of Cardiac Surgery)

Mural granulosa cell gene expression associated with oocyte developmental competence

Journal of Ovarian Research — Sat, 06 Mar 2010 00:00:00 +0100

Conclusions: The data in the present study demonstrate a close association between specific gene expression in mural granulosa cells and the developmental competence of oocytes. This finding suggests that the most differentially expressed gene, lysyl oxidase, may be a candidate biomarker of oocyte health and useful for the selection of good quality oocytes for assisted reproduction. (Source: Journal of Ovarian Research)

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The 'question mark' sign as a new ultrasound marker of tetralogy of Fallot in the fetus.

The Ultrasound Review of Obstetrics and Gynecology — Thu, 04 Mar 2010 00:00:00 +0100

CONCLUSION: : The finding of an enlarged aorta with a marked '?' shape should raise a strong suspicion of tetralogy of Fallot, in particular the variant with pulmonary atresia. This sign may be useful in screening considering that prenatal diagnosis of Fallot's tetralogy in routine ultrasound remains a challenge. Copyright (c) 2010 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 20205151 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Balloon Valvuloplasty for Neonatal Critical Pulmonary Valvar Stenosis with IVC Interruption: Pitfalls of the Transumbilical Approach.

J Korean Med Sci — Mon, 01 Mar 2010 00:00:00 +0100

We report herein the case of a newborn baby with critical PVS with inferior vena cava interruption, severe tricuspid regurgitation and a severely enlarged right atrium. We tried BPV through the transumbilical approach with difficulty, but he was successfully treated with the assistance of a coronary artery guiding catheter. PMID: 20191053 [PubMed - in process] (Source: J Korean Med Sci)

Neuromuscular complication after liver transplant in children: a single-center experience.

Mon, 01 Mar 2010 00:00:00 +0100

Conclusions: We conclude that the most-common neurologic complication after liver transplant in children in contrast to other studies is tremor, same as adult patients. This may be due to higher rate of use of tacrolimus in our patients. PMID: 20199365 [PubMed - in process] (Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation)

A new rat model of prenatal bowel obstruction: development and early assessment

Journal of Pediatric Surgery — Mon, 01 Mar 2010 00:00:00 +0100

Conclusions: We presented in this study a reproducible model of prenatal intestinal obstruction in the rat with the macroscopical and histologic features of prenatal intestinal obstruction. (Source: Journal of Pediatric Surgery)

Congenital pouch colon with rectal atresia: a case report

Journal of Pediatric Surgery — Mon, 01 Mar 2010 00:00:00 +0100

We describe the third such case and the second instance to survive. Although the prior survivor was managed by a single-stage procedure, we successfully managed our case by staged procedures. In this case report, we discuss the etiology and surgical options available for this rare condition. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Mon, 01 Mar 2010 00:00:00 +0100

Recurrent tracheoesophageal fistula (RTF) complicates 5% to 11% of cases of children treated at birth for esophageal atresia with inferior tracheoesophageal fistula, and it represents an important problem of respiratory morbidity and mortality. The aim of this paper is to demonstrate the usefulness of large vascularized pleural flaps in the treatment of RTF and the prevention of recurrences. (Source: Journal of Pediatric Surgery)

Journal of Pediatric Surgery — Mon, 01 Mar 2010 00:00:00 +0100

Biliary atresia (BA) is one of the most serious liver disorders in children. The purpose of the presented study was to investigate serum levels of bone morphogenetic protein 7 (BMP7) in BA children compared with healthy controls and to evaluate the association between serum BMP7 and the clinical outcome of BA patients post Kasai operation. Sixty-two BA patients post Kasai operation and 14 healthy controls were enrolled in the study. The patients were divided into 2 groups according to their serum total bilirubin levels (no jaundice, 2 mg/dL) and alanine aminotransferase (ALT) levels (normal ALT, 45 IU/L). Serum BMP7 levels were determined by commercial enzyme-linked immunosorbent assay. The mean serum BMP7 was higher in BA patients as compared with healthy controls (P = .002). The BA patie...

Heterotopic pancreas of the esophagus and stomach associated with pure esophageal atresia

Journal of Pediatric Surgery — Mon, 01 Mar 2010 00:00:00 +0100

Abstract: Esophageal atresia with or without tracheoeosphageal fistula is a frequent congenital malformation that may be associated with other congenital anomalies. The combination of heterotopic pancreas of the esophagus and pure esophageal atresia, however, is rare. We treated a patient with esophageal atresia without tracheoesophageal fistula (type A) associated with heterotopic pancreas of the esophagus and stomach who developed a gastric perforation. (Source: Journal of Pediatric Surgery)

Sutureless rescue of a severely hypoplastic pulmonary artery [Case reports]

European Journal of Cardio-Thoracic Surgery — Fri, 26 Feb 2010 22:01:57 +0100

The management of severely hypoplastic pulmonary arteries in the adolescent or adult with congenital heart disease is challenging. A patient with pulmonary atresia and ventricular septal defect (PA-VSD) presented with severe cyanosis due to a kinked and thrombosed right ventricle to pulmonary artery conduit. Retrograde wedge angiography showed a patent right and a diminutive left pulmonary artery deep in the lung hilus. Treatment options were limited and therefore a different strategy had to be considered to rescue the lung segments. This case report describes the novel use of a covered stent to successfully obtain a sutureless connection between a vascular graft and a diminutive pulmonary artery during a hybrid procedure. (Source: European Journal of Cardio-Thoracic Surgery)

Cholestatic Liver Disease in Children

Current Gastroenterology Reports — Thu, 25 Feb 2010 17:58:51 +0100

Abstract Inherited syndromes of intrahepatic cholestasis and biliary atresia are the most common causes of chronic liver disease and the prime indication for liver transplantation in children. Our understanding of the pathogenesis of these diseases has increased substantially by the discovery of genetic mutations in children with intrahepatic cholestasis and the findings that inflammatory circuits are operative at the time of diagnosis of biliary atresia. Building on this solid foundation, recent studies provide new insight into genotype-phenotype relationships and how mutations produce altered bile composition and cholestasis. New evidence exists that although liver transplantation is curative for patients with end-stage liver disease owing to cholestasis, some patients m...

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Mesenchymal expression of Tbx4 gene is not altered in Adriamycin mouse model

Pediatric Surgery International — Thu, 25 Feb 2010 06:51:44 +0100

Conclusion Although previously reported morphological abnormalities of notochord and esophagus were observed in AMM, Tbx4 gene expression was unaltered, suggesting that esophageal anomalies can occur in the presence of normal Tbx4 gene expression in this model. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00383-010-2580-yAuthors Piotr Hajduk, Our Lady’s Children’s Hospital Children’s Research Centre Crumlin Dublin 12 IrelandPaula Murphy, University of Dublin Department of Zoology, Trinity College Dublin IrelandPrem Puri, Our Lady’s Children’s Hospital Children’s Research Centre Crumlin Dublin 12 Ireland Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

The benefit of stay sutures during thoracoscopic esophagoesophagostomy in patients with esophageal atresia: a technical report

Pediatric Surgery International — Thu, 25 Feb 2010 06:51:41 +0100

We report on technical modifications we developed for thoracoscopic esophagoesophagostomy in patients with esophageal atresia. They are: (1) placing stay sutures along the edges of the atretic esophagi and exteriorizing them through the thoracic wall to expose the luminal surfaces of the esophagi nicely and relieve tension on the anastomosis; (2) leaving 1/5 of the length of the distal and proximal ends of the atretic esophagi intact before placing the stay sutures, to avoid retraction of the mucosa into the lumen; (3) making the diameter of the proximal esophagus 1.5–2 times larger than the diameter of the distal esophagus to make the shape of the anastomosis more streamlined without notching. Content Type Journal ArticleCategory Technical InnovationDOI 10.1007/s00383-010-2560...

[First trimester fetal cardiac scanning for fetuses at higher risk for congenital heart disease.]

Gynecologie, Obstetrique & Fertilite — Thu, 25 Feb 2010 00:00:00 +0100

CONCLUSION: Our preliminary results confirmed that fetal heart scanning is feasible at the end of the first trimester of pregnancy. Severe CHD are amenable to prenatal diagnosis in the first trimester in the population at higher risk for CHD. PMID: 20189436 [PubMed - as supplied by publisher] (Source: Gynecologie, Obstetrique & Fertilite)

Modified Dynamic Gracilis Neosphincter for Fecal Incontinence: An Analysis of Functional Outcome at a Single Institution

World Journal of Surgery — Wed, 24 Feb 2010 06:47:42 +0100

Conclusions A modified dynamic gracilis neoanal sphincter for end-stage fecal incontinence helps restore and sustain continence with improvement in quality of life in the majority of patients. The procedure was most effective as augmentation in those who had suffered a traumatic injury, when compared with patients with congenital atresia and sepsis that had resulted in loss of the native anal sphincter. Content Type Journal ArticleDOI 10.1007/s00268-010-0489-1Authors M. Z. M. Hassan, International Islamic University Department of Surgery, Faculty of Medicine Kuantan MalaysiaM. M. G. Rathnayaka, University of Kelaniya Department of Surgery, Faculty of Medicine PO Box 6 Thalagolla Road Ragama Sri LankaK. I. Deen, University of Kelaniya Department of Surgery, Faculty ...

Symptomatic Neonatal Tetralogy of Fallot: Repair or Shunt? [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 23 Feb 2010 22:46:26 +0100

Conclusions Shunting or primary repair of neonates with symptomatic TOF provides equivalent mortality and results. Shunted patients had fewer transannular patch repairs despite having more emergent initial operations. Compared with the primary repair group, shunted patients had decreased intensive care unit and hospital stays for the first hospitalization, which were neutralized when the second operation (repair) values were added. (Source: The Annals of Thoracic Surgery)

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Kawashima Procedure After Staged Unifocalizations in Asplenia With Major Aortopulmonary Collateral Arteries [CASE REPORTS]

The Annals of Thoracic Surgery — Tue, 23 Feb 2010 22:46:26 +0100

We report a Kawashima procedure (total cavopulmonary shunt) successfully carried out for asplenia syndrome, pulmonary atresia, and major aortopulmonary collateral arteries. At the age of 8, the patient underwent staged bilateral unifocalizations using confluent central pulmonary arteries concomitant with bilateral modified Blalock-Taussig shunts. As the result of an interrupted inferior vena cava with azygous continuation, the patient required a Kawashima procedure with augmentation of the central pulmonary arteries for definitive palliation 1 year later. Cyanosis, respiratory distress, and ventricular function improved. (Source: The Annals of Thoracic Surgery)

Symptomatic Neonatal Tetralogy of Fallot: Repair or Shunt?

The Annals of Thoracic Surgery — Tue, 23 Feb 2010 13:10:33 +0100

CONCLUSIONS: Shunting or primary repair of neonates with symptomatic TOF provides equivalent mortality and results. Shunted patients had fewer transannular patch repairs despite having more emergent initial operations. Compared with the primary repair group, shunted patients had decreased intensive care unit and hospital stays for the first hospitalization, which were neutralized when the second operation (repair) values were added. PMID: 20172143 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Kawashima Procedure After Staged Unifocalizations in Asplenia With Major Aortopulmonary Collateral Arteries.

The Annals of Thoracic Surgery — Tue, 23 Feb 2010 13:09:08 +0100

Intestinal Atresia, Encephalocele, and Cardiac Malformations in Infants with 47,XXX: Expansion of the Phenotypic Spectrum and a Review of the Literature

Karger Publishers — Mon, 15 Feb 2010 23:00:00 +0100

Fetal Diagn Ther (DOI:10.1159/000284929) (Source: Karger Publishers)

KTP laser: An important tool in refractory recurrent tracheo-esophageal fistula in children

International Journal of Pediatric Otorhinolaryngology — Tue, 09 Feb 2010 14:19:38 +0100

Abstract: Secondary tracheo-oesophageal fistula in delayed primary repair of oesophageal atresia is rare. This paper reports the successful use of the KTP laser in the treatment of this condition in a refractory case. It also recommends the use of direct laryngotracheobronchoscopy (DLTB) in the diagnosis. We recommend the use of this laser in cases of recurrent tracheo-esophageal fistula especially when other means have failed. (Source: International Journal of Pediatric Otorhinolaryngology)

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Autosomal dominant microtia.

European Journal of Medical Genetics — Tue, 09 Feb 2010 00:00:00 +0100

Authors: Elalaoui SC, Jaouad IC, Rifai L, Sefiani A Microtia (MIM600674) is a congenital malformation which occurs in 1/8000-10000 births. It is characterized by a small, and abnormally shaped pinna. It ranges in severity from a bump of tissue to a partially formed ear cup. Microtia is often associated with atresia of the external auditory canal. Familial microtia with meatal atresia has been reported, either with dominant or recessive inheritance, which makes genetic counselling difficult in sporadic cases. In the present paper, we report the case of a family with congenital microtia and conductive deafness in two generations, suggesting autosomal dominant inheritance with variable expression and incomplete penetrance. PMID: 20152949 [PubMed - as supplied by publisher] (Source: Eu...

Differential genome-wide gene expression profiling of bovine largest and second-largest follicles: identification of genes associated with growth of dominant follicles

Reproductive Biology and Endocrinology — Fri, 05 Feb 2010 00:00:00 +0100

Conclusion: We demonstrated that global gene expression profiling of F1 and F2 clearly reflected a difference in their follicular status. Expression of stage-specific genes in follicles may be closely associated with their growth or atresia. Several genes identified in this study will provide intriguing candidates for the determination of follicular growth. (Source: Reproductive Biology and Endocrinology)

[Bronchial Atresia together with a Gonadal Teratoma: What is the relationship?]

Archivos de Bronconeumologia — Fri, 05 Feb 2010 00:00:00 +0100

Authors: Sacristán Bou L, Martín Serrano C, Romero Candeira S PMID: 20138697 [PubMed - as supplied by publisher] (Source: Archivos de Bronconeumologia)

Concavo-convex oblique anastomosis technique for jejuno ileal atresia

Journal of Indian Association of Pediatric Surgeons — Thu, 04 Feb 2010 15:42:45 +0100

Conclusions:</b> We recommend this technique, as anastomosis is wide with less angulations, flow of effluent is linear, and there is less force exerted over post-anastomotic side wall. (Source: Journal of Indian Association of Pediatric Surgeons)

Esophageal atresia with tracheo-esophageal fistula: Accidental transtracheal gastric intubation

Journal of Indian Association of Pediatric Surgeons — Thu, 04 Feb 2010 15:42:45 +0100

We report such a rare occurrence. In this situation, the diagnosis and further management of EA and TEF was delayed. (Source: Journal of Indian Association of Pediatric Surgeons)

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Esophageal atresia with tracheo-esophageal fistula: Role of nebulized N-acetylcysteine in the outcome

Journal of Indian Association of Pediatric Surgeons — Thu, 04 Feb 2010 15:42:45 +0100

Pandey A, Gangopadhyay A N, Sharma S P, Kumar VJournal of Indian Association of Pediatric Surgeons 2009 14(4):232-232 (Source: Journal of Indian Association of Pediatric Surgeons)

Ultrasound-Guided Percutaneous Drainage of Neonatal Pyometrocolpos Under Local Anesthesia

CardioVascular and Interventional Radiology — Thu, 04 Feb 2010 06:44:24 +0100

We report an unusual cause of obstructive uropathy in three infants: pyometrocolpos due to lower genital tract atresia. Ultrasound-guided percutaneous drainage of the pyometrocolpos resulted in dramatically improved clinical and laboratory findings in these patients. Ultrasound-guided percutaneous drainage under local anesthesia is a simple, minimally invasive, safe, and effective procedure that facilitates later successful corrective surgery and avoids the need for more complex drainage procedures. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00270-010-9814-1Authors Oktay Algin, Atatürk Training and Research Hospital Department of Radiology Bilkent, Ankara TurkeyCuneyt Erdogan, Uludag University Medical Faculty Department of Radiology Gorukle, Bursa TurkeyNizamet...

Colonic interposition for esophageal replacement in children remains a good choice: 33-year median follow-up of 65 patients

Journal of Pediatric Surgery — Mon, 01 Feb 2010 00:00:00 +0100

Conclusions: Colon conduits restored gastrointestinal continuity with limited mortality and considerable morbidity but good functional outcome and health perception in the long-term. Our study suggests that colon grafts are no worse than gastric pull-ups in the long-term. (Source: Journal of Pediatric Surgery)

Intussusception in infants younger than 3 months: a single center's experience.

World Journal of Pediatrics : WJP — Mon, 01 Feb 2010 00:00:00 +0100

CONCLUSIONS: Infants aged 3 months or younger may suffer from intussusception and most of them present with typical symptoms. Early diagnosis and treatment are needed for a good prognosis. Intrauterine intussusception may be an etiological factor for ileal atresia. PMID: 20143212 [PubMed - in process] (Source: World Journal of Pediatrics : WJP)

Outcomes using predominantly single-stage approach to interrupted aortic arch and associated defects.

The Annals of Thoracic Surgery — Mon, 01 Feb 2010 00:00:00 +0100

CONCLUSIONS: A good functional outcome and low reoperation rate can be achieved with a single-stage repair regardless of the presence of major additional cardiac abnormalities. Neonates with risk factors such as low birth weight and prematurity require an individualized approach. PMID: 20103342 [PubMed - indexed for MEDLINE] (Source: The Annals of Thoracic Surgery)

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A novel two-stage complete repair method for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Chinese Medical Journal — Mon, 01 Feb 2010 00:00:00 +0100

CONCLUSIONS: This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative surgical approach in the treatment of PA-VSD and MAPCAs. PMID: 20193241 [PubMed - in process] (Source: Chinese Medical Journal)

Ross and Yasui operations for complex biventricular repair in infants with critical left ventricular outflow tract obstruction [Original articles]

European Journal of Cardio-Thoracic Surgery — Thu, 28 Jan 2010 19:40:36 +0100

Conclusions: Case selection is key for complex biventricular repair and the importance of appropriate case selection is exaggerated at young ages. All available options should be considered before pursuing the Ross operation in the presence of co-existing functional morphological abnormalities or emergent iatrogenic aortic regurgitation. However, both the Ross and Yasui operations in children (including neonates and young infants) with favourable functional morphology offer good survival, at least matching that of other biventricular repair strategies. (Source: European Journal of Cardio-Thoracic Surgery)

Pre- and postnatal diagnosis and outcome of fetuses and neonates with esophageal atresia and tracheoesophageal fistula

Prenatal Diagnosis — Thu, 28 Jan 2010 00:00:00 +0100

Clinical symptoms and ultrasound signs during pregnancy could suggest the presence of esophageal atresia (EA). However, most often EA is diagnosed postnatally. The aim of our study is to evaluate the course and outcome for prenatally and postnatally diagnosed EA. In addition, we studied the outcome of isolated versus nonisolated EA.In a retrospective data analysis, ultrasound characteristics, maternal and neonatal variables as well as clinical outcome were compared for fetuses/neonates with prenatal (n = 30) or postnatal (n = 49) diagnosis of EA. Clinical outcome in terms of morbidity and mortality of isolated EA was compared with that of EA complicated by chromosomal or structural anomalies.Prenatally diagnosed children were born 2 weeks earlier than postnatally diagnosed children (36.4 w...

Outcomes Using Predominantly Single-Stage Approach to Interrupted Aortic Arch and Associated Defects [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 27 Jan 2010 19:27:19 +0100

Conclusions A good functional outcome and low reoperation rate can be achieved with a single-stage repair regardless of the presence of major additional cardiac abnormalities. Neonates with risk factors such as low birth weight and prematurity require an individualized approach. (Source: The Annals of Thoracic Surgery)

Left Main Coronary Artery Atresia Diagnosed with 128-MDCT

Heart, Lung & Circulation — Tue, 26 Jan 2010 13:54:20 +0100

A 61-year-old female with intermittent chest discomfort and an inconclusive exercise treadmill test was submitted for a non-invasive coronary angiography with 128-multidetector computed tomography (MDCT) with prospective triggering (1.7mSv). The coronary arteries were free of atherosclerotic coronary artery disease (CAD). However, MDCT clearly demonstrated a congenital anomaly of the coronary arteries, with absence of the left coronary ostium and left main trunk (black arrow). A dominant right coronary artery (RCA) provided blood supply via conus branch (white arrow) to the left anterior descending (LAD) and left circumflex (Cx) arteries, which were located at their normal position. The conus branch (white arrow) coursed anteriorly to the main pulmonary artery. Subsequent invasive coronary...

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Long Term Outcomes of Surgically Treated Biliary Atresia - Four Decades of Experience

Journal of Surgical Research — Mon, 25 Jan 2010 16:41:02 +0100

Conclusions: KP remains the mainstay treatment for BA with a success rate approaching forty percent. Our outcome data show improving success rates of KP over the last four decades. As outcomes of LTx have also improved over this time period, overall survival of patients with BA in the current era exceeds 95%. (Source: Journal of Surgical Research)

A Fetal Model of the Tracheal Stenosis/Atresia Spectrum

Journal of Surgical Research — Mon, 25 Jan 2010 16:41:01 +0100

Background/Purpose: The treatment of severe tracheal stenosis/atresia remains essentially unresolved, particularly in the perinatal period. Previous models of these diseases have been restricted to adult animals. We sought to establish the principles of a large, surgical animal model of the congenital form of this spectrum of anomalies. Methods: Fetal lambs (n=8) underwent open surgery at 90-112 days gestation (term=145 days). Their cervical tracheas were encircled by a biocompatible polytetrafluoroethylene wrap, so as to extrinsically restrict their external diameter by 25%. Pregnancy was allowed to continue. Survivors (n=7) were killed at different time points post-operatively, namely 15, 21, 28, and 41days, before term. The manipulated tracheal segments were compared with their respecti...

What Causes Respiratory Distress?

PediatricEducation.org — Mon, 25 Jan 2010 00:01:37 +0100

Discussion Pectus excavatum is a deformation of the chest wall where the sternum and ribs grow abnormally to form a concavity relative to normal positioning. Pectus carnitum is similar but forms a convexity. Pectus excavatum is much more common (90%) than pectus carnitum (7-8%) or other congenital chest wall deformities (2-3%). Pectus excavatum is usually noted at birth or in the first year of life. It may be relatively minor (the most common) or quite severe (rarer) with the concavity displacing internal organs and possibly causing pulmonary and/or cardiac abnormalities. Usually it is relatively stable, but may progress especially during times of rapid growth such as puberty. Thankfully most patients do not need treatment but severe pectus excavatum may cause psychological distress becaus...

Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003.

Circulation — Mon, 25 Jan 2010 00:00:00 +0100

Conclusions-This population-based study demonstrates significant improvement in overall 5-year survival, particularly in cases of hypoplastic left heart syndrome and single ventricle. Additional studies are needed to determine the factors causing racial/ethnic and regional differences in outcome. PMID: 20100974 [PubMed - as supplied by publisher] (Source: Circulation)

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome.

The Ultrasound Review of Obstetrics and Gynecology — Mon, 25 Jan 2010 00:00:00 +0100

CONCLUSIONS: Tricuspid atresia and the frequently associated intracardiac anomalies can be diagnosed in the fetus with considerable accuracy. A thorough search for extracardiac malformations should be performed in order to rule out chromosomal anomalies and multiple malformation syndromes. Elevated pulsatility in the ductus venosus does not indicate cardiac failure. The short-term overall survival in continued pregnancies in our study exceeded 89%, with the greatest rate of loss being in the first year of postnatal life. Copyright (c) 2010 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 20101636 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

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Cyclooxygenase-2 Overexpression is Associated with Clinical Outcome in Biliary Atresia

European Journal of Pediatric Surgery — Tue, 19 Jan 2010 15:40:18 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0029-1243628Abstract The purpose of this study was to investigate the association between cyclooxygenase-2 (COX-2) expression and clinical outcome in biliary atresia (BA) patients.[...]© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Colonic Atresia: A Clinicopathological Insight into its Etiology

European Journal of Pediatric Surgery — Tue, 19 Jan 2010 15:40:18 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0029-1242735Abstract The pathogenesis of colonic atresia (CA) has not been discussed in detail, although the theory of a prenatal vascular insult, hypothesized from patients with small bowel atresia, has been accepted in the past. This review questions this etiology by reviewing all patients with CA treated in two institutions.[...]© Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Pregnancy outcome in patients with complex pulmonary atresia: case report and review of the literature

European Journal of Heart Failure — Mon, 18 Jan 2010 18:16:39 +0100

We report here on the outcome of pregnancy in a woman, aged 36, with complex pulmonary atresia in whom palliative operation had been performed at the age of 23. We review the medical literature on pregnancy course as well as maternal and foetal outcome in cases involving this maternal congenital heart disease. (Source: European Journal of Heart Failure)

Thrombospondin-1 Inhibits Angiogenesis and Promotes Follicular Atresia in a Novel in Vitro Angiogenesis Assay.

Endocrinology — Fri, 15 Jan 2010 00:00:00 +0100

Authors: Garside SA, Harlow CR, Hillier SG, Fraser HM, Thomas FH Thrombospondin-1 (TSP-1) is a putative antiangiogenic factor, but its role in regulating physiological angiogenesis is unclear. We have developed a novel in vitro angiogenesis assay to study the effect of TSP-1 on follicular angiogenesis and development. Intact preantral/early antral follicles dissected from 21-d-old rat ovaries were cultured for 6 d in the presence or absence of TSP-1. At the end of the culture period, angiogenic sprouting from the follicles was quantified using image analysis. Follicles were fixed and sectioned, and follicular apoptosis was assessed by immunohistochemistry for activated caspase-3 in granulosa cells. The results showed that TSP-1 inhibited follicular angiogenesis (P < 0.01) and promot...

Peculiar type 1 congenital pyloric atresia: a case report

Italian Journal of Pediatrics — Thu, 14 Jan 2010 00:00:00 +0100

We describe the neonatal course of a peculiar case of type 1 pyloric atresia, in which the pyloric membrane was connected to a second duodenal membrane through a virtual duodenal lumen in a premature newborn. The atypical variant required an unusual side to side gastroduodenostomy. We emphasize the importance of a prompt diagnosis to avoid potentially fatal complications and to warrant a good outcome even in the presence of a strange form of PA in the neonatal period. (Source: Italian Journal of Pediatrics)

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Schmid-Fraccaro Syndrome: Severe Neurologic Features

Pediatric Neurology — Tue, 12 Jan 2010 15:55:40 +0100

We report on a 17-year-old girl with Schmid-Fraccaro syndrome and severe cognitive deficits and motor deficits, who presented at our healthcare unit for a medical consultation. Her physical examination was remarkable for bilateral coloboma of the iris, hypertelorism, bilateral preauricular tags, scoliosis, and cardiac systolic murmur. After her birth, she was evaluated for anal atresia and congenital cardiac disease, which led to a genetic investigation and a diagnosis of Schmid-Fraccaro syndrome. Life expectancy in Schmid-Fraccaro syndrome depends on the number and variety of malformations, but in most cases the prognosis is favorable. (Source: Pediatric Neurology)

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: intrauterine course and outcome of an unusual congenital heart defect

Ultrasound in Obstetrics and Gynecology — Tue, 12 Jan 2010 00:00:00 +0100

We report on the intrauterine course of a case diagnosed at 17 weeks of gestation that had a favorable postnatal outcome after palliation. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: intrauterine course and outcome of an unusual congenital heart defect.

The Ultrasound Review of Obstetrics and Gynecology — Tue, 12 Jan 2010 00:00:00 +0100

We report on the intrauterine course of a case diagnosed at 17 weeks of gestation that had a favorable postnatal outcome after palliation. Copyright (c) 2010 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 20069575 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Thiamazole: Choanal atresia and aplasia cutis in a neonate following in utero exposure: case report

Reactions — Mon, 11 Jan 2010 14:23:59 +0100

(Source: Reactions)

Detection of Follicular Apoptosis in Water Buffalo (Bubalus bubalis) Ovary by Histology and Nick End Labelling Technique

Reproduction in Domestic Animals — Mon, 11 Jan 2010 00:00:00 +0100

The objective of this experiment was to assess the features and extent of follicular apoptosis in the water buffalo (Bubalus bubalis) ovary using classical histology and nick end labelling technique. Ovaries (n = 40) procured from the slaughterhouse were used for the study. The sections (5 [mu]m) were used for detection of terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labelling (TUNEL) and classical histology (H&E). Those follicles showing [ge] 5% TUNEL positivity (TUNEL assay) and pyknotic nuclei (histology) in granulosa cells were classified as atretic. Based on histology, the atretic primary and secondary follicles (%) were 93.82 and 95.62 respectively. The histology study reveals that the rates (%) of atresia in 5 mm were 36.90, 40.50, 62.84 and 74.5 respectively....

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Congenital malformations in infants born after in vitro fertilization in Sweden

Birth Defects Research Part A: Clinical and Molecular Teratology — Fri, 08 Jan 2010 00:00:00 +0100

The risk for congenital malformations is increased in infants born after in vitro fertilization (IVF). Some specific malformations appear to be more affected than others.The presence of congenital malformations in 15,570 infants born after IVF with an embryo transfer between April 1, 2001, and the end of 2006 were compared with all infants born in Sweden during 2001 to 2007 (n = 689,157). Risk estimates were made after adjusting for year of birth, maternal age, parity, smoking, and body mass index. The risks of specific malformations were compared with data from a previous study (1982 to March 31, 2001) of 16,280 infants born after IVF. Different IVF methods were compared to respect to malformation risk.Increased risks of a similar magnitude were found for most cardiovascular malformations...

Association of Paternal Age and Risk for Major Congenital Anomalies From the National Birth Defects Prevention Study, 1997 to 2004

Annals of Epidemiology — Thu, 07 Jan 2010 00:00:00 +0100

Conclusions: Our findings suggest that paternal age may be a risk factor for some multifactorial birth defects. (Source: Annals of Epidemiology)

Successful cord blood transplantation for a CHARGE syndrome with CHD7 mutation showing DiGeorge sequence including hypoparathyroidism

European Journal of Pediatrics — Wed, 06 Jan 2010 06:49:02 +0100

Abstract It is rare that coloboma, heart anomalies, choanal atresia, retarded growth and development, and genital and ear anomalies (CHARGE) syndrome patients have DiGeorge sequence showing severe immunodeficiency due to the defect of the thymus. Although the only treatment to achieve immunological recovery for these patients in countries where thymic transplantation is not ethically approved would be hematopoietic cell transplantation, long-term survival has not been obtained in most patients. On the other hand, it is still not clarified whether hypoparathyroidism is one of the manifestations of CHARGE syndrome. We observed a CHARGE syndrome patient with chromodomain helicase DNA-binding protein 7 mutation showing DiGeorge sequence including the defect of T cells accompan...

Fetal gastrointestinal MRI: all that glitters in T1 is not necessarily colon

Pediatric Radiology — Wed, 06 Jan 2010 06:48:18 +0100

Conclusion In cases of fetal occlusion, T1 hypersignal should not be considered as a sign of distal ileal or colonic occlusion. The obstruction may be proximal, implying a risk of small bowel syndrome, which requires adequate parental counselling. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00247-009-1497-xAuthors Marina Colombani, La Timone Children’s Hospital Service de Radiopediatrie 264 rue saint-Pierre Marseille 13385 FranceMathilde Ferry, Groupe Rennais d’Imagerie Médicale Service de Radiologie Rennes FranceCatherine Garel, Hôpital d’Enfants Armand-Trousseau Service de Radiologie Paris FranceMarie Cassart, Erasme Hospital Medical Imaging Brussels BelgiumAlain Couture, Hôpital Arnaud de Villeneuve Pediatric Radiology Montpellier Fran...

Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia

Pediatric Pulmonology — Wed, 06 Jan 2010 00:00:00 +0100

We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood. Pediatr Pulmonol. ©2010 Wiley-Liss, Inc. (Source: Pediatric Pulmonology)

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Macrophages are targeted by rotavirus in experimental biliary atresia and induce neutrophil chemotaxis via Mip2/Cxcl2.

Pediatric Research — Wed, 06 Jan 2010 00:00:00 +0100

In conclusion, infected cholangiocytes did not promote chemotaxis of inflammatory cells. Investigating alternate cellular targets of RRV, we detected the virus in hepatic macrophages, and found that infected macrophages promoted neutrophil chemotaxis by release of Mip2/Cxcl2 in response to RRV. PMID: 20057339 [PubMed - as supplied by publisher] (Source: Pediatric Research)

1st International Workshop on Esophageal Atresia

NLH - Gastroenterology & Liver Diseases - Events — Tue, 05 Jan 2010 14:15:50 +0100

(Source: NLH - Gastroenterology & Liver Diseases - Events)

Association of Paternal Age and Risk for Major Congenital Anomalies From the National Birth Defects Prevention Study, 1997 to 2004.

Annals of Epidemiology — Tue, 05 Jan 2010 00:00:00 +0100

CONCLUSIONS: Our findings suggest that paternal age may be a risk factor for some multifactorial birth defects. PMID: 20056435 [PubMed - as supplied by publisher] (Source: Annals of Epidemiology)

Plectin expression patterns determine two distinct subtypes of epidermolysis bullosa simplex

Human Mutation — Tue, 05 Jan 2010 00:00:00 +0100

Plectin is a cytoskeletal linker protein that has a dumbbell-like structure with a long central rod and N- and C-terminal globular domains. Mutations in the gene encoding plectin (PLEC1) cause two distinct autosomal recessive subtypes of epidermolysis bullosa (EB): EB simplex with muscular dystrophy (EBS-MD), and EB simplex with pyloric atresia (EBS-PA). Here, we demonstrate that normal human fibroblasts express two different plectin isoforms including full-length and rodless forms of plectin. We performed detailed analysis of plectin expression patterns in six EBS-MD and three EBS-PA patients. In EBS-PA, expression of all plectin domains was found to be markedly attenuated or completely lost; in EBS-MD, the expression of the N- and C-terminal domains of plectin remained detectable, althou...

A histopathological study of liver and biliary remnants with clinical outcome in cases of extrahepatic biliary atresia.

Indian Journal of Pathology and Microbiology — Fri, 01 Jan 2010 00:00:00 +0100

Conclusions: Histopathological correl ations with clinical outcome in EHBA have been rarely reported from the Indian subcontinent. A greater degree of fibrosis at the time of hepatic portoenterostomy and presence of ductal plate malformation is associated with a significantly poorer clinical outcome. PMID: 20090233 [PubMed - in process] (Source: Indian Journal of Pathology and Microbiology)

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A missed bilateral choanal atresia.

Annals of the Academy of Medicine, Singapore — Fri, 01 Jan 2010 00:00:00 +0100

Authors: Irfan M, Suzina SA PMID: 20126823 [PubMed - in process] (Source: Annals of the Academy of Medicine, Singapore)

Monitoring Cyclosporine Microemulsion at Two Hours Post Dosing in Pediatric Maintenance Liver Transplant Recipients

Transplantation Proceedings — Fri, 01 Jan 2010 00:00:00 +0100

This study of 128 patients included 50.8% males and 64.8% recipients of cadaveric livers. Their main reasons for transplantation were as follows: 43.8% acute liver failure and 37.5% biliary atresia. Mean age at time of transplantation was 5.4 ± 4.5 years for boys and 3.4 ± 3.3 years for girls. Mean age at the beginning of C2 monitoring was 8.9 ± 4.8 years and time elapsed since transplantation was 53.6 ± 36.4 months. The initial Cyclosporine dose of 5.5 ± 5 mg/kg/d had been reduced by month 24 to 4.5 ± 1.5 mg/kg/d. Estimation of glomerular filtration rate (eGFR) was performed using the Schwartz formula. Baseline creatinine and eGFR were 0.73 ± 0.49 mg/dL and 111.99 ± 28.27 mL/min.m2 versus 24-month creatinine and eGFR of 0.69 ± 0.20 mg/dL and 122.26 ± 24.47 mL/min.m2, respectivel...

Neonatal intrahepatic cholestasis caused by citrin deficiency in two Malaysian siblings: outcome at one year of life.

Singapore Medical Journal — Fri, 01 Jan 2010 00:00:00 +0100

We report two Malaysian siblings with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). The younger sibling, a six-month-old Chinese girl, presented with prolonged neonatal jaundice, and was investigated for biliary atresia. Urine metabolic screen showed the presence of urinary-reducing sugars, and she was treated with a lactose-free formula. NICCD was suspected based on the clinical history, examination and presence of urinary citrulline. Mutation study of the SLC25A13 gene showed the compound heterozygotes, 851del4 and IVS16ins3kb, which confirmed the diagnosis of NICCD in the patient and her three-year-old female sibling, who also had unexplained neonatal cholestasis. Long-term dietary advice, medical surveillance and genetic counselling were provided to the family....

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:38 +0100

The prenatal or postnatal factors that predict complex gastroschisis in patients (atresia, volvulus, necrotic bowel and bowel perforation) remain controversial. The authors from Mott Children's Hospital in Ann Arbor evaluated the prognostic value of prenatal ultrasonographic parameters and early postnatal factors in predicting clinical outcomes. (Source: Journal of Pediatric Surgery)

Prevention of biliary atresia in the experimental mouse model provides a new approach to elucidate its immune pathomechanism

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:38 +0100

In Cincinnati, Children's Hospital Medical Center, Division of Gastroenterology, Hepatology and Nutrition, Bezzera and colleagues established a highly ranked basic research program on the artificial biliary atresia (BA) mouse model , which is running successfully . Just recently, their colleagues from the pediatric surgical department started separate studies, one of which was published by Journal of Pediatric Surgery in August 2009. Herein, Bondoc et al show that rotavirus and its variants protect the offspring from Rhesus-rotavirus triggered BA, when the substances are prenatally and intraperitoneally applied. They used pure Rhesus rotavirus (RRV), a live pentavalent rotavirus vaccine (RotaTeq, Sanofi Pasteur MSD, Paris, France) containing 5 human-bovine reassortant rotaviruses, each con...

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Reply to Letter to the Editor by Petersen et al

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:38 +0100

We appreciate the comments of Drs Petersen, Turowski, and Ure, and we thank them for their interest in our recent publication in the Journal of Pediatric Surgery as well as their ongoing contributions to the understanding of both human biliary atresia (BA) and the murine experimental model previously described by Riepenhoff-Talty et al . Specifically, a study from their group published in 2001 provided a foundation for future studies exploring a protective immunologic mechanism transmitted from rhesus rotavirus (RRV)-infected BALB/c dams to their offspring in the murine model of BA . In our publication, we unintentionally omitted a citation of this study that demonstrated clinical protection against the development of obstructive cholangiopathy in newborn pups born to dams that were infect...

Intestinal complications after antenatal fetoscopic laser ablation in twin-to-twin transfusion syndrome

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:38 +0100

We report 3 cases of intestinal injury in TTTS after fetoscopic laser ablation of the communicating vessels: 2 cases of intestinal atresia, and 1 case of necrotizing enterocolitis of 1 twin. Intestinal ischemic diseases have been reported after prenatal laser treatment in TTTS; with this report, we add 3 more cases. (Source: Journal of Pediatric Surgery)

Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:26 +0100

Conclusions: In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population. (Source: Journal of Pediatric Surgery)

Cholangiocyte apoptosis is an early event during induced metamorphosis in the sea lamprey, Petromyzon marinus L

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:26 +0100

Conclusions: DNA fragmentation is identified at the earliest stages of metamorphosis during induced metamorphosis in lampreys. Additional studies are necessary to validate this potentially valuable animal model. (Source: Journal of Pediatric Surgery)

New approaches to pectus and other minimally invasive surgery in Argentina

Journal of Pediatric Surgery — Thu, 31 Dec 2009 15:18:24 +0100

Abstract: This is a presentation about the development of pediatric minimally invasive surgery and other pediatric surgical practices in Argentina. For the last 15 years, based on a collaborative approach, many surgical and nonsurgical procedures have been used in the treatment of long gap esophageal atresia and thoracic wall deformities. These procedures are described and detailed in original algorithms that are currently being used to treat our patients. (Source: Journal of Pediatric Surgery)

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Endoscopic choanoplasty without stenting for congenital choanal atresia repair

International Journal of Pediatric Otorhinolaryngology — Tue, 29 Dec 2009 14:14:27 +0100

Conclusions: Transnasal endoscopic choanoplasty repair for both unilateral and bilateral choanal atresia without intranasal stenting was found to be an expedient and safe procedure that affords minimal blood loss, minimal complications with a high rate of success. (Source: International Journal of Pediatric Otorhinolaryngology)

Orthopedic palatal expansion in the treatment of bilateral congenital choanal atresia: An additional tool in the long term follow up of patients?

International Journal of Pediatric Otorhinolaryngology — Sat, 26 Dec 2009 14:09:39 +0100

Conclusion: The cooperation between ENT surgeon and orthodontist may allow for an reduction in the burden of treatment of children affected by BCCA. (Source: International Journal of Pediatric Otorhinolaryngology)

Increased ovarian follicle atresia in obese Zucker rats is associated with enhanced expression of the forkhead transcription factor FOXO1

Medical Molecular Morphology — Wed, 23 Dec 2009 23:03:13 +0100

Abstract It is well established that hyperinsulinemia, resulting from insulin resistance, plays a role in the pathophysiology of polycystic ovary syndrome (PCOS). The aim of this study was to investigate if ovarian follicular development and atresia are impaired in obese hyperinsulinemic (fa/fa) Zucker rats. To gain insight into the molecular mechanism of follicular atresia, we also examined the expression and localization of forkhead transcription factor FOXO1, a major regulator of cell fate decisions such as differentiation, cell-cycle arrest, and cell death. Serum insulin but not gonadotropin levels were significantly higher in obese (fa/fa) rats when compared to lean controls. Total ovarian follicle number and the percentage of atretic follicles were also significantly...

Duodenal and small intestinal atresia

Surgery (Medicine Publishing) — Wed, 23 Dec 2009 15:21:09 +0100

Abstract: Bile-stained vomiting in a neonate indicates significant pathology and suggests mechanical obstruction. Congenital discontinuity of the bowel (atresia) is a common cause of mechanical intestinal obstruction in neonates. Key features of duodenal/intestinal atresia and their management are discussed in this article. (Source: Surgery (Medicine Publishing))

Oesophageal atresia

Surgery (Medicine Publishing) — Wed, 23 Dec 2009 15:21:09 +0100

Abstract: Oesophageal atresia is a congenital abnormality in which a variable portion of the mid-oesophagus is not formed. This is usually associated with a distal tracheo-oesophageal fistula. Important associated structural anomalies include heart defects, duodenal atresia, anorectal malformations. The anatomy, diagnosis and management of this condition are described. (Source: Surgery (Medicine Publishing))

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Role of 64-MDCT in Evaluation of Pulmonary Atresia With Ventricular Septal Defect

American Journal of Roentgenology — Tue, 22 Dec 2009 19:02:37 +0100

CONCLUSION. The management of this complex condition has been largely determined by the morphology of pulmonary arteries and the extracardiac sources of pulmonary blood supply. Although echocardiography and catheter angiography are the traditional imaging techniques used to diagnose PA-VSD, 64-MDCT has become a valuable noninvasive imaging technique in comprehensive evaluation of this condition. (Source: American Journal of Roentgenology)

Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis

Pediatric Transplantation — Tue, 22 Dec 2009 00:00:00 +0100

We report a case of a LDLT for a 12-yr-old Japanese girl with BA accompanied by HES. Histological examination of the resected liver showed biliary cirrhosis with dense eosinophilic infiltration of portal tracts and the lobules of the liver. She developed portal vein thrombosis on post-operative day 10 and the histopathological findings of the thrombus revealed dense eosinophilic deposition, suggesting that HES might have influenced the formation of this thrombus. Liver graft biopsies also demonstrated the presence of activated eosinophilils with biliary damage. Blood chemistry findings suggested liver dysfunction as a result of the eosinophilic infiltrations. Prednisolone treatment improved the liver dysfunction. Four years after LDLT, she remains clinically well on prednisolone at 0.3 mg/...

[Oesophageal atresia: Management in sub saharian countries.]

Archives de Pediatrie — Tue, 22 Dec 2009 00:00:00 +0100

Authors: Bandré E, Niandolo KA, Wandaogo A, Bankolé R, Mobiot ML PMID: 20034771 [PubMed - as supplied by publisher] (Source: Archives de Pediatrie)

Coordinated regulation of follicle development by germ and somatic cells.

Reproduction, Fertility, and Development — Sat, 19 Dec 2009 13:32:55 +0100

Authors: Binelli M, Murphy BD The continuum of folliculogenesis begins in the fetal ovary with the differentiation of the oogonia and their isolation within the primordial follicles. Primordial follicle activation is an enigmatic process, whereby some follicles enter the growing pool to become primary follicles, thereby embarking on an irreversible progression towards ovulation or atresia. This process is under the coordinated regulation of factors from the oocyte itself, as well as from the somatic cells of the ovary, in particular the theca and granulosa cells, which are structural components of the follicle. These two influences provide the principal stimuli for the growth of the follicle to the late preantral or early antral stage of development. The endocrine effects of the gonado...

The Performance of Hancock Porcine-Valved Dacron Conduit for Right Ventricular Outflow Tract Reconstruction [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Fri, 18 Dec 2009 23:25:12 +0100

Conclusions The Hancock valved conduit is a safe and reliable alternative to homografts. It appears to be appropriate in patients with limited pulmonary vascular bed and high pulmonary artery pressures. Caution is required in neonates because of the rigidity of the Dacron housing. Initial results with secondary percutaneous procedures are encouraging. (Source: The Annals of Thoracic Surgery)

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Clinical practice:

European Journal of Pediatrics — Fri, 18 Dec 2009 07:06:51 +0100

Abstract Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life remains the only surgical repair procedure. Two thirds of patients will clear their jaundice after a Kasai procedure, but only about one third will retain their livers after the first decade of life. Failure of this procedure leaves liver transplantation as the only chance for survival, and this disease is the commonest indication for liver transplantation in children. With modern medical care and refinements in surgical techniques, survival after either or both of these p...

Accessory Pathway Reciprocating Tachycardia Involving Twin AV Nodes in a Patient with Atrioventricular Discordance and Mitral Atresia

Pacing and Clinical Electrophysiology : PACE — Fri, 18 Dec 2009 00:00:00 +0100

We report a case of accessory pathway reciprocating tachycardia in atrioventricular discordance (AVD) and mitral atresia with twin AV nodes. In this case, the anterior AV node was located along the atretic mitral valve. The anterior AV node was involved in tachycardia and the posterior AV node acted as a bystander during tachycardia. The anterior AV node in AVD can be located along the atretic mitral valve, and one of twin AV nodes might act as a bystander during AV reciprocating tachycardia. (PACE 2009; 1[ndash]4) (Source: Pacing and Clinical Electrophysiology : PACE)

Multidetector computed tomography hepatic findings in children with end-stage biliary atresia

European Radiology — Thu, 17 Dec 2009 07:03:16 +0100

Conclusion MDCT can provide accurate morphological and vascular assessment of patients with end-stage biliary atresia and should be used for precise surgical planning. To the best of our knowledge this is one of the first studies to show the presence of numerous veno-venous communications in BA. Content Type Journal ArticleCategory PediatricDOI 10.1007/s00330-009-1681-2Authors Settimo Caruso, Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione (IsMeTT) Department of Radiology Via Tricomi 1 90127 Palermo ItalyRoberto Miraglia, Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione (IsMeTT) Department of Radiology Via Tricomi 1 90127 Palermo ItalyMariapina Milazzo, Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione (IsMeTT) Dep...

Pulmonary Hypoplasia in a Newborn with Goldenhar Syndrome

The Journal of Pediatrics — Tue, 15 Dec 2009 15:04:01 +0100

A female infant born to a 31-year-old mother (G2P2) by cesarean section at 35 weeks' gestation was referred for facial asymmetry and facial paralysis (, A). The parents are not relatives. The pregnancy was complicated by polyhydramnios with no known intrauterine teratogenic exposure. On physical examination, the infant appeared alert, active, and well. Her weight was 2280 g (50th percentile), length was 44 cm (10th to 50th percentile), and head circumference was 33 cm (50th percentile). The following dysmorphic features were noted: left facial hypoplasia, ipsilateral ear anomalies (i.e., malformed, low-set ear; microtia; preauricular tags; external auditory canal atresia), mandibular hypoplasia, and hypertelorism. A chest radiograph revealed nearly complete opacification of the left hemith...

Supracardiac anomalous pulmonary venous connection with unilateral pulmonary venous atresia: Diagnosis and management

Annals of Pediatric Cardiology — Sat, 12 Dec 2009 13:50:44 +0100

We report a case of a 6-day-old neonate referred to us for surgical correction of total anomalous pulmonary venous connection. Meticulous evaluation contributed to accurate diagnosis of associated unilateral pulmonary venous atresia. This unique association provides insights into the importance of evaluation of all pulmonary veins using various imaging tools. (Source: Annals of Pediatric Cardiology)

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Bilateral choanal atresia in an adult - management with mitomycin C and without stents: a case report

BioMed Central — Fri, 11 Dec 2009 00:00:00 +0100

Background: A 23-year-old female patient presented to the outpatient clinic with bilateral nasal obstruction and discharge since birth. Endoscopic examination and paranasal sinus tomography revealed bilateral choanal atresia. She did not have any other congenital abnormalities. Her parents reported cyanosis in childhood that worsened during feeding and improved during crying; however, they had not visited a physician. She did not have remarkable complaints during early childhood or adolescence. Methods: The patient was operated under general anesthesia, using a 0o 4 mm rigid endoscope. The orifice was widened with a curette and, to prevent stenosis, mitomycin-C (1 mg/ml) was applied topically to the nasopharyngeal orifice for 5 minutes. No stents were placed. Results: Follow-up evaluation ...

Fetal Eagle–Barrett syndrome and pulmonary atresia with intact ventricular septum

Congenital Anomalies — Mon, 07 Dec 2009 00:00:00 +0100

(Source: Congenital Anomalies)

Aortic Atresia With Marked Collaterals Percutaneously Reconstructed by a Covered Stent

Journal of the American College of Cardiology — Sat, 05 Dec 2009 13:48:59 +0100

A 46-year-old man had poorly controlled arterial hypertension despite triple antihypertensive medication for years before by chance aortic coarctation was diagnosed. He was admitted to our institution for operation. Given the high operative risk due to the marked collaterals, we decided on an attempt at percutaneous dilation of the coarctation in surgical stand-by. However, via the femoral artery, the ascending aorta could not be reached. Angiography in the ascending aorta via the right brachial artery (A, Online Videos 1 and 2) and descending aorta (B) confirmed the diagnosis of aortic atresia with blind-ended proximal and distal aorta separated by 3 mm and marked collaterals. As an attempt of recanalization from the brachial approach was unsuccessful, interruption of the aortic arch was ...

Early Detection of Anomalous Origin of Left Coronary Artery from the Right Pulmonary Artery After Successful Repair of Critical Coarctation of the Aorta

Pediatric Cardiology — Fri, 04 Dec 2009 07:19:19 +0100

This report describes a patient who underwent successful repair of critical coarctation with normalization of LV function despite signs of endocardial fibroelastosis but with persisting elevation of cardiac troponin T. Cardiac catheterization showed the rare coincidence of anomalous origin of left coronary artery from the right pulmonary artery (ALCAPA) and coronary sinus orifice atresia with left superior vena cava. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00246-009-9595-yAuthors Levent Celik, Klinikum Links der Weser Department for Congenital Heart Disease/Pediatric Cardiology Senator Wessling Strasse 1 28277 Bremen GermanyVera Becker, Klinikum Links der Weser Department for Cardiothoracic Surgery Senator Wessling Strasse 1 28277 Bremen GermanyDieter Hammel, K...

The Immunohistochemical Localization of Desmin and Smooth Muscle Actin in the Ovary of the African Giant Rat (Cricetomys gambianus) During the Oestrous Cycle

Anatomia, Histologia, Embryologia: Journal of Veterinary Medicine Series C — Thu, 03 Dec 2009 00:00:00 +0100

The aim of this study was to describe the distribution of smooth muscle actin and desmin immunopositive cells in the ovary of the giant rat. In addition, the study describes the morphological changes in the ovary of this species during the oestrous cycle. Healthy secondary and tertiary follicles dominated the ovary during pro-oestrus and oestrus. The theca externa of the tertiary follicles was immunopositive for smooth muscle actin, but immunonegative for desmin. Oestrus was also characterized by the presence of corpora haemorrhagica, which had an outer layer of smooth muscle actin immunopositive cells. Differentiating corpora lutea were observed during metoestrus. A further notable feature of the ovary during metoestrus was the presence of numerous atretic secondary and tertiary follicles...

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Drugs affecting intestinal secretions

Wed, 02 Dec 2009 14:14:05 +0100

Published July 2009 Aims: Bile acids (ursodeoxycholic and chenodeoxycholic acid) may be used as dietary supplements in children with inborn errors of bile acid synthesis. Ursodeoxycholic acid is used to improve the flow of bile in children with cholestatic conditions such as familial intrahepatic cholestasis, biliary atresia in infants, cystic-fibrosis-related liver disease, and cholestasis caused by total parenteral nutrition or following liver transplantation. Ursodeoxycholic acid may also relieve the severe itching associated with cholestasis. In sclerosing cholangitis, ursodeoxycholic acid is used to lower liver enzyme and serum-bilirubin concentrations. (Source: Gastroenterology and Liver Diseases Specialist Library - Biliary tree and gallbladder)

Drugs affecting intestinal secretions

Gastroenterology and Liver Diseases Specialist Library - Liver — Wed, 02 Dec 2009 14:13:56 +0100

Drugs affecting intestinal secretions

Gastroenterology and Liver Diseases Specialist Library - Pancreas — Wed, 02 Dec 2009 13:48:41 +0100

Drugs affecting intestinal secretions

Wed, 02 Dec 2009 12:08:39 +0100

Published July 2009 Aims: Bile acids (ursodeoxycholic and chenodeoxycholic acid) may be used as dietary supplements in children with inborn errors of bile acid synthesis. Ursodeoxycholic acid is used to improve the flow of bile in children with cholestatic conditions such as familial intrahepatic cholestasis, biliary atresia in infants, cystic-fibrosis-related liver disease, and cholestasis caused by total parenteral nutrition or following liver transplantation. Ursodeoxycholic acid may also relieve the severe itching associated with cholestasis. In sclerosing cholangitis, ursodeoxycholic acid is used to lower liver enzyme and serum-bilirubin concentrations. (Source: NHS Evidence - gastroenterology and liver diseases - Updates for Therapeutic Nutrition)

Plectin Gene Defects Lead to Various Forms of Epidermolysis Bullosa Simplex

Dermatologic Clinics — Tue, 01 Dec 2009 14:33:05 +0100

Plectin is an important organizer of the keratin filament cytoskeleton in basal keratinocytes. It is essential for anchoring these filaments to the extracellular matrix via hemidesmosomal integrins. Loss of plectin or incorrect function of the protein due to mutations in its gene can lead to various forms of the skin blistering disease, epidermolysis bullosa simplex. Severity and subtype of the disease is dependent on the specific mutation and can be associated with (late-onset) muscular dystrophy or pyloric atresia. Mouse models mimicking the human phenotypes allow detailed study of plectin function. (Source: Dermatologic Clinics)

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Epidermolysis Bullosa with Pyloric Atresia

Dermatologic Clinics — Tue, 01 Dec 2009 14:33:05 +0100

This article describes the clinical and pathologic features and molecular genetics of EB-PA, the mutations in the α6β4 integrin and plectin genes that cause EB-PA, and the clinical implications of molecular genetics on EB-PA. (Source: Dermatologic Clinics)

Prenatal diagnosis of congenital agenesis of the fetal portal venous system.

The Ultrasound Review of Obstetrics and Gynecology — Tue, 01 Dec 2009 00:00:00 +0100

CONCLUSION: CAPVS can be detected prenatally. An abnormal course of the umbilical vein necessitates prompt sonographic evaluation of the umbilical-portal venous system and meticulous investigation for additional anomalies. Complete CAPVS may be associated with remote clinical consequences of which the parents should be informed. Partial CAPVS has a favorable prognosis. Copyright (c) 2009 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 19953566 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Doppler indices of the middle cerebral artery in fetuses with cardiac defects theoretically associated with impaired cerebral oxygen delivery in utero: is there a brain-sparing effect?

The Ultrasound Review of Obstetrics and Gynecology — Tue, 01 Dec 2009 00:00:00 +0100

CONCLUSIONS: Fetuses with cardiac defects theoretically associated with markedly impaired cerebral oxygen delivery in utero (TGA and HLH) have smaller HCs at birth. However, only fetuses with HLH have cerebrovascular alterations that are detectable by evaluation of the Doppler indices MCA-PI, MCA-RI and CPR. Copyright (c) 2009 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 19953563 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

The oxidative state of children with cyanotic and acyanotic congenital heart disease.

Anadolu Kardiyol Der... — Tue, 01 Dec 2009 00:00:00 +0100

CONCLUSION: The level of oxidative stress in patients with cyanotic congenital heart disease was significantly higher than in the acyanotic and control groups, which were similar. PMID: 19965321 [PubMed - in process] (Source: Anadolu Kardiyol Der...)

Esophageal atresia/tracheoesophageal fistula in very low-birth-weight neonates: improved outcomes with staged repair

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Abstract: Introduction: The surgical management of esophageal atresia with distal tracheoesophageal fistula (EA/TEF) involves early division of the TEF and primary esophageal anastomosis. However, in premature infants, the morbidity associated with primary repair remains high, and the optimal surgical approach has not been well defined.Methods: Medical records of very low-birth-weight infants ( (Source: Journal of Pediatric Surgery)

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Influence of thoracoscopic esophageal atresia repair on esophageal motor function and gastroesophageal reflux

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Conclusion: There are unlikely to be benefits from thoracoscopic repair of EA in terms of postoperative esophageal motor function. (Source: Journal of Pediatric Surgery)

Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Conclusions: Most cases of BA with a hilar cyst achieved excellent clinical outcome after a hepaticojejunostomy. (Source: Journal of Pediatric Surgery)

Clinical value of pelvic 3-dimensional magnetic resonance image reconstruction in anorectal malformations

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Conclusions: Pelvic magnetic resonance 3D reconstructed images were able to show the dimensional anatomical relations of pelvis, bladder, urethra, rectum, and SMC. Both a 3D image and positional information with MRI offers the surgeon a simulated operative profile of the SMC superior to MRI slices alone, which will help in providing morphological data for image diagnosis and operation of the ARM. (Source: Journal of Pediatric Surgery)

Individualized management of upper rectal atresia

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Conclusions: Colon atresia, especially at the level of the upper rectum, is uncommon. Whether to proceed with an ostomy or to individualize the operative procedure according to the location of the atresia is still controversial. Transanal mucosectomy was a useful technique at the time of the definitive pull-through for the treatment of upper rectal atresia. In cases of upper CA associated with imperforate anus, delay in diagnosis and potential complications may result if the diagnosis of upper rectal atresia is missed. (Source: Journal of Pediatric Surgery)

Extrathoracic esophageal elongation (Kimura's technique): a feasible option for the treatment of patients with complex esophageal atresia

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Conclusions: We believe that the EEE is a useful surgical option for a selected group of patients with complex long-gap EA who required a primary esophagostomy and also for patients with any type of EA who developed severe complications after a primary repair and required a secondary esophagostomy. With this technique, we avoided an esophageal replacement in 80% of cases, and given that the EEE does not invalidate a later esophageal replacement, we believe that the EEE is a feasible initial option for these patients. (Source: Journal of Pediatric Surgery)

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A rare anatomical variation of type B esophageal atresia

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

Esophageal atresia (EA) is the most common congenital anomaly of the esophagus, occurring in 1 of 3500 neonates . The most common type of EA is that of a distal tracheoesophageal fistula (TEF) (gross classification type C, 86%) . Esophageal atresia with proximal TEF (gross type B) occurs only in 2% of cases. Once diagnosed, other anomalies of the VACTERL association, which is present in 50% of neonates with EA, should also be investigated . (Source: Journal of Pediatric Surgery)

Mechanical compression with secondary ischemia as a possible cause of atresias associated with omphalocele

Journal of Pediatric Surgery — Tue, 01 Dec 2009 00:00:00 +0100

We report 2 cases of omphalocele associated with intestinal atresia. In the context of this unusual sequence, we discuss the etiopathogenesis of intestinal atresias in association with tight abdominal wall defects. (Source: Journal of Pediatric Surgery)

Etiological spectrum of acute intestinal obstruction.

Indian Pediatrics — Tue, 01 Dec 2009 00:00:00 +0100

Authors: Annigeri VM, Mahajan JK, Rao KL Gastrointestinal obstruction in children may be due to a variety of conditions. 150 cases of acute intestinal obstructions seen over a period of 180 months were reviewed. Intestinal atresia was the comonest cause (34, 22.4%). PMID: 20061589 [PubMed - in process] (Source: Indian Pediatrics)

Natural History of Spinal Anomalies and Scoliosis Associated With Esophageal Atresia

PEDIATRICS — Mon, 30 Nov 2009 12:01:59 +0100

CONCLUSIONS: The risk of scoliosis is 13-fold after repair of EA in relation to general population. Nearly half of the patients have vertebral anomalies predominating in the cervical spine. Most of these deformities were not diagnosed primarily or during growth. Spinal surgery is rarely indicated. (Source: PEDIATRICS)

Assessment of estrogen receptors and apoptotic factors in cryopreserved human ovarian cortex.

Systems Biology in Reproductive Medicine — Sun, 29 Nov 2009 04:08:11 +0100

Authors: Depalo R, Lorusso F, Bettocchi S, Selvaggi L, Cavallini A, Valentini AM, Caruso ML, Lippolis C The aims of this study were: (i) to examine frozen-thawed ovarian tissues for features of follicular health and atresia by histology; (ii) to assess the expression of estrogen receptors alpha (ERalpha) and beta (ERbeta) by real-time PCR; (iii) to evaluate the Bax/Bcl-2 ratio, as an apoptotic index, in the ovarian tissues before and after cryopreservation. Ovarian cortical biopsies were obtained from 11 patients. The fragments were subdivided into two groups, fresh (control tissues) and cryopreserved tissues obtained by direct plunging into liquid nitrogen. Both tissue groups were subjected to a histological evaluation of the healthy and atretic follicles, immunohistochemical localiza...

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The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome.

The Annals of Thoracic Surgery — Sat, 28 Nov 2009 00:08:15 +0100

CONCLUSIONS: Clinically important RAAO occurred in 24% of the hypoplastic left heart syndrome patients in this series. If RAAO is detected at birth or early interstage, a Norwood operation is now favored. Palliative interventional catheterization remains very important mid and late interstage for continuing the hybrid strategy toward comprehensive stage 2. PMID: 19932266 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Prenatal diagnosis of left atrial isomerism

Heart — Fri, 27 Nov 2009 18:01:44 +0100

Conclusion: Left atrial isomerism presents a varied spectrum of cardiac malformations when it is detected prenatally. Complete heart block, complex cardiac abnormalities and fetal hydrops are poor prognostic features. Those with only minor cardiac malformations are at risk postnatally for biliary atresia and for bowel obstruction due to malrotation. (Source: Heart)

Anomalous origin of the pulmonary arteries from the left coronary artery in tetralogy of Fallot with pulmonary atresia

Cardiology in the Young — Fri, 27 Nov 2009 13:52:36 +0100

MiscellaneousLin Wu, Fang Liu, Cardiology in the Young, Volume 19 Issue 06 , pp 620-621Abstract (Source: Cardiology in the Young)

Diagnosis and management of congenital right pulmonary venous atresia

Cardiology in the Young — Fri, 27 Nov 2009 13:52:36 +0100

Brief ReportI. Ricardo Argueta-Morales, Ruchira Garg, William M. DeCampli, Cardiology in the Young, Volume 19 Issue 06 , pp 648-651AbstractCongenital unilateral pulmonary venous atresia is a rare anatomical defect. Patients present with pulmonary symptoms, and diagnosis may be ellusive. Pulmonary arterial wedge angiography is the gold standard with which to achieve diagnosis, and surgical intervention is often required for symptomatic patients. (Source: Cardiology in the Young)

Is It Possible to Differentiate between Choledochal Cyst and Congenital Biliary Atresia (Type I Cyst) by Antenatal Ultrasonography?

Fetal Diagnosis and Therapy — Thu, 26 Nov 2009 23:00:00 +0100

Fetal Diagn Ther 1997;12:306-308 (DOI:10.1159/000264493) (Source: Fetal Diagnosis and Therapy)

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Fluoroscopy-guided Topical Application of Mitomycin-C in a Case of Refractory Esophageal Stricture

Journal of Vascular and Interventional Radiology : JVIR — Wed, 25 Nov 2009 00:00:00 +0100

Esophageal strictures refractory to conservative treatment (balloon dilation, bouginage) are difficult to manage. Recently, some investigators reported great success using endoscopy-guided topical application of mitomycin-C to prevent scar formation and restenosis in children with benign esophageal strictures unresponsive to balloon dilation. Herein, the authors report a fluoroscopic approach for topical application of mitomycin-C in a case of refractory esophageal stricture after surgical repair in a child with congenital esophageal atresia. This technique is a feasible alternative to endoscopic techniques. (Source: Journal of Vascular and Interventional Radiology : JVIR)

Follicular, Oocyte and Embryo Features Related to Metabolic Status in Primiparous Lactating does Fed with High-Fibre Rearing Diets

Reproduction in Domestic Animals — Tue, 24 Nov 2009 00:00:00 +0100

In conclusion, the enhancement of reproductive management by using highly lignified products in rearing diets does not seem to report physiological reproductive benefits affecting oocyte maturation rate and embryo viability in primiparous lactating does. (Source: Reproduction in Domestic Animals)

Routine microsurgical biliary reconstruction decreases early anastomotic complications in living donor liver transplantation

Liver Transplantation — Tue, 24 Nov 2009 00:00:00 +0100

In conclusion, routine MB is a technical innovation that leads to decreased early anastomotic complications in living donor liver transplantation. Liver Transpl 15:1766-1775, 2009. © 2009 AASLD. (Source: Liver Transplantation)

The Retrograde Aortic Arch in the Hybrid Approach to Hypoplastic Left Heart Syndrome [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Mon, 23 Nov 2009 19:29:54 +0100

Conclusions Clinically important RAAO occurred in 24% of the hypoplastic left heart syndrome patients in this series. If RAAO is detected at birth or early interstage, a Norwood operation is now favored. Palliative interventional catheterization remains very important mid and late interstage for continuing the hybrid strategy toward comprehensive stage 2. (Source: The Annals of Thoracic Surgery)

Sphenoid sinus development in choanal atresia

International Journal of Pediatric Otorhinolaryngology — Fri, 20 Nov 2009 14:12:45 +0100

Conclusions: In unilateral and bilateral choanal atresia patients, there is normal growth of the width of the sphenoid sinuses. (Source: International Journal of Pediatric Otorhinolaryngology)

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Sialodochostomy as treatment for imperforate submandibular duct: A systematic literature review and report of two cases

International Journal of Pediatric Otorhinolaryngology — Fri, 20 Nov 2009 14:12:41 +0100

Conclusions: Congenital floor of mouth cysts arising from imperforate salivary ducts are rare, but they may mimic the appearance of a ranula. Unlike ranulas, these cysts may be safely and effectively cured by simple sialodochostomy. To date, 26 other cases have been described in the literature, and nearly all have been successfully treated with simple incision or marsupialization without recurrence. Care should be taken to exclude ranula as a diagnosis, as incomplete excision may likely result in recurrence. (Source: International Journal of Pediatric Otorhinolaryngology)

Successful airway management with use of a laryngeal mask airway in a patient with CHARGE syndrome

Journal of Anesthesia — Tue, 17 Nov 2009 23:58:53 +0100

This report describes a case of CHARGE syndrome observed in a 6-year-old male patient with a difficult airway, in whom anesthesia was managed successfully using a laryngeal mask airway. Content Type Journal ArticleCategory Short CommunicationDOI 10.1007/s00540-009-0791-yAuthors Yusuke Hara, Kyoto University Hospital Department of Anesthesia 54 Shogoin-Kawaracho, Sakyo-ku Kyoto 6060-8507 JapanKiichi Hirota, Kyoto University Hospital Department of Anesthesia 54 Shogoin-Kawaracho, Sakyo-ku Kyoto 6060-8507 JapanKazuhiko Fukuda, Kyoto University Hospital Department of Anesthesia 54 Shogoin-Kawaracho, Sakyo-ku Kyoto 6060-8507 Japan Journal Journal of AnesthesiaOnline ISSN 1438-8359Print ISSN 0913-8668 Journal Volume Volume 23 Journal Issue Volume 23, Number 4 / November, 200...

Cerebral and cerebellar white matter abnormalities with magnetic resonance imaging in a child with Feingold syndrome

American Journal of Medical Genetics Part A — Mon, 16 Nov 2009 00:00:00 +0100

We present a patient who was found to have areas of cerebral and cerebellar white matter hyperintensity with T2 weighted magnetic resonance (MR) imaging. To the best of our knowledge, this finding has not been previously described. While the significance and pathologic basis of this finding are unknown, its recognition is important since it has potential to be confused with imaging findings in other conditions. Moreover, it is likely to be observed in the future due to increased use of MR imaging. © 2009 Wiley-Liss, Inc. (Source: American Journal of Medical Genetics Part A)

Usefulness of a scoring system in the interpretation of histology in neonatal cholestasis.

World Journal of Gastroenterology : WJG — Fri, 13 Nov 2009 14:46:26 +0100

CONCLUSION: A 7-feature, 15-point histological scoring system had good diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis. PMID: 19908342 [PubMed - in process] (Source: World Journal of Gastroenterology : WJG)

[Seminar] Biliary atresia

LANCET — Fri, 13 Nov 2009 00:00:00 +0100

Biliary atresia is a rare disease of infancy, which has changed within 30 years from being fatal to being a disorder for which effective palliative surgery or curative liver transplantation, or both, are available. Good outcomes for infants depend on early referral and timely Kasai portoenterostomy, and thus a high index of suspicion is needed for investigation of infants with persistent jaundice. In centres with much experience of treating this disorder, up to 60% of children will achieve biliary drainage after Kasai portoenterostomy and will have serum bilirubin within the normal range within 6 months. 80% of children who attain satisfactory biliary drainage will reach adolescence with a good quality of life without undergoing liver transplantation. Although much is known about managemen...

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The illusion of severe carpal tunnel syndrome (CTS)

Muscle and Nerve — Fri, 13 Nov 2009 00:00:00 +0100

In this report we present a young woman with mild bilateral CTS, based on electrophysiological studies, in whom marked thenar atrophy was on a congenital basis related to the VATER association (vertebral anomalies, anal atresia, tracheoesophageal fistula, and radial or renal abnormalities). Muscle Nerve, 2010 (Source: Muscle and Nerve)

The primordial pool of follicles and nest breakdown in mammalian ovaries

Molecular Human Reproduction — Thu, 12 Nov 2009 15:49:49 +0100

The creation of the pool of follicles available for selection and ovulation is a multi-faceted, tightly regulated process that spans the period from embryonic development through to the first reproductive cycle of the organism. In mice, this development can occur in mere weeks, but in humans, it is sustained for years. Embryonic germ cell development involves the migration of primordial germs cells to the genital ridge, and the mitotic division of germ cell nuclei without complete cytokinesis to form a multi-nucleated syncytia, or germ cell nest. Through combined actions of germ cell apoptosis and somatic cell migration, the germ cell nuclei are packaged, with surrounding granulosa cells, into primordial follicles to form the initial follicle pool. Though often dismissed as quiescent and p...

Health-related quality of life and its determinants in children and adolescents born with oesophageal atresia

Acta Paediatrica — Thu, 12 Nov 2009 00:00:00 +0100

Conclusion: In this first study describing health-related quality of life in children and adolescents born with oesophageal atresia, we demonstrated that general health remains impaired because of a high incidence of concomitant anomalies and gastrointestinal symptoms in patients with oesophageal atresia when compared with the healthy reference population. (Source: Acta Paediatrica)

The complete three-vessel view in prenatal detection of congenital heart defects

Prenatal Diagnosis — Thu, 12 Nov 2009 00:00:00 +0100

To evaluate the effectiveness of the complete three-vessel view (c3VV) in assessment of fetuses with prenatally diagnosed congenital heart defects (CHD).Fetuses with prenatal suspicion of CHD were prospectively recruited into the study. All examinations included a c3VV for assessment of abnormalities in alignment, arrangement, vessel size, number of vessels, aortic arch sidedness and color flow mapping. Definite diagnoses of CHD were based on neonatal cardiologic exams or postnatal autopsy.Of 106 consecutive fetuses with CHD, 75 were shown to have at least one abnormality on the c3VV, a detection rate of 70.8%. When septal wall defects were excluded, the detection rate increased to 89%. All cases of transposition of great arteries (TGA), aortic stenosis, pulmonary stenosis, truncus arterio...

Childhood Esophageal Atresia Linked to Adult Scoliosis

Modern Medicine — Thu, 12 Nov 2009 00:00:00 +0100

Adults with a history of esophageal atresia repair during infancy face a substantially higher risk of scoliosis, according to research published online Nov. 9 in Pediatrics. (Source: Modern Medicine)

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Lesion-specific outcomes in neonates undergoing congenital heart surgery are related predominantly to patient and management factors rather than institution or surgeon experience: A Congenital Heart Surgeons Society Study

The Journal of Thoracic and Cardiovascular Surgery — Thu, 12 Nov 2009 00:00:00 +0100

Conclusion: Survival of neonates with complex congenital heart disease is influenced more by patient and management factors than by institution or surgeon experience. Institutional excellence in managing some diagnostic groups does not indicate similar performance for all diagnostic groups. Weighted risk-adjusted comparisons could provide a mechanism to improve results in institutions with less than optimal outcomes. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia

Journal of Indian Association of Pediatric Surgeons — Tue, 10 Nov 2009 15:59:27 +0100

We report two such cases. Radiology showed dilated ileal segment and distal micro colon in both the cases. At laparotomy there was atresia of ileocecal junction in the first case and isolated ileocaecal valve atresia with normal ileocecal junction in the other case. Both the babies were managed by ileocolic resection with an end to end anastomosis. The prognosis of ileocecal atresias is satisfactory. (Source: Journal of Indian Association of Pediatric Surgeons)

Tetralogy of Fallot with Persistent Fifth Aortic Arch: Echocardiographic Diagnosis

Pediatric Cardiology — Tue, 10 Nov 2009 08:04:22 +0100

This report describes a new case of P5A in an infant with tetralogy of Fallot but not pulmonary atresia, which was diagnosed echocardiographically. The echo-Doppler characteristics of P5A in the presence of right ventricular outflow obstruction are described, and the implications regarding surgery for the accompanying defects are discussed. To the authors’ best knowledge, the described case is the first reported instance of P5A in this specific setting. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00246-009-9573-4Authors Gerard Holmes, Pediatrix Cardiology Associates of New Mexico 201 Cedar SE, Suite 700 Albuquerque NM 87106 USAJoanna L. Holmes, Pediatrix Cardiology Associates of New Mexico 201 Cedar SE, Suite 700 Albuquerque NM 87106 USAWilliam Berman, Pediatrix...

Two Multivitamin Preparations Ineffective for Biliary Atresia

Medscape PublicHealth Headlines — Wed, 04 Nov 2009 15:10:24 +0100

Commonly used multivitamin supplements containing fat-soluble vitamins fail to prevent fat-soluble vitamin deficiencies. Medscape Medical News (Source: Medscape PublicHealth Headlines)

Great vessel development requires biallelic expression of Chd7 and Tbx1 in pharyngeal ectoderm in mice

Journal of Clinical Investigation — Tue, 03 Nov 2009 15:54:21 +0100

Aortic arch artery patterning defects account for approximately 20% of congenital cardiovascular malformations and are observed frequently in velocardiofacial syndrome (VCFS). In the current study, we screened for chromosome rearrangements in patients suspected of VCFS, but who lacked a 22q11 deletion or TBX1 mutation. One individual displayed hemizygous CHD7, which encodes a chromodomain protein. CHD7 haploinsufficiency is the major cause of coloboma, heart defect, atresia choanae, retarded growth and development, genital hypoplasia, and ear anomalies/deafness (CHARGE) syndrome, but this patient lacked the major diagnostic features of coloboma and choanal atresia. Because a subset of CHARGE cases also display 22q11 deletions, we explored the embryological relationship between CHARGE and V...

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ARTICLE: Antibacterial Medication Use During Pregnancy and Risk of Birth Defects: National Birth Defects Prevention Study

Archives of Pediatrics — Mon, 02 Nov 2009 21:01:14 +0100

Conclusions Reassuringly, penicillins, erythromycins, and cephalosporins, although used commonly by pregnant women, were not associated with many birth defects. Sulfonamides and nitrofurantoins were associated with several birth defects, indicating a need for additional scrutiny. (Source: Archives of Pediatrics)

Antibacterial Medication Use During Pregnancy and Risk of Birth Defects: National Birth Defects Prevention Study [Article]

Archives of Pediatrics — Mon, 02 Nov 2009 21:01:14 +0100

Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: A 12-year single-center survey

The Journal of Thoracic and Cardiovascular Surgery — Mon, 02 Nov 2009 00:00:00 +0100

Conclusion: The Norwood operation can now be performed with low mortality. Patients with mitral stenosis/aortic atresia still constitute the most challenging subgroup. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction—an experimental study in growing animals

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

Conclusions: Hepatic fibrosis induced by bile duct ligation in young rats could be modulated by pharmacologic interventions. Administration of PTX or PRED, or the combination of both, resulted in diminished collagen-filled areas in portal spaces. (Source: Journal of Pediatric Surgery)

Tracheal diverticula after tracheoesophageal fistula repair: case series and review of the literature

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

Conclusion: We hypothesize that the diverticula impaired airway clearance and contributed to persistent respiratory symptoms. Possible mechanisms for the diverticulum contributing to poor airway clearance include facilitating the pooling of secretions and acting as a “barrier” to the lower airway clearance mechanism. The diagnosis of a diverticulum should be considered early in patients with persistent respiratory symptoms after management of GER and tracheomalacia. Early obliteration of tracheal diverticula might improve respiratory status in some patients. (Source: Journal of Pediatric Surgery)

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Staged reduction of gastroschisis using preformed silos: practicalities and problems

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

Conclusions: Staged reduction of gastroschisis with PFS is simple, convenient, and safe. The low rates of associated complications and mortality appear favorable when compared to infants managed with more traditional techniques. We recommend that PFS should be used for the routine management of gastroschisis. (Source: Journal of Pediatric Surgery)

Pharyngeal perforation masquerading as esophageal atresia

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

We present a case of pharyngeal perforation from a nasogastric tube mistakenly diagnosed as esophageal atresia with tracheoesophageal fistula in a full term infant female. The correct diagnosis was identified with rigid bronchoscopy and esophagoscopy immediately prior to the planned thoracotomy which was aborted. After one week of oral restriction and antibiotics, the healed perforation did not demonstrate leakage on a contrasted pharyngoesophagogram and bottle feeds were initiated. The infant was discharged to home on day of life 13 without any further complications. (Source: Journal of Pediatric Surgery)

Tripartite esophagus

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

We present a variation not previously described. (Source: Journal of Pediatric Surgery)

In utero diagnosis of congenital pyloric atresia in a single twin using MRI and ultrasound

Journal of Pediatric Surgery — Sun, 01 Nov 2009 00:00:00 +0100

Abstract: Congenital pyloric atresia (CPA) is a rare disorder that has traditionally been diagnosed in the postnatal period. With improvements in ultrasound technology and increasing use of MRI, CPA is now diagnosed with increasing accuracy in utero. This also allows for identification of concomitant anomalies, which greatly affects infant survival. In addition, antenatal diagnosis of CPA and associated anomalies allow for family counseling and planning of treatment for the perinatal period including early referral to a center with pediatric surgical services. Here, we present a case of CPA with preoperative diagnosis using ultrasound and MRI. (Source: Journal of Pediatric Surgery)