Pediatric and Developmental Pathology
This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader, such as GoogleReader, or to display this data on your own website or blog. 
Subscribe to this data using MyMedWorm.
Subscribe to this data using GoogleReader.
Subscribe to this data using Bloglines.
Subscribe to this data using MyYahoo.
Read news and analysis about clinical lab software and the clinical lab industry at the most widely read lab blog - Lab Soft News.
This page shows you the latest items in this publication.
Morphologic Observations in a Case of Lethal Variant (Type I) Metatropic Dysplasia with Atypical Features: Morphology of Lethal Metatropic Dysplasia
Email this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
In this report, we present the radiologic and histopathologic features in a case of type
I metatropic dysplasia, with the unusual features of a persistent tail, unique lung dysmorphology, and thyroidal agenesis.
Content Type Journal ArticleAuthors
M.J. O'Sullivan, Department of Pathology, St. Louis Children's Hospital, Washington University Medical Center, 660 S. Euclid Avenue, St. Louis, MO 63110, USA USW.H. McAllister, Department of Radiology, St. Louis Children's Hospital, Washington University Medical Center, 660 S. Euclid Avenue, St. Louis, MO 63110, USA USR.H. Ball, Department of Radiology, St. Louis Children's...
Source: Pediatric and Developmental Pathology - November 1, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Hodgkin-like Post-transplant Lymphoproliferative Disorder in Children: Does It Differ from Post-transplant Hodgkin Lymphoma?Email this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
In conclusion, HL-PTLD and HD appear to be two related but immunophenotypically and biologically distinct
forms of lymphoproliferation in post-transplant patients and may require different protocols for their management.
Content Type Journal ArticleCategory Original articleDOI 10.1007/s10024-003-9877-yAuthors
S. Ranganathan, Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine Department of Pathology 3705 5th Avenue Pittsburgh PA 15213 USAS. Webber, Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine Department of Cardiology Pittsburgh PA 15213 USAS. Ahuja, Chi...
Source: Pediatric and Developmental Pathology - April 11, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Very Low Birthweight Placenta: Clustering of Morphologic CharacteristicsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We examined 1146 placentas of live-born infants with a birth weight of 500–1500 g. We then conducted
analyses of pairs of characteristics and multiple characteristics to identify “associated groups” and “factors,” respectively.
We found an associated group and factor that had placental features associated with acute inflammation and another associated
group and factor that had features associated with vasculopathy. Acute umbilical vasculitis had the strongest correlation
with other features of the acute inflammation associated group and factor. Gross evidence of acute inflammation (opacification
and green ap...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
LETTER TO THE EDITOR: Lysozyme Immunostaining in Renal Tubular DysgenesisEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content Type Journal ArticleDOI 10.1007/s100249910025Authors
M. Moldavsky, Department of Pathology, Rebecca Sieff Government Hospital, Safed, Israel IL
Journal Pediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal Volume Volume 3
Journal Issue Volume 3, Number 2 / March, 2000
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
CURRENT PRACTICE IN PEDIATRIC PATHOLOGY: Peripheral Neuroblastic Tumors: Pathologic Classification Based on Recommendations of International Neuroblastoma Pathology Committee (Modification of Shimada Classification)Email this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content Type Journal ArticleDOI 10.1007/s100249910024Authors
Vijay V. Joshi, Department of Pediatric Pathology, Connecticut Children's Medical Center and Hartford Hospital, 80 Seymour Street, Hartford, CT 06102, USA US
Journal Pediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal Volume Volume 3
Journal Issue Volume 3, Number 2 / March, 2000
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Evaluation of a Neuraminidase Detection Assay for the Rapid Detection of Influenza A and B Virus in ChildrenEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT A prototype version of a new diagnostic assay for influenza A and B (Zstat Flu™) based on detection of viral neuraminidase
was evaluated and compared to culture in 196 clinical samples. Children with respiratory illnesses were prospectively evaluated
at a pediatrician's office and at a large children's hospital using the neuraminidase assay and viral culture performed on
respiratory secretions. Influenza virus was isolated from 51 samples and 83 were positive by the neuraminidase assay. When
compared to culture the sensitivity of the assay was 96%, specificity was 77%, positive predictive value w...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Clinical and Histopathological Study of Merosin-deficient and Merosin-positive Congenital Muscular DystrophyEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
In this study, the clinical and histopathological findings of 39 merosin-deficient and
37 merosin-positive CMD patients were compared. Merosin-deficient CMD patients were found to be younger, with earlier onset
of symptoms, age of diagnosis, and a more severe clinical state (reflecting maximum motor capacity and contractures). On histopathological
evaluation, endomysial fibrosis, perimysial fibrosis, and histopathological state (reflecting fibrosis, adiposis, necrosis,
and variation in fiber size) were more severe in merosin-deficient CMD. There was a correlation between clinical and histopathological
states only in m...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
PERSPECTIVES IN PEDIATRIC PATHOLOGY: Progressive Familial Intrahepatic Cholestasis: A Personal PerspectiveEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT Progressive familial intrahepatic cholestasis (PFIC), originally described as “Byler disease” in an Amish kindred, has been
distinguished from other forms of cholestatic liver disease in childhood by clinical findings, clinical–laboratory observations,
and morphologic studies in biopsy, hepatectomy, and autopsy specimens. Correlation with genetic analyses has permitted both
more precise definition of PFIC and distinctions within PFIC. Two types of PFIC now are recognized: PFIC-1, resulting from
mutations in a gene called FIC1 (familial intrahepatic cholestasis, type 1), and PFIC-2, resulting ...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
ORIGINAL ARTICLES: Definitive Classes of Childhood Supratentorial Neuroglial TumorsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
In this study, we used the scores on the factors in cluster analyses and identified
eight classes of neuroglial tumors. Each of these classes had significant differences in histology, allowing the separation
of many of the conventional types of neuroglial tumors into two or more classes. For instance, fibrillary astrocytoma, pilocytic
astrocytoma, subependymal giant cell astrocytoma, anaplastic astrocytoma, oligodendroglioma, and ependymoma were represented
in two or more classes. Often these classes had statistically significant differences in survival distributions. For instance,
the two classes of “anaplastic ast...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Light Microscopic, Immunophenotypic, and Molecular Genetic Study of Autoimmune Lymphoproliferative Syndrome Caused by
fas
MutationEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT This case provides a complete light microscopic, immunophenotypic, and molecular genetic analysis of autoimmune lymphoproliferative
syndrome (ALPS), a rare benign cause of dramatic lymphadenopathy with atypical histology and phenotype that may be mistaken
for malignancy. The patient is 3-year-old child who was first clinically evaluated at the age of 16 months because of marked
generalized lymphadenopathy and hepatosplenomegaly. Histologic sections of a cervical lymph node demonstrated a marked paracortical
proliferation of occasional small and intermediate-sized lymphocytes and numerous large immu...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Juxtaposed Cystic Nephroma and Wilms' TumorEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We report a case of juxtaposed Wilms' tumor (WT) and cystic nephroma (CN) in a 21-month-old girl which gave rise to radiological
diagnostic difficulty. Preoperative chemotherapy was given, resulting in marked tumor necrosis but the cystic nephroma remained
untouched. Histological examination showed characteristic features of a triphasic WT and a CN; the two lesions were separated
by a thick fibrous capsule. While everybody agrees that WT and cystic partially differentiated nephroblastoma (CPDN) are closely
related, there are two opposite views about their relationship to CN. One is that CN may represent the final step ...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Phosphaturic Mesenchymal Tumor-Induced RicketsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We describe two prepubertal girls with oncogenic rickets. The first patient, 9 years of age, presented with recent-onset
lower-extremity pain. The second girl, presented at 4 years of age following a 9-month period of muscle weakness, bone pain,
and poor linear growth. Laboratory analyses in both patients revealed hypophosphatemia and hyperphosphaturia; elevated circulating
alkaline phosphatase activity was present in one of them. Radiographic evidence of a generalized rachitic process was evident
in both cases. Computerized tomography of the paranasal sinuses and facial bones in patient 1 revealed a small lesion erodi...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Occult Pulmonary Synovial Sarcoma Confirmed by Molecular TechniquesEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We report a unique case of a minute, occult synovial sarcoma of the lung detected intraoperatively during a pneumothorax
repair in a 17-year-old boy. No alternative primary site could be detected upon complete body imaging studies and physical
examinations. The diagnosis was confirmed by demonstration of the characteristic SYT/SSX gene fusion by reverse transcriptase
polymerase chain reaction (RT-PCR) performed upon RNA extracted from the paraffin block of the biopsy. This case demonstrates
the utility of this technique in diagnostic pathology.
Content Type Journal ArticleDOI 10.1007/s100249910011Authors
Pedram Ar...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Prognostic Value of Pre- and Postoperative Cardiac Troponin I Measurement in Children Having Cardiac SurgeryEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
The objective of this study was to determine if perioperative elevation of cardiac troponin I (cTnI) predicts mortality in
infants and children after surgical correction of congenital heart defects. One hundred infants and children having open heart
surgery were studied. Blood samples for cTnI analysis were collected before cardiopulmonary bypass (CPB) and at 4, 8, 12,
and 24 h after initiation of CPB. Demographic information, cardiac defect, repair performed, duration of CPB, complications,
and outcome were recorded. Cardiac defects were categorized as atrial septal defect (ASD), ventricular septal defect (VSD),
hypo...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Bile Acid Synthetic Defects and Liver DiseaseEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content Type Journal ArticleDOI 10.1007/s100249910001Authors
Kevin E. Bove, Division of Pediatric Pathology, Children's Hospital Medical Center, and the University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA USCynthia C. Daugherty, Division of Pediatric Pathology, Children's Hospital Medical Center, and the University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA USWes Tyson, Department of Pathology, Denver Children's Hospital, 1056 East 19th Avenue, Denver, CO 80218, USA USGary Mierau, Department of Pathology, Denver Children's Hospital,...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Project Pró-Natal: Population-based Study of Perinatal and Infant Mortality in Natal, Northeast BrazilEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT The Pró-Natal project is a collaborative initiative that aims to improve maternal and infant health in a deprived community
in Natal, Northeast Brazil. To assess the perinatal and infant mortality in this population of 40,000, we have collected over
a 2-year period a consecutive series of 39 autopsy examinations on deaths under 1 year of age. During this period there were
2212 live births in the study population. The 14 perinatal deaths are described using the Wrigglesworth classification, and
the 25 infant deaths, using a clinicopathological system. The contribution of normally formed stillbirths...
Source: Pediatric and Developmental Pathology - March 21, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Significance of Lymphoid Follicles and Aggregates in Gastric Mucosa of ChildrenEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
This study was designed to evaluate the significance of gastric lymphoid follicles (LF) and aggregates (LA) in children with
and without Helicobacter pylori (HP) infection. All 605 antrum biopsies performed during 1994 were reviewed and classified according to the presence or absence
of inflammation, LF, or LA. HP was searched with a DiffQuik stain in all biopsies showing gastritis, LF, or LA. Gastritis
was diagnosed in 80 biopsies (16 with LF, 18 with LA and 46 without LA or LF). Identification of HP in these biopsies was
as follows: (a) cases with LF: 12/16; (b) cases with LA: 3/18; (c) cases without LF or LA: 8/46. ...
Source: Pediatric and Developmental Pathology - February 18, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Congenitally Malformed Hearts from a Population of Children Undergoing Cardiac Transplantation: Comments on Sequential Segmental Analysis and DissectionEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We describe in detail the congenital
malformations found in 65 cardiac explants acquired from a population of children over a period of 15 years. The specimens
were examined and diagnosed using the method of sequential segmental analysis. The most prevalent type of cardiac malformation
was severe obstruction of the left heart (29.2%), followed by double-outlet right ventricle (15.4%), complete transposition
(13.8%), hearts with left-hand ventricular topology (10.8%), ventricular septal defect(s) (9.2%), tricuspid valvar agenesis
(4.6%), and tetralogy of Fallot (4.6%). These abnormalities accounted for 87.6% of the spe...
Source: Pediatric and Developmental Pathology - February 18, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Partial Molar Transformation of the Placenta of Presumably Monozygotic TwinsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT A pregnancy with one normal female fetus and a placenta that was divided into halves, one normal the other molar, is described.
Genetic analysis shows the molar component to be hyperdiploid/tetraploid but having an identical DNA composition as the normal
twin. Because there was no trophoblastic proliferation and the hyperdiploid cells were confined to the villous stroma, and
because the molar component was still being perfused by diploid vessels from the normal twin, we believe the mole is derived
from polyploidization of the mesenchymal epiblast in a monozygotic twin pregnancy.
Content Type Jou...
Source: Pediatric and Developmental Pathology - February 18, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Immunohistochemical Detection of Myocardial Necrosis in Stillbirth and Neonatal DeathEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT The aims of this study were to determine whether immunohistochemical staining for C9 can demonstrate myocardial necrosis
in the fetus and neonate. Hearts from cases of stillbirth or neonatal death with confirmed myocardial necrosis (in neonates)
or with ischemic lesions outside the heart (in neonates and stillborns) were stained immunohistochemically with antibodies
to C9. All five cases with confirmed myocardial infarction showed positive immunohistochemical staining for C9, largely localized
to the infarcted areas. The youngest subject was born at 24 weeks gestation and died at 4 days of age. One...
Source: Pediatric and Developmental Pathology - February 18, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Normal Hepatic Growth: Study of the Proliferative Capacity and Apoptosis of the Normal Liver during ChildhoodEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT We explored the mechanism of normal hepatic growth in children by evaluating the proliferation index (with Ki-67 antibody)
and apoptosis index (with the ApopTag™ kit). The proliferative index is almost constant, but the level of apoptosis is very
low in childhood and increases with age. Thus the growth of the liver in children is not due to a high proliferation index,
but to a very low apoptosis index.
Content Type Journal ArticleDOI 10.1007/s100249910009Authors
D. Gheban, Pathology Department, University of Medicine and Pharmacy, Str. Peana nr. 18, bl. V6, ap. 4, 3400 Cluj-Napoca, Romania RO...
Source: Pediatric and Developmental Pathology - February 18, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Letters to the EditorEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content Type Journal ArticleDOI 10.1007/s100249910015Authors
Arthur G. Weinberg, Department of Pathology, Children's Medical Center, Dallas, TX, USA USGeorge Reed, Monterey, CA, USA USWilliam Q. Sturner, University of Arkansas for Medical Sciences, Arkansas State Crime Laboratory, Little Rock, AR, USA US
Journal Pediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal Volume Volume 3
Journal Issue Volume 3, Number 1 / January, 2000
Source: Pediatric and Developmental Pathology - February 18, 2008 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Subchorionic Hematoma Associated with Thrombophilia: Report of Three CasesEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content Type Journal ArticleDOI 10.1007/s10024-002-0107-9Authors
Debra S. Heller, University of Medicine and Dentistry of New Jersey–New Jersey Medical School Department of Pathology UH/E158 185 South Orange Avenue Newark NJ 07103 USADemaretta Rush, Weill Medical College of Cornell University Department of Pathology Ithaca New York USARebecca N. Baergen, Weill Medical College of Cornell University Department of Pathology Ithaca New York USA
Journal Pediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal Volume Volume 6
Journal Issue Volume 6, Number 3 / June, 2003
Source: Pediatric and Developmental Pathology - September 23, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Beta-Cell Hyperplasia in Macrosomic Infants and Fetuses of Nondiabetic MothersEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
The objective of this study was to test the hypothesis that macrosomic infants of nondiabetic mothers have beta-cell hyperplasia
in their pancreases. Pancreatic tissues were examined from 10 macrosomic fetuses and liveborn infants and from 10 comparison
cases matched for gestational age and gender. None of the mothers had a history of diabetes and all had normal glucose screening
during pregnancy. Tissues were stained with hematoxylin and eosin and a monoclonal antibody against beta cells and were analyzed
using an image analysis program to evaluate the size and surface area of beta-cell clusters. Brain/liver weight ra...
Source: Pediatric and Developmental Pathology - September 5, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Infantile GM1 Gangliosidosis: Complete Morphology and Histochemistry of Two Autopsy Cases, with Particular Reference to Delayed Central Nervous System MyelinationEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT Inborn metabolic errors causing lysosomal storage, such as β-galactosidase deficiency (GM1 gangliosidosis [GM1]), have well-recognized effects on cellular function and morphology. In some classically “neuronal” storage diseases, including
GM1, neuroradiologic observations of infants have suggested a delay in myelination on the basis of persistently “immature” signal
intensities monitored over time. We sought to evaluate in a semiquantitative fashion the pattern and degree of myelination
in two infantile GM1 patients, one boy and one girl, autopsied at 15 months of age. We assigned myelinati...
Source: Pediatric and Developmental Pathology - September 5, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Morphometric Analysis of Preterm Fetal Pulmonary Development in the Sheep Model of Congenital Diaphragmatic HerniaEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
This study
aimed to quantitate structural differences between normal and CDH-affected preterm lamb lungs. We hypothesized that (a) pulmonary hypoplasia is present in preterm CDH-affected lungs; (b) the relative degree of pulmonary hypoplasia increases with gestation; and (c) the left upper lobe (LUL) is affected most. Fetal lambs were allocated to two groups. One group underwent surgery (72–74
days gestation) inducing CDH. Both groups (n = 7, n = 7) were delivered by cesarean section at 129 days (term: 145–149). Lungs were obtained at autopsy, were inflation-fixed,
processed for histology, and morphometry wa...
Source: Pediatric and Developmental Pathology - September 5, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Bannayan-Riley-Ruvalcaba Syndrome: Spectrum of Intestinal Pathology Including Juvenile PolypsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We describe the intestinal
pathology in multiple specimens from three children with BRRS. Examination of gastrointestinal biopsies from these children
revealed predominantly colonic and rectal polyps with the histology of juvenile polyps. Additionally, two cases with clusters
of ectopic ganglion cells within the lamina propria, one in a colonic polyp and one in a duodenal biopsy, and an atypical
polyp were observed. Bannayan-Riley-Ruvalcaba syndrome should be included in the list of differential diagnostic considerations
when a child or young adult presents with a juvenile polyp, particularly if unusual histologic fea...
Source: Pediatric and Developmental Pathology - September 5, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
CASE REPORT: Novel Point Mutation (W184R) in Neonatal Type 2 Gaucher DiseaseEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
ABSTRACT Gaucher disease is the most prevalent inherited sphingolipidosis and results from deficient glucocerebrosidase activity.
Three clinical forms of Gaucher disease have been described: type 1, or non-neuronopathic; type 2, or acute neuronopathic;
and type 3, or subacute neuronopathic. We have identified a novel mutation in a patient of Russian-British descent who died
of type 2 Gaucher disease a few hours after birth. A heterozygous T → C transition mutation in exon 6, cDNA nucleotide position
667, results in the substitution of tryptophan by arginine at amino acid residue 184 (W184R) of glucocerebr...
Source: Pediatric and Developmental Pathology - September 5, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Letter to the EditorEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
PDP insightsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Clinical guide to inherited metabolic diseasesEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Extrapleural benign solitary fibrous tumor in the shoulder of a 9-year-old girl: Case report and review of the literatureEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We report a case of a benign solitary fibrous tumor that occurred in the right shoulder of a 9-year-old girl. This case is
remarkable due to the unusual location of its occurrence and the young age of the patient. In addition, cytogenetic analysis
revealed a karyotype unreported in this neoplasm: 46,XX,der(4)t(4;9)(q31.1;q34), del(9)(p22p24),der(9)t(4;9)(q31.1;q34)ins(9;?)(q34;?)(17
cells)/46,XX (3 cells).
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Systemic form of juvenile xanthogranuloma: Report of a case with liver and bone marrow involvementEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Abstract Systemic form of juvenile xanthogranuloma with involvement of liver and bone marrow is reported in a 2-month-old female infant
who presented with hepatosplenomegaly, severe anemia, and thrombocytopenia. There was no skin lesion, nor bone lesion. The
enlarged liver has generalized yellowish spots. The diagnosis of juvenile xanthogranuloma was made by pathologic findings
of marrow and portal tract infiltration by S-100 negative, CD1a negative, CD68 positive, and Factor XIIIa positive large pale
to foamy histiocytes with Touton giant cells, and lack of Langerhans cell granule by electron microscopic ex...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Mitochondrial muscle pathologyEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Summary The diagnosis of mitochondrial disease is challenging and requires a multidisciplinary approach for investigation. Despite
the complexity, a specific diagnosis can be achieved in many cases. A specific diagnosis may provide prognostic information
for the patient and the option of prenatal testing for the family in a subsequent pregnancy. the case reports in this section
nicely illustrate several common pediatric clinical presentations of mitochondrial disease and a systematic approach to investigation.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-574...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Conjugated hyperbilirubinemia in infancy (mitochondrial DNA depletion syndrome, liver)Email this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Neonatal mitochondrial cardiomyopathyEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Langerin (CD207) staining in normal pediatric tissues, reactive lymph nodes, and childhood histiocytic disordersEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We describe the distribution of staining in autopsy pediatric tissues, dermatopathic and other reactive lymph nodes,
and childhood histiocytic lesions using the 12D6 antibody (Novocastra). We also correlate CD1a (antibody O1O) staining to
these factors. Langerin on epidermal LCs has a coarsely granular cell membrane and a cytoplasmic staining pattern that is
always associated with CD1a expression. All 6 skin samples had Langerin+/CD1a+ LCs within the epidermis. Six of 8 thymuses showed single scattered dendriticshaped cells in the medulla and rare cells within
Hassall corpuscles that coexpressed Langerin and CD1a. Cort...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Subclassification of anencephalic human fetuses according to morphology of the posterior cranial fossaEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Abstract Anencephaly is a designation for congenital absence of the cranial vault with cerebral hemispheres completely missing or decreased
to small masses attached to the base of the skull. The etiology is unknown. Whether the bony tissue or soft brain tissue is
a primary factor is also unknown. The present study has focused on the posterior cranial fossa in anencephaly. The goal is
to determine whether differences in the posterior cranial fossa could provide a basis for subclassification of anencephalic
fetal skeletons. Twenty-three human anencephalic fetuses, at gestational ages 13 to 22 weeks, were studi...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Sclerosing rhabdomyosarcomas in children and adolescents: A clinicopathologic review of 13 cases from the intergroup rhabdomyosarcoma study group and children’s oncology groupEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Abstract In recent reports, investigators have described a variant of adult sclerosing rhabdomyosarcoma (RMS) that is characterized
by a hyalinizing, matrix-rich stroma. To determine whether this variant occurs in children we investigated this phenomenon
in a recent series of 1207 pediatric patients who had RMS accessioned by the Intergroup Rhabdomyosarcoma Study Group, now
part of Children's Oncology Group. Thirteen patients had features of sclerosing RMS; 9 had been diagnosed with alveolar RMS
(ARMS), 3 with embryonal RMS (ERMS),FKHR). Cytogenetic testing in 2 patients who had ARMS-like foci demonstrated m...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Heat-accelerated fixation and rapid dissection of the pediatric brain at autopsy: A pragmatic approach to the difficulties of organ retentionEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Abstract We investigated whether it is possible to accelerate the examination of a pediatric brain at autopsy and thus facilitate its
return to the body before a fueral without compromising the quality of the neuropathologic examination. Accelerated fixation
and next-day dissection of the brain was performed in selected cases over a 2-year period by using a microwave histologic
tissue processor (MicroMed T/T MEGA, Milestone, Sorisole, Italy). Direct comparison of the histolotic appearance and immunohistochemical
reactivity of 2 cases, 1 fixed by conventional methods and 1 fixed with the accelerated method, w...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Expression of WT-1, Bcl-2, and CD34 by primary renal spindle cell tumors in childrenEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Abstract The confident diagnosis of renal spindle cell tumors in children is often difficult. An immunohistochemical study of WT-1,
Bcl-2, and CD34 was performed to determine their expression profiles and to assess the potential utility of these immunohistochemical
markers in the differential diagnosis of 36 cases of renal spindle cell tumors of childhood. The cases included 11 stromal
predominant Wilms tumors, 12 cellular mesoblastic nephromas, 9 clear cell sarcomas of the kidney (CCSK), and 4 monophasic
synovial sarcomas. WT-1 was uniformly positive in primitive undifferentiated stromal Wilms tumors (6 of ...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Expression of laminin and fibronectin in renal dysplasiaEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
In this study, the expressions of adhesive glycoproteins, laminin, and fibronectin were studied immunohistochemically
in 25 autopsy cases of renal dysplasia and normal age-matehed control cases. These cases of renal dysplasia were categorized
into 3 groups based on the period of gestation: 20 to 26 weeks, 17 to 33 weeks, and 34 to 40 weeks. The immunohistochemical
findings were graded from 0 to 4+ based on the visual intensity. Chi-square analysis was used to calculate the difference
in expressions of laminin and fibronectin in cases and controls as a whole and within and between age groups. Immunostaining
for laminin...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Reply to Dr. Sredni and colleaguesEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Biochemical approach to the investigation of pediatric mitochondrial diseaseEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Pediatric mitochondrial disease: Do we have the energy to make the diagnosis?Email this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Conclusions It is impossible for the anatomic or clinical pathologist to reliably “rule out mitochondrial disease”. Nevertheless, we should
gather the energy to work with our clinical colleagues and those in the research arena to better define these diseases over
the next decade. One needs only to look at the progress made in peroxisomal diseases and fatty oxidation defects to know that
such collaboration would lead to a better understanding of these complicated disorders [19].
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Jou...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Pentalogy of cantrell: A case report with pathologic findingsEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We present the case of a 28-h-old female infant born at 37 weeks of gestation with a rare congenital malformation consisting
of a pentad of findings: ectopia cordis, a midline supraumbilical wall defect, a defect of the lower sternum, absent pericardium,
and an anterior diaphragmatic defect. This constellation of defects is known as the pentalogy of Cantrell. Additional autopsy
findings included a bilateral cleft lip and palate, bilateral pulmonary hypoplasia, an atrial septal defect, and a patent
ductus arteriosus. We present this case because of its rarity and discuss the pathologic findings.
Content TypeJournal A...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Congenital pulmonary airway malformation (congenital cystic adenomatoid malformation) with multiple extrapulmonary anomalies: Autopsy report of a fetus at 19 weeks of gestationEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
We present a unique case of type 2 congenital pulmonary airway malformation with a previously undocumented combination of
multiple extrapulmonary anomalies, featuring ipsilateral multicystic renal dysgenesis, contralateral renal agenesis, and ovarian
germ cell hypoplasia, diagnosed in a 19-week gestational age fetus by autopsy. Epithelial cells comprising the pulmonary lesions
were positive for thyroid transcription factor-1, surfactant protein-B, and cytokeratin-7 but negative for cytokeratin-20
immunostainings, with the pattern seen in normal terminal bronchioles. Chromosomal analysis showed a normal female karyotype...
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Intrathyroidal branchial cleft-like cyst with heterotopic salivary gland-type tissueEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 7
Journal IssueVolume 7, Number 6 / December, 2004
Source: Pediatric and Developmental Pathology - June 7, 2007 Category: Pathology Tags: Pediatric and Developmental Pathology Source Type: journals
Myongenic Markers in the Evaluation of Embryonal Botryoid Rhabdomyosarcoma of the Female Genital TractEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 8
Journal IssueVolume 8, Number 6 / December, 2005
Source: Pediatric and Developmental Pathology - December 21, 2005 Category: Pathology Source Type: journals
Epilepsy-Induced Microarchitectural Changes in the BrainEmail this article to a colleague.
Save this article to My Clippings.
Discuss or comment on this article.
In this report, the neuropathology of other conditions associated with intractable epilepsy is considered, suggesting that in them similar epilepsy-produced alterations in microarchitecture can be observed. The idea is important to define the optimum time for epilepsy surgery and the underlying etiology of these seizure-genic lesions.
Content TypeJournal Article
JournalPediatric and Developmental PathologyOnline ISSN 1615-5742Print ISSN 1093-5266
Journal VolumeVolume 8
Journal IssueVolume 8, Number 6 / December, 2005
Source: Pediatric and Developmental Pathology - December 7, 2005 Category: Pathology Source Type: journals
