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Pediatric and Developmental Pathology

Isabelle Viel-Thériault, Jeremy Saban, Alana Lewis, Erika Bariciak, David Grynspan
Bacillus cereus is a ubiquitous Gram-positive rod seldom considered pathogenic in clinical isolates. However, it possesses multiple virulence factors explaining why it has been linked to fulminant and pyogenic infections in vulnerable hosts. Its recovery from sterile samples in immunocompromised patients cannot be disregarded. Premature infants would fall into this category. We describe the case of a neonate born at 26 weeks of gestational age, who died of a rapidly progressive B. cereus necrotizing pneumonia following suspected nosocomial acquisition...
February 13, 2019: Pediatric and Developmental Pathology
Diana K Sung, Rebecca N Baergen
INTRODUCTION: Chorangiosis is a proliferation of capillaries in terminal chorionic villi and is considered to be a marker for hypoxia and poor clinical outcome. Not all cases with hypervascular villi meet the generally accepted diagnostic criteria as reported by Altshuler. Our aim was to evaluate cases with villous hypervascularity that do not meet the diagnosis of chorangiosis, in which increased vascularity was present in a significant portion of the villous tissue but was not a diffuse process, which we call focal chorangiosis, to ascertain whether there were clinical or pathologic associations...
February 13, 2019: Pediatric and Developmental Pathology
Amy Ruffle, Malcolm Cameron, Nicolaas Jonas, Samantha Levine, Caroline Mills, C Elizabeth Hook, Matthew J Murray
Alveolar soft part sarcoma (ASPS) is an exceptionally rare non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), characterized by the translocation t(X;17) p(11.2;q25). This translocation results in the chimeric ASPSCR1-TFE3 transcription factor which drives tumorigenesis. Complete surgical resection is crucial in allowing a successful outcome in these cases. Here, we describe an 11-month-old female infant who presented with a well-circumscribed lesion of the tongue, with the clinical and radiologic appearances of an infantile hemangioma...
February 11, 2019: Pediatric and Developmental Pathology
Hao Wu, William Ferguson, Eumenia Castro, Debra Kearney, Milton Finegold, Kalyani Patel
Autopsy reports of 78 stillbirths and early infant deaths (up to age 8 weeks) were reviewed to investigate the prevalence of extrahepatic nonreticuloendothelial siderosis (EHNRS) in the context of neonatal liver failure. Of these, 10 liveborns (12.8%), M:F 3:2, with mean gestational age 37.6 weeks (range: 35-39) and mean age at the time of demise 19.1 days (range: 7-42), showed significant liver injury: infection (n = 7, viral > fungal), congenital malformations (n = 2), and ischemia (n = 1)...
February 5, 2019: Pediatric and Developmental Pathology
Adrian Dc Chan, David Grynspan, Robert P Jankov
No abstract text is available yet for this article.
February 2, 2019: Pediatric and Developmental Pathology
Gina M Gallucci, Ming Tong, Xiaodi Chen, Barbara S Stonestreet, Amy Lin, Suzanne M de la Monte
BACKGROUND: Perinatal ischemia-reperfusion (I/R) injury of cerebral white matter causes long-term cognitive and motor disabilities in children. I/R damages or kills highly metabolic immature oligodendroglia via oxidative stress, excitotoxicity, inflammation, and mitochondrial dysfunction, impairing their capacity to generate and maintain mature myelin. However, the consequences of I/R on myelin lipid composition have not been characterized. OBJECTIVE: This study utilized matrix-assisted laser desorption/ionization mass spectrometry (MALDI-MS) to assess alterations in cerebral supraventricular white matter myelin lipid profiles in a fetal sheep model of perinatal I/R...
January 25, 2019: Pediatric and Developmental Pathology
Beata Hargitai, Gergely Toldi, Tamas Marton, Velmurugan Ramalingam, Andrew K Ewer, Alison R Bedford Russell
A rare complication of umbilical venous catheter (UVC) insertion is the extravasation of the infusate into the peritoneal cavity. We report 3 cases of abdominal extravasation of parenteral nutrition (PN) fluid via UVCs. Two of these cases presented as "acute abdomen" which were assumed to be necrotizing enterocolitis clinically; however, during postmortem, PN ascites and liver necrosis were found. A further case is described in an infant with congenital diaphragmatic hernia. While we were unable to ascertain direct vessel perforation by the catheter in any of these cases, based on pathological and histological examination, the proposed mechanism of PN fluid extravasation is leakage through microinjuries of liver vessel walls and necrotic parenchyma...
January 25, 2019: Pediatric and Developmental Pathology
Elena Daoud, David Zwick
Noonan syndrome is a genetic condition with a heterogeneous phenotype and multisystem involvement. The pathogenesis of this disorder has been attributed to the mutations in the RAS/MAPK signaling pathway involved in cell proliferation and differentiation. The most common clinical presentations are related to cardiovascular abnormalities with congestive heart failure as the most common mechanism of death. We present the autopsy findings from a Noonan syndrome patient who died as a result of an unusual form of right ventricular obstruction associated with a rare PTPN11 variant previously reported without details of the cardiac findings...
January 21, 2019: Pediatric and Developmental Pathology
Philip J Katzman, Joseph Blitman, Leon A Metlay
BACKGROUND: Hypertensive disorders of pregnancy (HDP) are a common cause for preterm delivery. Prior studies showed that chronic villitis (CV) is associated with intrauterine growth restriction, preeclampsia, intrauterine fetal death, and morbidly adherent placenta (MAP). The authors hypothesize that disorders of the placental basal plate, especially basal chronic villitis (BCV), are associated with HDP. METHODS: The laboratory information system was queried over 12 years to identify placentas with or without the clinical history of HDP and with or without multifocal/focal CV or BCV...
January 21, 2019: Pediatric and Developmental Pathology
Lara Berklite, Selma F Witchel, Svetlana A Yatsenko, Francis X Schneck, Miguel Reyes-Múgica
45,X/46,XY mosaicism is one of a heterogenous group of congenital conditions known as differences (disorders) of sex development (DSD) that results in abnormal development of internal and external genitalia. Patients with DSD, particularly those with segments of the Y chromosome, are at increased risk for germ cell tumors including gonadoblastoma. Gonadoblastoma is a neoplasm comprised of a mixture of germ cells and elements resembling immature granulosa or Sertoli cells with or without Leydig cells or lutein-type cells in an ovarian type stroma...
January 15, 2019: Pediatric and Developmental Pathology
Nino Rainusso, Victor Seghers, Rachel Egler, John Hicks, Heidi V Russell
Children with neuroblastoma rarely present with metastatic disease without identifiable primary tumors. We describe the clinical and histopathologic characteristics of 4 patients aged 1, 7, 7, and 11 years with neuroblastoma involving bone or bone marrow without an apparent primary site. One patient presented with a periorbital bone lesion, 1 presented with a distal femoral lesion, and 2 presented with diffuse bone marrow involvement. All tumors were negative for MYCN amplification. All patients were alive without evidence of disease 5 years after completion of multimodality therapy...
January 2, 2019: Pediatric and Developmental Pathology
Karen M Chisholm, Christopher Denton, Sioban Keel, Amy E Geddis, Min Xu, Burton E Appel, Alan B Cantor, Mark D Fleming, Akiko Shimamura
Germline mutations in RUNX1 result in autosomal dominant familial platelet disorder with associated myeloid malignancy (FPDMM). To characterize the hematopathologic features associated with a germline RUNX1 mutation, we reviewed a total of 42 bone marrow aspirates from 14 FPDMM patients, including 24 cases with no cytogenetic clonal abnormalities, and 18 with clonal karyotypes or leukemia. We found that all aspirate smears had ≥10% atypical megakaryocytes, predominantly characterized by small forms with hypolobated and eccentric nuclei, and forms with high nuclear-to-cytoplasmic ratios...
January 2, 2019: Pediatric and Developmental Pathology
Anthony I Squillaro, Shengmei Zhou, Stefanie M Thomas, Eugene S Kim
Precocious puberty in an infant is an alarming and infrequent finding, making the differential diagnosis difficult for practitioners. Precocious puberty secondary to a sclerosing stromal tumor (SST) of the ovary is rare. We present a case of a child that began precocious puberty at 3 months of age including development of breast buds, pubic hair, growth spurt, and menarche 5 days prior to presenting to pediatric endocrinology at 10 months. She underwent right salpingo-oophorectomy which demonstrated a soft tissue mass occupying almost the entire ovary with a tan-pink fleshy cut surface...
December 21, 2018: Pediatric and Developmental Pathology
Pamela Singer, Christine B Sethna, Laura Castellanos-Reyes, Oksana Yaskiv, Vanesa Bijol
A 7-year-old boy with a history of febrile illness-related epilepsy syndrome presented with proteinuria and elevated creatinine. His severe epileptic disorder has been treated since age 2 with multiple antiepileptic medications, including valproic acid. More recently, he was noted to have features of Fanconi syndrome with acidosis, hypophosphatemia, hypokalemia, glucosuria, and nephrotic-range proteinuria. This was managed with supplements; however, in the setting of rising creatinine and prominent proteinuria, a kidney biopsy was performed...
December 19, 2018: Pediatric and Developmental Pathology
Elaine S Chan, Julie Lauzon, Lothar Resch, Bob Argiropoulos, Laura Schmitt, Rati Chadha
Alpha thalassemia major is a hemoglobinopathy caused by the inactivation or deletion of all 4 α-globin alleles. We describe a case of α-thalassemia major with atypical ultrasound and neuropathological findings. The mother had her first prenatal visit at 27 4/7 gestational weeks. Ultrasound revealed a hydropic fetus with multiple anomalies. However, the middle cerebral artery peak systolic velocity (MCA-PSV) suggested that the likelihood of fetal anemia was low. Given the poor prognosis of hydrops fetalis, the parents opted for the termination of pregnancy...
December 14, 2018: Pediatric and Developmental Pathology
Raj P Kapur, Michael A Arnold, Miriam R Conces, Lusine Ambartsumyan, Jeffrey Avansino, Marc Levitt, Richard Wood, Kelley J Mast
BACKGROUND: After pullthrough surgery for Hirschsprung disease (HSCR), Glut1-positive submucosal nerve hypertrophy is used to diagnose retained transition zone in the neorectum. We hypothesized that pelvic nerves, severed during pullthrough surgery, sprout into the neorectum to mimic transition zone. METHODS: The density (nerves/100x field) and maximum diameter of Glut1-positive submucosal nerves were measured in biopsies and redo resections from 20 patients with post-pullthrough obstructive symptoms...
December 12, 2018: Pediatric and Developmental Pathology
Emma Grottling, David Gisselsson
The aim of this study was to examine the prevalence of embryologic remnants in umbilical cords of different gestational ages. Sections from 392 umbilical cords were examined using light microscopy. Of these, 52% contained at least 1 remnant, most commonly of the allantoic duct type. Although there was a significant decrease in vitelline duct remnants over increasing gestational age, from 11% at weeks 11-25 to 1.6% at weeks 36-42 ( P = .009; χ2 test), the allantoic duct remnants remained constant in prevalence irrespective of gestational age...
December 12, 2018: Pediatric and Developmental Pathology
James R Wright
The objective of this study is to better understand embryonic vitelline vascular remnants in the umbilical cord, to assess their prevalence, to categorize their morphology, and then finally to describe and assess inflammation arising from these structures. During routine placental sign out, the author noted the presence or absence of vitelline vessel remnants for 1 year; when present, he assessed their histologic patterns and noted whether there were neutrophils marginating from the remnants and into the adjacent Wharton's jelly and whether there was any other evidence of amniotic fluid infection in sections of placental disc, membranes, or cord...
December 12, 2018: Pediatric and Developmental Pathology
Dina El Demellawy, James Yj Lee, Laura McDonell, David A Dyment, A S Knisely, Jean McGowan-Jordan, Bo Ngan, Milton Finegold, Raj P Kapur, Ahmed Nasr
Hepatic mesenchymal hamartoma is a rare benign neoplasm principally encountered in young children. Its origin is unknown. We report an unusual hepatic mesenchymal hamartoma in a 7-month-old girl, including histopathologic findings, immunophenotype, and karyotype. Chromosomal microarray analysis of tumoral tissue and circulating lymphocytes found 4 copies of a segment at 1q44 and fluorescence in situ hybridization indicated tandem triplication, ascribed to expansion of a paternal tandem duplication. This genetic abnormality may have played a role in pathogenesis...
November 29, 2018: Pediatric and Developmental Pathology
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No abstract text is available yet for this article.
November 27, 2018: Pediatric and Developmental Pathology
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