Fetal and pediatric pathology
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Cysts within the lung are one of the more common pulmonary pathological findings seen in an active pediatric surgical pathology service. Of both developmental and acquired origin, they may present as respiratory distress in infants or may be asymptomatic lesions incidentally discovered by images studies of the thoracic for "non-pulmonary" reasons. ⋯ Other congenital cystic lesions include bronchogenic cysts (usually in older patients) and some of the enteric duplication lesions that contain cysts. Acquired cystic lung lesions include acute and persistent pulmonary interstitial emphysema, postinfarction peripheral cysts, postinfectious pulmonary cysts, and the cystic form of pleuropulmonary blastoma.
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Fetal Pediatr Pathol · Jan 2008
Assessment of muscarinic and nicotinic acetylcholine receptor expression in primitive neuroectodermal tumor/ewing family of tumor and desmoplastic small round cell tumor: an immunohistochemical and Western blot study of tissue microarray and cell lines.
The primitive neuroectodermal tumor (PNET)/Ewing family of tumors (EFT) and desmoplastic small round cell tumor (DSRCT) portend a grave prognosis. Ongoing research in similar neurocrest-derived neoplasms has implicated both the muscarinic acetylcholine receptor (mAChR) and nicotinic acetylcholine receptor (nAChR) in the pathogenesis of these neoplasms. Acetylcholine has been reported to impart a modulatory effect on chemotaxis and proliferation, an effect ameliorated by anticholinergic drugs. ⋯ We found that AChRs are overexpressed in a significant number of PNET/EFT and DSRCT. The Western blot analysis of 3 human Ewing sarcoma cell lines confirms the presence of AChRs. Future studies are planned to confirm these results as well as to investigate their potential therapeutic implications.
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Fetal Pediatr Pathol · Jan 2007
ReviewHereditary and familial syndromes of bone and blood. Genetic pathways, diagnostic pitfalls.
Numerous metabolic disorders, teratogenic agents, and in utero infections lead to congenital bone disease and malformation. This review focuses on hereditary and familial disorders of bone with particular emphasis on impaired hematopoiesis, myelofibrosis, pathologic fractures, and dysmorphology of the forearm and craniofacial structures. ⋯ Both can impart difficulty to the appropriate evaluation and delay the correct diagnosis. Many of these disorders are phenotypically quite similar but require very different therapeutic intervention.
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Fetal Pediatr Pathol · May 2006
Case ReportsPortal venous gas following trauma in a preterm infant.
During the neonatal period, pneumatosis intestinalis (PI) and portal venous gas (PVG) are pathognomonic of necrotizing enterocolitis (NEC). Rare cases of such findings evolving after abdominal trauma have been reported in adults and children, but none in premature infants. We report a case of a 3-week-old premature infant where both PI and PVG were found following a fall from a scale. The presence of these findings prior to the evolvement of clinical symptoms suggestive of NEC strongly supports a traumatic etiology.