Pyloric duplication presenting with hemorrhagic ascites.
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Biomedical subjects
Publications and source records attributed to R W Novak.
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A review of 106 cases of Wilms' tumor revealed five examples of a distinctive, monomorphic, sarcomatous renal tumor. Electron microscopic studies suggested that such tumors have their origin from the blastemal cap cells of the developing metanephros. These variants of Wilms' tumor are more likely to metastasize to bone and have a poorer prognosis than the usual type of nephroblastoma.
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Four cases and a literature survey show acute transverse myelopathy to be a dramatic but rare pediatric problem. Neurologic deficits can be significant but generally improve with the passage of time. The prognosis for complete recovery, however, is poor.
Hemolytic anemia, thrombocytopenia, and renal failure form a constellation of complications observed in patients infected with neuraminidase-producing pneumococci. The circulating enzyme causes exposure of the T-cryptantigen on cell surfaces to which most people possess a naturally occurring antibody. Antigen-antibody interaction activates effector systems that lead to the clinical manifestations. The syndrome is most frequently seen in infants, in whom it carries more than a 50% mortality rate. T-cryptantigen activation can be detected by demonstrating red cell agglutination by the peanut lectin Arachis hypogea. Plasma exchange and avoidance of blood products containing immunoglobins are of value in the management of this devastating complication of pneumococcal infection.
A 3-year-old girl with rhizomelic chondrodysplasia punctata (RCDP) had severe microcephaly but a normal gyral pattern, neuronal density, and cortical cytoarchitecture. The white matter was diffusely decreased in mass but normally myelinated. There was optic atrophy and cerebellar degeneration. Leukodystrophy in peroxisomal disorders is caused by elevated very long chain fatty acids. The absence of a fatty acid abnormality in RCDP explains the normal myelination. Cerebellar and retinal degeneration and possible stunted dendritogenesis may be due to plasmalogen deficiency, which is the most severe biochemical abnormality in RCDP.