Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The paper reports on a case of "late onset disease" (congenital German measles). Diagnostically significant for this disease in an infant appearing perfectly healthy when new-born, were mainly the findings of an interstitial pneumonia, hepatosplenomegaly, as well as findings indicating the presence of a meningoencephalitis and retinopathy. The diagnosis of this rare course of congenital German measles was finally established via the identification of specific IgM rubeola antibodies, of a raised hemagglutination inhibition titer, and of the German measles virus itself. An attempt to treat the interstitial pneumonia with cortisone preparations remained unsuccessful. The clinical and diagnostic problem complexes are discussed briefly.
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PMID:["Late onset disease" (congenital german measles) (author's transl)]. 56 31

The effect of tocopherol (vitamin E) on oxygen-induced retinopathy was studied in 75 kittens following the development of a 12-point scoring system to quantitate the degree of retinopathy seen at three weeks. The tocopherol was found to be beneficial when given daily from the day of birth (P less than .0001) with oxygen exposures of two to three days (79% FiO2) beginning on day 3. Caution is urged in applying these data to humans because (1) hepatosplenomegaly was noted in the treated animals, and (2) the kitten model for oxygen-induced retinopathy is not entirely satisfactory, Pediatrics, 59:998-1005 1977, RETROLENTAL FIBROPLASIA, OXYGEN TOXICITY, VITAMIN E, RETINOPATHY, KITTEN.
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PMID:The role of tocopherol in oxygen-induced retinopathy: kitten model. 57 6

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition with high mortality. We report an extremely premature girl, born in the 24th gestational week (BW 732 g), that during her second month developed a severe HLH subsequent to a Serratia marcescens septicemia, with hepatosplenomegaly, cytopenias, hyperbilirubinemia (mostly conjugated, total bilirubin 916 mumol/L), hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia (21266 mug/L), and elevated sIL-2 receptor levels. Genetic analysis revealed no PRF1, STX11 or UNC13D gene mutations. Treatment was provided according to the HLH-2004 protocol with etoposide, dexamethasone, and immunoglobulin, but no cyclosporin because of immature kidneys. She recovered fully from the HLH but developed a severe retinopathy as well as green teeth secondary to the hyperbilirubinemia. We conclude that secondary, bacteria-associated HLH can develop in premature infants, and that HLH can be treated with cytotoxic therapy also in premature infants. It is important to be aware of HLH in premature infants, since it is treatable.
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PMID:Severe bacteria-associated hemophagocytic lymphohistiocytosis in an extremely premature infant. 1788 50