[Familial lymphohistiocytosis associated with lymphocytic meningitis].
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Biomedical subjects
Publications and source records attributed to M Kaplan.
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Coxsackie infections in 14 infants born in a single neonatal unit were studied. The clinical presentation was mild nonspecific disease in eight infants and an overwhelming multiorgan disease in six. Three infants with systemic disease died. Vertical transmission of the virus from a colonized mother was documented in five mothers who infected seven infants. The remaining seven infants were infected horizontally, presumably from infected personnel. Coxsackie B1 was isolated in four of the cases and type B3 in two cases of systemic disease. Intravenous gammaglobulin combined with human leukocyte interferon was utilized in three cases of overwhelming disease and two infants of these infants survived.
Recently it has been described in the literature a subpopulation of PBC patients with negative AMA that might represent patients with ANA positive autoimmune cholangitis. We review 25 cases of PBC (23 females) with AMA negative. Our aim was to determine the frequency of ANA(+) in this group. We studied serum transaminases, total bilirubin, AMA and ANA by indirect immunofluorescence considering positive dilutions of 1:40. All patients had elevated alkaline phosphatase (641 +/- 389 U/l). Total bilirubin was below 2.5 in 59%. Thirteen patients had esophageal varices. The histologic stage was I-II in eleven and III-IV in 14 cases. Fourteen patients (56%) has ANA(+) (dilution 1:40), 44% had lower dilutions. We confirm the presence of a subpopulation of PBC AMA negative, ANA positive patients and the observations of different and diverse immune alterations in PBC patients.
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Two nursing neonates deficient in glucose-6-phosphate dehydrogenase developed severe hyperbilirubinemia despite phototherapy. Mothers of both the infants had recently eaten fava beans. The hemolytic triggers found in fava beans may have been absorbed by the mothers and excreted in their breast milk. Carboxyhemoglobin determination performed on one of the infants reflected ongoing hemolysis.