Biotin Deficiency: An Unusual Complication of Parenteral AlimentationBIOTIN deficiency has been documented in human beings only in association with prolonged ingestion of raw egg white. In this paper, we report that clinically important biotin deficiency occurred in a child with short-gut syndrome during parenteral alimentation.Case ReportThe clinical history and growth of this 12-month-old girl were normal until four months of age, when malrotation and midgut volvulus resulted in extensive infarction of the small and large bowel. After the initial bowel resection, her clinical course was complicated by anastomosis failure, peritoneal infection, and bowel obstruction. Ultimately, the patient retained only 30 cm of jejunum, 0.5 cm . . .
Development of a Scleroderma-like Illness during Therapy with L-5-Hydroxytryptophan and CarbidopaA scleroderma-like illness developed in a patient treated with L-5 hydroxytryptophan (L-5HTP) and carbidopa for intention myoclonus. The patient had high plasma kynurenine levels that remained high when the L-5HTP-carbidopa combination was discontinued, However, levels rose futher on drug rechallenge, suggesting that the drug unmasked an abnormality in one of the enzymes that catabolize kynurenine. Plasma kynurenine was also determined to be high in seven of 15 patients wth idiopathic scleroderma, but not in eight patients with intention myoclonus treated with L-5HTP and a decarboxylase inhibitor and in whom scleroderma did not develop or in 10 patients with Parkinson's disease treated wth L-dopa and carbidopa. Our data and studies in the literature suggest that two factors may be important in the pathogenesis of some scleroderma-like illness: high plasma serotonin and the abnormality associated with elevated kynurenine.