Ciclosporin
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Hepatotoxicity: case report A male infant [exact age not stated] developed hepatotoxicity during treatment with ciclosporin for immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. The boy was born at 38 weeks of gestation. He had a family history of multiple deceased male infants on the maternal side (including his older brother). At the age of 1 month, he was hospitalised with chronic high-output diarrhoea, respiratory and urinary tract infections, catheter-associated bacteremias and deteriorated nutritional status, which led to growth retardation. At the hospital, his initial symptoms were suspected as an allergy to cow’s milk protein. He received treatment with hydrolyzed formula and free amino acids with no clinical response. Hence, he started receiving parenteral-nutrition-supplements [total parenteral nutrition] for nutritional support. Subsequent laboratory tests were noted to be abnormal. At 3 months and 15 days of age, he was referred to another hospital, where he was diagnosed with autoimmune enteropathy by endoscopic study and biopsy. Due to family history, his symptoms raised a suspicion of autoimmune enteropathy secondary to X-linked primary immunodeficiency. Thereafter, the trio exome sequencing study was requested. For the treatment, the parameter of parenteral-nutrition-supplements was adjusted. Subsequently, he started receiving low volumes of free amino acid-based formula, achieving gradual weight gain, despite the high stool output. Then, he received first line therapy with methylprednisolone. Even with 4 weeks of methylprednisolone treatment, he had persistent high fecal output. Therefore, he received ciclosporin [cyclosporine; route and dosage not stated]. Subsequently, he developed hepatotoxicity secondary to ciclosporin [duration of treatment to reaction onset not stated]. Additionally, his diarrhoea persisted. Hence, the boy’ ciclosporin was switched to tacrolimus [outcome not stated]. At the age of 7 months, he developed thrombosis involving multiple blood vessels. It was difficult to achieve new venous access for parenteral-nutrition-supplements. Therefore, the parenteral-nutrition-supplements was discontinued. Eventually, he died due to vascular complications. After death, the sequencing report was received, which showed a variant in a hemizygous state in the FOXP3 gene. Garcia CP, et al. New mutation in foxp3 gene identified in an infant with chronic diarrhea as manifestation of autoinmune enteropathy-ipex syndrome. [Spanish]. Revista 803500607 Chilena de Pediatria 91: 584-590, No. 4, Jan 2020. Available from: URL: http://doi.org/10.32641/rchped.v91i4.1467
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Reactions 12 Sep 2020 No. 1821
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