RESUMEN
Abstract Encapsulating Peritoneal Sclerosis (EPS) is a severe and rare condition frequently associated with peritoneal dialysis, characterized by bowel obstruction, with lethal consequences in 20% of the patients. The disease presents as a mass of fibrous tissue encapsulating visceral organs that may potentially compromise digestive tract function. This report describes the case of a patient under peritoneal dialysis (PD) due to chronic kidney disease secondary to focal segmental glomerulosclerosis diagnosed with EPS. The patient had undergone two living-donor kidney transplant procedures. Surgical techniques and clinical measures employed to unravel bowel obstruction are described, which have been shown to ameliorate EPS secondary complications. Parenteral nutrition has significantly contributed to afford adequate nutrition, improving tissue healing as well as serum protein levels, vitamins and electrolytes. Therapy with tamoxifen and sodium thiosulfate effectively delayed the development of EPS.
Resumo A peritonite esclerosante encapsulante (PEE) é uma condição rara e grave, frequentemente associada à diálise peritoneal, caracterizada por obstrução intestinal, que pode ter uma evolução fatal em 20% dos pacientes. Apresenta-se como uma massa de tecido fibroso, recobrindo os tecidos viscerais, e pode comprometer o funcionamento fisiológico de todo o aparelho digestivo. O presente estudo relata um caso de PEE decorrente de diálise peritoneal (DP) devido à insuficiência renal por glomeruloesclerose focal e segmentar. No caso relatado, a paciente foi submetida a DP e passou por dois transplantes renais intervivos. São descritas as técnicas cirúrgicas e as medidas clínicas adotadas, que se revelaram úteis na resolução da obstrução intestinal, com melhora dos efeitos secundários da PEE. A dieta parenteral mostrou ser um importante fator para a manutenção do aporte nutricional, auxiliando na cicatrização e no nível sérico de proteínas, vitaminas e eletrólitos. A terapia com tamoxifeno e a administração de hipossulfito de sódio foram eficientes para retardar o avanço da PEE.
Asunto(s)
Humanos , Femenino , Preescolar , Adulto , Peritonitis , Trasplante de Riñón , Diálisis Peritoneal , Fibrosis Peritoneal , InmunosupresoresRESUMEN
Encapsulating Peritoneal Sclerosis (EPS) is a severe and rare condition frequently associated with peritoneal dialysis, characterized by bowel obstruction, with lethal consequences in 20% of the patients. The disease presents as a mass of fibrous tissue encapsulating visceral organs that may potentially compromise digestive tract function. This report describes the case of a patient under peritoneal dialysis (PD) due to chronic kidney disease secondary to focal segmental glomerulosclerosis diagnosed with EPS. The patient had undergone two living-donor kidney transplant procedures. Surgical techniques and clinical measures employed to unravel bowel obstruction are described, which have been shown to ameliorate EPS secondary complications. Parenteral nutrition has significantly contributed to afford adequate nutrition, improving tissue healing as well as serum protein levels, vitamins and electrolytes. Therapy with tamoxifen and sodium thiosulfate effectively delayed the development of EPS.