1- Unidade de Nefrologia, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
2- Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
- 47th ESPN Annual Scientific Meeting of the European Society of Paediatric Nephrology, 18-20/9/2014, Porto (Poster)
- Revista Pediatr Nephrol 2014;29(9):1772
Introduction: Encapsulating Peritoneal Sclerosis is a extremely rare life-threatening complication of APD.
Material and methods: Consultation of clinical records.
Results: We present this 13-year-old boy with CKD since birth due to bilateral renal artery hypoplasia. He was on conservative therapy until 15-month-old, when APD was started. During his 9 year duration treatment he had 13 peritonitis. At age 9 he was transplanted. Graft failure due to chronic rejection was diagnosed 10 months after, which motivated return to APD. Peritonitis by unusual agents, fever, weight loss and painful abdominal mass corresponding to the graft led to transplant nephrectomy. During this extremely difficult procedure, many adherences with extensive peritoneal fibrosis were noted. He started HD while waiting for a second transplant. Around 2.5 years later, he had a 26.5% weight loss in 5 months due to malabsorption syndrome. He had constipation but no occlusion/subocclusion episodes occurred. A abdomino-pelvic CT scan was performed, which revealed signs of probable peritoneal sclerosis. Anaemia (despite of treatment with IV iron and ESA), leukopenia with neutropenia and mild thrombocytopenia were found, but his myelogram was normal. He also had some episodes of disequilibrium, without focal signs and with normal cranio-encephalic MRI. Although peritoneal biopsy was not performed, clinical evolution, surgeon´s description of peritoneum appearance and CT scan result, encapsulating peritoneal sclerosis was considered and treatment with prednisolone (1 mg/kg/day, 6 months), tamoxifen (10 mg/day, one year) and hypercaloric diet was started. An increase of body weight (80% of the lost) and normalization of CBC and biochemical values were noted.
Conclusions: Despite this good evolution and included on the transplant list some aspects concern us: no one knows when he will be submitted to a second transplant, which could ameliorate his prognosis; after stopping tamoxifen, will a clinical deterioration occur? And, in that case, which therapeutic options can be offered?
Palavras Chave: encapsulating peritoneal sclerosis, peritoneal dyalisis