Individualized concept for the treatment of autosomal recessive polycystic kidney disease with end‐stage renal disease
| Autor: | Yasuyuki Sato, Hideki Ishida, Masataka Hisano, Hiroko Chikamoto, Hideaki Imamura, Shohei Fuchinoue, Tomoo Yabuuchi, Kazunari Tanabe, Seisuke Sakamoto, Naoto Kaneko, Takayuki Miyai, Motoshi Hattori, Yuri Nawashiro, Yuko Akioka, Masayoshi Okumi, Kiyonobu Ishizuka, Kenichiro Miura, Mureo Kasahara |
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| Rok vydání: | 2020 |
| Předmět: |
Male
medicine.medical_specialty Adolescent Cholangitis Pancytopenia medicine.medical_treatment Splenectomy 030232 urology & nephrology Renal function 030230 surgery Gastroenterology End stage renal disease 03 medical and health sciences Liver disease 0302 clinical medicine Recurrence Internal medicine medicine Humans Precision Medicine Child Kidney transplantation Polycystic Kidney Autosomal Recessive Retrospective Studies Transplantation business.industry Infant medicine.disease Kidney Transplantation Autosomal Recessive Polycystic Kidney Disease Liver Transplantation Treatment Outcome Child Preschool Splenomegaly Pediatrics Perinatology and Child Health Kidney Failure Chronic Female business Follow-Up Studies |
| Zdroj: | Pediatric Transplantation. 24 |
| ISSN: | 1399-3046 1397-3142 |
| Popis: | Management of children with autosomal recessive polycystic kidney disease (ARPKD) who develop end-stage renal disease (ESRD) remains challenging because of concomitant liver disease. Patients with recurrent cholangitis are candidates for liver-kidney transplantation, while the treatment for patients with splenomegaly and pancytopenia due to portal hypertension is controversial. Herein, we report 7 children who were treated using an individualized treatment strategy stratified by liver disease. Two patients with recurrent cholangitis underwent sequential liver-kidney transplantation, while 4 patients with splenomegaly and pancytopenia but without recurrent cholangitis underwent splenectomy followed by isolated kidney transplantation. The remaining patient, who did not have cholangitis and pancytopenia, underwent isolated kidney transplantation. Blood cell counts were normalized after splenectomy was performed at the median age of 8.7 (range, 7.4-11.7) years. Kidney transplantation was performed at the median age of 8.8 (range, 1.9-14.7) years in all patients. Overwhelming post-splenectomy infections and cholangitis did not occur during the median follow-up period of 6.3 (range, 1.0-13.2) years. The estimated glomerular filtration rate at the last follow-up was 53 (range, 35-107) mL/min/1.73 m2 . No graft loss occurred. Our individualized treatment strategy stratified by recurrent cholangitis and pancytopenia can be a feasible strategy for children with ARPKD who develop ESRD and warrants further evaluation. |
| Databáze: | OpenAIRE |
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