Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?

Background: Several renal and urinary tract complications have been reported in patients with epidermolysis bullosa. Objective: This study investigated kidney and urinary tract involvement in patients with epidermolysis bullosa. Patients and Methods: Patients with epidermolysis bullosa in tre...

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Autores principales:	Neslihan Cicek, Nurdan Yildiz, Ruslan Asadov, Ayse Deniz Yucelten, Halil Tugtepe, Harika Alpay
Formato:	article
Lenguaje:	EN
Publicado:	Mattioli1885 2021
Materias:	epidermolysis bullosa peritoneal dialysis urinary involvement end stage renal disease Dermatology RL1-803
Acceso en línea:	https://doaj.org/article/400a9c85e62e4a9c9c914e2fd4f1211f
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spelling	oai:doaj.org-article:400a9c85e62e4a9c9c914e2fd4f1211f2021-11-17T08:27:55ZKidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?10.5826/dpc.1103a512160-9381https://doaj.org/article/400a9c85e62e4a9c9c914e2fd4f1211f2021-05-01T00:00:00Zhttp://dpcj.org/index.php/dpc/article/view/1552https://doaj.org/toc/2160-9381 Background: Several renal and urinary tract complications have been reported in patients with epidermolysis bullosa. Objective: This study investigated kidney and urinary tract involvement in patients with epidermolysis bullosa. Patients and Methods: Patients with epidermolysis bullosa in treatment at the Dermatology Unit were included in the study. Glomerular and tubular functions were investigated. Results: The study included 16 patients (4 females, 12 males) of mean 11.1 years (SD = 8.1 years). Estimated GFR was normal in all patients except one with end-stage renal disease. Excluding this patient, the urinary albumin/creatinine ratio and the fractional excretion of sodium were normal. The mean beta-2 microglobulin/creatinine ratio was 278.8 µg/g, and it was abnormally high in 2 patients. The mean tubular phosphorus reabsorption was 92.6%; it was abnormally low in 1 patient. Severe kidney or urinary tract involvement was present in 2 patients with recessive dystrophic EB-generalized severe (RDEB-GS): one patient had obstructive bullous lesions in the urethra; the other had end-stage renal disease secondary to focal segmental glomerulosclerosis and was on peritoneal dialysis for 3 years. Conclusions: Assessment for renal and urinary tract involvement should become a routine part of the evaluation of patients with any type of EB, but especially of patients with RDEB-GS. Patients with mild tubular dysfunction need long-term follow-up to detect early deterioration of renal function. Neslihan CicekNurdan YildizRuslan AsadovAyse Deniz YuceltenHalil TugtepeHarika AlpayMattioli1885articleepidermolysis bullosaperitoneal dialysisurinary involvementend stage renal diseaseDermatologyRL1-803ENDermatology Practical & Conceptual (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	epidermolysis bullosa peritoneal dialysis urinary involvement end stage renal disease Dermatology RL1-803
spellingShingle	epidermolysis bullosa peritoneal dialysis urinary involvement end stage renal disease Dermatology RL1-803 Neslihan Cicek Nurdan Yildiz Ruslan Asadov Ayse Deniz Yucelten Halil Tugtepe Harika Alpay Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?
description	Background: Several renal and urinary tract complications have been reported in patients with epidermolysis bullosa. Objective: This study investigated kidney and urinary tract involvement in patients with epidermolysis bullosa. Patients and Methods: Patients with epidermolysis bullosa in treatment at the Dermatology Unit were included in the study. Glomerular and tubular functions were investigated. Results: The study included 16 patients (4 females, 12 males) of mean 11.1 years (SD = 8.1 years). Estimated GFR was normal in all patients except one with end-stage renal disease. Excluding this patient, the urinary albumin/creatinine ratio and the fractional excretion of sodium were normal. The mean beta-2 microglobulin/creatinine ratio was 278.8 µg/g, and it was abnormally high in 2 patients. The mean tubular phosphorus reabsorption was 92.6%; it was abnormally low in 1 patient. Severe kidney or urinary tract involvement was present in 2 patients with recessive dystrophic EB-generalized severe (RDEB-GS): one patient had obstructive bullous lesions in the urethra; the other had end-stage renal disease secondary to focal segmental glomerulosclerosis and was on peritoneal dialysis for 3 years. Conclusions: Assessment for renal and urinary tract involvement should become a routine part of the evaluation of patients with any type of EB, but especially of patients with RDEB-GS. Patients with mild tubular dysfunction need long-term follow-up to detect early deterioration of renal function.
format	article
author	Neslihan Cicek Nurdan Yildiz Ruslan Asadov Ayse Deniz Yucelten Halil Tugtepe Harika Alpay
author_facet	Neslihan Cicek Nurdan Yildiz Ruslan Asadov Ayse Deniz Yucelten Halil Tugtepe Harika Alpay
author_sort	Neslihan Cicek
title	Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?
title_short	Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?
title_full	Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?
title_fullStr	Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?
title_full_unstemmed	Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?
title_sort	kidney and urinary tract involvement in epidermolysis bullosa: is routine follow-up necessary?
publisher	Mattioli1885
publishDate	2021
url	https://doaj.org/article/400a9c85e62e4a9c9c914e2fd4f1211f
work_keys_str_mv	AT neslihancicek kidneyandurinarytractinvolvementinepidermolysisbullosaisroutinefollowupnecessary AT nurdanyildiz kidneyandurinarytractinvolvementinepidermolysisbullosaisroutinefollowupnecessary AT ruslanasadov kidneyandurinarytractinvolvementinepidermolysisbullosaisroutinefollowupnecessary AT aysedenizyucelten kidneyandurinarytractinvolvementinepidermolysisbullosaisroutinefollowupnecessary AT haliltugtepe kidneyandurinarytractinvolvementinepidermolysisbullosaisroutinefollowupnecessary AT harikaalpay kidneyandurinarytractinvolvementinepidermolysisbullosaisroutinefollowupnecessary
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Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?

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