Neslihan Cicek 1 , Nurdan Yildiz 1 , Ruslan Asadov 2 , Ayse Deniz Yucelten 3 , Halil Tugtepe 4 , Harika Alpay 1 Show Affiliations »
Abstract
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. ©2021 Cicek et al.
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. ©2021 Cicek et al.
Entities: Chemical
Keywords:
end stage renal disease; epidermolysis bullosa; peritoneal dialysis; urinary tract involvement
Year: 2021
PMID： 34123558 PMCID： PMC8172055 DOI： 10.5826/dpc.1103a51
Source DB: PubMed Journal: Dermatol Pract Concept ISSN： 2160-9381