Kidney involvement and associated risk factors in children with Duchenne muscular dystrophy
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ORIGINAL ARTICLE
Kidney involvement and associated risk factors in children with Duchenne muscular dystrophy Muhammet Gültekin Kutluk 1
&
Çağla Serpil Doğan 2
Received: 22 February 2020 / Revised: 21 April 2020 / Accepted: 23 April 2020 # IPNA 2020
Abstract Background Kidney dysfunction is a common complication in adults with Duchenne muscular dystrophy (DMD); however, little attention has been paid to kidney function in pediatric patients. Methods Medical records of patients with DMD who were followed up for ≥ 12 months were retrospectively reviewed. Inclusion criteria were (i) aged 5–18 years, (ii) proven mutations in the dystrophin gene, and (iii) absence of structural anomalies of the kidney and urinary tract. Serum creatine kinase (CK) was used as an indirect marker of muscle destruction. Results Forty-four patients (mean age, 10.9 ± 3.3 years) were included. Blood pressure was evaluated by 24-h ambulatory blood pressure monitoring in 28 patients. Hypertension was found in 9 (32.1%), eight of whom were using steroids. Mild proteinuria, hypercalciuria, hypocalciuria, and hyperphosphaturia in 24-h urine collection (n = 36) were detected in 3 (8.3%), 5 (13.9%), 7 (19.7%), and 6 (16.7%) patients, respectively. Twenty-one (58.3%) demonstrated hyperuricosuria, associated with hyperuricemia in 4. Logarithmic cystatin C (CysC) had a positive correlation to creatinine (Cr) (p = 0.001, r = 0.54), CK (p = 0.048, r = 0.30), and parathormone (PTH) (p = 0.001, r = 0.49). Moreover, the patients were divided into two groups according to median CysC value: group 1 (n = 20, CysC ≤ 0.76 mg/l) and group 2 (n = 24, CysC > 0.76 mg/l). Mean CK, PTH, and Cr levels were significantly elevated in group 2 compared with group 1 (p = 0.010, 0.033, and 0.023, respectively). Conclusions Long-term exposure to the excessive burden of intracellular components released from damaged muscles may be associated with an increased risk over time of chronic kidney impairment in pediatric DMD patients.
Keywords Duchenne muscular dystrophy . Child . Cystatin C . Creatine kinase . Kidney function
Introduction Duchenne muscular dystrophy (DMD) is an X-linked disorder caused by mutations in the dystrophin gene, which encodes
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00467-020-04587-3) contains supplementary material, which is available to authorized users. * Çağla Serpil Doğan [email protected] Muhammet Gültekin Kutluk [email protected] 1
Department of Pediatrics, Division of Pediatric Neurology, Antalya Training and Research Hospital, 07059 Antalya, Turkey
2
Department of Pediatrics, Division of Pediatric Nephrology, Antalya Training and Research Hospital, 07059 Antalya, Turkey
dystrophin protein involved in muscle strength and stability, and is characterized by progressive muscle degeneration. In these patients, cardiac and respiratory complications are related to high morbidity and mortality [1–3]. However, in a few adult studies, kidney dysfunction has been reported as a comm
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