Bartter씨 증후군에서 발현된 저칼륨성 근육병 1예 |
손일홍, 이지용, 조성진, 박영관, 이성수, 김승민, 선우일남* |
연세대학교 원주의과대학 신경과학교실, 연세대학교 의과대학 신경과학교실* |
A Case of Hypokalemic Myopathy Associated with Bartter's Syndrome |
Il Hong Son, M.D., Ji Yong Lee, M.D., Sung Jin Cho, M.D., Young Kwan Park, M.D., Sung Soo Lee, M.D., Seung Min Kim, M.D., Il Nam Sunwoo, M.D.* |
Department of Neurology, Yonsei University Wonju College of Medicine
Department of Neurology, Yonsei University College of Medicine |
|
|
Abstract |
Bartter's syndrome is characterized by hyperreninemia, hyperaldosteronism, hypokalemic hypochlorenlic alkalosis, normal blood pressure, juxtaglomerular apparatus hyperplasia, general weakness, and muscle weakness. We experienced a case of hypokalemic myopathy associated with Bartter's syndrome in 15 years old male. He had experienced paroxysmal muscle weakness without sensory change and myalgia since 10 years old. Subsequently, he had complaints of progressive muscle weakness, especially proximal muscles. Prominent juxtaglomerular apparatus with cellular proliferation biopsy was seen
in the kidney. And there were mild perivascular inflammatory cell infiltration, small degenerating and/or regenerating muscles fibers, and normal muscle fiber distribution without evidence of chronic myopathy in the muscle biopsy. The patient was sucessfully managed with indomethacin and oral potassium chloride. |
|