Bartter씨 증후군에서 발현된 저칼륨성 근육병 1예

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.