title: Hypokalemic rhabdomyolysis in congenital tubular disorders: a case series and a systematic review.
abstract: Hypokalemia is a recognized cause of rhabdomyolysis but very few reports document its association with inborn renal tubular disorders. We report our experience with hypokalemic rhabdomyolysis in 5 pediatric patients affected by inborn renal tubular disorders and the results of a careful review of the literature disclosing 9 further cases for a total of 14 patients (8 male and 6 female subjects, aged between 1.6 and 46, median 16 years). The inborn renal tubular disorders underlying rhabdomyolysis were classic distal renal tubular acidosis (n = 7), Gitelman syndrome (n = 5), classic Bartter syndrome (n = 1), and antenatal Bartter syndrome (n = 1). In 8 patients rhabdomyolysis followed an acute intestinal disease, an upper respiratory illness or the discontinuation of regular medication. Five patients experienced two or more episodes of rhabdomyolysis. In 10 patients the underlying renal tubular disorder was recognized concurrently with the episode of rhabdomyolysis or some weeks later. In conclusion some congenital renal tubular disorders predispose to hypokalemic rhabdomyolysis. Prevention of discontinuation of regular medication and electrolyte repair in the context of acute intercurrent illnesses might avoid the development of hypokalemic rhabdomyolysis.
authors.affiliation: Pediatric Nephrology, University Children's Hospital Bern and University of Bern, Bern, Switzerland.
authors.forename: Rodo O
authors.initials: RO
authors.lastname: von Vigier
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Ortisi
authors.forename: Angela
authors.initials: A
authors.lastname: La Manna
authors.forename: Mario G
authors.initials: MG
authors.lastname: Bianchetti
authors.forename: Alberto
authors.initials: A
authors.lastname: Bettinelli
mesh_terms: D000293:Adolescent; D001477:Bartter Syndrome / Q000150:complications; D002648:Child; D002675:Child, Preschool; D005260:Female; D053579:Gitelman Syndrome / Q000150:complications; D006801:Humans; D007008:Hypokalemia / Q000209:etiology* / Q000517:prevention & control; D007223:Infant; D008297:Male; D015499:Renal Tubular Transport, Inborn Errors / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012206:Rhabdomyolysis / Q000209:etiology* / Q000517:prevention & control; D016896:Treatment Outcome