Bartter’s Syndrome – A Dilemma of Cause and Effect

Abstract

Six interrelated abnormalities of Bartter’s syndrome are analyzed – juxtaglomerular hyperplasia, angiotensin resistance, altered kallikrein-kinin system, hyperprostaglandinuria, hypokalemia, and chloride-losing nephropathy. Arguments are advanced that any one of these could be the proximate cause and result in all the others. By the same token, each abnormality could be a consequence of any of the others and, furthermore, modulate the others by negative or positive feedback. Despite many recent insights, available data do not permit a definitive conclusion as to the locus of the primary abnormality. Rather, the syndrome presents as a remarkable biological counterpart to an electronic integrated circuit. The altered physiology of Bartter’s syndrome is reviewed and the pathogenesis of the syndrome analyzed in the light of recent literature.

Infantile variant of Bartter syndrome and sensorineural deafness: A new autosomal recessive disorder

“…The pathogenesis of this, as well as the other BS variants, is not well understood. Different theories exist regarding the primary renal defect, and include, among others: impairment in sodium and chloride reabsorption [RodriguezPortales et al, 19861, increased tubular potassium loss [Korff et al, 19841, increased prostaglandin synthesis [Fujita et al, 1982;Calo et al, 19901 and angiotensin resistance [Bourke and Delaney, 1981;Sasaki et al, 1976;Kurtzman and Gutierrez, 19751. Treatment with prostaglandin synthetase inhibitors, mainly indomethacin, with or without potassium supplements or potassium sparing diuretics, has been shown to be effective in this disorder [Bommen and Brook 1982;Seyberth et al, 1985;Matsumoto et al, 19891. Sensorineural deafness (SND) has been described in association with various congenital renal diseases.…”

Hypokalemia and Prostaglandin Overproduction in Bartter’s Syndrome

“…In both cases 1 and 2, ingestion of diuretics or laxatives and surreptitious vomiting were safely ruled out by careful observation, and urinary chloride excretion was persist ently normal to high in the hospital. Some aspects of these 2 patients, however, were atypi cal for the classic Bartter's syndrome: in both of them, the disease was discovered incidentally at middle age, while in most of the patients with Bartter's syndrome, the dis ease is symptomatic and is diagnosed before the age of 25 [4] , However, those without symptoms have been report ed, possibly reflecting the heterogeneity of the syndrome [5] . That PAC at basal state in both case 1 and case 2 was normal is also atypical, although similar cases have been reported sporadically [6,7], Although the precise expla nation for this is yet undetermined, hypokalemia was most likely responsible, at least partially, since potassium balance is known to significantly modulate aldosterone production.…”

Total Body Potassium in Bartter’s Syndrome before and during Treatment with Enalapril

“…Patients are normotensive and exhibit subnormal in crease in blood pressure after administration of angioten sin II [1], The primary defect is postulated to be a disturb ance in tubular reabsorption of sodiumchloride in the thick ascending limb of the loop of Henle or in the early distal tubule [2][3][4][5].…”