A Case of Bartter's Syndrome with Surgical Treatment Followed for Four Years

Takayasu, Hisao; Aso, Yoshio; Nakauchi, Koji; Kawabe, Kazuki

doi:10.1210/jcem-32-6-842

Cited by 21 publications

(8 citation statements)

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“…Amelioration of hypokalemia with K-sparing diuretics (spironolactone and/or triamterene) has already been re ported in some patients with alleged Bartter's syndrome [19][20][21][22][23], though in other patients hypokalemia was unre sponsive to any maneuver aimed at reducing aldosterone effects on the renal tubule, including spironolactone ad ministration [24.25] and both functional (with aminoglu téthimide) [25] and subtotal surgical adrenalectomy [26]. One cannot presently settle whether so divergent effects resulted from inadequate drug dosage -only 75 mg/day of spironolactone was administered to a 9-year-old unre sponsive patient [25], though up to 1,000 mg/day was administered to a second unresponsive patient [24] -, unequal efficacy of treatment modalities (subtotal adre nalectomy may not completely suppress aldosterone se cretion), or different diseases in some patients.…”

Section: Gs Is Currently Believed To Results From a Renalmentioning

confidence: 99%

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman’s Syndrome

Colussi

Rombolà²,

Ferrari

et al. 1994

Am J Nephrol

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Six adult patients (4 females and 2 males, age range 26-57 years) with Gitelman’s syndrome (GS) were treated with spironolactone 200-300 mg/day (n = 5) and/or amiloride 10-30 mg/day (n = 3) for 1-18 months. The patients had hypokalemia, hyperreninemia, chloride-resistant metabolic alkalosis, renal hypomagnesemia (n = 5), and hypocalciuria (n = 5). Free water clearance studies during maximal water diuresis and furosemide administration were suggestive of a solute reabsorptive defect beyond the loop of Henle. Antialdosterone therapy induced a significant increase of Pk (from 2.6 ± 0.4 to 3.4 ± 0.4 τaM\ p < 0.0001) and a decrease of C_ĸ (from 21.4 ± 13.2 to 10.6 ± 4.8 ml/ min, p < 0.02) and FE_K (from 21.0 ± 13.6 to 13.4 ± 5.7%; p < 0.03); P_Mgincreased from 1.38 ± 0.38 to 1.64 ± 0.21 mg/dl (p < 0.03) with a parallel fall of C_Mg (from 5.5 ± 2.3 to 2.9 ± 1.5 ml/min; p < 0.02) and FE_Mg (from 5.7 ± 2.6 to 2.9 ± 0.6%; p < 0.05); arterial blood pH and HCO^-₃ did not change (P = plasma, C = clearance, FE = fractional excretion). The creatinine clearance fell (from 90.5 ± 16.8 to 65.8 ± 20.9 ml/min; p < 0.05), and P_renin rose (from 16.6 ± 8.9 to 35.3 ± 25.3 ng/ml/h;p < 0.02, as did P_aldo (from 26.1 ± 12.3 to 109 ± 82.6ng/dl; p < 0.01), indicating extracellular fluid volume contraction; however no significant clinical symptoms of hypovolemia ensued. Despite increased P_aldo levels, estimated transtubular K gradient in K secretory sites fell (from 8.0 ± 4.0 to 6.7 ± 3.4; p < 0.01), confirming blunted aldosterone tubular effect. At the dosages employed, spironolactone induced a greater increase of P_ĸ (0.81 ± 0.52 mM) than amiloride (0.07 ± 0.41; p < 0.001). In conclusion, antialdosterone therapy is effective in ameliorating hypokalemia and hypomagnesemia in GS. Its effects appear to result mainly from a direct tubular effect on K secretion and Mg reabsorption; extracellular fluid volume contraction appears also to occur during therapy, but has no relevant clinical effects. These results confirm that hypokalemia in GS is more a consequence of increased tubular secretion in the cortical collecting tubule than of impaired tubular K reabsorption; moreover, impaired tubular Mg conservation in GS might also occur in more distal segments of the nephron than previously supposed.

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Section: Gs Is Currently Believed To Results From a Renalmentioning

confidence: 99%

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman’s Syndrome

Colussi

Rombolà²,

Ferrari

et al. 1994

Am J Nephrol

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“…x 36,000. al. [15]. In some instances, a considerable degree of flat tening of the macula densa cells has been observed with out a decrease the number of these cells [16][17][18][19].…”

Section: Discussionmentioning

confidence: 97%

Nephropathologic Characteristics of a Woman with Bartter’s Syndrome after Prolonged Treatment with Spironolactone

Nakada

Shigematsu

Bartter

et al. 1980

Nephron

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A study was made of two renal biopsy specimens obtained from a 22-year-old woman with Bartter’s syndrome, the first to substantiate the diagnosis, the second 2 years later after the treatment with spironolactone. The first renal biopsy revealed remarkable hyperplasia and hypertrophy of the juxtaglomerular apparatus. The numerous proliferating cells were characterized by abundant lysosomal granules and dilated endoplasmic reticulum. The macula densa revealed slight proliferation, with narrow intercellular space. The ultrastructural picture of the second biopsy specimen showed increased lipofuscin-like granules in the juxtaglomerular cells, with epitheloid cells of the macula densa showing degeneration and irregular dilatation of the intercellular spaces. However, the light microscopic finding was compatible with that of the first. These findings suggest that the treatment was inadequate although clinical features or biochemical laboratory data were improved.

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“…These findings suggest that hypokalemia may cause rather than be caused by hyperreninemia and aldosteronism. In keeping with this formulation is the observation that bilateral adrenalectomy does not correct the hypokalemia in patients with Bartter's syndrome (55,56). Furthermore, hypokalemia in dogs caused increased excretion of prostaglandin E, elevated PRA, and hyposensitivity to angiotensin II infusions; indomethacin restored these abnormalities toward normal (47).…”

Section: Discussionmentioning

confidence: 98%

The Kallikrein-Kinin System in Bartter's Syndrome and Its Response to Prostaglandin Synthetase Inhibition

Vinci¹,

Gill²,

Bowden³

et al. 1978

J. Clin. Invest.

120

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A B S T R A C T The kallikrein-kinin system was characterized in seven patients with Bartter's syndrome on constant metabolic regimens before, during, and after treatment with prostaglandin synthetase inhibitors. Patients with Bartter's syndrome had high values for plasma bradykinin, plasma renin activity (PRA), urinary kallikrein, urinary immunoreactive prostaglandin E excretion, and urinary aldosterone; urinary kinins were subnormal and plasma prekallikrein was normal. Treatment with indomethacin or ibuprofen which decreased urinary immunoreactive prostaglandin E excretion by 67%, decreased mean PRA (patients recumbent) from 17.3+5.3 (S.E.M.) ng/ml per h to 3.3±1.1 ng/ml per h, mean plasma bradykinini (patients recumbent) from 15.4±4.4 ng/ml to 3.9±0.9 ng/ml, mean urinary kallikrein excretion from 24.8±3.2 tosyl-arginine-methyl ester units (TU)/ day to 12.4±2.0 TU/day, but increased mean urinary kinin excretion from 3.8±1.3 ,ug/day to 8.5±2.5 ,ug/day. Plasma prekallikrein remained unchanged at 1.4 TU/ml. Thus, with prostaglandin synthetase inhibition, values for uriniary kallikrein and kinin and plasma bradykinin returned to normal pari passu with changes in PRA, in aldosterone, and in prostaglandin E. The

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A Case of Bartter's Syndrome with Surgical Treatment Followed for Four Years

Cited by 21 publications

References 0 publications

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman’s Syndrome

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman’s Syndrome

Nephropathologic Characteristics of a Woman with Bartter’s Syndrome after Prolonged Treatment with Spironolactone

The Kallikrein-Kinin System in Bartter's Syndrome and Its Response to Prostaglandin Synthetase Inhibition

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A Case of Bartter's Syndrome with Surgical Treatment Followed for Four Years

Cited by 21 publications

References 0 publications

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman&rsquo;s Syndrome

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman&rsquo;s Syndrome

Nephropathologic Characteristics of a Woman with Bartter’s Syndrome after Prolonged Treatment with Spironolactone

The Kallikrein-Kinin System in Bartter's Syndrome and Its Response to Prostaglandin Synthetase Inhibition

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Product

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Correction of Hypokalemia with Antialdosterone Therapy in Gitelman’s Syndrome

Correction of Hypokalemia with Antialdosterone Therapy in Gitelman’s Syndrome