A case of Bartter's syndrome with abnormal renin response to salt load

Imai, Masashi; Yabuta, Keijiro; Murata, Hiroo; Takita, Seiji; Ohbe, Yoshio; Sokabe, Hirofumi

doi:10.1016/s0022-3476(69)80136-8

Cited by 18 publications

(4 citation statements)

References 34 publications

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“…[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]. These patients, however, appear to have glomerulonephritis, vide infra.…”

Section: Discussionmentioning

confidence: 96%

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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Section: Discussionmentioning

confidence: 96%

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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“…Evidence on the response of these patients to infusions of norepinephrine is contradictory. Brackett and associates (7) observed a normal pressor response in their patient, but in the patient reported by Imai et al (21) there was a reduced response to norepinephrine infusion. It has been suggested that the refractoriness of the vasculature to angiotensin in these patients may be due to tachyphylaxis as a result of prolonged high concentrations of circulating angiotensin (3, 7, 43).…”

Section: Nature Of the Basic Defectmentioning

confidence: 86%

“…To date, at least 22 cases have been recorded (21,23,42), but our knowledge of the syndrome's pathogenesis has progressed little since its original description. Although the patients described below were originally investigated from 1954 to 1963, and were briefly recorded in two abstracts in the latter year (1, 40), they are being reported in detail now because re-evaluation has brought to light certain features which may add to our understanding of this rare but very important entity.…”

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confidence: 99%

A Report of Four Cases, Including Three in One Sibship, With Comparative Histologic Evaluation of the Juxtaglomerular Apparatuses and Glomeruli

Sutherland¹,

Hartroft

Balis

et al. 1970

Acta Paediatrica

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Summary and Conclusions An electrolyte‐losing syndrome is described in three siblings, including one set of twins, and in an unrelated infant who was the product of incest. In most respects the clinical and laboratory findings in these patients were compatible with the condition which has come to be known as Bartter's syndrome. In all four of our patients, however, symptoms were manifested early in life, the disease ran a malignant course and death occurred early. Electron microscopic examination of the kidneys from two of our patients showed characteristic structural changes in the glomeruli including immaturity of the podocytes and extensive loss or fusion of their foot processes. Light microscopy of the kidneys from all four cases showed an unusually large proportion of immature glomeruli that seemed to diminish slowly with age. Some ninety per cent of these small glomeruli were located peripherally. The incidence and size of hypertrophied JGA, also the degree and extent of hyperplastic arterial thickening, appeared to increase with age. These findings are discussed in relation to the hypothesis that the basic abnormality in Bartter's syndrome may involve a defect of sodium reabsorption in the proximal tubule, and that this leads to hypertrophy of the JGAs of functioning nephrons with excessive production and release of renin, which causes vasoconstrictive dormancy of the more peripheral glomeruli.

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“…2 -6 Although numerous theories about the pathogenesis of this disorder have been advanced, including primary vascular hyporesponsiveness to pressor substances, autonomous renin oversecretion, reduced renal sodium or chloride reabsorption, overproduction of renal prostaglandins, and excessive renal potassium loss, the etiology of this syndrome remains unknown. 3 -4 -7 In 1969 Imai et al, 8 proposed that the primary abnormality in Bartter's syndrome was the overproduction of a natriuretic factor. This proposal was later extended by Grekin et al, 9 who suggested that Bartter's syndrome resulted from an excess of a "chloriuretic hormone," which would account for the characteristic renal potassium wasting, hypokalemia, fluid volume depletion, excessive renal prostaglandin Ep roduction, and most of the other features of the disorder.…”

mentioning

confidence: 99%

Bartter's syndrome and the atrial natriuretic factor gene.

Graham¹,

Bloch²,

Delaney³

et al. 1986

Hypertension

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We investigated whether the gene for atrial natriuretic factor, a recently discovered peptide hormone with potent natriuretic, diuretic, and vasorelaxant properties, was pathogenetically linked to an uncommon but well-defined fluid and electrolyte disorder, Bartter's syndrome. Restriction fragment length polymorphisms in the atrial natriuretic factor gene were sought in a large kindred with six of 23 family members being affected. A Bgl II polymorphism, identified in two of 40 (5%) apparently normal subjects, was found in one of two first-generation family members. This polymorphism was also present in five of seven unaffected second-generation siblings but in only three of six affected siblings. The failure of the absence or the presence of the polymorphism to cosegregate with the disease clearly indicates that in this kindred, the gene for atrial natriuretic factor is not linked to Bartter's syndrome.

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A case of Bartter's syndrome with abnormal renin response to salt load

Cited by 18 publications

References 34 publications

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

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

A Report of Four Cases, Including Three in One Sibship, With Comparative Histologic Evaluation of the Juxtaglomerular Apparatuses and Glomeruli

Bartter's syndrome and the atrial natriuretic factor gene.

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