The pathogenetic spectrum of Bartter's syndrome

Stein, Janice Gross

doi:10.1038/ki.1985.123

Cited by 130 publications

(82 citation statements)

References 24 publications

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“…Norby et al (1976), Kurtz et al (1984) and Seyberth et al (1985) have also reported similar cases of Bartter's syndrome lacking an abnormality in the distal tubular function. As discussed in literatures (Kurtz et al 1984;Stein 1985), the variables of renal distal tubular function are influenced easily by the procedure adopted (i.e., oral water loading vs. intravenous saline infusion) or the volume as well as endocrine status of the patient.…”

Section: Discussionmentioning

confidence: 99%

A Case of Secondary Aldosteronism Similar to Bartter's Syndrome with No Abnormality in Renal Chloride Reabsorption.

Takeuchi

Imai

Omata

et al. 1993

Tohoku J. Exp. Med.

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We had a 20-year-old male patient of secondary aldosteronism similar to Bartter's syndrome, which had proved to be evident after the remission of nephrotic syndrome. In the patient, hypokalemic alkalosis and hyperreninemic hyperaldosteronemia were observed, although the blood pressure was normal. Hyperplasia of juxtaglomerular cells was observed and no abnormalities indicating either glomerulonephritis or renal artery stenosis were found; the pressor response to intravenously infused angiotensin (ang) II was markedly decreased ; urinary prostaglandin (PG) E2, kallikrein and kinin excretion were elevated. The inhibition of PG synthesis with indomethacin decreased renal PG production and partially corrected both hypokalemia and pressor responsiveness to ang II. Thus, this case is considered to be a case of Bartter's syndrome. Contrary to the previously reported observations, the effective fractional chloride reabsorption rate in the renal distal tubules was normal (>80%) and not changed by PG inhibition. Plasma atrial natriuretic peptide level was normal. An interaction between renin-angiotensin and PG systems appears to play a prior role in this case. To explain the pathophysiology, we have hypothesized an abnormal function of ang II receptor signal transduction which excessively stimulates PLA2, resulting in overproduction of PG synthesis in tissues.prostaglandin ; nephrotic syndrome ; angiotensin II receptor ; kallikrein-kinin system ; atrial natriuretic peptide Secondary aldosteronism refers to an appropriately increased production of adrenal aldosterone in response to activation of the renin-angiotensin (R-A) system, while primary aldosteronism is due to an adrenal adenoma autonomously secreting mineralocorticoids.Secondary aldosteronism, therefore, occurs in such a pathophysiologic condition as a decrease in effective plasma volume (i.e.,

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Section: Discussionmentioning

confidence: 99%

A Case of Secondary Aldosteronism Similar to Bartter's Syndrome with No Abnormality in Renal Chloride Reabsorption.

Takeuchi

Imai

Omata

et al. 1993

Tohoku J. Exp. Med.

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“…It has been reported that renal tubular dysfunction leading to impaircd renal acidification is present in Bartter's syndrome (Stein, 1985). Bartter's syndrome happens to present features resembling renal tubular acidosis and vice versa (Rodriguez-Soriano et al, 1978 ;Takeda et al, 1973).…”

Section: Discussionmentioning

confidence: 99%

A Case of Pseudo-Bartter's Syndrome Associated with Hypergastrinemia, Thrombocytosis and Increased Serum Thyroxine-Binding Globulin.

et al. 1990

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A housewife, 40 years of age, was admitted with dysesthesia of the extremities, muscle weakness, and attacks of adynamia and thirst. She had been taking a laxative for more than 20 years. On physical examination, blood pressure was 94/56 mmHg. Laboratory tests revealed thrombocytosis, low serum K and marked increases in both plasma renin activity and serum aldosterone.Serum TBG was increased. Serum gastrin was also markedly increased and could not be enhanced by exogenous secretin. Both angiotensin 11 loading test and noradrenalin loading test failed to increase blood pressure. Ammonium chloride loading to examine the disturbance of urinary acidification was abnormal in the short term test and borderline in the long term test. Following a diagnosis of pseudo-Bartter's syndrome induced by long term intake of laxative and repeated diarrhea, the administration of laxative was interrupted and potassium, indomethacin and spironolactone were administered. However, serum K remained low. Hypergastrinemia, thrombocytosis and a high serum TBG level also persisted, the causes of which remain unknown. This is the first reported case of pseudo-Bartter's syndrome associated with hypergastrinemia, thrombocytosis and increased serum TBG.

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“…Ciddi hipokalemi ve metabolik alkalozuna yönelik, potasyum infüzyonu (120 meq/gün) ve spiranolakton (optimum dozu titre edilerek, 100 mg/gün) tedavileri uygulandı. Tedavinin 48. saatinde kan gazı ve meta- (5,6) . Bu bozuklukların patogenezisi, loop ve tiyazid diüretikleriyle görülen tabloyla aynıdır, çünkü burada sırasıyla Henle kulpundaki (loop diüretiği) ve distal tübüldeki (tiyazid diüre-tiği) NaCl geri emiliminde bir azalma vardır (7) .…”

Section: Introductionunclassified

“…Bartter sendromu genellikle çocukluk yaşında (6 yaş öncesinde) ortaya çıkarken, Gitelman sendromunun erişkin yaşta ortaya çıktığı bilinmektedir. Sodyum kaybına olan eğilim, hiperreninemiye, hiperaldosteronizme ve hipokalemik alkaloza yol açar (5) . Vazodilatatör prostaglandinlerin (prostaglandin E ve prostasiklin) sekresyonunun artması, kan basıncının neden bu olgularda normal kaldığını açıklayabilir (14) .…”

Section: Introductionunclassified

A bartter syndrome case who illustrating atypical clinical presentation

Tekçe¹,

Tekçe²,

Dağıstan³

et al. 2015

Tepecik Dergisi

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ÖZETBartter sendromu Henle kulpunun çıkan kalın kolundaki taşıyıcı proteinlerin etkisini ortadan kaldıran mutasyonlar nedeniyle ortaya çıkmaktadır. Oluşan klinik bulgular kompleksi, esas olarak tübüler sodyum ve klorür kaybının neden olduğu; ağır hipokalemi, metabolik alkaloz ve düşük-normal kan basıncı ile kendini göstermekte-dir. Son derece ender görülen bu kalıtımsal bozukluğa ait klinik bulguların genellikle perinatal ve çocukluk dönemlerinde ortaya çıktığı kabul edilmektedir. Bunun yanı sıra hastaların atipik yaşlarda da hekimin karşısına gelebileceği ve klinik beklentinin ötesinde her zaman normo/hipotansif olmayabileceği de akılda tutulmalıdır. Anahtar kelimeler: Bartter sendromu, hipokalemi, metabolik alkaloz ABSTRACTBartter syndrome is a consequence of the mutations in thick ascending loop of the Henle which cause deterioration in the effects of carrier proteins. Clinical spectrum complex includes severe hypokalemia, metabolic alkalosis and low-normal blood pressure which mainly caused by tubular Na and Cl loss. This a very rare condition usually accepted to occur in perinatal or pediatric age. It should be kept in mind that they may present in other age groups and contrary to clinical expectation, patients may not be normo/hypotensive.

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The pathogenetic spectrum of Bartter's syndrome

Cited by 130 publications

References 24 publications

A Case of Secondary Aldosteronism Similar to Bartter's Syndrome with No Abnormality in Renal Chloride Reabsorption.

A Case of Secondary Aldosteronism Similar to Bartter's Syndrome with No Abnormality in Renal Chloride Reabsorption.

A Case of Pseudo-Bartter's Syndrome Associated with Hypergastrinemia, Thrombocytosis and Increased Serum Thyroxine-Binding Globulin.

A bartter syndrome case who illustrating atypical clinical presentation

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