What is Bartter’s Syndrome?

Bartter’s Syndrome is an inherited defect in the renal tubules that causes low potassium levels, low chloride levels, which in turn causes metabolic alkalosis.¬†Bartter Syndrome, is not a single disorder but rather a set of closely related disorders. These Bartter-like syndromes share many of the same physiologic derangements, but differ with regard to the age of onset, the presenting symptoms, the magnitude of urinary potassium (K) and prostaglandin excretion, and the extent of urinary calcium excretion.

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Evidence for a tubular defect in the loop of Henle

Impaired response to furosemide in Hyperprostaglandin E syndrome: Evidence for a tubular defect in the loop of Henle

In hyperprostaglandin E syndrome (HPS) renal wasting of electrolytes and water is consistently associated with enhanced synthesis of prostaglandin E2 . In contrast to Bartter or Gitelman syndrome (BS/GS), HPS is characterized by its severe prenatal manifestation, leading to fetal polyuria, development of polyhydramnios, and premature birth. This disorder mimics furosemide treatment with hypokalemic alkalosis, hypochloremia, isosthenuria, and impaired renal conservation of both calcium and magnesium. Therefore the thick ascending limb of the loop of Henle seems to be involved in HPS.

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Bartter and Related Syndromes, the Puzzle is Almost Solved

It is now evident that the term Bartter syndrome does not represent a unique entity but encompasses a variety of disorders of renal electrolyte transport. Application of molecular biology techniques has permitted a better understanding of these “Bartter-like syndromes,” which at present can be divided into three different genetic and clinical entities.

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Calcification Lesions in Eyes of a Bartter Syndrome Patient

PURPOSE:

Bartter syndrome is characterized by hyperplasia of the renal juxtaglomerular apparatus, hyperaldosteronism, and hypokalemic alkalosis. We report a case of Bartter syndrome associated with normal serum calcium levels and posterior choroidal calcification.

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NSAID Drugs May Cause kidney Dysfunction

We have followed four patients with Bartter syndrome for a mean of 25.4 years (range 21.5-28.8 years) after diagnosis. All patients received non-steroidal anti-inflammatory drugs (NSAID). In all patients, various degrees of renal dysfunction were noted to be temporally associated with NSAID therapy. In two patients, renal dysfunction resolved after discontinuing NSAID therapy, while maintaining other chronic medications such as potassium-sparing diuretics. Renal dysfunction persisted after NSAID withdrawal in two patients. We report these cases as a warning that NSAID should be considered an important cause of either reversible or irreversible renal dysfunction in Bartter syndrome.

Pediatr Nephrol 1998 Nov;12(9):775-7, Schachter AD, Arbus GS, Alexander RJ, Balfe JW
Department of Pediatrics, The Hospital for Sick Children and University of Toronto, Ontario, Canada.
PMID: 9874326, UI: 99089722

Types of Bartter Syndrome

Bartter syndromes are defined as a family of inherited recessive autosomal tubulopathies. They are characterized by hypochloremia, hypokalemia, metabolic alkalosis associated with potassium renal leakage and normal blood pressure despite increased plasma renin activity. Read the rest »

Bartter Syndrome in Two Generations

Bartter’s syndrome (BS) is characterized by primary renal tubular hypokalemic metabolic alkalosis, hyperreninemia, hyperaldosteronism and normal blood pressure. The parents and siblings of a BS patient were evaluated for renal tubular function. The father and all 9 siblings of the patient had biochemical features of BS.
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Bartter Syndrome Suggested by Polyuria and Nocturia

Presentation

A 6-year-old boy who is developmentally delayed is brought to your office because he has been experiencing polydipsia, nocturia, and polyuria for several months. He is 3 to 4 years delayed in language, motor, and social skills. Physical examination reveals a healthy-looking child whose height and weight are between the 5th and 10th percentiles. All vital signs are normal, and no abnormalities are found on complete physical examination.

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Bartter Syndrome, Diagnosis in Children

Bartter syndrome is a rare form of renal potassium wasting characterized by hypokalemia, normal blood pressure, vascular insensitivity to pressor agents, and elevated plasma concentrations of renin and aldosterone. In certain families, the disorder may be inherited as an autosomal recessive trait.

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Bartter’s Syndrome Comes of Age

In 1962, Bartter and co-workers [1] published a description of two patients presenting with a new syndrome characterized by hypokalemia, metabolic alkalosis, hyperaldosteronism with normal blood pressure, decreased pressor responsiveness to infused angiotensin II, and hyperplasia of the juxtaglomerular complex. Since then, many reports, both in children and in adults, have appeared in the literature under the heading of “Bartter’s syndrome.” It is nowadays evident that this term does not represent a unique entity but encompasses a variety of disorders of renal electrolyte transport all characterized by a biochemical picture of hyperreninemic hypokalemic metabolic alkalosis. At the present time this ensemble of patients can be divided into three different genetic and clinical entities [2] :

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