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Bartter Syndrome

Madhavi Singh, MD Reviewed 05/2023
 


BASICS

  • Bartter syndrome and Bartter-like syndrome are a group of rare, autosomal recessive, salt-wasting nephropathies characterized by polyuria, hypokalemia, metabolic alkalosis, and normotension wit...

DIAGNOSIS

HISTORY

  • Polyuria and polydipsia are always present, and there is a history of episodes of dehydration.

  • In types I (pure furosemide), II, IV (furosemide-thiazide), and V, the presentation is us...

TREATMENT

  • The main goal in the neonatal period is to keep up with severe fluid and NA losses as well as correcting hypokalemia (6)[C].

  • In Gitelman, correction of hypomagnesemia is also important.

MEDICATION

ONGOING CARE

DIET

High in salt, K, and water 

PROGNOSIS

Good in general. With adequate management, patients can grow normally. 

COMPLICATIONS

Nephrocalcinosis, gastric ulcers, chronic kidney disease 

REFERENCES

1
Seyberth  HW. An improved terminology and classification of Bartter-like syndromes. Nat Clin Pract Nephrol.  2008;4(10):560–567. [View Abstract on OvidMedline]...

ADDITIONAL READING

  • Nozu  K, Iijima  K, Kanda  K, et al. The pharmacological characteristics of molecular-based inherited salt-losing tubulopathies. J Clin Endocrinol Metab. ...

CODES

ICD10

E26.81 Bartter’s syndrome 

SNOMED

  • 236465009 Bartter’s syndrome with hypercalciuria and nephrocalcinosis (disorder)

  • 700107006 Bartter syndrome antenatal type 1 (disorder)

  • 700109009 Bartter syndr...

CLINICAL PEARLS

Bartter and Bartter-like syndromes are rare autosomal recessive, hypokalemic, salt-losing nephropathies that mimic diuretic effects. 

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