What is Acquired Bartter Syndrome?

From the Guidelines

Acquired Bartter syndrome should be managed by addressing the underlying cause and providing supportive care with oral potassium supplementation and potassium-sparing diuretics, as recommended by the European Rare Kidney Disease Reference Network Working Group for Tubular Disorders 1.

Key Considerations

• The condition is characterized by electrolyte imbalances, particularly low potassium levels, metabolic alkalosis, and normal to low blood pressure.
• Common causes include prolonged use of loop diuretics, certain antibiotics, chemotherapy agents, and chronic conditions such as autoimmune disorders or sarcoidosis.
• Treatment focuses on addressing the underlying cause by discontinuing the offending medication when possible.

Management Strategies

• Oral potassium supplementation (typically potassium chloride 40-100 mEq daily in divided doses) to manage hypokalemia.
• Potassium-sparing diuretics like spironolactone (25-100 mg daily) to reduce urinary potassium wasting.
• Prostaglandin inhibitors such as indomethacin (25-50 mg three times daily) may be beneficial in reducing urinary potassium wasting.
• Regular monitoring of electrolytes, particularly potassium, magnesium, and calcium levels, is essential.
• Patients should maintain adequate fluid intake and may need to follow a high-potassium diet including foods like bananas, oranges, and potatoes.

Important Considerations

• The condition typically improves once the underlying cause is addressed, though some patients require long-term electrolyte supplementation and medication management to maintain normal kidney function and electrolyte balance.
• Genetic testing may be considered to identify the underlying cause of the condition, particularly in cases where the diagnosis is uncertain 1.
• Prenatal intervention with NSAIDs may be considered in cases of polyhydramnios secondary to Bartter syndrome, but close monitoring is mandatory due to the risks of fetal ductus arteriosus constriction and other complications 1.

From the Research

Definition and Causes of Acquired Bartter Syndrome

• Acquired Bartter syndrome is a rare condition that can be caused by various factors, including autoimmune disorders, endocrine diseases, and connective tissue diseases 2.
• It can also be associated with other conditions such as sarcoidosis, which can lead to a Bartter-like syndrome 3.
• The syndrome is characterized by hypokalemia, metabolic alkalosis, and other electrolyte imbalances.

Clinical Presentation and Diagnosis

• The clinical presentation of acquired Bartter syndrome can vary, but common symptoms include neuromuscular weakness, hypokalemia, and metabolic alkalosis 2.
• Diagnosis is often made through a combination of serum and urinary biochemistry analysis, as well as autoimmune profiling 2.
• It is essential to consider acquired Bartter syndrome in the differential diagnosis of patients with hypokalemia and metabolic alkalosis, especially those with underlying autoimmune or endocrine diseases.

Treatment and Management

• Treatment of acquired Bartter syndrome typically involves a combination of potassium supplementation, potassium-sparing diuretics, and other medications such as indomethacin and aldosterone antagonists 2, 4.
• In some cases, steroid-based regimens may be effective in managing the condition 2.
• Early diagnosis and treatment are crucial to prevent long-term consequences such as growth failure, nephrocalcinosis, and end-stage renal disease 5.

Related Conditions and Subtypes

• Bartter syndrome can be classified into different subtypes based on the underlying genetic cause, including types I, II, III, IV, and V 5.
• Each subtype has distinct clinical and biochemical features, and understanding these differences is essential for proper diagnosis and management.
• Acquired Bartter syndrome can also be distinguished from other conditions such as Gitelman's syndrome, which has similar clinical features but a different underlying cause 3.