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BETA v.3.0

2025 ICD-10-CM code E26.81

Bartter's syndrome

Use additional codes to specify any associated complications, such as nephrocalcinosis or cardiac arrhythmias.

Medical necessity for diagnostic testing and treatment is established by the presence of characteristic symptoms, electrolyte imbalances, and hormonal abnormalities consistent with Bartter syndrome. Genetic testing may be necessary to confirm the diagnosis and determine the specific subtype.

Diagnosis and management of Bartter syndrome involves a comprehensive assessment of clinical symptoms, blood and urine electrolyte levels, and hormone assays. Treatment focuses on correcting electrolyte imbalances with supplements, NSAIDs, and potentially potassium-sparing diuretics.

In simple words: Bartter syndrome is a rare, inherited kidney problem where the kidneys lose too much salt, leading to imbalances in essential minerals like potassium and calcium. This can cause symptoms like weakness, excessive thirst, and frequent urination.

Bartter syndrome is a group of rare inherited kidney disorders characterized by impaired salt reabsorption in the thick ascending limb of Henle's loop. This leads to excessive salt wasting, hypokalemic alkalosis, hypercalciuria, and increased levels of plasma renin and aldosterone, typically without hypertension.

Example 1: A newborn presents with polyuria, dehydration, and failure to thrive. Laboratory findings reveal hypokalemia, metabolic alkalosis, and hypercalciuria, leading to a diagnosis of antenatal Bartter syndrome., A 5-year-old child experiences frequent urination, muscle weakness, and fatigue. Blood tests show hypokalemia and metabolic alkalosis, while urine analysis reveals hypercalciuria, suggesting classic Bartter syndrome., A young adult is found to have hypokalemia and metabolic alkalosis during a routine check-up. Further investigation reveals hypocalcemia and increased urinary salt wasting, consistent with autosomal dominant hypocalcemia with Bartter syndrome.

Documentation should include detailed family history, clinical presentation, laboratory findings (serum and urine electrolytes, renin, aldosterone, calcium), and genetic testing results (if available).

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