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Question: NAGMA

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You are seeing a 15 year old girl who presents with acute bilateral lower leg weakness / near-paralysis. She is found to be hypokalemic, which led to her weakness and paralysis. She also has a hyperchloremic (non anion gap) metabolic acidosis. She was previously healthy and denies ingestion of any substances. She has not been having diarrhea. Her urine pH is 6.0. 

What condition do you suspect?


pemsou5_wp • May 30, 2023

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  1. Kelly June 5, 2023 - 5:56 pm Reply

    A) Type 1 Distal renal tubular acidosis
    Renal tubular acidosis are renal disorders of acid-base handling. Type 1 Distal tubule RTA is due to impaired acidification of urine. Urine pH is always > 5.4. Type 2 Proximal tubule RTA is due to impaired bicarbonate resorption. Urine pH is variable. Type 3 RTA is an extremely rare hereditary form. Type 4 RTA results in HYPERkalemia and is the most common type of RTA in adults due to diabetic nephropathy. A common cause of types 1 and 2 RTAs in older children and adults is autoimmune diseases. Other causes of RTAs are: hereditary forms, certain drugs (e.g. ifosfamide, ibuprofen, Toluene glue-sniffing), and renal diseases leading to tubule damage. Type 1 distal RTA may present with failure to thrive, Rickets and osteomalacia, nephrocalcinosis and recurrent UTIs, muscle weakness, and arrhythmia. Type 2 proximal RTA may also result in hypokalemia and Rickets. Periodic hypokalemic paralysis should have isolated hypokalemia. Other causes of non-anion gap metabolic acidosis (NAGMA) include: NS infusion, resolving DKA, diarrhea (or other GI bicarb losses such as high-output fistulas), and exogenous acid.

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