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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.
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June 5, 2023 at 5:56 pm
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.