We describe four pregnant patients with distal renal tubular acidosis (type
I) (DRTA) whose initial presentation was rhabdomyolysis (RML) secondary to
severe hypokalemia. We draw attention to the unusual presentation of DRTA
during pregnancy, the low frequency of DRTA in adult patients and RML as in
itial manifestation. In one case the DRTA was secondary to Sjogren Syndrome
and the etiology was unknown in the rest of the cases. We discuss the pote
ntial pathogenic mechanisms to explain hypokalemic RML and the various caus
es of DRTA in adult patients.