Online edition:ISSN 2758-089X

A Case of Hb S/A -α-thalassemia Exhibiting Quadriplegia Due to Distal Renal Tubular Acidosis

A 46-year-old negro seaman who called at Port Mizushima, Kurashiki City, from West Africa on May 21, 1981 developed quadriplegia shortly after having taken a tablet of an antipyretic agent on the ship. At the Port Clinic in Mizushima, hypopotassemia was detected and Guillain Barre syndrome was suspected. Adrenocorticosteroids therapy was started, but he became dyspneic because of the progression of the paralysis up to the level of respiratory muscle. He was, therefore, transferred to our emergency center and hospitalized. On the sixth hospital day (May 27), clinical manifestations improved by intravenous administration of potassium. Diagnosis of distal renal tubular acidosis was entertained on the basis of the presence of metabolic acidosis, hypopotassemia and the absence of acidification of urine by short duration NH4C1 acid-loading test. The hematological studies revealed a combination of sickle cell trait (Hb S/A) with α-thalassemia trait. It is well known that sickle cell anemia (Hb S/S) occasionally causes secondary distal renal tubular acidosis. However, the occurrence of renal tubular acidosis in sickle cell trait (Hb S/A) and in α-thalassemia trait (αTh/A) has not yet been reported in the literature. It is therefore thought that our observation on this case will deserve special description as one of the possible clinical signs of sickle cell trait.

Takemoto Y, et al