Succinyl-CoA:3-ketoacid CoA-transferase (SCOT) deficiency is, apparently, a rare disorder. Only nine patients have been reported since it was first described in 1972. The patients have various ethnic backgrounds: northern European,1-4 African-American, Japanese, and African. This communication is to report a tenth patient from another far eastern country, Pakistan, and to suggest that this disease may not be very rare but rather is underdiagnosed.

This infant's difficulties began in the newborn period. The pregnancy had been uneventful; the birth weight was 7 lb, 8 oz. He was readmitted to the hospital at 38 hours of age with a history of breathing difficulties since birth. He was lethargic and hypothermic; arterial blood gas determination revealed a pH of 7.16; the bicarbonate was 9.3, and there was a base deficit of −17.4. After correction of his acidosis he was started on a normal infant formula but became acidotic again....

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