RECURRENT D-LACTIC ACIDOSES WITH ENCEPHAL OPATHY IN A BOY WITH THE SHORT-BOWEL SYNDROME

For two years after surgical small-intestine duplication a 9-year-old boy with the short bowel syndrome had recurrent acidoses which caused severe failure to thrive. During the acidotic crises he had behavioura l disorders, unsteady gait, indistinct speech, lid raising weakness wi th vision paresis and occasional somnolence. These signs disappeared a fter the aciduria had been treated with high doses of bicarbonate. D-l actic acidosis was finally diagnosed by simultaneously determining D-l actate (8.9 mmol/l [normal < 0.5]) and L-lactate (1.4 mmol/l [normal ( 1.78]) during an episode of aciduria (pH 7.3, base excess -11.8 mmo/l ). Further acidotic crises were prevented by a carbohydrate-modified d iet, on which he gained 8 kg in one year.