Shigehiko Kamoshita

Congenital hyperparathyroidism secondary to maternal hypoparathyroidism.

Twin premature infants delivered by a mother who had been hypoparathyroid for several years, after thyroidectomy, died in the newborn period of hyaline membrane disease and intracranial hemorrhage. Both infants had marked parathyroid hyperplasia and severe osteoporosis and osteitis fibrosa of bones, typical of hyperparathyroidism. Reports of only 3 such infants were found in the literature. Congenital hyperparathyroidism due to maternal hypoparathyroidism is less common than neonatal hypocalcemia due to maternal hyperparathyroidism and appears to have a better prognosis than familial congenital hyperparathyroidism with clear-cell parathyroid hyperplasia.

Renal effects of dichloroacetate in vivo

We have investigated the effect of dichloroacetate (DCA) on the urinary excretion of lactate and pyruvate in a patient with congenital lactic acidosis and in healthy human controls. DCA administered orally in doses of 50 mg/kg and 30 mg/kg decreased plasma lactate and pyruvate of both the patient and the controls, while the urinary excretion of lactate and pyruvate was increased. However, these urinary increments were too small to contribute to the decrease of plasma lactate and pyruvate.