IDIOPATHIC HYPOPARATHYROIDISM WITH PAPILLEDEMA IN A BOY SIX YEARS OF AGE

A case is reported of a boy with idiopathic hypoparathyroidism, moniliasis and the celiac syndrome. Moniliasis developed at the age of 16 months and was apparently cured promptly but evidence of C. albicans infection was found at several subsequent visits. At the age of 5 years he began to pass loose stools and at 5 10/12 years was diagnosed as having the celiac syndrome. At 6 6/12 years he entered hospital in severe tetany and was found to have hypoparathyroidism. The bones were normal by roentgenogram and no intracranial calcification was observed. While he was in the hospital bilateral papilledema developed, then receded, apparently in response to therapy for the hypoparathyroidism. Treatment consisted of calcium gluconate intravenously and large doses of calcium gluconate, water-soluble vitamin D, AT-10 and creamalin® by mouth, as well as a high protein, low fat, low phosphorous diet. The stools became normal shortly after this diet was started. Brief reference is made to his brother in whom moniliasis developed at the age of 17/12 years and the celiac syndrome at about 3 years. At 9 years he still has no evidence of hypoparathyroidism. The four other siblings are well. The 22 previously reported cases of papilledema associated with hypoparathyroidism and the seven previously reported cases of moniliasis associated with hypoparathyroidism are briefly reviewed.