IDIOPATHIC HYPOPARATHYROIDISM AND IDIOPATHIC ADRENAL CORTICAL INSUFFICIENCY OCCURRING WITH CYSTIC FIBROSIS OF THE PANCREAS

Abstract

Only 2 previous cases of this duoglandular disturbance have been reported with autopsy confirmation. This patient is unique in that she also had cystic fibrosis of the pancreas. The patient was a 21-year-old white female with hypocalcemic tetany from infancy, hypoprothrombinemic bleeding and steatorrhea. She was 140 cm tall, weighed only 24 kg, and had chronic moniliasis, chronic pulmonary histoplasmosis, and vitiligo. She required innumerable hospitalizations during her lifetime for episodes of tetany, bleeding or diarrhea. But she was maintained in fair control with a regimen of calcium salts, vitamins K and D in large doses, and a gluten-free diet. Three years before her demise, she developed symptoms of Addison''s disease, confirmed by failure to respond to adrenocorticotropic hormone with increased 17-hydroxycorticosteroid excretion. Subsequently she did fairly well with cortisone replacement therapy. In Jan, 1958, she was seen with anorexia, nausea, weakness, ataxia, dysphagia, and weight loss. Her serum Ca was 12.4 mg/100 ml, and serum phosphate was 2.1 mg/100 ml. The diagnosis of hyper-vitaminosis D was made, and she rapidly responded to discontinuing calcium salts and vitamin D, and administration of hydrocortisone. Shortly thereafter she developed an obscure fever. Cultures of blood, feces, urine and spinal fluid were negative. Post-mortem examination revealed multiple Staphylococcus aureus, coagulase positive abscesses, cystic fibrosis of the pancreas, absence of parathyroid tissue, and hypoplasia of the adrenal cortices.