Late onset of adrenocortical failure in GH deficiency with invisible pituitary stalk: A case report of a 48-year-old Japanese man and review of the literature

Abstract

ACTH deficiency gradually develops in patients with growth hormone deficiency (GHD) who have abnormalities of the pituitary stalk on magnetic resonance imaging (MRI) following perinatal complications. We report here a rare case of GHD manifesting ACTH deficiency in middle age. A 48-year-old male patient was admitted to our hospital due to fever and hyponatremia. He was diagnosed as GHD and hypothyroidism at the age of 9, and had received lysine treatment until age 20, which was then replaced by thyroid hormone. He was not mentally retarded, but was the shortest in his class throughout his schooldays, reaching a final height of 148 cm. Hormonal examination revealed the presence of hypoadrenalism as indicated by poor responses of plasma cortisol to intravenous administration of corticotropin-releasing hormone (CRH) and insulin-induced hypoglycemia. Plasma ACTH responded well to CRH, but not to insulin-induced hypoglycemia, indicating that his hypoadrenalism was of hypothalamic origin. MRI showed an invisible pituitary stalk and relatively small pituitary gland. Since he had a perinatal abnormality, the damage around the pituitary and GHD could have originated from birth. In the literature, around 60% of GHD patients with pituitary stalk abnormalities develop hypoadrenalism due to ACTH deficiency, and more than 90% of such cases are diagnosed by age 30. We suggest that the appearance of hypoadrenalism should be carefully monitored in GHD patients with pituitary stalk abnormalities even after they reach middle age.