Pseudohypoparathyroidism

Little is known of the long-term outcomes of pseudohypoparathyroidism (PHP) because the condition is rare, and published data are confined to case series and case reports. The underlying cause of PHP is incurable. However, some patients have resolution of hypocalcemia with time as calcium homeostasis adapts to parathyroid hormone (PTH) resistance. Those who do not adapt to PTH resistance require lifelong calcium supplementation. Patients with associated hypothyroidism require long-term levothyroxine therapy. Rarely, other endocrinopathies can occur in type Ia or Ic PHP. Gonadotropin resistance can cause delayed puberty or infertility. Growth hormone-releasing hormone (GHRH) resistance can produce growth hormone deficiency. It is important to remain alert for symptoms and signs of these endocrinopathies, and to initiate testing of the gonadotropin or GHRH axes if required.