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Responses to Glucagon Infusion in Pseudohypoparathyroidism^*

ARNOLD S. BRICKMAN, HAROLD E. CARLSON and SEYMOUR R. LEVIN

Medical and Research Services, Sepulveda Veterans Administration Medical Center Sepulveda,California 91343;
Wadsworth Veterans Administration Medical Center Los Angeles, California 90073

Address allcorrespondence andrequests for reprints to: Arnold S. Brickman, M.D., Mineral Metabolism Section (665/lllE), Veterans Administration Medical Center, Sepulveda, California 91343.

Single or graded doses of glucagon (Eli Lilly) were given to patients with pseudohypoparathyroidism (PsHP) type I to examine thepossible presence of hormone resistance. The doses ofglucagon ranged from 0.25-15 µg/kg. The following individuals were studied: 13normal subjects, 5patients withlow erythrocyte N-protein activity (PsHP type Ia),and7 patients with normal erythrocyte N-protein activity (PsHP type Ib). Two additional patients with treated primary hypothyroidismwho were relatives ofa patient with PsHP type Ibwere also studied. The patients with PsHP type Ia had blunted plasma cAMP responses to all glucagon doses. In contrast, the patients with PsHP type Ib hadnormal cAMP responses to glucagon infusion. However, the 2 relatives of the patient with PsHP type Ib had clearly decreased cAMP responses to glucagon infusion; both had normal renal responses to PTHand were clinically and biochemically euthyroid at the time of study. Glucose responses o t glucagon were normal in both PsHP groups; the glucose response perunit cAMP response was slightly, butnot significantly, enhanced in PsHP type la patients. Glucagon resistance appears tobea common finding inpatients with PsHP typela, but notin those with PsHP type Ib.However, the observation of reduced glucagon responsivity in association with familial hypothyroidism in a kindred with PsHP type Ib suggests the possibility that this disorder mayalso cause disturbances in several hormone systems.

^* This work was supported by funds from the Research Service of the V.A.

Current address: Endocrine Section, Northport Veterans Administration Hospital, Northport, NewYork 11768.

Received July 14, 1986.

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