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A 43-Year-Old Male with Untreated Panhypopituitarism due to Absence of the Pituitary Stalk: From Dwarf to Giant

Departments of Internal Medicine (D.T.d.O., P.H.L.M.G.-D.), Clinical Chemistry (M.K.), and Radiology (P.H.H.), MCH Westeinde Hospital, 2501 CK The Hague, The Netherlands; and Department of Pediatrics, Leiden University Medical Center (J.M.W.), 2300 RC Leiden, The Netherlands

Address all correspondence and requests for reprints to: Dr. D. T. den Ouden, Department of Internal Medicine, MCH Westeinde Hospital, P.O. Box 432, 2501 CK The Hague, The Netherlands. E-mail: denouden99{at}hotmail.com.

Abstract

A 43-yr-old male was referred because of an x-ray made after a fall, which showed open epiphysis of the arm. The man had always been short for his age; during childhood he once consulted a pediatrician because of short stature, but thereafter he never sought medical attention. At age 18 yr he was not allowed to join the army because of his height of 147 cm. He continued to grow steadily and finally reached 193 cm. He had no complaints and considered himself reasonably fit. Physical examination showed a disproportional man with a body mass index of 29.3 kg/m² and Tanner stage P1G1. Laboratory investigations showed hormone levels consistent with multiple pituitary deficiency, with dynamic tests consistent with hypothalamic or pituitary stalk disease. Magnetic resonance scanning of the brain showed a small anterior pituitary remnant, no pituitary stalk, and an ectopic neurohypophysis.

This case of untreated panhypopituitarism shows a particular growth curve with an average growth velocity of 2 cm/yr, resembling patients with estrogen receptor mutation or aromatase deficiency. A literature study of other adult patients with untreated panhypopituitarism shows a variable growth pattern. Some speculations about possible reasons for this variability in clinical characteristics are presented.

This article has been cited by other articles:

				M. Faustini-Fustini, A. Balestrieri, V. Rochira, and C. Carani The Apparent Paradox of Tall Stature with Hypopituitarism: New Insights from an Old Story J. Clin. Endocrinol. Metab., August 1, 2003; 88(8): 4002 - 4003. [Full Text]

				D. T. den Ouden, M. Kroon, P. H. Hoogland, P. H. L. M. Geelhoed-Duijvestijn, and J. M. Wit Author's Response: The Apparent Paradox of Tall Stature with Hypopituitarism: New Insights from an Old Story J. Clin. Endocrinol. Metab., August 1, 2003; 88(8): 4003 - 4003. [Full Text]