Endocrine Abstracts

Congenital hypopituitarism is a rare disease, occurring sporadically in the vast majority of cases, that is found more frequently in males. We are presenting the case of a 19 year old that addressed our clinic because of short stature and lack of development of secondary sexual traits. During the clinical assessment we found his height to be 146 cm, corresponding to -4.78 SDs, Tanner stage of pubertal development 1 and lack of apocrine axillary odor. The patient was inapetent and had progressive weight loss of about 10 kg in the last year. Hormonal tests confirmed that he had panhypopituitarism (the central adrenal insufficiency only becoming obvious shortly after starting levothyroxine replacement therapy). We performed a Synachten-stimulation test in which cortisole failed to stimulate adequately. Hypophyseal imaging showed pituitary hypoplasia - the most frequent neuroradiologic find associated with the disease. The unexpected find in this case, though, was marked hyperglicemia, further evaluation showing that the patient also had type 1 diabetes mellitus. Initial management consisted of replacement therapy with levothyroxine and hydrocortisone and insuline therapy -with the intent to start growth hormone replacement therapy as soon as possible - with good expected heightening prognostic, given that the patient has delayed bone age. The case stands out through the fact that it illustrates the intricate and particular interdynamic of the two co-occurring diseases - including the "sparing" effect of hypopituitarism on diabetes mellitus through insulinosensitivity and low levels of counterregulatory hyperglycemic hormones. Lastly, the interplaying patophysiology also offers a challenging management of such a case for the physician.