Sestre Milosrdnice University hospital and Institute of Clinical Medical Research
Abstract
A case of a 45-year-old woman with untreated panhypopituitarism is presented. Hypopituitarism is a rare disorder consisting of multiple deficiencies of hormones originating from the adenohypophysis. It is divided into primary and secondary. Primary disorders manifest at the pituitary level, whereas secondary hypopituitarism implies hypothalamic affection. Partial or total loss of all pituitary hormones is called panhypopituitarism. Hypopituitarism can be slow and insidious or severe and life-threatening. The expression of symptoms largely depends on the patient\u27s age and the hormones involved. The leading symptom is growth retardation when the disorder develops in childhood or puberty. Sometimes years may elapse without accurate diagnosis, as in the case described. To date, only a few cases of untreated hypopituitarism have been published. In our patient, clinical examination revealed short stature and weak osteomuscular constitution, primary amenorrhea and lack of development of secondary sex characteristics. The patient\u27s clinical history revealed signs of hypopituitarism from childhood, which had been untreated until she presented to our clinic. The results of complete testing showed the lack of all pituitary hormones and antidiuretic hormone, which was consistent with panhypopituitarism, including central diabetes insipidus.Prikazuje se slučaj 45-godišnje bolesnice s neliječenim panhipopituitarizmom. Hipopituitarizam je rijedak poremećaj koji obilježava manjak više hormona prednjega režnja hipofize. Može biti primaran ili sekundaran. Primarni poremećaji nastaju na razini hipofize, dok su sekundarni na razini hipotalamusa. Djelomičan ili potpun manjak svih hormona hipofize naziva se panhipopituitarizam. Tijek hipopituitarizma može biti spor i podmukao ili nagao i životno ugrožavajući. Izražajnost simptoma ovisi prvenstveno o dobi bolesnika te zahvaćenim hormonima. Vodeći simptom je zaostajanje u rastu, kada bolest zahvati bolesnika u djetinjstvu ili pubertetu. Ponekad može proći niz godina bez potpune dijagnoze, kao što je opisano i u naše bolesnice. Do danas je objavljeno svega nekoliko slučajeva neliječenog hipopituitarizma. Kliničkim pregledom naše bolesnice nađen je nizak rast i slaba osteomuskularna građa, primarna amenoreja i nerazvijene sekundarne spolne značajke. Anamnestički podaci otkrivaju sliku hipopituitarizma od dječje dobi, ali bolesnica nije liječena do dolaska u našu kliniku. Rezultati sveobuhvatne obrade pokazali su manjak svih hormona hipofize i antidiuretičnog hormona, što govori u prilog panhipopituitarizma, uključujući i centralni dijabetes insipidus
