Endocrine Abstracts

Histiocytosis is a group of syndromes /synonyms: eosinophilicgranulomatosis, pulmonary Langerhans cell histiocytosis, fibrous lung disease, disease of smokers, non-lipid reticuloendotheliosis; disease of Hand–Schuller–Christian; disease of Letterer–Siwe/. It is a rare disease and its real frequency is unknown. In a reference center in the U.S. the disease is found in 5% in patients with lung biopsy carried out in connection with the diagnosis of interstitial lung disease.

In adults inflammation leads to a loss of elasticity (fibrosis) and destruction of the walls of pulmonary alveoli. In some patients spontaneous improvement (remissions) can be observed, while others progress to an end-stage fibrotic lung disease.

A woman, 32, was born prematurely with a congenital atresia of the esophagus, with operational adjustment and plastic of the esophagus at an early age. Her development was according to the age. From the summer of 2009 complaints of polydipsia and polyuriaappeared – up to 7 l/day, headaches and dizziness, impaired memory, amenorrhea. MRI showed a tumor in chiasmaopticum and pressing on the third ventricle. Operation was madeon 05.02.2010 – right-sided craniotomy and partial extirpation of the tumor. Histological result-Langerhans histiocytic tumor cell / tests: plasma cortisol−54.6 in the morning at 0800 and 2200 h in the evening, 75.9 (normal 70–240 IU/l; prolactin −54 IU/l; LH −0.048 IU/l; FSH −0.06 IU/l). Concentration of sample Zimnitski-diuresis with relative weight -1000.

In the course of treatment there was a sequential adjustment of hipocorticismand hypothyroidism, electrolyte abnormalities. PET/CT did not reveal any dissemination process.

We present a clinical case of isolated hypothalamic–pituitary disease a form of insipid diabetes, panhypopituitarism, foudroyant stroke and death.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.