Hypothalamic hamartoma in children
https://doi.org/10.14341/probl200652421-27
Abstract
The paper presents the data of examination and treatment of children with pubertas precox (PP) resulting from hypothalamic hamartoma, a rare malformation of the brain. It analyzes the results of 27 cases that is one of the largest series of cases in the world literature. Of the greatest interest are the clinical and anatomic comparisons presented by the authors, which make it possible to define the involvement of different hypothalamic structures in the development of specific endocrine disorders. When located parahy-pothalamically, hamartomas (a peduncular type) are characterized by the symptoms of PP; when located intrahypothalamically (a sessile type), in addition to PP, there are convulsive typical symptoms (compulsive laughter attacks). At the suprahypothalamtc site, there are commonly diencephalic disorders (obesity, diabetes insipidus). The paper provides the results of surgical and hormonal treatment (the use of long-acting luteinizing hormone-releasing hormone analogues), evidence for the high efficiency of medical treatment and the low efficiency attended by a high risk of complications, surgical treatment.
About the Authors
T V Semicheva
ГУ Эндокринологический научный центр (дир. - акад. РАН и РАМН И. И. Дедов) РАМН
S K Gorelyshev
Институт нейрохирургии им. Н. Н. Бурденко (дир. - акад. РАМН А. Н. Коновалов) РАМН, Москва
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Review
For citations:
Semicheva T.V., Gorelyshev S.K. Hypothalamic hamartoma in children. Problems of Endocrinology. 2006;52(4):21-27. (In Russ.) https://doi.org/10.14341/probl200652421-27
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