Rami B, Schneider U, Wandl-Vergesslich K, Frisch H, Schober E
University Children's Hospital, Vienna, Austria.
Acta Paediatr. 1998 Jan;87(1):112-4. doi: 10.1080/08035259850158010.
We report on a girl with central diabetes insipidus, growth hormone deficiency and bone lesions in multisystem Langerhans cell histiocytosis. Thickening of the pituitary stalk was detected by magnetic resonance imaging, which progressed over the course of the disease. During the observation period she developed primary hypothyroidism, which might be due to the extremely rare involvement of the thyroid gland in this disease. The girl underwent chemotherapy, which led to a regression of the Langerhans cell histiocytosis-lesion, but the hormone deficiencies persisted and substitution had to be continued. Langerhans cell histiocytosis should be included in the differential diagnosis in cases with pituitary stalk thickening and additional hypothalamic/pituitary hormone deficiencies, and in cases of acquired primary hypothyroidism, with or without enlargement of the thyroid gland and ultrasound findings similar to thyroiditis.
我们报告了一名患有中枢性尿崩症、生长激素缺乏症以及多系统朗格汉斯细胞组织细胞增多症伴骨病变的女孩。磁共振成像检测到垂体柄增粗,且在病程中逐渐进展。在观察期内,她出现了原发性甲状腺功能减退症,这可能是由于该病极少累及甲状腺所致。该女孩接受了化疗,这使朗格汉斯细胞组织细胞增多症病变有所消退,但激素缺乏症仍然存在,必须继续进行替代治疗。对于出现垂体柄增粗并伴有其他下丘脑/垂体激素缺乏症的病例,以及对于获得性原发性甲状腺功能减退症病例,无论甲状腺是否肿大且超声检查结果是否类似于甲状腺炎,均应将朗格汉斯细胞组织细胞增多症纳入鉴别诊断。