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目的探讨儿童下丘脑垂体肿瘤的内分泌改变。方法总结下丘脑垂体肿瘤患儿18例临床资料。结果 18例下丘脑垂体肿瘤中,颅咽管瘤3例,错构瘤2例,生殖细胞瘤13例。男12例,女6例,平均年龄(8.91±4.47)岁。颅咽管瘤3例临床首发症状分别为生长发育迟缓、中枢性尿崩症和颅内压增高。2例错构瘤均以婴幼儿中枢性同性性早熟为首发症状。13例颅内生殖细胞瘤首发症状均为中枢性尿崩症,同时4例伴有视野、视力改变,1例伴中枢性早熟,3例伴颅内压增高改变。结论对于儿童生长发育迟缓、中枢性尿崩症和婴幼儿中枢性性早熟均需注意有无下丘脑垂体肿瘤的可能。对儿童青少年中枢性尿崩症者不仅要常规做MRI检查,而且即使无肿瘤占位的也要定期复查。如同时出现多种内分泌改变时,应定期进行相关激素、垂体MRI的随访。
Objective To investigate the endocrine changes of hypothalamus pituitary tumors in children. Methods The clinical data of 18 patients with hypothalamic pituitary tumor were summarized. Results 18 cases of hypothalamic pituitary tumors, craniopharyngioma in 3 cases, hamartoma in 2 cases, 13 cases of germ cell tumors. There were 12 males and 6 females, with an average age of (8.91 ± 4.47) years. Three cases of craniopharyngioma clinical symptoms were growth retardation, central diabetes insipidus and increased intracranial pressure. 2 cases of hamartoma are infants with same-sex precocious puberty as the first symptom. 13 cases of intracranial germinoma were the first symptom of central diabetes insipidus, while 4 cases with vision, visual acuity, 1 case with precocious puberty, 3 cases with increased intracranial pressure changes. Conclusion For children with growth retardation, central diabetes insipidus and infant precocious puberty should pay attention to the possibility of hypothalamic pituitary tumors. On children and adolescents with central diabetes insipidus not only to do routine MRI examination, and even without tumor occupancy should also be regularly reviewed. If a variety of endocrine changes occur at the same time, should be regularly related hormone, pituitary MRI follow-up.