中国垂体柄阻断综合征临床特点和发病规律荟萃分析

Meta-analysis of clinical characteristics and pathogenetic regularity of pituitary stalk blocking syndrome in China

目的分析我国垂体柄中(阻)断综合征(pituitary stalk interruption syndrome,PSIS)患者的临床特点及发病规律。方法通过《中国期刊全文数据库》(CHKD)和万方数据检索中国PSIS相关报道,对PSIS进行荟萃分析。结果自2004年以来14省市文献33篇共报道311例,男女比例为257∶54,其中183例有分娩异常史或脑缺氧史。MRI检查239例垂体柄缺如,62例垂体柄变细。190例为多种腺垂体激素缺乏,75例为单一生长激素缺乏。垂体柄缺如者以多种腺垂体缺乏为主,而垂体柄变细者以单一生长激素缺乏为主,两组差异有统计学意义(P<0.01)。进入青春期者临床表现均为生长发育迟缓和第二性征缺如或延迟。该病患者治疗普遍不足且较晚,仅有12例(3.86%)报道接受生长激素治疗。结论新生儿异常分娩史或脑缺氧史可能是PSIS的一个重要病因,MRI是诊断PSIS的重要方法,疑诊者及时检查并测定腺垂体激素有利于早期诊断和治疗,加强围产期胎儿保护可能是防治PSIS的重要手段。

Objective To analyze the clinical characteristics and pathogenesis of the patients with pituitary stalk interruption （block） syndrome （PSIS） , there by better comprehending this disease. Methods A new case of PSIS was reported, and a literature review was conducted of Chinese reports on PSIS retrieved in CHKD and WANFANG Medical database. Results Since 2004, 311 cases of PSIS were reported in 33 papers from 14 provinces and cities, including this case. PSIS had a male predominance （257 male, 54 female）. 183 of 311 had an abnormal labor or cerebral anoxia history. Magnetic resonance imaging （MRI） showed pituitary stalk rupture in 239 cases and significant thin pituitary stalk in 62 cases. Multiple hormone deficits were observed in 190 patients and single growth hormone deficiency in 75 cases. The patients with pituitary stalk rupture have a predominance of multiple hormone deficits, and the patients with pituitary stalk necking have a predominance of growth hormone deficiencies （P 〈 0. 01 ）. The major clinical manifestations in adolescent patients are growth retardation and secondary sexual characters missing or delay, and the babies with PSIS are characterized by repeated attack of hypoglycemia and convulsions. Generally, the treatment for PSIS patients is insufficient and delayed. Only 12 cases （3.86%） received the growth hormone treatment. Conclusion Abnormal labor or cerebral anoxia history is probably one of the most important causes of PSIS ; MRI is an important method for diagnosis of PSIS. For suspected PSIS patients, cerebral MRI and endocrinological evaluation can be helpful for early diagnosis and treatment; strengthening protection for the perinatal fetus may be an important means for prevention and control of PSIS.