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·700·  http: //www.chinagp.net   E-mail: zgqkyx@chinagp.net.cn                    February  2023, Vol.26  No.6


           analysis was conducted on their clinical manifestations,laboratory and imaging test result,treatments and follow-up results.
           Results The age at the diagnosis of hypopituitarism for all cases ranged from 9 to 20 years old,and that at the diagnosis of
           hepatic cirrhosis was 16 to 24 years old,with an interval of 3 to 14 years old. The causes of hypopituitarism were germ cell tumor
           surgery (4 cases),craniopharyngioma surgery (2 cases),radiotherapy for nasopharyngeal carcinoma (1 case) and
           pituitary stalk interruption syndrome (1 case). All cases received no standardized hormone replacement therapy before cirrhosis
           was diagnosed by biopsy(2 cases) or imaging(6 cases). Fatigue,anorexia,hypoplastic external genitalia,short stature,
           recurrent upper respiratory tract infection and bleeding were the most common clinical manifestations. All cases had abnormalities
           in pituitary-thyroid axis,pituitary-gonadal axis,growth hormone,insulin-like growth factor-1,routine blood markers,
           four markers of hepatic fibrosis and liver imaging results. Moreover,it was found that 7 cases were also with abnormalities in
           antidiuretic hormone and hepatic function,and 6 cases were with abnormalities in pituitary-adrenal axis,coagulation function
           and serum lipids. All patients received treatment with desmopress in acetate,thyroid hormone,hydrocortisone and sex hormones
           as necessary. Four patients also received growth hormone replacement therapy. One-year follow-up indicated that,all cases
           had significantly improved levels of leukocyte,platelet,aspartate aminotransferase,alkaline phosphatase,total bilirubin,
           indirect bilirubin,total cholesterol,low-density lipoprotein,high-density lipoprotein,fibrinogen and four markers of hepatic
           fibrosis after treatment(P<0.05). However,no significant post-treatment improvement was found in the levels of hemoglobin,
           alanine aminotransferase,gamma-glutamyl transpeptidase,direct bilirubin,triacylglycerol and D-dimer,and in prothrombin
           time and endogenous prothrombin potential (P>0.05). Conclusion  All these 8 patients with hypopituitarism had hormone
           deficiency for several years before the diagnosis of hepatic cirrhosis,and abnormalities in thyroid hormone,sex hormone,
           growth hormone,insulin-like growth factor-1,routine blood markers,four markers of hepatic fibrosis and imaging results,
           with fatigue,anorexia,hypoplastic external genitalia,short stature,recurrent upper respiratory tract infection and bleeding
           as the most common clinical manifestations. One-year standardized hormone replacement therapy significantly improved the
           abnormalities in leukocyte,platelets,total cholesterol,high-density lipoprotein,low-density lipoprotein,fibrinogen and four
           markers of hepatic fibrosis.
               【Key words】 Hypopituitarism;Liver cirrhosis;Non-alcoholic fatty liver disease;Hormone replacement therapy;
           Signs and symptoms


               腺垂体功能减退症是指某种原因导致垂体前叶激素                          硬化原因明确和原发血液系统疾病患者。本研究获得
           分泌减少而出现的一组症候群,与中枢性尿崩症同时存                            郑州大学第一附属医院伦理委员会批准(编号:2022-
           在时称为全垂体功能减退症,轻度的正细胞性贫血是其                            KY-0229-002),患者均知情同意。
           最常见的血液系统表现          [1] ,白细胞和血小板同时减少                1.2 方法 总结所有患者的临床表现、实验室及影像
           的情况较为少见。非酒精性脂肪性肝病(non-alcoholic                     学检查结果、治疗方法及随访结果。
           fatty liver disease,NAFLD)是指除外酒精和其他明确肝              1.3 统计学方法 采用 SPSS 21.0 软件对数据进行统计
                                                                                           -
           损伤因素所致的,以弥漫性肝细胞大泡性脂肪变为主                             分析,正态分布的计量资料以(x±s)表示,治疗前后
           要特征的临床综合征,疾病谱包括单纯性肝脂肪变、                             比较采用配对 t 检验。以 P<0.05 为差异有统计学意义。
           非 酒 精 性 脂 肪 性 肝 炎(non-alcoholic steatohepatitis,    2 结果
           NASH)、 肝 硬 化 和 肝 细 胞 肝 癌。 垂 体 功 能 减 退 时             2.1 一般情况 本研究纳入的 8 例患者中男 6 例、女 2
           NAFLD 的发病风险增加,合并肝硬化时可能继发脾功                          例。诊断垂体功能减退症时年龄为 9~20 岁,平均年龄
           能亢进导致三系血细胞减少。本研究对近年收治的 8 例                          12 岁;诊断肝硬化时年龄为 16~24 岁,平均年龄 19.9 岁;
           垂体功能减退症合并肝硬化患者的临床资料进行回顾性                            中间相隔时间 3~14 年,平均时间 7.9 年,见表 1。1 例
           分析,以提高临床医师对该类疾病的认识。                                 患者为标准体质量,7 例患者为腹型肥胖。
           1 资料与方法                                             2.2 发病原因及诊断 发病原因:生殖细胞瘤手术史 4
           1.1 临床资料 回顾性分析 2010 年 1 月至 2021 年 12                例、颅咽管瘤手术史 2 例、鼻咽癌放疗后 1 例、垂体柄
           月郑州大学第一附属医院收治的 8 例垂体功能减退症合                          阻断综合征 1 例;腺垂体功能减退症 1 例、全垂体功能
           并肝硬化患者的临床资料。入组标准:(1)有不同程                            减退症 7 例。诊断:肝脏活检证实为肝硬化 2 例、影像
           度的垂体功能低下临床表现和 / 或鞍区疾病史,垂体 - 靶                       学诊断肝硬化 6 例。
           腺轴激素检测明确存在 1 项或多项腺垂体激素分泌功能                          2.3 临床表现及合并症 乏力、食欲不振 8 例;外生
           低下;(2)临床或病理诊断为肝硬化。排除标准:肝                            殖器发育不良 7 例;身材矮小 7 例;消化道出血 2 例;
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