ZOLLINGER-ELLISON SYNDROME CAN BE THE INITIAL ENDOCRINE MANIFESTATION IN PATIENTS WITH MULTIPLE ENDOCRINE NEOPLASIA-TYPE-I
ZOLLINGER-ELLISON SYNDROME CAN BE THE INITIAL ENDOCRINE MANIFESTATION IN PATIENTS WITH MULTIPLE ENDOCRINE NEOPLASIA-TYPE-I
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DOI：
10.1016/0002-9343(94)90323-9
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发表时间：
1994-11-01
影响因子：
5.9
通讯作者：
JENSEN, RT
中科院分区：
文献类型：
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作者：
BENYA, RV;METZ, DC;JENSEN, RT
目得：为了确定是否与多发性内分泌瘤I型（MEN-I）的患者可以最初呈现Zollinger-Ellison综合征（ZES），并了解ZES是否表现出任何显着的特点时，它发生作为MEN-I的第一个表现。患者和方法：60例患者谁被称为ZES的临床研究中心进行了检查队列分析。28人有MEN-I，32人没有。在MEN-I患者中，我们分析了ZES的临床和生化表现与肿瘤相关的其他内分泌疾病之间的时间关系。为了确定以ZES为MEN-I首发症状的患者（n = 8）是否具有任何显著的临床特征，我们将其与一组年龄、性别和自症状发作以来与ZES一致的散发性ZES患者（n = 32）进行了比较。在28例ZES和MEN-I患者中，11例最初表现为ZES和甲状旁腺功能亢进（HP），1例仅表现为垂体疾病。8例患者（29%）表现出ZES的特征，随后出现HP的临床和生化证据，而相同数量的患者以相反的顺序出现这2种内分泌疾病。无论ZES和HP发生的顺序如何，从第一次诊断到第二次诊断的时间相似。首次出现ZES的患者的病程为9 ~ 177个月，首次出现HP的患者的病程为12 ~ 264个月。在首次诊断时，与偶尔出现ZES综合征的患者相比，作为MEN-I表现的ZES患者没有明显的ZES相关症状、生化检测或肿瘤影像学结果。MEN-I患者最初可表现为症状性胰腺内分泌肿瘤综合征，而无任何其他疾病表现。在患有ZES和MEN-I的患者中，多达三分之一的患者可能患有ZES，而没有任何其他内分泌病的证据。因此，假定为散发性ZES的患者应进行持续的MEN-I特征性内分泌疾病的生化筛查，并在未来进行MEN-I基因的遗传学研究。
PURPOSE: To determine whether patients with multiple endocrine neoplasia type I (MEN-I) can initially present with Zollinger-Ellison syndrome (ZES), and to learn whether ZES exhibits any distinguishing features when it occurs as a first manifestation of MEN-I.PATIENTS AND METHODS: Sixty patients who had been referred to a clinical research center with ZES were examined by cohort analysis. Twenty-eight had MEN-I and 32 did not. In patients with MEN-I, we analyzed the temporal relationships between the clinical and biochemical manifestations of ZES and the other endocrinopathies associated with the neoplasia. To determine whether patients who had ZES as a first manifestation of MEN-I (n = 8) had any distinguishing clinical characteristics, we compared them to a cohort of patients with established sporadic ZES (n = 32) matched for age, sex, and time since the onset of symptoms consistent with ZES.RESULTS: Of the 28 patients with ZES and MEN-I, 11 initially presented with ZES and hyperparathyroidism (HP) and 1 with evidence only for pituitary disease. Eight patients (29%) presented with features of ZES and developed clinical and biochemical evidence for HP later, while the same number developed these 2 endocrinopathies in the opposite order. In whichever order ZES and HP occurred, the time from the diagnosis of the first to the diagnosis of the second was similar. It ranged from 9 to 177 months in patients who presented with ZES first, and from 12 to 264 months in patients who presented with HP first.At the time of initial diagnosis, the patients who presented with ZES as a manifestation of MEN-I had no distinguishing ZES-related symptoms, biochemical assays, or tumor imaging results compared to the cohort of patients who had the syndrome sporadically.CONCLUSION: Patients with MEN-I can initially present with a symptomatic pancreatic endocrine tumor syndrome without any other disease manifestations. In patients with ZES and MEN-I, up to one third may present with ZES without evidence of any other endocrinopathy. Consequently, patients with presumed sporadic ZES should undergo continual biochemical screening for other endocrinopathies characteristic of MEN-I and, in the future, genetic studies for the MEN-I gene.