当前位置: 首页 > 期刊 > 《中华泌尿外科杂志》 > 2000年第4期
编号:10648206
多发性内分泌腺瘤ⅡA家系及临床分析(附四例报告)
http://www.100md.com 《中华泌尿外科杂志》 2000年第4期
内分泌腺肿瘤|诊断|治疗,关键词:
多发性内分泌腺瘤ⅡA家系及临床分析(附四例报告)

     张辉 强万明 高玉琪 白人驹 张辉 强万明(300052 天津医科大学总医院泌尿外科);高玉琪(300052 天津医科大学总医院内分泌科);白人驹(300052 天津医科大学总医院放射科) 中华泌尿外科杂志 2000 0 21 4


    关键词:内分泌腺肿瘤;诊断;治疗 期刊 zhmnwkzz 0 242-244 论 著 fur -->


    

【摘要】 目的 探讨多发性内分泌腺瘤ⅡA的家系及临床特点。 方法 回顾调查1988~1998年收治此病4例家系情况及临床资料。 结果 每个亲代不是完全发病甚至晚于子代发病,甲状腺髓样癌早于或同时于嗜铬细胞瘤发病,4例均为甲状腺髓样癌+嗜铬细胞瘤,无甲旁亢。 结 论 此种常染色体显性遗传病可出现外显不全,所累及腺体并不同时发病使临床症状复杂。血降钙素、尿VMA、甲状旁腺素测定,颈部及肾上腺区CT检查是诊断和随访此病的常规方法。

Apedigree and clinical study of 4 cases of multiple endocrine neop lasia ⅡA

ZHANG Hui,QIANG Wanming,GAOYuqi,et al.

    (The General Hospital of Tianjin Medical University,Tianjin 300052,China)

【Abstract】 Objective To investigate the pedigree and c linical features of multipleendocrine neoplasia ⅡA. Methods 4 cases ofmultiple endocrine neoplasia treated from 1988 to 1998 were s urveyed retrospectively. Results The diseasemight not ocur in the parental generation or it might occur even later then their filialgeneration.Medullary thyroid carcinoma might occur simultaneously or intermitten tly inthe two thyroid lobes and it might occur at the same time or earlier than the pheochromocytoma. Conclusions The autosomal domin ant inheritance is often incomplete.Theclinical feature is often complicated as the neoplasm in the concerned glands might notdevelop simultaneously.Assay of urine VMA and plasma calcitonin,CT scan of neck andadrenal areas are the routin e methods for the diagnosis and follow up studies.

     ......


您现在查看是摘要页,全文长 10019 字符