Maaz E
Z Gesamte Inn Med. 1982 Aug 1;37(15):500-4.
The clinical picture of the hereditary chronic relapsing calcifying pancreatitis is demonstrated on the basis of a family. In 7 members of the family the disease could be ascertained, in other 3 members it is probably existing. Main symptom in all patients are severe relapsing epigastric pains with manifestations already in childhood or time of youth. Calcifications of the pancreas were always found and except one female patient an exocrine insufficiency of the pancreas with superficial steatorrhoea. A diabetes mellitus became manifest only in three of the seven patients. A larger attention is to be paid to the diagnosis of this special form of the chronic relapsing pancreatitis, in order to avoid long diagnostic wrong ways and non-indicated laparotomies in etiologically not clarified epigastric complaints. The therapy of the hereditary pancreatitis is conservative. Only in complications or in a not controllable so-called syndrome of pain it is recommended to decide on an operative approach, as in the family demonstrated in two female patients.
基于一个家族展示了遗传性慢性复发性钙化性胰腺炎的临床表现。在该家族的7名成员中确诊了该病,另外3名成员可能患有此病。所有患者的主要症状是严重的复发性上腹部疼痛,在儿童期或青年期就已出现症状。总是能发现胰腺钙化,除一名女性患者外,其他患者均有胰腺外分泌功能不全伴轻度脂肪泻。仅7名患者中的3名出现了糖尿病。对于这种特殊形式的慢性复发性胰腺炎的诊断应给予更多关注,以避免在病因不明的上腹部疼痛中出现长期的诊断错误和不必要的剖腹手术。遗传性胰腺炎的治疗是保守的。只有在出现并发症或所谓的疼痛综合征无法控制时,才建议采取手术治疗,如在该家族中的两名女性患者所示。