das Neves M M, Oliveira C V, Chebli J M, de Oliveira S C
Disciplina de Gastroenterologia Clínica da Escola Paulista de Medicina, São Paulo.
Rev Assoc Med Bras (1992). 1994 Oct-Dec;40(4):297-9.
Hereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. Recurrent abdominal pain is the most common clinical manifestation, with onset in childhood or adolescence. PURPOSE--Report of a case of HP with atypical presentation and a review of the literature. METHODS--A non-alcoholic patient, without history of abdominal pain, with steatorrhea and malnutrition was investigated. The diagnostic evaluation revealed severe chronic pancreatitis. Two close relatives with early onset calcifying pancreatitis were detected. Epidemiologic and clinical features of HP were reviewed. CONCLUSION--Although uncommon, HP should be regarded in the differential diagnosis of chronic pancreatitis. Familial screening of suspected cases should be routinely performed.
遗传性胰腺炎（HP）是慢性胰腺炎的一种罕见病因。反复腹痛是最常见的临床表现，多在儿童期或青春期发病。目的——报告一例表现不典型的HP病例并复习相关文献。方法——对一名无腹痛病史、患有脂肪泻和营养不良的非酒精性患者进行调查。诊断评估显示为重度慢性胰腺炎。发现两名近亲患有早发性钙化性胰腺炎。对HP的流行病学和临床特征进行了复习。结论——尽管不常见，但在慢性胰腺炎的鉴别诊断中应考虑HP。对疑似病例应常规进行家族筛查。
