Hoshina K, Kimura W, Ishiguro T, Tominaga O, Futakawa N, Bin Z, Muto T, Makuuchi M
Department of Surgery, University of Tokyo, Japan.
Hepatogastroenterology. 1999 Mar-Apr;46(26):1192-8.
The patient was a 22 year-old male. Hereditary chronic pancreatitis was suspected as a diagnosis since his mother's uncle had been operated on for chronic pancreatitis 14 years previously at the age of 64 years and his mother had been operated on for chronic pancreatitis with calculi 5 years previously at the age of 40 years. Surgery was needed, since: 1) he had experienced abdominal pain for 8 years; 2) endoscopic retrograde cholangiopancreatography (ERCP) revealed a marked irregular dilatation in the main pancreatic duct and a marked irregular dilatation and protein plugs in the ductule of the tail of the pancreas; and, 3) pancreatic functional diagnostic (PFD) test examination showed a 75% decrease in exocrine function. If a surgical procedure had not been performed, the patient would likely have experienced calculi formation in the pancreas and a further decrease in exocrine function. Since the patient was very young and had many protein plugs in the dilated ductule of the tail of the pancreas, we decided to perform a spleen-preserving Puestow's procedure with removal of the tail of the pancreas. Clinical and pathological findings of hereditary pancreatitis are reviewed.
该患者为一名22岁男性。由于其母亲的叔叔14年前64岁时因慢性胰腺炎接受了手术，其母亲5年前40岁时因慢性胰腺炎伴结石接受了手术，故怀疑该患者的诊断为遗传性慢性胰腺炎。由于以下原因需要进行手术：1) 他有8年的腹痛病史；2) 内镜逆行胰胆管造影（ERCP）显示主胰管明显不规则扩张，胰尾小导管明显不规则扩张并有蛋白栓；3) 胰腺功能诊断（PFD）试验检查显示外分泌功能下降75%。如果不进行手术，患者可能会出现胰腺结石形成，外分泌功能进一步下降。由于患者非常年轻，且胰尾扩张的小导管中有许多蛋白栓，我们决定行保留脾脏的Puestow手术并切除胰尾。本文对遗传性胰腺炎的临床和病理表现进行了综述。
