Feigelson J, Pécau Y, Poquet M, Terdjman P, Carrère J, Chazalette J P, Ferec C
Radiologie, Paris, France.
J Pediatr Gastroenterol Nutr. 2000 Feb;30(2):145-51. doi: 10.1097/00005176-200002000-00010.
Pathologic changes of the pancreas have been observed as early as the recognition of the disease termed initially "cystic fibrosis of the pancreas". Atrophy of the gland and its fatty infiltration were considered as usual features. The aim of this study was to follow-up the evolution of cystic fibrosis pancreas and to define its successive stages in correlation with the clinical, biochemical, and imaging findings.
Fifty-five patients were followed up during 9 years. The patients' genetic backgrounds were systematically performed. Blood lipase levels were analyzed systematically at each consultation of the patients and in the event of bouts of abdominal pains. Imaging using mainly echograms and tomodensitometric scans were regularly performed: echograms every 6 months, and tomodensitometric scans every 1 to 2 years. Magnetic resonance imaging was performed in four patients.
Five groups of patients were identified on the basis of tomodensitometric scan findings: normal pancreas (n = 4), incomplete lipomatosis of the pancreas (n = 9), complete lipomatosis of the pancreas (n = 23), cystic pancreas (n = 5), macrocystic pancreas (n = 1), atrophic pancreas (n = 13). Pancreas exocrine function was not correlated with findings. Forty episodes of pancreatitis were observed in seven patients. They had bouts of abdominal pain and elevation of lipase levels. Five of these patients were composite heterozygotes (D508/other). Incomplete lipomatosis represents an intermediate stage leading toward complete lipomatosis or toward atrophy after pancreatitis.
Studies of pancreatic function should be performed routinely in cystic fibrosis, especially in pancreatic sufficiency or in patients with normal pancreas images. Acute pancreatitis should be diagnosed and properly identified to be differentiated from other acute abdominal syndromes occurring in cystic fibrosis.
早在最初被称为“胰腺囊性纤维化”的疾病被认识时，就已观察到胰腺的病理变化。腺体萎缩及其脂肪浸润被视为常见特征。本研究的目的是随访胰腺囊性纤维化的演变过程，并根据临床、生化和影像学表现确定其连续阶段。
55例患者接受了9年的随访。系统分析了患者的基因背景。在每次患者就诊时以及出现腹痛发作时，系统分析血脂肪酶水平。主要定期进行超声检查和计算机断层扫描成像：每6个月进行一次超声检查，每1至2年进行一次计算机断层扫描。对4例患者进行了磁共振成像检查。
根据计算机断层扫描结果确定了五组患者：正常胰腺（n = 4）、胰腺不完全脂肪化生（n = 9）、胰腺完全脂肪化生（n = 23）、囊性胰腺（n = 5）、大囊状胰腺（n = 1）、萎缩性胰腺（n = 13）。胰腺外分泌功能与检查结果无关。在7例患者中观察到40次胰腺炎发作。他们有腹痛发作和脂肪酶水平升高。其中5例患者为复合杂合子（D508/其他）。不完全脂肪化生是一个中间阶段，可导致完全脂肪化生或胰腺炎后萎缩。
在囊性纤维化患者中应常规进行胰腺功能研究，尤其是在胰腺功能正常或胰腺影像正常的患者中。应诊断并正确识别急性胰腺炎，以与囊性纤维化中发生的其他急性腹部综合征相鉴别。
