Friesen S R, Kimmel J R, Tomita T
Am J Surg. 1980 Jan;139(1):61-72. doi: 10.1016/0002-9610(80)90231-7.
Prospective screening was carried out in 12 members of three families with multiple endocrine adenopathies, type I (MEA,I) and in 14 patients with no multiple endocrine adenopathies with and without other endorcinopathies. Elevated basal and responsive (after a meal) plasma concentrations of a relatively new candidate-hormone, human pancreatic polypeptide (hPP), were associated with pancreatic apudoma tumors in three asymptomatic patients with multiple endocrine adenopathies, type I. Two of these patients had excision of the tumors that resulted in normal plasma hPP concentrations postoperatively. Both tumors contained hPP predominantly by immunocytochemistry; one, a pure pancreatic polypeptide apudoma, was studied extensively demonstrating also by radioimmunoassay a high content of hPP and negligible amounts of insulin, glucagon, somatostatin, vasoactive intestinal polypeptide and gastrin. In this patient plasma concentrations of other polypeptides including insulin, glucagon, somatostatin, vasoactive intestinal polypeptide, gastrin, parathyrin, thyrocalcitonin, prolactin, corticotropin, growth hormone, thyrtropin and amine, serotonin, were within normal limits. The other patient, after excision of an hPP-detected pancreatic mixed hPP-gastrinoma, also became eugastrinemic postoperatively. Normal basal plasma hPP concentrations, but with exaggerated hPP responses to a meal in 11 patients, were associated with various combinations of islet cell hyperplasia, antral G cell hyperplasia with moderate hypergastrinemia and parathyroid hyperplasia. The patients with multiple endocrine adenopathies who have demonstrated this type of increased hPP response to a meal have not been operated on but are at risk for islet hyperplasia. Four of the 12 patients with multiple endocrine adenopathies, type I, with both normal basal and normally responsive hPP concentrations have no evidence as yet of pancreatic involvement.
对三个患有多发性内分泌腺瘤病I型（MEA, I）的家族中的12名成员以及14名无多发性内分泌腺瘤病、有或无其他内分泌病的患者进行了前瞻性筛查。一种相对较新的候选激素——人胰多肽（hPP）的基础血浆浓度和反应性（餐后）血浆浓度升高，与三名无症状的I型多发性内分泌腺瘤病患者的胰腺APUD瘤有关。其中两名患者切除了肿瘤，术后血浆hPP浓度恢复正常。通过免疫细胞化学检测，这两个肿瘤主要含有hPP；其中一个是纯胰多肽APUD瘤，通过放射免疫测定法广泛研究表明，其hPP含量高，而胰岛素、胰高血糖素、生长抑素、血管活性肠肽和胃泌素的含量可忽略不计。在该患者中，包括胰岛素、胰高血糖素、生长抑素、血管活性肠肽、胃泌素、甲状旁腺素、甲状腺降钙素、催乳素、促肾上腺皮质激素、生长激素、促甲状腺激素和胺、血清素在内的其他多肽的血浆浓度均在正常范围内。另一名患者在切除通过hPP检测到的胰腺混合性hPP - 胃泌素瘤后，术后也恢复了正常胃泌素水平。11名患者的基础血浆hPP浓度正常，但餐后hPP反应过度，这与胰岛细胞增生、伴有中度高胃泌素血症的胃窦G细胞增生和甲状旁腺增生的各种组合有关。表现出这种餐后hPP反应增强的多发性内分泌腺瘤病患者尚未接受手术，但有胰岛增生的风险。12名I型多发性内分泌腺瘤病患者中有4名基础hPP浓度和反应性均正常，目前尚无胰腺受累的证据。
