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胰腺内分泌细胞增生(胰岛细胞增殖症)的症状及早期诊断可能性

[Symptoms and early diagnostic possibilities of pancreatic endocrine cells hyperplasia (nesidioblastosis)].

作者信息

Przybylik-Mazurek Elwira, Pach Dorota, Hubalewska-Dydejczyk Alicja, Sowa-Staszczak Anna, Gilis-Januszewska Aleksandra, Kulig Jan, Matyja Andrzej, Chrapczyński Paweł

机构信息

Katedra i Klinika Endokrynologii UJ CM w Krakowie.

出版信息

Przegl Lek. 2012;69(1):9-14.

Abstract

Nesidioplastosis in adults is one of a rare causes of hyperinsulinemic hypoglycemia. Symptoms include chronic or recurrent hypoglycemias, often with neurological signs. Due to the looses of consciousness with coexisting seizures, in many cases patients are treated on epilepsy. Right diagnosis is usually late established, when the damages in the central nervous system (CNS) are irreversible. Early diagnosis of the disease and appropriate treatment might help to avoid serious disability in these patients. The aim of the study was to asses modern diagnostics of the nesidioblastosis with an emphasis on the biochemical and hormonal tests and imaging modalities. Patients enrolled to the study were aged between 18 and 72 years of age, and had chronic or recurrent hypoglycemia caused by hyperinsulinemia. In all patients fasting glucose and fasting insulinemia tests were performed, as well as the fasting blood test or in the 24-hour profile tests. Several techniques were used including ultrasound (US), abdominal computer tomography (CT), in two patients magnetic resonance imaging, scintigraphy of somatostatin receptors in seven patients, and in two patients scintigraphy with glucagone-like peptide-1 (GLP-1) analogue-labeled marker was done. In the performed tests low values of the blood glucose were found, whereas insulin levels, however not adequate to the blood glucose, were nearly always within the normal range. In the standard imaging only in one patient tumor lesion in the pancreatic tail was revealed, though not confirmed in the intraoperative histology. In the scintigraphy examination with the somatostatin analogue in one patient slightly increased collection of the marker in whole pancreas was reported and in the other patient focal collection in the pancreatic tail was observed. Scintigraphy with GLP-1 analogue revealed focal collection of the marker in one case. Five patients were underwent surgical treatment. In the histopathology in all operated patients hyperplasia of the endocrine pancreatic cells with positive immuno. histochemic reaction on the insulin was found. In the three cases despite hyperplasia of pancreatic islets, small sizes insulinomas were detected as well. 1. The diagnosis of nesidioblastosis should be taken into consideration in all patients with unclear-cause hypoglycemias, in whom simultaneously insulin blood level is inadequate to the level of glucose. 2. Widely available imaging examinations: US, CT, MRI are useless in the diagnosis of nesidioblastosis. 3. Among the imaging methods in preoperative diagnostics of hypoglycemia with concomitant hyperinsulinemia somatostatin receptor scintigraphy seems to have specific, though limited role - it is valuable only in the severe, diffused lesions. 4. Recurrent hypoglycemias after 70% excision of the pancreas may indicate the possibility of coexistence of pancreatic islets hyperplasia and insulin secreting insulinoma.

摘要

成人胰岛细胞增生症是高胰岛素血症性低血糖症的罕见病因之一。症状包括慢性或复发性低血糖症,常伴有神经学体征。由于意识丧失并伴有癫痫发作,在许多情况下患者按癫痫进行治疗。通常在中枢神经系统(CNS)出现不可逆转的损害时才迟迟做出正确诊断。该疾病的早期诊断和适当治疗可能有助于避免这些患者出现严重残疾。本研究的目的是评估胰岛细胞增生症的现代诊断方法,重点是生化和激素检测以及成像方式。纳入该研究的患者年龄在18至72岁之间,患有由高胰岛素血症引起的慢性或复发性低血糖症。对所有患者进行了空腹血糖和空腹胰岛素血症检测,以及空腹血液检测或24小时血糖监测。使用了多种技术,包括超声(US)、腹部计算机断层扫描(CT),两名患者进行了磁共振成像,七名患者进行了生长抑素受体闪烁扫描,两名患者进行了胰高血糖素样肽-1(GLP-1)类似物标记物闪烁扫描。在进行的检测中发现血糖值较低,而胰岛素水平虽然与血糖水平不匹配,但几乎总是在正常范围内。在标准成像中,仅一名患者的胰尾发现了肿瘤病变,但术中组织学未证实。在使用生长抑素类似物的闪烁扫描检查中,一名患者报告整个胰腺的标记物摄取略有增加,另一名患者观察到胰尾有局灶性摄取。使用GLP-1类似物的闪烁扫描在一例中显示标记物有局灶性摄取。五名患者接受了手术治疗。在组织病理学检查中,所有接受手术的患者均发现内分泌胰腺细胞增生,胰岛素免疫组化反应呈阳性。在三例中,尽管胰岛增生,但也检测到了小的胰岛素瘤。1. 对于所有病因不明的低血糖症患者,若同时胰岛素血水平与血糖水平不匹配,应考虑胰岛细胞增生症的诊断。2. 广泛应用的成像检查:US、CT和MRI对胰岛细胞增生症的诊断无用。3. 在伴有高胰岛素血症的低血糖症术前诊断的成像方法中,生长抑素受体闪烁扫描似乎具有特定但有限的作用——仅在严重的弥漫性病变中才有价值。4. 胰腺切除70%后复发的低血糖症可能表明存在胰岛增生和胰岛素分泌性胰岛素瘤并存的可能性。

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