發(fā)布時(shí)間：2018-03-10 14:56

  本文選題：糖原累積病　切入點(diǎn)：肝臟病理　出處：《廣西醫(yī)科大學(xué)》2014年碩士論文　論文類型：學(xué)位論文

【摘要】：目的：總結(jié)糖原累積病患者的臨床表現(xiàn)、實(shí)驗(yàn)室檢查結(jié)果以及肝臟病理特點(diǎn)，加深對糖原累積病的認(rèn)識。 方法：回顧性分析廣西醫(yī)科大學(xué)第一附屬醫(yī)院2003年1月至2011年12月期間收治住院的49例肝糖原累積病患者的臨床資料，總結(jié)其臨床表現(xiàn)、實(shí)驗(yàn)室檢查結(jié)果以及其肝臟病理特點(diǎn)。 結(jié)果:（1）49例患者中男32例，，女17例，最小年齡為3個(gè)月，最大年齡為25歲，8例患者首次確診時(shí)年齡大于5歲。（2）最常見的臨床表現(xiàn)是腹部膨隆和肝臟腫大（100%），其次是生長遲緩（70.5%）和脾腫大（42.8％）。（3）實(shí)驗(yàn)室檢查陽性發(fā)現(xiàn)最常見的為低血糖、AST和ALT升高，其次為GGT升高、輕度貧血、高膽固醇血癥、高甘油三酯血癥、高尿酸血癥。（4）42例患者超聲檢查未發(fā)現(xiàn)肝腺瘤，所有患者心電圖檢查均正常。（5）28例行肝組織病理檢查，14例（28.6％）只有肝細(xì)胞內(nèi)糖原腫脹，11例（22.4％）有肝細(xì)胞內(nèi)糖原腫脹和門脈周圍纖維化；3例（6.1％）具有肝細(xì)胞糖原累積和肝細(xì)胞脂肪變性；28例患者中都均無支鏈淀粉沉淀。結(jié)論：（1）肝糖原累積病發(fā)病率低，當(dāng)發(fā)現(xiàn)肝腫大伴低血糖的患者應(yīng)注意鑒別該病。大部分的肝糖原累積病可在＜5歲兒童中發(fā)現(xiàn)。（2）腹部膨隆、肝腫大、脾腫大是最主要的臨床表現(xiàn)。（3）大多數(shù)患者ALT、AST,、GGT升高，其他實(shí)驗(yàn)室檢查可表現(xiàn)有低血糖、高尿酸血癥、高乳酸血癥和貧血。（4）肝組織病理檢查發(fā)現(xiàn)肝細(xì)胞糖原累積、門靜脈周圍纖維化和肝細(xì)胞脂肪變性。
[Abstract]:Objective: to summarize the clinical manifestations, laboratory findings and liver pathological features of patients with glycogen accumulative disease. Methods: the clinical data of 49 patients with liver glycogen accumulation disease admitted from January 2003 to December 2011 in the first affiliated Hospital of Guangxi Medical University were analyzed retrospectively. The results of laboratory examination and the pathological features of the liver. Results among 49 patients, 32 were male and 17 female, with a minimum age of 3 months. The most common clinical manifestations of 8 patients aged 25 years or older at the time of first diagnosis are abdominal distension and hepatomegaly 100%, followed by 70.5% of growth retardation) and 42.8% of splenomegaly.) the most common positive laboratory findings are low. The levels of AST and ALT were increased. The second was elevated GGT, mild anemia, hypercholesterolemia, hypertriglyceridemia and hyperuricemia. No hepatic adenoma was found in 42 patients with hyperuricemia. All patients had normal electrocardiogram and 28 cases had liver histopathologic examination. Only hepatocyte glycogen swelling was found in 11 cases (22.4%) there were hepatocyte glycogen swelling and portal fibrosis in 3 cases (6. 1) there was hepatocyte glycogen accumulation and liver fine. There was no amylopectin precipitate in 28 patients with cellular fatty degeneration. Conclusion the incidence of liver glycogen accumulation disease is low. Patients with hepatomegaly and hypoglycemia should pay attention to distinguishing the disease. Most of the liver glycogen accumulative diseases can be found in children under 5 years old.) abdominal swelling, hepatomegaly and splenomegaly are the most important clinical manifestations. Other laboratory tests showed hypoglycemia, hyperuricemia, hyperlactic acidemia and anemia. 4) Hepatocyte glycogen accumulation, periportal fibrosis and hepatocyte steatosis were found by pathological examination of liver tissue.
【學(xué)位授予單位】：廣西醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R575

【共引文獻(xiàn)】

相關(guān)博士學(xué)位論文 前1條

1 盧超霞;糖原累積癥的致病基因突變研究先天性皮膚松弛癥的致病基因突變研究[D];北京協(xié)和醫(yī)學(xué)院;2009年

相關(guān)碩士學(xué)位論文 前4條

1 張曉紅;解酮障礙SCOT基因突變與臨床研究[D];廣州醫(yī)學(xué)院;2010年

2 王薇;肌肉中的糖原脫支酶活力測定及糖原定量、定性以診斷Ⅲa型糖原貯積癥[D];中國協(xié)和醫(yī)科大學(xué);2008年

3 劉璐;兒童肝糖原累積病12例臨床研究[D];中國醫(yī)科大學(xué);2010年

4 賀宏梅;肌糖原累積病的臨床及病理學(xué)研究[D];河北醫(yī)科大學(xué);2014年

本文編號：1593832