肝病合并溶血性疾病的漏诊分析——附3例报道

临床诊治思维倾向于尽量用一元论来解释整个病情,但是有时一个患者同时患有几种有相同症状的疾病时,如果用一元论解释,会将复杂的疾病简单化,从而影响治疗甚至预后。肝病本身即可引起溶血,如同时患有肝病和溶血性疾病,因两者症状重叠(如都可出现黄疸、肝脾肿大、贫血等),容易漏     

以肝功能不良就诊的地中海贫血患者83例临床分析

目的:探讨地中海贫血合并肝功能不良患者的临床特征。方法:以2012年1月至2021年7月华中科技大学同济医学院附属同济医院收治的83例以肝功能不良就诊的地中海贫血病例为研究对象,分析其一般情况、地中海贫血基因型、血常规及肝功能、影像学等临床资料。结果:地中海贫血合并肝功能不良患者表现为谷丙转氨酶和谷草转氨酶升高、小细胞低色素性贫血,其总胆红素异常程度不一,且与基因型无关(P>0.05)。但黄疸严重的患者其红细胞及血红蛋白明显减少(P<0.05),且常易合并其他肝胆疾病,如病毒性肝炎、胆系感染等。结论:湖北地区地中海贫血基因携带也较为多见,临床医生在诊治疑难性肝病时应考虑到地中海贫血可能。     

Analysis of 259 cases of jaundice in youth

目的 探讨18～44岁青年黄疸患者的临床表现与病因的构成.方法 收集我院2009年1月～2011年11月以黄疸为首发表现入院的青年患者的临床资料,统计患者病因构成与临床表现、性别、严重程度的关系,并与中老年人群中的黄疸病因构成进行比较.结果 可引起青年黄疸的疾病较多,其中最常见的病因依次为病毒性肝炎、胆石病、药物性肝病、自身免疫性肝病等.病毒性肝炎、胆石病、败血症、肝衰竭等疾病伴有的临床症状和体征较多,重度黄疸主要见于肝炎及肿瘤患者.结论 青年黄疸的病因以常见病、多发病为主,黄疸的病因构成有其自身的特点.     

Coomb用于区别黄疸型肝炎和免疫性溶血性贫血1例

自身免疫性溶血性贫血是一种获得性溶血性疾病,患者由于免疫功能紊乱产生抗自身红细胞的抗体.与红细胞表面的抗原结合.或激活补体使红细胞加速破坏而致溶血性贫血。患者具有：贫血、黄疸、乏力、肝脾肿大、肝功能检查异常等表现,     

溶血性黄疸患者实验室检测结果分析

目的：从实验室的角度分析溶血性黄疸的特点,为临床鉴别诊断溶血性黄疸提供实验室依据。方法选取2011年6月－2013年12月在广州医科大学附属沙井医院就诊的44例患者,其中包括遗传性溶血性疾病和溶血性疾病合并肝炎病毒感染的患者,对实验室检查结果包括血液学检查,生化指标和葡萄糖－6－磷酸脱氢酶（G －6－PD）活性等进行分析。结果G －6－PD 缺乏症所致的溶血性黄疸,患者不一定有贫血表现。地中海贫血所致的溶血性黄疸,绝大多数患者红细胞呈小细胞低色素,平均红细胞体积（MCV）和平均红细胞血红蛋白含量（MCH）低于正常,α－地中海贫血血红蛋白（Hb）A2偏低,β－地中海贫血 Hb A2增高。溶血性黄疸网织红细胞可增高也可在正常范围内。尿液检查胆红素呈阴性,尿胆原含量正常或增高。生化检查非结合胆红素轻中度增高,结合胆红素正常,间接胆红素的量占到总胆红素量的70％～80％。ALT、AST、ALP 基本正常。溶血性黄疸患者合并肝炎病毒感染时,ALT、AST、ALP、GGT、总胆红素、直接胆红素、间接胆红素可显著升高。结论对原因不明或者不能用肝炎解释的黄疸以及轻中度黄疸,间接胆红素占到总胆红素的70％～80％的患者,都应该警惕溶血性疾病的可能,建议作 G －6－PD 酶活性测定和血红蛋白电泳筛查。     

熊去氧胆酸联合ERCP治疗梗阻性黄疸的疗效观察

随着经内镜逆行胰胆管造影(ERCP)及相关技术的广泛应用,梗阻性黄疸患者均可通过ERCP以及鼻胆管引流术、胆管支架植入术或胆总管结石取石术来减轻黄疸,是目前此类患者的首选治疗手段[1]。熊去氧胆酸广泛应用于临床,对原发性胆汁性肝硬化、胆汁淤积性肝病等肝胆疾病疗效确切[2],本院应用熊去氧胆酸联合ERCP治疗梗阻性黄疸,取得了较好的临床疗效。     

溶血性黄疸的临床特征及鉴别诊断

溶血性黄疸病因繁多,临床表现复杂多样。部分患者虽然存在溶血,但贫血不明显或因贫血程度轻微而以黄疸为首发表现,长期反复,难以治愈,可误诊为原因不明的慢性肝炎、黄疸原因待查等,尤其容易与Gilbert综合征等先天性非结合胆红素血症混淆,出现漏诊和延误诊治。作为肝病科医师,应了解并掌握溶血性黄疸以及慢性溶血性疾病的相关知识。总结了溶血性黄疸的临床特征及鉴别诊断思路,并简述了常见慢性溶血性疾病的临床特点,以供临床医师参考。     

肝病患者肝肾阴虚证型临床相关特征的观察与研究——2001年湖北省中医院肝病科出院患者病案资料分析

目的：探讨肝病患者肝肾阴虚证的分布规律。方法：采用临床流行病学调查方法，以中医病历为信息来源，以国家标准《中医病证分类与代码》为规范，利用现代计算机技术，编制相关分析软件，分析2001年湖北省中医院肝病科出院的肝病患者肝肾阴虚证分布规律。结果：肝肾阴虚证多见于慢性肝病病程较长者，中老年男性患者出现频率较高。肝肾阴虚证多见于中医的积聚、臌胀、胁痛、黄疸和西医病的肝硬化、原发性肝癌、病毒性肝炎等疾病中。结论：肝肾阴虚证是肝病科慢性肝病患者常见证候之一，提示慢性肝病患者病情进入中、晚期须注重肝肾阴虚证的辨证论治。     

复方降压药所致药物性肝病2例

目的:分析2例口服复方降压片和北京降压O号所致的药物性肝病的临床资料及特点,以加深临床医师对药物性肝病的认识.方法:根据患者服药史,临床表现,血象,肝炎病毒标记物,肝功能试验,影像学检查及药物性肝病的诊断标准,加以临床分析,讨论.结果:降压药所致药物性肝病的临床表现主要为黄疸.实验室检查丙氨酸转氨酶,γ-谷氨酰转酞酶,碱性磷酸酶均升高,血总胆红素定量明显升高,尤以直接胆红素升高为著.按Zakim临床分型属胆汁淤积型.结论:对黄疸患者应注意排除药物性肝病的可能,早期诊断、及时治疗.     

肝细胞性黄疸的诊断与治疗

正黄疸是临床上常见的一类症状和体征,表现为患者皮肤黏膜和巩膜黄染,通常因肝脏损伤引起,根据病因不同可将其分为溶血性、肝细胞性、梗阻性和先天性非溶血性黄疸,其中肝细胞性黄疸最常见。肝细胞性黄疸是因肝细胞病变造成其摄取、转化和排泄胆红素的能力降低所致。凡能造成肝细胞功能障碍,影响胆红素摄取、结合和排泌的疾病均可引起肝细胞性黄疸,常见于各种肝实质性疾病,如病毒性肝炎、自身免疫性肝病、药物性肝损伤、中     

Cirrhosis as a consequence of graft-versus-host disease

A 28-yr-old woman with severe idiopathic aplastic anemia received an HLA-identical mixed lymphocyte culture nonreactive bone marrow transplant from her brother. In the months after successful engraftment, she developed cutaneous and hepatic graft-versus-host disease, associated with marked cholestatic jaundice. Despite a series of therapeutic maneuvers, cholestasis persisted but remained relatively stable over the ensuing 10 yr. However, serial liver biopsies revealed progressive biliary-type fibrosis culminating in cirrhosis. Subsequently, her clinical course deteriorated and she developed signs of hepatic failure, and ultimately died 10.5 yr after bone marrow transplantation. The evolution of chronic graft-versus-host disease to cirrhosis may be a limiting factor in the long-term survival of this group of bone marrow transplant recipients. The lack of correlation between the stable clinical or biochemical indices and the progressive hepatic disease underscores the need for sequential liver biopsies in patients with sustained liver function abnormalities after bone marrow transplantation.     

Cholelithiasis in sickle cell anemia in a Caucasian population

Sixty-two Saudi Arab oasis dwellers with sickle cell anemia, aged 10 to 64 years, were evaluated for cholelithiasis. Five of them had multiple gallstones, the youngest of whom was 17 years old. Only one of these had symptoms and findings strongly suggestive of cholelithiasis and obstructive jaundice, and his serum bilirubin level was higher than is usually found in patients with the hepatic crises of sickle cell anemia. The remaining four had less severe abdominal pain and jaundice but still presented potential problems in differential diagnosis. The evaluation led to the finding of gallstones in three patients. The incidence of cholelithiasis in Saudi Arabs was found to be one-sixth to one-fourth as great as that in American Negroes. Fewer patients had impaired liver function, and over-all clinical findings were less severe. Anemia was milder and reticulocytosis was less striking. The lower incidence of cholelithiasis and the milder clinical manifestations in these patients were related to an unusually high level of alkali-resistant hemoglobin which averaged more than 20 per cent. Recommendations regarding appropriate routine evaluation of patients with sickle cell anemia by cholecystography may need to be altered for populations such as these Saudi Arabs in whom the disease is of a relatively mild type.     

Exacerbation of Sickle Cell Disease Itself as a Cause of Abnormal Liver Chemistry Tests

Liver pathology occurring in patients with sickle cell disease is commonly related to viral hepatitis or hepatic iron deposition due to repeated transfusions; cholestasis and cirrhosis may also occur. Consequently, the differential diagnosis of abnormal liver tests in patients with sickle cell anemia is often complicated. We report the case of a patient presenting with jaundice and abnormal liver biochemistries, without typical evidence of the liver diseases associated with sickle cell anemia. Biochemical markers for viral hepatitis were negative. CT scan only showed hepatomegaly. The liver biopsy revealed marked sinusoidal congestion with red blood cells without significant steatosis or increased iron deposition. The patient's medical history corroborated with biochemical tests and histological examination of the liver suggested that worsening hemolysis related to increased sickling of erythrocytes intrahepatically led to sinusoidal dilatation and probably caused the abnormal liver tests.     

Rapid progression of autoimmune hepatitis in the background of primary sclerosing cholangitis

"Overlap syndromes" have been reported among various autoimmune liver diseases, particularly between primary biliary cirrhosis and autoimmune hepatitis (AIH) in adults and between AIH and autoimmune cholangitis in children. The overlap syndrome of AIH and primary sclerosing cholangitis (PSC), however, has been scarcely reported. Furthermore, in most of the reported cases of AIH/PSC overlap syndrome, PSC and AIH were believed to occur simultaneously. We report a case of a 34-year-old woman who has ulcerative colitis and PSC (diagnosed by colonoscopy, histology, and cholangiogram) and 7 years later develops rapidly progressive liver failure and hemolytic anemia from AIH. Liver biopsy showed dense portal lymphoplasmacytic infiltrate with interface hepatitis and acidophil bodies confirming AIH. She responded well to immunosuppressive therapy with steroids, both with respect to her liver disease and her autoimmune hemolytic anemia. Additionally, her clinical symptoms of fatigue, jaundice, and pruritus improved markedly and quickly. Overlap or "crossover" syndrome should be considered in all patients with PSC when they present with sudden deterioration of the liver function and changes in liver enzymes. By making the diagnosis of AIH in a patient with well-established PSC, appropriate treatment can be initiated, resulting in the patient's prompt recovery.     

以黄疸为主要表现的轻型β-地中海贫血1例附肝组织活检病理

41岁女性,临床特点为间断黄疸、脾大、贫血,家族成员有发作性黄疸病史,经血红蛋白电泳等检查确诊为轻型β-地中海贫血。肝脏活检病理特点：肝细胞胞浆内可见色素颗粒沉积,肝窦内有巨核细胞,肝内轻度纤维化,有少量炎性细胞浸润。     

Idiopathic adulthood ductopenia: case report and review of the literature

The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. Serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. Patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.     

李秀惠教授运用截断逆挽法治疗慢性肝衰竭验案

肝衰竭是临床常见的严重肝病症候群,属于中医“急黄”“瘟黄”等范畴,病死率极高。李秀惠教授认为慢性肝衰竭的主要病机以气血不足为本,瘀、痰(湿)热毒为邪的本虚标实证,以“截断逆挽法”为主要治则,同时注重“病-证-症”紧密结合,在改善患者临床症状的同时又直达病机,在临床上取得了很好的疗效。     

Bridging use of plasma exchange and continuous hemodiafiltration before living donor liver transplantation in fulminant Wilson's disease

A 15-year-old girl presented with acute hepatic failure showing ascites and hepatic encephalopathy, accompanied by hemolytic anemia. She was diagnosed as having fulminant Wilson's disease (FWD). Plasma exchange (PE), continuous hemodiafiltration (CHDF) and D-penicillamine administration were started immediately. Copper [24,000 microg] was removed by PE and CHDF over three days, which relieved the jaundice and the consciousness disorder. A successful liver transplant followed. FWD progresses rapidly and often liver transplantation is the only possible therapy. In this case, PE and CHDF were an effective therapy bridge until liver transplantation.     

α1-抗胰蛋白酶缺乏症1例并文献复习

报道1例以黄疸为表现的α1-抗胰蛋白酶缺乏症病例,该例患者初始起病以黄疸为表现,病程10年,通过肝活检组织病理确诊。结合文献对α1-抗胰蛋白酶缺乏症的流行病学现状、病因机制、临床表现及治疗预后加以复习,藉此对临床遗传性肝病的诊治提供参考。     

Spur cell anemia associated with a cirrhotic non-alcoholic steatohepatitis patient

Spur cell anemia is an acquired hemolytic anemia, which may occur in patients with severe liver dysfunction, especially in those with alcoholic cirrhosis. Recently, non-alcoholic steatohepatitis (NASH) has been highlighted as one of the causes of chronic liver diseases, which could progress to cirrhosis. Histological features of NASH are indistinguishable from those of alcoholic hepatitis. We report on a cirrhotic NASH patient who developed progressive indirect dominant jaundice and intractable hemolytic anemia during his follow-up. This report shows the first case of a cirrhotic NASH patient who died as a result of spur cell anemia.