中西医结合治疗家族性高黄疸两例报告

黄疸是指血清胆红素增高致使巩膜、皮肤、黏膜以及其他组织和体液发生黄染的现象.黄疸的传统分类方法将黄疸分为溶血性、肝细胞性和梗阻性三大类.近年倾向于根据增高的胆红素性质进行分类,即先确定患者的胆红素是非结合性胆红素增高为主,还是以结合性胆红素增高为主,然后再根据病史、体征并实验室及影像学特征进行鉴别,最后确立病因诊断.非结合胆红素血证的病因主要分为溶血性贫血、UGT基因变异、其他原因三类.结合性胆红素血证的发病主要有肝细胞性黄疸、胆汁淤积性黄疸、遗传性高结合胆红素血证三类,它们各自又有不同的病因[1].     

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     

肝内胆汁淤积症的诊断与治疗

肝内胆汁淤积症是由多种原因引起肝细胞和（或）毛细胆管胆汁分泌障碍,导致部分或完全性胆汁流阻滞为特征的综合征。应注意与肝外阻塞性黄疸类相鉴别,二者均表现为重度黄疸、皮肤瘙痒、大便颜色变浅、血胆红素升高,以直接胆红素升高为主。所以明确肝内胆汁淤积症机制、诊断及治疗甚为重要。     

胆汁淤积性黄疸的诊断和治疗

胆汁淤积性黄疸的发病机理,上世纪90年代研究已进入分子水平。目前尚不十分清楚。大家认为,肝脏对胆红质的摄取、运载及脂化能力均正常,主要是由致病因子引起的肝细胞的细胞器和毛细胆管的损害,造成胆汁排泄障碍,或毛细胆管内胆栓形成,脂型胆红素逆向进入血液而发生黄疸;不同病因引起胆汁淤积的过程和预后亦不同。一般产生胆汁淤积的病因有感染(包括病毒、细菌、毒素和寄生虫);代谢(如药物、酒精性肝病,囊性纤维化,妊娠,脂肪肝);免疫(如原发性胆汁性胆管炎,原发性胆汁性肝硬化);其他(如肝硬化、肝癌)等。由于各种病因损害肝细胞内细胞器,使…     

肝活检确诊以溶血性贫血为首发症状的肝豆状核变性1例

患者，男，14岁。因“反复右上腹痛、乏力、巩膜黄染1个月余”，于2003年6月12日第1次入院。入院体检：发育稍差，智力正常。神志清楚，呼吸平稳。轻度贫血貌，全身皮肤轻度黄染，未见肝掌、蜘蛛痣。浅表淋巴结无肿大。结膜略苍白，巩膜轻度黄染。心肺未闻及异常。腹平软，肝、脾肋下未触及肿大，肝区轻叩痛。双下肢无水肿。家族成员中     

超声诊断肝外梗阻性黄疸36例分析

现将我院经超声显像检查,并经手术、病理等证实的36例肝外梗阻性黄疸报告如下。一、资料与方法36例均为1983年8月～1987年12月我院门诊及住院病例。其中男20例,女16例,年     

中西医结合治疗新生儿母乳性黄疸50例

我们自2000年10月以来,采用自制茵栀黄口服治疗母乳性黄疸50例,疗效满意,现报告如下。1 资料与方法1.1 临床资料:患儿均为单纯母乳喂养,皮肤黄染出现时间为出生后3～7d,并逐渐加深持续不退>2周;黄疸以未结合总胆红素(TBil)升高为主;一般情况良好,精神及食欲好,生长发育正常,肝脏无肿大,     

肝性脑病的诊断与治疗

肝性脑病的诊断与治疗泰安市中心医院（２７１０００）肖德明万宝美封扬肝性脑病又称肝昏迷，是严重肝病或门－体分流的严重并发症之一。现结合文献及临床体会，对其发病机理及诊治情况作一介绍。１发病机理肝性脑病的发病机理迄今未完全阐明，目前认为，本病主要由于肠道...     

超声引导肝穿刺活检对不明原因肝性黄疸的诊断价值

目的:探讨超声引导肝穿刺活体组织检查术对不明原因肝细胞性黄疸的诊断意义。方法:2009年6月以来收住的肝细胞性黄疸29例,平均住院1周以上,经常规方法未能明确诊断,实施超声引导肝穿活检术,使用美国Bard自动活检枪,16/18G穿刺针。结果:29例穿刺肝活检均获得组织标本,作出病理诊断,无严重并发症发生。结论:超声引导穿刺活检安全性较高,对不明原因的肝细胞性黄疸临床诊断有重要价值。     

肝细胞型胆汁淤积诊治进展

胆汁淤积是由于肝内外胆管阻塞或肝细胞功能障碍所致的胆汁不能到达十二指肠而在肝细胞和血液积聚的一种病理学状态。肝细胞是胆汁合成和分泌的主要场所。当肝细胞上的胆盐输出泵(bile salt export,BSEP)、ATP结合盒(ATP-binding cassette,ABC)转运蛋白、多耐药相关蛋白2(multidrug resistance-associated protein,MRP2)功能障碍或受抑制,则可导致胆汁酸或其结合胆盐在肝细胞和血清中聚积引起肝细胞型胆汁淤积[1]。引起肝细胞型胆汁淤积的常见病因包括细菌、病毒、真菌和寄生虫等感染、酒精或非酒精性脂肪性肝炎、自身免疫性或药物相关性肝损伤、全胃肠外营养、重症相关(缺血性)肝内胆汁淤积、良性复发性肝内胆汁淤积(benign recurrent intrahepatic cholestasis,BRIC)、进展性家族性肝内胆汁淤积(progressive familial intrahepatic cholestasis,PFIC)、ABCB4基因缺乏、妊娠期肝内胆汁淤积(intrahepatic cholestasis of pregnancy,ICP)、霍奇金病、转移性肿瘤、肉芽肿性肝炎和肉芽肿病、血管性疾病(如布加综合征和肝窦阻塞综合征)和各种原因的肝硬化等[2]。     

肝前脂肪厚度与多囊卵巢综合征不孕症患者胰岛素抵抗的相关性研究

目的：探讨肝前脂肪厚度与多囊卵巢综合征（ PCOS）不孕症患者胰岛素抵抗相关指标的关系,为PCOS不孕症的治疗提供相关依据。方法对91例PCOS不孕症患者行超声检测肝前脂肪厚度,并测量身高、体质量、腰围（ WC）、臀围（ HC）等指标,检测性激素、胰岛素等核医学指标;检测血脂、血糖等生化指标,分析肝前脂肪厚度与上述指标间的关系。结果肝前脂肪厚度与体质量、体重指数（ BMI）、WC、HC等人体测量值呈正相关（P＜0.05）,与身高无明显相关（P＞0.05）;与空腹胰岛素、血糖、尿酸、胰岛素抵抗指数等呈正相关（P＜0.05）,与高密度脂蛋白呈负相关（P＜0.05）;与性激素、甲状腺功能、总胆固醇、甘油三酯、低密度脂蛋白及其他肝肾功能指标无明显相关（ P＞0.05）。结论肝前脂肪厚度在PCOS不孕症患者中与胰岛素抵抗有关的指标有一定的相关关系;在临床诊治该类患者时应全面评估,以提高妊娠率及改善妊娠结局。     

Altered blood-brain barrier permeability in rats with prehepatic portal hypertension turns to normal when portal pressure is lowered

AIM: To study the blood-brain barrier integrity in prehe-patic portal hypertensive rats induced by partial portal vein ligation, at 14 and 40 d after ligation when portal pressure is spontaneously normalized. METHODS: Adult male Wistar rats were divided into four groups: GroupⅠ: Sham14d, sham operated; GroupⅡ: PH14d, portal vein stenosis; (both groups were used 14 days after surgery); GroupⅢ: Sham40d, Sham operated and GroupⅣ: PH40d Portal vein stenosis (GroupsⅡandⅣused 40 d after surgery). Plasma ammonia, plasma and cerebrospinal fluid protein and liver enzymes concentrations were determined. Trypan and Evans blue dyes, systemically injected, were investigated in hippocampus to study blood-brain barrier integrity. Portal pressure was periodically recorded. RESULTS: Forty days after stricture, portal pressure was normalized, plasma ammonia was moderately high, and both dyes were absent in central nervous system parenchyma. All other parameters were reestablished. When portal pressure was normalized and ammonia level was lowered, but not normal, the altered integrity of blood-brain barrier becomes reestablished. CONCLUSION: The impairment of blood-brain barrier and subsequent normalization could be a mechanism involved in hepatic encephalopathy reversibility, Hemo-dynamic changes and ammonia could trigger blood-brain barrier alterations and its reestablishment.     

Altered blood-brain barrier permeability in rats with prehepatic portal hypertension turns to normal when portal pressure is lowered

AIM: To study the blood-brain barrier integrity in prehepatic portal hypertensive rats induced by partial portal vein ligation, at 14 and 40 d after ligation when portal pressure is spontaneously normalized.METHODS: Adult male Wistar rats were divided into four groups: Group I: Sham_14d , sham operated; Group II: PH_14d , portal vein stenosis; (both groups were used 14 days after surgery); Group III: Sham_40d, Sham operated and Group IV: PH_40d Portal vein stenosis (Groups II and IV used 40 d after surgery). Plasma ammonia, plasma and cerebrospinal fluid protein and liver enzymes concentrations were determined. Trypan and Evans blue dyes, systemically injected, were investigated in hippocampus to study blood-brain barrier integrity. Portal pressure was periodically recorded.RESULTS: Forty days after stricture, portal pressure was normalized, plasma ammonia was moderately high, and both dyes were absent in central nervous system parenchyma. All other parameters were reestablished. When portal pressure was normalized and ammonia level was lowered, but not normal, the altered integrity of blood-brain barrier becomes reestablished.CONCLUSION: The impairment of blood-brain barrier and subsequent normalization could be a mechanism involved in hepatic encephalopathy reversibility. Hemodynamic changes and ammonia could trigger blood-brain barrier alterations and its reestablishment.     

Diagnosis and initial management of cholangiocarcinoma with obstructive jaundice

Cholangiocarcinoma is the second most common primary hepatic cancer. Despite advances in diagnostic techniques during the past decade, cholangiocarcinoma is usually encountered at an advanced stage. In this review, we describe the classification, diagnosis, and initial management of cholangiocarcinoma with obstructive jaundice.     

溶血性疾病中的双重杂合因素及溶血系统分析的临床意义 (附506例贫血黄疸病因分析)

目的：分析初诊为贫血或溶血患者的发病原因。方法：常规筛查506例贫血、黄疸溶血、脾大患者，经试验确定存在溶血后，针对性进行血红蛋白病、红细胞膜病、红细胞酶病和后天获得性溶血指标系统分析及家系调查。结果：506例患者中，确诊病因的溶血性疾病384例，病因不明的溶血性贫血21例，非溶血性血液病33例，非血液疾病24例，失访44例。在病因明确的384例溶血性疾病中，遗传性溶血病因356例，其他溶血病因28例。遗传性溶贫中血红蛋白病114例、红细胞膜病133例、红细胞酶病65例、双重杂合子44例。将双重杂合子中的各个病因出现次数与前3大类病因组合并，则先天溶贫病因百分比分别为血红蛋白病34．62％、红细胞膜病42．56％、红细胞酶病22．82％。红细胞缺陷酶的比例为葡萄糖-6-磷酸脱氢酶41．57％、丙酮酸激酶44．94％、嘧啶-5’核苷酸酶3．37％、磷酸果糖激酶3．37％、NADH-高铁血红蛋白还原酶4．49％、醛缩酶1．12％、谷胱甘肽还原酶1．12％。结论：双重杂合子红细胞存在两种遗传缺陷，易互相干扰诊断提示。溶血系统分析和家系调查可以提高溶血性贫血病因确诊率，尤其对红细胞酶病和双重杂合子具有鉴别诊断意义。     

Preoperative biliary drainage (stenting) for treatment of obstructive jaundice

The role of preoperative biliary drainage in malignant obstructive jaundice has been controversial. Laboratory studies suggest that relief of jaundice prior to major pancreatic resection would be associated with improved morbidity and mortality. However, clinical experience has not supported the laboratory results. Obstructive jaundice can be relieved preoperatively via an endoprosthesis introduced either percutaneously or endoscopically. Cohort studies have not shown any clinical benefit and in some the endoprostheses have been implicated in postoperative complications. The only randomized study has shown no benefit in preoperative drainage, but one recent study has confirmed that endoscopic drainage, whilst not conferring an advantage, did no harm. Hence, whilst preoperative drainage is not recommended, if for any reason operation needs to be delayed, endoscopic drainage via an endoprosthesis can be used without fear of adversely influencing the outcome.     

覆膜支架治疗梗阻性黄疸的价值分析

[目的]探讨覆膜支架在治疗梗阻性黄疸患者中的临床应用价值。[方法]选择梗阻性黄疸行胆道覆膜支架置入患者30例,随访1年,对其术后产生的并发症进行分析。[结果]30例手术成功率、有效率100%,胆道感染发生率10.0%,胆道出血发生率6.7%,胆囊炎发生率3.3%,胰腺炎发生率6.7%,支架滑脱、移位发生率10.0%,支架阻塞发生率6.7%。[结论]覆膜支架可以有效防止胆道再狭窄或闭塞;其滑脱、移位发生率较高;不易跨越胆囊管开口放置,可以跨越壶腹放置。     

Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia

Exact diagnosis of the subtype has essential therapeutic consequences in autoimmune hemolytic anemia. Cold-antibody types include primary chronic cold agglutinin disease (CAD) and rare cases of cold agglutinin syndrome (CAS) secondary to cancer or acute infection. Primary CAD is a clonal lymphoproliferative disorder. Not all patients require pharmacological therapy, but treatment seems indicated more often than previously thought. Corticosteroids should not be used to treat primary CAD. Half of the patients respond to rituximab monotherapy; median response duration is 11 months. The most efficient treatment to date is fludarabine and rituximab in combination, resulting in responses in 75%, complete responses in 20% and median response duration of more than 66 months. Toxicity may be a concern, and an individualized approach is discussed. Erythrocyte transfusions can be given provided specific precautions are undertaken. No evidence-based therapy exists in secondary CAS, but optimal treatment of the underlying disorder is essential when feasible.     

Antenatal treatment of fetal alloimmune cytopenias

Summary The antenatal use of intravenous immunoglobulin (IVG) was explored in 9 cases of alloimmune cytopenias affecting fetuses. In 7 cases of alloimmune thrombocytopenia, IVG at a dose of 1 gm/kg/week appeared to be uniformly effective in elevating the fetal platelet count and preventing a recurrence of antenatal intracranial hemorrhage (2 cases). In 2 cases of Rh disease the results were more equivocal. There did not appear to be any significant toxicity associated with its use. The mechanism of IVG effect in the successfully treated cases remains uncertain.Presented at the International Workshop on ITP, August 26 and 27, 1988, Lucerne, Switzerland