Extreme hyperbilirubinemia: An indicator of morbidity and mortality in sickle cell disease

BACKGROUND Sickle cell disease(SCD) is a disorder that results in increased hospitalizations and higher mortality. Advances in management have resulted in increases in life expectancy and led to increasing awareness of sickle cell hepatopathy(SCH).However, its impact in patients on the natural history and outcomes of SCD is not known. Our study aims to describe the prevalence of extreme hyperbilirubinemia(EH), one form of SCH, its effect on morbidity and mortality,and correlations between sickle cell genotype and SCH type. We hypothesize that EH is associated with higher morbidity and mortality.AIM To investigate the effects of EH on morbidity and mortality among patients with SCD.METHODS This retrospective cohort study was performed using a database of patients with SCD treated at Grady Memorial Hospital between May 2004 and January 2017.Patients with EH(defined as total bilirubin above 13.0 mg/dL) were identified. A control group was identified from the same database with patients with total serum bilirubin ≤ 5.0 mg/dL. Electronic medical records were used to extract demographic information, laboratory values, radiology results, current medications, need for transfusions and mortality data. Two samples T-test, chi-squared test and Fisher's exact test were then used to compare the parameters between the two groups.RESULTS Out of the database, fifty-seven charts were found of patients with bilirubin 13 mg/dL. Prevalence of severe SCH as defined by EH was 4.8%(57/1172). There were no demographic differences between patients with and without EH.Significant genotypic differences existed between the two groups, with hemoglobin SS SCD being much higher in the EH group(P 0.001). Patients with severe EH had a significant elevations in alanine aminotransferase(157.0 ± 266.2 IU/L vs 19.8 ± 21.3 IU/L, P 0.001), aspartate aminotransferase(256.5 ± 485.9 U/L vs 28.2 ± 14.7 U/L, P 0.001) and alkaline phosphatase(218.0 ± 176.2 IU/L vs 85.9 ± 68.4 IU/L, P 0.001). Patients with EH had significantly higher degree of end organ failure measured with quick Sequential Organ Failure Assessment scores(0.42 ± 0.68 vs 0.01 ± 0.12, P 0.001), increased need for blood products(63% vs 5%, P 0.001), and exchange transfusions(10.5% vs 1.3%, P = 0.022).CONCLUSION Among patients with SCD, elevated levels of total bilirubin are rare, but indicative of elevated morbidity, mortality, and need for blood transfusions.Large differences in sickle cell genotype also exist, but the significance of this is unknown.     

基于《素问》运气七篇探讨壬辰年慢性肝病的诊治规律

《素问》运气七篇大论是基于"天人合一"的整体观,以五运六气立说,重点揭示疾病的发生与中医运气学说的关系,提出了具有指导意义的病因病机理论、诊治理论及方药理论,建构了独具特色的中医运气理论体系,对临床辨证论治起着越来越重要的作用。文章在《素问》运气七篇大论的基础上对壬辰年(2012年)的运气变化进行论述,并结合慢性肝病的病机特点,探讨壬辰年慢性肝病的中医诊治规律,以期为2012年慢性肝病的中医药诊治及养生调护提供参考。     

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non‐SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.     

FTL在慢性肝病患者血清中的表达及临床意义

目的 探讨血清铁蛋白轻链(Ferritin light chain, FTL)在慢性肝脏疾病进展过程中表达水平的变化及其潜在的临床应用价值。方法 利用iTRAQ定量蛋白组学技术对随机选取的慢性肝病患者与健康者血清进行比较研究, 酶联免疫吸附法（ELISA）检测显著性差异蛋白FTL在不同阶段慢性肝病患者和健康者血清中的含量, 应用统计学方法对FTL在不同阶段慢性肝病中表达量进行组间比较,并分析其与血清丙氨酸氨基转移酶(ALT)、天门冬氨酸氨基转移酶(AST)、血清铁蛋白(Serum Ferritin, SF)的相关性。结果 通过对慢性肝病患者与健康者血清样品进行质谱分析, 筛选并鉴定出差异表达蛋白69个,其中FTL是慢性肝病患者血清中表达明显上调的差异蛋白。ELISA检测不同阶段慢性肝病患者和健康者血清中的FTL表达水平,结果显示不同阶段慢性肝病患者血清中的FTL表达均高于健康者,且随着肝病病情进展呈下降趋势;统计学分析显示:FTL与ALT、AST、SF呈正相关性(P均＜0.01）。不同阶段慢性肝病患者血清中FTL表达差异均具有统计学意义(P＜0.01）,其表达水平和肝病进展程度呈负相关性(rs = -0.405,P ＜0.01)。结论 FTL与慢性肝脏疾病密切相关, 其表达水平不仅能体现肝脏的损伤状况,还能反映肝脏疾病的进展情况,在慢性肝病诊断、监测和治疗方面均具有良好的应用前景。     

Effects of Silymarin on Diabetes Mellitus Complications: A Review

Diabetes mellitus is a common metabolic disorder that is caused by a deficit in the production of (type 1) or response to (type 2) insulin. Diabetes mellitus is characterized by a state of chronic hyperglycemia and such symptoms as weight loss, thirst, polyuria, and blurred vision. These disturbances represent one of the major causes of morbidity and mortality nowadays, despite available treatments, such as insulin, insulin secretagogues, insulin sensitizers, and oral hypoglycemic agents. However, many efforts have been made to discover new drugs for diabetes treatment, including medicinal plant extracts. Silymarin is a powder extract of the seeds from Silybum marianum, a plant from the Asteraceae family. The major active ingredients include four isomers: silybin, isosilybin, silychristin, and silydianin. Silymarin is indicated for the treatment of hepatic disorders, such as cirrhosis, chronic hepatitis, and gallstones. Moreover, several studies of other pathologies, including diabetes, sepsis, osteoporosis, arthritis, hypercholesterolemia, cancer, viral infections, and Alzheimer's and Parkinson's diseases, have tested the effects of silymarin and reported promising results. This article reviews data from clinical, in vivo, and in vitro studies on the use of silymarin, with a focus on the complications of diabetes, including nephropathy, neuropathy, healing delays, oxidative stress, hepatotoxicity, and cardiomyopathy. Copyright © 2017 John Wiley & Sons, Ltd.     

上海青浦地区维持性血液透析患者的现况调查

目的：了解上海郊县维持性血液透析（maintenance hemodialysis,MHD）患者的治疗状况,为优化郊县和农村地区的血液透析治疗提供依据。方法：2009年10月对上海市青浦区患者的治疗情况进行横断面调查。结果：共调查维持性血液透析治疗患者133例,其中男性73例,女性60例。患者年龄55.28±12.36（26~80）岁,透析龄40.71±30.56（3~131）个月。患者中高血压病患病率、治疗率和达标率分别为88.0%（117/133）、99.1%（116/117）和12.8%。49.6%的高血压病患者服用1种降压药,应用2种、3种和以上者分别占40.0%和10.4%。单药用药以钙通道阻滞剂最多（87.9%）,其次为血管紧张素受体阻断剂（22.9%）。贫血治疗的达标率为29.3%（39/133）。促红细胞生成素治疗率为94.7%,口服铁剂应用率为85.7%。患者血钙、血磷、钙磷乘积和甲状旁腺激素（iPTH）的治疗达标率依次为54.9%、25.6%、51.1%和32.3%;仅有5例（3.76%）上述4项指标均达到要求。患者中30.1%（40/133）的患者合并血吸虫肝病,前者较无血吸虫肝病患者年龄高（58.8±11.7岁比53.8±12.4岁,P〈0.05）,血清白蛋白和血红蛋白（hemoglobin,Hb）水平低（39.2±6.4g.L-1比41.1±3.1g.L-1;96.1±9.8g.L-1比99.9±10.0g.L-1,P〈0.05）。结论：上海青浦地区MHD患者合并血吸虫肝病、贫血、高血压病的患病率高且相应治疗不充分,血压和Hb的达标率低。相当比例的血液透析患者未能满意地控制钙磷代谢及继发性甲状旁腺功能亢进。     

急性中毒性肝病的临床特征研究

目的：探索急性中毒对肝脏功能的特征性影响。方法：应用临床资料,对8种毒物引起的52例急性中毒性肝病患者的临床资料进行分析,并与85例急性病毒性肝炎患者对照研究。结果：8种毒物中,杀虫脉,砷化氢,毒蕈的肝损害发生率最高（P＜0．01）,各毒物间比较所致肝功能化验指标升高的程度无明显差异。急性中毒性肝病肝脏叩击痛及肝脏肿大的发生率非常显著高于急性病毒性肝炎患者,而发热及黄疸则刚好相反（P＜0．01）,肝功能化验丙氨酸转氨酶（ALT）,天门冬氨酸转氨酶（AST）,血清总胆红素（TBIL）等指标的升高程度,则非常显著低于急性病毒性肝炎（P＜0．01）,肝功能恢复时间显著短于急性病毒性肝炎（P＜0．01）,结论：急性中毒性肝病可由多种毒物引起,一般急性中毒性肝损害是可逆的,起病快,恢复快,治疗效果及预后较急性病毒性肝炎为好。     

聚合酶链反应检测肝病患者血液中纳米细菌的研究

目的 评价PCR检测慢性肝病和肝癌患者血液中纳米细菌(NB)的临床价值.方法 对68例慢性乙型肝炎(CHB)、54例慢性重型乙型肝炎(CSHB)、66例肝硬化(CL)、23例肝癌(HCC)患者采用免疫组化染色、透射电镜扫描和PCR技术,检测血液中的纳米细菌,并与40例健康人结果比较.结果 PCR检测纳米细菌阳性率分别为27.69%、50.00%、61.29%、52.38%和5.00%,慢性肝病和肝癌患者阳性率明显高于健康人(P＜0.005).慢性乙型肝炎阳性率低于其他肝病(P＜0.005).PCR测定结果与免疫组化染色比较,两法阳性率无明显差异(P＞0.05),PCR灵敏度为46.51%,特异性为73.95%,总有效率66.67%.结论 PCR对纳米细菌的检测具有重要的参考价值.慢性肝病和肝癌患者血液中纳米细菌感染率高于健康人.     

武汉市2005～2008年疟疾发病情况及流动人口的管理措施

目的为了做好武汉市疟疾防控工作,评价疟防工作成效,为今后开展疟疾监测和防控提供依据。方法收集武汉市2005～2008年疟疾疫情报告、个案调查资料,分析武汉市2008年疟疾发病情况,比较2005～2008年疟疾发病趋势。结果武汉市近4年疟疾发病率均在1/10万以下,流动人口疟疾管理工作切实有效,国外输入性病例成为疟疾发病的主要群体,且恶性疟病例逐渐增多。结论武汉市疟疾发病率低,但不稳定因素增加,应加强流动人口疟疾管理工作。     

职业性中毒性肝病实验室诊断研究

本文对临床已诊断为急、慢性中毒性肝病160例患者,进行了23种实验室肝脏功能试验研究。急性患者的肝功能试验阳性发生率为PA70%(14/20),ADA55%(4/20)、ALT45%(9/20),CG40%(8/20),与其余19种试验比较有显著差异(P<0.01)。对慢性患者,以ILG100%(11/11),ITTT45 64。75%(90/139),Tf 52。59%(71/135),CG31.75%(40/126)最为敏感,与常用肝功能的ALT 比较有显著差异(P<0.01)。中毒性肝病的检验阳性频率,急性病例有30%患者同时出现6种以上试验阳性。慢性病例有35%患者可同时出现4种以上试验阳性。肝脏功能试验的灵敏度,在急性病例血清酶和PA 检查出现明显的阳性,而在慢性病例以廓清试验(ICG,ITTT45)及Tf 最为敏感。