hyperbilirubinemia

高胆红素血症
  • 文章类型: Case Reports
    血小板减少症,Anasarca,发烧,骨髓活检/肾功能不全的网状蛋白纤维化,器官肿大(TAFRO)综合征是罕见的疾病,具有与多器官损伤相关的多种临床和病理特征。很少有TAFRO综合征伴有高胆红素血症肝损害的报道。我们描述了一名61岁男性的病例,该男性出现突然发作的腹痛并伴有高胆红素血症的肝损害。他的症状恶化了,导致发烧,肝功能不全,浆液腔积液,血小板减少症,和急性肾衰竭.类固醇停药后发热和anasarca复发。通过从腋窝淋巴结进行活检,最终诊断出患者患有TAFRO综合征。然后他服用了类固醇,几乎完全解决了他的症状.我们的病例以其非典型体征和TAFRO综合征完全缓解而著称。
    Thrombocytopenia, anasarca, fever, reticulin fibrosis on bone marrow biopsy/renal dysfunction, and organomegaly (TAFRO) syndrome are infrequent conditions with diverse clinical and pathological characteristics related to multi-organ damage. There are few reports of TAFRO syndrome accompanied by liver damage with hyperbilirubinemia. We describe the case of a 61-year-old male who presented with sudden onset abdominal pain accompanied by liver damage with hyperbilirubinemia. His symptoms worsened, leading to fever, hepatic insufficiency, serous cavity effusions, thrombocytopenia, and acute renal failure. Fever and anasarca relapsed after steroid discontinuation. The patient was ultimately diagnosed with TAFRO syndrome by biopsies taken from the axillary lymph nodes. He was then administered steroids, which resolved his symptoms almost completely. Our case was notable for its atypical signs and total remission of TAFRO syndrome.
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  • 文章类型: Case Reports
    钩端螺旋体病,由钩端螺旋体属螺旋体引起的急性人畜共患病感染,在全球范围内构成重大健康风险。通过接触受感染动物的尿液传播,血,或组织。此病例报告检查了一名44岁的严重钩端螺旋体病男子,表现为韦尔的疾病,以急性低氧性呼吸衰竭和横纹肌溶解继发的急性肾损伤(AKI)为特征,并发严重低钠血症.该病例强调了与钩端螺旋体病相关的诊断和管理挑战,强调跨学科合作和综合诊断评估的重要性。
    Leptospirosis, an acute zoonotic infection caused by spirochetes of the genus Leptospira, poses significant health risks worldwide. Transmission occurs through contact with infected animals\' urine, blood, or tissue. This case report examines a 44-year-old man with severe leptospirosis, presenting as Weil\'s disease, characterized by acute hypoxic respiratory failure and acute kidney injury (AKI) secondary to rhabdomyolysis, complicated by severe hyponatremia. The case underscores the diagnostic and management challenges associated with leptospirosis, highlighting the importance of interdisciplinary collaboration and comprehensive diagnostic evaluation.
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  • 文章类型: Journal Article
    背景:胎儿和新生儿溶血病(HDFN)是由母体抗体攻击胎儿血细胞抗原引起的。尽管常规产前抗D预防,在某些HDFN病例中仍需要进行宫内输血(IUTs)。方法:我们对华沙医科大学第一妇产科出生的HDFN新生儿进行了回顾性队列研究。我们分析了274例HDFN新生儿,确定46名因胎儿贫血而需要IUT的人和228名未需要IUT的人。实验室结果,管理,并比较这些组的结局.结果:对比分析显示,接受IUT治疗的新生儿更容易出现明显的贫血,高胆红素血症,铁过载,表明铁蛋白浓度高。这些新生儿更经常需要输血,光疗,静脉注射免疫球蛋白,交换输血。接受IUT的新生儿的停留时间更长。结论:与不需要IUT的HDFN相比,需要IUT的HDFN与新生儿期更多的并发症有关,并且通常需要额外的治疗。
    Background: Hemolytic disease of the fetus and newborn (HDFN) is caused by maternal antibodies attacking fetal blood cell antigens. Despite routine antenatal anti-D prophylaxis, intrauterine transfusions (IUTs) are still needed in some HDFN cases. Methods: We conducted a retrospective cohort study on newborns with HDFN born in the 1st Department of Obstetrics and Gynecology of the Medical University of Warsaw. We analyzed 274 neonates with HDFN, identifying 46 who required IUT due to fetal anemia and 228 who did not. The laboratory results, management, and outcomes were compared between these groups. Results: Comparative analysis showed that newborns treated with IUT were more likely to have significant anemia, hyperbilirubinemia, and iron overload, indicated by a high ferritin concentration. These neonates more often required top-up transfusions, phototherapy, intravenous immunoglobulin infusions, and exchange transfusions. The length of stay was longer for newborns who received IUT. Conclusions: HDFN requiring IUT is associated with a greater number of complications in the neonatal period and more often requires additional treatment compared to HDFN not requiring IUT.
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  • 文章类型: Journal Article
    先前关于口服硫酸锌治疗对新生儿血清胆红素水平影响的研究产生了相互矛盾的结果。因此,这项临床研究的目的是评估口服硫酸锌对新生儿重症监护病房的足月儿血清胆红素水平的影响.这项研究是在Sanandaj的Besat医院的新生儿护理部门进行的,库尔德斯坦省,作为一项双盲随机对照试验。参与者包括需要光疗并进入新生儿重症监护病房的足月婴儿(妊娠37-42周)。共纳入290名婴儿,随机分为两组。干预组除接受光疗外,还接受每日1mg/kg剂量的口服硫酸锌补充剂,而安慰剂组每天接受等量的安慰剂。在干预开始时以及随后每24小时获得胆红素测量值,直到出院。收集的数据使用STATA软件版本17进行分析。在将婴儿随机分配到硫酸锌组和安慰剂组之后,研究结果,包括干预后胆红素水平的平均变化,光疗的时间,以及住院天数,对硫酸锌组160名婴儿和安慰剂组130名婴儿进行了分析和比较。接受硫酸锌的婴儿的胆红素水平降低为(-3.75±0.19CI95%-4.12,-3.37),安慰剂组为(-1.81±0.15CI95%-2.12,-1.50)干预后24小时。此外,干预后48和72小时,干预组胆红素水平下降幅度更大.硫酸锌组住院时间较短(2.13±0.04vs.2.83±1.42),并且所需的光疗时间少于安慰剂组(6.21±2.16vs.8.78±1.40)。结论:高胆红素血症足月新生儿口服硫酸锌可降低胆红素水平,光疗的持续时间,住院。试用注册:IRCT,IRCT20220806055625N1。研究注册2022年12月25日,http://irct。ir/审判/66,722。
    Previous investigations on the impact of oral zinc sulfate treatment on newborns\' serum bilirubin levels have produced conflicting results. As a result, the goal of this clinical study was to evaluate how oral zinc sulfate affected the levels of serum bilirubin in term infants who were admitted to the neonatal intensive care unit. The study was conducted at the Neonatal Care Unit of Besat Hospital in Sanandaj, Kurdistan Province, as a double-blind randomized controlled trial. The participants included term infants (37-42 weeks of gestation) who required phototherapy and were admitted to the neonatal intensive care unit. A total of 290 infants were enrolled and randomly divided into two groups. The intervention group received oral zinc sulfate supplementation at a dosage of 1 mg/kg per day in addition to phototherapy, while the placebo group received an equivalent amount of placebo daily. Bilirubin measurements were obtained at the initiation of the intervention and subsequently every 24 h until discharge. The collected data were analyzed using STATA software version 17. After the infants were randomly allocated to the zinc-sulfate and placebo groups, the study outcomes, including the average changes in bilirubin levels after intervention, the hours of phototherapy, and the number of days of hospitalization, were analyzed and compared for a total of 160 infants in the zinc sulfate group and 130 infants in the placebo group. The reduction in bilirubin levels in infants receiving zinc sulfate was (- 3.75 ± 0.19 CI 95% - 4.12, - 3.37) and for placebo group was (- 1.81 ± 0.15 CI 95% - 2.12, - 1.50) 24 h after the intervention. Furthermore, 48 and 72 h following the intervention, bilirubin levels in the intervention group demonstrated a more substantial decline. The zinc sulfate group had a shorter hospital stay (2.13 ± 0.04 vs. 2.83 ± 1.42) and required less phototherapy hours than the placebo group (6.21 ± 2.16 vs. 8.78 ± 1.40).           Conclusions: Oral zinc sulfate supplementation in term neonates with hyperbilirubinemia decreased the level of bilirubin levels, duration of phototherapy, and hospital stay.           Trial registration: IRCT, IRCT20220806055625N1. Study Registered 25 December 2022, http://irct.ir/trial/66,722 .
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  • 文章类型: Case Reports
    血色素沉着症是一种以铁积累过多为特征的疾病,导致各种器官功能障碍。一个50岁的女人,以前身体健康,报告腹痛和皮肤和眼睛发黄一个月。经检查,她表现出广泛的黄疸,腿部肿胀,腹胀.入院时总胆红素水平为24.52mg/dL,提示高胆红素血症.影像学检查,包括USG和CT扫描,显示轻度至中度腹水和肝脏质地改变。血清铁蛋白(1443ng/mL)和转铁蛋白饱和度(84%)升高提示铁过载。肝活检证实肝细胞中存在铁沉积物,导致血色素沉着病的诊断。基因检测显示C282Y和H63D突变呈阴性,导致诊断为非稳态铁调节剂(非HFE)相关的遗传性血色素沉着症。患者每周开始放血,并定期进行监测,肝移植被认为是一种潜在的治疗方法。
    Hemochromatosis is a condition marked by excessive iron accumulation, causing dysfunction in various organs. A 50-year-old woman, previously in good health, reported abdominal pain and yellowing of the skin and eyes for one month. Upon examination, she exhibited widespread jaundice, leg swelling, and abdominal distention. Her total bilirubin level was 24.52 mg/dL at admission, indicating hyperbilirubinemia. Imaging studies, including USG and CT scans, revealed mild to moderate ascites and altered liver texture. Elevated serum ferritin (1443 ng/mL) and transferrin saturation (84%) suggested iron overload. A liver biopsy confirmed the presence of iron deposits in hepatocytes, leading to a diagnosis of hemochromatosis. Genetic testing was negative for the C282Y and H63D mutations, resulting in a diagnosis of non-homeostatic iron regulator (non-HFE) related hereditary hemochromatosis. The patient began weekly phlebotomy and was monitored regularly, with a liver transplant being considered as a potential treatment.
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  • 文章类型: English Abstract
    要设计和制备具有良好机械性能和生物相容性的高效胆红素吸附剂。
    在这项研究中,设计并合成了季铵吡啶,然后改性聚醚砜微球,或PES/p(4-VP-co-N-VP)@6微球,通过相转换和静电喷涂制备。通过核磁共振(NMR)光谱和扫描电子显微镜研究了聚合物组分和微球的形态。测试了微球的基本性能及其对胆红素的吸附效率,并进一步探讨了吸附机理。还测量了微球体的血细胞计数和凝固时间。
    研究中制备的改性聚醚砜微球的直径约为700-800μm。与原始PES微球相比,PES/p(4-VP-co-N-VP)@6微球的表面和内部结构没有明显变化,它们也有松散的多孔结构,除了不规则的大毛孔外,还散布着一些微孔。与对照组相比,改性微球在胆红素PBS缓冲溶液中静态吸附180min后,胆红素去除效果为(94.91±0.73)%,差异有统计学意义(P<0.0001)。根据凝血时间的发现,空白血浆组活化部分凝血活酶时间(APTT),对照PES组,改性PES微球组为(27.57±1.25)s,(28.47±0.45)s,和(30.4±0.872)s,分别,实验组与其他两组比较差异有统计学意义(P<0.01,P<0.05)。红细胞和白细胞计数无明显变化。
    本研究制备的微球具有较高的胆红素吸附效率,优异的机械性能和热稳定性,良好的血液生物相容性,并有望用于肝衰竭患者的临床治疗。
    UNASSIGNED: To design and prepare a high efficiency bilirubin adsorbent with good mechanical properties and biocompatibility.
    UNASSIGNED: In this study, quaternary ammonium pyridine was designed and synthesized, and then modified polyether sulfone microspheres, or PES/p(4-VP-co-N-VP)@6 microspheres, were prepared by phase conversion and electrostatic spraying. The morphology of the polymer components and the microspheres were studied by means of nuclear magnetic resonance (NMR) spectroscopy and scanning electron microscopy. The basic properties of the microspheres and their bilirubin adsorption efficiency were tested, and the adsorption mechanism was further explored. Blood cell counts and the clotting time of the microspheres were also measured.
    UNASSIGNED: The diameter of the modified polyether sulfone microspheres prepared in the study was approximately 700-800 μm. Compared with the original PES microspheres, the surface and internal structure of PES/p(4-VP-co-N-VP)@6 microspheres did not change significantly, and they also had a loose porous structure, with some micropores scattered around in addition to irregular large pores. Compared with the control group, the bilirubin removal effect of the modified microspheres was (94.91±0.73)% after static adsorption in bilirubin PBS buffer solution for 180 min, with the difference being statistically significant (P<0.0001). According to the findings for the clotting time, the activated partial thromboplastin time (APTT) of the blank plasma group, the control PES group, and the modified PES microsphere group were (27.57±1.25) s, (28.47±0.45) s, and (30.4±0.872) s, respectively, and the difference between the experimental group and the other two groups was statistically significant (P<0.01, P<0.05). There was no significant change in red blood cell and white blood cell counts.
    UNASSIGNED: The microspheres prepared in the study have high efficiency in bilirubin adsorption, excellent mechanical properties and thermal stability, and good blood biocompatibility, and are expected to be used in the clinical treatment of patients with liver failure.
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  • 文章类型: Journal Article
    这项研究旨在评估高胆红素血症新生儿的血液学毒性和相关因素的程度。2023年4月至12月进行了一项横断面研究。总共包括247名新生儿。使用问卷和数据提取表收集数据。收集4个4ml的血液。SysmexKX-21分析仪用于血液分析,使用MindrayBS-240分析仪测量胆红素。将数据输入Epi-data,并通过SPSS进行分析。采用logistic回归。P值设定为0.05。在光疗之前,血液毒性,比如贫血,白细胞减少症,和血小板减少症,占45.7%,22.2%,和6.1%,分别,而光疗后,贫血和血小板减少症,显著增加,但是白细胞减少症,显著下降。患贫血的风险增加,胆红素>18mg/dl的新生儿为3.5、2.7和2.1倍,Rh血型不相容,并接受强化光疗,分别。低出生体重和强化光疗都使血小板减少症的发生率增加了2倍和3.4倍。分别。发现血液毒性是新生儿的严重公共卫生问题。因此,严格的随访和考虑加重因素的早期毒性检测是必要的。
    This study aimed to assess the magnitude of hematological toxicity and associated factors in newborns with hyperbilirubinemia. A cross-sectional study was conducted from April to December 2023. A total of 247 newborns were included. The data were collected using questionnaires and a data extraction sheet. Four 4 ml of blood was collected. A Sysmex KX-21 analyzer was used for blood analysis, and a Mindray BS-240 analyzer was used for bilirubin measurement. The data were entered into Epi-data and analyzed by SPSS. The logistic regression was used. The P value was set at 0.05. Before phototherapy, the hematological toxicities, such as anemia, leucopenia, and thrombocytopenia, were 45.7%, 22.2%, and 6.1%, respectively, whereas after phototherapy, anemia and thrombocytopenia, significantly increased, but the leucopenia, significantly decreased. The risk of developing anemia increased, 3.5, 2.7, and 2.1-fold among newborns with bilirubin > 18 mg/dl, with Rh blood group incompatibility, and treated with intensive phototherapy, respectively. Both low birth weight and intensive phototherapy increased the incidence of thrombocytopenia by 2 and 3.4-fold, respectively. Hematological toxicity was found to be a severe public health issue in newborns. Thus, strict follow-up and early detection of toxicity by considering aggravation factors are necessary.
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  • 文章类型: Journal Article
    目的:确定交换输血的适应症,评估交换输血产品的使用和浪费(即,重组全血交换输血),并确定这些输血在荷兰的全国分布和流行程度。
    方法:所有9家新生儿重症监护病房(NICU)和15家非NICU医院都参与了这项回顾性研究,观察,队列研究。我们检索了参与中心在11年期间订购的所有交换输血产品的适应症和使用数据。
    结果:共有574名患者订购了1,265种产品,纳入分析。严重的ABO(32.6%)和非ABO(25.2%)免疫性溶血和随后的高胆红素血症是最常见的适应症。罕见的适应症是百日咳博德特氏菌的严重白细胞增多(2.1%)和严重贫血(1.5%)。大约一半的订购产品仍未使用。在574例新生儿中的278例(48.4%),没有使用一个或多个产品,其中229例(82.7%)是由于严重的高胆红素血症的解决以及进一步加强光疗。接受交换输血的新生儿的总体患病率为14.6:100,000活产新生儿。
    结论:相当比例的产品未使用,每年在荷兰,有限数量的患者接受交换输血治疗,强调了该程序在荷兰的罕见性。
    OBJECTIVE: To identify indications for exchange transfusions, assess the use and waste of exchange transfusion products (ie, reconstituted whole blood exchange transfusions), and determine nationwide distribution and prevalence of these transfusions in the Netherlands.
    METHODS: All 9 neonatal intensive care units and 15 non-neonatal intensive care unit hospitals participated in this retrospective, observational, cohort study. We retrieved data on the indications for and use of all exchange transfusion products ordered by participating centers over an 11-year period.
    RESULTS: A total of 574 patients for whom 1265 products were ordered were included for analyses. Severe ABO (32.6%) and non-ABO (25.2%) immune hemolysis and subsequent hyperbilirubinemia were the most frequent indications. Rare indications were severe leukocytosis in Bordetella pertussis (2.1%) and severe anemia (1.5%). Approximately one-half of all ordered products remained unused. In 278 of 574 neonates (48.4%), ≥1 products were not used, of which 229 (82.7%) were due to the resolving of severe hyperbilirubinemia with further intensification of phototherapy. The overall prevalence of neonates who received an exchange transfusion was 14.6:100 000 liveborn neonates.
    CONCLUSIONS: A considerable proportion of products remained unused, and annually a limited number of patients are treated with an exchange transfusion in the Netherlands, highlighting the rarity of the procedure in the Netherlands.
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  • 文章类型: Journal Article
    UDP-葡糖醛酸基转移酶(UGT1A1)变体的谱,与吉尔伯特综合征(GS)和Crigler-Najjar综合征(CNS-II)有关,在中国和西方国家都有报道。然而,GS和CNS-II中单个UGT1A1变异体的基因型-表型相关性尚待阐明.探讨UGT1A1变异模式和基因型-表型相关性,我们招募了310名中国患者,包括232例GS患者和78例CNS-II患者。收集外周血样品用于通过聚合酶链反应和Sanger测序筛选基因UGT1A1中的变体。分析不同UGT1A1变异与临床表型的相关性。总共鉴定了21种UGT1A1变体,包括九个新颖的变体,并在GS和CNS-II患者中构成42种UGT1A1基因型。最常见的UGT1A1变体是A(TA)7TAA,p.G71R,p.Y486D,p.P364L,和p.P229Q,这与西方国家不同。p.Y486D变体在CNS-II中的次要等位基因频率高于GS,而A(TA)7TAA变体在GS中的次要等位基因频率高于CNS-II。14种复发基因型UGT1A1患者血清总胆红素和甘油三酯有显著差异,其中复方患者血清总胆红素p.Y486D(纯合子)/p.G71R变异体显著高于纯合A(TA)7TAA,纯合p.G71R,复合杂合A(TA)7TAA/p。G71R和A(TA)7TAA/p。P364L,和组合杂合A(TA)7TAA/p。G71R/p。P229Q,而患者血清甘油三酯A(TA)7TAA(纯合子)/p。P229Q变体与复合杂合子A(TA)7TAA/p相比显著更高。G71R,单杂合子A(TA)7TAA,单杂合子p.G71R,和纯合A(TA)7TAA。中国患者的UGT1A1基因型谱与西方国家不同。UGT1A1基因型复发患者血清总胆红素和甘油三酯水平存在差异。
    The spectrum of UDP-glucuronosyltransferase (UGT1A1) variants, which are associated with Gilbert syndrome (GS) and Crigler-Najjar syndrome (CNS-II), has been reported in Chinese and western countries. However, the genotype-phenotype correlation of the individual UGT1A1 variants in GS and CNS-II remains to be clarified. To explore the UGT1A1 variant pattern and genotype-phenotype correlations, we enrolled 310 Chinese patients, including 232 patients with GS and 78 with CNS-II. Peripheral blood samples were collected for screening variants in the gene UGT1A1 by a polymerase chain reaction and Sanger sequencing. The correlation between different UGT1A1 variants and clinical phenotypes was analyzed. A total of 21 UGT1A1 variants were identified, including nine novel variants, and constituted 42 UGT1A1 genotypes in the GS and CNS-II patients. The most common UGT1A1 variants were A (TA)7TAA, p.G71R, p.Y486D, p.P364L, and p.P229Q, which were different from western countries. The p.Y486D variant had higher minor allele frequency in CNS-II than in GS whereas the A (TA)7TAA variant had higher minor allele frequency in GS than in CNS-II. The serum total bilirubin and triglyceride had significant differences among 14 recurrent genotypes of UGT1A1, in which the serum total bilirubin in patients with compound p.Y486D (homozygous)/p.G71R variant was significantly higher compared with homozygous A (TA)7TAA, homozygous p.G71R, compound heterozygous A (TA)7TAA/p.G71R and A (TA)7TAA/p.P364L, and combined heterozygous A (TA)7TAA/p.G71R/p.P229Q, while the serum triglyceride in patients with combined A (TA)7TAA (homozygous)/p.P229Q variant was significantly higher compared with compound heterozygous A (TA)7TAA/p.G71R, single heterozygous A (TA)7TAA, single heterozygous p.G71R, and homozygous A (TA)7TAA. The spectrum of UGT1A1 genotypes in Chinese patients was distinct from western countries. There were differential levels of serum total bilirubin and triglyceride in patients with recurrent genotypes of UGT1A1.
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  • 文章类型: Journal Article
    在重症监护病房(ICU)和非ICU设置中偶尔观察到极端高胆红素血症。这项研究检查了极端高胆红素血症(胆红素水平≥12mg/dL)的病因以及与30天死亡率相关的因素。
    这项回顾性观察性队列研究确定了2016年至2020年间庆尚国立大学昌原医院的439例极端高胆红素血症患者。根据病因将患者分为三组和11种疾病。使用Cox比例风险模型调查与基线时30天死亡率相关的危险因素。
    在439例极端高胆红素血症患者中,287、78和74分别在肝硬化/恶性肿瘤组中,缺血性损伤组,和良性肝胆胰腺病因学组,分别,相应的30天死亡率为42.9%,76.9%,和17.6%。导致高胆红素血症的最常见疾病是胰胆管恶性肿瘤(28.7%),其次是肝硬化(17.3%),肝细胞癌(10.9%),肝转移(8.4%)。高胆红素血症的病因,阻塞性黄疸,感染,白蛋白水平,肌酐水平,凝血酶原时间-国际标准化比率与30天死亡率独立相关.
    本研究提示ICU和非ICU环境中极端高胆红素血症的三种病因。极端高胆红素血症患者的预后在很大程度上取决于病因和阻塞性黄疸的存在。
    UNASSIGNED: Extreme hyperbilirubinemia is occasionally observed in intensive care unit (ICU) and non-ICU settings. This study examined the etiologies of extreme hyperbilirubinemia (bilirubin level ≥12 mg/dL) and the factors associated with the 30-day mortality.
    UNASSIGNED: This retrospective observational cohort study identified 439 patients with extreme hyperbilirubinemia at the Gyeongsang National University Changwon Hospital between 2016 and 2020. The patients were classified into three groups and 11 diseases according to their etiology. The risk factors associated with 30-day mortality at the baseline were investigated using the Cox proportional hazards model.
    UNASSIGNED: Of 439 patients with extreme hyperbilirubinemia, 287, 78, and 74 were in the liver cirrhosis/malignancy group, the ischemic injury group, and the benign hepatobiliary-pancreatic etiological group, respectively, with corresponding 30-day mortality rates of 42.9%, 76.9%, and 17.6%. The most common disease leading to hyperbilirubinemia was a pancreatobiliary malignancy (28.7%), followed by liver cirrhosis (17.3%), hepatocellular carcinoma (10.9%), and liver metastases (8.4%). The etiologies of hyperbilirubinemia, obstructive jaundice, infection, albumin level, creatinine level, and prothrombin time-international normalized ratio were independently associated with the 30-day mortality.
    UNASSIGNED: This study suggests three etiologies of extreme hyperbilirubinemia in the ICU and non-ICU settings. The prognosis of patients with extreme hyperbilirubinemia depends largely on the etiology and the presence of obstructive jaundice.
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