1977例急性、亚急性、慢加急性肝衰竭患者的病因与转归分析

目的 探讨急性肝衰竭(ALF)、亚急性肝衰竭(SALF)、慢加急性肝衰竭(ACLF)的病因. 方法回顾性总结1977例肝衰竭患者的临床资料,对病因、年龄、性别、转归等方面进行比较分析.结果 ALF的前三位病因是:HEV感染(33.96%)、HBV感染(13.21%)与药物性肝病(9.43%);SALF为药物性肝病(31.53%)、HEV感染(16.22%)、HBV感染(9.91%);ACLF为HBV感染(90.29%)、洒精性肝病(2.65%)、HBV与HEV重叠感染(2.26%).常见嗜肝病毒感染者占90.09%(1781例),其中单HBV感染占92.93%(1655例).在HBV感染者中(1655例),26～55岁患者占77.10%(1276例).2005-2007年酒精性肝衰竭患者39例,占酒精性病因患者的81.25%(48例);2006-2007年药物性肝衰竭共23例,占药物性病因的56.10%(41例).除药物性肝损伤外,其他病因均男性多于女性.三类肝衰竭总治愈,好转率为35.56%,HEV感染性肝衰竭的治愈,好转率高于药物性肝衰竭(x2=4.42,P<0.05),其他组间差异无统计学意义.结论 不同类型肝衰竭主要病因不同;HBV感染居肝衰竭病因之首,酒精性、药物性肝衰竭呈上升趋势;HEV感染性肝衰竭治愈、好转率相对较高.     

从2例患儿的不同结局探讨儿童肝豆状核变性筛查的必要性

对四川省妇幼保健院收治的2例肝功能异常儿童进行生物化学及基因检测,2例患儿均确诊为肝豆状核变性,病例1以肝衰竭并溶血性贫血为主要表现,反复行人工肝治疗但病情无好转,家属放弃治疗后死亡;病例2体格检查发现肝功能异常,口服锌剂治疗后肝功能正常,随访近2年无不适.肝豆状核变性患儿出现临床症状后再就诊往往存在诊断延迟,因此有必...     

79例亚急性肝衰竭患者的临床分析

目的分析79例亚急性肝衰竭患者的临床特征、预后及特殊治疗对预后的影响。方法收集2004年1月～2010年1月北京地坛医院收治的亚急性肝衰竭病例共79例,对其性别、年龄、病因、并发症、预后及特殊治疗方法进行归纳总结。结果 79例患者中,男性60例,女性19例,平均年龄(47.59±19.01)岁,最常见的病因为戊型肝炎病毒(HEV)感染(43.04%),其次为HBV感染(27.85%)。发生率最高的3类并发症依次为腹水、腹腔感染、肝性脑病,与死亡明显相关的并发症为肝肾综合征及肝性脑病。本组资料的病死率约为18.99%。结论亚急性肝衰竭好发于男性及40岁以上人群,病死率较高。HEV感染是本组资料中最常见的致病因素,腹水、腹腔感染为最常见的并发症,肝肾综合征是最常见的致死因素。     

重视肝活组织病理检查在临床肝病诊疗中的应用

目的探讨超声引导下经皮肝穿刺活组织病理检查在临床肝病诊疗中的意义。方法回顾性分析了吉林大学第一医院157例超声引导下经皮肝穿刺活组织病理检查病例,所有病例均为肝损害经常规检查方法未能明确诊断或临床诊断分期不明确。所有患者经临床生化学检查及影像学检查均未提示肝硬化。分为I组(HBV感染组)和II组(非HBV感染组)。结果 157例病例中,I组81例,其中IA组(ALT<2×ULN)43例,IB组(ALT≥2×ULN)38例。II组76例,其中自身免疫性肝病30例,药物性肝损害21例,脂肪性变11例,罕见病例8例,隐源性肝炎6例。结论超声引导下经皮肝穿刺活组织检查安全性较高,肝活组织病理检查在不明原因的肝损害或临床诊断分期不明确的肝损害诊断中有非常重要的价值,可减少临床漏诊及误诊,有利于明确肝损害的病因及程度。     

肝豆状核变性合并病毒性肝炎病例报道

本通过一例肝豆状核变性合并病毒性肝炎的报道．说明对于某些原因不明的肝硬化或并发病毒性肝炎的儿童和青少年,尤其有家族史．要高度警惕肝豆状核变性。对于肝硬化伴有凝血障碍的病人．要慎用活血药。     

血浆铜蓝蛋白在乙型肝炎不同肝功能状态的水平及意义

目的探讨肝豆状核变性、乙型肝炎不同肝功能状态时血浆铜蓝蛋白水平的差异及其临床意义。方法用终末期肝病模型(model for end-stage liver disease,MELD)评分衡量肝功能损害的严重性,采用散射比浊法检测并比较分析肝豆状核变性、慢加急性肝衰竭极期以及恢复期、慢性乙型肝炎患者的血浆铜蓝蛋白水平。结果肝豆状核变性(n=50)、慢加急性肝衰竭极期和恢复期(n=30)、慢性乙型病毒性肝炎患者(n=50)的MELD评分分别为11.1±6.5、20.4±4.2、10.9±4.9、9.6±9.8,血浆铜蓝蛋白分别为(0.065±0.036)g/L、(0.176±0.037)g/L、(0.210±0.056)g/L、(0.197±0.038)g/L;血浆铜蓝蛋白水平4组之间的两两比较存在差异(F=111.4,P<0.001),肝豆状核变性患者低于其他3组(P<0.001)。结论肝豆状核变性血浆铜蓝蛋白水平显著降低。相对于肝豆状核变性,没有肝衰竭的慢性乙型肝炎血浆铜蓝蛋白水平几乎正常。慢加急性肝衰竭极期血浆铜蓝蛋白水平仅轻度下降,随着肝功能衰竭的恢复,血浆铜蓝蛋白恢复正常。     

83例肝豆状核变性患者的临床特征分析

目的 总结肝豆状核变性患者的临床特征。方法 回顾性分析2013年4月—2021年8月河北医科大学第三医院收治的83例肝豆状核变性患者的临床表现、实验室/影像学/肝组织病理学检查、ATP7B基因检测结果。根据损伤部位不同区分临床类型。多组间比较采用单因素方差分析。结果 83例WD患者中,男女比例1∶1.075;发病年龄最小为3岁,平均(21.16±14.87)岁,其中≤18岁者39例(46.99%);以肝损伤为首发表现者(肝型)为主(63.86%),31例(37.35%)患者就诊时已进展为肝硬化,5例(6.2%)患者以急性或慢加急性肝衰竭就诊。角膜K-F环阳性62例(74.69%),其中肝型患者K-F环阳性率为66.04%;血铜蓝蛋白下降79例(95.18%),24 h尿铜升高73例(87.95%)。25例肝组织病理结果显示,肝组织均有不同程度的炎症、纤维化及脂变,以及铜颗粒沉积。25例ATP7B基因检测结果中,突变位点以外显子8的c.2333G>T/p.R778L最为常见。结论 肝豆状核变性患者以肝病表现为主,角膜K-F环、血清铜蓝蛋白、24 h尿铜指标均有一定局限性。诊断不明时...     

以血小板减少为首发表现的肝豆状核变性1例并文献复习

目的:重视以血小板减少为首发表现的肝豆状核变性的诊断、鉴别诊断及治疗。方法:报告1例以血小板减少为首发表现的肝豆状核变性的患者,总结肝豆状核变性的临床表现、诊断及治疗方法。结果:肝豆状核变性的临床首发表现多样,极易漏诊。本例以血小板减少为首发表现。结论:临床病例表现多样,切记思维定势,病因寻找要从常见病开始,亦不要忽略了少见、罕见病的可能,肝豆状核变性的早期诊断、治疗对其预后十分关键。     

暴发性肝豆状核变性的诊断

暴发性肝豆状核变性（fulminant Wilson disease，FWD）是肝豆状核变性病（WD）的一种少见而严重的临床类型，临床表现与其他原因引起的暴发型肝衰竭类似，国内外鲜见从临床、病理和分子生物学多个水平追踪观察的报道。我们对2例典型FWD的临床、病理和基因突变特点进行了分析，以提高对WD的认知性和诊断水平。     

2005年美国肝病学会关于急性肝衰竭诊疗现状 (Position Paper)要点

急性肝衰竭的定义 被广泛接受的急性肝衰竭(ALF)的定义是既往无肝硬化者在26周内出现肝功能恶性化而导致的凝血功能障碍(INR≥1.5)及任何程度的意识改变(脑病).对于有肝豆状核变性、垂直感染HBV者或自身免疫性肝炎,如果其疾病是在26周以内发现的,即使有肝硬化,也可包括在ALF内.     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Changes in connexin43, the gap junction protein of astrocytes, during development of the rat pineal gland

Abstract: The abundance of gap junctions between rat pineal astrocytes formed by connexin43 (Cx43) was studied during development. Levels and distribution of Cx43 were measured by immunoblotting and indirect immunofluorescence, respectively. The amount of Cx43 in cells located within the gland was low until about the 7th postnatal day and increased to adult values between the 14th and 21st days postpartum. Although astrocytes, recognized by their vimentin immunoreactivity, were scarce before birth, they were abundant by the 7th postnatal day suggesting that the low levels of Cx43 found at this age corresponded to a low expression of this protein. Localization of the immunoreactivity to Cx43 and vimentin showed a close correlation, indicating that mature or immature pineal astrocytes form gap junctions made of Cx43. Since Cx43 levels attained their adult values at about the time the innervation and the functional state of the gland reached maturity (2–3 weeks after birth), it is proposed that astrocyte gap junctions are involved in the function of the adult rat pineal gland.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Presence of immunoreactive growth hormone and prolactin in the ovine pineal gland

Abstract: The use of antisera raised against bovine growth hormone (GH) and ovine prolactin (PRL) enabled the detection of related immunoreactive (ir) sequences of proteins in ovine pineal tissue. The isolation of PRL-like ir-material was accomplished using a 0.25 M ammonium sulphate (pH 5.5) extraction followed by ethanol precipitation, whereas the resulting 2.0 M ammonium sulphate (pH 7.0) precipitate contained a GH-like immunoreactivity. Gel chromatography of the GH-like immunoreactivity (Sephadex G-100) indicated the presence of several GH-like fragments ranging in the M_r range of 7,000 to 55,000. Analyses of the PRL-like ir-material found in pineal tissue on HPLC using a TSK 545-DEAE column led to the resolution into a single peak of immunoreactivity. A single peak of activity was also observed following chromatofocusing and hydrophobic interaction chromatography of the ir-peak from the TSK 545-DEAE column. The PRL-like ir-material inhibited the binding of [¹²⁵I]ovine PRL-S14 to anti-ovine PRL antibodies without showing an affinity for binding to anti-rat PRL or anti-bovine GH antibodies. Scatchard analysis of the binding of pineal PRL-like ir-material and pituitary ovine PRL-S14 to liver membranes from day-20 pregnant rats revealed similar affinity constants (K_a of 4.7 ± 0.2 × 10⁹ M^-1). In addition, the replication of Nb 2 Node rat lymphoma cells was stimulated by pineal PRL-like ir-material, an effect known to be specific for lactogenic hormones. The pineal PRL-like immunoreactivity appeared on sodium dodecyl sulfate polyacrylamide gels as a single major band of M_r 24,000. The functional status of PRL-and GH-like ir-material in the ovine pineal remains to be determined, but evidence is presented that the overall protein synthesis rate of the rat pineal responded to circulating concentrations of PRL.     

Microbiology of human immunodeficiency virus anorectal disease

PURPOSE: Individuals who are seropositive for the human immunodeficiency virus are at high risk for opportunistic infection and anorectal disorders. Little prospective information is available regarding anorectal pathogens in these patients. METHODS: One hundred sixty-three HIV-seropositive patients presented to the colorectal clinic between 1989 and 1992. Forty-seven (29 percent) patients were thought to have an infectious process and were prospectively studied using a standardized multiculture protocol. RESULTS: Mean age was 33 (range, 19–59) years. All were male; high-risk behavior accounted for 87 percent of HIV transmissions. Presenting complaints included anorectal pain (79 percent), pus per anum (28 percent), and blood per anum (26 percent). Examination revealed perianal tenderness (60 percent), condyloma (38 percent), perianal ulcers (38 percent), and anal fissures (34 percent). Sixty-six sets of cultures were performed; 28 patients had one set, 15 had two sets, and 4 had three sets. Thirty-two of these 47 patients (68 percent) had positive cultures including herpes (50 percent), cytomegalovirus (25 percent),Neisseria gonorrhoeae (16 percent), chlamydia (16 percent), acidfast bacilli (2 percent), and others (9 percent). Six of 32 patients with positive cultures had more than one organism cultured. Sixteen (50 percent) patients with positive cultures were treated medically, 8 (25 percent) were treated surgically and 8 (25 percent) were treated with both modalities. Sixty-one procedures were performed on 17 patients for condylomata. Eighteen patients had 20 procedures for abscesses, 50 percent of whom had positive cultures for other than common bowel flora; all improved. Fourteen patients underwent 33 procedures for perianal fistulas.Mycobacterium fortuitum was cultured from one patient who required 13 procedures for abscesses and fistulas. Forty-five (96 percent) patients were followed for an average of 12.5 months ±2.9 SEM (range, 1–94 months). Symptoms were improved or resolved in 22 of 32 (69 percent) patients with positive cultures and in 11 of 13 (84 percent) with negative cultures. CONCLUSIONS: Specific pathogens may often be identified in human immunodeficiency virus-seropositive patients with anorectal disorders if aggressively sought. Although patients without specific pathogens identified may be expected to improve with planned empiric treatment, positive identification allows more directed therapy.