Nail changes in patients with liver disease

Background Liver cirrhosis, hepatitis C virus (HCV) and hepatitis B (HBV) virus infections are known to be associated with different skin disorders. Nail changes are additional important criteria, which would help in identification of these systemic diseases. Objective To record the nail abnormalities in patients with liver disease which were not reported before, especially those with HCV and HBV infection. Patients and methods The study comprised 100 patients with HCV, HBV and liver cell failure, and 100 normal healthy controls. Both groups underwent full history taking and thorough general examination, complete blood picture, hepatitis B antigen, hepatitis C antibody, liver function tests, abdominal ultrasonography and PCR were performed in patients with liver disease. Full nail examination was performed. Results Nail changes were more prevalent in patient group (68%) than in the control group (35%). The nail infection, onychomycosis, was the most common finding in 18% of patients and that in controls was 10% followed by in a descending order, longitudinal striations, brittle nails, onychorrhexis, clubbing of fingers, dystrophic nails, leukonychia and longitudinal melanonychia. Conclusion Nail changes are observed with not only liver cirrhosis but also with HCV and HBV infection, and this will add additional clinical criteria for general practitioners and dermatologists to help them with diagnosis of these common systemic infections.     

Liver transplantation in acute liver failure: Dilemmas and challenges

Acute liver failure (ALF) refers to a state of severe hepatic injury that leads to altered coagulation and sensorium in the absence of pre-existing liver disease. ALF has different causes, but the clinical characteristics are strikingly similar. In clinical practice, however, inconsistency in the definition of ALF worldwide and confusion regarding the existence of pre-existing liver disease raise diagnostic dilemmas. ALF mortality rates used to be over 80% in the past; however, survival rates on medical treatment have significantly improved in recent years due to a greater understanding of pathophysiology and advances in critical care management. The survival rates in acetaminophen-associated ALF have become close to the post-transplant survival rates. Given that liver transplantation (LT) is an expensive treatment that involves a major surgical operation in critically ill patients and lifelong immunosuppression, it is very important to select accurate patients who may benefit from it. Still, emergency LT remains a lifesaving procedure for many ALF patients. However, there is a lack of consistency in current prognostic models that hampers the selection of transplant candidates in a timely and precise manner. The other problems associated with LT in ALF are the shortage of graft, development of contraindications on the waiting list, vaguely defined delisting criteria, time constraints for pre-transplant evaluation, ethical concerns, and comparatively poor post-transplant outcomes in ALF. Therefore, there is a desperate need to establish accurate prognostic models and explore the roles of evolving adjunctive and alternative therapies, such as liver support systems, plasma exchange, stem cells, auxiliary LT, and so on, to enhance transplant-free survival and to fill the void created by the graft shortage     

Intracranial pressure monitoring in the perioperative period of patients with acute liver failure undergoing orthotopic liver transplantation

Acute liver failure (ALF) may result in severe neurological complications caused by cerebral edema and elevated intracranial pressure (ICP). Multiple pathogenic mechanisms explain the elevated ICP, and newer hypotheses have been described. While invasive ICP monitoring (ICPM) may have a role in ALF management, these patients are typically coagulopathic and at risk for intracranial hemorrhage. ICPM is the subject of much debate, and significant heterogeneity exists in clinical practice regarding its use. Contemporary ICPM techniques and coagulopathy reversal strategies may be associated with a lower risk of hemorrhage; however, most of the evidence is limited by its retrospective nature and relatively small sample size.     

血浆置换联合连续性血液透析滤过治疗儿童急性肝衰竭

目的探讨血浆置换联合连续性血液透析滤过治疗儿童急性肝衰竭的疗效及应用时机。方法17例急性肝衰竭患儿行血浆置换联合连续性血液透析滤过治疗,收集治疗前,血浆置换后,连续性血液透析滤过8h及结束时的血标本,检测多项生化和炎症指标并进行统计分析。结果17例患儿中死亡7例,存活率58．8％。总胆红素、凝血酶原活动度、谷丙转氨酶、血氨、血清肌酐、肿瘤坏死因子旺、白细胞介素6在人工肝治疗后较治疗前均显著改善（P〈0．05）;上述7项指标中总胆红素和凝血酶原活动度在血液透析滤过结束后较血浆置换结束时无显著差异（P〈0．05）,其余指标均进一步好转（P〈0．05）。肝性脑病分期和衰竭器官数在人工肝治疗后显著下降（P〈0．05）。结论血浆置换联合连续性血液透析滤过治疗儿童急性肝衰竭安全有效,应在确诊急性肝衰竭后及早进行。     

Laparoscopic liver resection for the treatment of hepatocellular carcinoma

Accumulation of experiences and technological advances after the first report of laparoscopic liver resection(LLR) are now revealing the characteristics and specific advantages of this approach, especially for hepatocellular carcinoma(HCC) patients with chronic liver diseases(CLD). Inlaparoscopic approach, there are minimum needs for:(1) laparotomy and dissection of the attachments and adhesion which may cause destructions in the collateral blood and lymphatic flows; and(2) compression of the liver which may cause parenchymal damage for the liver resection(LR). These are especially beneficial for the patients with CLD. LLR results in minimal postoperative ascites and the other complications, which could potentially lead to lowering the risk of fatal liver failure. These characteristics of LLR facilitate surgical treatment application to the patients of HCC with background CLD. Laparoscopic approach also results in improved vision and manipulation in a small operative field under several conditions, including the cases where it is necessary to perform repeat LR between adhesions. These characteristics make LLR safer and more accessible to the repeat treatment, such as multicentric and metachronous lesions in the cirrhotic liver. These advantages of LLR indicate it is a superior method than open LR under certain conditions in patients of HCC with background CLD.     

PE+PBA、PE+DPMAS治疗急性重症肝衰竭伴MODS的临床疗效及安全性

目的观察不同人工肝支持系统治疗急性重症肝衰竭伴多器官功能障碍综合征(MODS)的临床疗效及安全性。方法选取2014年5月--2019年3月百色市人民医院收治的急性重症肝衰竭伴MODS患者168例为研究对象,采用随机数字表法将其分为观察组、对照组,各84例,对照组予以血浆置换联合血浆胆红素吸附系统(PE+PBA)治疗,观察组采用血浆置换联合双重血浆分子吸附系统(PE+DPMAS)治疗,比较两组治疗有效率,分析其治疗前后肝功能[血清总胆红素(TBIL),直接胆红素(DBIL),丙氨酸氨基转移酶(ALT),门冬氨酸氨基转移酶(AST),谷.氨酰转肽酶(GGT)]、电解质(K^+,Na^+,Ca^2+,CI)、凝血功能[凝血酶原时间(PT),凝血酶原活动度(PTA),纤维蛋白原(FIB),活化部分凝血活酶时间(APTT)]、炎症因子[肿瘤坏死因子-a(TNF-α),白介素-6(IL-6),C反应蛋白(CRP)]、不良反应发生率、临床结局。结果两组治疗有效率比较差异无统计学意义(P>0.05);治疗后两组TBIL、DBILALT、AST、GGT均下降,且观察组治疗后TBILDBIL、ALT低于对照组(P<0.05);治疗后两组K^+、Na^+均升高,而Ca^2+CI水平均下降,且观察组治疗后Ca2+高于对照组(P<0.05);治疗后两组PT均缩短,PTA、FIB、APTT均上升,且观察组治疗后PT较对照组缩短,观察组FIB高于对照组(P<0.05);观察组治疗后血清TNF-α、IL-6、CRP水平较对照组低(P<0.05);两组不良反应发生率比较差异无统计学意义(P>0.05);观察组入院8周后死亡率低于对照组(P<0.05)。结论采用PE+DPMAS治疗急性重症肝衰竭伴MODS患者可明显改善其肝功能,对凝血功能、电解质水平影响小,能较好下调机体炎症因子水平,改善患者临床结局,且安全可靠,值得在临床推广实践。     

Pediatric metabolic liver diseases: Evolving role of liver transplantation

Metabolic liver diseases (MLD) are the second most common indication for liver transplantation (LT) in children. This is based on the fact that the majority of enzymes involved in various metabolic pathways are present within the liver and LT can cure or at least control the disease manifestation. LT is also performed in metabolic disorders for end-stage liver disease, its sequelae including hepatocellular cancer. It is also performed for preventing metabolic crisis’, arresting progression of neurological dysfunction with a potential to reverse symptoms in some cases and for preventing damage to end organs like kidneys as in the case of primary hyperoxalosis and methyl malonic acidemia. Pathological findings in explant liver with patients with metabolic disease include unremarkable liver to steatosis, cholestasis, inflammation, variable amount of fibrosis, and cirrhosis. The outcome of LT in metabolic disorders is excellent except for patients with mitochondrial disorders where significant extrahepatic involvement leads to poor outcomes and hence considered a contraindication for LT. A major advantage of LT is that in the post-operative period most patients can discontinue the special formula which they were having prior to the transplant and this increases their well-being and improves growth parameters. Auxiliary partial orthotopic LT has been described for patients with noncirrhotic MLD where a segmental graft is implanted in an orthotopic position after partial resection of the native liver. The retained native liver can be the potential target for future gene therapy when it becomes a clinical reality.     

Orthotopic liver transplantation for giant liver haemangioma: A case report

In liver haemangiomas, the risk of complication rises with increasing size, and treatment can be obligatory. Here we present a case of a 46-year-old female who suffered from a giant haemangioma causing severe portal hypertension and vena cava compression, leading to therapy refractory ascites, hyponatremia and venostasis-associated thrombosis with pulmonary embolism. The patients did not experience tumour rupture or consumptive coagulopathy. Surgical resection was impossible because of steatosis of the non-affected liver. Orthotopic liver transplantation was identified as the only treatment option. The patient's renal function remained stable even though progressive morbidity and organ allocation were improbable according to the patient's lab model for end-stage liver disease(lab MELD) score. Therefore, non-standard exception status was approved by the European organ allocation network "Eurotransplant". The patient underwent successful orthotopic liver transplantation 16 mo after admission to our centre. Our case report indicates the underrepresentation of morbidity associated with refractory ascites in the lab MELD-based transplant allocation system, and it indicates the necessity of promptly applying for non-standard exception status to enable transplantation in patients with a severe clinical condition but low lab MELD score. Our case highlights the fact that liver transplantation should be considered early in patients with non-resectable, symptomatic benign liver tumours.     

妊娠期合并急性脂肪肝研究进展

妊娠期合并急性脂肪肝是严重的并发症之一,笔者总结近年相关研究文献,从发病机制、遗传因素、产科处理、内科处理、母婴预后等方面进行了阐述.     

多中心网状组织细胞增多症并发肝脏肿瘤

报告1例多中心网状组织细胞增多症并发肝脏肿瘤.患者男,61岁.面部、胸部和上肢红斑4个月,伴关节肿痛、低热1个月就诊.皮损组织病理检查示真皮内有单核、双核及多核胞质丰富的组织细胞,胞质呈毛玻璃状,细胞核呈圆形,有明显的核仁位于核中央.免疫组化染色结果示lyso( ),CD68( ),Vim( ),S-100(-),CDlα(-).患者有乙肝病史,甲胎蛋白(AFP)6820μg/L,腹部B超示肝脏右叶实质性占位,考虑恶性肿瘤可能.     

Two cases of pressure ulcer healing after liver transplantation in cirrhosis patients

We report two cases of pressure ulcers in liver cirrhosis patients. In case 1, a 64-year-old Japanese woman had suffered from liver cirrhosis caused by hepatitis C virus and developed a pressure ulcer on her sacral and coccygeal area due to long-term bedrest. After she received a living donor liver transplantation from her child, the ulcer healed synchronizing with improvement of serum cholinesterase and bilirubin. Likewise, her systemic condition also got much better after the transplantation. In case 2, a 53-year-old Japanese man with hepatitis B virus cirrhosis and hepatocellular carcinoma developed a pressure ulcer on his sacral area. Although he received a living donor liver transplantation from his brother, his general status and pressure ulcer were fluctuating in conformity with the variance of serum bilirubin. However, at 5 weeks after the transplantation, the ulcer gradually started improving, entrained to serum bilirubin decrease. From these findings, the condition of the pressure ulcer in liver cirrhosis patients synchronized with serum bilirubin as well as systemic condition, suggesting a possible influence of bilirubin for wound healing.     

Caval reconstruction techniques in orthotopic liver transplantation

There are several caval reconstruction techniques currently in use for orthotopic liver transplantation. These include caval replacement or the conventional technique, performed with or without venovenous bypass, piggyback technique with anastomosis with two or three hepatic veins with or without cavotomy and modifications of the piggyback technique including end-to-side and side-to-side cavocaval anastomosis. There are few randomized controlled trials comparing the use of these techniques and our knowledge of their comparability is based on a few multi- and many single-center retrospective and prospective reviews. Although there are advantages and disadvantages for each technique, it is advisable that the surgeon perform the technique with which they have the most the experience and at which they are the most skilled as excellent outcomes can be obtained with any of the caval reconstruction options discussed.     

温肾软肝清化汤治疗肝硬化男性性功能减退综合征临床效果观察

目的：观察并分析温肾软肝清化汤治疗肝硬化男性性功能减退综合征的临床效果，以提高肝硬化男性性功能减退综合征的临床治愈率。方法：选择医院2011年1月至2012年1月收治的120例肝硬化男性性功能减退综合征患者为研究对象，采用数字随机方法将其随机分成观察组和对照组，每组60例。观察组患者采用温肾软肝清化汤治疗，对照组患者口服安络化纤丸治疗，比较两组患者治疗结束后性功能减退、纳差、乏力以及不适症状的变化情况，并对治疗后患者的白蛋白（ALB）、总胆红素（TBiL）、血清睾酮（T）值的水平、门冬氨酸氨基转移酶（AST）水平进行测定。结果：观察组患者的总有效率为85.0％，对照组患者的总有效率为58.3％，两组数据差异具有统计学意义（P＜0.05）；两组患者治疗后，TBiL、ALB、AST和T值水平均得到了明显改善；且观察组患者的各项指标水平改善程度明显优于对照组，数据差异显著，具有统计学意义（P＜0.05）。结论：温肾软肝清化汤治疗肝硬化男性性功能减退综合征的临床疗效显著，值得推广。     

Cutaneous lesions as the presenting sign of acute graft-versus-host disease following liver transplantation

Acute graft-versus-host disease (GVHD) is a frequent complication of bone marrow transplantation but is only rarely observed after solid organ transplantation. We describe a 68-year-old man who developed a maculopapular eruption 7 days following orthotopic liver transplantation for cirrhosis with malignant transformation due to haemochromatosis. At day 20, the patient complained of nausea, vomiting, diarrhoea and fever. Skin biopsy revealed a lymphocytic infiltrate at the dermoepidermal interface, vacuolization of basal cells and epidermal dyskeratosis. Immunohistochemistry showed HLA-DR and intercellular adhesion molecule-1 expression of lesional keratinocytes. HLA-typing of peripheral blood lymphocytes demonstrated circulating lymphocytes of donor origin. Endoscopy revealed extensive erosions of the oesophagus, stomach and duodenum that on histology disclosed multifocal loss of crypts, lymphocytic infiltrates and epithelial cell death. A diagnosis of acute GVHD was made, and high-dose immunosuppressive therapy with azathioprine and methylprednisolone was instituted. The skin and gastrointestinal symptoms subsided within 4 weeks, but the patient died from severe infectious complications 105 days after transplantation. We conclude that acute GVHD is a rare but potentially fatal complication of liver transplantation. Skin lesions are an early sign of acute GVHD and thus represent an important tool for early diagnosis.     

The value of amino-terminal propeptide of type III procollagen in routine screening for methotrexate-induced liver fibrosis: a 10-year follow-up

BACKGROUND: Methotrexate (MTX) -induced liver damage is an important complication in patients treated with this drug for skin disease. Reliable non-invasive monitoring tests would have considerable importance. OBJECTIVES: This retrospective study was designed in order to evaluate if serial normal serum levels of amino-terminal propeptide of type III procollagen (PIIINP) might indicate that no significant fibrosis is taking place in the liver, and thereby reduce the need for repeated liver biopsies in psoriatic patients treated with MTX. METHODS: The clinical records of 70 patients with psoriasis, who in the years 1989/90 were on MTX and had both a liver biopsy without fibrosis and a normal PIIINP, were examined and followed until the patient stopped taking the drug. The follow-up time was from 1 to 11 years. RESULTS: A total of 189 liver biopsies and 329 analyses of PIIINP were recorded. Twenty-one patients had only one and no further biopsies, but their data included at least two to three PIIINP samples obtained within a year around the time of the biopsy, and at least two were taken either prior to or at the time of the biopsy. The remaining patients had from two to seven liver biopsies each and a total of 267 analyses of PIIINP. In the study period only four patients developed fibrosis of the liver as shown by liver biopsies, and all four of these patients developed elevated serum PIIINP levels. In addition two further patients, one of them with psoriatic arthritis, had elevated PIIINP, but normal liver biopsy. Thus no liver fibrosis was missed in the 63 patients with consistently normal PIIINP levels. CONCLUSIONS: Present data support the view that, as long as PIIINP is consistently normal in serial investigations, there is minimal risk of development of substantial liver fibrosis.     

Obesity and liver transplantation

The percentage of overweight and obese patients (OPs) waiting for a liver transplant continues to increase. Despite the significant advances occurred in bariatric medicine, obesity is still considered a relative contraindication to liver transplantation (LT). The main aim of this review is to appraise the literature on the outcomes of OPs undergoing LT, treatments that might reduce their weight before, during or after surgery, and discuss some of the controversies and limitations of the current knowledge with the intent of highlighting areas where future research is needed.     

Pre-and-post transplant considerations in patients with nonalcoholic fatty liver disease

Non-alcoholic fatty liver disease (NAFLD) is currently the third most common indication for liver transplantation in the United States. With the growing incidence of obesity, NAFLD is expected to become the most common indication for liver transplantation over the next few decades. As the number of patients who have undergone transplantation for NAFLD increases, unique challenges have emerged in the management and long-term outcomes in patients. Risk factors such as obesity, hypertension, diabetes, and hyperlipidemia continue to play an important role in the pathogenesis of the disease and its recurrence. Patients who undergo liver transplantation for NAFLD have similar long-term survival as patients who undergo liver transplantation for other indications. Research shows that post-transplantation recurrence of NAFLD is commonplace with some patients progressing to recurrent non-alcoholic steatohepatitis and cirrhosis. While treatment of comorbidities is important, there is no consensus on the management of modifiable risk factors or the role of pharmacotherapy and immunosuppression in patients who develop recurrent or de novo NAFLD post-transplant. This review provides an outline of NAFLD as indication for liver transplantation with a focus on the epidemiology, pathophysiology and risk factors associated with this disease. It also provides a brief review on the pre-transplant considerations and post-transplant factors including patient characteristics, role of obesity and metabolic syndrome, recurrence and de novo NAFLD, outcomes post-liver transplantation, choice of medications, and options for immunosuppression.     

Psoriasis:Biologic treatment and liver disease

Patients with moderate or severe psoriasis have a high prevalence of chronic liver disease. Chronic liver disease in these patients is related to metabolic syndrome, alcohol abuse or viral infections. Therefore,treatment of these patients is challenging. Classic systemic treatments may be contraindicated because of their immunosuppressive and hepatotoxic potential.First-line therapy in this setting is generally ultraviolet B phototherapy combined with topical treatment, but its feasibility and efficacy are sometimes limited. The therapeutic options are further restricted by concomitant psoriatic arthritis. Biologic treatments have shown to be effective in psoriasis and psoriatic arthritis, and they are largely devoid of liver toxicity. Anti-tumor necrosis factor-alpha(TNF-α) treatments have proven to be effective and safe in patients with chronic hepatitis C virus(HCV) infections and other non-infectious chronic liver disorders, including alcoholic and non-alcoholic liver diseases. However, in chronic hepatitis B virus(HBV), anti-TNF-α treatments carry a high risk of HBV reactivation. Anti-interleukin-12/23 treatments are also effective in patients with psoriasis, but data regarding their safety in chronic hepatitis infections are still limited. Safety reports in patients with psoriasis and chronic HCV infection are contradictory, and in chronic HBVevidence indicate a potential risk of viral reactivation. Moreover, concerns remain about the long-term safety of both TNF-α antagonists and ustekinumab. Non-viral liver diseases such as alcoholic and non-alcoholic liver diseases are more prevalent in patients with psoriasis than in the general population. TNF-α antagonists have also been prescribed in these patients. Although data are still scarce in this setting, results suggest a favorable profile in patients with psoriasis and non-alcoholic liver diseases. We review the literature regarding all these aspects.