促进糖尿病患者足溃疡愈合的干预措施指南(2023年更新版)——《国际糖尿病足工作组:糖尿病相关的足病预防与管理指南(2023)》的一部分

创面处理的原则,包括清创、创面准备和使用涉及改变创面生理以促进愈合的新技术,在试图治愈慢性糖尿病相关的足溃疡时是至关重要的。此外,糖尿病相关的足溃疡的发病率和治疗成本不断上升,这就需要结合作为金标准的多学科治疗已有共识,运用高质量的疗效和成本效益证据来支持和加强慢性糖尿病相关的足溃疡创面愈合的干预措施。本指南是2023年国际糖尿病足工作组(IWGDF)针对促进糖尿病患者足溃疡愈合的干预措施的循证指南。它是对2019年IWGDF指南的更新。我们遵循推荐、评估、发展、评价分级(GRADE)方法,以患者-干预-对照-结果(PICO)形式设计临床问题从而得出重要结果,进行系统综述,制定评判表汇总,并为每个问题撰写推荐和理由。每条推荐的形式都是基于系统综述中发现的证据,并使用GRADE评判汇总项目,包括理想和不理想的效果、证据的可信度、对患者的价值、所需的资源、成本效益、公平性、可行性和可接受性;我们制定的推荐由作者同意并由独立专家和利益相关者审查。根据系统综述和从证据到决策过程的结果,我们提出29条推荐。我们对使用干预措施改善糖尿病患者足溃疡的愈合提出了一些有条件的支持性推荐。这些推荐包括蔗糖八硫酸盐敷料的使用,对手术后的创面使用负压疗法,使用胎盘衍生产品,使用自体白细胞、血小板、纤维蛋白贴剂,使用局部氧疗,以及使用高压氧,但我们强调在任何情况下这些推荐应在单独应用最佳标准治疗无法治愈伤口且有资源可用于干预的情况下使用。这些关于创面愈合的推荐应该有助于提高糖尿病伴足溃疡患者的疗效,我们希望这些推荐能得到广泛的实施。然而,尽管作为推荐基础的许多证据的准确性正在提高,但总体上仍然很差,我们鼓励在这一领域进行更多、质量更好的试验,包括那些有关卫生经济学分析的试验。

What,why and how to monitor blood glucose in critically ill patients

Critically ill patients are prone to high glycemic variations irrespective of their diabetes status.This mandates frequent blood glucose(BG)monitoring and regulation of insulin therapy.Even though the most commonly employed capillary BG monitoring is convenient and rapid,it is inaccurate and prone to high bias,overestimating BG levels in critically ill patients.The targets for BG levels have also varied in the past few years ranging from tight glucose control to a more liberal approach.Each of these has its own fallacies,while tight control increases risk of hypoglycemia,liberal BG targets make the patients prone to hyperglycemia.Moreover,the recent evidence suggests that BG indices,such as glycemic variability and time in target range,may also affect patient outcomes.In this review,we highlight the nuances associated with BG monitoring,including the various indices required to be monitored,BG targets and recent advances in BG monitoring in critically ill patients.

Prehospital transdermal glyceryl trinitrate for ultra- acute ischaemic stroke: data from the RIGHT- 2 randomised sham- controlled ambulance trial

Background The effect of transdermal glyceryl trinitrate(GTN,a nitrovasodilator)on clinical outcome when administered before hospital admission in suspected stroke patients is unclear.Here,we assess the safety and efficacy of GTN in the prespecified subgroup of patients who had an ischaemic stroke within the Rapid Intervention with Glyceryl trinitrate in Hypertensive stroke Trial-2(RIGHT-2).Methods RIGHT-2 was an ambulance-based multicentre sham-controlled blinded-endpoint study with patients randomised within 4hours of onset.The primary outcome was a shift in scores on the modified Rankin scale(mRS)at day 90.Secondary outcomes included death;a global analysis(Wei-Lachin test)containing Barthel Index,EuroQol-5D,mRS,telephone interview for cognitive status-modified and Zung depression scale;and neuroimaging-determined‘brain frailty’markers.Data were reported as n(%),mean(SD),median[IQR],adjusted common OR(acOR),mean difference or Mann-Whitney difference(MWD)with 95%CI.Results 597 of 1149(52%)patients had a final diagnosis of ischaemic stroke;age 75(12)years,premorbid mRS>2107(18%),Glasgow Coma Scale 14(2)and time from onset to randomisation 67[45,108]min.Neuroimaging‘brain frailty’was common:median score 2[2,3](range 0–3).At day 90,GTN did not influence the primary outcome(acOR for increased disability 1.15,95%CI 0.85 to 1.54),death or global analysis(MWD 0.00,95%CI-0.10 to 0.09).In subgroup analyses,there were non-significant interactions suggesting GTN may be associated with more death and dependency in participants randomised within 1hour of symptom onset and in those with more severe stroke.Conclusions In patients who had an ischaemic stroke,ultra-acute administration of transdermal GTN in the ambulance did not improve clinical outcomes in a population with more clinical and radiological frailty than seen in previous in-hospital trials.WHAT IS ALREADY KNOWN ON THIS TOPIC⇒Transdermal glyceryl trinitrate(GTN)was associat-ed with less death and dependency in those with acute stroke treated within 6hours of stroke onset in a systematic review and individual patient data meta-analysis from two randomised controlled tri-als.The Rapid Intervention with Glyceryl trinitrate in Hypertensive stroke Trial-2(RIGHT-2)assessed the effect of GTN given prehospital in patients with pre-sumed stroke within 4hours of onset.This subgroup analysis details the effect of GTN in those with clini-cally diagnosed ischaemic stroke.WHAT THIS STUDY ADDS⇒Transdermal GTN did not influence clinical or radio-logical outcomes despite lowering blood pressure compared with sham.GTN may be associated with more death and dependency in those randomised within 1hour of symptom onset and in those with more severe stroke,but these interactions were non-significant.The population recruited in RIGHT-2 was more dependent and frailer(both clinically and radiologically)than in prior trials of transdermal GTN within 6hours of stroke onset performed in hospital,and may account for the differences in results.HOW THIS STUDY MIGHT AFFECT RESEARCH,PRACTICE OR POLICY⇒Transdermal GTN should not be administered to pa-tients with presumed stroke prehospital outside of a trial environment.Clinical and radiological frailty should be taken into consideration in the design and interpretation of future ultra-acute stroke trials.

慢性肾脏病患者铁剂应用的争议：来自改善全球肾脏病预后组织(KDIGO)会议的结论

慢性肾脏病和终末期肾病患者常需补铁治疗。但近年来铁剂的使用逐年增加,带来了铁超载、氧化应激和超敏反应等新问题,并可能促进感染的发生,其使用的安全性受到关注。为此,改善全球肾脏病预后组织(KDIGO)召开专家组会议,全面评估铁剂使用的利和弊,并且提供合理使用方案以减轻急性反应及其他毒副作用。

Changing face of hepatic encephalopathy:Role of inflammation and oxidative stress

The face of hepatic encephalopathy(HE) is changing.This review explores how this neurocognitive disorder,which is associated with both acute and chronic liver injury,has grown to become a dynamic syndrome that spans a spectrum of neuropsychological impairment,from normal performance to coma.The central role of ammonia in the pathogenesis of HE remains incontrovertible.However,over the past 10 years,the HE community has begun to characterise the key roles of inflammation,infection,and oxidative/nitrosative stress in modulating the pathophysiological effects of ammonia on the astrocyte.This review explores the current thoughts and evidence base in this area and discusses the potential role of existing and novel therapies that might abrogate the oxidative and nitrosative stresses inflicted on the brain in patients with,or at risk of developing,HE.

Ruptured hepatocellular carcinoma following chemoembolization:a western experience

BACKGROUND: Transcatheter arterial chemoembolization (TACE) is a recommended first line therapy for unresectable hepatocellular carcinoma (HCC). Serious complications such as neutropenic sepsis and hepatic decompensation are well known, but rupture of HCC following TACE is a rare and potentially fatal complication. The aim of this study was to identify the incidence of ruptured HCC following TACE and the associated risk factors. METHODS: A retrospective analysis was performed using our liver database with 'chemoembolization', 'ruptured HCC' covering the patients who received chemoembolization from January 1995 to December 2005. There were no exclusions. RESULTS: A total of 294 patients received chemoemboliza- tion in 530 sessions during the 10-year period. Of these, 2 ruptured following treatment (incidence 0.68%). The mean age was 65 years and the interval between the treatment and rupture was 2 and 24 days. The common factors were male sex, large tumor size (range 11-13 cm), and exophytic tumor growth. One patient died 2 days after rupture with hepatic decompensation while the second is alive after a 6-month follow up without tumor recurrence. CONCLUSIONS: Ruptured HCC following TACE is a rare but serious complication. Large tumor size, male sex, and exophytic growth of tumor may be predisposing factors for rupture.

Aetiopathogenesis of autoimmune hepatitis

The histological hallmark of autoimmune hepatitis(AIH) is a dense portal mononuclear cell infiltrate that invades the surrounding parenchyma and comprises T and B lymphocytes,macrophages,and plasma cells.An unknown but powerful stimulus must be promoting the formation of this massive inflammatory cellular reaction that is likely to initiate and perpetuate liver damage.An autoimmune attack can follow different pathways to inflict damage on hepatocytes.Liver damage is likely to be orchestrated by CD4+ T lymphocytes recognizing an autoantigenic liver peptide.To trigger an autoimmune response,the peptide must be embraced by an HLA class Ⅱ molecule and presented to na?ve CD4+ T helper(Th0) cells by professional antigen presenting cells,with the co-stimulation of ligand-ligand fostering interaction between the two cells.Th0 cells become activated,differentiate into functional phenotypes according to the cytokines prevailing in the microenvironment and the nature of the antigen,and initiate a cascade of immune reactions determined by the cytokines produced by the activated T cells.Th1 cells,arising in the presence of the macrophage-derived interleukin(IL) -12,secrete mainly IL-2 and interferon-gamma(IFN-g),which activate macrophages,enhance expression of HLA classⅠ(increasing liver cell vulnerability to a CD8+ T cell cytotoxic attack),and induce expression of HLA class Ⅱ molecules on hepatocytes.Th2 cells,which differentiate from Th0 if the microenvironment is rich in IL-4,produce mainly IL-4,IL-10,and IL-13 which favour autoantibody production by B lymphocytes.Physiologically,Th1 and Th2 antagonize each other.Th17 cells,a recently described population,arise in the presence of transforming growth factor beta(TGF-β) and IL-6 and appear to have an important effector role in inflammation and autoimmunity.Theprocess of autoantigen recognition is strictly controlled by regulatory mechanisms,such as those exerted by CD4+CD25+ regulatory T cells,which derive from Th0 in the presence of TGF-β,but in the absence of IL-6.If regulatory mechanisms fail,the autoimmune attack is perpetuated.Over the past three decades different aspects of the above pathogenic scenario have been investigated.In particular,a defect in immunoregulation affecting CD4+CD25+ regulatory T cells(T-regs) has been demonstrated in AIH,particularly at diagnosis or during relapse.Advances in the study of autoreactive T cells have occurred mostly in AIH type 2,since the knowledge that CYP2D6 is the main autoantigen has enabled the characterization of both CD4 and CD8 T cells targeting this cytochrome.CD4 T cells from patients with type 2 AIH positive for the predisposing HLA allele DRB10701 recognize seven regions of CYP2D6,five of which are also recognized by CD8 T cells.High numbers of IFN-g producing CD4 T cells and CD8 T cells are associated with biochemical evidence of liver damage,suggesting a combined cellular immune attack.

Autoimmune liver serology:Current diagnostic and clinical challenges

Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseas-es(AiLD),namely autoimmune hepatitis types 1 and 2(AIH-1 and 2),primary biliary cirrhosis(PBC),and the sclerosing cholangitis variants in adults and children.AIH-1 is specified by anti-nuclear antibody(ANA) and smooth muscle antibody(SMA).AIH-2 is specified by antibody to liver kidney microsomal antigen type-1(anti-LKM1) and anti-liver cytosol type 1(anti-LC1).SMA,ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation.PBC is specified by antimitochondrial antibodies(AMA) react-ing with enzymes of the 2-oxo-acid dehydrogenase complexes(chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly react-ing with nuclear pore gp210 and nuclear body sp100.Sclerosing cholangitis presents as at least two variants,first the classical primary sclerosing cholangitis(PSC) mostly affecting adult men wherein the only(and non-specific) reactivity is an atypical perinuclear antineutro-phil cytoplasmic antibody(p-ANCA),also termed peri-nuclear anti-neutrophil nuclear antibodies(p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis(ASC) with serological features resembling those of type 1 AIH.Liver diagnostic serol-ogy is a fast-expanding area of investigation as new purified and recombinant autoantigens,and automatedtechnologies such as ELISAs and bead assays,become available to complement(or even compete with) tradi-tional immunofluorescence procedures.We survey for the first time global trends in quality assurance impact-ing as it does on(1) manufacturers/purveyors of kits and reagents,(2) diagnostic service laboratories that fulfill clinicians' requirements,and(3) the end-user,the physician providing patient care,who must properly interpret test results in the overall clinical context.

Cancer of the uncinate process of the pancreas:surgical anatomy and clinicopathological features

BACKGROUND:The clinicopathological features of uncinate process pancreatic cancer(UPPC) are poorly described.Furthermore the anatomy of the uncinate process and its division during surgery are central to pancreaticoduodenectomy for UPPC.We set out to describe the embryology and anatomy of the uncinate process and the clinicopathological features of UPPC.DATA SOURCES:All published case series of UPPC were reviewed and included in this review.RESULTS:The true incidence of UPPC is difficult to quantify,with the reported incidence ranging from 2.5% to 10.7% of pancreatic cancer.There are 5 published series of UPPC including 117 patients,72 males and 45 females,aged from 45-53 years to 61-84 years.The median survival was 5 or 5.5 months in 3 of the series,12.1 months in another based only on potentially resectable lesions and 17 months in another based only on resected cases.CONCLUSIONS:The number of reported series of UPPC is limited,with vague symptoms as the predominant presenting features of the disease.The prognosis is poor with synchronous venous resection demonstrating a survival advantage.

Helicobacter pylori and autoimmune disease:Cause or bystander

Helicobacter pylori(H.pylori)is the main cause of chronic gastritis and a major risk factor for gastric cancer.This pathogen has also been considered a potential trigger of gastric autoimmunity,and in particular of autoimmune gastritis.However,a considerable number of reports have attempted to link H.pylori infection with the development of extra-gastrointestinal autoimmune disorders,affecting organs not immediately relevant to the stomach.This review discusses the current evidence in support or against the role of H.pylori as a potential trigger of autoimmune rheumatic and skin diseases,as well as organ specific autoimmune diseases.We discuss epidemiological,serological,immunological and experimental evidence associating this pathogen with autoimmune diseases.Although over one hundred autoimmune diseases have been investigated in relation to H.pylori,we discuss a select number of papers with a larger literature base,and include Sj grens syndrome,rheumatoid arthritis,systemic lupus erythematosus,vasculitides,autoimmune skin conditions,idiopathic thrombocytopenic purpura,autoimmune thyroid disease,multiple sclerosis,neuromyelitis optica and autoimmune liver diseases.Specific mention is given to those studies reporting an association of anti-H.pylori antibodies with the presence of autoimmune disease-specific clinical parameters,as well as those failing to find such associations.We also provide helpful hints for future research.

Diagnosis in bile acid-CoA:Amino acid N-acyltransferase deficiency

Cholate-CoA ligase(CCL) and bile acid-CoA:amino acid N-acyltransferase(BAAT) sequentially mediate bile-acid amidation.Defects can cause intrahepatic cholestasis.Distinction has required gene sequencing.We assessed potential clinical utility of immunostaining of liver for CCL and BAAT.Using commercially available antibodies against BAAT and CCL,we immunostained liver from an infant with jaundice,deficiency of amidated bile acids,and transcription-terminating mutation in BAAT.CCL was normally expressed.BAAT expression was not detected.Immunostaining may facilitate diagnosis in bileacid amidation defects.

Risk-adjustment in hepatobiliarypancreatic surgery

AIM: The present study evaluates the performance of the POSSUM, the American Society of Anesthetists (ASA),APACHE and Childs classification in predicting mortality and morbidity in hepatopancreaticobiliary (HPB) surgery.We describe especially the limitations and advantages of risk in stratifying the patients.METHODS: We investigated 177 randomly chosen patients undergoing eledive complex HPB surgery in a single institution with a total of 71 pre-operative and intra-operative risk factors.Primary endpoint was in-hospital mortality and morbidity.Ordered logistic regression analysis was used to identify individual predictors of operative morbidity and mortality.RESULTS: The operative mortality in the series was 3.95% .This compared well with the p-POSSUM and APACHE predicted mortality of 4.31% and 4.29% respectively. Postoperative complications amounted to 45% with 24 (13.6%)patients having a major adverse event. On multivariate analysis the pre-operative POSSUM physiological score (OR = 1.18,P = 0.009) was superior in predicting complications compared to the ASA (P = 0.108), APACHE (P = 0.117)or Childs classification (P = 0.136). In addition, serum sodium, creatinine, international normalized ratio (INR),pulse rate, and intra-operative blood loss were independent risk factors. A combination of the POSSUM variables and INR offered the optimal combination of risk factors for risk prognostication in HPB surgery.CONCLUSION: Morbidity for elective HPB surgery can be accurately predicted and applied in everyday surgical practice as an adjunct in the process of informed consent and for effective allocation of resources for intensive and high-dependency care facilities.

Novel ABCB11 mutations in a Thai infant with progressive familial intrahepatic cholestasis

Progressive familial intrahepatic cholestasis(PFIC) type 2 is caused by mutations in ABCB11,which encodes bile salt export pump(BSEP).We report a Thai female infant who presented with progressive cholestatic jaundice since 1 mo of age,with normal serumγ-glutamyltransferase.Immunohistochemical staining of the liver did not demonstrate BSEP along the canaliculi,while multidrug resistance protein 3 was expressed adequately.Novel mutations in ABCB11,a four-nucleotide deletion in exon 3,c.90_93delGAAA,and a single-nucleotide insertion in exon 5,c.249_250insT, were identified,with confirmation in her parents. These mutations were predicted to lead to synthesis of truncated forms of BSEP.Immunostaining and mutation analysis thus established the diagnosis of PFIC type 2.

Autoimmune paediatric liver disease

Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis(AIH),autoimmune sclerosing cholangitis(ASC),and de novo AIH after liver transplantation.AIH is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody(SMA/ANA,type 1) or liver kidney microsomal antibody(LKM1,type 2).There is a female predominance in both.LKM1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity,presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both groups.The most common type of paediatric sclerosing cholangitis is ASC.The clinical,biochemical,immunological,and histological presentation of ASC is often indistinguishable from that of AIH type 1.In both,there are high IgG,non-organ specific autoantibodies,and interface hepatitis.Diagnosis is made by cholangiography.Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters,and decreased inflammatory activity on follow up liver biopsies.However,the cholangiopathy can progress.There may be evolution from AIH to ASC over the years,despite treatment.De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies,hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and collapse.Like classical AIH,it responds to treatment with prednisolone and azathioprine.De novo AIH postliver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection.Whether this condition is a distinct entity or a form of atypical rejection in individuals susceptible to the development of autoimmune phenomena is unclear.Whatever its etiology,the recognition of this potentially life-threatening syndrome is important since its management differs from that of standard anti-rejection therapy.

Clinical features and treatment of sump syndrome following hepaticojejunostomy

BACKGROUND:Cholangitis after Roux-en-Y hepaticojejunostomy is usually caused by anastomotic stricture.A small number of cases present without evidence of obstruction and are ascribed to reflux of gastro-intestinal content into the biliary tree above the anastomosis (sump syndrome).Despite prophylactic rotating antibiotic therapy,the cholangitic episode may be severe and life-threatening.METHODS:From 2001 to 2006,six patients who had undergone an end-to-side hepaticojejunostomy presented to our institution with recurrent episodes of biliary sepsis.Anastomotic stricture was excluded by liver MRI/MRCP and percutaneous transhepatic cholangiogram (PTC).Barium meal showed reflux of contrast into the biliary tree in all patients.Three patients had a short jejunal Roux limb (less than 50 cm) on pre-operative imaging.RESULTS:Five patients underwent surgery and two of them had two operations.One patient had a Tsuchida antireflux valve and subsequently underwent lengthening of the Roux loop.Three patients had lengthening of the Roux loop;one underwent re-do hepaticojejunostomy and one had concomitant revision of the hepaticojejunostomy and lengthening of the Roux loop.The latter underwent further lengthening of the Roux loop.Three patients are cholangitis-free 6,36 and 60 months after surgery;two still experience mild episodes of cholangitis.CONCLUSIONS:An adequate length of the Roux loop is important to prevent reflux.However,Roux loop lengthening to 70 cm or more does not always resolve the problem and cholangitis,although generally less frequent and severe,may recur despite appropriate reconstructive or antireflux surgery.In these cases,life-long rotating antibiotics is the only available measure.

What we have learned and what to expect from capsule endoscopy

Capsule endoscopy was conceived by Gabriel Iddan and Paul Swain independently two decades ago. These applications include but are not limited to Crohn's disease of the small bowel, occult gastrointestinal bleeding, non steroidal anti inflammatory drug induced smallbowel disease, carcinoid tumors of the small bowel,gastro intestinal stromal tumors of the small bowel andother disease affecting the small bowel. Capsule endoscopy has been compared to traditional small bowelseries, computerized tomography studies and pushenteroscopy. The diagnostic yield of capsule endoscopyhas consistently been superior in the diagnosis of smallbowel disease compared to the competing methods(small bowel series, computerized tomography, pushenteroscopy) of diagnosis. For this reason capsule en-doscopy has enjoyed a meteoric success. Image qualityhas been improved with increased number of pixels,automatic light exposure adaptation and wider angle ofview. Further applications of capsule endoscopy of other areas of the digestive tract are being explored. Theincreased transmission rate of images per second hasmade capsule endoscopy of the esophagus a realisticpossibility. Technological advances that include a double imager capsule with a nearly panoramic view of the colon and a variable frame rate adjusted to the movement of the capsule in the colon have made capsuleendoscopy of the colon feasible. The diagnostic ratefor the identification of patients with polyps equal to orlarger than 6 mm is high. Future advances in technology and biotechnology will lead to further progress.Capsule endoscopy is following the successful moderntrend in medicine that replaces invasive tests with lessinvasive methodology.

Novel en-bloc resection of locally advanced hilar cholangiocarcinoma: the Rex recess approach

Loco-regional recurrence after potentially curative resection remains a problem in hilar cholangiocarcinoma. Hilar dissection risks local spillage of tumor cells leading to suboptimal disease free survival. We have developed a new technique of radical resection for hilar cholangiocarcinoma based on the distinctive anatomy of the Rex recess of the liver, which has been assessed in two patients with locally advanced hilar cholangiocarcinoma. This technique included a right hepatectomy with en-bloc resection of the hepatoduodenal ligament and portal venous reconstruction to the left portal vein at the Rex recess. Both patients had R0 resection and have been disease-free for 26 and 38 months, respectively.

Liver transplantation for acute intermittent porphyria:a viable treatment?

BACKGROUND:Acute intermittent porphyria (AIP) is the most common hepatic porphyria.Its clinical presentation includes severe disabling and life-threatening neurovisceral symptoms and acute psychiatric symptoms.These symptoms result from the overproduction and accumulation of porphyrin precursors,5-aminoleuvulinic acid (ALA) and porphobilinogen (PBG).The effect of medical treatment is transient and is not effective once irreversible neurological damage has occurred.Liver transplantation (LT) replaces hepatic enzymes and can restore normal excretion of ALA and PBG and prevent acute attacks.METHOD:Two cases of LT for AIP were identified retrospectively from a prospectively maintained LT database.RESULT:LT was successful with resolution of AIP in two patients who suffered from repeated acute attacks.CONCLUSION:LT can correct the underlying metabolic abnormality in AIP and improves quality of life significantly.

Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma

Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management.Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1（complete） shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.