Cholestatic liver disease is a common disease in infancy,and due to its complex etiology and different outcomes,conservative medical treatment and conventional surgery lack therapeutic effect.Liver transplantation has obvious advantages in the treatment of decompensated intrahepatic cholestatic liver disease and biliary atresia.This article summarizes the etiology,clinical manifestations,diagnosis,and treatment of infantile cholestatic liver disease,as well as related perspectives.

Objective To investigate the role of multi-disciplinary team (MDT) in the treatment of complex cholestatic liver injury after liver transplantation. Methods MDT consultation was conducted to clarify the causes and therapeutic strategies for one case of complex cholestatic liver injury after liver transplantation admitted to Liver Transplantation Center of the First Hospital of Jilin University on June 23, 2020. And the role of MDT in the treatment of complex cholestatic liver injury after liver transplantation was summarized. Results The patient presented with abnormal liver function after liver transplantation. The diagnosis of biliary stricture, rejection and biliary tract infection was confirmed successively. Endoscopic retrograde cholangiopancreatography (ERCP) stent internal and external double drainage, glucocorticoid shock and anti-infection therapy yielded low clinical efficacy. After MDT consultation, complex cholestatic liver injury after liver transplantation was confirmed. It was suggested to optimize the immunosuppressive regimen based on the exclusion of rejections by pathological examination, deliver targeted anti-infection interventions and prevent the potential risk of concomitant drug-induced liver injury. The patient was discharged after proper recovery. Conclusions The causes of complex cholestatic liver injury after liver transplantation are diverse, and the condition changes dynamically. MDT consultation are performed to deepen the understanding of this disease, strengthen the classification of diagnosis and treatment ideas and enhance the precision and efficacy of corresponding treatment.

BACKGROUND AND OBJECTIVEHuman achaete-scute homolog 1 (hASH1) gene plays a critical role in development of the central nervous system, automatic nervous system, adrenal medullary chromaffin cells, thyroid C cells and pulmonary neuroendocrine cells. The aim of this study is to determine hASH1 gene expression in the normal lung tissue and various types of lung tumors, to analyze whether its expression correlated with pulmonary neuroendocrine markers, and to explore the possibility of hASH1 as clinical pathological markers in the neuroendocrine tumors compared with previous neuroendocrine tumor markers.

METHODShASH1, Chromogranin A, Synaptophysin and CD56 expression were examined in lung tumor specimens (lung inflammatory pseudotumor, squamous cell carcinoma, adenocarcinomas, large cell carcinoma, typical carcinoids, atypical carcinoids, large cell neuroendocrine carcinomas and small cell lung carcinoma and corresponding normal lung specimens) using immunohistochemistry (S-P method). Western blot and reverse transcription polymerase chain reaction (RT-PCR) assay were applied to detect the expressions of hASH1 protein and mRNA in lung cancer tissues.

RESULTShASH1 expression was positive in 2/16 (12.5%) typical carcinoids, 15/20 (75%) atypical carcinoids, 6/10 (60%) large cell neuroendocrine carcinomas and 31/40 (77.5%) small cell lung carcinoma, respectively, but not in any normal lung tissue (0/10), lung inflammatory pseudotumor (0/49), squamous cell carcinoma (0/30), adenocarcinomas (0/30) or large cell carcinoma (0/20). There was a significant difference in hASH1 expression between typical carcinoids and atypical carcinoids (P < 0.01), but not in large cell neuroendocrine carcinomas and small cell lung carcinoma (P > 0.05). hASH1 expression highly closely correlated with Chromogranin A, Synaptophysin and CD56 expression (P < 0.05).

CONCLUSIONhASH1 is a new kind of highly specific markers of pulmonary neuroendocrine tumours, and may be applied to clinical pathology diagnosis of the pulmonary neuroendocrine tumors.

Adenocarcinoma ; genetics ; metabolism ; pathology ; Basic Helix-Loop-Helix Transcription Factors ; genetics ; metabolism ; Carcinoma, Large Cell ; genetics ; metabolism ; pathology ; Carcinoma, Neuroendocrine ; genetics ; metabolism ; pathology ; Carcinoma, Squamous Cell ; genetics ; metabolism ; pathology ; Gene Expression Regulation, Neoplastic ; genetics ; physiology ; Humans ; Immunohistochemistry ; Lung Neoplasms ; genetics ; metabolism ; pathology ; Neuroendocrine Tumors ; genetics ; metabolism ; pathology ; Reverse Transcriptase Polymerase Chain Reaction ; Small Cell Lung Carcinoma ; genetics ; metabolism ; pathology

Objective:To investigate the influencing factors for anastomotic biliary stric-ture after liver transplantation.Methods:The retrospective case-control study was conducted. The clinical data of 428 recipients who underwent allogeneic orthotopic liver transplantation in the First Hospital of Jilin University from September 2014 to August 2021 were collected. There were 324 males and 104 females, aged (52±10)years. Observation indicators: (1) surgical conditions of recipients; (2) occurrence of anastomotic biliary stricture after liver transplantation and its treat-ment; (3) analysis of influencing factors for anastomotic biliary stricture after liver transplantation. Follow-up was conducted using outpatient examination to detect occurrence of anastomotic biliary stricture and treatment up to August 30, 2021. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was analyzed using the t test. Measure-ment data with skewed distribution were represented as M( Q1, Q3) or M(range), and comparison between groups was analyzed using the Mann-Whitney U test. Count data were represented as absolute numbers, and the chi-square test was used for comparison between groups. Logistic regression model was used for multivariate analysis. Results:(1) Surgical conditions of recipients: the operation time of 428 recipients was 465(420,520)minutes, the cold ischemia time was 368(320,450)minutes, and the volume of intraoperative blood loss was 2 500(1 500,4 000)mL. Of the 428 recipients, 142 cases were performed continuous biliary posterior wall anastomosis + interrup-ted anterior wall anastomosis by polygluconate sutures, 286 cases were anastomosed with polypro-pylene sutures, including 169 cases undergoing continuous biliary posterior wall anastomosis combined with interrupted anterior wall anastomosis, 73 cases undergoing completely interrupted biliary anterior and posterior wall anastomosis, and 44 cases undergoing completely continuous biliary anterior and posterior wall anastomosis. None of the 428 recipients had indwelling T tubes. (2) Occurrence of anastomotic biliary stricture after liver transplantation and its treatment:all the 428 recipients were followed up for 3 to 72 months, with a median follow-up time of 28 months. During the follow-up, 50 patients developed anastomotic biliary stricture, of which 41 patients were treated with endoscopic retrograde cholangiopancreatography, 8 patients were treated with percutaneous transhepatic cholangial drainage, and 1 patient was treated with surgery, showing no recurrence. (3)Analysis of influencing factors for anastomotic biliary stricture after liver transplanta-tion: results of univariate analysis showed that anastomosis method and donor liver cold ischemia time were related factors for postoperative anastomotic biliary stricture of recipients undergoing allogeneic orthotopic liver transplantation ( χ2=15.74, Z=-2.04, P<0.05). Results of multivariate analysis showed that completely interrupted biliary anterior and posterior wall anastomosis and donor liver cold ischemia time were independent influencing factors for postoperative anastomotic biliary stricture of recipients undergoing allogeneic orthotopic liver transplantation ( odds ratio=0.25, 1.00, 95% confidence interval as 0.08-0.85, 1.00-1.01, P<0.05). Conclusions:Suture type is not an influencing factor for postoperative anastomotic biliary stricture of recipients undergoing allogeneic orthotopic liver transplantation. Completely interrupted biliary anterior and posterior wall anastomosis and donor liver cold ischemia time were independent influencing factors.

Objective:To explore the feasibility and advantages of planned initiation of extracorporeal membrane oxygenation(ECMO)prior to liver transplantation.Methods:From November 2017 to July 2021, clinical data were retrospectively reviewed for 3 liver transplantation recipients assisted by ECMO.There were such preoperative symptoms of right ventricular dysfunction as fatigue, chest tightness and palpitations.In the first case, right heart catheterization was not performed due to patient refusal; another two patients were screened by transthoracic Doppler echocardiography(TDE)and diagnosed through right heart catheterization as portopulmonary hypertension(POPH)and pulmonary hypertension.Results:Three recipients with pulmonary hypertension received catheterization in right femoral artery and vein.After freeing of diseased liver and before blocking inferior vena cava, V-A ECMO support was performed.The dose of heparin was adjusted according to activated clotting time(ACT)and perioperative vital signs remained stable.They were ventilated for 54, 12 and 62 hours and supported by ECMO for 27, 61 and 14 hours.All were smoothly discharged.During a mean follow-up period of 26(9-22)months, liver functions were normal.Conclusions:Patients with end-stage liver disease with pulmonary hypertension should undergo routine TDE examinations during waiting period before liver transplantation.Those with pulmonary hypertension should undergo further right heart catheterization to confirm the diagnosis and severity of the disease.Planned application of ECMO through multidisciplinary consultations can expand surgical indications for liver transplantation, maintain intraoperative hemodynamic stability and facilitate smooth liver transplantation and postoperative patient recovery.