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Autosomal recessive congenital ichthyosis caused by CERS3 mutations is extremely rare in clinical practice. We recently identified a family of autosomal recessive congenital ichthyosis and performed multigene exome sequencing for hereditary skin diseases to identify causative genes. Mutation analysis revealed compound heterozygous mutations of c.746A>G(from the mother) and exon12 deletion(from the father)in CERS3 were detected in the proband, which were verified by Sanger sequencing and co-segregated with the ichthyosis phenotype in the proband and her parents. These mutations were both reported for the first time. For the treatment, the proband received an oral acitretin capsules of 20 mg once daily. After 3-month follow up, the patient's lesion improved significantly.
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Objective:To explore the risk factors for the occurrence of thrombocytopenia (TCP) within 2 weeks after pediatric liver transplantation (LT) and examine the relationship between the occurrence of TCP and prognosis.Methods:From January 2021 to November 2021, clinical data were retrospectively reviewed for 162 pediatric LT recipients aged under 4 years at Organ Transplantation Center of Tianjin First Central Hospital.Based upon the lowest value of platelet count at Week 2 post-operation, they were assigned into two groups of TCP (n=90) and non-TCP (n=72). General preoperative profiles, intraoperative findings, postoperative complications, types of commonly used antibiotics, anticoagulant dosing and prognosis of two groups were compared.Univariate and multivariate analyses were utilized for examining the independent risk factors for TCP.Receiver operating characteristic (ROC) curve was plotted for examining the cut-off value of independent risk factors for diagnosing TCP.Results:Among them, 90 (55.56%) developed TCP within 2 weeks post-operation and 25(15.43%) developed TCP at Day 1 post-operation.The median preoperative platelet count was 178×10 9/L and the lowest value was 65×10 9/L at Day 3(1-4) post-operation with a declining rate of 63.5% and platelet count of recipient normalized at Day 6(4-7.25) post-operation.The results of univariate analysis showed statistically significant inter-group differences in operative duration[(574.43±80.53)min vs.(526.75±72.42)min], intraoperative blood loss[400(300, 550)ml vs.320(300, 400)ml], red blood cell transfusion[2(2, 3)U vs.2(1.5, 2.0)U], preoperative platelet count[178.5(141.75, 242.5)×10 9/L vs.257 (209.75, 357)×10 9/L], postoperative infection rate[27.8%(25/90)vs.13.9%(10/72)] and dosing rates of piperacillin sodium and tazobactam sodium[8.9%(8/90)vs.25.0%(18/72)] ( P<0.05). Multivariate Logistic regression analysis revealed statistically significant inter-group differences in operative duration( P=0.008), red blood cell transfusion( P=0.01), preoperative platelet count( P<0.01) and postoperative infection rate ( P=0.02). The results of ROC curve analysis showed that the cut-off values of operative duration, red blood cell transfusion and preoperative platelet count were 535 min, 2.75 U and 183.5×10 9/L respectively.Length of ICU stay was higher in TCP group than that in non-TCP group, and the difference was statistically significant [4(3, 5) vs.3(3, 4) day, P=0.006]. Conclusions:LT children aged under 4 years with intraoperative red blood cell transfusion>2.75 U, operative duration>535 min and preoperative platelet count<183.5×10 9/L are more likely to develop post-transplantation TCP.And occurrence of TCP prolongs the length of ICU stay in pediatric recipients.
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Objective:To investigate the clinical application of continuous renal replacement therapy (CRRT) in infants with acute kidney injury (AKI) after liver transplantation.Methods:A retrospective study was conducted on infants with AKI after liver transplantation in Tianjin First Center Hospital from January 1, 2019 to June 1, 2021. Infants with AKI within 1 year after liver transplantation were divided into CRRT group and non-CRRT group according to whether CRRT was performed. The preoperative and intraoperative condition, the postoperative complications were compared, the risk factors of CRRT for AKI infants, the clinical characteristics of CRRT were analyzed, and the prognosis between CRRT group and non-CRRT group were compared.Results:① A total of 512 cases of pediatric liver transplantation were performed. A total of 189 cases (36.9%) developed AKI within 1 year after surgery, including 18 cases in CRRT group and 171 cases in non-CRRT group. ② There was no significant difference in preoperative conditions between the two groups. The duration of liver transplantation (hours: 8.8±1.5 vs. 7.5±1.3) and intraoperative blood loss [mL: 370 (220-800) vs. 310 (200-400)] in CRRT group were significantly higher than those in non-CRRT group. CRRT group had significantly higher incidence of postoperative complication [unplanned operation: 8 cases (44.4%) vs. 14 cases (8.2%), primary nonfunction: 1 case (5.6%) vs. 0 case (0%), retransplantation: 3 cases (16.7%) vs. 0 case (0%), hepatic artery thrombosis: 3 cases (16.7%) vs. 4 cases (2.3%), intestinal fistula: 2 cases (11.1%) vs. 2 cases (1.2%)] than non-CRRT group (all P < 0.05). ③ The average start time of CRRT was 10 (1-240) days. The per capita frequency of CRRT treatment was 3.3 (1.0-14.0) times. The average duration of each CRRT treatment was 10.1 (6.0-19.3) hours, the average reduction rate of serum creatinine (SCr) was 25.6% (13.5%-45.0%) after CRRT. ④ In CRRT group, 5 patients died, the 1-year and 2-year survival rates were both 72.22%. In non-CRRT group, 6 patients died, the 1-year and 2-year survival rates were 97.1% and 96.5%, respectively. There were significant differences in 1-year and 2-year survival rates between the two groups (both P < 0.01). Conclusions:The incidence of AKI after pediatric liver transplantation was high, and most infants treated with CRRT were associated with serious surgical complications. CRRT was a powerful means to remove inflammatory factors and maintain the stability of circulation and internal environment, which could improve the multi-organ dysfunction effectively.
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Objective To summarize the experience of diagnosis and treatment of Takotsubo syndrome (TTS) after liver transplantation. Methods Clinical data of one TTS patient after liver transplantation was retrospectively analyzed. Clinical features, diagnosis and treatment strategies were summarized, and literature review was conducted. Results A 43-year-old female patient successfully underwent split liver transplantation due to primary biliary cirrhosis for 8 years. At postoperative 3 d, the patient developed anxiety, irritation, dyspnea, disorientation, hypotension, N-terminal pro-brain natriuretic peptide (NT-proBNP) of > 35 000 pg/mL, creatine kinase isoenzyme (CK-MB) of 5.9 U/L and troponin I (TnI) of 1.78 μg/L. Electrocardiogram indicated the signs of sinus rhythm. Echocardiography indicated diffuse weakening of the left ventricular wall motion and spherical dilatation of the apex, accompanied with moderate and severe regurgitation of the mitral valve and tricuspid valve. The left ventricular ejection fraction (LVEF) declined to 23%, whereas no abnormal segmental motion of ventricular wall or corresponding electrocardiogram changes were observed. The possibility of acute coronary syndrome was excluded. The InterTAK diagnostic score was 73. The diagnosis of TTS after liver transplantation was considered. Metoprolol, coenzyme Q10, recombinant human brain natriuretic peptide, deacetyl lanatoside and lorazepam were given. Echocardiography at postoperative 10 d showed that the left ventricular function was significantly improved and the LVEF recovered to 50%. The patient was discharged 40 d after liver transplantation. The liver function was recovered well. During postoperative follow-up, she was given with metoprolol till the submission date, and no recurrence was reported. Conclusions TTS after liver transplantation is rare in clinical practice. It is difficult to make the diagnosis. The condition of TTS is severe and clinical prognosis is poor. Prompt diagnosis and interventions should be implemented.
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Objective:To detect causative gene mutations in 1 patient with ADULT syndrome mainly presenting with ectodermal dysplasia.Methods:Clinical data were collected from a proband with ADULT syndrome, and genomic DNA was extracted from peripheral blood samples obtained from the proband and his parents. Exome sequencing was performed in the proband by using targeted panels for hereditary skin diseases to determine mutation sites, and then the candidate mutation sites were verified by Sanger sequencing in the family members.Results:The 22-year-old male patient presented with sparse and thin hair, scattered facial freckles, missing permanent teeth, cloudy corneas, palmoplantar erythema and keratosis, nail/toenail dystrophy, and nipple dysplasia. Genetic testing of the peripheral blood genomic DNA of the proband revealed a heterozygous mutation (c.1040G>T) in exon 8 of the TP63 gene, resulting in an amino acid change at position 347 (p.C347F) . The mutation was not detected in his father or mother with normal phenotypes, suggesting the cosegregation of the gene mutation with the disease phenotype in the family.Conclusion:The de novo heterozygous missense mutation in the TP63 gene may be the causative mutation in the proband, and combined with clinical manifestations, the proband was diagnosed with ADULT syndrome without finger/toe deformities.
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Objective:To detect gene mutations and make a diagnosis in a family with ichthyosis accompanied by liver injury.Methods:Clinical data were collected from the proband, and genomic DNA was extracted from peripheral blood samples from the proband and his parents. Exome sequencing was performed in the proband by using a gene panel targeting hereditary skin diseases to identify mutation sites, and then the candidate mutation site was verified by PCR and Sanger sequencing in the family members. Results of peripheral blood smear examination and other auxiliary examinations were collected from the proband and his parents and analyzed.Results:The proband presented with generalized dry skin and tiny white scales on the lower limbs, accompanied by elevated transaminase levels, mild sensorineural hearing loss in both ears and fatty liver. Exome sequencing revealed a homozygous mutation c.933dupA in exon 6 of the ABHD5 gene encoding CGI-58 protein in the peripheral blood genomic DNA of the proband, resulting in a frameshift mutation p.R312Tfs*45 in the amino acid sequence. Heterozygous mutations at this site were identified in his father and mother. The mutation cosegregated with the disease phenotype in the family. The peripheral blood smear examination of the proband showed lipid vacuoles in neutrophils, which were called Jordan anomaly. Conclusion:The diagnosis of Chanarin-Dorfman syndrome was made in the proband based on the presentation of ichthyosis-like skin lesions and abnormal liver function, as well as the homozygous mutation in the ABHD5 gene and Jordan anomaly in peripheral blood smears.
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Objective:To study the use of digital subtraction angiography (DSA) guided transnasal ileus tube placement in management of abdominal compartment syndrome (ACS) after liver transplantation.Methods:From January 2015 to December 2019, a total of 30 patients who developed ACS after liver transplantation who were admitted to the Transplantation Intensive Care Unit of Tianjin First Central Hospital were retrospectively studied. According to the way of decompression, these patients were divided into the study group and the control group. Patients in the control group were treated with conventional abdominal decompression, while patients in the study group were treated with DSA guided transnasal ileus tube placement based on management principles developed in conventional abdominal decompression. Changes in intra-abdominal pressure, treatment efficacy rates and liver functions were monitored in the two groups up to 7 days after abdominal decompression.Results:There were 23 males and 7 females, aged (53.4±11.6) years. After treatment, the IAP, portal venous blood flow velocity, bile drainage volume, ALT and AST in the study group were significantly better when compared with the findings before treatment: [IAP: (7.13±3.87) vs (22.73±2.09) mmHg, portal vein blood flow velocity: (34.76±10.31) vs (21.45±6.47) cm/s, bile drainage volume: (198.43±19.94) vs (80.72±9.52) ml/d, ALT: (158.92±67.56) vs (278.73±99.17) U/L, AST: (79.36±15.63) vs (196.71±89.05) U/L], ( P<0.05). After treatment, when compared with the control group, the IAP, portal vein blood flow velocity, bile drainage and TBil in the study group were significantly better [IAP: (7.13±3.87) vs (13.47±6.19) mmHg, portal vein blood flow velocity: (34.76±10.31) vs (24.98±8.54) cm/s, bile drainage: (198.43±19.94) vs (108.73±21.30) ml/d, TBil: (258.85±91.95) vs (343.69±89.45) μmol/L], ( P<0.05). In the control group, the IAP significantly decreased on the fourth day after treatment, ( P<0.05); compared with the significant difference in the study group on the second day after treatment ( P<0.05). After 7 days of treatment, the efficacy rate of the control group was 46.7% (7/15), compared to 86.7% (13/15) in the study group. The difference between the two groups was significant (χ 2=5.400, P<0.05). Conclusion:DSA guided transnasal ileus tube placement for treatment of abdominal compartment syndrome after liver transplantation resulted in a better treatment efficacy rate than conventional treatment.
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Objective:To explore the application of extracorporeal membrane oxygenation (ECMO)for severe acute respiratory distress syndrome(ARDS)in children after liver transplantation.Methods:The clinical data were retrospectively analyzed for two ECMO-supported children with severe ARDS after liver transplantation. There were suspected pneumocystis carinii pneumonia(n=1)and identified pneumocystis carinii pneumonia(n=1).Results:Veno-arterial ECMO(VA-ECMO)was performed and oxygen saturation index(OSI)before an initiation of ECMO was 31.8 and 23.9 respectively. Both were successfully separated from ECMO after 219 h and 168 h support respectively, and both were weaned from ventilator after 342 h and 232 h invasive mechanical ventilation respectively. The length of ICU stay was 31 and 18 days and the length of hospital stay 57 and 33 days respectively. During ECMO support, liver function remained stable and there was no new onset of organ dysfunction or life-threatening complications.Conclusions:ECMO is a potential therapy for children with severe ADRS after liver transplantation and the assessment and management of complications with ECMO support should be further studied.
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Objective:To study the clinical course and underlying causes of early severe neurological complication (ESNC) after pediatric liver transplantation.Methods:A retrospective study was conducted on 309 pediatric liver transplantation recipients treated at Tianjin First Central Hospital from January 2018 to December 2018. ESNC occurred in 15 patients (4.8%, 15/309) within 1 month after liver transplantation. There were 7 males and 8 females, aged from 6 to 46 months. Liver transplantation was carried out for biliary atresia ( n=12), fulminant liver failure ( n=1), Niemann-Pick disease ( n=1) and Alagille syndrome ( n=1). The causes of ESNC and the prognosis were analyzed. Results:The onset of ESNC was 10.7 (0-28) d after liver transplantation. Twelve patients developed encephalopathy with epilepsy in 2 patients. Four of these patients were caused by severe infection, 4 by heart failure combined with respiratory failure which led to ischemic hypoxic encephalopathy, 2 by transplant liver failure, 1 by intracranial infection, and 1 by severe brain swelling which led to brain death. Epilepsy occurred in 3 patients, 2 caused by neurotoxicity of calcineurin inhibitors, and 1 caused by reversible posterior leukoencephalopathy syndrome. Three children with ESNC died after operation, including 1 brain death, 1 due to severe heart failure and 1 due to severe infection.Conclusions:ESNC occurred in 4.8% of patients which seriously affected long-term prognosis of patients. Measures to reduce the incidence of ESNC include prevention of postoperative infection and drug toxicity, and good control of cardiac insufficiency.
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Hyperammonemia syndrome (HS) is a comparatively rare but often fatal clinical syndrome characterized by progressive respiratory alkalosis and abrupt mental status alteration associated with markedly elevated plasma ammonium levels. Although the exact mechanism of HS remains unclear, infection with urease producing microbes is proposed as the main etiology of HS recently. A patient with HS after repeated autologous skin transplantation was admitted to Tianjin First Center Hospital in March 2018, presented with fever, coma and epilepsy. The infection of Mycoplasma hominis was confirmed in blood sample by high throughput gene detection. The patient was survived after multimodal management including antimicrobial treatment, aggressive ammonia removal by continuous renal replacement therapy in combination with lactulose, and mechanical ventilation. She was successfully discharged from intensive care unit (ICU) with clear consciousness, normal temperature and smooth breath. In view of the experience of the case treatment, a review of literature was conducted to discuss the epidemiology and clinical characteristics, possible etiologies and mechanisms, and outcomes with emphasis on treatment strategies of HS and to promote more clinicians to recognize this rare disease.
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Objective To review the development of adult and pediatric liver transplantation in Tianjin First Center Hospital, and to enhance academic exchanges, improve technological innovation, and jointly promote the progress and maturity in the field of liver transplantation. Methods The development of liver transplantation in Tianjin First Center Hospital was analyzed. The clinical data of adult and pediatric liver transplantation from September 1998 to September 2018 were collected. The important events and technological innovation achievements of liver transplantation during the 20 years were summarized. Results The first clinical liver transplantation was attempted in Tianjin First Central Hospital in April 1980. The first long-term survival adult liver transplantation in China was completed in 1994 (11 years survival after the operation). The specialized team of liver transplantation was formally established in September 1998. The 20-year clinical exploration and progress reflected the characteristics of era changes and technological innovation during the rapid development of liver transplantation in China. Our center performed liver re-transplantation in January 1999, reduced-size pediatric liver transplantation in August 2000. In May 2001, we organized the formulation for the preventive and treatment plan for hepatitis B recurrence after liver transplantation. We performed combined liver and kidney transplantation in July 2002, split liver transplantation (SLT) in April 2004, the first domino liver transplantation (DLT) in August 2005. Pediatric living donor liver transplantation (LDLT) was initiated in October 2006, adult LDLT was carried out in August 2007. In September 2007, the first living donor combined liver and kidney transplantation from the same donor in Asia was performed. The first domino+living donor double grafts liver transplantation in the world was performed in January 2009. In March 2011, we performed laparoscopically assisted right hepatic lobe liver transplantation (LDLT) with middle hepatic vein. In May 2014, living donor laparoscopic left lateral lobe procurement was successfully established. In April 2016, simultaneous liver, pancreas and kidney multi-organ transplantation was completed. Domino donor-auxiliary liver transplantation was performed in February 2017. In December 2017, extracorporeal membrane oxygenation (ECMO)-supported liver transplantation in a patient with severe pulmonary hypertension was successfully completed. Liver transplantation combined with partial splenectomy was established in April 2018. Cross-domino liver transplantation (hypersensitive kidney transplantation with auxiliary liver transplantation+pediatric liver transplantation) was performed in May 2018. During the 20 years, the team has performed or assisted other centers in Beijing, Shanghai, Guangzhou and Shenzhen to carry out more than 10 000 cases of liver transplantations. A total of 7 043 cases of various types of liver transplantation were performed in the single center of the hospital (6 005 adult liver transplantations and 1 038 pediatric liver transplantations). Concerning adult liver transplantation, the cumulative 1-year, 3-year and 5-year survival rate from September 1998 to March 2003 were 83.1%, 73.0% and 69.0%, from April 2003 to March 2009 were 85.3%, 76.2% and 72.1% and from April 2009 to September 2018 were 87.5%, 79.2% and 75.1%, respectively. The cumulative 1-year, 3-year and 5-year survival rate for pediatric liver transplantation were 93.5%, 92.2% and 90.2%, respectively. The nucleoside (acid) analogue combined with low dose hepatitis B immunoglobulin (HBIG) was developed to prevent the recurrence of hepatitis B after liver transplantation, this plan has reduced the recurrence rate of hepatitis B and the 5-year re-infection rate of hepatitis B virus (HBV) after liver transplantation significantly. The risk assessment system for tumor recurrence after liver transplantation was established and individual treatment method was established based on this assessment system. Continuous exploration and improvement of liver transplantation for liver cancer, liver re-transplantation, liver transplantation with portal vein thrombosis, SLT, DLT and multi-organ combined transplantation have significantly improved the clinical efficacy of patients and the post-operative survival rate. Conclusions The liver transplantation team of Tianjin First Center Hospital has carried out a scientific and technological exploration on the key problems and technical difficulties of clinical liver transplantation. This work strongly has initiated and promoted the rapid development of liver transplantation in China. The restrictive barrier of hepatitis B recurrence after liver transplantation has been overcome. The risk prevention and control system of tumor recurrence after liver transplantation has been established. A series of innovative achievements that can be popularized have been achieved in the field of complex liver transplantation and expansion of donor liver source. The iterative progress and sustainable development of liver transplantation have been realized.
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With the continuous improvement of liver transplantation techniques and the innovation of related disciplines, great changes have been observed in the etiology and clinical outcome of middle- and long-term complications after liver transplantation, and thus related prevention and treatment strategies are worthy of exploration and adjustment. In recent years, breakthroughs have been made in the prevention and treatment of post-transplantation complications including the recurrence of hepatitis, but standardized diagnosis and treatment should be strengthened in order to maximize the benefits of recipients. So far, the prevention and treatment of middle- and long-term complications with unknown pathogenesis and complicated causes after liver transplantation still rely on the long-term monitoring, prevention, and control of related risk factors. The main tasks of long-term management of liver transplantation recipients is to maintain the function of transplanted liver, control the recurrence of primary diseases, prevent and treat the new-onset diseases, and continue to treat the concomitant diseases before surgery. This article elaborates on the tasks and strategies for the prevention and treatment of middle- and long-term complications after liver transplantation.
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Objective To investigate the clinical efficacy and influencing factors in patients with acute-on-chronic liver failure grade 3 after liver transplantation.Methods 33 patients with acute-on-chronic grade 3 liver failure who were treated in Tianjin First Center Hospital from January 2015 to December 2017 was retrospectively analyzed,including 21 patients in liver transplantation group and 12 patients in control group.Among them,28 patients were males and 5 patients were females,aged (43.4± 12.3) years.The data and follow-up information of all patients were collected.The survival condition was analyzed by Kaplan-Meier.Univariate and multivariate Cox regression analysis was used to analyze the risk factors of death in patients after liver transplantation.Results There was no significant difference in Child-Pugh score,total bilirubin,creatinine and infection before operation between liver transplantation group and control group (P＞0.05).The age of patients in liver transplantation group was older than the control group,the difference was statistically significant (P＜0.05).The 1-year and 3-year cumulative survival rates in the liver transplantation group were 61.9％ and 61.9％ respectively and the rates in control group were 8.3％ and 8.3％ respectively by Kaplan-Meier survival analysis.There was significant difference between the two groups (P＜0.05).Twenty-one patients in the liver transplantation group were followed up for a long time,13 patients survived and followed up for 163～ 1 123 days.Except for renal insufficiency complicated with renal anemia in 1 case,the other 12 cases had normal liver function,and 8 cases died in 2～54 days after liver transplantation.Postoperative shock was an independent risk factor for death after liver transplantation by univariate and multivariate Cox regression analysis.Conclusion Acute-on-chronic grade 3 liver failure was indication for liver transplantation,postoperative shock was an independent risk factor for death after liver transplantation.
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Objective To discuss the surgical strategy for children with complex congenital heart disease (CHD) and end-stage liver disease (ESLD).Methods We reported two eases of pediatric liver transplantation in patients with complex CHD and ESLD.Medical data including operation procedure,ICU management and outcomes were reviewed retrospectively.Also we reviewed the literature on the topic of clinical outcomes resulted from different surgery options.Results The first case was a seven-month-old male patient with biliary atresia and complex CHD (unroofed coronary sinus syndrome,persistent left superior vena cava,patent foramen ovale,and peripheral pulmonary stenosis).Liver transplantation was successfully performed without corrective heart surgery.The operation time was 6 h and 35 min.The patient suffered acute cardiac dysfunction and significant hypoxemia after extubation,then pneumonia developed,and eventually the patient died on post-operative day 12.The second case was a seven-month-old male patient with biliary atresia and complex CHD (ventricular septal defect,patent foramen ovale,patent ductus arteriosus,pulmonary stenosis).Liver transplantation was performed on the same day following total correction of cardiac defects by open-heart surgery.The operation time was 16 h and 15 min.The patient was extubated after 60 h ventilation,and was transferred to ward from ICU on post-operative day 6 with stable cardiopulmonary function.However,hepatic artery occlusion occurred on early postoperative stage,and consequently the patient received the second liver transplantation for ischemic biliary complication on post-operative day 40.The second liver transplantation procedure was uneventful.The liver graft recovered smoothly with stable hemodynamics.Conclusion Children with complex CHD undergoing liver transplantation are at an increased perioperative risk.The surgical strategy for each patient must be tailored individually according to specific cardiovascular status and limited hepatic reserve.
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Objective To explore the effectiveness of octreotide therapeutic strategy to attenuate portal hyperperfusion resulted from small-for-size graft in infant liver transplantation.Methods A total of 22 infants received small-for-size liver graft (defined as GV/SLV＜0.5,and GV＜ 150 g) in our hospital from December 2013 to August 2016.Twelve cases (octreotide group) were treated with intravenous octreotide infusion (300 g daily for 24-96 h) to attenuate the portal hyperperfusion after transplantation,and the rest 10 cases given liver transplantation at the early stage did not receive the intervention of octreotide and served as control group.Results The initial portal vein flows (PVFs) in octreotide group and control group were (413.43 ± 76.24) (390.83 ± 107.89) ml/(min 100 g),and there was no significant difference between two groups (P＞0.05).The PVFs on postoperative day (POD) 3 and POD5 in octreotide group and control group were (334.90 ± 96.67) and (441.04 ± 117.41),and (322.20 ± 81.04) and (423.23 ± 100.81) mL/(min 100 g) respectively (P＜0.05 for all).However,there were no significant differences in serum AST and bilirubin levels at four time points (initial,POD3,POD5 and POD7) after transplantation between two groups (P＞0.05).The incidence of hepatic artery occlusion,and biliary complications in octreotide group and ontrol group was 33.33％ and 44.44％,and 33.33％ and 11.11％ respectively (P ＞ 0.05 for all).Conclusion Octreotide treatment attenuated portal hyperperfusion resulted from small-for-size graft in infant liver transplantation.However,the effects of octreotide therapy on graft biochemical tests,the hepatic artery and biliary complications were still unclear,and further investigation is needed.
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Objective To investigate the effect of active immunization on prevention of post-transplantation de novo hepatitis B virus (HBV) infection in patients receiving liver grafts from hepatitis B core antibody (HBcAb) positive donors.Methods A retrospective analysis was conducted. Eighty-seven children undergoing liver transplantation from HBcAb positive donors admitted to Tianjin First Center Hospital from October 2012 to December 2016 were enrolled, and the data of donors and recipients were collected. The hepatitis B vaccine was given before operation for hepatitis B surface antibody (HBsAb) >1000 U/L; hepatitis B immunoglobulin (HBIG) 100 U/kg was given during the operation, in order to prevent children from HBV infected by obtaining passive immunity quickly, children with HBsAb< 200 U/L after operation were injected with hepatitis B vaccine for booster immunization. HBV markers and liver function of recipients were determined before liver transplantation and during the follow-up, which up to April 2017. According to the children got de novo HBV infection after operation or not, the preventive effect of active immunization before and after transplantation operation on HBV infection was analyzed and compared.Results In 87 children who received HBcAb positive donor livers, 9 (10.3％) developed de novo HBV infection, which occurred in 16 (10, 25) months after liver transplantation. Among the 9 children with HBV infection, 7 children had HBsAb < 1000 U/L before the operation, the ratio was statistically increased as compared with the children without HBV infection [77.8％ (7/9) vs. 37.2％ (29/78),P < 0.05]. After the transplantation, 62 children of 78 without HBV infection showed a good response to hepatitis B vaccination, 1 child after inoculation of hepatitis B vaccine, the titer of HBsAb was still less than 200 U/L, 15 children without administration of hepatitis B vaccine, only with HBIG injection for prevention. The HBsAb of children with de nove HBV infection were less than 200 U/L after operation, the ratio was significantly increased as compared with children without HBV infection [100.0％ (9/9) vs. 20.5％ (16/78),P < 0.01].Conclusions The establishment of active immunization method can effectively prevent children with de novo HBV infection occurred inpediatric recipients from HBcAb positive donors with preventive treatment.
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Objective To explore the effectiveness of octreotide therapeutic strategy to attenuate portal hyperperfusion resulted from small-for-size graft in infant liver transplantation.Methods A total of 22 infants received small-for-size liver graft (defined as GV/SLV＜0.5,and GV＜ 150 g) in our hospital from December 2013 to August 2016.Twelve cases (octreotide group) were treated with intravenous octreotide infusion (300 g daily for 24-96 h) to attenuate the portal hyperperfusion after transplantation,and the rest 10 cases given liver transplantation at the early stage did not receive the intervention of octreotide and served as control group.Results The initial portal vein flows (PVFs) in octreotide group and control group were (413.43 ± 76.24) (390.83 ± 107.89) ml/(min 100 g),and there was no significant difference between two groups (P＞0.05).The PVFs on postoperative day (POD) 3 and POD5 in octreotide group and control group were (334.90 ± 96.67) and (441.04 ± 117.41),and (322.20 ± 81.04) and (423.23 ± 100.81) mL/(min 100 g) respectively (P＜0.05 for all).However,there were no significant differences in serum AST and bilirubin levels at four time points (initial,POD3,POD5 and POD7) after transplantation between two groups (P＞0.05).The incidence of hepatic artery occlusion,and biliary complications in octreotide group and ontrol group was 33.33％ and 44.44％,and 33.33％ and 11.11％ respectively (P ＞ 0.05 for all).Conclusion Octreotide treatment attenuated portal hyperperfusion resulted from small-for-size graft in infant liver transplantation.However,the effects of octreotide therapy on graft biochemical tests,the hepatic artery and biliary complications were still unclear,and further investigation is needed.
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Objective To investigate the effect of active immunization on prevention of post-transplantation de novo hepatitis B virus (HBV) infection in patients receiving liver grafts from hepatitis B core antibody (HBcAb) positive donors.Methods A retrospective analysis was conducted. Eighty-seven children undergoing liver transplantation from HBcAb positive donors admitted to Tianjin First Center Hospital from October 2012 to December 2016 were enrolled, and the data of donors and recipients were collected. The hepatitis B vaccine was given before operation for hepatitis B surface antibody (HBsAb) >1000 U/L; hepatitis B immunoglobulin (HBIG) 100 U/kg was given during the operation, in order to prevent children from HBV infected by obtaining passive immunity quickly, children with HBsAb< 200 U/L after operation were injected with hepatitis B vaccine for booster immunization. HBV markers and liver function of recipients were determined before liver transplantation and during the follow-up, which up to April 2017. According to the children got de novo HBV infection after operation or not, the preventive effect of active immunization before and after transplantation operation on HBV infection was analyzed and compared.Results In 87 children who received HBcAb positive donor livers, 9 (10.3％) developed de novo HBV infection, which occurred in 16 (10, 25) months after liver transplantation. Among the 9 children with HBV infection, 7 children had HBsAb < 1000 U/L before the operation, the ratio was statistically increased as compared with the children without HBV infection [77.8％ (7/9) vs. 37.2％ (29/78),P < 0.05]. After the transplantation, 62 children of 78 without HBV infection showed a good response to hepatitis B vaccination, 1 child after inoculation of hepatitis B vaccine, the titer of HBsAb was still less than 200 U/L, 15 children without administration of hepatitis B vaccine, only with HBIG injection for prevention. The HBsAb of children with de nove HBV infection were less than 200 U/L after operation, the ratio was significantly increased as compared with children without HBV infection [100.0％ (9/9) vs. 20.5％ (16/78),P < 0.01].Conclusions The establishment of active immunization method can effectively prevent children with de novo HBV infection occurred inpediatric recipients from HBcAb positive donors with preventive treatment.
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Objective To summarize the clinical course of acute interstitial pneumonitis (AIP) associated pediatric acute respiratory distress syndrome (PARDS) in 8 recipients after liver transplantation,and further discuss the potential risk factors and therapeutic highlights.Methods A total of 476 pediatric patients received liver transplantation in Tianjin First Center Hospital from January 2012 to September 2016.Among them,8 cases of AIP associated PARDS in ICU were recruited in this study.Medical data including clinical presentation,ICU management and outcomes were analyzed retrospectively.Results The onset time-window of AIP associated PARDS was (2.67 ± 0.77) months after liver transplantation,and the time interval between initial symptom and ICU administration was (6.75 ± 5.82) days.Five cases had the history of acute rejection therapy,and 5 cases had CMV and/or EBV viremia history.All 8 cases received mechanical ventilation,2 cases given nasal non-invasive ventilation and the rest 6 cases given invasive ventilation,3 of which were switched to high frequency oscillatory ventilation (HFOV) combined with inhaled nitric oxide.At the stage of hypoxic climax,the fraction of inspired oxygen (FiO2) was up-regulated to 1.0 to maintain the oxygenation index (OI) of (25.24 ± 5.94).Temporary replacement of immunosuppressants with intravenous glucocorticoids was implemented in all 8 cases without acute rejection episode.Of 8 cases,2 cases died from PARDS,1 case died from portal thrombosis associated hepatic failure,and the rest 5 cases survived.Conclusion AIP associated PARDS is a critical complication with high mortality in pediatric patients after liver transplantation.Excessively strong immunosuppression therapy at early post-transplant stage shows a risk factor for AIP.Lung protective ventilation strategy and HFOV are recommended to reduce ventilator induced lung injury in pediatric patients.Temporary intravenous glucocorticoids may reduce acute inflammatory reaction in PARDS patients without increasing the risk of acute rejection.
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Objective To investigate the correlation between indocyanine green plasma disappearance rate (ICG-PDR) and allograft function,postoperative complications after liver transplantation.Methods A prospective? study was done on 115 cases of adult liver transplantation from Jun 1st,2016 to December 1st,2016.115 patients were divided into ICG-PDR ＜ 18％/min group (group A,n =50) and ICG-PDR≥18％/min group (group B,n =65).The recovery of liver function,complications and survival rate were analyzed.Results 111 out of 115 cases recovered well and discharged,and 4 cases died at the first month postoperation.There was significant difference in the MELD score,bleeding volume during operation and Hb,PA,TB of the first week postoperation.The incidence of hepatic artery complications and pneumonia was significantly higher in group A than in group B (P＜0.05).The survival rate within 3 and 6 months was 94％ (47/50) in group A,and 98.5％ (64/65) in group B (P＞0.05).Conclusion Early postoperative ICG-PDR was closely related to graft function,and ICG-PDR was a good predictor of postoperative arterial complications.