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Objective:To explore the clinicopathological characteristics, treatments and outcomes of posttransplant lymphoproliferative disorder(PTLD)in pediatric liver transplant recipients.Methods:From October 2016 to October 2021, retrospective data analysis was performed for 11 pediatric liver transplant recipients with PTLD. There were 5 males and 6 females with a diagnostic age of 1-8 years. Living donor liver transplantation(LDLT, n=9)and deceased donor liver transplantation(DDLT, n=2)were performed. All recipients received tacrolimus plus methylprednisolone. The major clinical manifestations included lymphadenopathy, splenomegaly, anemia, fever and digestive system symptoms(diarrhea, abdominal pain, ascites, hematochezia & intussusception, etc.). Laboratory tests hinted at hypoproteinemia, elevated transaminases and serum positivity of EBV-DNA. Positron emission tomography and computed tomography(PET-CT)revealed PTLD( n=9). Ten children were diagnosed by pathology, including lymphoid hyperplasia( n=3), plasmacytic hyperplasia PTLD( n=1), polymorphic PTLD( n=2), diffuse large B-cell lymphoma( n=2), infectious mononucleosis PTLD( n=1)and Burkitt lymphoma( n=1). Results:After a definite diagnosis of PTLD, tacrolimus was tapered or discontinued. And rituximab was prescribed. Two patients received chemotherapy(R-COP & R-CHOP)while 2 cases of local masses were operated. Up until February 2022, 10 cases survived and their conditions improved. One patient died of infection.Conclusions:PTLD is one of the most serious and fatal complications after liver transplantation in children. Clinical manifestations are diverse and an early diagnosis is difficult. The changes of EBV-DNA load should be closely monitored after liver transplantation. Imaging and pathological examinations may aid in an early diagnosis of PTLD. A treatment regimen based on immunosuppression reduction and rituximab improves the prognosis of PTLD in pediatric liver transplant recipients.
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Pediatric acute liver failure (PALF) is a rare syndrome with high mortality, and at present, liver transplantation is still the only effective treatment method for PALF. In recent years, the technology of liver transplantation in children has become more and more mature and has significantly improved the prognosis of PALF patients in China. However, there are still many problems in liver transplantation for PALF patients. Comprehensive discussion of objective problems before, during, and after liver transplantation may further improve the overall prognosis of PALF patients.
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Although pediatric acute liver failure (PALF) is rare in clinical practice, it seriously threatens the life and health of children due to acute onset and rapid progression. PALF has various etiologies, and at present, it is still unable to identify the etiology in a relatively large proportion of children. The clinical manifestations of PALF are also different from those of adults, and it is difficult to judge early hepatic encephalopathy in infants and young children. It is very important to maintain the stability of internal environment, provide etiological treatment, and avoid drug abuse and the abuse of blood products. Blood purification can be performed for patients with related indications to win more time for autogenous liver function recovery and liver transplantation, and the precise diagnosis and treatment of PALF should be taken seriously in clinical practice.
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Objective:To assess the transient elastography (TE) in assessing hepatic fibrosis in pediatric chronic liver disease.Methods:Children with chronic liver disease who were both examined with TE and percutaneous transhepatic puncture for grading Scheuer scores in the Children′s Hospital of Fudan University from January 2017 to September 2017 were recruited.Ordinal Logistic regression analysis was used to analyze the interfering factors for hepatic fibrosis.Receiver operating characteristic (ROC) curves were plotted to acquire the optimal cut-off value and to calculate the area under the curve (AUC). Results:Thirty-eight children were enrolled finally, including 28 boys and 10 girls with the mean age of 7.4 years old (1.1-16.0 years). The success rate of detecting hepatic fibrosis was 85%.Ordinal Logistic regression analysis discovered that only the value of liver stiffness measured by TE was correlated with the grade of hepatic fibrosis ( β=0.055, P<0.001). TE was effective to differentiate hepatic fibrosis[<S2 vs.≥S2 (6.6±4.0) kPa vs.(21.2±18.6) kPa, P=0.001], significant hepatic fibrosis[<S3 vs.≥S3 (7.4±4.0) kPa vs.(34.8±19.0) kPa, P=0.000 1, and cirrhosis[<S4 vs.S4 (10.6±12.3) kPa vs.(35.8±15.1) kPa, P=0.002]. At the cut-off values of liver stiffness measurement with 6.89 kPa and 14.39 kPa, AUC of it to predict the grade of hepatic fibrosis ≥S2 and ≥S3 were 0.81 ( P=0.001) and 0.94( P<0.001), respectively. Conclusions:TE is a useful and reliable noninvasive tool to assess hepatic fibrosis in pediatric chronic liver disease.
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A retrospective analysis was performed on the clinical data of a child with X-linked hyper IgM syndrome (XHIGM) with cholangiectasis as a major manifestation in Children′s Hospital of Fudan University in March 2017.The patient was a 4-year-old boy who was admitted to the hospital due to repeated diarrhea for half a year and yellow skin for 5 days.No abnormalities were found in his fetal period and birth history; The patient had 2 severe pneumonias and suppurative infection of the left axillary lymph node in infancy.Physical examination revealed delayed physical development, severe malnutrition, moderately stained yellow, lymphadenopathy and hepatomegaly.Laboratory examinations showed elevated leukocyte, eosinophils and C-reactive protein, low hemoglobin and albumin, high gamma-glutamyl transpeptidase (GGT), low IgG and normal IgM.Imaging examination revealed diffuse expansion of intrahepatic and extrahepatic bile ducts.Hepatic pathology showed hyperplasia in the bile canaliculus and some fibrous tissues around the large bile ducts.High-throughput sequencing identified a pathogenic mutation in the XHIGM gene CD 40LG (exon5 c. 506A>G, p.Y169C), with his mother as a carrier.After admission, the patient was given anti-infection, diet adjustment, albumin, intravenous immunoglobulin and ursodeoxycholic acid.The patient was discharged after the improvement in his condition.This case suggested that in addition to the common infection characteristics, XHIGM can also be manifested as diffuse intrahepatic, extrahepatic cholangiectasis and significantly elevated eosinophil.c.506A>G mutation in CD 40LG was the pathogenic mutation of this disease.
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Objective@#To explore the relationship between genotype and phenotype of ABCB11 deficiency.@*Methods@#Clinical data of two siblings with ABCB11 deficiency were retrospectively analyzed. Related literature from PubMed, CNKI and Wangfang databases was reviewed to date (up to August 2017) with 'ABCB11 gene’ or 'bile salt export pump’, 'cholestasis’ and 'child’ as key words.@*Results@#The patients were siblings. Both of them presented as jaundice, pruritus and hepatosplenomegaly since 3 days after birth. Significant laboratory findings on admission of the older sister included high total bilirubin, 170 µmol/L;conjugated bilirubin, 115.8 µmol/L;alanine aminotransferase, 168 U/L;total bile acid 186.3 µmol/L and normal gamma-glutamyl transpeptidase. While routine laboratory data of the younger brother were as follows: total bilirubin, 148.8 µmol/L;conjugated bilirubin, 96.3 µmol/L;alanine aminotransferase, 232.8 U/L;total bile acid 226 µmol/L, and normal gamma-glutamyl transpeptidase.Both received ursodeoxycholic acid and fat-soluble vitamins. Liver pathology of the younger brother showed giant hepatocytes with ballooning degeneration, focal necrosis and intrahepatic cholestasis. Both the patients harbor the same compound heterozygous mutations in ABCB11 gene, c.145C>T (p.Q49X) and c.1510G>A (p.E504K). The sister is 9 years old now, with normal liver function. Jaundice faded around 3 months after birth, pruritus relieved at age 5, and medications was stopped since then. The brother progressed to liver failure after an operation on perianal abscess when he was 8-month-old, and received living-related liver transplantation when he was 9 month and 20 days old (from his mother). Now he is 1 year and 5 months old, with normal liver function. Both are under our follow-up. Literature review revealed 18 ABCB11 deficiency patients from 7 families who had apparent different prognoses, within each family the siblings had the same ABCB11 gene mutation. Seven cases relieved after ursodeoxycholic acid therapy and/or partial external biliary diversion, 5 received orthotopic liver transplantation, 2 developed hepatocellular carcinoma and 4 cases died in childhood.@*Conclusions@#The clinical manifestations of ABCB11 deficiency may vary greatly in patients carrying the same genotype, even in siblings. Patients should be managed in individualized maner.
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ObjectiveTo understand the clinical epidemiology of enterovirus 71 (EV71) in children with hand,foot,and mouth disease (HFMD) in Shanghai during 2010 to 2011.Methods The demographic,etiological and clinical data of children with HFMD were analyzed retrospectively.EV71 was tested in stool samples by one-step quantitative reverse transcription-polymerase chain reaction (RT-PCR).The date were analyzed by Chi-square test.ResultsEV71 was detected in 820 (54.45％) of 1506 inpatients in 2010 and in 924 (59.84％) of 1544 inpatients in 2011,respectively.The detection rates in severe cases and uncomplicated cases were 86.31％ and 46.67％ (x2 =247.146,P＜0.01) in 2010 and 88.78％ and 48.75％ (x2 =201.664,P＜0.01) in 2011,respectively.The detection rate of EV71 was the highest (60％- 67 ％) during May and June when HFMD peaked.Among 1744 EV71-infected HFMD inpatients,the male-to-female ratio was 1.78 ∶ 1 ; the proportion of cases was the lowest in infant ＜6 months of age (0.46％,8/1744),and the highest in children 1 years of age (34.92％,609/1744); children aged 1-3 years accounted for 76.78％ (1339/1744);and migrant children accounted for 72.76 ％ (1269/1744).The demographic characteristics in severe cases were similar to those in general EV71-infected children.Nine severe cases of pulmonary edema/hemorrhage were all infected with EV71.Conclusions EV71 was a major pathogen causing the outbreaks of HFMD and severe complications in Shanghai in 2010 and 2011.Most severe cases and all critically severe cases were associated with EV71 infection.
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Objective To understand the clinical characteristics of pediatric patients who developed H1N1 influenza A virus-associated pneumonia during the outbreak of H1N1 influenza A in Shanghai. MethodsA dcscriptivc study was done to analyze the clinical and epidemiologic characteristics of 30 hospitalized children who developed complicated pneumonia caused by H1N1 influenza A virus infection in 2009 in Shanghai. The comparison of medians was done using rank sum test and comparison of rates was done using exact chi-square test. Results Among thirty pediatric patients with H1N1 influenza A virus-associated pneumonia, the median age was 5.9 years old, five cases (16.7 %) had pre-existing medical conditions. Twenty cases (66.7 % ) had been exposed to the classmates or family membcrs with fever. All cases had fever and cough. Eleven cases (36.7 %00 ) had tachypnca and ten (33.3%) had wheeze. Eleven cases (36.7%) showed white blood cell (WBC)＜4.0 × 109/L and 2 (6. 7%) had thrombocytopenia. All patients had bilateral or unilatcral patchy infiltrates in the lung indicated by chest X-ray and four (13. 3%) had extensive infiltrates with the evidence of pulmonary edema. One (3. 3%) critically ill child with pneumonia, chest computed tomography scan revealed lung fibrosis 3 months and 9 months after illness onset. Three(10. 0%) cases had pneumomediastinum and subcutaneous emphysema. Six cases (20. 0%) were complicated with acute respiratory failure, three (10. 0%) with acute asthmatic attack and one (3. 3%) with encephalitis. All patients were treated with oseltamivir plus antibiotics and four required mechanical ventilation. All patients survived. The median duration of fever in group with oseltamivir given within 2 days of fever onset was statistically shorter than that in group with oseltamivir given 2 days after fever onset (2 days vs 5 days, Z= -8. 015, P<0. 01). Conclusions Both pre-school age and schoolage children may develop complicated severe respiratory diseases after H1N1 influenza A virus infection. Early initiation of oseltamivir may shorten the duration of fever and reduce the occurrence of severe complications.
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Objective To investigate the impact of injecting hepatitis B immune globulin(HBIG)at third trimester of pregnancy on the nucleotide sequences of precore and basal core promoter(BCP)regions of hepatitis B virus(HBV)DNA.Methods One hundred and twenty pregnant women(67 in HBIG group and 53 in no-HBIG group)were enrolled in this study.Serum HBV DNA level was determined using quantitative real-time polymerase chain reaction(RT-PCR).Relevant serum markers (HBeAg,HBsAg)of HBV were detected by enzyme-linked immunosorbent assay(ELISA).Nucleotide fragments of HBV precore and BCP regions were amplified by nested PCR and then sequenced by automated DNA sequencer.Data were analyzed using t test and chi-square test.Results Sera of 33 women in HBIG group were collected before interruption with HBIG and at delivery.Precore and BCP regions of HBV DNA were amplified and sequenced successfully from double sera of 23 among 33 women. The rates of total nucleotide substitute in precore and BCP regions, that in precore region, and that in BCP region before and after interruption were 1.5% and 1.4%, 0.7% and 0.6%, 1.7% and 1.7%, respectively (Fisher's exact test, X2 =0.627, 0.689, 1.000, respectively,all P＞0.05). The rates of total mutations of hot points including 1896G→A,1899G→A,1762A→T,1764G→A before and after interruption were 27.2% and 13.0%, respectively (x2=5.717, P=0. 017). But the prevalences of these hot points mutations before and after interruption were 30.4%and 17.4%, 17.40/00 and 4.3%, 26.1% and 13.0%, 34.80/00 and 17.4%, respectively, which were all not significantly different (P＞0.05). The rates of nucleotide substitute in precore and BCP regions,that in precore region, and that in BCP region of 53 women in HBIG group and 47 women in no-HBIG group at delivery were 0.9% and 0.8%, 0.3% and 0.3%, 1.1% and 0.9%, respectively (Fisher's exact test, )x2=0.434, 0.839, 0.340, respectively, all P＞0. 05). The rates of total mutations of hot points of women in HBIG group and those in no-HBIG group at delivery were 5.7% and 10.1%,respectively, which was not significantly different (P＞0.05). These hot points mutations including 1896G→A,1899G→A,1762A→T, 1764G→A of women in HBIG group and those in no-HBIG group at delivery were 9.4% and 14.9%, 0 and 2. 1%, 7.5%0 and 10.6%, 5.7% and 12.8%, respectively,which were all not significantly different ( P＞0.05). Conclusions Antepartum interruption of HBV intrauterine infection with HBIG may not raise the nucleotide mutations in precore and BCP regions of HBV DNA. On the other hand, antepartum interruption may decrease mutations of hot points in the precore and BCP regions of HBV DNA.