Prevelance of hepatitis D co-enfection in children with hepatitis B infection: Cross-sectional analyses from Western Turkey

WOS: 000326481900008PubMed ID: 24254267Background/aims: Effective hepatitis B virus control has warranted a decline in hepatitis B virus prevalence over the world with a relevant reduction in hepatitis B virus-associated delta hepatitis. However, despite the dramatic decline in hepatitis D virus infection rate, no further decrease was recorded after 2000. This cross-sectional study aims to investigate: I- The prevalence of hepatitis D virus co-infection in children with hepatitis B virus infection in Western Turkey; II- The influence of neonatal hepatitis B virus vaccination on hepatitis D virus co-infection rate; and III- The impact of co-infection on prognosis of liver disease. Materials and Methods: Serological markers of hepatitis B virus and hepatitis D virus infections were determined by ELISA in patients with chronic hepatitis during immune tolerance, immunoactive, HBeAg-negative chronic, and inactive carrier state. Delta co-infection rate was evaluated in two groups, children born before and after the national neonatal mass vaccination has started (before and after 2000). Viral load, serum alanine aminotransferase, and histological grade were evaluated in co-infected cases. Results: Overall hepatitis delta virus infection rate was 1,76% (3/170); two patients with eAg-negative chronic hepatitis B and one patient in the immunoactive phase were infected with hepatitis D virus. Mean fibrosis score of hepatitis D virus -infected cases and hepatitis B virus -infected counterparts were 4 +/- 1,7 and 1,3 +/- 1, respectively (p: 0,006). Hepatitis D virus infection was detected in 2 out of 158 children born before and in 1 of 12 born after the neonatal vaccination program. Hepatitis B e-antibody was detected in two patients with delta co-infection (11 and 6 years old), and all mothers of delta hepatitis cases were chronically hepatitis B virus-infected. Conclusions: Delta hepatitis is rare among hepatitis B virus-infected children in the Western region of Turkey. Despite the success of the national vaccination program, delta hepatitis is not a vanishing disease and it has a grave prognosis due to development of early cirrhosis

Mannose-binding lectin gene polymorphism and chronic hepatitis B infection in children

Background/Aims: Mannose-binding lectin (MBL) is a member of innate immune system that activates complement system through lectin pathway. MBL deficiency is associated with susceptibility to infectious diseases. In this study, the relation between MBL gene polymorphism and chronic hepatitis B infection in children is evaluated. Patients and Methods: The study included 67 children with chronic hepatitis B and 99 healthy controls. The hepatitis B patients were divided into immuntolerant, chronic inactive, and treatment groups according to their laboratory findings. MBL gene codon 52, 54, and 57 polymorphisms were studied with polymerase chain reaction in all patients and controls. The associations of MBL gene polymorphism with clinical, laboratory, and histopathologic findings were evaluated. Results: Homozygous codon 54 polymorphism of MBL was found significantly higher in chronic hepatitis B patients than controls. Rate of the polymorphism was similar in all groups and, responsive and nonresponsive patients in the treatment group. Conclusions: The hepatitis B patients who are homozygous for codon 54 of MBL are prone to develop chronic infection. Longitudinal studies with larger groups are needed

Successful Management of L-Asparaginase-Associated Pancreatitis With Octreotide and Pegylated Asparaginase in 2 Patients With Acute Lymphoblastic Leukemia: Is There a Different Rare Warning Sign of Hypoglycemia for L-Asparaginase-Associated Pancreatitis?

L-Asparaginase (L-Asp) is a critical component of chemotherapy for acute lymphoblastic leukemia (ALL). However, toxic effects associated with L-Asp, such as hepatic dysfunction, pancreatitis, hypercholesterolemia, and hyperglycemia, have occurred. In addition, acute pancreatitis is a significant life-threatening adverse event associated with ALL. We describe 2 patients with ALL who had L-Asp-associated pancreatitis (AAP), with one patient presenting with hyperglycemia and the other presenting with hypoglycemia during induction treatment. When octreotide was administered to both of these patients, the clinical findings and laboratory data were improved. AAP was not repeated after treatment with pegylated asparaginase. Although AAP has a high risk of mortality and morbidity in childhood, APP treatment with appropriate agents, such as octreotide, can be successful. (C) 2020 Elsevier Inc

Histological Chorioamnionitis: Effects on Premature Delivery and Neonatal Prognosis

WOS: 000323359600009PubMed ID: 23639744Background: Chorioamnionitis is closely related to premature birth and has negative effects on neonatal morbidity and mortality. Methods: In this prospective study, 43 mothers who delivered earlier than 35 gestational weeks and their 57 infants were evaluated clinically and with laboratory findings. Placentas and umbilical cords were investigated histopathologically for chorioamnionitis and funisitis. Results: The overall frequency of clinical and histological chorioamnionitis (HCA) was 8.3% and 23.2%, respectively. The frequency of HCA was 47.3% and 83.3% in mothers delivered <32 weeks and <30 weeks, respectively. Maternal demographic and clinical findings and also leukocyte and C-reactive protein values were not indicative of HCA. Infants of mothers with HCA had significantly lower Apgar scores together with higher SNAP-PE-II and CRIB scores. These infants had increased mechanical ventilator and surfactant requirements, higher incidences of patent ductus arteriosus, early sepsis, and bronchopulmonary dysplasia, and higher mortality rates. The effect of HCA on neonatal morbidity and mortality was more prominent than the effect of low birthweight and lower gestational age. Conclusion: Chorioamnionitis not only causes premature deliveries, but is also associated with neonatal complications and increased mortality. Clinical findings and infectious markers in mother or infant do not predict the diagnosis of histological chorioamnionitis. Therefore, placental histopathology may have a role in predicting neonatal outcome in premature deliveries, especially those below 30 weeks. Copyright (c) 2013, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved

Molecular Investigations to Improve Diagnostic Accuracy in Patients With ARC Syndrome

Arthrogryposis, Renal dysfunction and Cholestasis (ARC) syndrome is a multi-system autosomal recessive disorder caused by germline mutations in VPS33B. The detection of germline VPS33B mutations removes the need for diagnostic organ biopsies (these carry a >50% risk of life-threatening haemorrhage due to platelet dysfunction); however, VPS33B mutations are not detectable in similar to 25% of patients. In order further to define the molecular basis of ARC we performed mutation analysis and mRNA and protein studies in patients with a clinical diagnosis of ARC. Here we report novel mutations in VPS33B in patients from Eastern Europe and South East Asia. One of the mutations was present in 7 unrelated Korean patients. Reduced expression of VPS33B and cellular phenotype was detected in fibroblasts from patients clinically diagnosed with ARC with and without known VPS33B mutations. One mutation-negative patient was found to have normal mRNA and protein levels. This patient`s clinical condition improved and he is alive at the age of 2.5 years. Thus we show that all patients with a classical clinical course of ARC had decreased expression of VPS33B whereas normal VPS33B expression was associated with good prognosis despite initial diagnosis of ARC. (C) 2008 Wiley-Liss, Inc.Hershkovitz D, 2008, ARCH DERMATOL, V144, P334Gissen P, 2006, HUM GENET, V120, P396, DOI 10.1007/s00439-006-0232-zBull LN, 2006, J PEDIATR-US, V148, P269, DOI 10.1016/j.jpeds.2005.10.005Lo B, 2005, BLOOD, V106, P4159, DOI 10.1182/blood-2005-04-1356Maina EN, 2005, ONCOGENE, V24, P4549, DOI 10.1038/sj.onc.1208649Gissen P, 2005, HUM MOL GENET, V14, P1261, DOI 10.1093/hmg/ddi137Gissen P, 2004, NAT GENET, V36, P400, DOI 10.1038/ng1325HARLOW E, 1998, USING ANTIBODIES LABHORSLEN SP, 1994, J MED GENET, V31, P62KARNOVSKY MJ, 1971, J CELL BIOL, V284, P146

The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease

Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study