Infant increase liver enzymes

INTRODUCTION/AIMS: New therapeutic strategies to increase survival motor neuron protein levels in spinal muscular atrophy (SMA) have focused on replacing the SMN1 gene. Onasemnogene abeparvovec was approved by the US Food and Drug Administration in 2019 for treatment of children with SMA less than 2 years of age. Postmarketing studies are limited, especially outside of Europe and the United States. Herein we describe a single-center experience with onasemnogene abeparvovec from the Middle East...

BACKGROUND: Due to different growth and metabolic processes, reference values of alkaline phosphatase (AP) for children aged 3 month to 18 years are dependent on age and sex. They are not constant and differ from those of adults due to the growth processes taking place. Accordingly, reference levels of AP continuous across these ages were generated for boys and girls based on of a large German health- and population-based study, LIFE Child. We considered AP at different growth and Tanner stages and additionally its association with other anthropometric parameters...

BACKGROUND: The adverse health influences of polycyclic aromatic hydrocarbons (PAHs) exposures have been examined in several previous research. However, the evidence on the health influences of PAHs exposure during pregnancy and childhood is scarce, with no study on the infant's liver function. Therefore, in this study, the association of in-utero exposure to particulate matter-bound PAHs (PM-bound PAHs) on the umbilical liver enzymes was investigated. METHODS: A total of 450 mother-pair samples were assessed in this cross-sectional study in Sabzevar, Iran (2019-2021)...

BACKGROUND: Hepatitis B infection is one of the world's most serious public health problems, causing significant morbidity and mortality. More than 2 billion individuals around the world have been infected with the hepatitis B virus (HBV), and approximately 400 million people are chronically infected with the virus, with more than a million dying each year from hepatitis B virus-related liver disease. A newborn infant whose mother is positive for both HBsAg and HBeAg has a 90% chance of developing chronic infection by the age of 6...

UNLABELLED: Consumptive hypothyroidism (CH) is a rare and potentially overlooked complication of hepatic hemangiomas (HH) overexpressing the enzyme deiodinase, which converts thyroxine (T4) to reverse triiodothyronine (rT3). MATERIALS AND METHODS: Here, we report a case series of 3 patients and a systematic review of the literature. RESULTS: Hypothyroidism (mean serum TSH 52.03 mIU/L) was detected at a mean age of 4.6 months (range 3-6) in 3 infants with infantile hepatic hemangiomas, treated with thyroxine (mean dose 12 µg/kg/day)...

Autism spectrum disorder (ASD) is a neurodevelopmental disorder that affects 0.6%-1.7% of children. The etiology of autism is hypothesized to include both biological and environmental factors (Watts, 2008). In addition to the core symptoms of social-communication delay and restricted, repetitive interests, co-occurring irritability/aggression, hyperactivity, and insomnia negatively impact adaptive functioning and quality of life of patients and families. Despite years of effort, no pharmacologic agent has been found that targets the core symptoms of ASD...

Despite considerable progress in understanding drug metabolism in the human pediatric population, data remains scarce in preterm neonates. Improving our knowledge of the ADME properties in this vulnerable age group is of utmost importance to avoid suboptimal dosing, which may lead to adverse drug reactions. The juvenile (mini)pig is a representative model for hepatic drug metabolism in human neonates and infants, especially phase I reactions. However, the effect of prematurity on the onset of hepatic phase I and phase II enzyme activity has yet to be investigated in this animal model...

PURPOSE: Congenital diaphragmatic hernia (CDH) pathogenesis is poorly understood. We hypothesize that fetal CDH lungs are chronically hypoxic because of lung hypoplasia and tissue compression, affecting the cell bioenergetics as a possible explanation for abnormal lung development. METHODS: To investigate this theory, we conducted a study using the rat nitrofen model of CDH. We evaluated the bioenergetics status using H1 Nuclear magnetic resonance and studied the expression of enzymes involved in energy production, the hypoxia-inducible factor 1α, and the glucose transporter 1...

BACKGROUND: Propolis has become one of the most preferred supplements due to its beneficial biological properties. Organic (water and vegetable oils) and chemical (ethyl alcohol, propylene glycol, and glycerol) solvents are used for propolis extraction. However, the effects of these chemicals on health should be taken into account. OBJECTIVES: In this study, the effects of propolis extracts on health were evaluated. METHODS: 32 pregnant Wistar albino rats and 64 neonatal/young adults were given three different extractions of propolis (propylene glycol, water, and olive oil)...

Cholestasis refers to impaired bile flow from the liver to the intestine. In neonates, cholestasis causes poor growth and may progress to liver failure and death. Normal bile flow requires an intact liver-gut-microbiome axis, whereby liver-derived primary bile acids are transformed into secondary bile acids. Microbial bile salt hydrolase (BSH) enzymes are responsible for the first step, deconjugating glycine- and taurine-conjugated primary bile acids. Cholestatic neonates often are treated with the potent choleretic bile acid ursodeoxycholic acid (UDCA), although interactions between UDCA, gut microbes, and other bile acids are poorly understood...

OBJECTIVE: The aim of study was to investigate at what extent breastfeeding and vaginal delivery can increase mother-to-child transmission of cytomegalovirus (CMV) and to observe the clinical outcomes of postnatal infection in term or moderate and late preterm infants. METHODS: In this retrospective study of prospectively collected clinical data and serum samples, during 2012-2015, 380 women with CMV IgG positive/IgM negative and their 384 infants (4 twin pairs) with gestational age ≥32 weeks were included...

OBJECTIVES: Methotrexate (MTX) has been used as maintenance therapy for Crohn's disease (CD) in adults and children. However, there are only a few studies on the MTX's effectiveness in thiopurine-naïve CD adult patients and children. This study aimed to evaluate the MTX's effectiveness and safety as first immunomodulator for maintenance therapy in pediatric CD. METHODS: This retrospective cohort study recruited 64 pediatric CD patients treated with MTX as a first-line immunomodulator...

OBJECTIVES: We aimed to determine whether severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in pregnancy is associated with an increased risk of hypertensive disorders of pregnancy (HDP). METHODS: A multicenter retrospective cohort study of all pregnant patients who had SARS-CoV-2 testing and delivered in a large health system between March 2020 and March 2021. Cases were stratified into two groups: patients who tested positive for SARS-CoV-2 during pregnancy vs...

INTRODUCTION: Autoantibody testing has contributed to both biological and clinical insights in managing patients with liver disease. These autoantibodies often have clinical value for the diagnosis, disease activity and/or prognosis. AIM OF THE STUDY: We aimed to investigate the potential application of auto-antibodies in different etiologies of non-autoimmune liver diseases. MATERIAL AND METHODS: This study was conducted on 53 infants and children with chronic liver diseases...

OBJECTIVE: The rate of preeclampsia with severe features has increased. Previous studies have shown elevated liver enzymes are an indicator of worsening hypertensive disease of pregnancy and adverse outcomes, therefore leading to their inclusion as a diagnostic criterion for severe features of preeclampsia. Despite this, there are limited data to support an aspartate aminotransferase (AST) or alanine aminotransferase (ALT) concentration ≥ two times the upper limit of normal as the critical point at which maternal harm from ongoing pregnancy exceeds neonatal harm from delivery...

BACKGROUND: Olipudase alfa is a recombinant human acid sphingomyelinase (ASM) enzyme replacement therapy (ERT) for non-central-nervous-system manifestations of acid sphingomyelinase deficiency (ASMD). We report 2-year cumulative safety and efficacy data after olipudase alfa treatment in 20 children (four adolescents [12-17 year], nine children [6-11 year], and seven infants/early child [1-5 year]) with baseline splenomegaly and growth deficits who completed the 1-year ASCEND-Peds clinical trial (NCT02292654) and who continue to receive olipudase alfa in a long-term study (NCT02004704)...

OBJECTIVE: Bile acid intermediates, 3α,7α,12α-trihydroxycholestanoic acid (THCA) and 3α,7α-dihydroxycholestanoic acid (DHCA), are metabolized in peroxisomes. Some peroxisomal disorders (PDs), such as Zellweger spectrum disorder (ZSD), show an accumulation of bile acid intermediates. In particular, ABCD3 deficiency and acyl-CoA-oxidase 2 deficiency are characterized by these metabolite abnormalities. In patients with ZSD, levels of bile acid intermediates can be lowered by a primary bile acid supplementation treatment; therefore, measuring their levels could help evaluate treatment effectiveness...

Nudix hydrolase 7 (NUDT7) is an enzyme that hydrolyzes CoA species, is highly expressed in the liver, and resides in the peroxisomes. Peroxisomes are organelles where the preferential oxidation of dicarboxylic fatty acids occurs and where the hepatic synthesis of the primary bile acids cholic acid and chenodeoxycholic acid is completed. We previously showed that liver-specific overexpression of NUDT7 affects peroxisomal lipid metabolism, but does not prevent the increase in total liver CoA levels that occurs during fasting...

Background: While prior research has shown differences in the risk of malaria infection and sickness between males and females, little is known about sex differences in vaccine-induced immunity to malaria. Identifying such differences could elucidate important aspects of malaria biology and facilitate development of improved approaches to malaria vaccination. Methods: Using a standardized enzyme-linked immunosorbent assay, IgG antibodies to the major surface protein on Plasmodium falciparum (Pf) sporozoites (SPZ), the Pf circumsporozoite protein (PfCSP), were measured before and two weeks after administration of a PfSPZ-based malaria vaccine (PfSPZ Vaccine) to 5-month to 61-year-olds in 11 clinical trials in Germany, the US and five countries in Africa, to determine if there were differences in vaccine elicited antibody response between males and females and if these differences were associated with differential protection against naturally transmitted Pf malaria (Africa) or controlled human malaria infection (Germany, the US and Africa)...

RATIONALE: HELLP syndrome, a rare but serious obstetric complication, is often overdiagnosed due to its nonspecific symptoms and inappropriate clinical testing. PATIENT CONCERNS: A 30-year-old nulliparous pregnant Chinese woman at gestational age of 28+1 weeks was admitted to our hospital because Doppler ultrasonography at a local hospital had detected loss of fetal umbilical artery end-diastolic blood flow lasting 12 hours. On admission to our hospital, the patient showed elevated blood pressure (148/84 mm Hg), but blood pressure and laboratory indicators after admission were normal...