Hypertriglyceridemia children

Objective: To analyze the efficacy and safety of the L-DEP regimen (asparaginase, liposome doxorubicin, etoposide and methylprednisolone) as a salvage therapy for the refractory primary hemophagocytic lymphohistocytosis triggered by Epstein-Barr virus infection (EBV-pHLH) in children. Methods: In this retrospective case study, clinical and laboratory data before and after L-DEP regimen of 4 children diagnosed with EBV-pHLH in Beijing Children's hospital between January 2016 and June 2022 were collected, and the efficacy and safety of L-DEP regimen for the treatment of EBV-pHLH were analyzed...

CONTEXT: Several studies have investigated the relationship between serum vitamin D and dyslipidemia in children and adolescents, but the findings have been contradictory. OBJECTIVE: The current systematic review and dose-response meta-analysis investigated the serum vitamin D - dyslipidemia relationship in children and adolescents. DATA SOURCES: ISI Web of Science, Scopus, MEDLINE (PubMed), EMBASE databases, and Google Scholar, were searched up to December 2022...

BACKGROUND AND AIM: APO CII, one of several cofactors which regulate lipoprotein lipase enzyme activity, plays an essential role in lipid metabolism. Deficiency of APO CII is an ultra-rare autosomal recessive cause of familial chylomicronemia syndrome. We present the long-term clinical outcomes of 12 children with APO CII deficiency. METHODS AND RESULTS: The data of children with genetically confirmed APO CII deficiency were evaluated retrospectively. Twelve children (8 females) with a mean follow-up of 10...

Objective: To analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH). Methods: This was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children's hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate. Results: Among all 9 cases, there were 6 males and 3 females, with the age ranged from 0...

BACKGROUND: Transient infantile hypertriglyceridemia (TIH) is a syndrome of hypertriglyceridemia, fatty liver, and deranged liver functions with progression to fibrosis and cirrhosis. It is an autosomal recessive disorder caused by mutations in Glycerol-3-phosphate dehydrogenase 1 gene present on Chromosome 12q12-q13, and has been reported in Israeli Arab families with high consanguinity. TIH is suspected by high serum triglyceride levels and steatosis on liver biopsy; however, diagnosis is confirmed on clinical exome sequencing ...

INTRODUCTION: Metabolic syndrome (MetS) is a cluster of risk factors that may predispose to cardiovascular diseases and type 2 diabetes mellitus. These risk factors include hypertension, central obesity, hyperglycaemia, hypertriglyceridemia and low high-density lipoprotein cholesterol (HDL-C). The presence of at least three of these risks factors defines MetS. Globally, MetS now affects children and adolescents with increasing obesity prevalence. This study determined the prevalence of MetS and associated risk factors among secondary school adolescents in Gombe Local Government Area(LGA), Northeast Nigeria...

PURPOSE: The PNOC001 phase II single-arm trial sought to estimate progression-free survival (PFS) associated with everolimus therapy for progressive/recurrent pediatric low-grade glioma (pLGG) on the basis of phosphatidylinositol 3-kinase (PI3K)/AKT/mammalian target of rapamycin (mTOR) pathway activation as measured by phosphorylated-ribosomal protein S6 and to identify prognostic and predictive biomarkers. PATIENTS AND METHODS: Patients, age 3-21 years, with progressive/recurrent pLGG received everolimus orally, 5 mg/m2 once daily...

OBJECTIVE: Fosinopril and amlodipine are commonly prescribed as first-line pharmacotherapeutic agents for pediatric hypertension, but there is a lack of comparative studies regarding the efficacy of these two drugs. We aimed to evaluate and compare the efficacy of fosinopril and amlodipine monotherapy in pediatric primary hypertension. METHODS: This was a single-center, bidirectional observational study. A total of 175 children and adolescents with primary hypertension receiving antihypertensive monotherapy from July 2020 to February 2023 were enrolled...

BACKGROUND: Combined use of inhibitors of mammalian target of rapamycin (mTOR) and vascular endothelial growth factor (VEGF-2) receptors is a potential strategy to overcome resistance to either class of drugs when used alone. PATIENTS AND METHODS: We designed a phase 1 trial to test the drug combination of a multikinase VEGF receptor 2 inhibitor, vandetanib, and an mTOR inhibitor, everolimus, in a pediatric and young adult patient cohort with advanced cancers. Exceptional responders were probed for tumor mutational profile to explore possible molecular mechanisms of response...

Diabetic ketoacidosis (DKA) was observed in a 12-year-old female child recently diagnosed with type 1 diabetes mellitus. A rise in triglyceride (TG) levels accompanied this. High TG levels were suspected due to the lipemic appearance of the serum. Acute pancreatitis and lipemia retinalis are potential complications for hypertriglyceridemia patients. Treating the DKA and insulin deficiency reverted the TG levels to normal without using lipid-lowering agents.

BACKGROUND: Atopic dermatitis (AD) is a common inflammatory skin disease that affects both children and adults. However, limited research has been conducted on gender differences in AD. OBJECTIVES: This study aimed to assess gender differences in adult AD patients, focusing on demographic and clinical features, comorbidities, and treatment approaches. METHODS: In this multicenter, observational, cross-sectional study, we enrolled 686 adult patients with AD (357 males and 329 females)...

OBJECTIVES: This study aimed to evaluate the side effect profile of soybean oil lipid injectable emulsion -(SO-ILE) and soybean oil, medium-chain triglyceride, olive oil, fish oil lipid injectable emulsion (SO,MCT,OO,FO-ILE) in critically ill children requiring parenteral nutrition (PN). METHODS: This is an observational study of children admitted to our pediatric intensive care unit requiring PN for ≥7 days. Patients were divided into 2 cohorts: SO,MCT,OO,FO-ILE (n = 34) and SO-ILE (n = 111)...

BACKGROUND: The established association between acute lymphoblastic leukemia (ALL) and hyperlipidemia has, in some studies, been linked to toxicities such as pancreatitis, thrombosis, and osteonecrosis. However, a systematic review investigating the incidence, management, and clinical implications of hyperlipidemia during childhood ALL treatment is lacking. OBJECTIVES: Systematically assess the incidence of hyperlipidemia during ALL treatment, explore associations with risk factors and severe toxicities (osteonecrosis, thrombosis, and pancreatitis), and review prevalent management strategies...

Long-term medication with valproic acid has been associated with acute pancreatitis. The purpose of this report is to gain insight into the features of this pancreatitis. A preregistered literature search (CRD42023438294) was performed on the National Library of Medicine, Excerpta Medica, Web of Science, and Google Scholar. Patients with alcohol abuse disorder, gallstone disease, hypertriglyceridemia or hypercalcemia, patients with acute valproic acid intoxication, and patients with a pre-existing pancreatitis were excluded...

OBJECTIVES: Cardiovascular diseases are the main causes of death in the world. They are associated with the presence of risk factors such as obesity and dyslipidemia. Our objectives were to verify association between body mass index (BMI) and abnormalities in children's lipid profile evaluated during well-child visits, seeking to identify the frequency of each disorder in this population. METHODS: A cross-sectional study examined anthropometric data and laboratory results of children aged 2 to 9 (n=363) at a pediatric clinic between 2014 and 2017...

Background : Lipoprotein lipase (LPL) deficiency is a genetic condition. Affected individuals typically develop symptoms related to severe and persistent hypertriglyceridemia, such as abdominal pain and recurrent pancreatitis, before 10 years of age. No pharmacological treatment sustainably lowering triglycerides (TGs) in LPL deficiency patients has been proven to be effective. This study investigated whether a long-chain triglyceride (LCT)-restricted, medium-chain triglyceride (MCT)-supplemented diet enables a meaningful reduction in TGs and reduces LPL-related symptoms in children with LPL deficiency...

BACKGROUND: A prospective evaluation of non-alcoholic fatty liver disease (NAFLD) during induction therapy for acute lymphoblastic leukemia (ALL) has not been performed. Herein, we prospectively investigated the frequency, risk factors, and outcomes of NAFLD during induction therapy in children and adolescents with B-cell precursor ALL (BCP-ALL). METHODS: This study enrolled 74 newly diagnosed BCP-ALL cases aged 1 year and older who were admitted to our department between January 2011 and December 2020...

INTRODUCTION: Data on the prevalence of pediatric fatty liver disease remain limited, partly due to challenges in diagnosis. A novel concept of metabolic-associated fatty liver disease (MAFLD) makes it possible to establish the diagnosis in overweight children with sufficiently elevated alanine aminotransferase (ALT). We investigated the prevalence, risk factors, and metabolic co-morbidities of MAFLD in a large group of overweight children. METHODS: Data on 703 patients aged 2-16 years examined due to overweight in different levels of healthcare in 2002-2020 were collected retrospectively from patient records...

OBJECTIVES: Severe and very severe hypertriglyceridemia although rare within the pediatric population occur more often among oncology patients, secondary to chemotherapeutic agents. Currently there exists minimal literature to guide management of severe hypertriglyceridemia among pediatric patients. Very-low-fat dietary restriction should be considered over nil per os (NPO) for initial management of severe hypertriglyceridemia in stable pediatric patients. Pediatricians caring for oncology patients must consider chylomicronemia as a potential etiology for presenting symptoms...

Hypertriglyceridemia has been recognized as a common complication of diabetes ketoacidosis (DKA), whereas severe hypertriglyceridemia, also known as diabetic lipemia, rarely occurs and is associated with an increasing risk of acute pancreatitis. We report the case of a 4-year-old girl with new-onset DKA associated with remarkable hypertriglyceridemia. Her serum triglyceride (TG) level was as high as 2490 mg/dL on admission and 11,072 mg/dL on day two during treatment with hydration and intravenous insulin infusion, whereas the critical situation was successfully stabilized by standard treatment for DKA without the occurrence of pancreatitis...