Metabolics syndrome pediatrics

The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...

OBJECTIVES: To characterize children and adolescents with type 2 diabetes mellitus (T2DM) insured by a large health maintenance organization, and to identify variables associated with treatment quality and disease outcome. STUDY DESIGN: Children and adolescents diagnosed with T2DM over a 9-year period were identified from the database of Clalit Health Services, a large health maintenance organization in Israel (1 213 362 members aged 0-18 years). Demographic, anthropometric, clinical, and laboratory data were analyzed...

Epilepsy with mental retardation limited to females (EFMR) is a syndrome characterized by early onset heat-sensitive epilepsy of infancy or early childhood and generally limited to females, which previously reported that the cadherin gene superfamily subtype protocadherin 19 (PCDH19) gene is its pathogenic gene. We retrospectively analyzed the clinical data for 2 cases of EFMR patients with PCDH19 mutation diagnosed by Department of Pediatric Neurology of Xiangya Hospital, Central South University in 2015. Literature on PubMed, OMIM and HGMD relevant to this syndrome was reviewed, and the clinical characteristics were summarized accordingly...

Correction of craniosynostosis can result in blood loss when the patient already has physiologic anemia. The aim of this study was to determine whether patients benefit from early blood transfusion and whether the timing of blood transfusion affects metabolic disturbances and the postoperative course. In this retrospective review, 71 patients who underwent open calvarial vault remodeling for correction of craniosynostosis were separated into 2 groups according to whether they received blood transfusions early (within the first 30 minutes of surgery) or later (after the first 30 minutes of surgery)...

BACKGROUND: The incidence of metabolic syndrome (MetS), is rapidly increasing in developing countries. However, the epidemiology of MetS is not well reported in the pediatric and young adult population. We determined the prevalence of MetS and its components among overweight and obese Nigerian adolescents and young adults presenting for university admission. MATERIALS AND METHODS: A cross-sectional study of overweight and obese adolescents and young adults was performed...

BACKGROUND: Individuals with Down syndrome (DS) experience various comorbidities in excess of the prevalence seen among the non-DS population. However, the extent of the excess burden of comorbidities specifically within commercially and publicly insured DS populations aged < 21 years is not currently known. OBJECTIVES: To (a) describe the most common diagnoses among individuals with DS who have either commercial or Medicaid insurance and (b) compare the prevalence of those diagnoses between DS cases and non-DS controls...

Folate is an important B vitamin naturally found in the human diet and plays a critical role in methylation of nucleic acids. Indeed, abnormalities in this major epigenetic mechanism play a pivotal role in the pathogenesis of cognitive deficit and intellectual disability in humans. The most common cause of cognitive dysfunction in children is Down syndrome (DS). Since folate deficiency is very common among the pediatric population, we questioned whether chronic folate deficiency (CFD) exacerbates cognitive dysfunction in a mouse model of DS...

In October 2016, the American Association for Cancer Research held a meeting of international childhood cancer predisposition syndrome experts to evaluate the current knowledge of these syndromes and to propose consensus surveillance recommendations. Herein, we summarize clinical and genetic aspects of RASopathies and Sotos, Weaver, Rubinstein-Taybi, Schinzel-Giedion, and NKX2-1 syndromes as well as specific metabolic disorders known to be associated with increased childhood cancer risk. In addition, the expert panel reviewed whether sufficient data exist to make a recommendation that all patients with these disorders be offered cancer surveillance...

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is an underrecognized co-morbidity of obesity. The characteristic features and severity of NAFLD in severe childhood obesity remain unknown. OBJECTIVES: To investigate features of NAFLD in obese children and identify predictors of significant disease. SETTING: Academic center with a standardized pathway for pediatric bariatric surgery and a dedicated multidisciplinary team. DESIGN: This is a baseline cross-sectional analysis for a data set obtained from a prospective clinical outcome study that included severely obese children (≤14 yr of age) and adolescents (14-21 yr of age) who underwent laparoscopic sleeve gastrectomy between March 2008 and March 2015...

Objective: To invesigate the epidemic status of the metabolic syndrome (MS) among mainland Chinese adolescents aged 10-17 in 2010-2012. Methods: Data were collected from Chinese Nutrition and Health Surveillance in 2010-2012. Multi-stage stratified proportion to the population cluster random sampling method was conducted to determine 16 872 adolescents in 150 counties from 31 provinces in mainland China. The epidemic status of metabolic syndrome was analyzed by China criterion (defined by Chinese Pediatric Society, Chinese Medical Association) and Cook criterion, respectively...

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis are two rare but life-threatening diseases characterized by detachment of epidermis, bullous skin lesions and mucous membrane erosions. Anti-epileptic drugs are highly suspected to be the causative agents. Although carbamazepine (CBZ) is the most associated anti-epileptic drug, oxcarbazepine (OXC), which is a monohydrated derivative of CBZ, is proposed to be safer because of the different metabolism of the two drugs. Herein, we report a case of SJS induced by oxcarbazepine...

OBJECTIVES: Hemoglobin (Hb) and red blood cell distribution width (RDW) have been reported to be a risk marker of metabolic syndrome (MS) and nonalcoholic fatty liver disease (NAFLD). No study exists on pediatric populations. We aimed to determine the association between hematological parameters, and the severity of disease in children with biopsy proven NAFLD. METHODS: 117 children (85 males, mean age 12 years) with ultrasound (US) evidence of NAFLD undergoing liver biopsy for diagnosis of non alcoholic steatohepatitis (NASH), were prospectively enrolled between January 2011 and May 2013 in the setting of a tertiary care centre...

Amelogenesis imperfecta (AI) is a heterogeneous group of inherited dental enamel defects. It has rarely been reported in association with multiorgan syndromes and metabolic disorders. The metabolic disorders that have been reported in association with AI include hypocalciuria, impaired urinary concentrating ability, and Bartter-like syndrome. In literature, only three cases of AI and distal renal tubular acidosis (dRTA) have been described: two cases in adults and a solitary case in the pediatric age group...

PURPOSE: The aim of this study was to verify if serum chemerin in children correlates with body weight, fat mass, selected inflammatory markers, parameters of liver function, lipid and glucose metabolism. MATERIALS AND METHODS: The study included children aged 5-17 years with normal body weight (<85th BMI percentile, n=43) or overweight (≥85th BMI percentile, n=58). Serum concentrations of chemerin were determined with ELISA. RESULTS: Children with excess body weight presented with significantly higher serum concentrations of chemerin...

OBJECTIVES: Metabolic syndrome components, such as being overweight or having hypertension, hyperlipidemia, or diabetes mellitus, are common complications after liver transplant in pediatric patients with probable multifactorial causes and increase the risk of cardiovascular complications in adulthood. In this study, our aim was to evaluate the prevalence of these components both before and after transplant surgery. MATERIALS AND METHODS: Our study included all children having liver transplant at our institution over a period of 20 years who were under 18 years old and had at least 6 months of posttransplant follow-up...

BACKGROUND: Cardiovascular and metabolic consequences of obesity in children, unlike adults, are still not well understood nor have they been subject to extensive research in Africa. We aimed to identify the cardio-metabolic complications associated with childhood obesity at the early phase of the management of obese children in a reference center in Cameroon. METHODS: In this cross-sectional study conducted from November 2013 to September 2014 and based on World Health Organization (WHO) classification of Obesity (BMI > 3SD under 5 years and BMI > 2SD from 5 and above), we included children aged 3 to 17 years who were being followed up for obesity at the pediatric endocrinology unit of the Mother and Child Center of the Chantal BIYA Foundation in Yaounde, Cameroon...

Lysosomal storage disorders (LSDs) are a group of about 50 genetic metabolic disorders, mainly affecting children, sharing the inability to degrade specific endolysosomal substrates. This results in failure of cellular functions in many organs, including brain that in most patients may go through progressive neurodegeneration. In this study, we analyzed the brain of the mouse model for Hunter syndrome, a LSD mostly presenting with neurological involvement. Whole transcriptome analysis of the cerebral cortex and midbrain/diencephalon/hippocampus areas was performed through RNA-seq...

Loss of lean body mass is a relevant component of the cachexia, or protein energy wasting (PEW), syndrome. Reduced muscle mass seems to be the most solid criterion for the presence of cachexia/PEW in patients with chronic kidney disease (CKD), and those with greater muscle mass loss have a higher risk of death. Children with CKD have many risk factors for lean mass and muscle wasting, including poor appetite, inflammation, growth hormone resistance, and metabolic acidosis. Mortality risks in patients with CKD increases as body mass index (BMI) and weight decreases...

OBJECTIVES: 'Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections' (PANDAS) identified a unique subgroup of patients with abrupt onset of obsessive compulsive disorder (OCD) symptoms clinically related to Streptococcus infection and accompanied by neuropsychological and motor symptoms. After almost 20 years, PANDAS has not been accepted as distinct disorder and new criteria for paediatric acute-onset neuropsychiatric syndrome (PANS) have been replaced it, highlighting the fact that several agents rather than only Streptococcus might be involved...

Non-alcoholic steatohepatitis (NASH) is the more aggressive form of non-alcoholic fatty liver disease (NAFLD). NASH can progress to hepatic fibrosis, cirrhosis, portal hypertension and primary liver cancer. Therapy is evolving with a substantial number of trials of promising new agents now in progress. In this article however, we will examine data for several older forms of therapy which have been fairly extensively studied over the years: Polyunsaturated Fatty Acid (PUFA) supplements, vitamin E, insulin sensitizing agents with a focus on pioglitazone and statin agents...