Metabolics syndrome pediatrics

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...

OBJECTIVE: Obstructive sleep apnea (OSA) is a prevalent condition, especially in children with obesity, and is associated with increased risk for metabolic syndrome (MetS). Angiopoietins have been identified as potential biomarkers of endothelial dysfunction and MetS. In adults, angiopoietin-2 (Ang-2) and its soluble receptor (sTie-2) are associated with diabetes, hypertension, and obesity and could be increased in children with OSA and obesity, particularly those with evidence of cardiometabolic alterations...

BACKGROUND: Similarly to psoriasis in adult, Recent research has linked psoriasis to several comorbidities in children. Our objective was to describe comorbidities associated with pediatric psoriasis, to investigate their relationship with psoriasis characteristics and severity, and to perform a review of the literature. METHODS: A cross-sectional study was performed on a sample of Moroccan psoriatic children, during two years 2014 -2016. RESULTS: 64 psoriatic children had metabolic comorbidities in association with psoriasis, 20 children had non-metabolic comorbidities and 76 children didn't have any comorbidities...

Pediatric overweight and obesity is an emerging public health priority as rates have rapidly increased worldwide. Obesity is often clustered with other metabolic abnormalities including hypertension, dyslipidemia, and insulin resistance, leading to increased risk of cardiovascular disease. This cluster of risk factors, termed the metabolic syndrome, has traditionally been reported in adults. However, with the increased prevalence of pediatric obesity, the metabolic syndrome is now evident in children and adolescents...

OBJECTIVE: The development of these guidelines is mandated by the American Association of Clinical Endocrinologists (AACE) Board of Directors and American College of Endocrinology (ACE) Board of Trustees and adheres with published AACE protocols for the standardized production of clinical practice guidelines (CPGs). METHODS: Recommendations are based on diligent reviews of the clinical evidence with transparent incorporation of subjective factors, according to established AACE/ACE guidelines for guidelines protocols...

BACKGROUND AND AIM: Short bowel syndrome (SBS) is a common cause of intestinal failure and can be divided into three types depending on intestinal anatomy. Gut dysbiosis has been observed in pediatric SBS patients and is associated with impaired outcome. Little is known about the changes in gut microbiota of adult SBS patients. Therefore, we aim to characterize the fecal microbiota of adult patients with different types of SBS. METHODS: Fifteen fecal samples from healthy controls and adult patients with type II or type III SBS were collected (five in each group)...

INTRODUCTION: We present a child with unexplained splenomegaly to highlight this feature as a presenting sign of the RASopathy CBL syndrome and to draw attention to the power and utility of next generation genomic sequencing for providing rapid diagnosis and critical information to guide care in the pediatric clinical setting. CLINICAL REPORT: A 7-year-old boy presented with unexplained splenomegaly, attention deficit hyperactivity disorder, mild learning difficulties, easy bruising, mild thrombocytopenia, and subtle dysmorphic features...

INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...