Global burden of disease attributable to metabolic risk factors in adolescents and young adults aged 15-39, 1990-2021.

Background: Metabolic risk factors are a significant cause of global burden among adolescents and young adults, but there is a lack of attention to the burden attributable to these metabolic risk factors globally.

Aims: This study aims to provide comprehensive estimates of five important metabolic risk factors and the attributable disease burden in people aged 15-39 years from 1990 to 2021, based on the Global Burden of Disease Study (GBD) database.

Methods: Global total deaths and disability-adjusted life years (DALYs) were used to describe the burden attributable to five common metabolic risk factors, including high fasting plasma glucose (FPG), high low-density lipoprotein cholesterol (LDL-C), high systolic blood pressure (SBP), high body mass index (BMI), and kidney dysfunction, in adolescents and young adults.

Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents.

Objective: To determine predictors of native liver survival (NLS) in children and adolescents with autoimmune hepatitis (AIH).

Study Design: The medical records of children and adolescents with AIH were reviewed. A questionnaire was used to collect data on clinical presentation, biochemical and histologic findings, and treatment.

Glycogen storage diseases: Twenty-seven new variants in a cohort of 125 patients.

Background: Hepatic glycogen storage diseases (GSDs) are a group of rare genetic disorders in which glycogen cannot be metabolized to glucose in the liver because of enzyme deficiencies along the glycogenolytic pathway. GSDs are well-recognized diseases that can occur without the full spectrum, and with overlapping in symptoms.

Methods: We analyzed a cohort of 125 patients with suspected hepatic GSD through a next-generation sequencing (NGS) gene panel in Ion Torrent platform.

"Milk of calcium": a rare presentation of calcinosis.

Rheumatic diseases such as juvenile dermatomyositis (JDM), juvenile sistemic lupus erythematosus (JSLE) and sistemic sclerosis may have calcium deposits in the subcutaneous and muscle tissues known as calcinosis. Extensive calcium-laden fluid collections referred as "milk of calcium" are rare forms of calcinosis in JDM. We describe a 15-year old patient with overlap syndrome (sclerodermatomyositis and JSLE), whose magnetic resonance imaging (MRI) showed perimusculares fluid collections in the lower limbs.

Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl.

Context: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasturés syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener's granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution.

Gastric-tube esophagoplasty in children.

From 1979 to 1996, 19 patients underwent gastric-tube esophagoplasty. There were 10 boys and 9 girls, aged between 1 year 4 months and 4 years 11 months at the time of surgery. In 1 patient the esophagoplasty was performed due to a long stenosis secondary to reflux; 3 others had caustic stenoses; and the remaining patients had esophageal atresia.

Esophageal carcinoma secondary to a chemical injury in a child.

Twin sisters were clinically and endoscopically followed due to chemical injuries to the esophagus after ingestion of muriatic acid at 10 months of age. One of the girls developed esophageal carcinoma 10 years later and died after esophagectomy because of progression of the disease. Her twin sister has a severe stenosis at the distal esophagus and is waiting for surgical treatment.

Enteropathogens associated with acute diarrheal disease in urban infants in São Paulo, Brazil.

To determine the prevalence and epidemiology of enteropathogens in acute infantile diarrhea, 500 infants less than or equal to 12 months of age with diarrhea and 500 age-matched control subjects coming to a São Paulo emergency room were studied. Enteropathogens were identified in 55% of case infants and 10% of controls; enteropathogenic Escherichia coli (EPEC) of classic EPEC serotypes producing EPEC adherence factor (EAF) (26% of case infants), rotavirus (14%), Salmonella species (8%), enterotoxigenic E. coli (7%), and Shigella species (5%) were associated with diarrhea.

[Sickle cell anemia: cerebral angiography changes].

Two cases of sickle cell disease with neurological complications are described. Cerebral angiograms were performed and a characteristic moyamoya-like angiographic pattern was demonstrated. This pattern results from a compensatory vascularization which may follow occlusion of the arteries at the base of the brain in several diseases.

[Incidence of rotavirus in the Menino Jesus Pediatric Hospital (SP): ELISA--a practical and efficient method for its detection].

Seventy infants and children with age from 2 months to 4 years old were admitted to "Hospital Infantil Menino Jesus", in São Paulo, Brazil, in the period from March 1983 to June 1984 with acute diarrhea. Feces from these patients were analysed in the search of rotavirus and enteropathogenic bacteria (Shigella, Salmonella and E. coli), for the purpose of defining the role of rotavirus as an agent of acute diarrhea in infants and young children in this country.