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ANTHROPOMETRIC AND DIETARY ASSESSMENT OF PATIENTS WITH GLYCOGENOSIS TYPE I

Jorge,Natália Bauab; Tommaso,Adriana Maria Alves de; Hessel,Gabriel

ABSTRACT Objective: To perform anthropometric and dietary evaluation of patients with glycogenosis type Ia and Ib. Methods: This cross-sectional study is composed of a sample of 11 patients with glycogenosis divided into two subgroups according to the classification of glycogenosis (type Ia=5 and type Ib=6), aged between 4 and 20 years. The analyzed anthropometric variables were weight, height, body mass index,...

Date: 2021   |   Origin: Oasisbr

Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and la...

Porta,Gilda; Carvalho,Elisa de; Santos,Jorge L.; Gama,Jorge; Borges,Cristian V.; Seixas,Renata B.P.M.; Ferreira,Alexandre R.; Miura,Irene K.

Abstract Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, bio...

Date: 2019   |   Origin: Oasisbr

PREVALENCE OF HEPATIC STEATOSIS AMONG CHILDREN AND ADOLESCENTS WITH CYSTIC FIBR...

Gobato,Amanda Oliva; Vasques,Ana Carolina Junqueira; Ribeiro,Antonio Fernando; Yamada,Roberto Massao; Hessel,Gabriel

ABSTRACT Objective: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status. Methods: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps ...

Date: 2019   |   Origin: Oasisbr

PRE AND POST-OPERATIVE OTORHINOLARYNGOLOGY SURGERY CARE IN PATIENTS WITH GLYCOG...

Tommaso,Adriana Maria Alves de; Hessel,Gabriel; Riccetto,Adriana Gut; Semenzati,Graziela de Oliveira; Gusmão,Reinaldo Jordão

ABSTRACT Objective: To discuss aspects of pre and post-operative otorhinolaryngology surgery in patients with glycogen storage disease type 1b. Case description: Description of three clinical cases with probable glycogen storage disease type 1b who underwent otorhinolaryngology surgery, showing the importance of multidisciplinary interaction to avoid episodes of hypoglycemia. Comments: Patients with glycogen st...

Date: 2019   |   Origin: Oasisbr

Selenium supplementation in pediatric patients using parenteral nutrition: Is i...

Freitas,Renata Germano Borges de Oliveira Nascimento; Nogueira,Roberto José Negrão; Hessel,Gabriel

Summary Objective: To analyze the nutritional status of selenium and verify the effect of its supplementation in pediatric patients during 14 days of parenteral nutrition (PN). Method: This is a series of cases with patients followed for two weeks while using PN. Data collection was performed at the beginning (T0), in the 7th (T1) and 14th days of PN (T2). The supplemented group received 2 µg/kg/day of selenous...

Date: 2018   |   Origin: Oasisbr

IMPORTÂNCIA DA BIÓPSIA HEPÁTICA NO DIAGNÓSTICO DA DEFICIÊNCIA DE LIPASE ÁCIDA L...

Tommaso,Adriana Maria Alves De; Barra,Flávia Fonseca de Carvalho; Hessel,Gabriel; Moreno,Carolina Araújo; Giugliani,Roberto

RESUMO Objetivo: Descrever a doença de depósito de ésteres de colesterol (DDEC) e a importância da biópsia hepática na realização do diagnóstico. Descrição do caso: Paciente feminina, dois anos e dez meses de idade, com queixa de aumento do volume abdominal secundário à hepatomegalia há quatro meses. Ultrassonografia abdominal demonstrou hepatomegalia e esteatose hepática. Exames laboratoriais mostraram aumento...

Date: 2018   |   Origin: Oasisbr

Brazilian guidelines for the diagnosis and treatment of cystic fibrosis

Athanazio,Rodrigo Abensur; Silva Filho,Luiz Vicente Ribeiro Ferreira da; Vergara,Alberto Andrade; Ribeiro,Antônio Fernando; Riedi,Carlos Antônio

ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening ...

Date: 2017   |   Origin: Oasisbr

Factors impacting the growth and nutritional status of cystic fibrosis patients...

Hortencio,Taís Daiene Russo; Nogueira,Roberto José Negrão; Marson,Fernando Augusto de Lima; Hessel,Gabriel; Ribeiro,José Dirceu

OBJECTIVE: The aim of this study was to evaluate by clinical and laboratory parameters how cystic fibrosis (CF) affects growth and nutritional status of children who were undergoing CF treatment but did not receive newborn screening. METHODS: A historical cohort study of 52 CF patients younger than 10 years of age were followed in a reference center in Campinas, Southeast Brazil. Anthropometric measurements wer...

Date: 2015   |   Origin: Oasisbr

Metabolic syndrome and insulin resistance in obese adolescents

Gobato,Amanda Oliva; Vasques,Ana Carolina J.; Zambon,Mariana Porto; Barros Filho,Antonio de Azevedo; Hessel,Gabriel

Objective:To verify the prevalence of metabolic syndrome and insulin resistance in obese adolescents and its relationship with different body composition indicators.Methods: A cross-sectional study comprising 79 adolescents aged ten to 18 years old. The assessed body composition indicators were: body mass index (BMI), body fat percentage, abdominal circumference, and subcutaneous fat. The metabolic syndrome was...

Date: 2014   |   Origin: Oasisbr

Selenium deficiency and the effects of supplementation on preterm infants

Freitas,Renata Germano B. O. N.; Nogueira,Roberto José N.; Antonio,Maria ngela R. G. M.; Barros-Filho,Antonio de Azevedo; Hessel,Gabriel

Objective: This study aimed to review the literature about blood concentrations of selenium associated with gestational age, feeding, supplementation and related clinical features in preterm infants.Data sources: Systematic review in the following databases: MEDLINE, PubMed, Google academics, SciELO. org, ScienceDirect (Elsevier) and CINAHL-Plus with Full Text (EBSCO). Articles published up to January 2013 with...

Date: 2014   |   Origin: Oasisbr

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