ABSTRACT
A doença celíaca (DC) é uma enteropatia caracterizada pela intolerância permanente ao glúten desencadeada por mecanismos autoimunes nos indivíduos geneticamente predispostos. A DC com seu quadro clínico típico e principalmente atípico tem se mostrado mais frequente do que se imaginava. Seu diagnóstico é baseado em suspeita clínica, exames sorológicos e biópsia intestinal. Devido à evolução dos marcadoressorológicos e revisão dos critérios diagnósticos, discute-se sobre a real necessidade da realização da biópsia intestinal em casos selecionados. O tratamento da DC continua sendo a dieta isenta de glúten.
Celiac disease (CD) is an enteropathy characterized by permanent intolerance to gluten triggered by autoimmune mechanisms in genetically predisposed individuals. The frequency of CD, with its typical clinical condition and mainly atypical, has been higher than expected. Its diagnosis is based on clinical suspicion, serologic tests, and intestinal biopsy. The evolution of the knowledge about serological markers and revision of thediagnostic criteria prompts questions about the real need of intestinal biopsy in selected cases. The treatment of CD remains the gluten-free diet.
ABSTRACT
Introdução: a nutrição parenteral (NP) é utilizada no tratamento de crianças que não podem ser alimentadas completamente por via oral ou enteral. Dessa forma, seu principal objetivo é manter ou restituir o estado nutricional ideal. Devido à particularidade da faixa etária pediátrica em relação às necessidades nutricionais, seu uso pode se tornar um desafio. A participação de equipe multidisciplinar é essencial. Objetivos: o objetivo deste artigo é apresentar uma revisão em NP sobre seus principais elementos e atuais recomendações para o restabelecimento do estado nutricional da criança. Métodos: realizou-se a revisão de diretrizes e artigos específicos sobre NP em Pediatria disponíveis do período de 2000 a 2011 em português e inglês. Conclusões: os atuais avanços no conhecimento das demandas nutricionais bem como as melhorias na disposição dos nutrientes e manejo das complicações da NP fazem desta uma ferramenta eficiente e segura para ser utilizada quando bem indicada.
Introduction: parenteral nutrition (PN) is used in the treatment of children that cannot be completely fed via oral or enteral feeding. Its main purpose is to maintain or restore optimal nutritional status. However, because of the particularity of the pediatric age group in relation to nutritional requirements, its use can become a challenge. The participation of a multidisciplinary team is essential. Objectives: the aim of this article is to present a review in PN, its main elements, and current recommendations for the reestablishmentof a nutritional status in the child. Methods: the review of guidelines and specific articles about PN in Pediatrics, available from 2000 to 2011, in Portuguese and English, was performed. Conclusions: the current advances in the knowledge about nutritional demands and improvements in the provision of nutrients and handling of PN complications make this procedure an efficient and safe tool to be used when indicated.
ABSTRACT
OBJECTIVE: The aim of this study was to describe the prevalence of main hereditary thrombophilias, Janus kinase 2 (JAK2) V617F mutation, antiphospholipid antibody syndrome (APS), and hyperhomocysteinemia in Brazilian children and adolescents diagnosed with portal vein thrombosis (PVT) without associated hepatic disease. METHODS: A cross-sectional study was carried out with 32 children with PVT in accompaniment at Hospital das Clínicas of the Universidade Federal de Minas Gerais from January 1990 to July 2011. Laboratory evaluation of thrombophilias was performed from September 2010 to July 2011. RESULTS: Thirty-two patients were evaluated; 59% were boys. Median age at diagnosis was 2.4 years. Mean time of patients' accompaniment was between 4.7 and 5.2 years. The presence of hereditary and acquired thrombophilias occurred in 34.4% of patients, and 9 of them also showed other risk factors in the previous history evaluation. Risk factors were absent in the previous history of 18 patients (56.3%). Two patients showed persistent high titres of anticardiolipin antibodies. Hyperhomocysteinemia was not observed. One patient was heterozygous for factor V Leiden and prothrombin G20210A mutation (3.1%). Eleven patients (34.4%) showed heterozygous methylenetetrahydrofolate reductase (MTHFR) C677T, and no patient had the JAK2V617F mutation. CONCLUSIONS: Even after investigation of main hereditary and acquired thrombophilia, PVT remains without apparent cause in most patients. Nevertheless, association of local and systemic risk factors seems to be important also in the pediatric age group. Therefore, despite the low prevalence, a complete investigation, which includes both hereditary and acquired thrombophilias, may be necessary.
Subject(s)
Mutation , Portal Vein/pathology , Thrombophilia/complications , Venous Thrombosis/etiology , Adolescent , Antibodies/blood , Brazil/epidemiology , Cardiolipins/immunology , Child , Child, Preschool , Cross-Sectional Studies , Factor V/genetics , Female , Heterozygote , Humans , Hyperhomocysteinemia/complications , Infant , Infant, Newborn , Janus Kinase 2/genetics , Male , Methylenetetrahydrofolate Reductase (NADPH2)/genetics , Prevalence , Prothrombin/genetics , Risk Factors , Thrombophilia/epidemiology , Thrombophilia/genetics , Thrombophilia/immunology , Venous Thrombosis/genetics , Venous Thrombosis/immunologyABSTRACT
CONTEXT: Portal vein thrombosis refers to a total or partial obstruction of the blood flow in this vein due to a thrombus formation. It is an important cause of portal hypertension in the pediatric age group with high morbidity rates due to its main complication - the upper gastrointestinal bleeding. OBJECTIVE: To describe a group of patients with portal vein thrombosis without associated hepatic disease of the Pediatric Hepatology Clinic of the Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil with emphasis on diagnosis, presentation form and clinical complications, and the treatment of portal hypertension. METHODS: This is a descriptive study of a series of children and adolescents cases assisted from January 1990 to December 2010. The portal vein thrombosis diagnosis was established by ultrasound. RESULTS: Of the 55 studied patients, 30 (54.5%) were male. In 29 patients (52.7%), none of the risk factors for portal vein thrombosis was observed. The predominant form of presentation was the upper gastrointestinal bleeding (52.7%). In 20 patients (36.4%), the initial manifestation was splenomegaly. During the whole following period of the study, 39 patients (70.9%) showed at least one episode of upper gastrointestinal bleeding. The mean age of patients in the first episode was 4.6 ± 3.4 years old. The endoscopic procedure carried out in the urgency or electively for search of esophageal varices showed its presence in 84.9% of the evaluated patients. The prophylactic endoscopic treatment was performed with endoscopic band ligation of varices in 31.3% of patients. Only one died due to refractory bleeding. CONCLUSIONS: The portal vein thrombosis is one of the most important causes of upper gastrointestinal bleeding in children. In all non febrile children with splenomegaly and/or hematemesis and without hepatomegaly and with normal hepatic function tests, it should be suspect of portal vein thrombosis. Thus, an appropriate diagnostic and treatment approach is desirable in an attempt to reduce morbidity and mortality.
Subject(s)
Esophageal and Gastric Varices/etiology , Gastrointestinal Hemorrhage/etiology , Portal Vein , Splenomegaly/etiology , Venous Thrombosis , Adolescent , Child , Child, Preschool , Endoscopy , Esophageal and Gastric Varices/therapy , Female , Gastrointestinal Hemorrhage/therapy , Humans , Infant , Infant, Newborn , Ligation , Male , Risk Factors , Sclerotherapy , Splenomegaly/therapy , Ultrasonography , Venous Thrombosis/complications , Venous Thrombosis/diagnostic imaging , Venous Thrombosis/therapyABSTRACT
CONTEXT: Portal vein thrombosis refers to a total or partial obstruction of the blood flow in this vein due to a thrombus formation. It is an important cause of portal hypertension in the pediatric age group with high morbidity rates due to its main complication - the upper gastrointestinal bleeding. OBJECTIVE: To describe a group of patients with portal vein thrombosis without associated hepatic disease of the Pediatric Hepatology Clinic of the Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil with emphasis on diagnosis, presentation form and clinical complications, and the treatment of portal hypertension. METHODS: This is a descriptive study of a series of children and adolescents cases assisted from January 1990 to December 2010. The portal vein thrombosis diagnosis was established by ultrasound. RESULTS: Of the 55 studied patients, 30 (54.5%) were male. In 29 patients (52.7%), none of the risk factors for portal vein thrombosis was observed. The predominant form of presentation was the upper gastrointestinal bleeding (52.7%). In 20 patients (36.4%), the initial manifestation was splenomegaly. During the whole following period of the study, 39 patients (70.9%) showed at least one episode of upper gastrointestinal bleeding. The mean age of patients in the first episode was 4.6 ± 3.4 years old. The endoscopic procedure carried out in the urgency or electively for search of esophageal varices showed its presence in 84.9% of the evaluated patients. The prophylactic endoscopic treatment was performed with endoscopic band ligation of varices in 31.3% of patients. Only one died due to refractory bleeding. CONCLUSIONS: The portal vein thrombosis is one of the most important causes of upper gastrointestinal bleeding in children. In all non febrile children with splenomegaly and/or hematemesis and without hepatomegaly and with normal hepatic function tests, it should be suspect of portal vein thrombosis. Thus, an appropriate diagnostic and treatment approach is desirable in an attempt to reduce morbidity and mortality.
CONTEXTO: Trombose da veia porta refere-se a uma obstrução total ou parcial do fluxo de sangue nesta veia devido à formação de um trombo. É uma causa importante da hipertensão porta na faixa etária pediátrica, com taxas elevadas de morbidade devido a sua principal complicação - a hemorragia digestiva alta. OBJETIVO: Descrever o grupo de crianças e adolescentes com trombose de veia porta sem doença hepática associada do Ambulatório de Hepatologia Pediátrica do Hospital das Clínicas da UFMG, Belo Horizonte, MG, Brasil, com ênfase no diagnóstico, forma de apresentação, complicações clínicas e na abordagem da hipertensão porta. MÉTODOS: Trata-se de estudo descritivo de uma série de casos de crianças e adolescentes atendidos de janeiro de 1990 a dezembro de 2010. O diagnóstico de trombose de veia porta foi estabelecido por ultrassonografia. RESULTADOS: Dos 55 pacientes analisados, 30 (54,5%) eram do gênero masculino. Em 29 pacientes (52,7%) não foi identificado nenhum fator de risco para trombose de veia porta. A forma de apresentação predominante foi hemorragia digestiva alta (52,7%). Em 20 pacientes (36,4%), a manifestação inicial foi esplenomegalia. Durante todo o período de seguimento, 39 pacientes (70,9%) apresentaram, pelo menos, um episódio de hemorragia digestiva alta. A média de idade dos pacientes neste primeiro episódio foi de 4,6 ± 3,4 anos. O exame endoscópico, seja realizado na urgência ou eletivamente para pesquisa de varizes esofágicas, mostrou sua presença em 84,9% dos pacientes avaliados. O tratamento endoscópico profilático foi realizado com ligadura elástica de varizes em 31,3% dos pacientes. Apenas um evoluiu para óbito devido a sangramento refratário. CONCLUSÕES: A trombose de veia porta é uma das causas mais importantes de hemorragia digestiva alta em crianças. Deve-se suspeitar de trombose de veia porta em toda criança com esplenomegalia afebril e/ou hematêmese, sem hepatomegalia e com testes de função hepática normais. Desta forma, uma abordagem diagnóstica e terapêutica adequada é desejável na tentativa de reduzir a morbimortalidade.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Esophageal and Gastric Varices/etiology , Gastrointestinal Hemorrhage/etiology , Portal Vein , Splenomegaly/etiology , Venous Thrombosis , Endoscopy , Esophageal and Gastric Varices/therapy , Gastrointestinal Hemorrhage/therapy , Ligation , Risk Factors , Sclerotherapy , Splenomegaly/therapy , Venous Thrombosis/complications , Venous Thrombosis/therapy , Venous ThrombosisABSTRACT
Trombose de veia porta (TVPO) refere-se à obstrução total ou parcial do fluxo sanguíneo nessa localização, secundária à formação de trombos, Essa entidade se mostra importante na faixa etária pediátrica, por ser causa importante de hipertensão porta, com elevadas taxas de morbidade devido à sua principal complicação - a hemorragia digestiva alta (HDA). Aproximadamente 79% das crianças com diagnóstico de trombo- se de veia porta apresentarão ao menos um episódio de HDA. A etiologia da trombose é variada e, na maioria dos casos, há associação de fatores locais e sistêmicos, sendo o cateterismo umbilical o principal fator etiológico encontrado em crianças. A importância dos distúrbios de coagulação entre esses fatores está se tornando mais acentuada à medida que novos estudos e métodos diagnósticos são disponibilizados. O tratamento varia de acordo com a forma de apresentação - aguda ou crônica - e com a existência ou não de complicações associadas à TVPO, No quadro agudo a anticoagulação está indicada na maioria dos casos, enquanto no quadro crônico sua indicação acontece geralmente quando há trombofilias associadas, Na evolução crônica, o tratamento se direciona às complicações decorrentes da hipertensão porta secundária à TVPO.
Portal Vein Thrombosis (PVT) refers to a total or partial obstruction of the blood flow in this location, secondary to a thrombus tormation.': 2 PVT is important in the pediatric age group because it is one of lhe most frequent causes of portal hypertension, with high morbidity rates due to its main complicalion - the upper gastrointestinal bleeding. Approximately 79% of lhe children diagnosed with PVT will show at least one episode of upper gastrointestinal bleeding during their lives. The etiology of portal vein lhrombosis is varied and, in most cases, there is an associalion of local and systemic tactors, and lhe umbilical calheterization is lhe main etiological tactor found in children. The importance of coagulation disorders among these factors is becoming greater as new studies and diagnostic methods are available. Treatment varies according to the form of presentation - acute or chronic - and the presence or absence of complications associated with PVT. In acute thrombosis, anticoagulation is indicated in most cases, while in the chronic condition is usually indicated when the patient has thrombophilia.In chronic disease, treatment is directed at complications of portal hypertension secondary to PVT.