Júlio Rocha Pimenta

Doença de Wilson em crianças e adolescentes: diagnóstico e tratamento

OBJETIVO: Descrever as formas de apresentacao, as alteracoes laboratoriais ao diagnostico e o tratamento de criancas e adolescentes com doenca de Wilson. METODOS: Estudo descritivo e retrospectivo de 17 criancas e adolescentes com doenca de Wilson atendidos no Ambulatorio de Hepatologia Pediatrica do Hospital das Clinicas da Universidade Federal de Minas Gerais no periodo de 1985 a 2008. Os dados foram coletados dos prontuarios e durante as consultas ambulatoriais. RESULTADOS: A idade ao diagnostico variou de 2,8 a 15,1 anos, com media de 8,8±0,9 anos. A forma de apresentacao predominante foi hepatica (53%), seguida por assintomaticos provenientes de triagem familiar. O anel de Kayser-Fleischer foi encontrado em 41% dos pacientes. A ceruloplasmina encontrava-se alterada em 15/17 pacientes e o cobre urinario variou de 24 a 1000mcg/24h (mediana: 184mcg/24h). O tratamento instituido foi a D-penicilamina. Observaram-se efeitos colaterais em cinco criancas, sem necessidade de interrupcao ou troca da medicacao. As respostas clinica e laboratorial, com niveis normais de aminotransferases, foram evidenciadas em 14 pacientes apos mediana de 10,7 meses de tratamento. Tres criancas morreram (uma por hepatite fulminante e duas com complicacoes da insuficiencia hepatica grave), apesar do tratamento. CONCLUSOES: A doenca de Wilson e rara na faixa etaria pediatrica. A forma de apresentacao predominante e a hepatica. Seu diagnostico se baseia principalmente em dosagem de ceruloplasmina baixa, cobre livre e cobre em urina de 24 horas elevados, mas exige alto grau de suspeicao. Apresenta boa resposta e tolerância ao tratamento medicamentoso.

Management of digestive bleeding in children and adolescentes

The term gastrointestinal bleeding includes bleeding from both the upper digestive tract with location above the Treitz angle, called the upper gastrointestinal bleeding (UGB), as the source below Treitzangle lower gastrointestinal bleeding (LGB). In most cases the expectant observational approach is advocated, based on history and detailed physical examination. The classification by age and clinical status, both as to UGB and LGB, are the most used today. The initial approach for cases of gastrointestinal bleeding, both high as low, aims hemodynamic stabilization of the patient, and according to the site of bleeding other propaedeutic and therapeutic options are performed. Unlike to that in adults, the frames of gastrointestinal bleeding in children have low morbidity and mortality rates, due to the absence of comorbidities, as well as a better haemodynamic response to gastrointestinal bleeding. The purpose of this article is to help the systemized care to patients with gastrointestinal hemorrhage.

Intestinal Inflamatory disease in childood

The incidence of inflammatory bowel disease in the Western countries has increased in recent years. Classically it is divided into Crohn’s Disease and Ulcerative Colitis. The most common clinical manifestations are abdominal pain, diarrhea, weight loss and blood in the stool. The intestinal inflammation reduces the absorption of nutrients, which increases susceptibility to micronutrient deficiency. extra-intestinal manifestations may be present. Primary immunodeficiency research in children diagnosed with IBD before two years of age should be performed. Laboratory tests should include screening of acute and/or chronic, evaluation of anemia and nutritional status. Endoscopic examinations and feces are needed, especially before the start of treatment. Outpatient follow-up and complementary tests should be judicious. Treatment is difficult because it is a chronic disease with phases of relapse and remission. The drugs have adverse effects sometimes severe. The most current view of IBD therapy is based on the modification of the natural history of the disease, with the goal of achieving mucosal healing, reducing complications of the disease and improve the patient’s quality of life. It is essential to monitor the linear growth, muscle development, skeletal and pubertal. The general pediatrician should be aware of the diagnosis, requesting additional examinations and evaluating the need for referral to the pediatric gastroenterologist.

EVALUATION OF PRIMARY PROPHYLAXIS WITH PROPRANOLOL AND ELASTIC BAND LIGATION IN VARICEAL BLEEDING IN CIRRHOTIC CHILDREN AND ADOLESCENTS

Background The efficacy of nonselective β-blocker and endoscopic procedures, such as endoscopic variceal ligation, as primary prophylaxis of variceal hemorrhage in cirrhotic adults was demonstrated by numerous controlled trials, but in pediatric population, few are the number of studies. Objective The objective of this study is to evaluate the primary prophylaxis with β-blocker in cirrhotic children and adolescents with portal hypertension. Methods This is a cohort study encompassing 26 cirrhotic patients. β-blocker prophylaxis was performed with propranolol. When contraindicated the use of β-blocker, or if side effects presents, the patients were referred to endoscopic therapy with band ligation. Patients were evaluated by endoscopy, and those who had varicose veins of medium and large caliber or reddish spots, regardless of the caliber of varices, received primary prophylaxis. Results Of the 26 patients evaluated, 9 (34.6%) had contraindications to the use of propranolol and were referred for endoscopic prophylaxis. Six (35.3%) of the 17 patients who received β-blocker (propranolol), had bled after a median follow-up time of 1.9 years. β-blockage dosage varied from 1 mg/kg/day to 3.1 mg/kg/day and seven (41.2%) patients had the propranolol suspended due to fail of the β-blockage or adverse effects, such as drowsiness, bronchospasm and hypotension. Patients who received endoscopic prophylaxis (elastic bandage) had no bleeding during the follow-up period. Conclusion All of the patients that had upper gastroinstestinal bleeding in this study were under propranolol prophylaxis. The use of propranolol showed a high number of contraindications and side effects, requiring referral to endoscopic prophylaxis. The endoscopic prophylaxis was effective in reducing episodes of bleeding.

Viral Hepatitis A, B, and C in children and adolescents

Viral hepatitis are a serious public health problem in Brazil and the world. According to estimates, billions of people have already had contact with the hepatitis virus and millions are chronic carriers. The term viral hepatitis usually refers to hepatotropic viruses, which the currently best known A, B, C, D, and E are responsible for over 90% of cases of acute hepatitis. Viral hepatitis cause liver disease in the pediatric population and a wide variety of clinical presentations, ranging from an asymptomatic carrier state, acute hepatitis, fulminant or chronic to cirrhosis and hepatocellular carcinoma in other populations. Basic attention teams have a relevant role in the diagnosis and follow-up of hepatitis affected patients, symptomatic or not. In this review, hepatitis caused by viruses A, B, and C will be emphasized, which are frequent in our environment and involve high morbidity and mortality.