Enfermedad celíaca en el adolescente y adulto joven: un desafío para gastroenterólogos de niños y adultos / Celiac disease in adolescents and young adults: a follow up study

Background: Follow-up of celiac patients in Chile is often interrupted when adolescents are referred to adult gastroenterologists. Aim: To study the evolution of patients with celiac disease when they reach adolescence or young adulthood. Patients and methods: Current adherence to gluten-free diet and its relation to symptoms and circulating antiendomysial antibodies were evaluated in the 58 confirmed celiac patients older than 12 years of age controlled at 3 hospitals in Santiago. Results: Mean age at the moment of this assessment was 17.8 ñ 5 years, 65.5 percent were women, 12.5 percent were at nutritional risk (-IDS) while 20 percent were overweight/obese. Although all patients declared themselves asymptomatic, a focused questionnaire revealed that 26 percent suffered some symptoms. Only 24.1 percent followed a strict gluten-free diet. Eight of 20 patients who ate gluten-containing diets had negative antiendomysial antibodies (EMA), three of whom turned positive within 6 to 9 months. In three of four (asymptomatic) cases that accepted a new jejunal biopsy, histology was abnormal. One patient who followed a strict diet had EMA (+) and normal histology. Conclusions: These results confirm that after childhood, symptoms abate significantly in celiac patients. The observed sensitivity and specificity of EMA makes necessary to maintain small intestinal biopsies as the gold standard for diagnosis and confirmation of the disease

La dieta sin gluten del celíaco ¿afecta la dieta familiar? / Does a gluten free diet affects the celiac patientïs family diet?

En 59 pacientes celíacos (12-28 años) se estudió la adherencia a la dieta sin gluten y el estado nutricional (al diagnóstico y en la actualidad), por antropometría (P/E, P/T y T/E en < 15 años e IMC en > 15 años). En 136 de sus familiares se evaluó el estado nutricional, la ingesta, la adecuación según requerimientos y el patrón de consumo de alimentos. Pacientes: todos se consideraron asintomáticos; solo 24,1 por ciento seguía la dieta estrictamente; 15 por ciento presentaban desnutrición leve mientras que 19,3 por ciento tenían sobrepeso/obesidad. Familiares: ninguno presentaba desnutrición, 12 por ciento de los < 15 años y 23,5 por ciento de los > 15 años tenían sobrepeso/obesidad. La ingesta de nutrientes principales estaba entre los P50-P75 descritos para población chilena; el pan y cereales fueron las mayores fuentes energéticas. Los resultados sugieren que tanto los pacientes celíacos > 12 años como sus familiares no presentan riesgo nutricional; más bien, siguen la tendencia a sobrepeso/obesidad de la población chilena actual

Enfermedad de Wilson: una revisión a propósito de experiencia clínica en 16 pacientes / Wilson's disease: a review apropos of clinical experience in 16 patients

Wilson's disease is an inherited disorder of copper metabolism. We report 16 patients (6 males) with the disease; 6 had hepatic involvement and 3 were asymptomatic. The age onset was 9 years for hepatic and 17 years for neurologic involvement. The mean delay in diagnosis was 14 months. Chronic hepatitis, cirrhosis and fulminant hepatic failure were the clinical forms of liver disease. Patients with neurologic disorders had behavioral disturbances and extrapyramidal manifestations such as dystonia and parkinsonism. Patients had a good response to penicillamine, except 3 that died of liver complications, in whom the treatment was delayed or discontinued. We conclude that this metabolic disease must be suspected in pubertal children and in adults of less than 30 years old with liver disease of unknown origin or behavioral alterations associated to an extrapyramidal syndrome