RESUMO
A 13-year-old girl with controlled coeliac disease who had been on a gluten-free diet for the past 3 months was admitted with respiratory distress and hypoxia for the past week. Chest radiograph and CT scan showed bilateral widespread alveolar shadowing suggestive of a hypersensitive pneumonitis. There was a dramatic radiological and clinical response to oral corticosteroids. Bronchial lavage was suggestive of recurrent aspiration and histology of a transbronchial biopsy showed pulmonary haemosiderosis. We conclude that pulmonary haemosiderosis may occur in children with coeliac disease who are on a gluten-free diet.
Assuntos
Doença Celíaca/complicações , Doença Celíaca/terapia , Dieta Livre de Glúten , Hemossiderose/diagnóstico , Pneumopatias/diagnóstico , Administração Oral , Adolescente , Corticosteroides/administração & dosagem , Anti-Inflamatórios/administração & dosagem , Doença Celíaca/tratamento farmacológico , Feminino , Hemossiderose/tratamento farmacológico , Hemossiderose/patologia , Humanos , Hipóxia/diagnóstico , Pulmão/patologia , Pneumopatias/tratamento farmacológico , Pneumopatias/patologia , Pneumonia/diagnóstico , Pneumonia/tratamento farmacológico , Pneumonia/patologia , Radiografia Torácica , Síndrome do Desconforto Respiratório/diagnóstico , Síndrome do Desconforto Respiratório/tratamento farmacológico , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
Familial Mediterranean fever is quite prevalent among Arabs. We reviewed the files of 56 patients diagnosed with familial Mediterranean fever and followed up at King Hussein Medical Centre in Jordan over 4 years for their clinical profile, course, genotype, treatment and complications. There were 30 males and 26 females with a mean age at onset of 5.2 years. Abdominal pain (79%) was the commonest manifestation, followed by arthritis (13%) and chest pain (4%). Family history was positive in 50% of patients. Regarding treatment, 97% of patients responded well to colchicine, and amyloidosis was not documented in any patients after 5 years follow-up. The commonest genotype was M694 (64%), followed by heterozygous M694V-V726A (23%) and E148Q (8%).
Assuntos
Árabes , Febre Familiar do Mediterrâneo/epidemiologia , Febre Familiar do Mediterrâneo/genética , Dor Abdominal/genética , Centros Médicos Acadêmicos , Adolescente , Distribuição por Idade , Idade de Início , Árabes/genética , Árabes/estatística & dados numéricos , Artrite/genética , Dor no Peito/genética , Criança , Pré-Escolar , Colchicina/uso terapêutico , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/terapia , Feminino , Seguimentos , Frequência do Gene , Triagem de Portadores Genéticos , Genótipo , Humanos , Lactente , Jordânia/epidemiologia , Masculino , Linhagem , Vigilância da População , Prevalência , Distribuição por SexoRESUMO
Familial Mediterranean fever is quite prevalent among Arabs. We reviewed the files of 56 patients diagnosed with familial Mediterranean fever and followed up at King Hussein Medical Centre in Jordan over 4 years for their clinical profile, course, genotype, treatment and complications. There were 30 males and 26 females with a mean age at onset of 5.2 years. Abdominal pain [79%] was the commonest manifestation, followed by arthritis [13%] and chest pain [4%]. Family history was positive in 50% of patients. Regarding treatment, 97% of patients responded well to colchicine, and amyloidosis was not documented in any patients after 5 years follow- up. The commonest genotype was M694 [64%], followed by heterozygous M694V- V726A [23%] and E148Q [8%]
