RESUMEN
BACKGROUND: Cirrhosis is rare in pediatrics. The children cirrhosis is particular by the ascendancy of biliairy cirrhosis and cirrhosis due to an innate error of metabolism and by the relative frequency of the cases where an etiological treatment is possible. However in developing countries, the children cirrhosis still put problems of etiological diagnosis and of therapeutic coverage. AIM: To study epidemiological and etiological particularities, therapeutic modalities and outcome of cirrhosis in the south of Tunisia. METHODS: We led a retrospective study over 15 years (1990-2004) having allowed to depict 71 cirrhotic children followed in the service of general pediatric department of SFAX University hospital (Tunisia). RESULTS: Our patients divide up into 36 girls and 35 boys. The age of revelation of the disease was variable (15 days to 15 years). Jaundice and hepatomegaly were the most two clinical signs frequently found in the clinical exam. On the etiological plan, biliairy cirrhosis (Extra hepatic biliary atresia, dilatation of choledocal cyst, progressive familial intrahepatic cholestasis...) were the most frequent (40%) followed by metabolic cirrhosis(tyrosinemia type I, Wilson disease...) (17 %) and post-hepatitic cirrhosis (17%). In 27% of cases, no etiology was found. Besides the symptomatic treatment, an etiological treatment was tempted in some cases. No patient benefited from hepatic transplantation. The follow-up of the children cirrhosis was grave because 30 children (42%) died, 19 children are lost-sight and 22 children (31%) are still alive. CONCLUSION: The children cirrhosis pose still in our country of the problem of etiological diagnosis because of the not availability of some specific additional exams and especially problems of coverage for lack of a program of hepatic transplantation.
Asunto(s)
Cirrosis Hepática Biliar , Cirrosis Hepática , Adolescente , Niño , Preescolar , Femenino , Departamentos de Hospitales , Hospitales Universitarios , Humanos , Lactante , Recién Nacido , Cirrosis Hepática/epidemiología , Cirrosis Hepática/etiología , Cirrosis Hepática/terapia , Cirrosis Hepática Biliar/epidemiología , Cirrosis Hepática Biliar/etiología , Cirrosis Hepática Biliar/terapia , Masculino , Pediatría , Estudios Retrospectivos , Túnez/epidemiologíaRESUMEN
Cystic fibrosis was regarded a long time as exceptional in the Nord Africaine population and in particular in Tunisia what was at the origin of the ignorance of its various diagnostic and therapeutic aspects in our country. Nevertheless, with the development of the means of the diagnosis, several cases of cystic fibrosis were diagnosed these last years what will pose true problems of assumption of responsibility of these children like illustrates it well our experiment in the pediatric department of Sfax university hospital. In 10 years going of 1991 to 2000 we reported 7 cases of cystic fibrosis in the pediatric department of Sfax university hospital. Our patients are 3 boys and 4 girls. The age of revelation of the disease varied from 3 months to 14 years with 4 years and 10 months an average age. All our patients had a respiratory symptomatology in the foreground. The evolution was marked by the death of 4 patients at an average age of 5 years and half whereas the 3 surviving patients for the moment are more or less balanced on the nutritional level, digestive and respiratory. Cystic fibrosis is not exceptional in our area, its assumption of responsibility therapeutic is difficult, and it requires the collaboration of several experts and a good compliance of the child and of his family.
Asunto(s)
Fibrosis Quística , Niño , Preescolar , Fibrosis Quística/diagnóstico , Fibrosis Quística/epidemiología , Fibrosis Quística/genética , Fibrosis Quística/terapia , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , TúnezRESUMEN
Idiopathic pulmonary hemosiderosis, a rare clinical entity, is characterized by bleeding into the alveoli and progressive pulmonary fibrosis. It is usually treated with systemic corticosteriod during acute bleeding episodes and with prolonged courses of oral corticosteroids and/or other immunosuppressant drugs for maintenance therapy. Because prolonged treatment with these agents is frequently associated with significant side effects, favorable response to inhaled corticosteroids would be ideal in this condition. The authors reports 2 cases of idiopathic pulmonary hemosiderosis diagnoses in the pediatric department of Sfax: a 20 month old boy and an 8 year old girl successfully treated with inhaled beclomethazone dipropionate using doses of 500_g/day and low dose oral prednisolone (0.5 mg/kg one day/2).
Asunto(s)
Beclometasona/administración & dosificación , Glucocorticoides/administración & dosificación , Hemosiderosis/tratamiento farmacológico , Enfermedades Pulmonares/tratamiento farmacológico , Prednisolona/administración & dosificación , Administración por Inhalación , Niño , Femenino , Hemosiderosis/diagnóstico , Humanos , Lactante , Enfermedades Pulmonares/diagnóstico , Masculino , Resultado del TratamientoRESUMEN
BACKGROUND: Cow's milk allergy is defined as reproducible adverse reaction to a food protein antigen which is immune medieted. About 80 to 90 % become clinically tolerated within the first three year of live. The aim of this study is to evaluate the clinical, immunological and evolutive characteristics of the 10 % to 20 % of persistent form of cow's milk allergy. METHODS: This study included six persistent form of cow's milk allergy (2 boys and 4 girls). The provocation challenge test according to Rance recommendation, have been made from 6 months to one year. RESULTS: 4/6 infants with persistent cow's milk allergy have an atopic familial disease. Clinical presentation changed over time at once symptoms were preventely gastrointestinal, at the end there was an increased frequency of atopic disease (asthma: 4/6 infants) CONCLUSION: Persistent form of cow's milk allergy are characterized by considerable importance of familial atopic disease; change in cow's milk allergy manifestations over time and more prolonged delay between cow's milk persistent consumption and manifestations of symptoms.
Asunto(s)
Hipersensibilidad a la Leche/complicaciones , Leche/efectos adversos , Animales , Asma/complicaciones , Niño , Preescolar , Femenino , Humanos , Inmunoglobulina E/sangre , Masculino , Hipersensibilidad a la Leche/inmunología , Pruebas CutáneasRESUMEN
UNLABELLED: The current immunosuppressive treatment of patients with autoimmune hepatitis consists of prednisone and azathioprine. High doses of prednisone used to obtain the remission of the disease are associated with serious adverse effects. To avoid harmful consequences of prednisone, we proposed to treat patients with oral cyclosporine to obtain the remission of the inflammatory process. CONCLUSION: This case and review of the cases in the literature show that cyclosporine can induce remission of liver disease in children with autoimmune hepatitis, with few and well tolerated adverse effects.