RESUMO
BACKGROUND: Diabetes in children is undergoing a profound change in terms of frequency and age at onset worldwide. Algeria is particularly impacted by the epidemic to the point of appearing in the "top 10" 2016 of countries with very high incidence of T1D. Our study was aimed to present the epidemiological evolution of T1D in children using data from the register of the department of Oran. METHODS: New cases of T1D are children under 15 years old coming from primary care units and pediatric departments. Registration was centralized at the level of the "C" pediatric unit of Oran University Hospital since 1973 with an assessment close to 100%. RESULTS: From 1973 to 2017, 2358 T1D new cases aged less than 15 years at T1D diagnosis have been registered. The average annual incidence under 15 years old for the last 5 years 2013-2017 is 31.12±3.60 cases per 100,000 and 22.62±5.18, 36.92±6.88 et 37.93±6.53 for children 0-4, 5-9 et 10-14 years old, respectively. The average annual evolution was at 12.78% in the last 25 years for the whole group and 15.03, 15.50 and 9.10% for children of 0-4, 5-9 and 10-14 years old, respectively. The estimated prevalence on December 31st 2017 was 207 per 100,000 equivalent to 1 T1D for 482 children under 15. The sex ratio for all cases is 0.94 with non-significant fluctuations. The winter/summer seasonality, significant from 1973 to 2013 in favor of winter months is no longer present starting from 2013. CONCLUSION: We confirm, in agreement with other teams, the outsized increase in the incidence of T1D and the younger age at onset in children under 15 in our country. This evolution, mainly related to environmental problems poses new difficulties to families and teams in charge of the disease.
Assuntos
Diabetes Mellitus Tipo 1/epidemiologia , Adolescente , Idade de Início , Argélia/epidemiologia , Criança , Pré-Escolar , Feminino , História do Século XX , História do Século XXI , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Prevalência , Sistema de Registros/estatística & dados numéricos , Estações do AnoRESUMO
Wolfram syndrome (WS) is a rare autosomal recessive progressive neurodegenerative disorder, and it is mainly characterized by the presence of diabetes mellitus and optic atrophy. Other symptoms such as diabetes insipidus, deafness, and psychiatric disorders are less frequent. The WFS1 gene, responsible for the disease and encoding for a transmembrane protein called wolframin, was localized in 1998 on chromosome 4p16. In this report, we present a familial observation of Wolfram syndrome (parents and three children). The propositus was a 6-year-old girl with diabetes mellitus and progressive visual loss. Her family history showed a brother with diabetes mellitus, optic atrophy, and deafness since childhood and a sister with diabetes mellitus, optic atrophy, and bilateral hydronephrosis. Thus, association of these familial and personal symptoms is highly suggestive of Wolfram syndrome. The diagnosis was confirmed by molecular analysis (biology), which showed the presence of WFS1 homozygous mutations c.1113G>A (p.Trp371*) in the three siblings and a heterozygote mutation in the parents. Our observation has demonstrated that pediatricians should be aware of the possibility of Wolfram syndrome when diagnosing optic atrophy in diabetic children.
