Children with FSs encounter a minor risk of mortality and morbidity. While recurrent seizures are observed in these children, only a minority of these patients develop epilepsy.

Background: Intellectual disability is a form of neurodevelopmental disorders that begin in childhood and is characterized by substantial intellectual difficulties as well as difficulties in conceptual, social, and practical areas of living. Several genetic and nongenetic factors contribute to its development; however, its most severe forms are generally attributed to single-gene defects. Highthroughput technologies and data sharing contributed to the diagnosis of hundreds of single-gene intellectual disability subtypes.

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Les accidents domestiques représentent un problème sérieux de santé publique en pédiatrie. Ils sont responsables d’une lourde morbidité et mortalité parmi la population pédiatrique. Nous avons mené une étude rétrospective colligeant 231 cas d’accidents domestiques chez l’enfant au Service de Pédiatrie de l’Hôpital Hédi Chaker de Sfax durant une période de 5 ans (2008 - 2012). Durant la période d’étude, nous avons colligé 231 cas d’accidents domestiques. Il s’agissait de 124 garçons (53,7%) et 107 filles (46,3%). L’âge moyen était de 2 ans avec des extrêmes de 1 jour et 14 ans. Les enfants âgés de moins de 4 ans étaient les plus exposés (88,7%). Les intoxications accidentelles étaient les accidents les plus fréquents (105 cas). Les caustiques étaient les agents toxiques les plus fréquents (33 cas), suivis par les médicaments (28 cas) puis les hydrocarbures (16 cas). Les accidents par corps étrangers ont représenté le deuxième mécanisme accidentel (64 cas). Il s’agissait de 43 cas d’inhalation de corps étrangers et 21 cas d’ingestion de corps étranger. Nous avons enregistré 28 cas de traumatismes; il s’agissait d’une chute d’une certaine hauteur dans 25 cas. Nous avons recensé 26 cas d’envenimations scorpioniques, 5 noyades, 2 cas de brulure et un seul cas d’électrisation. Les intoxications accidentelles et les accidents par corps étrangers représentent les principaux accidents domestiques dans notre série et la tranche d’âge entre 1 et 4 ans est la plus exposée aux accidents domestiques.

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Background:Bacillus Calmette-Guérin (BCG) vaccine is a widely used vaccine. Management of local BCG complications differs between clinicians, and the optimal approach remains unclear.Aims:We aim to describe the epidemiological, clinical and therapeutic aspects of the BCG vaccine side effects in Sfax.Patients and Methods:This was a retrospective study of all the cases of BCG vaccine adverse reactions recorded in the Dermatology and Paediatrics Departments of Hedi Chaker University Hospital of Sfax over a period of 10 years (2005–2015).Results:Twenty cases of BCG adverse reactions were notified during the study period. Actually, 80% of the patients presented local adverse reactions. The outcome was good in all the followed patients. The rate of disseminated BCG disease was 20%. Biological tests of immunity showed a primary immunodeficiency in three cases, whereas the outcome was fatal in two cases.Conclusion:BCG vaccine adverse reactions range from mild to severe. However, the management of benign local reactions remains unclear. Disseminated BCG disease must alert clinicians to the possibility of a primary immunodeficiency.

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: Factor XIII deficiency is a rare autosomal recessive disorder of hemostasis characterized by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. The aim of the study is to describe the clinical features and the outcome of the patients and to determine molecular characteristics. A retrospective study, was conducted on seven patients with factor XIII deficiency in the department of hematology and pediatrics, Hedi Chaker Hospital, Sfax, Tunisia during the period of 14 years (2001-2014). The activity of factor XIII in plasma of the patients was less than 1%. Seven patients from five unrelated families were recorded (four men and three women). Median age at diagnosis was 3.5 years. All patients had consanguineous parents. Six patients presented umbilical bleeding and only three patients had intracranial bleeding. Other bleeding features were seen, including skin and mucosal bleeding, muscular hematoma, and splenic rupture. Recurrent abortions were observed in one patient. The standard screening tests were normal. Genetic analysis identified two mutations interesting the subunit A of factor XIII. All patients received transfusion of fresh frozen plasma monthly. One patient was died because of intracranial hemorrhage.Factor XIII deficiency is a rare bleeding disorder which frequently increases in areas with high consanguinity. In our study, we identified a founder mutation. The prognosis of the disorder is related to hemorrhagic complications especially to life-threatening intracranial bleeding. Prophylaxis consists of factor XIII concentrate or recombinant factor XIII. If these are unavailable, fresh frozen plasma may be used.