Cardiac iron overload detection using longitudinal strain in asymptomatic children with beta thalassemia major.

Cardiomyopathy mediated by iron disposition in cardiomyocytes is a dreadful cause of morbidity and mortality in patients with beta thalassemia major (BTM). Conventional transthoracic echocardiography (TTE) parameters are preserved at late stages of cardiomyopathy induced by iron overload. Therefore, cardiac imaging modalities based on myocardial deformation such as strain imaging are used for early detection of cardiac iron overload. To demonstrate the contribution of longitudinal strain (LS) in early detection of cardiac iron overload in children with BTM. Sixty children (30 children with BTM and 30 healthy controls) were enrolled in this study. Conventional TTE study was performed in both patient and control groups. LV regional longitudinal strain (RLS) were determined and compared between the two study groups. Mean age was 10.4 ± 5 years in BTM group compared to 10.2 ± 5 years in control group (p = 0.876). Compared to control group, there was no significant difference in conventional TTE parameters except for indexed left atrium (LA) area and volume. LA was significantly larger in BTM children (27.59 ± 13.1 ml/m2 vs. 18.23 ± 4.33 ml/m2, p = 0.001). RLS was lower in anterior, septal and inferior walls in basal and middle segments of LV in BTM group while there was no significant difference in RLS in apical segment between the two groups (- 27.30 ± 5.1 vs.- 28.83 ± 4.33, p = 0.22). In asymptomatic BMT children with normal conventional TTE parameters, LS could be used for the detection of subclinical myocardial dysfunction.

Diagnostic challenges in childhood sarcoidosis.

The aim of our clinical image was to emphasize the value of a careful skin examination in the diagnosis of early-onset sarcoidosis in children.

[Epidemiological, etiological and evolutionary aspects of children cirrhosis in a developing country: experience of the pediatric department of SFAX University hospital, Tunisia]. / Aspects épidémiologiques, étiologiques et évolutifs des cirrhoses de l'enfant dans un pays en développement: expérience du service de pédiatrie du CHU de Sfax, Tunisie.

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.

Epidemiological study of non-polio enterovirus neurological infections in children in the region of Monastir, Tunisia.

The epidemiological, virological, and clinical syndromes of 86 children younger than 13 years suspected of having neurological diseases and admitted to Fattouma Bourguiba Hospital of Monastir from January 2002 to November 2003 were analyzed. The presence of enterovirus was studied in cerebrospinal fluid (CSF) samples by reverse transcription-polymerase chain reaction (RT-PCR) and by isolation on cell culture. Sixty-one (71%) specimens were positive by RT-PCR, whereas 45 (52.3%) were detected by cell culture. Eighty percent (n = 36) of cultured enteroviruses were identified, whereas 20% were untyped. Echoviruses were isolated most frequently, with 32 cases (71.1%) in children. Echovirus 6 was the most commonly identified serotype (22.2%), followed by echovirus 13 (20%). The highest incidence on neurological infection (19.6%) occurred in children less than 6 months of age. The infected children were predominantly male (62.3%). Enteroviruses were detected in all the period of the year with the highest rate in the spring and summer months. Aseptic meningitis was the most commonly diagnosed disease (49%).