Effect of diabetes self-management education on health-related quality of life of Tunisian children with type1 diabetes mellitus and their parents: A randomized controlled trial.

AIM: To assess the effect of diabetes self-management education (DSME) on health related quality of life (HRQoL) of Tunisian children/adolescents with type 1 diabetes mellitus and their parents. METHODS: This monocentral study used a randomized controlled trial design, during five-month intervention and five-month follow-up and including 110 patients (54 in the DSME intervention group and 56 in the Individual Education by Pediatrician (IEP) control group) and their parents. Pediatric Generic Core Quality-of-Life Inventory 4.0-Scale (PedsQL4.0) evaluated HRQoL. RESULTS: At baseline, both groups had similar clinical features and PedsQL4.0 scores (p>0.05). In DSME, clinical outcomes were significantly improved from baseline to follow-up (p<0.001), while in the IEP group, which received no intervention, these outcomes remained unchanged. During follow-up, DSME showed higher PedsQL4.0 scores in parents' proxy-report and children/adolescents self-report (p<0.001). According to parents' proxy-report, PedsQL4.0 scores were significantly higher during follow-up compared to baseline in DSME (p<0.001) while they remained the same in IEP (p>0.05). DSME had higher percentage of change in the PedsQL4.0 scores than IEP (p<0.01). The median change varied from -5.01% to 0% vs 5.41% to 36.36% in IEP and DSME, respectively. CONCLUSION: Encouraging healthcare professionals to incorporate these interventions could enhance the HRQoL of diabetic children and bolster their self-esteem.

Access to Care and Therapy for Kawasaki Disease in the Arab Countries: A Kawasaki Disease Arab Initiative (Kawarabi) Multicenter Survey.

Kawasaki Disease (KD) is still the most common acquired heart disease in children below the age of five years; it has been well described in the developed world; however, data from the Arab world are limited to case reports or single-center case series. In an effort of optimizing KD research in the Arab world, a group of physicians and researchers established the KD Arab Initiative (Kawarabi) in 2021, and published the first survey, which showed disparities in the availability of intravenous immunoglobulin (IVIG); this had prompted Kawarabi to assess the access to care and therapy of KD patients in Arab countries. A 32 structured questions survey was conducted in thirteen Arab countries and addressed KD patients' access to healthcare in urban and rural settings. The survey results showed that access to care was uniform across large, mid-size cities and rural areas in 7/13 (54%) countries, while in 6/13 (46%) countries, it was in favor of large and mid-size cities over rural areas. The quality of medical services received by children with KD in large cities was rated as excellent in 6/13 or good in 7/13 countries compared to fair in 4/13 or poor in 4/13 countries in rural areas. Availability of IVIG was limited (23%) in mid-size cities and almost impossible (23%) in rural areas. The KD patients in mid-size cities and rural areas have limited access to standard healthcare in the Arab world. This survey laid the foundation for future Kawarabi endeavors to improve the care of children with KD.

Reversibility of pancreatic ß-cells dysfunction after vitamin D and calcium supplementation: a pilot study in a population of obese and prepubescent North-African children.

The mechanisms of diabetogenesis in children remain largely obscure. This study aimed to determine the impact of vitamin D and calcium supplementation on pancreatic ß-cells function in terms of insulin secretion and sensitivity. This was a quasi-experimental study involving 30 obese and prepubescent Tunisian children (57% boys). During three months, the children received calcium and vitamin D supplementation at therapeutic doses. An oral glucose tolerance test (OGTT) was performed at the beginning and at the end of the study. The following metabolic definitions were applied: i) hyperinsulinism: insulinemia sum > 300 µ UI/ml during OGTT, ii) insulin-resistance: homeostatic model assessment of insulin-resistance > 2, iii) normal glycaemic profile: normal plasma levels during OGTT without any spike, and iv) pancreatic ß-cells dysfunction reversibility: disappearance of the aforementioned disorders. The means ± standard-deviation of age and body mass index were 10.87 ± 1.9 years, and 30.17 ± 4.99 kg/m2, respectively. All children were at the stage of hyperinsulinism associated with insulin-resistance. These disturbances were noted even in children having a normal glycaemic profile at OGTT. After calcium and vitamin D supplementation, glycaemic profile as well as insulin-secretion improved significantly (p < 0.0001). Hyperinsulinism and insulin-resistance decreased significantly by 56.67% (p < 0.0001) and 70.00% (p < 0.0001), respectively. Complete reversibility of these two disorders was noted in 26.6% of children. To conclude, in obese and prepubescent children, vitamin D and calcium supplementation led to the reversibility of the pancreatic ß-cells dysfunction.

Disseminated BCG infection revealing a severe combined immunodeficiency: A case report.

INTRODUCTION: Bacillus Calmette Guerin (BCG) vaccine, which is administered to all newborns in Tunisia, can lead to serious complications ranging from local disease to disseminated disease in a group of patients with primary immunodeficiency diseases. CASE REPORT: A 3-month-old boy presented with persistent fever, hepato-splenomegaly and multiple osteolytic lesions. He was diagnosed with severe combined immunodeficiency disease and disseminated BCG infection. Despite anti-tubercular therapy combined with intravenous immunoglobulin, the evolution was fatal. CONCLUSION: The case highlights the possible risk of such rare yet lethal complication of BCG vaccine. In suspected cases of primary immunodeficiency disease, inoculation of BCG should be postponed until appropriate screening tests exclude such diagnosis to prevent serious complications.

[Coronary artery aneurysm in Kawasaki disease and its risk factors : a retrospective study about 65 Tunisian children]. / Les complications coronaires de la maladie de Kawasaki et ses facteurs de risque : à propos de 65 cas d'enfants tunisien.

INTRODUCTION: Kawasaki syndrome (KS) is a systemic vasculitis of unknown etiology that affects medium and small blood vessels. The aim of our study is to analyze coronary artery lesions in children with KS and their risk factors. MATERIAL AND METHODS: All children under the age of 15 years-old presenting KS and admitted in the pediatric department of three university hospital (Sahloul hospital, and Farhat Hached hospital of Sousse, Ibn El Jazzar hospital of Kairoun) from January 2000 to December 2018 were included. RESULTS: Sixty-five patients were included in our study. The mean age at diagnosis was of 29.9 months [2-120 months] and the sex ratio was of 1.7. Echocardiography was performed in all patients. It showed coronary dilation in 37% of patients with coronary artery diameter of 4.2 mm on average [3.2-7mm]. The coronary aneurysm was small in 19 cases and medium in 5 cases. No giant aneurysm has been identified. In univariate analysis, the predictors of coronary artery lesions were male sex, atypical form, fever duration more than 10 days, hepatic cytolysis, thrombocytosis and anemia. In multivariate analysis, only the last four parameters were the predictive factors of the coronary artery involvement. CONCLUSION: Several risk factors can be used to determine which children are predisposed to develop coronary dilations. In case of patient with risk factors, intravenous immunoglobulins should be initiated early to avoid these serious complications.

Temporal and climate characteristics of respiratory syncytial virus bronchiolitis in neonates and children in Sousse, Tunisia, during a 13-year surveillance.

This study established the correlation between respiratory syncytial virus (RSV) bronchiolitis and climate factors in the area of Sousse, Tunisia, during 13 years (2003-2015), from neonates and children <= 5 years old and hospitalized in Farhat Hached University-Hospital of Sousse. The meteorological data of Sousse including temperature, rainfall, and humidity were obtained. RSV detection was carried out with the direct immunofluorescence assay. The impact of climate factors on viral circulation was statistically analyzed. From 2003 to 2015, the total rate of RSV bronchiolitis accounted for 34.5% and peaked in 2007 and 2013. RSV infection was higher in male cases and pediatric environment (p<0.001) and was detected in 47.3% of hospitalizations in intensive care units. The epidemic of this pathogen started in October and peaked in January (41.6%). When the infectivity of RSV was at its maximum, the monthly average rainfall was high (31 mm) and the monthly average temperature and the monthly average humidity were at their minimum (11 °C and 66%, respectively). RSV activity was negatively correlated with temperature (r = - 0.78, p = 0.003) and humidity (r = - 0.62, p = 0.03). Regression analysis showed that the monthly average temperature fits into a linear model (R2 = 61%, p < 0.01). No correlation between RSV activity and rainfall was observed (p = 0.48). The meteorological predictions of RSV outbreaks with specific Tunisian climate parameters will help in determining the optimal timing of appropriate preventive strategies. In the area of Sousse, preventive measures should be enhanced since October especially, when the temperature is around 11 °C and humidity is above 60%.

Long term effect of a school based intervention to prevent chronic diseases in Tunisia, 2009-2015.

BACKGROUND & OBJECTIVES: We aimed to evaluate the long term effect of school based intervention to prevent non- communicable disease risk factors. METHODS: It was a quasi experimental study conducted during the period of 2009-2015. We involved school children aged from 11 to 16 years old. For the assessment of the program's effectiveness, subjects in both groups were examined at baseline, at the end of the 3-year intervention period and at the follow-up, one year after program's cessation. RESULTS: In the intervention group, the prevalence of school children who reported to be eating 5 fruits and vegetable sdaily increased significantly from 30.0% at pre-assessment to 33.2% at post-assessment, one year after (p=0.02, p=0.41 respectively). For the control group, this prevalence had significantly decreased from 40.2% at baseline to 35.0% at post-intervention, at the follow up, this proportion increased to 44.5%(p=0.001, p<10-3 respectively). Concerning smoking habits, we observed a decreasing trend in the intervention group from 5.7% at pre-assessment, to 4.8% at post-assessment and to 3.4% at the follow-up (p=0.19 and p=0.25 respectively). There was also a significant decrease in school children who did recommended physical activity in the same group. CONCLUSION: The present work showed that interventions promoting healthy lifestyles should be maintained. Developing countries should be encouraged and supported to design, conduct, and evaluate robust preventive interventions.

Socio-demographic association of non communicable diseases' risk factors in a representative population of school children: a cross-sectional study in Sousse (Tunisia).

INTRODUCTION: A better understanding of socio-demographic characteristics of subgroups, which have a high risk to develop chronic diseases, is essential to develop more efficient interventional programs especially for youth. This study aimed to determine the association between clusters of non communicable diseases (NCDs') risk factors and the socio-demographic characteristics among a sample of Tunisian school children. MATERIALS AND METHODS: We conducted, in 2013/2014, a cross-sectional study among a proportional and stratified school children sample, selected in 17 elementary public schools in Sousse (Tunisia). A cluster analysis was used to identify different NCDs risk factors clusters, based on tobacco use, physical inactivity, unhealthy diet, and excess weight. Subsequent χ2-tests were used to identify differences between the NCDs risk factors clusters in regards to socio-demographic characteristics. RESULTS: Four clusters of NCDs risk factors were found: 1) Cluster 1: physical inactivity behavior with normal weight, 2) Cluster 2: physical inactivity behavior associated to excess weight, 3) Cluster 3: unhealthy diet associated to excess weight and low practice of physical activity, and 4) Cluster 4: smoking behavior with physical activity behavior. The pattern of cluster membership differed across sex (<10-3), school level, and socioeconomic level (<10-3) but there was no significant difference between clusters for mother's education levels and household tenure. CONCLUSION: This study can have important implications for health policy and practice. Indeed, it found that many subjects have simultaneous multiple NCDs risk factors which leads to identify groups at risk and implement integrated intervention program.

Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012).

PURPOSE: Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility to not only recurrent infections but also autoimmune diseases and malignancies. The aim of this study was to describe and analyze the distribution, clinical features and eventual outcome of PID among Tunisian patients. METHODS: We reviewed the record of 710 patients diagnosed with Primary Immunodeficiency Diseases (PIDs) from the registry of the Tunisian Referral Centre for PIDs over a 25-year period. RESULTS: The male-to-female ratio was 1.4. The median age at the onset of symptoms was 6 months and at the time of diagnosis 2 years. The estimated prevalence was 4.3 per 100,000 populations. The consanguinity rate was found in 58.2 % of families. According to the International Union of Immunological Societies classification, spectrums of PIDs were as follows: combined T-cell and B-cell immunodeficiency disorders account for the most common category (28.6 %), followed by congenital defects of phagocyte (25.4 %), other well-defined immunodeficiency syndromes (22.7 %), predominant antibody deficiency diseases (17.7 %), diseases of immune dysregulation (4.8 %), defect of innate immunity (0.4 %) and complement deficiencies (0.4 %). Recurrent infections, particularly lower airway infections (62.3 %), presented the most common manifestation of PID patients. The overall mortality rate was 34.5 %, mainly observed with combined immunodeficiencies. CONCLUSION: The distribution of PIDs was different from that reported in Western countries, with a particularly high proportion of Combined Immunodeficiencies and phagocyte defects in number and/or function. More is needed to improve PID diagnosis and treatment in our country.

School-Based Intervention as a Component of a Comprehensive Community Program for Overweight and Obesity Prevention, Sousse, Tunisia, 2009-2014.

INTRODUCTION: Combating obesity at an early age, by improving physical activity and nutrition-related behaviors, is vital to the prevention of more critical health concerns in adulthood. This intervention study evaluated the effectiveness of a school-based component of a community behavioral intervention on overweight and obesity rates of adolescents in Sousse, Tunisia. METHODS: A quasi-experimental school-based intervention was conducted with an intervention group (in Sousse Jawhara and Sousse Riadh) and a control group (in Sousse Msaken). The intervention (which was a physical activity and nutrition program) lasted 3 years, with data at preintervention collected during the 2009-2010 school year and at postintervention collected during the 2013-2014 school year. Descriptive statistics and multivariate analysis were used to determine the effect of the intervention on risk of excess weight. RESULTS: Results showed a significant increase in fruit and vegetable intake by the intervention group (P = .04). The intervention group had an increase in students in the normal weight category (P = .03) and a decrease in students in the overweight category (P = .03).The intervention effect was a protective factor against excess weight for the participating schoolchildren (OR, 0.84; P = .02). CONCLUSION: This study showed that a school-based intervention is successful in increasing healthy dietary habits and in reducing risk of excess weight. It also showed the importance of a multisectoral approach to provide an environment conducive to healthy behaviors for adolescents.

Baseline characteristics of a school based intervention to prevent non communicable diseases risk factors: Project "together in Health".

BACKGROUND: Tobacco use, unhealthy diet, and physical inactivity are among the leading causes of the major non communicable diseases. So, prevention should take place early in childhood. AIM: In this paper, we will present an overview of project "Together in health" in schools, a component of a community based intervention. It consists on a school based intervention with the aim to improve knowledge, attitudes and behaviors concerning the main chronic disease risk factors such as unhealthy diet, physical inactivity and smoking. METHODS: We conducted a quasi experimental design with intervention and control groups. The study concerned pupils of colleges of Sousse aged 11 to 16 years old in 7th and 9th grade. The pre-assessment concerned a randomized sample of schoolchildren. The proportional and stratified sample was composed of 4003 schoolchildren with 1929 and 2074 respectively in intervention and control groups. We used chi square test to compare percentages with 0.05 level of significance. RESULTS: The sex ration was been 1 in the intervention group and 0.87 in control group. The mean age of our population was been 13.48±1.29 and 13.24±1.25 respectively in intervention and control groups with significant difference (p<10-3). Schoolchildren who reported practicing physical activity daily represented 19.1% and 12.7% respectively in intervention and control groups. Concerning eating habits, the schoolchildren reported frequency (number of days per week) of consuming various foods and beverages included respectively in the intervention and control group: vegetables 3.9 days/week and 4.81 days/week, fruits 5.41 days/week and 5.7 days/week, high fat food 2.49 days/week and 2.48 days/week, sweetened beverage 3.84 days/week and 3.3 days/week, sweets 4.33 days/week and 4.57 days/week. The proportion of irregular smokers was been respectively 6.8% and 2.2% among boys and girls in the intervention group and 11.3% and 0.9% in control group. CONCLUSION: Integrated and sustainable interventions against non communicable disease risk factors in this region are needed to prevent these diseases early in childhood.

A 1,100-year-old founder effect mutation in IL12B gene is responsible for Mendelian susceptibility to mycobacterial disease in Tunisian patients.

Mendelian susceptibility to mycobacterial disease (MSMD) is a rare disorder predisposing apparently healthy individuals to infections caused by weakly virulent mycobacteria such as bacille Calmette-Guerin (BCG), environmental mycobacteria, and poorly virulent Salmonella strains. IL-12p40 deficiency is the first reported human disease due to a cytokine gene defect and is one of the deficiencies that cause MSMD. Nine mutant alleles only have been identified in the IL12B gene, and three of them are recurrent mutations due to a founder effect in specific populations. IL-12p40 deficiency has been identified especially in countries where consanguinity is high and where BCG vaccination at birth is universal. We investigated, in such settings, the clinical, cellular, and molecular features of six IL-12p40-deficient Tunisian patients having the same mutation in IL12B gene (c.298_305del). We found that this mutation is inherited as a common founder mutation arousing ~1,100 years ago. This finding facilitates the development of a preventive approach by genetic counseling and prenatal diagnosis especially in affected families.

Giant cell hepatitis with autoimmune hemolytic anemia in a nine month old infant.

Giant cell hepatitis (GCH) with autoimmune hemolytic anemia is a rare entity, limited to young children, with an unknown pathogenesis. We report the case of 9-mo old who presented with fever, diarrhea and jaundice four days before hospitalization. Physical examination found pallor, jaundice and hepatosplenomegaly. The laboratory workup showed serum total bilirubin at 101 µmol/L, conjugated bilirubin at 84 µmol/L, hemolytic anemia, thrombocytopenia and immunoglobulin G (IgG) and anti-C3d positive direct Coombs' test. The antinuclear, anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies, antiendomisium antibodies were negative. Serological assays for viral hepatitis B and C, cytomegalovirus, herpes simplex and Epstein Barr virus were negative. The association of acute liver failure, Evan's syndrome, positive direct Coomb's test of mixed type (IgG and C3) and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH. The diagnosis was confirmed by a needle liver biopsy. The patient was treated by corticosteroids, immunomodulatory therapy and azathioprine but died with septicemia.

Thyroid-related autoantibodies in Tunisian patients with type 1 diabetes.

AIM: To evaluate, retrospectively, the frequency of antithyroid antibodies (ATA) in patients with type 1 diabetes (T1D). MATERIALS AND METHODS: Antithyroperoxidase antibodies (TPO-Ab), antithyroglobulin antibodies (TG-Ab), and antithyroid-stimulating hormone receptor antibodies (TSHR-Ab) were determined by enzyme-linked immunosorbent assay. Sera of 312 patients (166 children and 146 adults) with T1D were analyzed. Sera of 276 healthy subjects (87 children and 189 blood donors) served as controls. RESULTS: Out of 312 patients with T1D, 44 (14%) had ATA (TPO-Ab or TG-Ab or TSHR-Ab). The frequency of ATA in patients with T1D was significantly higher than in the control group (14% vs. 2.8%; p<10(-5)). ATA were significantly more frequent in adult patients with T1D than in the blood donor group (20% vs. 1.6%; p<10(-8)). The frequency of ATA in adult patients was significantly higher than in pediatric patients (20% vs. 9%; p=0.006). The frequency of TPO-Ab and TG-Ab was significantly higher in patients with T1D than in the control group (13.5% vs. 2%; p<10(-8) and 7% vs. 2.2%, p=0.008), respectively. Out of 312 patients with T1D, only one had TSHR-Ab. The simultaneous presence of three autoantibodies was found in one patient with T1D. CONCLUSION: ATA were frequent in patients with T1D. Serological screening of autoimmune thyroid disease is suggested in patients with T1D.