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Background and Aims: To study the independent and combined associations of diet and cardiorespiratory fitness with the prevalence of the metabolic syndrome (MetS).Methods and Results: We studied a population-based random sample of 663 men and 671 women 57-78 years of age at baseline of an ongoing randomised controlled trial. Based on a 4-day food record a diet score was created according to goals achieved (vegetables ≥400 g/day, fish ≥2 servings/week, fibre ≥14 g/1000 kcal, saturated fat <10 E%/day). Cardiorespiratory fitness was measured as maximal oxygen uptake (VO(2 max)) in a maximal symptom-limited bicycle ergometer test. MetS was defined by the National Cholesterol Education Program criteria. The lowest prevalence of MetS (5%) was observed among individuals in the highest VO(2 max) tertile and achieving 3-4 dietary goals. The highest prevalence (55%) was observed among those in the lowest VO(2 max) tertile and achieving none of the dietary goals. Among individuals in the highest VO(2 max) tertile, the odds ratio of having MetS was 0.04 (95% CI 0.02-0.10) for those achieving 3-4 dietary goals, 0.07 (0.04-0.14) for those achieving 1-2 dietary goals, and 0.16 (0.07-0.37) for those achieving none of the dietary goals compared with individuals in the lowest VO(2 max) tertile and achieving none of the goals after adjustment for confounding factors.Conclusion: Healthy diet and higher levels of cardiorespiratory fitness are associated with a reduced risk of having MetS. However, fitness seems to have a stronger association with MetS than diet. [ABSTRACT FROM AUTHOR]

Objective: We studied the association of maximum oxygen uptake (Vo(2max)) with the development and resolution of metabolic syndrome (MetS) for 2 years in older individuals.Research Design and Methods: Subjects were a population sample of 1,226 men and women aged 57-78 years. We assessed Vo(2max) directly by respiratory gas analysis during maximum exercise testing and used dichotomous and continuous variables for MetS.Results: One SD increase in baseline Vo(2max) associated with 44% (95% CI 24-58) decreased risk of developing MetS. Individuals in the highest third of baseline Vo(2max) were 68% (37-84) less likely to develop MetS than those in the lowest third. One SD increase in Vo(2max) increased the likelihood to resolve MetS 1.8 (1.2-2.8) times. Individuals in the highest Vo(2max) third were 3.9 (1.5-9.9) times more likely to resolve MetS than those in the lowest third.Conclusions: Higher levels of cardiorespiratory fitness protect against MetS and may resolve it in older individuals. [ABSTRACT FROM AUTHOR]

Abstract Background Major histocompatibility complex class II deficiency, also referred to as bare lymphocyte syndrome is a rare primary Immunodeficiency disorder characterized by a profondly deficient human leukocyte antigen class II expression and a lack of cellular and humoral immune responses to foreign antigens. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections. The infections begin in the first year of life and involve usually the respiratory system and the gastrointestinal tract. Severe malabsorption with failure to thrive ensues, often leading to death in early childhood. Bone marrow transplantation is the curative treatment. Case reports Here we report two cases with a late outcome MHC class II deficiency. They had a long term history of recurrent bronchopulmonary and gastrointestinal infections. Bone marrow transplantation could not be performed because no compatible donor had been identified. At the age of 12 years, they developed oral papillomatous lesions related to HPV (human papillomavirus). The diagnosis of HPV infection was done by histological examination. HPV typing performed on the tissue obtained at biopsy showed HPV type 6. The lesions were partially removed after two months of laser treatment. Conclusions Viral infections are common in patients with MHC class II and remain the main cause of death. Besides warts caused by HPV infection do not exhibit a propensity for malignant transformation; they can cause great psychosocial morbidity.

The impact of trace metal elements (TMEs) on plants is one current pollution problem, the severity of which is increasing with industrial development, population growth and inappropriate agricultural practices. The latter can have irreversible effects on ecosystems, including species extinction, trophic chain contamination and altered human health, particularly in the case of consumed plants such as zucchini squash ( Cucurbita pepo L.). This study aims to investigate the effects of nickel on various physiological and biochemical parameters of zucchini growth, with a particular focus on how this toxic metal impacts the quality of fruit that is consumed by humans. To achieve this, plants aged 45 days were grown for one month on solid media loaded with different concentrations of Ni (0, 100, 300 and 500 µM). The results showed that exposure of plants to Ni resulted in significantly altered growth and higher accumulation of Ni in the shoots (1314 µg·g -1 DW) than in roots and fruits. Concerning non-enzymatic antioxidants, the results showed that Ni toxicity significantly increased total polyphenols, especially in shoots at 300 µM Ni, while flavonoid content decreased in the roots and shoots in response to Ni treatment. Our results also show that nickel tolerance in C. pepo is ensured by a combination of several mechanisms such as an increase in the content of proline. This species can survive and tolerate, to different degrees, toxic cations at concentrations up to 500 µM but with visible symptoms of toxicity such as chlorosis of the leaves. Indeed, based on thresholds of hyperaccumulation, we can qualify Cucurbita pepo as a hyperaccumulator species of nickel.

The main objective of this study was to assess the impact of environmental pollution on the gastropod Stramonita haemastoma by examining various physiological and biochemical parameters. Trace metal elements (TME) were measured in the snail's foot. Over a one-year period from 2013 to 2014, the study investigated the variations in reduced glutathione, malondialdehyde, metallothionein, glutathione S-transferase, acetylcholinesterase and catalase activities in the foot of the gastropod at three different sites. The results showed spatiotemporal fluctuations in biomarkers, physiological parameters and TME. This integrative approach, being the first of its kind in the region using S. haemastoma as a model organism, represents a means of environmental assessment accessible to all countries allowing the monitoring and conservation of coastal marine environments on a national and international scale and which could be used in marine biomonitoring programs to indicate the impact of pollution and assess the quality of the Algerian coastal marine environment., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Purpose: Osteonecrosis of the femoral head (ONFH) is a degenerative and progressive disorder that mainly affects people with sickle cell disease (SCD). Herein, we aimed to search for a better understanding of markers that can act as risk factors for ONFH in patients with SCD., Methods: We conducted a retrospective study including 510 SCD patients followed over 23 years. Patients were divided into the ONFH group and the no-ONHF control group. Univariate and multivariate logistic regression analyses were performed to identify risk factors., Results: Among 510 SCD patients, 41(8%) were diagnosed with ONFH at a mean age of 167 months ± 64 (72-288). The cumulative incidence of ONHF increased from 2.3% at ten years to 18.3% at 20 years of age. The radiological grade 3 ONHF was predominant. No significant differences in sex, age at diagnosis of SCD, and Hb genotype were found between groups. The patient age and the time since diagnosis of SCD were statistically higher in patients with ONHF in univariate and multivariate analysis. ONHF was also associated with higher creatinine level (p = 0.001) lower LDH level (p = 0.006), and higher number of vaso-occlusive crisis (VOC)/patient/year (p < 0.001). The cumulative incidence of ONHF in patients having more than 3 VOC/year was significantly higher (43% versus 18.9% at 20 years, p < 0.001). In addition, infections, gallstones, growth delay, delayed initiation of hydroxyurea, and a higher transfusion rate were significantly associated with ONFH., Conclusion: These findings confirm that ONFH is closely related to the age, severity, and duration of SCD. Better management of this disease prevents acute and chronic complications, and early screening of the ONFH as soon as the first signs of the severity of the disease are detected provides a better functional prognosis., (© 2023. The Author(s) under exclusive licence to SICOT aisbl.)

Introduction: This study aimed to assess osteoarticular manifestations in patients with common variable immunodeficiency (CIVD) and to identify the predictive factors., Methods: This was a retrospective and prognostic study conducted in the pediatrics: immuno-hematology and stem cell transplantation department, including patients who fit the definition of CVID. A Cox model analysis was used to identify predictive factors., Results: A total of 36 patients were enrolled. Osteoarticular involvement was noted in 15 patients (42%) with a cumulative incidence of 90% after a median follow-up of 25 years. Non-infectious manifestations were reported in 14 patients (39%). The cumulative risk of inflammatory or autoimmune osteoarticular etiology was 74%. Well-characterized rheumatic diseases were retained in six patients and unlabeled autoimmune or inflammatory mechanism in five cases. Bone mineral density revealed osteoporosis in six cases leading to a cumulative risk of degenerative complications of 72%. The cumulative incidence of infectious complications was 17%. In multivariate analysis, predictors of osteoarticular complications were low body weight (HR = 8.67, CI: 1.496-50.278, p = 0.01) and hepatomegaly at diagnosis (HR = 6.2, CI: 1.537-25.075, p = 0.01). Reduced CD4 cells rate < 600 cells/mm 3 and hepatomegaly were predictors of autoimmune or inflammatory complications, while chronic diarrhea and iron deficiency were associated with degenerative manifestations., Conclusions: Osteoarticular manifestations have emerged as a real health problem for CVID patients. Risk increases with low body weight, hepatomegaly, chronic diarrhea, iron deficiency, and CD4 cells rate under 600 cell/mm 3 . Elucidating the mechanisms of these complications in CVID is important for developing preventive strategies. Key Points • This retrospective and prognostic study described the clinical characteristics of osteoarticular manifestations in 36 patients with CVID to ensure better recognition and understanding of this association by clinicians. • Identification of predictive factors of osteoarticular complications according to its etiology is crucial to establish appropriate, optimal and early management of patients at risk., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Trace metal element (TME) pollution is a major threat to plants, animals and humans. Agricultural products contaminated with metals may pose health risks for people; therefore, international standards have been established by the FAO/WHO to ensure food safety as well as the possibility of crop production in contaminated soils. This study aimed to assess the accumulating potential of aluminum and barium in the roots, shoots and fruits of Abelmoschus esculentus L., and their effect on growth and mineral nutrition. The content of proline and some secondary metabolites was also evaluated. After treating okra plants with aluminum/barium (0, 100, 200, 400 and 600 µM) for 45 days, the results showed that Al stimulated the dry biomass production, whereas Ba negatively affected the growth and the fructification yield. The okra plants retained both elements and exhibited a preferential accumulation in the roots following the sequence: roots > shoots > fruits, which is interesting for phytostabilization purposes. Al or Ba exposure induced a decline in mineral uptake (K, Ca, Mg, Zn and Fe), especially in roots and shoots. In order to cope with the stress conditions, the okra plants enhanced their proline and total phenol amounts, offering better adaptability to stress.

Aluminum (Al) is an ubiquitously present element in soil; it is considered as a major problem in crop production that affects plant growth and development on acid soils. The aim of this work was to determine the germination parameters, to quantify the water status and growth, to assess the Al accumulation, and antioxidant enzyme activities in plants to evaluate the stress exerted by aluminum in Cucumis sativus L. For germination test, increasing doses of Al were used (0, 200, 500, 1,000, and 2,000 μM). Results showed that germination was stimulated with 500 of Al. Aluminum effects on development were studied by treating the plants with different concentrations of Al (100, 200, 300, and 500 µM, Al) during 45 days. As regards to the plant's growth, water content, and dry biomass production there was a slight increase. On the other hand, the activities of the antioxidant enzymes were disturbed by aluminum stress. Data indicate that the catalase (CAT) activity showed a decrease in the different parts of the plant. However, guaiacol peroxidase (GPX) and ascorbate peroxidase (APX) activities were significantly stimulated. Studying the effects of Al-induced stress allowed us to conclude that cucumber has a high ability to accumulate this element in the roots., Competing Interests: The authors have declared that no conflicts of interests exist., (© 2021 The Authors. Food Science & Nutrition published by Wiley Periodicals LLC.)

Barium (Ba) is a nonessential element that can cause several deleterious effects in most organisms. Elevated Ba concentrations can be toxic for plants and may affect growth and disturbances in homeostasis. This study aimed to evaluate the Ba stress, the plant-tolerance limits, and the detoxification strategy adopted by Cucumis sativus L. The effect of Ba on seed's germination and vegetative development of this species was evaluated. For germination test, different Ba concentrations were used (0, 200, 500, 1,000, and 2,000 μM). Results showed that germination was stimulated with 500 and 2,000 µM of Ba. The toxicity effect on plant development was studied by treating the plants with increasing doses of Ba (100, 200, 300, and 500 μM) during 45 days. Shoot and root dry biomass production decreased significantly with elevated Ba concentrations, although water content enhanced in the roots. The concentration of Ba, 500 µM, induced high Ba accumulation in shoots and roots (9 times higher than in the control plants). Moreover, results showed that catalase, guaiacol peroxidase, and ascorbate peroxidase activities were stimulated in the different tissues of cucumber plants which highlight the occurring of an oxidative damage through Ba treatments and the involvement of the plant enzymatic antioxidant defense system., Competing Interests: The authors have declared that no conflicts of interests exist., (© 2021 The Authors. Food Science & Nutrition published by Wiley Periodicals LLC.)

This study was aimed to identify the predictors of splenic sequestration crisis (SSC) among pediatric patients with sickle cell disease (SCD). This prognosis study was carried out in the pediatric immuno-hematology unit, over 20 years (1998 to 2017), enrolling patients with SCD. The cox model was used in multivariate analysis. Among 423 patients with SCD (240 S/S phenotype, 128 S/B0, 30 S/B+, 14 S/O arab and 11 S/C), 150(35.4%) had at least one episode of SSC. The average age of patients at the first episode was 48.3 months ± 32.4(2-168). Recurrence of SSC was observed in 117 patients (78%). Spleen size ≥3 cm at baseline was the strongest predictor of SSC occurrence (HR = 7.27, CI: 4.01-13.20, p = 0.05) and recurrence (HR = 6.37, CI: 1,46-27.83, p = 0.01). Pallor revealing the disease, age at onset of symptoms <24 months and reticulocytosis ≥300,000/mm 3 increased the risk of SSC. Pain crisis revealing the disease as well as neutrophilia was associated with a lower risk of SSC. In conclusion, this study confirmed the high prevalence of SSC in SCD and the high frequency of recurrence after a first episode. The SSC occurrence and recurrence were intimately linked to the presence of splenomegaly, chronic pallor revealing the disease as well as reticulocytosis., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Consanguinity increases the incidence of recessive diseases such as beta-thalassaemia major (βTM), one of the most prevalent lethal inherited diseases in the world. Aim: This study aims to identify the frequency of endogamy and consanguinity in two Mediterranean βTM populations and to study the implication of socio-economic factors. Subjects and methods: A trans-sectional study was conducted in 203 Tunisian families and 75 Italian families. Data were collected using a questionnaire completed by patients and parents. Results: Complete endogamy and consanguinity were observed in 82.75% and 62.56% of Tunisian families, respectively. Complete endogamy was found in 90.67% of Italian families, no consanguinity was noted. The low occupation status of Tunisian mothers was associated with an increasing frequency of consanguinity ( p  = .01) and endogamy ( p  = .0003). Consanguinity was associated with low education level ( p  = .012) and low occupation status ( p =.047) of fathers. No significant association was found between endogamy and socio-economic factors in the Italian sample. Conclusions: High consanguinity and endogamy rates in Tunisian families may explain the frequency of βTM in Tunisia. The high endogamy rate in Italian families could also increase the frequency of βTM. Identification of geographical distribution and socio-economic factors leading to endogamy and consanguinity in these populations might help to improve βTM prevention.

In beta-thalassemia patients, erythrocyte autoantibodies can remain silent or lead to Autoimmune Hemolytic Anemia (AIHA).The aim of this study was to identify predictors of AIHA in beta-thalassemia patients with positive Direct Antiglobulin Test (DAT), in Tunisia. This longitudinal prognosis study was carried out on beta-thalassemia patients with a positive confirmed DAT. Predictors of AIHA were identified the Kaplan-Meier method. A Cox model analysis was used to identify independent predictors. Among 385 beta thalassemia patients, 87 developed positive DAT (22.6%). Autoimmune hemolytic anemia was occurred in 25 patients. Multivariate analysis showed that AIHA was independently associated with beta-thalassemia intermedia and similar family history of AIHA. Splenectomy in patients with positive DAT was independently associated with an increased risk of AIHA (HR = 6.175, CI: 2.049-18.612, p < 0.001). The risk of developing AIHA was higher during the first 72 transfusions. Autoimmune hemolytic anemia was significantly associated with polyspecific DAT (anti-complement and anti-IgG), blood group AB and prior alloimmunization. Whereas transfusion by phenotypic and leukoreduced blood was a protective factor. In summary, splenectomy after autoimmunization, prior alloimmunization, DAT specificity (IgG with complement), thalassemia intermedia, AB blood group and family history of AIHA were strongly associated with AIHA. Leukoreduced blood transfusion had a proven preventive role., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children. We conducted a prospective study on two hundred and ninety children with immune deficiency. Demographic details (including age, sex, and consanguinity) with personal and family history were recorded. Special attention was paid to cutaneous manifestations. Dermatological involvements were grouped according to the etiology of their most prominent sign. Cutaneous manifestations were found in 164 patients (56.5%). They revealed the diagnosis of PIDs in 71 patients (24.5 %). The mean age at presentation was 21 months. Overall the most prominent cutaneous alterations were infectious. They accounted for 106 cases (36.55%). The most prevalent causes of cutaneous infections were bacterial: 93 cases (32.06%). Immuno-allergic skin diseases were among the common findings in our study. These include eczematous dermatitis found in 62 cases (21.38%). Malignancy related PIDs was seen in a boy with Wiskott Aldrich syndrome. He developed Kaposi's sarcoma at the age of 14 months. Cutaneous changes are common among children with PIDs. In pediatric patients with failure to thrive, chronic refractory systemic manifestations often present in other family members, recurrent cutaneous infections unresponsive to adequate therapy, atypical forms of eczematous dermatitis or unusual features should arouse the suspicion of PIDs and prompt specialized immunologic consultation should be made., Competing Interests: Competing interests: The authors have declared that no competing interests exist.

β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. This single center investigation enrolled 28 β-TM patients (16 males, 12 females) regularly transfused with packed red cell since early years of life. For each patient were determined: age, sex, number of transfusions received, history of splenectomy and anthropometric parameters. All patients underwent an evaluation of hormonal status including growth, gonadal, thyroid, adrenal cortex, and parathyroid glands. Dual-energy X-ray absorptiometry was used to diagnose low bone mass. Assessment of iron overload status was performed by measuring the serum ferritin concentration and the results of magnetic resonance imaging T 2 *. Growth retardation was found in 16 of the 28 studied patients (57 %). Thirteen among them had delayed puberty. Spontaneous puberty was achieved in 16 cases. Growth hormone (GH) deficiency was found in 10 cases (35 %). Seventeen among the studied patients (60 %) developed disorders of glucose homeostasis. Subclinical hypothyroidism was found in six patients (21 %). Intensive chelation therapy had allowed the reversibility of this complication in five cases. Adrenal Insufficiency was observed in 9 cases (32%). Hypoparathyroidism has occurred in one case. Ten of the 28 studied patients had low bone mass (35%). Twenty-three of the 28 studied patients (82%) had at least one endocrine complication., Competing Interests: Competing interests: The authors have declared that no competing interests exist.

Aim: To report a new case of hypoparathyroidism in a child with ß-thalassemia major, Case: We report a case of a 17-year-old Tunisian girl with transfusion-dependent thalassemia major presented with paresthesia and pubertal delay. Laboratory investigations showed hypocalcaemia and hyperphosphatemia. Parathyroid hormone level was low (2 ng/l, normal range: 12-72 ng/l) than expected for the degree of hypocalcaemia. Serum ferritin concentration was 1770ng/ml. The patient was started on oral daily calcium supplementation, Alfa calciferol and intensive iron chelation therapy. Follow-up after 6 and 12 months revealed normal Calcium and ECG showed QT interval within normal range., Conclusion: Investigating calcium homeostasis at regular intervals and early management of any abnormality can preclude the occurrence of complications.

The aim of this study was to access average delays for novogeneration of myeloid and lymphoid cells after allogeneic bone marrow transplantation (BMT) outcome and factors affecting this organization. A prospective analysis over 2 years (01/01/07 to 31/12/08) enrolling 19 children treated with allogeneic intrafamilial bone marrow transplantation. Indications for bone marrow transplantation were: aplastic anemia (3 cases), bemoglobinopathies (9 cases), myelodysplastic syndrome (1 case) and primary immunodeficiency (6 cases). Different conditioning regiments were used according to the indication. The study of immune reconstitution was based on the quantitative determination of immunoglobulin and lymphocyte subpopulation. These tests were routinely requested to 1 month, 2 months, 3 months, 6 months, 9 months and 12 months. The average time of engraftment was 18 days (12-24). A rate of CD4+T lymphocytes>200/mm3 was provided within an average of 2,5 months (1-7). The average time to obtain CD8+T lymphocytes>200/mm3 was 2 months (1-5). The humoral immune reconstitution was made within an average of 2 months (1-4). A report of CD4+/CD8+T lymphocytes>I was obtained within 10 months and a half (1-24). Univaried analysis showed a correlation between the bone marrow sex matched and the faster reorganization of CD8+T cells (p=0.042). A quantity of CD34+>6 10(6)/kg was significantly associated with the recapture of a formula lymphocyte CD4+/CD8+T>1 (p=0.03) Immune recovery post bone marrow transplantation in children begins with myeloid lineage then lymphoid B then lymphoid T The inversion of the report CD4+/CD8+T lymphocytes, seems to be influenced by the high contain of CD34+cells in the graft as well as the type of conditioning.

Background: Permanent reception at the level of hospital emergencies should be a sanitary priority., Aim: In order to set up a data base that allows identifying failures, to bring forward a certain number of solutions and to elaborate a strategy for promoting first-line emergency services, Methods: We carried out a retrospective descriptive survey of 20853 consultants in the first-line emergency ward of the district hospital of Nefza over a one-year period (2001)., Results: At the end of our analysis, we found the following main results: the profile of the emergency consultant is a young person (average age 31.76), of female sex in 52% of cases and benefiting from social security (CNSS or CNRPS) in 40.5% of cases. The average number of consultations is of 57 consultations per 24 hours: essentially during August, October and March. The reason for having recourse to emergency services is dominated by the medico-surgical pathology in 78% of cases and 15.5%. In our survey 3% of the patients were referred to second-line emergencies; the referring motives are essentially dominated by traumatisms (39%)., Conclusion: one patient out of two is consulting for a false emergency case, probably for reasons of easy access to the emergency services and because of the absence of a day consultation in the basic health care units. On the other hand, the non-availability of diagnostic means in the afternoon and evening increases the number of 2nd line emergency recourse.