Particularities of Sjögren Syndrome in elderly.

INTRODUCTION: Diagnosis of Sjögren syndrome (SS) can be difficult in the elderly in whom sicca syndrome can be related to senescence, comorbidities or to iatrogenesis. METHODS: We performed a retrospective study including of SS patients records (AECG criteria) in the internal medicine departement, La Rabta Hospital over 18 years. Epidemiological, clinical, biological and therapeutic features of elderly patients (EP) and young patients(YP) were compared Results: A total of 323 patients with SS were enrolled, 35 were over 65 years of age (33 females/2 males). The mean age at disease onset was 68.8±4.4 years. Comparative analysis showed that SS diagnosis was made earlier in elderly (p=0.02). Fatigue was more frequent in elderly (p<0.01). Positivity of anti-SSA was more frequent in YP (p=0.04). Anti-malarial agents were less prescribed in elderly (p=0.03). There was no significant differences concerning the other clinical features, laboratory findings, treatment and outcomes. CONCLUSION: The SS in elderly seems not to be a distinct subset of disease. However, treatment and follow-up of elderly patients with SS must obey to closer attention considering their vulnerability and the complexity of their management.

Influence of diet quality on nutritional status of school-aged children and adolescents in Zanzibar, Tanzania.

BACKGROUND: Malnutrition among young children and adolescents poses a serious health challenge in developing countries which results in many health problems during adulthood. Poor diet quality is known as the root cause of malnutrition which is caused by unhealthy food choices and bad eating habits among young children and adolescents. However, limited evidence is available on diet quality and its association with nutrition status among young children and adolescents in Zanzibar. This study examined the diet quality and its relationship with the nutritional status of school-aged children and adolescents in Zanzibar. METHODS: Data for this study was obtained from the cross-sectional survey of School Health and Nutrition (SHN) conducted in Zanzibar. The survey recruited children aged 5-19 years from 93 schools in Zanzibar. A seven-day food frequency questionnaire (FFQ) was used to assess dietary intake. Prime Dietary Quality Score (PDQS) consisted of 21 food groups was then constructed to assess the diet quality of school-aged children and adolescents. Body mass index (BMI-for-age Z-score) was used as the indicator of nutrition status. Both linear and logistic regression analysis techniques were used to determine the associations between BMI and PDQS. RESULTS: A total data of 2,556 children were enrolled in the survey. The prevalence of thinness was 8.1%, normal 82.1%, overweight 7.2% and obesity 2.6%. The mean (SD) PDQS score was 18.8 (3.2) which ranged from 8 to 33. Consumptions of green leafy vegetables (49.3%), yellow or red fruits (37.8%), legumes (38.3%), fish (36.3%), and vegetable oil (31.5%) were up to three times per week, whereas consumptions of white vegetables (77.3%), cooked vegetables (32.6%), citrus fruits (66.8%), other types of fruits (66.2%), nuts (46.4%), poultry (49.6%), whole grains (61%) and eggs (67.8%) were less than once per week. In terms of unhealthy foods, eating fried foods was reported by 26.3% up to three times per week, and 31.5% reported consuming sweets and ice cream up to three times in the past week. High PDQS was significantly associated with a reduction in BMI of children (p< 0.005). For each unit increase in the consumption of unhealthy foods such as fried foods, cooked vegetables and refined grains there is a significant increase in BMI. The odds of being obese decrease significantly as diet quality increases from the first to third quintile of PDQS (AOR = 0.2, 0.04-0.89 95% CI, p = 0.035). CONCLUSION: Consumption of high quality diet was found to be associated with a reduction in excessive weight among school-aged children and adolescents in Zanzibar. There is a need for interventions targeting to reduce unhealthy food consumption in school environment. Further research should be conducted to assess diet quality using PDQS among young children and adolescents.

Adult-onset Still's disease after SARS-Cov-2 infection.

Adult-onset Still's disease (AOSD) is an uncommon inflammatory disorder. AOSD and SARS-Cov-2 infection share clinical and laboratory features, including systemic inflammation. A 19-year-old woman had prolonged fever for 3 weeks, joint pain, and biological inflammatory syndrome. Post COVID-19 AOSD was diagnosed. SARS-Cov-2 infection induces many inflammatory diseases including AOSD.

Erdheim-Chester disease: A multisystem disease case illustration with rare manifestations and treatment challenges.

Erdheim-Chester disease is a rare multisystemic disease. A 50-year-old woman, presented with a recurrent pain and swelling of the left knee. Bone scintigraphy showed increased tracer uptake of peripheral skeleton. The computed tomography showed tissular infiltration in the retroperitoneum, around the vessels. Immunohistochemistry showed CD68 (+) and CD1a (-).

Particularities of infectious complications in systemic lupus erythematosus.

AIM: To describe characteristics of systemic lupus erythematosus (SLE) patients with infectious complications and to determine frequency, clinical and microbiological features and outcomes of reported infections. METHODS: This is a descriptive, retrospective study conducted over an 11-year period at the Internal Medicine Department La Rabta Hospital Tunis, collecting medical records of SLE patients who had experienced infectious complications. RESULTS: Fifty-six patients were included, consisting of 52 females and 4 males (gender ratio M/F= 0.07). The mean age at SLE diagnosis was 35±13.8 years. The mean duration of the disease was 4.8±3.1 years. A total of seventy-eight infections were documented. Infection revealed the disease in 12 patients (21%) and occurred after an average delay of 36 months [1-156 months] of SLE diagnosis. Forty-three patients (74%) were receiving corticosteroid therapy, associated in 37.5% of cases with immunosuppressive treatment. Urinary and pleuro-pulmonary infections were most common infectious sites. An infectious agent was identified in 59 cases (76%). Bacterial infections were the most common (76%), dominated by the enterobacteria pathogen agent. Viral infections (n=12) were mainly caused by varicella-zoster virus and cytomegalovirus. Five patients required intensive care. Twenty patients experienced a lupus flare during the infectious episode. The outcome was favorable in 52 (93%) patients. Three patients died, two due to septic shock caused by pulmonary infection in two cases and cutaneous infection in one patient. One patient died from a probable pulmonary embolism. CONCLUSION: Infectious complications are responsible for significant morbidity and mortality during SLE. Hence the importance of early diagnosis and adequate management.

Heritage language learners of English: Linguistic gaps and cognitive strengths.

PURPOSE: This study examined whether Heritage Language Learners (HLLs) of English display profile effects in their performance on knowledge- and processing-dependent measures relative to the standardised mean scores of monolingual speakers. The study also investigated the influence of several experiential factors on HLL performance. METHOD: Participants were 59 Arabic-speaking HLLs from six to nine years old. The children completed a battery of linguistic tests in their L1 and L2, as well as cognitive measures of short-term and working memory and non-verbal intelligence. RESULT: Significantly lower standardised scores were observed for HLLs as compared to the standardised mean scores on all Arabic/English language tasks except L2 word reading. HLLs scored at or above age-level expectations on cognitive measures except the Arabic nonword repetition task. Stepwise regression analyses examining variance in HLLs' performance, age and richness of environment consistently explained HLLs' performance in L1 Arabic, but different factors accounted for HLLs' performance in English depending on the task. Age was the only variable that consistently explained variance in performance on the cognitive measures. CONCLUSION: The results suggest that processing-dependent measures may be less sensitive to difference in language experience than traditional knowledge-based measures such as standardised measures of language and vocabulary.

Management of patients with cardiovascular diseases during Ramadan.

During the month of Ramadan, over one billion Muslims observe a water and food fast from sunrise to sunset. The practice of this religious duty causes marked changes in eating and sleeping habits. With the increasing incidence of cardiovascular (CV) risk factors, the number of patients with CV pathologies who wish to fast is increasing worldwide, and in Tunisia, which is ranked as a high CV risk country. If fasting has been shown to be beneficial for the improvement of some metabolic parameters, its practice in patients with CV pathology remains debated. The Tunisian Society of Cardiology and Cardiovascular Surgery (STCCCV) in consultation with the National Instance of Evaluation and Accreditation in Health (INEAS) has established this document in the form of a consensus after having analysed the literature with the aim of addressing these questions: -What is the impact of fasting in patients with CV pathologies? -How to stratify the risk of fasting according to CV pathology and comorbidities? -How to plan fasting in patients with CV diseases? -What are the hygienic and dietary measures to be recommended during fasting in patients with CV pathologies? -How to manage medication during the month of Ramadan in patients with CV diseases?

Unilateral pseudouveitis revealing a pancreatic neuroendocrine carcinoma: A case report.

Neuroendocrine tumors are a heterogeneous group of tumors with a wide range of malignant potential that tend to have a relative prolonged course. These tumors infrequently metastasize to the orbit. To the best of our knowledge, ocular metastases from pancreatic neuroendocrine tumors (PNETs) have never been reported in the literature. We report the case of a 61-year-old man who presented with progressive deterioration of general condition with unilateral recurrent episodes of non-granulomatous panuveitis of the left eye related to a choroidal metastasis. Radiological imaging and histopathological analyses led to the diagnosis of metastatic pancreatic neuroendocrine carcinoma as the primary tumor. Choroidal metastases from neuroendocrine tumors are extremely rare, but compromise patients' well-being because of visual impairment. Uncommonly, these metastases can be the first manifestation of unknown tumors, warranting further investigations to detect the primary cancer.

Aplastic anemia secondary to tyrosine kinase inhibitor therapy in a patient with chronic myeloid leukemia.

INTRODUCTION: Nilotinib, as the second generation of tyrosine kinase inhibitor, has significant efficacy in patients with chronic myeloid leukemia resistant or intolerant to Imatinib. Aplastic anemia induced by tyrosine kinase inhibitors is an uncommon complication. CASE REPORT: A 34-year-old female case with CML in the chronic phase was treated with Imatinib in first-line therapy. Nilotinib was switched because of failure to achieve complete cytogenetic response at 6 months following Imatinib. Three years with Nilotinib, the patient developed a persistent pancytopenia grade 4 while a major molecular response was achieved. MANAGEMENT & OUTCOME: Nilotinib was discontinued. However, the hematologic finding of the patient had not recovered after three months. A bone marrow biopsy showed marked hypocellularity and fatty tissue without evidence of myelofibrosis. Immunosuppressive therapy was started. Unfortunately, the patient died due to septic and hemorrhagic shock nine months after Nilotinib interruption. According to Naranjo's algorithm, the causality relationship with the drug is probable with a score of 5. DISCUSSION: Aplastic anemia is an uncommon adverse event of tyrosine kinase inhibitors but it can be a fatal complication. The early diagnosis of aplastic anemia related to Nilotinib therapy is needed to avoid further detrimental effects of the drug.

Prevalence and Determinants of Anemia among Children in Zanzibar, Tanzania: Analysis of Cross-Sectional Population Representative Surveys.

The aim of this study was to assess the prevalence of anemia and its determinants among children aged 6-59 months in Zanzibar, Tanzania, from 2005 to 2015. We used secondary data from the Tanzania Demographic and Health Surveys from three different periods: 2005, 2010, and 2015. A sample of 3502 child-mother pairs from three survey datasets was used to analyze the overall prevalence of anemia and reveal its determinants. Hierarchical logistic regression was used to demonstrate the prevalence odds ratio of factors, both from the mothers and children, for anemia prevalence. The study indicated a significant decrease in anemia prevalence from 76.1% in 2005 to 65.4% in 2015 (p < 0.001). Hierarchical logistic regression between variables and anemia showed a significant association (p = 0.02) with households that improperly disposed of stool, children with minimum dietary diversity (p = 0.041), children in low age quartiles (p = < 0.001), and underweight children (p = 0.025). Maternal, household characteristics, and child factors were associated with childhood anemia in Zanzibar, Tanzania. Despite the significant decrease of anemia in Zanzibar, the overall prevalence rate is still a significant public health concern. Designing and strengthening comprehensive interventions to address anemia in the general population and different categories should be given special consideration.

TH17 cells expressing CD146 are significantly increased in patients with Systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune disorder characterized by vascular damage, excessive fibrosis and abnormal T cells immune-regulation. CD146 is an adhesion molecule essentially expressed in the vascular system, but also on TH17 lymphocytes. In view of the recently described role of CD146 in SSc, we hypothesized an involvement of CD146 positive TH17 cells in this disease. Compared to healthy controls, we showed that both soluble form of CD146 (sCD146), and IL17A levels were increased in patients with SSc with a positive correlation between both factors. A significant increase in TH17 cells attested by an increase of RORγT, IL17A mRNA and CD4+ IL17A+ cell was observed in patients with SSc. Interestingly, the percentage of TH17 cells expressing CD146 was higher in patients with SSc and inversely correlated with pulmonary fibrosis. In vitro experiments showed an augmentation of the percentage of TH17 cells expressing CD146 after cell treatment with sCD146, suggesting that, in patients the increase of this sub-population could be the consequence of the sCD146 increase in serum. In conclusion, TH17 cells expressing CD146 could represent a new component of the adaptive immune response, opening the way for the generation of new tools for the management of SSc.

Association of systemic beta-defensin-1 and -20G/A DEFB1 gene polymorphism with Behçet's disease.

BACKGROUND: Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology. Beta-defensins are antimicrobial peptides involved in epithelial host defense. To explore whether beta-defensins might be involved in BD pathogenesis, we examined plasma human beta-defensin-1 (hBD-1) and DEFB1 -20G/A polymorphism in BD patients. METHODS: This case-control study included 106 BD patients fulfilling the criteria of the International Study Group for BD and 156 controls. The -20G/A genotypes were determined by PCR-RFLP analysis in all participants, and plasma hBD-1 was assessed by ELISA in 77 BD patients and 44 controls, only. Stepwise multiple regression models were applied to determine independent predictors for plasma hBD-1 in BD patients. RESULTS: Distribution of -20G/A genotypes was different between BD patients and controls. Compared to GG genotype, "GA" genotype [OR (95% CI), 3.12 (1.56-6.16); p = .001] and "AA" genotype [2.57 (1.10-5.96); p = .027)] were associated with increased risk for BD. Plasma hBD-1 concentrations were significantly higher in BD patients than controls (9.81 ±â€¯3.52 ng/mL vs. 5.30 ±â€¯3.02 ng/mL; p < .001), and in BD patients with neurological involvement than those without (11.1 ±â€¯4.12 ng/mL vs. 9.19 ±â€¯3.10 ng/mL; p = .040). No variation was noted according to other clinical features, treatment received or -20G/A genotypes. In multivariate analysis, neurological involvement was the only predictor for plasma hBD-1 (ß, 0.274; p = .029). CONCLUSIONS: Findings suggest that hBD-1 and its encoding gene DEFB1 could modulate the risk for BD, especially for BD neurological involvement. Further work is needed for a better understanding of role of hBD-1 and its genetic variants in the pathogenesis of BD.

[Panniculitis in patient undergoing treatment for dermatomyositis with methotrexate]. / Panniculite au cours d'un traitement d'une dermatomyosite par du méthotrexate.

Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM). The appearance of panniculitis during treatment with methotrexate (MTX) is exceptional and has only been described in 3 cases. We report a case of a 50-year-old woman suffering from DM since 1997 who was treated with corticosteroids showing favorable clinical and biological evolution. When a relapse occurred 2 years later, she was treated with higher-dose of corticosteroids in combination with a 7,5 mg weekly dose of methotrexate. The evolution was rapidly favorable. Eighteen months later, the patient had multiple subcutaneous nodules on limbs and buttocks. Anatomopathological examination showed panniculitis. There was no evidence supporting progression in DM. Prednisone dose was increased to 0.5 mg/kg/day, always in combination with MTX, without any clear signs of improvement. MTX treatment was stopped and the cutaneous lesions completely disappeared in 2 months without any relapse. This objective response lasted for 42 months. Our observation is particular given the occurrence of panniculitis in a patient undergoing treatment for dermatomyositis with methotrexate and illustrates the difficulties in the diagnosis. This entity must be known despite its exceptional nature since cutting off MTX treatment generally induces the disappearance of subcutaneous nodules.

Pleural myelomatous involvement in multiple myeloma: five cases.

Pleural myelomatous involvement in multiple myeloma (MM) is rare, occurring in less than 1% of cases. We retrospectively studied five cases of patients with MM who developed myelomatous pleural effusions. Three men and 2 women with a mean age of 61 years presented with myelomatous pleural effusion. The pleural fluid electrophoresis revealed a peak of IgG in three cases, of IgA in one case, and of lambda light chains in one case, which were identical to that in the sera of the patients. Detection of typical plasma cells in pleural fluid cytology was contributive, and histologic confirmation by pleural biopsy was positive in four cases. Treatment consisted of chemotherapy. The clinical outcome was initially good, but relapses occurred in all cases early and were complicated by fatal infections. Myelomatous pleural effusion is a rare affection. It is usually a late complication associated with poor prognosis.

[Gaucher's disease uncovered late]. / Maladie de gaucher de decouverte tardive: a propos de deux cas.

Gaucher's disease is an uncommon inborn recessive autosomal disease, due to a deficient activity of the lysosomal enzyme beta glucocerebrosidase. This disease is usually diagnosed in the first or second decade of life with the arising of bone pains, splenomegaly and hemorragic manifestations due to thrombocytopenia. When the enlarged spleen is not evident, or after splenectomy, patients may be mis-identified as having Gaucher's disease. We present here two cases of elderly patients aged 70 and 46 years respectively, in whom the disease was a surprising finding of bone marrow examination, during check up for pancytopenia.