[Vascular manifestations of Behcet's disease]. / Les complications vasculaires de la maladie de Behçet.

Behçet disease is a multi-systemic complex vasculitis with unknown etiology characterized by different clinical involvements, including mucocutaneous, ocular, vascular, articular, neurological and gastrointestinal manifestations. Growing evidence supports that different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, the vascular phenotype identifies a specific group of patients who suffer from recurrent inflammatory thrombosis and arterial involvement. Vascular disease develops in up to 40% with a definite male preponderance and is usually an early manifestation. It is one of the main causes of death in Behçet's disease. Venous involvement is significantly more common than arterial disease and lower extremity deep vein thrombosis is its most frequent manifestation. Arterial disease involves mostly pulmonary arteries and aorta and manifests mainly in the form of aneurysms. Glucocorticoids and immunosuppressant's are the recommended first-line treatments in vasculo-Behçet. Furthermore, randomized controlled trials are still needed to assess the role of adding anticoagulation to current standard therapy in venous thrombosis in Behçet's disease and to assess the role of anti-TNF alpha therapy in vasculo-Behçet.

ADHD and addictive behavior in crack-cocaine users.

BACKGROUND: Crack consumption is a major public health issue in Martinique with a poor prognosis. A preliminary study has found a high prevalence of history of childhood ADHD (C-ADHD) in crack users. OBJECTIVE: To determine the prevalence of C-ADHD and adult ADHD (A-ADHD) in crack users and their potential associations with substance use behavior. METHODS: All consecutive patients consulting in the public academic hospital covering 376,000 inhabitants were included in the present study and received a comprehensive battery measuring addictive behavior, psychiatric and somatic comorbidities. C-ADHD groups and A-ADHD groups were defined with the Wender-Utah Rating Scale-25 and the Brown ADD Rating Scale, respectively. Impulsivity was evaluated with the Barratt Impulsiveness Scale (BIS-11). FINDINGS: In total, 111 participants were evaluated. Among them, 50 (45%) were classified in the C-ADHD group and 20 (18%) in the A-ADHD group. Compared to the patients without ADHD, those with ADHD were found to have higher impulsivity (C-ADHD: BIS total score 67.90 (10.1) vs. 63.28 (10.5), P=0.021, BIS attentional score 17.5 (3.6) vs. 15.3 (3.4), P=0.002, A-ADHD: BIS total score 75.1 (11.3) vs. 63.4 (9.2), P<0.001, BIS motor impulsivity 26.9 (5.3) vs. 22.6 (4.3), P<0.001, BIS attentional score 19.3 (3.3) vs. 15.6 (3.5), P<0.001, BIS planification 28.9 (5.7) vs. 25.10 (4.7), P=0.003). Fifty percent of A-ADHD patients were found with high impulsivity vs. 15% of patients without A-ADHD (P<0.001). However, ADHD was not associated with more severe addictive behavior or history of legal consequences. INTERPRETATION: ADHD prevalence is high in cocaine-crack users and associated with increased impulsivity. However, neither ADHD nor impulsivity explains addictive behaviors or legal consequences.

Ibuprofen is deleterious for the development of first trimester human fetal ovary ex vivo.

STUDY QUESTION: Does ibuprofen use during the first trimester of pregnancy interfere with the development of the human fetal ovary? SUMMARY ANSWER: In human fetuses, ibuprofen exposure is deleterious for ovarian germ cells. WHAT IS KNOWN ALREADY: In utero stages of ovarian development define the future reproductive capacity of a woman. In rodents, analgesics can impair the development of the fetal ovary leading to early onset of fertility failure. Ibuprofen, which is available over-the-counter, has been reported as a frequently consumed medication during pregnancy, especially during the first trimester when the ovarian germ cells undergo crucial steps of proliferation and differentiation. STUDY DESIGN, SIZE, DURATION: Organotypic cultures of human ovaries obtained from 7 to 12 developmental week (DW) fetuses were exposed to ibuprofen at 1-100 µM for 2, 4 or 7 days. For each individual, a control culture (vehicle) was included and compared to its treated counterpart. A total of 185 individual samples were included. PARTICIPANTS/MATERIALS, SETTING, METHODS: Ovarian explants were analyzed by flow cytometry, immunohistochemistry and quantitative PCR. Endpoints focused on ovarian cell number, cell death, proliferation and germ cell complement. To analyze the possible range of exposure, ibuprofen was measured in the umbilical cord blood from the women exposed or not to ibuprofen prior to termination of pregnancy. MAIN RESULTS AND THE ROLE OF CHANCE: Human ovarian explants exposed to 10 and 100 µM ibuprofen showed reduced cell number, less proliferating cells, increased apoptosis and a dramatic loss of germ cell number, regardless of the gestational age of the fetus. Significant effects were observed after 7 days of exposure to 10 µM ibuprofen. At this concentration, apoptosis was observed as early as 2 days of treatment, along with a decrease in M2A-positive germ cell number. These deleterious effects of ibuprofen were not fully rescued after 5 days of drug withdrawal. LARGE SCALE DATA: N/A. LIMITATIONS, REASONS FOR CAUTION: This study was performed in an experimental setting of human ovaries explants exposed to the drug in culture, which may not fully recapitulate the complexity of in vivo exposure and organ development. Inter-individual variability is also to be taken into account. WIDER IMPLICATIONS OF THE FINDINGS: Whereas ibuprofen is currently only contra-indicated after 24 weeks of pregnancy, our results points to a deleterious effect of this drug on first trimester fetal ovaries ex vivo. These findings deserve to be considered in light of the present recommendations about ibuprofen consumption pregnancy, and reveal the urgent need for further investigations on the cellular and molecular mechanisms that underlie the effect of ibuprofen on fetal ovary development.

Design of a typical residence for the elderly in Morocco.

The population of Moroccan elders is in full increase; their requirements for quality and quantity of services are becoming increasingly important. On the basis of this, reality and extension of many major innovative projects in Morocco (industrial expansion plan, renewable energy stations, the road infrastructure network, rural electrification, drinking water, accelerated urbanization, globalization...) gain importance. Reflection on the design of a typical residence for the elderly has become an ambitious idea possessing all the chances of its success; it is also worth noting that it is a citizen opportunity to be seized by all political decision-makers for the promotion of health and the improvement of the quality of life of a growing category of the population. The typical residence of the elderly remains not only a place of life but also an environment of therapeutic care and at different levels of autonomy and dependence of our elders.

Venous thromboembolism and hyperhomocysteinemia as first manifestation of pernicious anemia: a case series.

BACKGROUND: Hyperhomocysteinemia has been suspected of favoring thrombosis. Several case-control studies and even a meta-analysis have confirmed a link between venous thrombosis and hyperhomocysteinemia. Homocysteine is due to genetic and acquired factors (poor diet in folate and vitamin B12, older age, renal impairment, thyroid diseases, and malignancies) induced by the intake and the concentrations of vitamin B9 or B12 in the majority of cases. CASES PRESENTATION: We report the cases of four Moroccan patients who presented with acute vein thrombosis of different sites: a 34-year-old man, a 60-year-old man, a 58-year-old man, and a 47-year-old woman. All patients had a low level of cobalamin with marked hyperhomocysteinemia with normal serum and red cell folic acid. Venous thrombosis revealed pernicious anemia in all patients. Their low levels of cobalamin, atrophic gastritis, and positive results for gastric parietal cell antibodies confirmed the diagnosis of pernicious anemia. There was no evidence of immobilization, recent surgery, malignancy, antiphospholipid antibody, myeloproliferative disorder, or hormone replacement therapy. No deficiencies in protein C and protein S were detected; they had normal antithrombin III function and factor V Leiden; no prothrombin gene mutations were detected. Treatment included orally administered anticoagulation therapy and cobalamin supplementation. The outcome was favorable in all cases. CONCLUSIONS: These reports demonstrate that pernicious anemia, on its own, can lead to hyperhomocysteinemia that is significant enough to lead to thrombosis. Understanding the molecular pathogenesis of the development of thrombosis in patients with hyperhomocysteinemia related to Biermer disease would help us to identify patients at risk and to treat them accordingly. The literature concerning the relationship between homocysteine and venous thrombosis is briefly reviewed.

Kikuchi-Fujimoto disease: report of 4 cases and review of the literature.

INTRODUCTION: Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign disease of unknown origin predominantly affecting young women and presenting in the form of cervical lymphadenopathy and/or prolonged fever. MATERIAL AND METHODS: The authors report 4 cases of Kikuchi-Fujimoto disease diagnosed in the Internal Medicine Department of Ibn Sina university hospital in Rabat between 2009 and 2010. RESULTS: These 4 women with a mean age of 27±8.6years [16-37] were admitted with febrile syndrome and cervical lymphadenopathy. The diagnosis was based on histological examination of a lymph node biopsy. The disease was associated with systemic lupus erythematosus in one case and actinomycosis in another case. A favourable course was observed in response to corticosteroid therapy in two patients, antibiotic therapy in one patient and antipyretic treatment alone in the fourth patient. CONCLUSION: In the light of these four cases, the authors discuss the diagnostic difficulties, the modalities of treatment of Kikuchi-Fujimoto disease and its clinical course.

[Kikuchi-Fujimoto's disease: Report of 4 cases and literature review]. / Maladie de Kikuchi-Fujimoto : à propos de quatre cas et revue de littérature.

INTRODUCTION: Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis is a benign disease predominantly occurring in young women which etiology remains unknown and revealed by cervical lymphadenitis and/or prolonged fever. OBSERVATIONS: This report describes a survey of four patients who developed Kikuchi's lymphadenitis occurring concomitantly with LES in one case and actinomycosis in another case. The definite diagnosis is usually made through histopathological examination of a lymph node biopsy. The evolution was favorable under corticosteroid therapy in two patients, antibiotics in the third and only antipyretic in the fourth. CONCLUSION: The authors bring report through these three observations, the diagnostic difficulties, the therapeutic means of the disease of Kikuchi, as well as its evolutionary aspects.

Association of stromal cell-derived factor-1-3'A polymorphism to higher mobilization of hematopoietic stem cells CD34+ in Tunisian population.

We explored the influence of polymorphisms in genes encoding the chemokine stromal cell-derived factor-1 (SDF-1)/CXCL12 in a cohort of Tunisian patients with malignant hematologic diseases multiple myeloma [MM], non-Hodgkin's lymphoma [NHL], Hodgkin's disease, and acute myeloid leukemia [AML], who underwent stem cell mobilization for autologous transplantation versus a group of healthy donors for allogeneic transplantation. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLp) analysis was used for rapid identification of genotypes. Significant associations for SDF1-3'A polymorphism were observed exclusively in patients with MM and NHL. While there was a lack of all association of SDF-1 polymorphism with AML patients. However, considering that the ability of mobilization varies among subjects, we have observed that the SDF1-3'A allele was associated with good mobilization capacity. Interestingly, the association was mainly observed among healthy allogeneic transplant donors where the analysis was not biased by background disease or chemotherapy (P=.010; odds ratio=2.603; confidence interval [95%]=1.239-5.466).

[Unusual presentation of Wegener's granulomatosis]. / Présentation inhabituelle d'une granulomatose de Wegener.

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved.

[Catastrophic antiphospholipid syndrome and rituximab: a new report]. / Syndrome catastrophique et anti-CD20: une nouvelle observation.

BACKGROUND: Catastrophic antiphospholipid syndrome is a distinctly rare dramatic condition characterized by widespread thrombosis of small vessels. Early diagnosis and aggressive therapies are essential in this condition because of its extremely high mortality rate. Therapeutic management include heparine, high dose steroids, cyclophosphamide, plasma exchange, intravenous immunoglobuline, however a number of patients are refractory to treatment. AIM: We review and discuss alternative and emerging treatment options by rituximab for patients who fail or cannot tolerate conventional therapy. CASE-REPORT: A 36-year-old female with a two mounths history of dyspnea, palpitation and chest pain was admitted. Physical examination upon admission revealed a fever, ischemic digital necrosis, scleroderma of the hands and beaking of the nose. Laboratory tests showed normal level of liver enzymes, elevation of creatinine level, lymphopenia, haemolytic anaemia with negative Coombs tests, low platelet count, prolonged partial thromboplastin time. The D-Dimer value was 158 ng/ml. Urinalysis revealed a proteinuria. Antinuclear antibody tests and lupus anticoagulant were strongly positive. Echocardiography revealed severe pulmonary hypertension and pericarditis. There was no pulmonary embolism on thoracic angio tomodensitometry. The diagnosis of catastrophic antiphospholipid antibody syndrome associated with systemic lupus and scleroderma was established. She was treated with anticoagulants, corticotherapy, one pulse of intravenous cyclophosphamide, 2 doses of intravenous immunoglobuline and 5 sessions of plasmapheresis. Because of lack of response 2 doses of 375 mg weekly rituximab i.v. were added but she developed pulmonary embolism, alveolar haemorrhage and she died. CONCLUSION: Effectiveness of Rituximab for the CSAPL should be demonstrated by further studies.

Systemic lupus erythematosus and multiple myeloma: an uncommon association. Two cases and literature review.

The association of systemic lupus erythematosus and multiple myeloma is uncommon. We report two cases of systemic lupus erythematosus associated to multiple myeloma. The cases are discussed in the light of a review of the literature. The clinical, laboratory and radiographic findings of the patients, as well as the subsequent therapeutic approach are discussed. A systematic review of all the other cases of this association is performed. We report two female patients of 50 and 35 years old who developed a multiple myeloma seven and three years respectively after the diagnosis of systemic lupus erythematosus. In the second case, systemic lupus erythematosus was associated to monoclonal gammopathy. One patient died after three months and one patient is still in remission after three years of the diagnosis of multiple myeloma. The coexistence of systemic lupus erythematosus and multiple myeloma is very rare and the possible pathogenetic mechanisms underlying this association remain unclear.

Contribution of spinal MRI for unsuspected cobalamin deficiency in isolated sub-acute combined degeneration.

Sub-acute combined degeneration (SCD) is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord and is a neurological complication due to vitamin B12 deficiency. Revealing forms of SCD without anemia are rare. We report a case of SCD of the spinal cord in a 33-year-old woman without anemia but with a 10-month history of paresthesis and urine imperiosity. Magnetic resonance imaging (MRI) of the spine showed intramedullary hyperintensity seen on T2-weighted images in the posterior column of the cervico-dorsal spinal cord, extending from C1 to D1. A diagnosis of SCD of the spinal cord was considered and confirmed by a low serum cobalamin. The patient was treated with vitamin B12 supplements and showed gradual improvement in her clinical symptoms.

[Sneddon's syndrome: 15 cases with cerebral angiography]. / Le syndrome de Sneddon: étude de 15 cas avec artériographie cérébrale.

Sneddon's syndrome is a rare disease defined by the presence of ischemic cerebrovascular events associated with livedo reticularis. We report a retrospective study of fifteen cases, thirteen women and two men, mean age of 37.93+/-9.77 years. All patients presented one or more cerebral infarcts. Six patients had dementia. Brain magnetic resonance imaging showed several cortical infarcts with white matter involvement. Cerebral angiography performed in all patients, showed a distal arteriopathy in twelve and thrombosis of the right carotid internal artery in one. One patient had antiphospholipid antibodies. Ten patients were treated with antiplatelet agents and five with anticoagulants. The course was favorable in eight patients and stationary in three. Four patients had several recurrent infarcts, one when anticoagulants were discontinued, one taking an anti-sludge-platelet agent and two who were not initially taking any treatment.

Fever revealing Behçet's disease: Two new cases.

Behçet's disease (BD) is an uncommon cause of fever of unknown origin. We report two cases, both involving 42-year-old males, who initially presented with prolonged fever and who were ultimately diagnosed as having BD after a delay of 12 and 21 months, respectively. Both patients developed pulmonary aneurysms. Although fevers resolved after therapy, both patients died within the first year after diagnosis. Clinicians should be aware that long-term fever may be an inaugural sign of BD, especially in individuals living in countries along the ancient Silk Road or Mediterranean basin.