[Pulmonary embolism in the context of severe envenomation by a Moroccan viper]. / Embolie pulmonaire dans les suites d'une envenimation grave par une vipère marocaine.

Heparin, which was widely used thirty years ago for the treatment of viper envenomations, is now contra-indicated during the acute phase, which is at risk for hemorrhage and death. We report a case of pulmonary embolism, a rare situation in the context of viper envenomation. By means of this case report, we want to discuss the pathophysiological links between envenomation and thromboembolic disease, and on the other hand, the potential heparin usefulness, not during the acute, hemorrhagic phase, but as a prophylactic treatment when hemorrhagic risk has been replaced by an inflammatory syndrome, with increased fibrinogen and platelets which are then prothrombotic factors.

[Haemophagocytic syndrome as a complication of acute pancreatitis during systemic lupus erythematosus]. / Syndrome hémophagocytaire compliquant une pancréatite aiguë dans le cadre d'un lupus érythémateux systémique.

BACKGROUND: Acute pancreatitis and haemophagocytic syndrome (HS) are rarely seen in systemic lupus erythematosus (SLE). PATIENTS AND METHODS: We report the case of a young female patient without any noteworthy prior history, who was hospitalised for abdominal pain associated with acute pancreatitis possibly related to SLE with associated haemophagocytic syndrome. DISCUSSION: Screening for cutaneous symptoms of lupus in patients with pancreatitis can help avoid diagnostic errors.

[Incidence of malaria among United Nations troops deployed in the Ituri district of Democratic Republic of Congo (ex-Zaire) during a 12-month period spanning 2005 and 2006]. / Evaluation de l'importance du paludisme chez les militaires de l'ONU déployés sur le district de i'Ituri en ex-Zaïre pendant une période de 12 mois des années 2005-2006.

The purpose of this study was to determine the incidence of malaria in United Nations (UN) troops deployed in northeastern Democratic Republic of Congo (ex-Zaire). A one-year study was conducted from June 2005 to May 2006. The study was retrospective for the first six months and prospective for the second. During the study period, a total of 99 cases of malaria requiring hospitalization at the UN Level II Hospital in the town of Bunia, Democratic Republic of Congo were recorded among UN troops. Malaria accounted for approximately 36% of all hospitalizations for medical diseases. The offending species in 98% of cases was Plasmodium falciparum. Transmission was highest from April to September. There were no deaths. Parasitemia was less than or equal to 2% in 91% of cases.

[Efficacity of rituximab in hemolytic anemia with cold autoantibodies case]. / Efficacité exemplaire du rituximab dans un cas d'anémie hémolytique à autoanticorps froids.

INTRODUCTION: Autoimmune hemolytic anemia with cold autoantibodies or cold agglutinin disease is a rare chronic disorder in which no treatment has, until now, evidence of its effectiveness. CLINICAL CASE: We report a patient who successfully responded to rituximab for a cold agglutinin disease refractory to conventional therapy with very good tolerance and a complete remission. CONCLUSION: There are only few observations that have been reported in the literature regarding the efficacity of rituximab in the treatment of cold agglutinin disease. This promising therapy could, in the future, constitute a real alternative.

[Ulcerative colitis: exceptional consequence after rituximab therapy]. / La colite ulcérative : une conséquence exceptionnelle après traitement par rituximab.

INTRODUCTION: Possible adverse complications related to rituximab (RTX) are low, some of which are extremely rare. The authors describe one situation visibly waning exceptional treatment with RTX for SLE refractory to conventional therapies. COMMENT: The authors report a patient of 34 years followed for months for an illness in its bullous lupus, with cutaneous, articular, hematologic and immunologic. Given a corticosteroid resistance, several therapeutic background based hydroxychloroquine, cyclophosphamide and methotrexate, were initiated without any improvement. Immunomodulatory therapy type RTX was introduced to this form refractory at a rate of 375mg/m(2)/week. The waning of the second infusion, the patient presented a sudden intense abdominal pain syndrome, revealing an acute catarrhal appendicitis. At distance from the appendectomy, the consequences of which were favorable, treatment with RTX was resumed. In the aftermath of the third infusion, the patient presented in table tract marked by profuse watery diarrhea whose explorations reveal a morphological endoscopic appearance of erythematous, ulcerative colitis, reversible upon discontinuation of treatment. Histological data revealed important infiltrates composed mainly of CD8T lymphocytes. CONCLUSION: Gastrointestinal immunological consequences to the requirements of the targeted therapies deserved very careful and rigorous monitoring. However, at the slightest sign of digestive, a detailed morphological exploration is essential, to avoid in particular surgical emergency, evolution without treatment could engage in short-term vital prognosis.

[Durable efficacity and remission after treatment with imatinib mesylate for FIP1L1-PDGFRA transcript negative associated eosinophilic cardiomyopathy]. / Efficacité et rémission durables après traitement par imatinib mésylate pour une cardiomyopathie éosinophilique à transcrit FIP1L1-PDGFRA négatif.

INTRODUCTION: The cardiac involvement in hypereosinophilia remains a major cause of morbidity and mortality. Recent advances have identified new molecular mechanisms responsible for the expansion of the eosinophilic lineage, allowing a better classification of the different forms of Hypereosinophilic syndrome (HES) and especially targeted therapy. Since the discovery of the involvement of deregulated tyrosine kinases in the pathophysiology of these diseases, and particularly the identification of the fusion gene FIP1L1-PDGFRA, new molecules inhibiting specifically this signaling pathway (imatinib) were individualized, leading to dramatic therapeutic benefits in proliferative forms of HES considered before that of very poor prognosis. CASE REPORT: We report here the dramatic effectiveness of imatinib used as second line therapy for dilated cardiomyopathy revealing a hypereosinophilic syndrome in a patient in whom the search for FIP1-L1-PDGFRA fusion gene was negative. CONCLUSION: If hypereosinophilia has varied clinical and morphological outcome, its clinical consequences, particularly on heart function, are sometimes dreadful, and are not correlated either with blood eosinophil levels or with a specific etiology. We report here a case of HES lacking the FIP1-L1-PDGFRA fusion gene showing that despite the absence of this molecular defect, imatinib mesylate may have therapeutic interest in those cases of HES resistant to first line therapies.

[Pseudo-tumoral and ischemic encephalic Erdheim-Chester disease]. / Maladie d'Erdheim-Chester cérébrale avec double lésion pseudotumorale et ischémique.

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-langerhans cell histiocytosis of unknown etiology. It is a multi-systematic xanthogranulomatous infiltration with almost constant bone involvement; the neurological manifestations are not specific and occur in 15-20% of cases. METHODS: We report the case of a 59-year-old woman hospitalized for a frontal syndrome and right hemiparesis. RESULTS: Imaging revealed a left caudate nucleus process with recent infarct. Cardiovascular involvement and bilateral osteosclerosis of long bones strongly suggested ECD, confirmed after biopsies of the pericardium and bone. CONCLUSION: Pseudo-tumor encephalic ECD is very rare; the caudate nuclei is an unusual localization; ischemic stroke has been exceptionally described. Prognosis depends largely on the involvement of the central nervous and cardiovascular systems.

[Spontaneous spleen rupture in the course of malaria]. / Rupture spontanée de la rate au cours du paludisme.

The spontaneous rupture of malarial spleen is an uncommon complication. It usually occurs to a child and a recent expatriate. Its diagnosis is misleading into a context of infection. A 28-year-old European man, newly affected to Gabon, under chimioprophylactic drugs by chloroquine and proguanil, has been hospitalized in September 2000 because of an acute attack of malaria to Plasmodium falciparum. After four days treatment by quinine, he presented an abdominal pain with a Sub capsular Haematoma of the spleen confirmed by Scanner. The evolution was favourable under conservative treatment. The spontaneous rupture of malarial spleen is caused by Plasmodium vivax and falciparum. Twenty cases are reported in the literature. Conservative management permits to preserve the role of the spleen in immune response especially of the child and of people who regularly travel to endemic zone.

[Chronic calcifying pancreatitis revealing a primary hyperparathyroidism]. / Pancréatite chronique calcifiante révélant une hyperparathyroïdie primaire.

INTRODUCTION: The association of pancreatitis and hyperparathyroidism is rare. CASE RECORD: A 41-year-old man had consulted for a strong abdominal pain reliant to a chronic calcifying pancreatitis. A major hypercalcaemia led to a primary hyperparathyroidism diagnosis. The evolution was favourable after parathyroid adenoma surgery. DISCUSSION: The hypercalcaemia activate the transformation of trypsinogen into trypsin which is toxic for the pancreas. The role of parathyroid hormone remains unclear.

[Gleich syndrome. A case report and review of the literature]. / Le syndrome de Gleich. Un cas et revue de la littérature.

INTRODUCTION: The Gleich syndrome associates episodic angioedema, hypereosinophilia and elevation of immunoglobulin M. It's a rare cause of nonallergic angioedema and is characterised by no organ involvement. EXEGESIS: We report a case of a 27-years-old african women, with five years history of recurrent angioedema of face and extremities, associated with a major hypereosinophilia. Serum IgM elevation, elimination of other etiologies and spectacular response to corticoid treatment permitted to retain diagnosis. CONCLUSION: The majority of cases of Gleich syndrome were reported in USA, Europe and Japan. The nonepisodic angioedema, which is not accompanied by elevation of immunoglobulin M, was described in Japan. There are currently no case reported in Africa where parasites are the principal cause of hypereosinophilia. The immunohistochemical studies permit to explain cytochemical disturbances responsible for the release of disease whose initial mechanism is unknown.

[Is mild hyperhomocysteinemia an additional risk factor of the metabolic syndrome?]. / L'hyperhomocystéinémie est-elle un facteur de risque supplémentaire du syndrome métabolique?

INTRODUCTION: Metabolic syndrome is a cardiovascular risk factors cluster and hyperhomocysteinemia an obvious independant risk factor. OBJECTIVE: To ascertain if hyperhomocysteinemia is associated with the metabolic syndrome. METHODS: "Epimil" is a prospective epidemiologic survey, which began by a cross-sectionnal study of cardiovascular risk factors in a French population, which then will be followed for ten years for supervision and intervention. Initial data collection, blood pressure measurement, ECG and blood samples (biology and DNA) have been performed. For the metabolic syndrome, we used the criterias of the third report of the national cholesterol education program expert panel on detection, evaluation, and treatment of high blood cholesterol in adults (NCEP ATP III). RESULTS: Out of 2045 men aged from 20 to 58 years (37.7+/-8.7 years), 185 (9%) have metabolic syndrome (at least three criterias), 587 (29%) a blood homocysteine>or=12 micromol/L and 202 (10%)>or=15 micromol/L. Homocysteinemia is 10.97+/-5.01 micromol/L for the whole population and doesn't differ with (11.4+/-6 micromol/L) or without (10.9+/-5 micromol/L) the metabolic syndrome, as does its values distribution. It's not correlate with the body mass index, waist and hip measurements, nor with glycemia, HbA1c, insulin resistance and cardiovascular risk markers (CRPus, microalbuminuria). It weakly correlates with systolic and diastolic blood pressure, creatinine clearance, tobacco use, cholesterolemia, triglycerides and free fatty acids but not with HDL and LDL fractions, nor lipoprotein (a). Among this population, it slightly contributes to the ten years vascular risk evaluated according to Framingham equations or Score system. CONCLUSIONS: Homocysteinemia and the prevalence of hyperhomocysteinemia don't differ with or without the metabolic syndrome and doesn't correlate its main criterias.

A controlled study of sacroiliitis in Behçet's disease.

The aim of the study was to evaluate the prevalence of sacroiliitis in a group of patients with Behçet's disease (BD). Pelvic X-rays of 27 patients with BD responding to the International Study Group of BD and 30 controls (15 AS and 15 sciatica) were read blind and sacroiliac involvement was graded according to the New York criteria. In a second step, patients or controls with equivocal sacroiliitis had a sacroiliac CT scan. Two patients with BD (7.4%) and all patients with AS had evident bilateral sacroiliitis (at least grade 2). One patient with BD and two patients with sciatica had equivocal sacroiliitis (grade 1). CT confirmed sacroiliitis in the two patients with BD and eliminated inflammatory sacroiliitis in the three other patients with equivocal sacroiliitis showing mild degenerative lesions. A review of the literature showed that sacroiliitis and AS are rarely associated with BD. There remains insufficient evidence to suggest that sacroiliitis is an intrinsic feature of BD and that BD belongs to the group of SpA.

Idiopathic retroperitoneal fibrosis and ankylosing spondylitis. A new case report.

The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the HLA-B27 antigen. Prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases.

[Sneddon's syndrome: 4 cases and a review of the literature]. / Le syndrome de Sneddon: 4 cas et revue de la littérature.

Sneddon syndrome is an association of livedo racemosa and cerebrovascular ischemic events generally occurring in young adults. This is an uncommon chronic progressive arterio-occlusive disorder of unknown cause involving small and medium sized vessels. We report four cases. One case was disclosed by cerebral hemorrhage. One pathogenic hypothesis suggests the involvement of an idiopathic progressive inflammatory arteriopathy or secondary thrombotic disorder comparable with antiphospholipid syndrome.

[McDuffie hypocomplementemic urticarial vasculitis. Two cases and review of the literature]. / Vascularite urticarienne hypocomplémentémique de McDuffie. Deux observations et revue de la littérature.

INTRODUCTION: Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We report two new cases. EXEGESIS: Case 1: a 41-year-old woman presented in 1994 with inflammatory polyarthralgia, diffuse urticaria, fever, and weight loss. Biology showed proteinuria, positive rheumatoid factor with hypocomplementemia and negative immunological tests. Skin and renal biopsies showed leukocytoclastic vasculitis and extramembranous glomerulopathy, respectively. Outcome within steroid therapy was marked by alternating clinical improvement and relapses. Case 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis, diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays and CT scan showed interstitial fibrosis and echocardiography revealed pericarditis. Biology showed positive rheumatoid factor with hypocomplementemia and negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vasculitis. Corticosteroids and cyclophosphamide improved the patient's condition. McDuffie HUV is a disease with varied systemic manifestations. Its existence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids. CONCLUSION: McDuffie HUV is a defensible entity among urticarial vasculitis because of its particular clinical and biological features.

[Tropical pyomyositis simulating septic arthritis in AIDS patients. Two cases]. / Les myosites tropicales simulant une arthrite septique chez les patients vivant avec le VIH (deux observations).

Tropical pyomyositis (TP) is an acute bacterial infection of skeletal muscles characterized by rapid formation of abscesses. Since the beginning of the AIDS epidemic, interest in TP has increased because of its rising incidence in association with HIV infection and of the problems that it poses for differential diagnosis. Occurrence of TP is a criterion for classification of HIV infected patients in WHO disease stage III. The purpose of this report is to describe two HIV-infected patients who presented TP simulating septic arthritis of the hip and knee respectively. Medical imaging was particularly useful in establishing accurate topographic diagnosis and needle drainage in decreasing the duration of hospitalization and avoiding the need for surgical debridement. Needle puncture must be guided by ultrasound or scan imaging.

[Ischaemic stroke secondary to viper envenomation in Morocco in the absence of adequate antivenom]. / Accident vasculaire cérébral ischémique à la suite d'une envenimation vipérine grave au Maroc, traitée par un antivenin inadapté

An ischaemic stroke is a rare complication of viper envenomation that is due to multifactorial pathophysiological mechanisms. The authors describe the case of a 55-year-old patient bitten by the viper Cerastes cerastes. The patient was admitted to the intensive care unit with multiple organ failure, disseminated intravascular coagulopathy, rhabdomyolysis, anuria and elevated troponin level. The persistent disturbance of consciousness has motivated a brain scan which has revealed a bifocal ischemic stroke. The complex venom of the species C. cerastes may induce hypotension, tissue necrosis, acute renal failure, bleeding disorders or DIC. With the cessation of a non-indicated heparintherapy and haemodialysis, the patient recovered in a few weeks despite the initial infusion of an unsuitable antivenom due to the late identification of the reptile. The preventive treatment of the complications of this envenomation is based on the infusion of the polyvalent antivenom Favirept(®).

[Ocular involvement in polyarteritis nodosa: two cases]. / Atteinte oculaire au cours de la périartérite noueuse. Deux observations.

INTRODUCTION: Polyarteritis nodosa (PAN) is a systemic vasculitis with polymorphic manifestations. Ocular involvement is rare; we report two such cases. CASES: The first case was a 56-year-old man with PAN treated with corticosteroids. A year and a half later, during a disease outbreak associated with decreased visual acuity, ocular examination objectified a localized pigment epithelial detachment in the right eye. The outcome was favorable with corticoid bolus followed by azathioprine. The second case was a 41-year-old man presenting with PAN and decreased visual acuity. Ophthalmologic examination showed inferotemporal branch vein occlusion in the right eye with serous retinal detachments in the left eye. The outcome was favorable with intravenous cyclophosphamide followed by azathioprine. DISCUSSION: The incidence of ocular involvement in PAN varies from 10 to 20%. It is most often associated with vasculitis of retinal and choroidal arteries. Fluorescein angiography is the gold standard of diagnosis. Multifocal acute ischemia of the choriocapillaris is very pathognomonic. Immunosuppressant drugs are effective and should be reserved for severe forms.

[Septic pylephlebitis associated with Enterobacter cloacae septicemia]. / Pyléphlébite septique associée à une septicémie à Enterobacter cloacae.

Septic pylephlebitis or purulent thrombosis of the portal venous system generally results from a progressive extension of suppurated thrombophlebitis, secondary to an intrabdominal infection. Germs most often found are Escherichia coli and Streptococcus, isolation of Enterobacter cloacae is unusual. We report a particular observation of septic pylephlebitis associated with E. cloacae bacteremia, without biliary, digestive or pancreatic lesion on the CT-scan. The antibiotic sensitivity pattern of the isolated germ and the negative epidemiologic investigation pled in favour of community acquired infection. The infection resolved with antibiotics and anticoagulation, followed by total repermeation of the portal system.