[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.

[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heparin usefulness, not during the acute, hemorrhagic phase, but as a prophylactic treatment when hemorrhagic risk has been replaced by an inflammatory syndrome, with increased fibrinogen and platelets which are then prothrombotic factors.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.

[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(®).

[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.

[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.

[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.

[RACAND syndrome associated with primary biliary cirrhosis]. / Syndrome RACAND associé à une cirrhose biliaire primitive.

The acronym RACAND means the association of Raynaud's phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynaud's phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells.

[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.

[McArdle disease revealed by exercise intolerance associated with severe rhabdomyolysis]. / Révélation d'une maladie de Mc Ardle par une intolérance musculaire à l'effort associée à une rhabdomyolyse sévère.

McArdle's disease (MAD) is a rare hereditary myopathy secondary to a deficit in myophosphorylase, an essential enzyme for the use of muscular glycogen reserves. Exercise intolerance to a variable degree is the fundamental manifestation. Muscular enzymes are usually normal or slightly elevated, except during episodes of rhabdomyolysis. Generally, the electromyogram has poor sensitivity for the diagnosis of exercise myopathies. The muscular biopsy can be misleadingly normal. The role of MRI in the diagnosis of MAD is not well clarified in the literature. We report the case of a 16-year-old patient, hospitalized in July 2008 for exercise intolerance. On admission, he was asymptomatic and the physical examination was non contributive. Serum creatine kinase levels and renal function measures were normal. Cycloergometer exercise testing unmasked the disease. EMG and muscular biopsies were normal. During the second hospitalization, this time for rhabdomyolysis, T2 weighted MRI of the thighs showed high intensity signals from the gracilis muscles. The control MRI, made after 2 weeks of rest, was normal. Right gracilis muscle biopsy demonstrated excess glycogen with myophosphorylase deficiency, establishing the diagnosis of MAD. MAD is a rare metabolic myopathy to consider in patients with a history of exercise intolerance. The muscle biopsy can be misleadingly normal and should be, to our opinion, be guided by MRI findings.

[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.

[Three case-reports of viperin envenoming in Morocco]. / A propos de trois observations d'envenimations vipérines graves au Maroc.

Snake bites are responsible for a high mortality rate in Africa. Problems for the early care of the victims are many. We published three observations of Moroccan typical viperin severe envenomings (rapidly extensive edema, necrosis, haemorrhagic shock) are presented. The overall mortality of those bites is 4%. In the Maghreb, viperin syndromes are the result of the lebetin viper (Vipera lebetina), the horned viper or sand viper (Cerastes cerastes), sometimes Bitis or Echis sp. Immunotherapy remains effective against haemorrhage, even when administered late, in severe disease (bleeding). Death remains inevitable if antivenin is not being administered or if it is at the stage of multiorgan failure. Heparin is contra-indicated in the acute context, but at distance from the bite, the persistence of inflammatory syndrome can cause phlebitis or pulmonary embolism. Fresh frozen plasma and corticosteroids are ineffective and unnecessary. Signs of gravity are rapidly extensive swelling and systemic manifestations, particularly bleeding. The severity of poisoning is related to the difficulties of access to health centers, the use of traditional medicine for more than half of the victims, the lack of training of caregivers, finally and most importantly, the lack of antivenomous serum,that is not within reach of prescribing at the appropriate places and times. Despite its cost and allergic risk (become rare with the current serums), immunotherapy which is the only weapon effective against the venom, should be part of emergency essential drugs.

[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.