[Pulmonary alveolar microlithiasis: A report on two familial cases in Morocco]. / Microlithiase alvéolaire pulmonaire : à propos de deux cas familiaux au Maroc.

INTRODUCTION: Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive disease. The majority of patients are asymptomatic. The disease is often diagnosed on routine radiological examination. CASE REPORTS: We report two familial cases of PAM. A 17-year-old girl with a chest X-ray showing an alveolar syndrome, especially on the right side, a bronchointerstitial syndrome, and diffuse calcifications. The thoracic CT scan showed calcified micro- and macronodules with pleural and pericardial calcifications. Respiratory function tests showed restrictive syndrome and normal blood gas values suggestive if PAM, which was confirmed by the presence of microliths in bronchoalveolar lavage (BAL). Family investigation led to chest radiograph of a 14-year-old sister who was asymptomatic but presented with an aspect of "sandstorm" calcifications. CONCLUSION: PAM is known to be radio-clinically dissociative. In typical cases, radiology can suggest the diagnosis, which is often confirmed by SLC34A2 mutation or microliths in BAL or sputum. The prognosis is compromised in the long-term. The only effective treatment nowadays is lung transplantation.

Détermination des concentrations plasmatiques de l'isoniazide et la rifampicine chez les patients sous antituberculeux

Introduction : La tuberculose est un problème de santé publique dans le monde entier. Son traitement est basé sur quatre antituberculeux majeurs dont l'isoniazide et la rifampicine qui ont bouleversé le pronostic de cette maladie. L'objectif de cette étude était de montrer l'apport du suivi thérapeutique pharmacologique dans la prévention des échecs thérapeutiques en cas de sous-dosage et de toxicité en cas de surdosage.Matériels et méthodes : Il s'agit d'une étude rétrospective faite à l'Hôpital Moulay Youssef et le laboratoire de toxicologie et de pharmacologie du centre anti-poison et de pharmacovigilance du Maroc sur une période de 9 mois, et menée sur 142 patients ayant tous bénéficié d'un suivi thérapeutique pharmacologique de l'isoniazide et la rifampicine. Les dosages étaient réalisés par chromatographie liquide haute performance après extraction liquide-liquide. Résultats : Notre étude a montré qu'à dose thérapeutique, 78,2% des patients tuberculeux avaient des concentrations plasmatiques de rifampicine au-dessous de la fourchette thérapeutique et 35,4% des patients avaient des concentrations plasmatiques d'isoniazide au-dessus de la fourchette thérapeutique.Conclusion : Dans le cadre du traitement antituberculeux, le recours au suivi thérapeutique pharmacologique permet aux cliniciens

Refeeding syndrome: a forgotten and potentially lethal entity.

Refeeding syndrome (RS) is defined as the fluid, electrolyte, metabolic, and clinical disturbances that occur after nutrition of patients who have been undernourished or fasting for a prolonged period. This syndrome has been recognized for several decades but is most often overlooked and underdiagnosed by health professionals. The authors report a RS in a patient subjected to prolonged fasting after being kidnapped by a criminal gang. Refeeding resulted in severe hypophosphatemia, neurological impairments, and hematologic disorders. The patient died due to septic shock with multiple organ failure secondary to aspiration pneumonia. In this case, the failure to recognize RS was decisive in the fatal outcome. Professionals must be aware of this disease to prevent and treat it properly.

[Association of ischemic and hemorrhagic cerebral stroke due to severe envenomation by the Sahara horned viper (Cerastes cerastes)]. / Association de lésions cérébrales thrombotiques et hémorragiques lors d'une envenimation grave par la vipère à cornes du Sahara (Cerastes cerastes).

Viper envenomation can cause complex disorders of hemostasis. These disorders usually lead to hemorrhagic syndrome. The occurrence of thrombosis is exceptional. This case report describes a severe envenomation by the Saharan horned viper (Cerastes cerastes), complicated by unusual brain injuries: hemorrhagic and ischemic stroke. The physiopathologic link between this serious toxic coagulopathy and the multitude of toxins in the venom of Cerastes cerastes is highlighted.

[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.

[Cerebral metastasis of a "ghost" bronchopulmonary tumor]. / Métastase cérébrale d'une tumeur bronchopulmonaire "fantôme".

Carcinoma of unknown primary (CUP) is the metastasis of epithelial malignancy whose origin remains unknown. Bronchial carcinoma is the most common cause (45%), however, in more than a quarter of cases, its origin is unknown and the choice of therapy often justifies a histopathological precision brought by the immuno-histochemistry. We present a rare case of cerebral metastasis, revealing an unknown lung cancer after a comprehensive review. This is a patient aged 53 years, chronic smoker, who presented a year and a half ago a sudden loss of consciousness with afebrile tonic-clonic seizure of spontaneous resolution. Clinical examination was unremarkable. Brain imaging by MRI has objectified the presence of two parietal tissue formations, which stereotactic biopsy with histological and immuno-histochemical favored metastasis of lung adenocarcinoma origin. An etiologic in search of the primitive lung remained negative (chest X-ray of face, chest CT and bronchoscopy with sampling upper gastrointestinal endoscopy). The rest of the etiologic, looking for another primitive, remained negative. The diagnosis of a bronchogenic carcinoma with brain metastasis is therefore withheld. The patient received two brain radiotherapies (gamma kniff) with regression of the two nodular formations. We have not given specific treatment and have called for very close monitoring of the patient who remained stable after 18 months. Through this observation, we stress the interest of the histology and immuno-histochemistry of carcinoma of unknown primary (CUP) to the diagnosis, guide therapy and determine prognosis.

[Multiple sclerosis associated with antiphospholipid syndrome: diagnostic and therapeutic difficulties]. / Sclérose en plaques associée à un syndrome des antiphospholipides : difficultés diagnostiques et thérapeutiques.

Strokes are the main neurological manifestation of antiphospholipid syndrome. Other clinical presentations are possible and may mimic classic symptoms of multiple sclerosis (MS). A 46-year-old woman, with a history of two miscarriages, presented four subacute neurological episodes (optic neuritis, right facial paralysis, paraparesis of the thigh, and right brachial monoparesis). Using McDonald criteria, the diagnosis of multiple sclerosis was retained. Because of the occurrence of thrombocytopenia during a final relapse, we reconsidered the diagnosis of MS. Search for antiphospholipid antibodies was positive. All clinical manifestations and complementary tests were compatible with the diagnosis of antiphospholipid syndrome associated with multiple sclerosis. Given the great similarity of clinical, radiological and biological findings in the two diseases, non-thrombotic neurological manifestations of antiphospholipid syndrome can be difficult to distinguish from MS associated with antiphospholipid syndrome.

[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.

[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.

[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.