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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.

The molecular basis of genetic predisposition to pulmonary tuberculosis in adults remains largely elusive. Few candidate genes have consistently been implicated in tuberculosis susceptibility, and no conclusive linkage was found in two previous genome-wide screens. We report here a genome-wide linkage study in a total sample of 96 Moroccan multiplex families, including 227 siblings with microbiologically and radiologically proven pulmonary tuberculosis. A genome-wide scan conducted in half the sample (48 families) identified five regions providing suggestive evidence (logarithm of the odds [LOD] score >1.17; P < 0.01) for linkage. These regions were then fine-mapped in the total sample of 96 families. A single region of chromosome 8q12-q13 was significantly linked to tuberculosis (LOD score = 3.49; P = 3 x 10(-5)), indicating the presence of a major tuberculosis susceptibility gene. Linkage was stronger (LOD score = 3.94; P = 10(-5)) in the subsample of 39 families in which one parent was also affected by tuberculosis, whereas it was much lower (LOD score = 0.79) in the 57 remaining families without affected parents, supporting a dominant mode of inheritance of the major susceptibility locus. These results provide direct molecular evidence that human pulmonary tuberculosis has a strong genetic basis, and indicate that the genetic component involves at least one major locus with a dominant susceptibility allele.

The aim of this study was to identify the spectrum of abnormalities revealed on high-resolution computerized tomography (HRCT) in patients with ankylosing spondylitis (AS), to compare findings with those of plain radiography and pulmonary function testing (PFT), and to look for correlations between lung involvement and AS severity. We prospectively studied 55 consecutive patients with a diagnosis of AS according to the modified New York criteria who attended our department over a period of 2 years. All patients had a detailed rheumatological examination and underwent plain chest radiography, chest HRCT and PFT. HRCT revealed abnormalities in 29 patients (52.7%), whereas plain chest radiography was abnormal in only 2. Abnormalities consisted of interstitial lung disease (ILD) ( n=4), apical fibrosis ( n=5), emphysema ( n=5), bronchiectasis ( n=4), ground glass attenuation ( n=2), and non-specific interstitial abnormalities ( n=26). Only apical fibrosis and bronchiectasis were statistically more frequent with increasing disease duration (significant trend chi(2)test, p=0.0029 and 0.028, respectively). PFT showed a restrictive process in 19 patients (34.5%). No correlation was noted between HRCT and PFT, nor with AS symptomatic and structural severity parameters. However, there was a statistically significant correlation between PFT and AS symptomatic and structural severity parameters. In conclusion,: this study confirms that the chest HRCT of patients with AS showed a great number of abnormalities undetectable by standard X-rays. The high incidence of lung abnormalities emphasizes the importance of excluding such a diagnosis in patients with AS even without respiratory symptoms.

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.

Introduction: The authors report four cases of non Hodgkin's lymphomas (NHL) among patients infected by the hepatitis C virus (HCV). They discuss the epidemiological, physiopathological and clinical features of this association., Current Knowledge and Key Points: The role of the HCV in the development of B-cell NHL is probable but the reported frequency of the association with HCV infection is variable especially in different countries. Most of the reported cases are low-grade NHL with frequent extranodal involvement., Future Prospects and Projects: New studies will improve the understanding of the physiopathological mechanisms that might explain the occurrence of NHL in the course of HCV infection. The recent evidence that the antiviral treatment is effective in splenic lymphoma with villous lymphocytes leads to reconsider the relationship between HCV and NHL and to think about new therapeutic possibilities.

Purpose: Gastrointestinal bleeding, an uncommon complication of polycythemia can be the inaugural sign in exceptional cases., Case Report: A 35-year-old patient was hospitalized for upper gastrointestinal bleeding. Physical examination and laboratory tests led to the diagnosis of polycythemia (Vaquez disease). Gastroscopy showed an hemorrhagic bulber ulcer. Outcome was favorable after chemotherapy and antinuclear treatment., Conclusion: Based on this observation and data in the literature we propose that this unusual presentation would suggest a relationship between polycythemia and ulcer disease.

Non-Hodgkin's malignant lymphoma is rarely located in the soft tissue of the chin making diagnosis difficult. Clinical signs are non-specific: progressive weight loss, asthenia, fever, an inflammatory tumefaction unresponsive to medical treatment. Imaging shows a homogeneous tumefaction of the soft tissues. Pathology and immunohistochemistry establish the diagnosis.

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.

Unlabelled: Ankylosing spondylitis is a very uncommon finding in patients with sarcoidosis. Thirteen cases have been reported in the literature. We report a new case. Observation. - A 40-year-old man had inflammatory low back pain since 1983 which formerly responded to nonsteroidal inflammatory drugs (NSAIDs). He developed dyspnea and skin rash in 1993. Physical exam found cervical and lumbar spine stiffness and violaceous and circular lesions on the forehead, nose and right cheek. Laboratory tests showed: ESR at 50 mm, increased serum angiotensin-1-converting enzyme, and negative HLA B27. X-rays showed sacroiliac ankylosis, cervical and lumbar syndesmophytes and cervical facet joint ankylosis. The plain chest x-ray showed an interstitial syndrome. Chest CT scan showed mediastinal adenopathies. Skin biopsy disclosed non-caseating epitheliod and giant-cell granuloma. Outcome was good with steroid therapy but back pain was only improved by NSAIDs., Discussion: - This association raises a diagnostical problem because spine involvement in sarcoidosis can mimic ankylosing spondylitis. It also suggests the hypothesis of a pathophysiological link between the two diseases.

The authors report the results of prospective study, which compared 75 sports subjects and 45 witnesses. All subjects were male, the differentiating parameter being the type of sport practiced. This work analysed surface Electrocardiogramme, thransthoracic echocardiography and high amplification ECG. The statistical study used the student test t compare means and the Chi2 test for the percentages, the signification limit was fixed to 5%. Clinically, our two series didn't show a significant statistical difference, concerning: age, weight, height or arterial pressure. On the electrocardiographical level, the sport's men have a lower cardiac frequency (p = 0.005), a larger PR space (p = 0.05), an important Sokolow parameter (p < 0.005), and repolarisation disorders represented, essentially by negative T waves (p = 0.02) and an upper movement of ST segment in V2-V3 (p < 0.005). Echocardiography showed a dilatation of the right cavities: right auricular (p = 0.0125) and right ventricular (p = 0.025). Move over, it has been showed that the sport's men left ventricular walls were tabor (septal wall, p = 0.0125), (posterior wall, p = 0.025), despite a difference in the values of the left ventricular telediastolic diameter (4 mm average in the two series). The signification limit was not reached and it was also showed that the left auricular was also dilated (p = 0.025). The study of the delayed Potentials, tried to bring an explication to certain sudden deaths of sport's men which are to date unexplained and which could have a rhythmical origin? It is also to be noted that sport's men present more delayed ventricular Potentials. However, the statistical signification was not reached (p = 0.07).

Introduction: Malacoplakia, a chronic granulomatous disease, rarely involves the digestive tube and, when it does, takes on a pseudotumoral aspect., Observation: A 37 year-old man was hospitalized for chronic diarrhea that had progressed over 15 years. He exhibited an edematous-ascitic syndrome and bilateral pleurisy together with, biologically, a malabsorption syndrome. The endoscopic examinations (fibroscopy and colonoscopy) revealed polypoid tumor-like formations. An image of tumoral stenosis of the sigmoid-colic junction was revealed on barite lavage. Histological examination of the surgical sample (wide left colectomy of one third of the transverse) was suggestive of malacoplakia (histiocytes with characteristic Michaelis-Gutmann bodies)., Discussion: Malacoplakia is an inflammatory disease predominantly affecting the urogenital tract. Other organs can be involved. Its clinical symptomatology is highly disparate. The interest of this disease is in its pathogenesis, on which, in fact, its treatment is based.

Introduction: The main issue in venous thrombotic events is their etiology. Several clinical and/or biological data can be helpful in that research. In the two cases we report here, a macrocytic anemia led to the diagnosis of probably acquired hyperhomocysteinemia., First Case: a 24-year-old man was admitted for severe anemia and a superior vena cava syndrome. Biological data showed megaloblastic anemia and mild features of blood destruction that were explained by a pernicious anemia. Second case: a 35-year-old man had two deep venous thrombotic events in one year (involving the right leg, then the left leg); biological findings showed a macrocytic mild anemia that was diagnosed as a pernicious anemia. In both of the patients, deep venous thrombosis was mainly explained by a hyperhomocysteinemia that was a consequence of vitamin B12 deficiency. The two patients improved under anticoagulant treatment combined with subcutaneous vitamin B12., Conclusion: Pernicious anemia can cause acquired hyperhomocysteinemia, which is considered a risk factor for deep venous thrombosis. Thus, the connectivity of these conditions should remain in the practitioner's mind, especially when thrombosis occurs along with a macrocytic anemia.

Real progresses have been made during the past years in the comprehension of hemostasis mechanisms, along with rising of new antithrombotic drugs. The later include: 1) direct inhibitors of thrombin such as hirudin, bivalirudin, argatroban, melagatran and ximelagatran; 2) inhibitors of factor Xa such as the synthetic pentasaccharid and DX-9065a; 3) inhibitors of factor IXa; 4) inhibitors of tissue factor-factor VIIa complex such as tissue factor pathway inhibitor (TFPI) or NAPc2 (nematode anticoagulant peptide); 5) drugs enhancing endogenous anticoagulant activity, such as protein C or activated protein C; 6) drugs modulating endogenous fibrinolytic activity. These new drugs are promising a real decrease in mortality and morbidity due to venous thrombo-embolic disease, which is considered as a public health issue. Both physicians and biologists are concerned by these new antithrombotic agents, the former to think about new treatment strategies, the later to monitore, if necessary, the effects of such new drugs. Our review does not include antiplatelet agents which are indicated only in arterial thrombosis.

Endomyocardial fibrosis is very rare in Behcet's disease. The authors report the case of a 28 year old patient with Behcet's disease complicated by a pseudo-tumoral right ventricular formation on echocardiography. This misleading appearance suggested the diagnosis of cardiac thrombus or tumour and led to a surgical approach which revealed a fibrous moderator band suggesting endomyocardial fibrosis, confirmed by antomopathological analysis. Besides the originality of this case and the unusual pseudo-tumoral presentation, the authors underline the difficulties of establishing the diagnosis, despite the advances of medical imaging. The pseudo-tumoral intra-cardiac lesion in a suggestive clinical context (Behcet's disease) should raise suspicion of the diagnosis of endomyocardial fibrosis.

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.

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.

Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.

The authors report on 30 cases of cardiothyrotoxicosis in the young adult with Basedow's disease. Cardiac arrhythmia represented the most frequent clinical form of dysfunction, mainly atrial fibrillation. Conductive disorders came second, with seven cases of first-degree atrioventricular block [AVB], one case of second-degree AVB, and two cases of sinusoidal bradycardia. Three cases of ballooning of the mitral valve were detected by echocardiography. Myocardial hypertrophy was found in one case. The authors discuss the various physiopathological hypotheses regarding conduction and myocardial hypertrophy anomalies. No cases of severe cardiac insufficiency or coronaropathy were noted, which is explained by the absence of cardiopathic antecedents and the young age of the patient population. Treatment is more complicated in the case of a preexisting cardiac event.

Thirty-four observations of papillary fibroelastoma (PFE) of the mitral valve have to date been reported in the literature. Our findings are the tenth so far documented on PFE revealed by a cerebral ischaemic vascular accident. The echocardiographic investigation detected a pedunculated tumour of the mitral valve 9 mm in diameter, and associated with moderate mitral leakage. The tumour was surgically excised tumour confirmed the original PFE diagnosis. The evolution seven years after surgery is favourable, with no signs of tumour recurrence and good functioning of the mitral valve system.

Objectives: We prospectively studied 783 consecutive Moroccan patients to define: 1) the prevalence of anti-hepatitis C virus (HCV) antibody (Ab), 2) the prevalence of other viral infections: HBs Ag, anti-HAV IgM, anti-HGV, HGV RNA, 3) the risk factors of spreading HCV infection, and 4) the distribution of HCV genotypes., Results: 60/783 (7.7%) patients had anti-HCV Ab (48 H/12 F), 26 (3.3%) HBs Ag, and 3 (0.3%) IgM anti-HAV. Anti-HGV Ab was found in 11/60 (18.3%) anti-HCV positive patients, and 6/38 (15.8%) anti-HCV negative patients. 2/22 (9%) serum anti-HCV positive and anti-HGV negative patients were positive for HGV RNA. The 60 HCV positive patients rarely had other viral infections: 3 (5%) HBs Ag, 11 (18.3%) anti-HGV positive, 2 (9%) HGV RNA positive, and none had anti-HBc, IgM anti-HAV, or anti-HIV. HCV positive patients had more often undergone transfusion of blood products (21.7 vs 5.5%; P < 0.0001), and dental treatment (55% vs 8.3%; p < 0.0001). Patients with anti-HCV Ab frequently had hepatitis lesions on liver biopsy, i.e. chronic active hepatitis (n = 44) or cirrhosis (n = 16). HCV RNA was positive in 45/60 (75%) anti-HCV positive patients. HCV genotypes were: 1b (n = 21, 47%), 2a/2c (n = 13, 29%), 1a (n = 6, 13%), et 3 (n = 1, 2%)., Conclusions: In our Moroccan population, the prevalence of HCV was high (7.7%). Other viral infections (HBV, HAV, HGV) were rare.

The authors report the case of an acquired left ventricle-right atrial communication after open chest trauma. The initial clinical presentation was a haemothorax and haemopericardium responding well to emergency surgical drainage. Secondarily, a systolic murmur suggesting a ventricular septal defect and signs of right heart failure were observed. Colour Doppler echocardiography led to the diagnosis of a left ventricle-right atrial communication associated with tricuspid regurgitation with dilatation of the right heart chambers and pulmonary hypertension. At surgery, a defect in the membranous interventricular septum was confirmed with rupture of the septal tricuspid leaflet causing tricuspid regurgitation. The surgeon closed the defect with a patch and performed a De Vega tricuspid valvuloplasty. The postoperative outcome was uneventful.

Unlabelled: INTRODUCTION AND EXEGESIS: The authors report a case of sinus node dysfunction that occurred in a 22-year-old patient with Graves' disease and disappeared after thyroidectomy., Conclusion: Bradycardia is uncommon in hyperthyroidism, the sinus node dysfunction would be due to thyrotoxicity-related myocarditis.

At present, Morocco is looking into ways and means for the setting up of a health system liable to satisfy the requirements of the end of this century while taking into account the socio-economic constraints. The current health system comprises three sectors: a public sector consisting of the structures of the ministry of public Health together with those of the Health services of the Royal Armed Forces, a semipublic sector, and a private sector. The health system has been undeniably instrumental in the improvement of the health standards in independent Morocco. One figure is evidence enough of that: mortality rate dropped from 160% in 1956 to 57.4% in 1993. This score, however, does not make up for the inadequacies of the system: insufficient human and material means, uneven geographic distribution of health cover and a very limited health insurance. In order to remedy this situation, a health system development program is to be elaborated in the next few months. With the aim of improving the quality and the geographic expansion of the health cover, this program rests upon a number of fundamental principles: progressively generalized compulsory health insurance, improved health cover in the rural area, and a greater commitment to health matters on the part of local elected councils. The financing of the program will rely on health insurance revenues and the budget of the public Health ministry. The latter item will be devoted to the development of health infrastructures and, prior to generalized compulsory health insurance, to the financing of health-care for the poorest.

The authors report a new observation of Köhler's disease. Since Köhler's description in 1975, 40 observations have been noted. It concerns a particular entity characterized by a hyperostosis usually bilateral sternocosto clavicular able to associate other osseous lesions and cutaneous eruptions. Its etiology remains unknown.

The authors report the case of a 26-year old male patient who had Graves' disease with a first degree atrioventricular block (AVB) and intermittent episodes of a second degree AVB of the Lucciani-Wenckebach type. These disorders of conduction had features characteristic of a nodal block and disappeared after treatment of the hyperthyroidism. The pathogenesis of atrioventricular conduction disorders in hyperthyroidism remains controverted. The authors put forward the following hypothesis: under the influence of thyroid hormones in excessive amounts, the autonomic nervous system would act by reciprocal excitation and exacerbate a patent or latent hypervagotonia which was pre-existent to the hyperthyroidism. This hypothesis needs to be tested by intracardiac electrophysiological studies with atrial stimulation.