Ann Med Interne (Paris)
· 2002 Sep · PMID 12442081
First described in 1948, Bernard-Soulier syndrome is an uncommon hereditary thrombopathy characterized by abnormal expression of the GPIb-IX-V complex which inhibits platelet migration to the site of endothelial trauma....First described in 1948, Bernard-Soulier syndrome is an uncommon hereditary thrombopathy characterized by abnormal expression of the GPIb-IX-V complex which inhibits platelet migration to the site of endothelial trauma. Our case illustrates the pathophysiological mechanisms involved and points out the similarity with idiopathic thrombopenic purpura.
Lévêque C, Sabbah P, Helie O
… +3 more, Minvielle F, Dion AM, Cordoliani YS
Ann Med Interne (Paris)
· 2002 Sep · PMID 12442080
The authors describe the radiological pattern of meningitis neurological complications. These complications are vascular lesions, arteritis and thrombophlebitis, brain swelling, hydro-cephalus, extra-cerebral collections...The authors describe the radiological pattern of meningitis neurological complications. These complications are vascular lesions, arteritis and thrombophlebitis, brain swelling, hydro-cephalus, extra-cerebral collections, neuritis and labyrinthitis. When meningitis complication is suspected, MRI must be the first neuroradiological investigation.
Neuville S, Dromer F, Chrétien F
… +2 more, Gray F, Lortholary O
Ann Med Interne (Paris)
· 2002 Sep · PMID 12442078
Cryptococcus neoformans is an encapsulated yeast mainly responsible for meningoencephalitis, especially in AIDS patients. Recent observations using an experimental model of systemic cryptococcosis that mimics the human i...Cryptococcus neoformans is an encapsulated yeast mainly responsible for meningoencephalitis, especially in AIDS patients. Recent observations using an experimental model of systemic cryptococcosis that mimics the human infection have reinforced the knowledge on the pathogenesis of cryptococcosis. Cryptococcosis may occur several years after inhalation of infecting particles from the environment. A stage of fungemia that reflects the dissemination of infection usually precedes the development of meningoencephalitis. The capsule mainly composed of glucuronoxylomannan constitutes the main virulence factor of C. neoformans. It has several deleterious effects including the inhibition of the host immune responses. The central nervous system involvement differs between AIDS patients and HIV-negative patients. In AIDS patients, histological studies of the brain show numerous cryptococci without significant inflammatory cell response. In other immunodepressed hosts, a granulomatous inflammation containing few yeasts is usually seen. This may reflect an altered local immunological defect against C. neoformans in AIDS patients with cryptococcosis.
Perrocheau A, De Benoist AC, Six C
… +3 more, Goulet V, Decludt B, Levy-Bruhl D
Ann Med Interne (Paris)
· 2002 Sep · PMID 12442076
In France, two sources of data, the mandatory notification and the laboratory network EPIBAC, allow the health authorities to follow the incidence of bacterial meningitis (BM) and to assess the relative frequency of the...In France, two sources of data, the mandatory notification and the laboratory network EPIBAC, allow the health authorities to follow the incidence of bacterial meningitis (BM) and to assess the relative frequency of the micro-organisms responsible for such infection. In 1999, more than 1,000 cases of BM were notified in France. The more common micro-organisms were: Streptococcus pneumoniae 46%, Neisseria meningitidis 32% and Streptococcus agalactiae(or Streptococcus B) 11%. Listeria monocytogenes and Haemophilus influenzae accounted for 6% and 5% of the cases respectively. In 1999, the incidence per 100,000 inhabitants of meningitis due to pneumococci (0.81), to streptococci B (0.19) and tuberculosis meningitis (0.17) were stable since 1995. The incidence rate of meningitis due to Listeria (0.10) and to H. influenzae (0.08) shows a regular decrease since 1992. The impact of preventive measures of meningitis due to Listeria and H. influenzae B has been clearly demonstrated through the dramatic decrease of meningitis due to these micro-organisms.
Ann Med Interne (Paris)
· 2002 Sep · PMID 12442075
Botulism is still found in France. Botulism is life-threatening and may have epidemic occurrences, which justifies an epidemiological watch with systematic notification of cases. In order to complete these recent epidemi...Botulism is still found in France. Botulism is life-threatening and may have epidemic occurrences, which justifies an epidemiological watch with systematic notification of cases. In order to complete these recent epidemiological data, older cases since 1940 have been reviewed through a survey sent to the French Ministry of Health and the Pasteur Institute (National Reference Center). A Medline search has also been completed. In France, foodborne botulism is the main source of contamination. Type B is nearly always involved but type E botulism has been regularly reported. Fatal outcome due to botulism-induced respiratory muscle weakness can now be avoided by mechanical ventilation, leading to better prognosis. Microbiological diagnosis has been confirmed in two-thirds of the cases by isolation of toxin in the patient's serum. It is important to identify the foodstuff incriminated to prevent the development of a botulism outbreak. Foodstuffs responsible for foodborne botulism are the same since 1940. However, the increased consumption of vacuum-packed foodstuffs might facilitate the persistence of botulism. In conclusion, this review points out the significance of epidemiological screening of foodstuffs in the alimentary chain as well as the systematic notification of botulism. The regular educational information for young physicians regarding botulism should be continued.
Bligny D, Cador B, Jolivet-Gougeon A
… +6 more, Le Strat A, Cazalets C, Laurat E, Jego P, Bouget J, Grosbois B
Ann Med Interne (Paris)
· 2002 Sep · PMID 12442074
OBJECTIVES AND METHODS: A retrospective study of 45 patients with Clostridium difficile infection over a 4-year period in a department of Internal Medicine. RESULTS: Mean age was 79 years; sex-ratio (F/M)=1.5; 38% of the...OBJECTIVES AND METHODS: A retrospective study of 45 patients with Clostridium difficile infection over a 4-year period in a department of Internal Medicine. RESULTS: Mean age was 79 years; sex-ratio (F/M)=1.5; 38% of the patients had neurological or severe psychiatric disorders; 20% had a neoplastic disease. Ninety-three percent of cases had received one or more antibiotics before onset of diarrhea, prescribed mainly for a pulmonary infection. Amoxicillin clavulanic acid and cephalosporins were the most frequently used treatments, respectively in 48% and 40% of cases. For 25 patients (56%) Clostridium difficile-associated diarrhea was considered as a nosocomial infection, and as community-acquired diarrhea in 20 cases (44%). Treatment included isolation of the patient as soon as bacteriological diagnosis was known and specific therapy was instituted by metronidazole or vancomycin for a mean of 18 days. The addition of Saccharomyces boulardii was used in of cases. The clinical course was rapidly favorable for 80% of patients. Five patients died with complications of severe colitis in 2 cases. Mean hospital stay was 49 days (annual mean of the department=10 days). CONCLUSION: Clostridium difficile diarrhea concerns above all elderly patients with one or more underlying pathologies. Amoxicillin clavulanic acid and third-generation cephalosporins are the most frequently prescribed antibiotics in these cases and have the highest correlation with this infectious complication. This medical problem requires greater knowledge as it causes significant morbidity and increases the risk of prolonged hospital stays.
Cavard A, Lidove O, Aflak F
… +8 more, Drupt F, Goldenberg P, Baglin AC, Taksin AL, Malek-Chehire N, Wasserman MR, Terré C, Blétry O
Ann Med Interne (Paris)
· 2002 May · PMID 12218908
IgG-secreting lymphoplasmocytoid leukemia is a rare disease associating splenomegaly and B cell proliferation. Lymphoid phenotype is usually different from chronic lymphocytic leukemia. We report a case disclosed by hype...IgG-secreting lymphoplasmocytoid leukemia is a rare disease associating splenomegaly and B cell proliferation. Lymphoid phenotype is usually different from chronic lymphocytic leukemia. We report a case disclosed by hyperviscosity syndrome. Diagnosis was confirmed at routine ophthalmologic study.
Bouraoui H, Mahdhaoui A, Zaaraoui J
… +4 more, Mejdoub M, Ernez-Hajri S, Jeridi G, Ammar H
Ann Med Interne (Paris)
· 2002 May · PMID 12218907
TWO CASES: Candida albicans prosthetic valve endocarditis (PVE) is a rare entity with serious complications. We report two cases of Candida albicans PVE, confirmed by culture of the prosthetic valve. The first patient di...TWO CASES: Candida albicans prosthetic valve endocarditis (PVE) is a rare entity with serious complications. We report two cases of Candida albicans PVE, confirmed by culture of the prosthetic valve. The first patient died twenty days after surgery with cerebral bleeding secondary to multiple mycotic aneurysms, the second patient was still alive eight months following a Saint-Jude aortic valve replacement and prolonged antifungal therapy. The difficulty of diagnosis and management are discussed.
Salliot C, Escaut L, Minoui P
… +3 more, Minozzi C, Coumbaras J, Vittecoq D
Ann Med Interne (Paris)
· 2002 May · PMID 12218906
An HIV-infected man developed primary polymyositis (fever, myalgia, elevated serum muscle enzymes, and signs of inflammation) associated with serum anti-Jo1 antibodies and pulmonary fibrosis. Anti-Jo1 antibodies are exce...An HIV-infected man developed primary polymyositis (fever, myalgia, elevated serum muscle enzymes, and signs of inflammation) associated with serum anti-Jo1 antibodies and pulmonary fibrosis. Anti-Jo1 antibodies are exceptional in HIV-infected patients. We discuss the role of infection in polymyositis. HAART and corticosteroids led to rapid clinical and radiological improvement.
Ann Med Interne (Paris)
· 2002 May · PMID 12218905
A 61-year-old man developed an extrapyramidal syndrome 15 days after an episode of carbon monoxide poisoning. Two months later he developed severe deafness. Brain imaging revealed ischemic lesions in the region of the ba...A 61-year-old man developed an extrapyramidal syndrome 15 days after an episode of carbon monoxide poisoning. Two months later he developed severe deafness. Brain imaging revealed ischemic lesions in the region of the basal ganglia. Audiometric studies disclosed neurosensorial deafness. The patient died nine months later due to respiratory complications.
Gross C, Lecocq J, Isner ME
… +3 more, Vautravers P, Meyer C, Kehrli P
Ann Med Interne (Paris)
· 2002 May · PMID 12218904
Constipation is a symptom not to be neglected, especially if the complaint is recent. We report a case where this symptom was the only clinical manifestation of a voluminous retroperitoneal tumor. The tumor was found to...Constipation is a symptom not to be neglected, especially if the complaint is recent. We report a case where this symptom was the only clinical manifestation of a voluminous retroperitoneal tumor. The tumor was found to be a neurilemmoma of the fourth right lumbar roots, confirmed at pathological examination. Manifestations of extradural neurinomas generally result from compression of neighboring structures. The best preoperative diagnosis approach is magnetic resonance imaging but histology is required for confirmation. The close relations the tumor mass maintains with the unaffected nerve fibers makes resection difficult without postoperative functional consequences. Surgeons discuss the relevance of partial excision in order to preserve the nerve root in case of a benign slow-growing tumor. There does however appear to be a significant recovery of muscle strength even in case of total resection.
Ann Med Interne (Paris)
· 2002 May · PMID 12218903
- The frequency of concurrent scleroderma and pregnancy is low because scleroderma is a rare connective-tissue disease and the mean age of symptom onset is in the early 40s. The reciprocal influence of pregnancy and scle...- The frequency of concurrent scleroderma and pregnancy is low because scleroderma is a rare connective-tissue disease and the mean age of symptom onset is in the early 40s. The reciprocal influence of pregnancy and scleroderma and management of pregnancy are the purpose of this review. Current knowledge and key points. - There is no increase in infertility in women with scleroderma. The effect of pregnancy on scleroderma is a greater risk of renal crisis, especially when scleroderma is evolutive, diffuse and recent. Conversely, pregnancy is characterized by prematurity and small full-term infants because scleroderma induce placentar vascular abnormalities. There is no increase in miscarriages and spontaneous abortions. The pregnant scleroderma patient is a potential anaesthetic challenge because of physical difficulties and visceral involvement. Future and projects. - Planning of pregnancy and follow-up by a multidisciplinary experienced team in high risk pregnancies are guarantees for successful pregnancies. Future research will try to determine the role of microchimerism in scleroderma physiopathology and specify placental findings in order to ameliorate the obstetrical prognosis.
Ann Med Interne (Paris)
· 2002 May · PMID 12218902
Several studies have shown an association between the presence of systemic sclerosis in females and the presence of fetal cells in peripheral blood. These results have led to the hypothesis that systemic sclerosis (SSc)...Several studies have shown an association between the presence of systemic sclerosis in females and the presence of fetal cells in peripheral blood. These results have led to the hypothesis that systemic sclerosis (SSc) may indeed be the consequence of an allogeneic fetomaternal reaction. However, certain normal female controls also exhibit microchimerism. In addition, there are several clinical and histological differences between SSc and sclerodermoid graft versus host reaction. If microchimerism plays a role in SSc, it would therefore comprise one step in a multistep process. This review focuses on recent papers targeting microchimerism and its evaluation in SSc.
Ann Med Interne (Paris)
· 2002 May · PMID 12218901
CREST syndrome has been described as a form of progressive systemic sclerosis in which there is relatively limited involvement of the skin, prominence of calcinosis, Raynaud's phenomenon, esophageal dysfunction and telan...CREST syndrome has been described as a form of progressive systemic sclerosis in which there is relatively limited involvement of the skin, prominence of calcinosis, Raynaud's phenomenon, esophageal dysfunction and telangiectasia. The acronym CREST was coined in 1964 by Winterbauer in the USA but the very first case report was by French physicians Thibierge and Weissenbach in 1910. Antinuclear antibodies recognizing chromosomal centromere proteins are characteristic of CREST syndrome and are present in more than 50% of the cases. The prognosis of CREST syndrome is relatively good with a long lasting disease duration (>10 years). Two complications are seldom associated with CREST syndrome: digital gangrene with finger losses and pulmonary hypertension (3 to 14% of CREST syndrome). Pulmonary hypertension is a very late event and the prognosis is very severe (mortality rate of 50% after 2 years).
Mouthon L, García De La Peña-Lefebvre P, Chanseaud Y
… +3 more, Tamby MC, Boissier MC, Guillevin L
Ann Med Interne (Paris)
· 2002 May · PMID 12218899
Systemic sclerosis (SSc) is a connective tissue disorder that is characterized by excessive collagen synthesis by fibroblasts and by vascular hyperreactivity and obliteration phenomena. Excessive collagen production is t...Systemic sclerosis (SSc) is a connective tissue disorder that is characterized by excessive collagen synthesis by fibroblasts and by vascular hyperreactivity and obliteration phenomena. Excessive collagen production is the consequence of abnormal interactions between endothelial cells, fibroblasts and mononuclear cells. Immunological abnormalities are present very early in the development of SSc. Mononuclear cells, particularily macrophages and T lymphocytes play a prominent role in fibroblast activation and collagen synthesis through the cytokines they produce. Thus, lymphocytic infiltrates in the skin and in the lung are preferentially composed of CD8+ T lymphocytes, that produce important amounts of interleukin 4 (IL-4). The effects of IL-4 are added to these of transforming growth factor B (TGF-B) and connective tissue growth factor (CTGF) that stimulate collagen synthesis by fibroblasts. T lymphocytes produce important amounts of gamma interferon (INF-gamma) that is the best inhibitor of collagen synthesis by fibroblasts. However, the inhibitory effect of INF-gamma on collagen synthesis is diminished in SSc patients. Numerous autoantibodies can be evidenced in the serum of SSc patients. Three of them are specific for SSc and mutually exclusive: anti-centromere antibodies (Ab) in limited SSc, anti-Scl70 Ab in diffuse SSc and anti-RNA polymerase III Ab in diffuse SSc with renal involvement. These autoantibodies are good prognosis markers but their pathogenic role remains uncertain.
Coppo P, Veyradier A, Durey MA
… +4 more, Fremeaux-Bacchi V, Scrobohaci ML, Amesland F, Bussel A
Ann Med Interne (Paris)
· 2002 May · PMID 12218898
Thrombotic microangiopathies (TMA) encompass various severe diseases characterized by microangiopathic hemolytic anemia and peripheral thrombocytopenia, associated with fever, neurological signs and renal involvement. Mi...Thrombotic microangiopathies (TMA) encompass various severe diseases characterized by microangiopathic hemolytic anemia and peripheral thrombocytopenia, associated with fever, neurological signs and renal involvement. Microvascular thrombosis is the typical lesion, and results in tissue ischemia. Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are the two most classical forms. These two entities are clinically and histopathologically closely related. There is a body of evidence suggesting that endothelial cell injury is the initial event in TTP and HUS, and that it may be related to a large number of triggering factors, such as infection, connective tissue disease, drugs, cancer and chemotherapy, transplantation, and pregnancy. Endothelial cell injury enhances the release of ultra large forms of von Willebrand factor (ULvWF) multimers and other prothrombotic agents, such as plasminogen activator inhibitor and platelet activating factor, whereas it decreases the release of prostaglandin-I2, a strong inhibitor of platelet aggregation. Recently however, it has been shown that TTP and HUS were pathophysiologically distinct. Actually, TTP is associated with a deficiency in von Willebrand factor-cleaving protease, an enzyme involved in cleavage of ULvWF into circulating 200 kDa and 350 kDa fragments. This deficiency may be either congenital or acquired, and then related to an IgG inhibitory autoantibody. This protease deficiency may account for the high amounts of plasmatic ULvWF in TTP patients. In HUS, vWF-cleaving protease activity is found normal. HUS encompasses two distinct entities. Epidemic, or diarrhea-associated HUS, is associated with verotoxin or Shiga toxin-associated enterobacteriaceae. These toxins are directly responsible for endothelial cell injury. Sporadic HUS (also termed atypical HUS in children) is closely related to TTP, and shares the same triggering factors. Familial HUS has been associated in some cases with hypocomplementemia and factor H dysfunction, the pathophysiological role of which remains unclear. The study of the different triggering factors and predisposing factors may be useful to define different subsets of TMA, that may be characterized by their course and prognosis.
Hassine E, Bousnina S, Marniche K
… +5 more, Fennira H, Ben Khelil J, Ben Mustapha MA, Megdiche ML, Chabbou A
Ann Med Interne (Paris)
· 2002 May · PMID 12218897
Pulmonary involvement in Behçet's disease is an uncommon condition (12%). Thromboembolism of the superior vena cava and/or other mediastinal veins, aneurysms of the aorta and pulmonary arteries are the main vascular mani...Pulmonary involvement in Behçet's disease is an uncommon condition (12%). Thromboembolism of the superior vena cava and/or other mediastinal veins, aneurysms of the aorta and pulmonary arteries are the main vascular manifestations in addition to pulmonary infarct and intrathoracic hemorrhage. Despite their scarcity, respiratory symptoms may be life-threatening. The aim of this study was to assess the contribution of thoracic imaging for one of the most serious aspects of the disease: pulmonary artery aneurysm. We report five patients with pulmonary artery aneurysms (mean age: 39.5 years). Hemoptysia revealed Behçet's disease in three. Initially explored by conventional radiography, computed tomography and angiography, pulmonary artery aneurysms are currently investigated well with helicoidal computed tomography, digital angiography, magnetic resonance imaging (MRI) and angio-MRI. These imaging techniques provide helpful information for the diagnosis of Behçet's disease.