[Rhabdomyolysis with tetraparesis secondary to hepatitis E virus infection in a patient on statins]. / Rhabdomyolyse avec tétraparésie secondaire à une hépatite virale E chez un patient sous statines.

INTRODUCTION: Neurologic and muscular damage associated with acute hepatitis due to hepatitis E virus (HEV) are rare and may be underdiagnosed. CASE REPORT: We report the case of a 56-year-old man, presenting with flaccid tetraparesis secondary to an acute rhabdomyolysis induced by acute E virus hepatitis. He fully recovered after one month under supportive treatment. DISCUSSION: Rare cases of acute rhabdomyolysis induced by HEV infection have been reported in the literature. We discuss the potential adjuvant role of statin treatment in our patient. Unexplained acute neurological conditions should prompt the search for HEV infection.

DLNO/DLCO ratio evolution under targeted therapy in patients with pulmonary hypertension.

The ratio of the diffusing capacity of the lung for carbon monoxide (DLCO) and for nitric oxide (DLNO) measured simultaneously is modified in patients with precapillary pulmonary hypertension (PH). The potential impact of targeted therapy on the DLCO/DLNO ratio is unknown. Simultaneous measurements of DLNO and DLCO were performed at baseline, 3-4 month follow-up (first evaluation) and 12-month follow-up (second evaluation) after initiation of targeted PH therapies in incident cases of precapillary PH. The main outcome was the change in DLNO/DLCO ratio under treatment between baseline and the first evaluation. Twenty-nine patients were included (mean age: 66.8 years, 62.1% female). No significant change in the DLNO/DLCO ratio was found between baseline and the first evaluation. Similarly, no significant differences were noted with regard to changes in Dm or Vc, the DLNO/DLCO ratio in different patient subgroups, or in the 20 patients evaluated at the second follow-up. Within the limitations of this study, the DLNO/DLCO ratio is not useful in monitoring the response to treatment in PH.

Bleeding risk of terminally ill patients hospitalized in palliative care units: the RHESO study.

Essentials Bleeding incidence as hemorrhagic risk factors are unknown in palliative care inpatients. We conducted a multicenter observational study (22 Palliative Care Units, 1199 patients). At three months, the cumulative incidence of clinically relevant bleeding was 9.8%. Cancer, recent bleeding, thromboprophylaxis and antiplatelet therapy were independent risk factors. SUMMARY: Background The value of primary thromboprophylaxis in patients admitted to palliative care units is debatable. Moreover, the risk of bleeding in these patients is unknown. Objectives Our primary aim was to assess the bleeding risk of patients in a real-world practice setting of hospital palliative care. Our secondary aim was to determine the incidence of symptomatic deep vein thrombosis and to identify risk factors for bleeding. Patients/Methods In this prospective, observational study in 22 French palliative care units, 1199 patients (median age, 71 years; male, 45.5%), admitted for the first time to a palliative care unit for advanced cancer or pulmonary, cardiac or neurologic disease were included. The primary outcome was adjudicated clinically relevant bleeding (i.e. a composite of major and clinically relevant non-major bleeding) at 3 months. The secondary outcome was symptomatic deep vein thrombosis. Results The most common reason for palliative care was cancer (90.7%). By 3 months, 1087 patients (91.3%) had died and 116 patients had presented at least one episode of clinically relevant bleeding (fatal in 23 patients). Taking into account the competing risk of death, the cumulative incidence of clinically relevant bleeding was 9.8% (95% confidence interval [CI], 8.3-11.6). Deep vein thrombosis occurred in six patients (cumulative incidence, 0.5%; 95% CI, 0.2-1.1). Cancer, recent bleeding, antithrombotic prophylaxis and antiplatelet therapy were independently associated with clinically relevant bleeding at 3 months. Conclusions Decisions regarding the use of thromboprophylaxis in palliative care patients should take into account the high risk of bleeding in these patients.

[Sustained response with tocilizumab in a case of refractory relapsing polychondritis]. / Réponse prolongée après traitement par tocilizumab d'une polychondrite atrophiante.

INTRODUCTION: Relapsing polychondritis (RP) is a rare inflammatory disease characterized by diffuse cartilage involvement, especially those of the respiratory tract, leading to potentially life threatening complications. Corticosteroids remain the first-line empirical therapy. Immunosuppressive drugs such as azathioprine, cyclophosphamide and tumor necrosis factor blockers (anti-TNFα) are commonly used as second-line therapy with varying degrees of success. CASE REPORT: We report a 40-year-old man with severe RP for whom conventional therapy and immunosuppressive treatments were ineffective. Prolonged clinical remission was obtained after introduction of the anti-interleukin-6 receptor antibody (tocilizumab), which was perfectly tolerated and allowed to taper steroids and methotrexate to a very low dosage. CONCLUSION: Our patient is the fifth published one documenting the efficacy of tocilizumab in severe refractory RP, which strengthens the use of anti-IL-6 in that indication.

[Hypertrophic osteoarthropathy and aortic graft infection: Two cases]. / Ostéoarthropathie hypertrophiante et infection de prothèse aortique : deux observations.

INTRODUCTION: Hypertrophic osteoarthropathy (HOA) located to lower extremities may be the initial manifestation of an infected aortic graft. CASE REPORTS: We report two patients with HOA secondary to aortobifemoral vascular prosthesis infection and aortoenteric fistula. As reported in the literature, initial manifestations included fever, painful swelling of limbs, joint pain, clubbing and in one case intestinal bleeding. These symptoms preceded frank episodes of septicaemia due to a wide variety of bacteria (microbial enteric flora). Bone scan was an appropriate tool for confirming the diagnosis of HOA. Abdominal computed tomography, and PET-scan were useful for detecting vascular infection. Aortoenteric fistula remained difficult to identify. CONCLUSIONS: Mechanisms involved in the pathogenesis of HOA associated with infected graft are poorly understood, but vascular endothelial growth factor (VEGF) could play a major role. The mortality of infected aortic grafts remains high, but knowledge of this association could lead to early diagnosis and accurate treatment.

[Autoimmune autonomic ganglionopathy: a case series of six patients and literature review]. / Ganglionopathie autonomique auto-immune : une série de six observations et revue de la littérature.

PURPOSE: Auto-immune autonomic ganglionopathy is a recently described clinical entity within the spectrum of autonomic neuropathies. Patients with auto-immune autonomic ganglionopathy typically present with rapid onset of severe autonomic failure. Acetylcholine receptor ganglionic antibodies, directed against ganglionic synapsis, disrupt synaptic transmission in autonomic ganglia and lead to autonomic failure. These antibodies are specific for auto-immune autonomic ganglionopathy and are present in 50% of patients. METHODS: Descriptive retrospective study. We report six French patients who presented with auto-immune autonomic ganglionopathy diagnosed between 1996 and 2002. RESULTS: Four patients were men and the median age at diagnosis was 45 years. All patients presented with a subacute autonomic failure with constant severe orthostatic hypotension. Serological testing of acetylcholine receptor ganglionic antibodies was performed in four out of the six patients and was found positive in three. All the patients received intravenous immunoglobulin and a clinical improvement was observed in four of them. CONCLUSION: Auto-immune autonomic ganglionopathy is an unusual and overlooked disorder. However, this autonomic neuropathy deserves to be better known as most of the patients respond to immunomodulatory therapy.

[Granulomatous liver disease as the presenting feature of alveolar echinococcosis in an hepatitis C infected cardiac transplant patient]. / Granulomatose hépatique révélatrice d'une échinococcose alvéolaire chez un adulte transplanté cardiaque, infecté par le virus de l'hépatite C.

Alveolar echinococcosis (AE) is a rare but potentially serious zoonosis for which an early diagnosis is of primary importance. We report the first observation of AE in a cardiac transplant patient infected by hepatitis C virus. He first presented with a single asymptomatic hepatic nodule. The liver biopsy showed an epithelioid granuloma with necrosis. We review the clinical features, diagnosis and outcome of this disease in immunocompromised hosts. In immunocompromised patients living in areas endemic for Echinococcus multilocularis, AE should be included in the differential diagnosis of tumor like lesions of the liver.

[Inappropriate low glycated hemoglobin and hemolysis]. / L'hémolyse : une cause d'erreur dans l'interprétation de l'hémoglobine glycosylée.

Hemoglobin A1c (HbA1c), measured regularly in the patients with diabetes, is the major form of stable glycated hemoglobin and has to be maintained below 6.5% to prevent or decrease the risk of chronic complications. HbA1c reflects mean blood glucose levels of the previous 3 months. We report the case of a particularly low HbA1c in a diabetic patient despite high plasma glucose levels, that was induced by auto-immune hemolysis related to an Evans syndrome. All hemolytic disorders can be responsible for falsely reassuring HbA1c values. Clinicians must be aware that hematologic status has to be considered for the correct interpretation of HbA1c results.

[Tropheryma whipplei and Whipple disease: false positive PCR detections of Tropheryma whipplei in diagnostic samples are rare]. / Tropheryma whipplei et maladie de Whipple: résultats de la recherche par polymerase chain reaction (PCR) dans les échantillons à visée diagnostique.

BACKGROUND: PCR can be used to detect T. whipplei (Tw) in samples from variable tissue types and body fluids. We report clinical, evolutive characteristics and final diagnosis in patients with positive Tw PCR assay. METHODS: Retrospective study of Tw PCR realized since 10years in a microbiology laboratory. RESULTS: Twenty-five Tw PCR assays were positive among 200 realized. Diagnosis was not confirmed in six cases. One patient was missing for follow up. Eighteen patients presented with Whipple's disease. Among these 18 patients, 14 had a classic Whipple's disease, three patients presented an endocarditis and one patient isolated neurological manifestations. Ten patients presented fever, seven a weight loss and 12 joint involvement. Four patients presented cutaneous manifestations, only six had gastrointestinal symptoms. Neurological involvement was reported in five cases, pulmonary symptoms in four cases, cardiac involvement in six cases and ocular signs in two cases. Anemia was reported in four patients and elevated levels of acute-phase reactants in 14 cases. Positive predictive value of Tw PCR for Whipple's disease diagnosis was 75%. Thirteen patients had a good evolution with antibiotics. Three patients presented recurrence and two cases with cardiovascular involvement died. CONCLUSION: Whipple's disease is rare but often mentioned in internist experience. The diagnosis should be every time confirmed. Tw PCR assay is an important diagnostic tool but is not sufficient to establish the diagnosis and must be interpreted with histopathology and immunohistochemical testing results.

[Systemic sclerosis in men]. / Sclérodermie systémique chez l'homme.

PURPOSE: To study the initial clinical features and describe the outcome of systemic sclerosis in a cohort of French men. METHODS: Patients with systemic sclerosis based on Leroy's criteria were included. In this retrospective study we compared a cohort of men to a cohort of women, diagnosed between 1997 and 2005 in departments of internal medicine and rheumatology. RESULTS: One hundred and twenty-one patients were included amongst which thirty-six men. The mean follow-up duration was 6.5 years. The time to diagnosis was significantly shorter in men than in women. Diffuse cutaneous systemic sclerosis, cutaneous ulcers and interstitial syndrome on chest radiograph were more frequent at diagnosis in men than in women. An environmental factor (silica) was observed in only nine men. During the follow-up, incidence of restrictive lung disease was significantly higher in men than in women (37% versus 14% p=0.01) with higher rates of oxygen dependency (22% versus 5% p<0.01). Cumulated survival rates in men were 92% at 5 years, 72% at ten years and 43% at 15 years, respectively. The mean survival was 13 years in men (IC 95%: 10-16) versus 23 years in women (IC 95%: 10-36) with no statistical difference (p=0.27). CONCLUSION: If interstitial and restrictive lung disease, oxygen dependency and diffuse systemic sclerosis were more frequent in men than in women, this data did not provide any evidence of survival difference between men and women with systemic sclerosis.

Disseminated cryptococcosis and histoplasmosis co-infection in a HIV-infected woman in France.

We report the first case in Europe of co-infection with disseminated cryptococcosis and histoplasmosis. The diagnosis of invasive histoplasmosis was confirmed by microscopic examination of the anatomic right colon specimen (hemicolectomy). Histoplasma antigen detection is not yet available in France but it could have a key role in the early diagnosis of disseminated histoplasmosis co-existing with a cryptococcal infection, especially in HIV-infected African people.