[Living... challenging our limits. Is wisdom a drug against feeling unease].
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Attitude to Death↗
Biomedical subjects
H Rousset
Publications and source records attributed to H Rousset.
Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases.
The aim of this study was to evaluate the response to treatment and the long-term outcome in a cohort of patients in whom severe autoimmune hemolytic anaemia (AHA) was the leading manifestation of systemic lupus erythematosus (SLE). Twenty-six women with severe isolated AHA were included. Corticosteroids were used as the initial treatment for all patients in our study. An initial response was obtained in all but one patient (96%). The overall recurrence rate was three per 100 person-years, with an expected recurrence-free proportion of 73% with a 180 months median follow-up. Seven patients (27%) experienced a relapse of AHA. We found a higher proportion of pleuritis in relapsing patients. Only three patients experienced multiple relapses despite splenectomy and several immunosuppressants. Steroid-sparing effect of hydroxychloroquine and azathioprine could not be assessed because most of the patients received these treatments for other reasons than AHA. Intravenous immunoglobulins induced transient response in three cases. Splenectomy was efficient to definitively control AHA in one patient but two patients quickly experienced relapses while one patient did not benefit. Five patients received immunosuppressants that induced only transient responses. Rituximab was long-term efficient in one case. In conclusion, severe AHA is a serious complication of SLE that warrants appropriate management. On the basis of our experience, the ideal treatment of isolated AHA should be oral corticosteroids in first-line treatment. Our study does not support an important role for splenectomy. Patients refractory to conventional therapy should be treated either with few toxic immunosuppressive drugs, danazol or rituximab.
Adolescent↗
[Venous thromboembolism and cancer].
OBJECTIVES: The risk of venous thrombosis during cancer is largely increased especially in case of chemotherapy, surgery, advanced stage disease, coagulation abnormalities. Survival of patients with cancer experiencing venous thrombosis seems to be worse. Although thrombosis may be a presenting feature of occult malignancy, there are insufficient data to support a more extensive screening than comprehensive medical history, physical examination, routine laboratory tests and chest radiography. CURRENT KNOWLEDGE AND KEY POINTS: Pathophysiology of venous thrombosis during cancer is unspecific: venous stasis, vessel wall damage, hypercoagulability). Other factors like platelet abnormalities or the direct responsibility of chemotherapy or hormonotherapy have recently been though to play a causative role. Treatment of cancer-associated thrombosis usually requires at least 6 months of low-molecular-weight heparin therapy rather than oral anticoagulant. Inferior vena cava filters are not indicated. Primary prophylaxis of thrombosis during cancer could safely been achieved with low-molecular-weight heparin. Central venous catheters can be associated with thrombotic complications. Many risks factors have been identified: catheter's type, modalities of catheter's implantation, type of perfusion, bulky mediastinal mass... Prophylactic anticoagulation is not routinely recommended. FUTURE PROSPECTS AND PROJECTS: Knew oral anticoagulants could facilitate the treatment of venous thrombosis occurring during cancer in the next years.
Administration, Oral↗
[Pentoxifylline: a cheap substitute for anti-TNFalpha agents?].
PURPOSE: Pentoxifylline (PTX) is a phosphodiesterase inhibitor drug used to improve peripheral vascular disease. In vitro studies demonstrated that PTX has anti-TNFalpha properties. We did a selective review of clinical trials which used PTX in patients with inflammatory rheumatic and non-rheumatic diseases. CURRENT KNOWLEDGE AND KEY POINTS: Most of the identified clinical trials were uncontrolled and involved a low number of patients. Use of PTX in systemic lupus erythematous, Behçet's disease and sarcoidosis yielded significant preliminary results. Moreover, PTX markedly reduced proteinuria in several glomerulonephritis (lupus nephritis, membranous nephropathy, diabetic nephropathy). FUTURE PROSPECTS AND PROJECTS: Further randomized and controlled clinical trials are required to examine whether PTX can improve outcome in patients with inflammatory diseases. Meanwhile, PTX should not be used for the treatment of these diseases.
Clinical Trials as Topic↗
[Whipple's disease endocarditis: report of 5 cases and review of the literature].
PURPOSE: Endocarditic lesions (infectious endocarditis) associated with Whipple's disease are exceptional. We report five cases from the cardiovascular and pneumologic hospital Louis Pradel in Lyon. METHOD: We have collected all cases of Tropheryma whipplei endocarditis diagnosed between 1995 and 2004. RESULTS: Five men with a mean age of 53 years at time of diagnosis. The symptoms were essentially cardiovascular: murmur, embolism in 3 cases, and heart failure secondary to valvular insufficiency in 2 cases. The valvular involvement, double in 3 cases, was more often aortic. Vegetations were present in all patients and valvular destruction sometimes very important. A low grade fever was present in 4 cases, associated with weight loss in 2 cases. The only extra-cardiac symptoms were arthralgias or arthritis in all cases, considered in 3 patients as seronegative rheumatoid arthritis, B27+ spondylarthritis, and psoriasic arthritis. Their was no other clinical manifestations of Whipple's disease, particularly digestive, ocular, neurologic or adenopathy, and duodenal biopsies secondarily performed in 4 cases were non contributive. This differs from literature as an extra-cardiac location was identified in 11 out of 17 cases. The diagnosis was obtained by histology and PCR on the cardiac valves, as all the patients underwent surgery. The evolution was favourable with a prolonged antibiotic therapy. CONCLUSIONS: These report confirms the existence of endocarditic forms of the Whipple's disease, in which the single extra-cardiac manifestation is rheumatologic, and reminds us the usefulness of histology and PCR on the cardiac valves at the time of valvular surgery.
Actinobacteria↗
Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: follow-up studies.
Marchiafava-Bignami disease (MBD), an acute toxic demyelination of the corpus callosum in alcoholics, is associated with poor evolution in the majority of patients. We report here the early and late diffusion magnetic resonance imaging (MRI) and apparent diffusion coefficient (ADC) studies of two patients suffering from MBD with favourable outcome. Diffusion and anatomical MRI changes were parallel to the clinical evolution, suggesting that MRI studies can be helpful for diagnosis and follow-up. Unlike in stroke, restricted diffusion on ADC maps does not seem to be a sign of irreversibility.
Adult↗
[Common variable immunodeficiency with autoimmune manifestations: study of nine cases; interest of a peripheral B-cell compartment analysis in seven patients].
PURPOSE: Autoimmune manifestations (AIM) are associated to common variable immunodeficiency (CVI) in about 20 to 25% of the cases. This study presents the clinical, biological characteristics and the evolution of nine patients developing CVI and AIM. A peripheral B-cell compartment analysis has been performed in seven cases. METHOD: This multicenter retrospective study analyses nine patients, six men and three women, within a population of 32 CVI. RESULTS: The mean age was 27 years at the time of diagnosis of AIM and 30 years at the time of diagnosis of CVI. The diagnosis of AIM preceded the diagnosis of CVI in five cases. Thirteen AIM of different types were observed: autoimmune hemolytic anemia (AHA, 3), immune thrombocytopenic purpura (ITP, 2), Evan's syndrome (2), primary biliary cirrhosis (1), rheumatoid arthritis (1), alopecia totalis (1), myasthenia gravis (1). The peripheral B-cell compartment was investigated in seven patients: five patients with autoimmune cytopenia presented with a diminution of memory B cells (CD27+IgD-) and immature B cells (CD21-) levels; the patient with primary biliary cirrhosis and myasthenia gravis had only a diminution of memory B cells level; the last patient with ITP presented with a normal level of memory B cells. Five among the seven patients with autoimmune cytopenia required a specific treatment using corticosteroids, high dosages of intravenous immunoglobulin, then splenectomy after failure of the medical management, with severe infectious complications in one case. CONCLUSION: The association of AIM and CVI is not fortuitous. The most common AIM is autoimmune cytopenia. The peripheral B-cell compartment analyses show that a majority of patients have a defect in memory B-cells. Treatment regimens are not standardized and splenectomy increases the risk of infectious complications.
Adolescent↗
[Pregnancy complications and baby blues...].
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Adipocytes↗
[Acute aseptic meningitis in adults. Retrospective study of 32 cases].
OBJECTIVE: The objective of this study was to analyze the characteristics of a series of acute aseptic meningitis (AAM) (defined by sterile cerebrospinal fluid (CSF) with more than 10 leucocytes per mm3 and a neutrophilic polynuclear-rich formula). We analysed the initial management, the reasons for antibiotic and/or antiviral treatment, the aetiologies, the need for lumbar puncture and the progression... METHOD: We retrospectively analyzed 32 cases of AAM (out of a total of 130 cases of meningitis) from two departments of internal medicine in Lyon, diagnosed between January 1996 and January 2003. Only the files fulfilling the AAM criteria were retained, selecting those with a minimum neutrophilic polynuclear level of 30% in the CSF. RESULTS: The mean age was 32.6 years (range: 18-75) and predominantly male patients (59%). On admission, 87% of the patients exhibited fever, but only 9% remaining so for 72 hours. Viral syndrome before admission was noted in 59% of cases, with seasonal predilection (summer: 39%, winter: 35%). The motivation for lumbar puncture (LP) was meningeal syndrome (44%), headache (94%) and vomiting (47%). The average rate of neutrophils in the CSF on admission was 63% (range: 30-96). A control LP on Day 3 was performed 16 times (50%): mean PNN rate at 18% (range: 0-80), lymphocyte rate=68% (range: 20-95). Most of the patients (77.4%) had a C reactive protein (CRP) lower than 50 mg/l on admission (range: 5-320). A cerebral scan was performed 10 times (31%) and was abnormal 2 times (multiple cerebral abscesses, possible intracranial hypertension). An antibiotic (84%) and/or antiviral (34%) treatment was initiated. The evolution on Day 3 was favourable (87.5%): no fever, regression of the meningeal syndrome, with a mean duration of hospitalisation of 8.3 days (range: 1-60). Search for Herpes simplex virus and Enterovirus was made with PCR analysis in 20 cases (62.5%): no positivity for the herpes, but 9 for the Enterovirus. The systematic blood cultures were positive only once (staphylococcal infective endocarditis with cerebral abscesses). The diagnosis of bacterial meningitis was evoked 3 times (prior antibiotic treatment). DISCUSSION: The frequency of Enterovirus AAM should encourage this type of investigation in order to withdraw the often initiated anti-infectious treatment rapidly, and hence avoid a second lumbar puncture.
Acute Disease↗
[Coeliac disease: disease of the future?].
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Celiac Disease↗
[Haemochromatosis screening in 120 patients complaining with persistant fatigue].
AIM: Chronic fatigue is the more frequent symptom identified in the course of hereditary haemochromatosis. A screening for this disorder was carried out in 120 primary care patients consulting for unexplained chronic fatigue. SUBJECTS AND METHODS: Transferrin saturation and serum ferritin were determined in all patients. If transferrin saturation was >or= 45% and serum ferritin >or= 300 microg/l, HFE1 genotyping for mutations C282Y and H63D was completed. RESULTS: One hundred and twenty patients were recruited, 19-86 years old, including 62 males and 58 females. 45 patients (38%) presented with serum ferritin >or= 300 microg/l. Thirty two patients (27%) presented with transferrin saturation >or= 45%. Twenty two patients (18%) presented with these two pathological values. Four C282Y/H63D compound heterozygous, one H63D/H63D homozygous, and eight simplex heterozygous (6 H63D and 2 C282Y) genotypes were found. Patients with serum ferritin >or= 300 microg/l were predominantly male (89%), older (57 year) and plethoric (BMI: 26.4) corresponding mainly to dysmetabolic hyperferritinemia. CONCLUSION: None of these 120 patients consulting for unexplained chronic fatigue was found with hereditary haemochromatosis. Therefore observed prevalence is 0, with upper limit of 95% confidence interval at 2.5%. But the high prevalence (38%) of serum ferritin >or= 300 microg/l must be emphasized, corresponding usually to dysmetabolic hyperferritinemia.
Adult↗
[Distal renal tubular acidosis ten years before Sjogren's syndrome].
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Acidosis, Renal Tubular↗
[MRI of central nervous system in a series of 58 systemic lupus erythematosus (SLE) patients with or without overt neuropsychiatric manifestations].
PURPOSE: Central nervous (CNS) involvement in SLE is common and can be evaluated with MRI. The primary goal of this study was to evaluate with high-field MRI the CNS involvement in a series of SLE patients with or without neuropsychiatric symptoms. The secondary goal was to detect a possible relationship between MRI and clinical or biological parameters in SLE. MATERIALS AND METHODS: We correlated the clinical and biological parameters of 58 patients with a lupus defined according to the American College of Rheumatology criteria, including 30 with neuropsychiatric manifestations with conventional and modern MRI (including diffusion weighted-images, high-resolution 3D T1 weighted-images). The population studied was compared to a group of 18 normal controls. RESULTS: In 69% of cases, MRI demonstrated involvement of the CNS both in asymptomatic patients (64.3%) and in patients with neuropsychiatric manifestations (73.3%): microembolic signals, cerebral infarctions (associated with the anti-phospholipid syndrome), atrophy, basal ganglia involvement, posterior leucoencephalopathy, subcortical calcification or hemosiderin deposits (T2*), dilated perivascular spaces. CONCLUSION: MRI with adapted sequences clearly demonstrated the cerebral involvement in approximately 70% of SLE patients with or without neuropsychiatric symptoms.
Adult↗
[A great imitator for the allergologist: intolerance to gluten].
Intolerance of gluten, resposible for Coeliac disease, is essentially shown by an auto-immune enteropathy, even if the cutaneous manifestation (herpetiform dermatitis) and perhaps certain neurological signs (cerebral syndrome, peripheral neuropathy) may be independent as well as associated with the intestinal illness. This affection is of immunological nature, occuring in a genetic field that predisposes to the illness (familial form: concordance of 70% in homozygote twins; 90% of patients show an HLA molecule of type DQ2, DQ8 in almost all the other cases. The exogenous factor is the gluten content contained in wheat, rye and barley, more precisely by the intermediary "the prolamines" which are the "reactive" element that induces a the same time an inflammatory reaction of type TH11 locally (expressed by the histological aspect of a duodenal biopsy evolving as villous atrophy) and a humoral response with production of anti-gliadine and anti-transglutaminase antibodies (the role of the latter enzyme is intervention in the local transformation of antigens to make them antigenic). It is an illness of adults as well as children and this point must now be emphasized. Recent epidemiological studies insist on a high prevalence (1/300 in Europe). Clinical expression, at the start very polymorphic and so misleading, before the appearance of the more classical signs of malabsorption and development, always feared, towards a lymphoma. These signs are haematological (anemia of various types, hyper platelets by hyposplenism, haemorrhagic signs) cutaneous (herpetiform dermatitis, cutaneous vasculitis) mucosal (aphtose), hepatic (cytolysis), neurophysical (fatigue, troubles of behaviour, cerebral syndrome, neuropathy) and osteo-articulitis (osteopenia, arthralgias, diffuse pains). The association of certain auto-immune illnesses must be emphasized (diabetes, Hashimoto thyroiditis, Gougerot disease, primitive biliary cirrhosis). To think early of the possibility of intolerance to gluten, is to give the means of a very easy diagnosis (measurement of anti-gliadin, anti-endomysium and anti-transglutaminase, and secondarily duodenal biopsy if necessary), and it is early elimination of gluten food which will make the various clinical manifestations disappear and so prevent the risk of evolution to a tumoral pathology.
Adolescent↗
Polycythaemia and iron deficiency.
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Adult↗
[Systemic lupus erythematosus with disease onset after age 65].
OBJECTIVES: Systemic lupus erythematosus with disease-onset in the elderly has rarely been studied (only one report about 21 patients with disease onset at 65 and older). Is the management of this pathology modified in this population? METHODS: Seventeen hospitalised cases of lupus patients with disease onset at 65 or older are retrospectively reported between 1988 and 2000. The results are compared with those of younger subjects. RESULTS: The female to male ratio is 1.83. Mean age at disease onset is 71.9 +/- 3.5 years. Mean duration of follow-up is 3.5 +/- 2.4 years. Main initial symptoms are: deterioration of general status (41%), arthritis (35%), cutaneous manifestations (35%), thrombo-embolism (24%) and pleuritis (18%). Malar rash is uncommon (12%). Nephropathy is never a revealing symptom and is rarely serious during the disease's evolution. Like in neurologic manifestations, the etiology has to be discussed in relation to associated co-morbidities. Concerning haematologic features, lymphopenia is found in 82% of the cases with a questionable specificity. Antinuclear antibodies are constant, anti-dsDNA antibodies are found in 82% of the cases, antibodies to extractable nuclear antigens in 50%, and anticoagulant circulating activity in 59%. Prognosis is difficult to assess in such a limited series but 5-years survival probability is 83%. Glucocorticoid lead to 50% of major complications. CONCLUSIONS: This study focuses on the particular initial manifestations of systemic lupus erythematosus in the elderly (deterioration of general status, thrombosis, unusual cutaneous symptoms), and on the specificity of differential diagnosis and treatment.
Age Distribution↗
[Inherited polycythemia].
PURPOSE: The majority of polycythemias occurs sporadically without any other familial case. Very occasionally polycythemia finds a familial support. This work is a review of the actual knowledge about inherited polycythemias. CURRENT KNOWLEDGE AND KEY POINTS: Although polycythemias linked to a anomaly of the haemoglobin affinity for oxygen are well understood (haemoglobin mutants with high oxygen affinity, 2-3 Diphosphoglycerate deficiency and methemoglobinemia), so called primary polycythemias (above all primary familial and congenital polycythemias) just begin to find an explanation for ten years (erythropoietin receptor gene mutation). FUTURE PROSPECTS AND PROJECTS: Progressively, the part of really idiopathic polycythemias is smaller and smaller. Although most of mechanisms to explain congenital polycythemias are understood, some of them are still unresolved (Chuvash polycythemia, the majority of primary familial and congenital polycythemias).
Hemoglobins↗