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B Godeau

Publications and source records attributed to B Godeau.

At least 19 recordsLinked to original sources
PubMed2006

Vasculitis restricted to the lower limbs: a clinical and histopathological study.

BACKGROUND: Rare cases of vasculitis restricted to the lower limbs have been reported, but the characteristics, outcome and response to treatment of this entity are not well known. OBJECTIVE: To describe the clinical, complementary examinations and response to treatment of this rare entity in the first retrospective series, and to compare data with historical pooled cases. METHODS: Retrospective analysis of all biopsy-proven cases observed over a 10-year period in four French tertiary medical units. Diagnosis of vasculitis restricted to the lower limb required the absence of any clinical symptom and complementary test finding, suggesting major extramuscular visceral involvement. RESULTS: 11 patients were included. Vasculitis restricted to the lower limb was associated with disabling muscle pain of the calves. Fever was present in 50% of cases; ankle arthralgia in 50% and skin involvement in 40%. MRI was the cornerstone of the diagnosis, showing hyperintense signal in T2 weight and in T1 weight after gadolinium injection. MRI findings correlated well with clinical outcome and were useful in guiding biopsy. Muscle biopsy was consistent with a polyarteritis nodosa-type vasculitis in only 40% cases, whereas a leucocytoclastic vasculitis was seen for all other cases. Treatment with corticosteroids was effective in all cases, but there were relapses requiring immunosuppressive agents in 54% of cases. CONCLUSION: Vasculitis of the calf muscles must be considered for patients with calf pain and with a biological inflammatory syndrome.

Adult↗
PubMed2006

[Hepatosplenic localization of cat scratch disease: two cases in immunocompetent adult patients].

INTRODUCTION: Cat-Scratch Disease (CSD) is a well-recognized benign cause of localized lymphadenopathy, which often recovers spontaneously. However systemic clinical presentations are described in immunodeficient adults (bacillary angiomatosis, bacillary splenitis) and are less common in immunocompetent ones. EXEGESIS: We report two cases of disseminated CSD in immunocompetent patients, presenting hepatosplenic nodules, associated in the second case with an endocarditis. CONCLUSION: Bartonella serology must be achieved in case of hepatosplenic nodules with fever. Treatment of disseminated CSD in immunocompetent adults is still empirical and recovery can occur without antibiotherapy when endocarditis is not associated.

Aged↗
PubMed2005

[Burkitt's lymphoma in a patient with systemic lupus erythematosus treated with immunosuppressive drugs. A case report].

INTRODUCTION: Neoplasia and lymphoproliferative disorders are sometimes reported in patients with systemic lupus erythematosus (SLE). However, the pathophysiological link between lymphoma and SLE is still a matter of debate. We report a new case of Burkitt's lymphoma occurring in a patient treated with immunosuppressive drugs for SLE. CASE REPORT: A 38-year-old woman with SLE treated for 10 years with immunosuppressive drugs was admitted for the rapid onset of multiple neuritis with cranial nerves palsy, without extra-neurological involvement. The cerebrospinal fluid was normal. A bone marrow biopsy revealed Burkitt's lymphoma. CONCLUSION: This is the third case reported of Burkitt's lymphoma occurring in SLE. Here we discuss the data of the literature and the possible pathophysiological links between Burkitt's lymphoma and SLE.

Adult↗
PubMed2005

[Calciphylaxis: a rare differential diagnosis of systemic vasculitis].

INTRODUCTION: Calciphylaxis is a rare phenomenon of medium- and small-vessel calcifications leading to cutaneous necrosis mimicking vasculitis. CASE REPORT: A 75 year-old-woman with chronic renal insufficiency was admitted for extensive cutaneous necrosis of the limb. Diagnosis of vasculitis was made, but inspite of corticosteroid therapy, the condition of the patient was worsening. After cutaneous biopsy, the diagnosis of calciphylaxis was established. CONCLUSION: Calciphylaxis must be suspected in cases of cutaneous necrosis occurring in a patient with chronic renal failure. Treatment requires rapid normalization of phosphocalcic balance. It is a condition with high mortality.

Aged↗
PubMed2005

[Acute splenic sequestration: a rare and severe complication of sickle cell anemia in adults. A case report].

INTRODUCTION: Acute splenic sequestration, a well-recognized complication of sickle cell syndromes, is characterized by a sudden decrease in haemoglobin concentration and marked painless splenomegaly. We report a case illustrating the outcome and the treatment options of this complication. CASE REPORT: A 45-year old homozygous woman developed acute splenic sequestration with severe anemia. Red blood cells transfusion led to transient improvement but a relapse-required splenectomy. Long-term outcome was favorable. CONCLUSION: Acute splenic sequestration is a severe complication mainly observed in children. Despite the severity of this complication, prompt diagnosis and appropriate therapy, and particularly red blood cells transfusions, led to a complete recovery. Splenectomy is required in the more severe form of the disease.

Acute Disease↗
PubMed2004

Intravenous immunoglobulin and autoimmune thrombocytopenic purpura: 22 years on.

Autoimmune thrombocytopenic purpura is now commonly treated with high doses of intravenous immunoglobulins. Twenty-two years after this treatment was first shown to be effective, several questions remain. We review here current knowledge concerning the frequency and type of side-effects and the probable mechanism of action of intravenous immunoglobulins. We suggest that the currently recommended dose of intravenous immunoglobulins (2 g/kg body weight) could be halved, that the total dose of intravenous immunoglobulins should be administered as a single infusion, that non-responders could be given another equal dose on day 3, and that intravenous immunoglobulins plus prednisolone should be considered as the gold standard for treatment of the most severe forms of the disease. Finally, as intravenous immunoglobulins have only a transient effect, they cannot be considered as a curative treatment for patients with chronic autoimmune thrombocytopenic purpura.

Humans↗
PubMed2003

[Recurrent Kikuchi's disease in a patient with discoid lupus].

INTRODUCTION: Histiocytic necrotizing lymphadenitis, also called Kikuchi-Fujimoto's disease (KD), usually occurs in young women and is characterized by localized lymphadenitis (mostly cervical) usually associated with fever. Diagnosis relies on histopathological and immunohistochemical analysis of involved lymph node. KD is considered to be the clinical expression of an inadequate immunological reaction, resulting in a self-limited hyperstimulation of the lymphoid tissue, trigerred by an antigenic infectious stimulation. The clinical course is usually benign with a spontaneous remission of symptoms. Recurrence is very rarely observed. KD may be associated with systemic lupus erythematosus, whereas the association with chronic discoid lupus has only been rarely reported. EXEGESIS: We report a case of KD recurrence, 9 years after the initial diagnosis, in a young eurasian woman, with a chronic discoid lupus erythematosus. CONCLUSION: KD is a benign disease with a spontaneously good outcome. Recurrence may rarely occur many years after the initial diagnosis but needs a new histopathological confirmation. Systemic or even cutaneous lupus erythematosus may be associated with KD. Although unfrequent, this association suggests that both diseases could share a common pathogenesis.

Adult↗
PubMed2002

[Characteristics of autoimmune hemolytic anemia in adults: retrospective analysis of 83 cases].

PURPOSE: To describe the main characteristics and outcome of adult's acquired immune hemolytic anemias (AIHA). To analyse the relevance of the complementary tests performed for the search of an underlying disease. METHODS: Retrospective (1980-2000) monocentric study. INCLUSION CRITERIA: age above 16, AIHA defined by an hemoglobin level below 12 g/dl in men and 11 g/dl in women, with hemolysis and/or a positive direct Coombs test and/or the presence of cold agglutinins (threshold 1/500) and/or in the absence of any other cause. RESULTS: Eighty three patients included (56 women and 27 men), with a mean age of 56 years (+/- 22) at AIHA onset including: 72 patients (87%) with warm antibody AIHA and 11 (13%) with cold agglutinin disease. The mean follow-up was 48 months (median 22 months). Among the 72 patients with warm antibody AIHA, the specificity of autoantibodies was: IgG + complement (43%), IgG (32%) or complement alone (25%); cold agglutinins (titre from 1/60 to 1/512) were detected in 15 (20%) of the patients. Antinuclear antibodies were detected (threshold: 1/80) in 33% of the cases. Hypogammaglobulinemia on serum protein electrophoresis (SPE) was significantively associated with the presence of an underlying non-Hogkin lymphoma (NHL). The CT-scan of the the chest and abdomen which was performed in 50% of the patients, showed abnormalities other than a spleen enlargement in 25% of the cases. The medullar biopsy (MB) was abnormal in 7 of 26 cases (27%) but lead by itself to the diagnosis of NHL in a single case. Thrirty seven (51%) of warm antibody AIHA cases were finally considered to be "secondary" to an underlying disease namely: NHLs (n = 14), Hogkin's disease (n = 1) connective tissue disease (CTD) (n = 14), drug-induced AIHA (n = 3), miscellaneous (n = 5). In 6 out of 14 cases (43%) of NHL's associated AIHA, the onset of AIHA precedes the NHL from 22 to 66 months. The response rates to different therapeutic regimens did not significatively differ when "secondary" and "idiopathic" AIHA were compared. Overall, 13 patients (15.6%) died mainly from infectious complications (n = 5) or an underlying NHL (n = 5). CONCLUSIONS: In more than half of the cases AIHA are associated with an underlying disease and AIHA may precede the onset of a NHL for a long period. In the absence of a clinically apparent underlying disorder, testing for the presence of antinuclear antibodies, a SPE and a CT-scan must be systematic. Conversely, if no abnormalities are found, the relevance of a systematic MB at AIHA onset seems very low.

Adult↗
PubMed2001

The nonexpression of CD36 on reticulocytes and mature red blood cells does not modify the clinical course of patients with sickle cell anemia.

It is thought that an increase in the adhesion of circulating reticulocytes to the vascular endothelium may initiate the vascular occlusion underlying the painful crises and organ failures typical of sickle cell disease (SCD). At least 2 receptors, usually present on reticulocytes, seem to be involved in this adhesion process: glycoprotein CD36 (glycoprotein IV) and integrin alpha(4)beta(1) (very late activation antigen--4). Recently, a high frequency of the platelet CD36--deficient phenotype was reported in black Africans. The frequency of this deficiency was similar in subjects with and without SCD. The role of CD36 in vaso-occlusion was then investigated by comparing the clinical course in 2 groups of black Africans homozygous for hemoglobin S, with and without CD36 deficiency, but similar in age, sex, geographical origin, number of alpha-globin genes, and beta-globin gene haplotype. Flow cytometry showed that CD36 was absent from the circulating red blood cells and reticulocytes of platelet CD36--deficient individuals but present on those from patients with normal platelet CD36 expression, and that alpha(4)beta(1) integrin levels were similar on the reticulocytes of the 2 groups. Neither clinical severity, as evaluated by the frequency and characteristics of vaso-occlusive events, nor biological data differed significantly in the 2 groups of patients. Finally, although CD36 has been suggested to play a critical role in the pathogenesis of vaso-occlusion, this study, despite including only a small number of patients, supports the idea that the modulation of expression of a single type of adhesion molecule is insufficient to counteract the pathological process leading to vaso-occlusion in SCD patients. (Blood. 2001;98:966-971)

Adolescent↗
PubMed2001

[Hemolytic anemia disclosing Wilson's disease. Report of 2 cases].

INTRODUCTION: The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease. EXEGESIS: We report two cases of Wilson's disease revealed by acute intravascular hemolytic anemia associated with liver failure. Blood smear analysis showed stippled red cells in one case; hemolytic anemia improved within a few weeks in both patients but progressive liver failure required transplantation in the other. Hemolysis probably results from the toxic effect of free serum copper on erythrocyte membrane. CONCLUSION: Diagnosis of Wilson's disease must be considered in case of acute hemolytic anemia associated with liver failure in young adults.

Adolescent↗
PubMed2001

[Sickle cell disease in adults: which emergency care by the internists?].

INTRODUCTION: Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the most prevalent genetic disease at birth in the Ile-de-France area. Internists are involved in the management of acute complications, particularly acute vaso-occlusive crisis. CURRENT KNOWLEDGE AND KEY POINTS: Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to the ischaemic process, and infectious complications. In adults, acute vaso-occlusive crisis is the major clinical problem prompting admission to the hospital and the main cause of death. It mainly manifests by osteoarticular pain but other clinical complications can be observed such as acute chest syndrome, priapism, ischaemic or haemorrhagic stroke, abdominal pain and acute multivisceral failure. The treatment of acute vaso-occlusive crisis is symptomatic. Simple transfusion or partial exchange transfusion is required in the more severe form of vaso-occlusive crisis. FUTURE PROSPECTS AND PROJECTS: The management of adult patients with sickle cell disease must be based on a multidisciplinary approach. At the present time, more than 50% of patients survive beyond the fifth decade. This better and longer life in developed countries has resulted from basic investigations and symptomatic treatments.

Acute Disease↗
PubMed2001

[Treatment of major sickle cell syndromes. The point of view of the internist].

Major sickle cell syndromes constitute at present the most important risk of genetic disease in the wider Paris area. Sickle cell patients are exposed to vaso-occlusive attacks and to chronic visceral complications of ischemic origin capable of affecting all organs. Acute vaso-occlusive complications represent the first cause of hospitalisation and the main cause of death for the adult patient. Functional asplenia also occurs with a risk of infection through encapsuled germs. The risk of infection must always be taken into account by clinicians treating adults even if serious infectious accidents are less frequent in adults than in children. Sickle cell disease is a public health problem. Better knowledge of acute vaso-occlusive complications and ways of treating them rapidly by simple symptomatic measures mean that the mortality rate and the risk of serious functional sequelae can be decreased. Such measures allow sickle cell patients to enjoy a life expectancy beyond 50 years.

Adult↗
PubMed2000

Acute chest syndrome in adults with sickle cell disease.

STUDY OBJECTIVES: Acute chest syndrome (ACS) is a frequent and potentially severe pulmonary illness in sickle cell disease (SCD). The aim of the study was to report the clinical features and outcome of consecutive ACS episodes in adult patients in a French SCD center. All patients were treated according to an uniform therapeutic protocol applying transfusion only in the more severe clinical form of ACS. RESULTS: There were 107 consecutive episodes in 77 adult patients (mean age, 29 +/- 7 years; 78% hemoglobin [Hb] SS; 14% Hb SC; and 8% Hb Sbeta + thalassemia) over a 6-year period. Seventy-eight percent of our patients had an associated vaso-occlusive crisis that preceded the chest signs in half of the cases. Comparison between acute and baseline levels showed a statistically significant difference in Hb levels (drop of 1.6 to 2. 25 g/dL depending on Hb genotype), WBC count (increase of 9.2 +/- 8. 3 x 10(9)/L); platelet count (increase of 67 +/- 209 x 10(9)/L); and lactate dehydrogenase values (increase of 358 +/- 775 IU/L) in ACS patients. Hypercapnia was detected in 42% of patients without sign of narcotic abuse. We identified a high percentage of alveolar macrophages containing fat droplets in 31 of 43 (77%) patients who underwent BAL. Bacterial culture findings were almost always negative, but were performed after starting antibiotic therapy that was administered in 96 episodes. Transfusion was required in 50 of 107 ACS events (47%). Five patients died, and all were transfused. CONCLUSIONS: These results confirm that fat embolism is probably a frequent mechanism of ACS in adult patients. However, fat embolism was not associated with a more severe clinical course, suggesting that bronchoscopy and BAL have little impact on the management of these patients. Restricting transfusion to the most severe ACS cases does not seem to increase the mortality rate.

Acute Disease↗
PubMed2000

Screening for autoimmune markers is unnecessary during follow-up of adults with autoimmune thrombocytopenic purpura and no autoimmune markers at onset.

In an attempt to evaluate the frequency of autoimmune markers in autoimmune thrombocytopenic purpura (AITP) and to determine if autoimmune markers in patients with isolated AITP were associated with particular disease manifestations, we analyzed records of 122 consecutive adults with AITP. Twenty-nine patients (24%) had significant titers of one or several autoimmune markers at AITP onset. Among them, 16 (13%) had antinuclear antibodies. The presence of autoimmune markers did not correlate with presenting feature, response to treatment or long-term outcome of AITP. Six patients (5%) developed seven autoimmune diseases during follow-up, comprising systemic lupus erythematosus, an antiphospholipid syndrome, autoimmune haemolytic anemia (n = 2), Grave's disease, Hashimoto's disease and primary biliary cirrhosis. At AITP onset, three of these patients had isolated biological markers of the autoimmune disease they later developed. The annual average incidence rate of autoimmune diseases was 1% per patient-year in the entire group and 0.4% in the group of patients with no autoimmune markers at AITP onset. This low rate is probably due to careful assessment at diagnosis for concomitant overt autoimmune disease. We recommend extensive screening for autoimmune markers at AITP onset, and careful follow-up of patients with autoimmune markers. Routine screening for autoimmune markers during AITP follow-up is not necessary for patients with no autoimmune markers at AITP onset. Systemic lupus erythematosus (SLE) and other autoimmune disorders can complicate autoimmune thrombocytopenic purpura (AITP) or be diagnosed concomitantly with otherwise unremarkable AITP (1, 2). However, the frequency and prognostic value of isolated autoimmune markers (i.e. not associated with an autoimmune disorder), particularly antinuclear antibodies (ANA) at AITP onset or during follow-up is controversial (3-8). For example, the committee organized by George et al. (9) to write guideline on the diagnosis and treatment of AITP stated that the search for ANA and lupus anticoagulant were of "uncertain appropriateness at diagnosis and during follow-up". In an attempt to help practicians to make decisions, we analyzed the frequency of autoimmune markers and autoimmune disorders at onset and during the follow-up in 122 adults with AITP and no overt autoimmune disease at diagnosis. These consecutive patients were followed by the same physician for a mean period of 6 years, and had routine screening tests for autoimmune markers and disorders at onset, before steroid therapy, and regularly during follow-up.

Adolescent↗