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B Godeau

Publications and source records attributed to B Godeau.

At least 37 records · Page 2Linked to original sources
PubMed1998

Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare variant of plasma cell dyscrasia with multiple systemic manifestations. We followed the progress of 20 patients with POEMS syndrome in our institution over a 10-yr period. Pulmonary hypertension (PH) was observed in five patients. All patients suffered dyspnea on exertion, which always appeared during an exacerbation of POEMS syndrome. The typical echocardiographic signs of PH were observed in all of these patients, and the median pulmonary-artery systolic pressure was 57 mm Hg (range, 50 to 65 mm Hg). Mean pulmonary-artery pressure during right side heart catheterization in two patients was 32 mm Hg. No other explanation for the PH could be found. Overproduction of cytokines was found in all cases, with high serum concentrations of interleukin-1beta, interleukin-6, tumor necrosis factor-alpha, and vascular endothelial growth factor. We suggest that PH should be added to the list of symptoms of POEMS syndrome. Cytokines may mediate POEMS syndrome-associated PH, as proposed for the other systemic manifestations of this disorder.

Adult↗
PubMed1998

Pulmonary embolism; lung scanning interpretation: about words.

OBJECTIVE: To assess clinicians' agreement on how they interpret lung scan reports with regard to the diagnosis of pulmonary embolism. DESIGN: In this prospective study, nuclear medicine physicians provided two types of reports for each lung scan: a routine descriptive report and a short form with a standardized conclusion on the likelihood of pulmonary embolism: "high probability," "no conclusion," and "diagnosis excluded." Three independent blinded senior clinicians reviewed all routine reports and chose one of the following conclusions: "high probability," "no conclusion," or "diagnosis excluded." SETTING: An acute care teaching hospital near Paris. SUBJECTS: Eighty-two lung scans were studied. MAIN OUTCOME MEASUREMENTS: Inter-clinician agreement and agreement between clinicians' conclusions and the nuclear medicine physicians' standardized reports were analyzed using the kappa index. RESULTS: The distribution of the clinicians' conclusions from routine reports strongly differed (p < 0.001). Agreement among the three clinicians was observed in 40.2% of the routine reports, and the inter-clinician agreement was poor to moderate (kappa range, 0.28 to 0.52). A complete agreement among the three clinicians and the nuclear medicine physicians' standardized conclusions was observed for 32.9% of the reports. The agreement between each clinician and the standardized conclusions was also poor to moderate (kappa range, 0.32 to 0.55). CONCLUSION: Reading the same routine reports, clinicians reached different conclusions. Furthermore these differed greatly from the nuclear medicine physicians' standardized conclusions. These results support the notion that physicians should be given standardized reports.

Adult↗
PubMed1997

Specific antiplatelet glycoprotein autoantibodies are associated with the thrombocytopenia of primary antiphospholipid syndrome.

Thrombocytopenia is a frequent complication of primary antiphospholipid syndrome (PAPL) and has been attributed to antibodies directed against platelet glycoproteins (Gp) and also to antiphospholipid antibodies. We tested patients with PAPL with and without thrombocytopenia for specific antiplatelet autoantibodies. Platelet autoantibodies were detected by means of platelet immunoassays which included MAIPA with a panel of monoclonal antibodies directed against all the platelet Gps known to be possible targets for platelet autoantibodies. A high prevalence of serum platelet antibodies was found in patients with thrombocytopenia (73%, 11/15 patients) whereas antiplatelet antibody was detected in only one of the 10 control patients (P < 0.01). The antibodies mainly recognized GpIIbIIIa (n = 7), but also CD9 (n = 5), GpIaIIa (n = 4). GpIbIX (n = 3) and GpIV (n = 3). Platelet-Gp antibodies eluted from the platelet surface had the same reactivity as those found in the original sera from three of the four patients tested, whereas no anticardiolipin activity was found in the platelet eluates, suggesting the absence of cross-reactivity between anticardiolipin and antiplatelet antibodies. The MAIPA assay was also performed with F(ab')2 fragments obtained by pepsin digestion of serum IgG from four patients. The same results were obtained with F(ab')2 fragments and the original serum, demonstrating that platelet antibodies specifically bind in vivo to platelet Gps via their F(ab')2 fragments. Our results suggest a link between specific platelet antibodies and the thrombocytopenia of PAPL.

Adult↗
PubMed1997

Dapsone for chronic autoimmune thrombocytopenic purpura: a report of 66 cases.

Sixty-six adults with chronic autoimmune thrombocytopenic purpura AITP and platelet count <50 x 10(9)/l were treated with dapsone (75-100 mg orally). A response was observed in 33 patients. The median duration of treatment required to obtain a response was 21 d (range 8-90). The median maximal platelet count on treatment was 130 x 10(9)/l (range 71-355). Dapsone was continued in 20/33 responders for a median of 12.5 months (range 1-48) and the response persisted in 19. Treatment was intentionally withdrawn in the other 13 responders and thrombocytopenia immediately recurred in 12. Reversible side-effects required cessation of treatment in seven patients. These results demonstrate that dapsone is an effective, inexpensive, and well-tolerated treatment for chronic AITP.

Adult↗
PubMed1997

Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides.

Twenty-five patients with good-prognosis polyarteritis nodosa or Churg-Strauss syndrome entered a prospective, randomized, multicentre study comparing two treatments: either oral corticosteroids and oral cyclophosphamide (CY; 2 mg/kg/day) for 1 yr (group A), or oral corticosteroids and monthly i.v. CY pulses (0.6 g/m2) (group B) for 1 yr. The objective was to determine the optimal CY regimen. Judgement criteria were the efficacy of the treatment in controlling the disease and the development of side-effects. Among the 25 patients who could be analysed, complete recovery was achieved with the experimental treatment in 9/12 patients in group A and 10/13 patients in group B. Two patients in each group relapsed after the end of therapy and were well controlled by corticosteroids or other drugs. One failure occurred in each group. The mean follow-up was 60.8 +/- 14.5 months after the beginning of the treatment. Side-effects associated with the administration of CY and steroids were noted 27 times in group A vs 14 times in group B (not significant). The oldest patient in these series (group B) died of pneumonia. No superiority in terms of efficacy could be established between the two regimens; however, the number of patients included was too small to conclude definitively. Toxic side-effects were significantly more frequent in women (P < 0.02). The high number of adverse effects leads us to recommend pulse over oral CY and an overall lowering of the doses of immunosuppression.

Adult↗
PubMed1997

Short and longterm outcomes for patients with systemic rheumatic diseases admitted to intensive care units: a prognostic study of 181 patients.

OBJECTIVE: To determine short and longterm outcomes and prognostic factors for patients with systemic rheumatic diseases admitted to intensive care units in 4 teaching hospitals. METHODS: All adult intensive care unit admissions over a 12 year period for systemic rheumatic diseases were retrospectively assessed. One hundred and eighty-one patients with a mean age of 57 +/- 17 years were studied. RESULTS: The death rate in intensive care units was 33% (59/181) and in-hospital mortality was 43% (77/181). One hundred and four patients were discharged alive from hospital; 40 died during followup (mean 105 +/- 7 mo). The estimated 5 year survival rate for the discharged patients was 69%. The 4 factors significantly associated with in-hospital mortality by multivariate analysis were simplified acute physiologic score (p = 10(-4)), poor prior health status (p = 10(-4)), corticosteroid administration (p = 0.005), and the reason for admission; mortality was higher in the group admitted to intensive care for infectious complication (55 versus 34% for others; p = 0.006). In contrast, in-hospital mortality was not influenced by age or by systemic rheumatic diseases. Using Cox's model, only age over 60 years was a prognostic factor significantly associated with an increase in longterm mortality (p = 10(-4)). CONCLUSION: The short term outcome for patients with systemic rheumatic diseases in intensive care units was poor. The longterm prognosis after hospital discharge appeared fair, although the standardized mortality ratio was 5-fold that of a nonselected population. Short and longterm prognoses were similar for different systemic rheumatic disease groups.

Adult↗
PubMed1996

[Patients with systemic disease admitted to intensive care units: course and prognostic factors].

Based on our experience, hospitalization in intensive care units of patients with systemic diseases occurs several years after diagnosis. Though mortality ranges from 30 to 45%, it is not statistically different from that in unselected patients. Outcome is clearly related to controllable infectious complications. Three other prognosis factors are the gravity of the patient's status at admission (APACHE or IGS scores), corticosteroid therapy and prior health status. Long-term prognosis for patients discharged from intensive care is good with a 30% mortality at 5 years despite the irreversible nature of their disease.

Connective Tissue Diseases↗
PubMed1996

Rabies encephalitis in a patient with AIDS: a clinicopathological study.

A 46-year-old man was bitten by a dog in Mali; anti-rabies vaccination was incomplete. Three months later he was admitted to hospital with fever and diarrhea. Human immunodeficiency virus (HIV) serology was positive and CD4 count was 70/mm3. His status worsened rapidly with confusion hydrophobia and hypersialorrhea. Despite anti-rabies serotherapy and vaccination, he died suddenly 12 days after admission. Immunofluorescence on cerebral tissue samples established rabies encephalitis. Neuropathology showed mild encephalitis with occasional Babès nodules and rare perivascular mononuclear cuffs. Intraneuronal Negri inclusion bodies were remarkably diffuse and abundant. They were clearly demonstrated by immunocytochemistry and electron microscopy. Apoptotic neurons were identified in the brain stem and hippocampus in the vicinity of inflammatory foci. In contrast, apoptosis could not be demonstrated in non-inflammatory areas, even where Negri bodies were numerous. There was no associated HIV encephalitis or opportunistic infection. The occurrence of rabies encephalitis in AIDS represents a random association, but is probably not exceptional as rabies is endemic in many countries and the AIDS epidemic is spreading worldwide. In this case, although the incubation duration and clinical presentation were comparable to those in classical rabies the T-cell-mediated immunosuppression may account for the weak inflammatory reaction and unusually abundant viral multiplication. This observation confirms that all those at risk for rabies, particularly immunocompromised patients, should receive complete anti-rabies treatment including vaccines and specific immunoglobulins, as soon as possible after infection.

Acquired Immunodeficiency Syndrome↗
PubMed1996

[Treatment of autoimmune thrombopenic purpura in adults].

Autoimmune thrombocytopenic purpura (AITP) is a common hematological problem. Steroids are the usual first-line treatment but give long-term remission in less than 20% of adults. High doses of human intravenous immunoglobulin G (IVIg) can increase the platelet count in 70 to 80% of patients, but the treatment is expensive and the platelet response usually only transient. Seventy to 80% of the adults have the chronic form of AITP (ie, disease duration of more than 6 months) that only improves on specific treatment. Splenoctomy is the treatment of choice when thrombocytopenia is severe and/or associated with life threatening bleeding, as it cures 60-80% of patients. The best treatment for patients with severe chronic AITP in whom splenectomy is ineffective or contra-indicated is a difficult challenge. In this situation, spontaneous remission is rare and 5% of patients will die from hemmorrhage. However, the treatments so far proposed in refractory chronic AITP (danazol, vinca alcaloids, azathioprine, cyclophosphamide, dapsone, etc) are inconsistently and transiently effective. The aim of the treatments in AITP are thus different in the acute and chronic forms of the disease. In the acute phase, the treatment should quickly increase the platelet count, even if the effect in transient, and aggressive treatments (ie, splenectomy, immunosuppressive drugs) must be avoided since spontaneous remission is possible. On the contrary, splenectomy is the treatment of choice for chronic AITP since it obtains complete persistent recovery in nearly 80% of patients. In the case of unsuccessfullness, treatments should then be administered with the aim to maintain a "safe" platelet count (> 20 to 30 x 10(9)/L).

Acute Disease↗
PubMed1996

Fusidic acid induced acute immunologic thrombocytopenia.

Fusidic acid is used in hospitals as second-line therapy for multidrug-resistant staphylococcal infections. We report the first fully documented case of fusidic acid induced thrombocytopenia, in a 48-year-old patient. The thrombocytopenia was abrupt and severe but resolved spontaneously 7 d after drug withdrawal. The thrombocytopenia transiently relapsed 6 d later, when fusidic acid was reintroduced. Haemorrhagic signs were observed, but no severe bleeding occurred. Platelet transfusions failed to increase the platelet count. We detected an IgG platelet antibody in the patient's serum, that specifically recognized platelet glycoprotein IIb/IIIa only in the presence of fusidic acid. Fusidic acid induced thrombocytopenia should be considered as a possible cause for the thrombocytopenia frequently seen in the intensive care setting.

Acute Disease↗
PubMed1996

Treatment of chronic autoimmune thrombocytopenic purpura with monoclonal anti-D.

BACKGROUND: The platelet count increases transiently after treatment with polyclonal anti-D in about 50 percent of D+ patients with autoimmune thrombocytopenic purpura (AITP). The effect is usually attributed to macrophage Fc-receptor blockade by antibody-coated red cells. As polyclonal anti-D is in limited supply, prospective testing was performed on a monoclonal anti-D (MoAb D) in such patients. STUDY DESIGN AND METHODS: Seven D+ patients with chronic AITP received MoAb D intravenously at doses of 47 to 95 microg per kg of body weight. Response was assessed by studying platelet count increment. Hemolysis and red cell-bound MoAb D were measured before and after MoAb D administration. RESULTS: MoAb D red cell binding was demonstrated in all patients at a ratio higher than that observed in AITP patients successfully treated with polyclonal anti-D. However, little or no platelet count increment was observed in six patients, while a transient response was observed in only one (platelet count 97 x 10(9)/L before MoAb D infusion and 163 x 10(9)/L 4 days later). Furthermore, because five patients showed signs of hemolysis and two became anemic, higher doses of MoAb D should be used only with caution in patients with AITP. CONCLUSION: The MoAb D used in this study cannot be proposed as an alternative treatment for patients with AITP.

Antibodies, Monoclonal↗
PubMed1996

Acute arterial obliteration: a new feature of the POEMS syndrome?

The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin changes) syndrome is a rare variant of plasma cell dyscrasia with multisystemic manifestations. We present 4 cases with arterial symptoms typical of acute arterial obliteration (AAO) and review 9 similar cases in the literature. The clinical course of AAO was unusual and particularly severe when affecting the lower limbs; recurrent events required amputations. As demonstrated by angiographic and histologic studies, thrombotic and atheromatous lesions were the main pathologic features of AAO. Atherosclerotic risk factors were absent or moderate in 3 of our cases, and no cause of thrombosis other than the POEMS syndrome was found. A high production of cytokines was found in all cases, with elevated serum levels of interleukin-1 beta (9/9 samples), interleukin-6 (7/9 samples), and tumor necrosis factor-alpha (6/9 samples). We suggest that arterial manifestations should be added to the spectrum of manifestations of the POEMS syndrome. Cytokines may mediate the POEMS syndrome-associated AAO, as previously proposed for the other systemic manifestations of this disorder.

Acute Disease↗
PubMed1996

Bronchoalveolar lavage in adult sickle cell patients with acute chest syndrome: value for diagnostic assessment of fat embolism.

Fat embolism of necrotic bone marrow could be a frequent cause of acute chest syndrome (ACS) in sickle cell syndromes (SC), as suggested by postmortem findings. To check this hypothesis in living patients, we evaluated the presence of fatty macrophages recovered by bronchoalveolar lavage (BAL) in ACS. We investigated 20 consecutive cases of ACS by BAL, and identification of alveolar cells containing fat droplets was performed using oil red O (ORO), a specific neutral fat stain. The specificity of the method was determined on control groups, including eight SC patients without acute chest syndrome and 15 non-SC patients. A cut-off of > 5% of alveolar macrophages containing fat droplets was determined from the control groups to assess the diagnosis of fat embolism. In 12 ACS episodes, BAL exhibited > 5% of fatty macrophages, ranging from 10% to 100% (median value 46.5%). In 11 cases, fat embolism was associated with proven (n = 8) or probable (n = 3) bone marrow infraction, which mostly predated ACS. Eight ACS episodes were associated with a low percentage (< or = 5%) of fatty alveolar macrophages and could be related to a cause other than fat embolism in six episodes, such as sepsis, in-situ thrombosis, or rib infarcts generating hypoventilation. This study supports the diagnostic yield of BAL for fat embolism, which can be incriminated in 60% of cases of ACS in this adult population.

Adolescent↗