[Pulmonary hypertension and heart valve diseases induced by fenfluramine? Hypothesis or certitude? Myth or reality?].
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Animals↗
Biomedical subjects
P Godeau
Publications and source records attributed to P Godeau.
[An unknown cause of prolonged fever: apropos of 6 cases of chronic aortic dissection].
PURPOSE: Aortic arch dissection may be sometimes misdiagnosed due to the lack of mild to moderate chest pain. Definite diagnosis is often made while dissection has already occurred more than 15 days ago, being thereafter considered as chronic. Aortic dissection may then present as a prolonged febrile illness with fever and/or inflammation as main symptoms, with little or no pain. METHODS: We retrospectively reviewed cases of chronic aortic dissections seen in a department of internal medicine and a department of neurology between 1975 and 1992. RESULTS: We report six cases of patients presenting with aortic dissection and describe their outcome and treatments after the diagnosis was made based on either thoracic computerized tomography or trans-esophageal echocardiography evidence. Four patients had surgical aortic arch repair while one patient was treated with beta-blockers. CONCLUSION: Chronic aortic dissection has rarely been reported to cause fever or increased sedimentation rate. Treatment has to be discussed between medical and surgical teams involved in the therapeutical management of these unusual patients.
Aged↗
[Infectious aneurysm due to Listeria monocytogenes: a new case and review of the literature].
INTRODUCTION: Infections due to Listeria monocytogenes usually occur in pregnant women, in the elderly and in immunocompromised patients. Arterial aneurysms due to this germ are rare. Only 16 cases have been previously described in the literature. EXEGESIS: We report the case of a patient who had been hospitalized for recurrent fever over the past 3 months. Aortic mycotic aneurysm was diagnosed; blood and aneurysm cultures revealed Gram-positive bacilli consistent with the presence of Listeria monocytogenes. We also review previous reports focusing on infections due to Listeria monocytogenes. Mycotic aneurysms due to this germ are mainly observed in elderly male patients and occur on large arteries. In the present study, only one patient was immunocompromised. Furthermore, all patients who were not operated on died. CONCLUSION: Arterial aneurysm due to Listeria moncytogenes is best managed via surgical resection in combination with antimicrobial therapy. Immunosuppression is not necessary for the development of arterial aneurysm due to Listeria. Bacteriological and histological examinations should be done systematically when surgical resection of an aneurysm is required.
Aged↗
Susac syndrome.
Susac syndrome is an occlusive arteriolar disease that provokes infarcts in the cochlea, retina, and brain of young subjects, mostly women. Its cause is unknown. Some infarcts may be asymptomatic and only revealed by ancillary investigation: 1) audiogram that shows bilateral sensorineural hearing loss predominating on low frequencies, 2) funduscopy and fluorescein retinal angiography demonstrating bilateral distal branch retinal artery occlusions, and 3) brain MRI T2-weighted images disclosing small multifocal hyperintensities in white and gray matter. Treatment options are not codified, ranging from antithrombotic drugs to immunomodulatory therapy. Course is self-limited after an active fluctuating phase. Dementia, blindness, and deafness are rare late sequelae, and half of patients return to normal life.
Adult↗
Chronic intestinal pseudo-obstruction in systemic lupus erythematosus.
BACKGROUND/AIMS: Chronic intestinal pseudo-obstruction (CIPO) reflects a dysfunction of the visceral smooth muscle or the enteric nervous system. Gastrointestinal manifestations are common in systemic lupus erythematosus (SLE) but CIPO has not been reported. Features of CIPO are reported in five patients with SLE. METHODS: From 1988 to 1993, five patients with SLE or SLE-like syndrome were hospitalised for gastrointestinal manometric studies. CIPO was the onset feature in two cases. Antroduodenal manometry (three hours fasting, two hours fed) was performed in all patients, and oesophageal manometry in four. RESULTS: Intestinal hypomotility associated with reduced bladder capacity and bilateral ureteral distension was found in four patients and aperistalsis of the oesophagus in three. Treatment, which consisted of high dose corticosteroids, parenteral nutrition, promotility agents, and antibiotics, led to remission of both CIPO and urinary abnormalities in all cases. Antroduodenal manometry performed in two patients after remission showed increased intestinal motility. One patient died, and postmortem examination showed intestinal vasculitis. CONCLUSIONS: CIPO in SLE is a life threatening situation that can be reversed by treatment. It may be: (a) a complication or onset feature of the disease; (b) secondary to smooth muscle involvement; (c) associated with ureteral and vesical involvement; (d) the result of intestinal vasculitis.
Adult↗
Apoptosis and expression of soluble Fas mRNA in systemic lupus erythematosus.
Our objective was to measure the relative proportions of messenger RNAs coding for soluble and membrane-bound Fas in peripheral blood mononuclear cells (PBMCs) and to quantify lymphocyte apoptosis in vitro in systemic lupus erythematosus (SLE). A RT-PCR (soluble/membrane Fas mRNA) ratio was calculated in 16 SLE, 11 RA and 16 healthy subjects' PBMCs. Apoptosis was quantitated in vitro using nick-end labeling and flow-cytometry analysis immediately and after overnight T cell activation. The mean soluble/membrane Fas ratio was 1.219+/-0.424 in the SLE group, significantly higher than in healthy subjects, 0.516+/-0.180 (P = 0.0001). There was no significant difference between inactive and active SLE groups whereas Fas ratio was higher in SLE patients with nephritis (1.460+/-0.365) compared to those without nephritis (1.031+/-0.380) (P=0.039). Mean soluble/ membrane Fas ratio in RA patients (0.975 +/- 0.37) was higher than in healthy subjects (P = 0.0003) and lower than lupus nephritis patients (P=0.015). A significantly higher proportion of mononuclear cells engaged apoptosis after T cell activation in active lupus patients, compared to control subjects. In comparison with healthy subjects, Fas alternative splicing was skewed toward the soluble form of Fas in SLE and RA. Apoptosis after T cell activation in vitro was increased in active SLE patients.
Adolescent↗
Ribavirin in hepatitis C related cryoglobulinemia.
OBJECTIVE: An open, uncontrolled trial of ribavirin, an oral guanosine nucleoside analog for treatment of hepatitis C, in patients with hepatitis C virus (HCV) associated cryoglobulinemia intolerant to interferon. METHODS: Five patients with cryoglobulinemia related to HCV infection unresponsive to interferon therapy received oral ribavirin (100 to 1200 mg daily) for 10 to 36 months. RESULTS: Patients treated with ribavirin had prompt decrease in serum aminotransferase levels and marked improvement of manifestations of cryoglobulinemia within a few weeks. Ribavirin did not eradicate HCV RNA from the sera, but a decrease in viral load was observed in 3 patients, from 232 to 86 x 10(5) copies HCV/ml. Relapse occurred within 3 months once therapy was discontinued. The drug was well tolerated, but mild dose related hemolysis was common. CONCLUSION: Ribavirin monotherapy may be effective in patients with symptomatic cryoglobulinemia related to HCV infection, but this effect is not sustained when ribavirin therapy is discontinued.
Aged↗
The natural course of cerebral lesions in Sneddon syndrome.
OBJECTIVES: To characterize the clinical, biological, and neuroradiological findings in Sneddon syndrome; to correlate magnetic resonance imaging abnormalities with disability, presence of hypertension and other vascular risk factors, presence of heart valvulopathy on echography, and titer of antiphospholipid antibodies; and to compare these findings in antiphospholipid-positive and antiphospholipid-negative patients. DESIGN: Retrospective review of the records of 32 consecutive patients with livedo reticularis and neurological events, followed up in our institution between January 1991 and August 1995. PATIENTS: Twenty-six patients (20 women and 6 men) who had at least 1 cerebral ischemic arterial event associated with generalized and pathological livedo reticularis. RESULTS: The age at the first cerebral ischemic event ranged from 22 to 58 years. Motor deficit was the most frequent sign (found in 73% of cases). Disability was found in 50%, systemic hypertension in 65%, heart valvulopathy in 61%, and antiphospholipid antibodies in 42% of cases. Patients were classified in 6 groups according to magnetic resonance imaging findings. No correlation was found between the presence of hypertension or other vascular risk factors, valvulopathy, antiphospholipids, and magnetic resonance imaging abnormalities. There was no significant difference between antiphospholipid-positive and antiphospholipid-negative patients except for the presence of antinuclear antibodies. There was a significant correlation between the extent of magnetic resonance imaging abnormalities and disability. CONCLUSION: The severity of the disease seems to be correlated with magnetic resonance imaging aspects, but not to the presence of antiphospholipid antibodies. Magnetic resonance imaging may help to understand the natural course of the cerebral involvement of the disease.
Adult↗
Pregnancy in relapsing polychondritis: twenty-five pregnancies in eleven patients.
OBJECTIVE: To determine maternal and fetal outcome in pregnant women with relapsing polychondritis (RP). METHODS: Retrospective review of 11 records selected from among those of 116 female RP (Michet's criteria) patients. RESULTS: In these 11 women, 25 pregnancies occurred after or concomitantly with the onset of RP. The mean (+/- SD) age at RP onset was 25 +/- 5 years (range 15-31). Therapeutic abortion was performed in 1 woman because of ongoing cyclophosphamide treatment. Disease flare occurred in 7 of 24 pregnancies, requiring treatment modification in 2 cases. RP was considered stable in 16 and asymptomatic in 1 of the 24. Treatment of RP was with nonsteroidal antiinflammatory drugs (2 of 24), steroids (13 of 24), dapsone (7 of 24), or plasma exchanges (1 of 24); no treatment was given in 8 of the 24 pregnancies. In 14 pregnancies, the course was uneventful. Ten pregnancies were complicated by ectopic nidation (n = 3), spontaneous abortion (n = 3), premature birth (n = 3), and premature rupture of membranes (n = 1). Eighteen normal children were born. CONCLUSION: Pregnancy does not seem to modify the course of RP. Successful pregnancies may be achieved in women with RP. No RP was observed in the neonates.
Abortion, Spontaneous↗
Endothelin-1 in the lungs of patients with pulmonary hypertension.
BACKGROUND: Pulmonary hypertension (PH) is characterized by an increase in vascular tone and an abnormal proliferation of muscle cells in the walls of pulmonary arteries. Recent studies have found high plasma endothelin-1 (ET-1) concentrations in patients with PH. This study was conducted to assess whether elevated circulating ET-1 levels in PH really reflect excessive local pulmonary production. METHODS: We prospectively studied ET-1 concentration in lung specimens from 6 control subjects and 13 patients with severe PH referred for lung or heart-lung transplantation (6 patients had primary PH and 7 PH secondary to congenital heart defect). Endothelin-like immunoreactivity (ET-LI) was measured in plasma and lung tissue, using a radioimmunoassay, after ET-1 extraction. Reverse-phase high-performance liquid chromatography was also performed. RESULTS: Peripheral venous plasma ET-LI concentrations in patients with PH, whatever the cause, were greater than those in controls (10.7 +/- 0.8 vs 5.3 +/- 0.7 pg/ml; P < 0.0005). Pulmonary ET-LI was significantly higher in patients with PH, irrespective of its cause, than in controls (25.2 +/- 5.1 vs 8.1 +/- 1.1 pg/mg, P < 0.03). ET-LI pulmonary concentrations were slightly higher in Eisenmenger than in primary PH, but this was not significant (27.1 +/- 8.6 vs 22.8 +/- 5.4 pg/mg). Linear regression analysis indicated a small but significant correlation between ET-LI pulmonary concentrations and pulmonary vascular resistance in the patients with PH (r = 0.38; P = 0.047). In each case, HPLC separation of ET indicated that most of the immuno-reactivity was detected in the same fraction as ET-1. CONCLUSIONS: The striking increase in ET-1 pulmonary concentration provides new evidence that excessive local pulmonary ET-1 production may contribute to the vascular abnormalities of pulmonary hypertension.
Adult↗
[A rare cause of chronic diarrhea].
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Adult↗
[Aneurysm of the thoracic aorta and spondylodiscitis disclosing brucellosis].
Brucellosis, which decreased during the eighties in France, remains a public health problem in many Mediterranean countries. We report the case of a 65-year old patient native of Morocco, of thoracic aorta aneurysm and lumbar spondylodiscitis due to Brucella melitensis, revealed by haemoptysis and lumbar pains, with a favourable outcome after aortic graft resection, spinal plaster immobilization and specific lengthy antibiotic treatment. This case report is characterized by the absence of endocarditis or infectious focus near the aneurysm. Diagnosis of aneurysm and spondilitis due to Brucella melitensis is based on imaging and bacteriological and serological examination. Because of a clinical and biological intolerance for rifampicin and cotrimoxazole, this patient received ofloxacin-doxycyclin-streptomycin. We discuss antibiotic recommendations and stress the interest of the early diagnosis of complicated forms of brucellosis for a better prognosis.
Aged↗
[Horton disease disclosed by a specific inflammatory arteriopathy of the lower limbs].
The authors report the case of a woman aged 67 years who presented an acute ischemia of the lower limbs. A surgical exploration showed no atherosclerosis or thrombus. A biopsy of the femoral artery found an extensive mononuclear cell infiltration of the media and a fragmentation of internal elastic lamina. The diagnosis of Horton disease was considered and confirmed by the presence of clinical signs of polymyalgia rheumatica and inflammatory syndrome (erythrocyte sedimentation rate: 75 mm). Corticosteroid therapy was started with success. A year after discharge there was no recurrence of ischemic manifestations and the erythrocyte sedimentation rate was normal. Involvement of lower limbs in Horton disease is rare and exceptionally proven. But postmortem studies indicate that it is more frequent than previously reported. The diagnosis should be considered in the differential diagnosis of any unexplained case of peripheral vascular disease occurring in the middle-aged or elderly.
Aged↗
[Thrombomodulin in internal medicine].
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Humans↗
[Antiphospholipid syndrome and atrophic polychondritis].
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Adult↗
'Localized amyloidosis': 123I-labelled SAP component scintigraphy and labial salivary gland biopsy.
In apparently localized amyloidosis, there is no appropriate test to determine whether systemic deposits exist. We studied the value of serum amyloid P component (SAP) scintigraphy and labial salivary gland (LSG) biopsy on patients with apparently localized amyloidosis in 12 patients who had neither clinical nor biological evidence of systemic amyloidosis. All patients had an LSG biopsy and echocardiography. Iodine-123-labelled serum amyloid P component (123I-SAP) scintigraphy was performed in all patients. Whole-body scintigraphy was done, and tissue retention was evaluated at 24 h and 48 h. Of these 12 patients, three had amyloidosis in their LSG and had abnormal 123I-SAP scintigraphy; these three had a secondary clinical history of systemic amyloidosis. Three other patients had abnormal 123I-SAP scintigraphy without detectable systemic amyloid deposits, but one had a previous history of bilateral carpal tunnel syndrome treated with infiltration. 123I-SAP scintigraphy in association with LSG biopsy may be helpful in determining the localized or systemic character of amyloid disease.
Adult↗
Complete recovery of right intraventricular thrombus and pulmonary arteritis in Behçet's disease.
We report on a patient who presented with sustained fever, weight loss, haemoptysis and elevated erythrocyte sedimentation rate. The diagnosis of Behçet's disease was based on recurrent oral and genital aphthae, pseudofolliculitis and a history of thrombophlebitis. A right intraventricular thrombus and bilateral pulmonary aneurysms were discovered. Their complete recovery was observed within 6 months after a combination of prednisone, azathioprine, colchicine and aspirin therapy was started.
Adrenal Cortex Hormones↗
Outcome of planned pregnancies in systemic lupus erythematosus: a prospective study on 62 pregnancies.
We conducted a prospective study in order to determine planned pregnancy outcome in systemic lupus erythematosus followed in a tertiary referral centre. Pregnancy was authorized if disease was inactive on 20 mg/day prednisone or less for at least 1 yr. Upon the diagnosis of pregnancy, systematic corticosteroids consisting of 10 mg/day prednisone or more were started. In the case of antiphospholipid antibodies, 100 mg/day aspirin was added, replaced by heparin in the pre-partum period. In the case of antiphospholipid syndrome complicated by previous thrombotic events or fetal losses despite aspirin, heparin was prescribed. One woman with a history of atrioventricular block was treated with dexamethasone. Patients were monitored by medical and obstetrical examination, and laboratory tests carried out at least monthly and a quarterly echography. Among 62 pregnancies in 38 women, lupus flare was observed in 27% of the cases, 6% of which occurred in the post-partum period. Flares were moderate except in one renal involvement in a woman with prior diffuse proliferative glomerulonephritis. Therapy was not modified in half of the cases. Pregnancy ended in early spontaneous abortion not related to lupus flare (n = 10), stillbirth (n = 2). induced abortion (n = 2), preterm birth (n = 29) and full-term birth (n = 19). Caesarean section was performed in nine cases. A severe infection occurred in two premature neonates. Another premature neonate was growth retarded. Two children had cutaneous neonatal lupus. No child died, neither had atrioventricular block. Stillbirth and severe prematurity were more common in mothers with antiphospholipid syndrome. After exclusion of early spontaneous and induced abortions, the live birth rate was 96%, that is close to the French general population. The main problem remains a high rate of prematurity, but without maternal or neonatal death.
Adrenal Cortex Hormones↗