[Aortitis after G-CSF injections]. / Aortite après injections de G-CSF.

INTRODUCTION: Aortitis consists in aortic wall inflammation from infectious or non infectious cause. It may lead to aortic aneurysm with a risk of rupture, which is life-threatening and may justify surgical procedures. The cause of the aortitis is sometimes obscure. CASE REPORT: We report the case of a 55 years old woman who developed acute aortitis of the descending aorta after G-CSF (granulocyte-colony stimulating factor) injections for blood stem cells graft. No cause was found to the aortitis, the evolution was favorable after corticosteroid treatment, without aneurysm at six months. CONCLUSION: The present case rises the question of G-CSF (Neupogen responsibility in aortic lesions. Neutrophilic mediated diseases (Sweet's syndrome, pyoderma gangrenosum) and leukocytoclastic vasculitis were reported after G-CSF therapy. Neutrophils induced by G-CSF injections present functional abnormalities which may play a role in the pathogenesis of these diseases.

An Atlas on genes and chromosomes in oncology and haematology.

The "Atlas of Genetics and Cytogenetics in Oncology and Haematology" http://www.infobiogen.fr/services/chromcancer is a peer-reviewed and free internet database aimed at genes involved in cancer, cytogenetics and clinical entities in cancer, and cancer-prone diseases. It contains concise and updated review articles, a huge portal towards genetics and/or cancer databases, and teaching materials in genetics for the students. This database is made for and by clinicians and researchers, who are encouraged to contribute. The Atlas is part of the genome project. It provides information in cancer epidemiology. It contributes to research, university and post-university teaching, and telemedicine. It contributes to 'meta-medicine', a mediation using new information technology, between the overflowing information provided by the scientific community and the individual practitioner.

Multiple atherosclerotic plaque rupture in acute coronary syndrome: a three-vessel intravascular ultrasound study.

BACKGROUND: To test the hypothesis of general atherosclerotic plaque destabilization during acute coronary syndrome (ACS), the present study sought to analyze the 3 coronary arteries by systematic intravascular ultrasound scan (IVUS). METHODS AND RESULTS: Seventy-two arteries were explored in 24 patients referred for percutaneous coronary intervention after a first ACS with troponin I elevation. Fifty plaque ruptures (mean, 2.08 per patient; range, 0 to 6) were diagnosed by the association of a ruptured capsule with intraplaque cavity. Plaque rupture on the culprit lesion was found in 9 patients (37.5%). At least 1 plaque rupture was found somewhere other than on the culprit lesion in 19 patients (79%). These lesions were in a different artery than the culprit artery in 70.8% and were in both other arteries in 12.5% of these 24 patients. Complete IVUS examination of all 3 coronary axes in patients who had experienced a first ACS revealed that multiple atherosclerotic plaque ruptures were detected by IVUS; these multiple ruptures were present simultaneously with the culprit lesion; they were frequent and located (in three quarters of cases) on the 3 principal coronary trunks; and the multiple plaque ruptures in locations other than on the culprit lesion were less severe, nonstenosing, and less calcified. CONCLUSION: Although one single lesion is clinically active at the time of ACS, the syndrome seems nevertheless associated with overall coronary instability.

[Multiple ruptures of atherosclerotic plaques in acute coronary syndrome. Endocoronary ultrasonography study of three arteries]. / Ruptures multiples de plaques d'athérosclérose dans les syndromes coronaires aigus. Etude échographique endocoronaire des trois artères.

The aim of this study was to assess the three coronary arteries systematically by endocoronary ultrasonography in patients with unstable angina to check the hypothesis of global destabilisation of atherosclerotic plaques in acute coronary syndromes (ACS). Sixty two coronary arteries were examined (2.6 per patient). Fifty plaque ruptures were diagnosed (2.08 per patient). Rupture of a plaque of the culprit lesion of the ACS was clearly detected in 9 patients (37.5%). At least one ruptured plaque on a site other than the culprit lesion was observed in 19 patients (79%), on another artery in 70.8% of cases and on two other arteries in 12.5% of cases. A complete endocoronary ultrasonic examination of the three coronary arteries in patients with a first ACS demonstrated that: multiple atherosclerotic plaque rupture may be detected by endocoronary ultrasonography; these multiple plaque ruptures occur simultaneously with the culprit lesion; they are frequent and can be situated on the three main coronary vessels and multiple plaque rupture other than the culprit lesion are less severe, non stenotic and less calcified. Thus, although a single lesion is clinically symptomatic, ACS seems to be associated with global coronary instability.

[Subacute infectious endocarditis due to the agent of cat scratch fever: Bartonella henselae]. / Endocardite infectieuse subaiguë due à l'agent de la maladie des griffes du chat: Bartonella henselae.

The diagnosis of severe mitral stenosis with left atrial thrombus was rectified at valvular replacement in a 48-year old immuno-competent man who was a cat owner. The mass in the left atrium was, in fact, a large endocarditic vegetation. Pre- and postoperative blood cultures were negative as was culture of the excised mitral valve. The diagnosis of infectious endocarditis (IE) due to Bartonella Henselae was made from a positive serological test (1600) and identification of the germ by genetic amplification. Antibiotic therapy was continued for 6 months and the patient was cured with a follow-up of 4 years. Bartonella Henselae IE is very rare (14 reported cases) and affects mainly the aortic valve, often giving rise to very large vegetations which, in half the cases, are complicated by systemic emboli. Germs like Batonella are sensitive to most antibiotics, especially the aminosides and macrolides. In Bartonella Henselae IE, valve replacement is the rule (13 out of 14 cases) and the prognosis is usually good. Sero-diagnosis of Bartonellosis should be part of the systematic investigation of all blood culture negative IE.

Atlas of Genetics and Cytogenetics in Oncology and Haematology, updated.

The 'Atlas of Genetics and Cytogenetics in Oncology and Haematology' (http://www.infobiogen.fr/services/chromcancer) is an Internet database aimed at genes involved in cancer, cytogenetics and clinical entities in cancer, and cancer-prone diseases. It presents information in concise and updated reviews (cards) or longer texts (deep insights), a (new) case report section, a huge portal towards genetics and/or cancer databases, and teaching items in genetics for students in medicine and the sciences. This database is made for and by clinicians and researchers in the above-mentioned fields, who are encouraged to contribute. It deals with cancer research, genomics and cytogenomics. It is at the crossroads of research, post-university teaching and telemedicine. The Atlas is available at no cost.

The "Atlas of genetics and cytogenetics in oncology and haematology" on the internet and a review on infant leukemias.

The "Atlas of Genetics and Cytogenetics in Oncology and Haematology" (URL: http://www.infobiogen.fr/services/chromcancer) is a database devoted to chromosome abnormalities in cancer, cancer-prone diseases, and genes involved in cancer. The information is concise and updated. This database is made for and by cytogeneticists, molecular biologists, clinicians in oncology and hematology, and pathologists, who are encouraged to contribute. The database is herein presented, together with an example concerning congenital leukemias.

Atlas of genetics and cytogenetics in oncology and haematology, an interactive database.

The 'Atlas of Genetics and Cytogenetics in Oncology and Haematology' (http://www.infobiogen.fr/services/chromcancer ) is a database devoted to chromosome abnormalities in cancer, cancer-prone diseases and genes involved in cancer. Information presented in each page is concise and updated. This database is made for and by: cytogeneticists, molecular biologists, clinicians in oncology and in haematology, and pathologists, who are encouraged to contribute.

A database on cytogenetics in haematology and oncology.

The aim of 'Atlas of Genetics and Cytogenetics in Oncology and Haematology' (http://www.infobiogen.fr/services/chromcancer) is to present summarized information on chromosome abnormalities in cancer, with extensions to genes involved in cancer and to cancer-prone diseases. Information is to be updated. This database is made for and by cytogeneticists, molecular biologists, clinicians in oncology and in haematology, and pathologists.

[Communication between the left ventricle and the right atrium in infectious endocarditis. Diagnosis using Doppler-echocardiography]. / Communication entre le ventricule gauche et l'oreillette droite au cours d'une endocardite infectieuse. Diagnostic par l'échocardiogramme-doppler.

The diagnosis of a communication between the left ventricle and right atrium was made by transthoracic and transoesophageal echocardiography in a 67 year old man with a recurrence of a methicillin-resistant staphylococcus aureus infectious endocarditis complicating aortic valve replacement with a bioprosthesis seven weeks previously. This diagnosis was confirmed at surgery; the left ventricular-right atrial communication was closed by suturing its edges and a new aortic valve prosthesis was implanted. Unfortunately, the patient died 4 months later of myocardial dysfunction although the infectious endocarditis seemed to have been sterilised by antibiotic therapy. Doppler echocardiography, especially using the transoesophageal approach is the best diagnostic method for rare complications of infectious endocarditis, usually of the aortic valve, the prognosis of which is improved by early surgery and appropriate antibiotic therapy for the causal organisms.

The naevoid basal-cell carcinoma syndrome (Gorlin syndrome) is a chromosomal instability syndrome.

The Gorlin syndrome, or naevoid basal-cell carcinoma syndrome (NBCS) is an autosomal dominant cancer prone disease (at risk of multiple basal cell carcinomas, and other malignant or benign proliferations). We have previously reported data from peripheral blood lymphocytes of patients with this condition, showing a significant level of spontaneous chromatid and chromosome rearrangements and an overall lengthening of the cell cycle. In this paper, we confirm this disease to be a chromosome instability syndrome from studies on fibroblasts of 5 patients. Spontaneous chromosomal rearrangements, an increased frequency of sister chromatid exchanges and a slowing of the cell cycle were found, compared to age-matched control material. There was also an increased sensitivity to aberration production by mechlorethamine in patient fibroblasts. The chromosome instability we found was not restricted to a given cell lineage, but appears to be part of the general condition of this syndrome. The recently discovered gene responsible for Gorlin syndrome, PTC (or PTCH), encodes a transmembrane protein with yet poorly known functions. However, the demonstration of Gorlin syndrome as a chromosome instability syndrome suggests that this protein has a role in DNA maintenance, repair and/or replication.

[Value of mass dosage of the MB isoenzyme of creatinine phosphokinase in the diagnosis of recent myocardial infarction]. / Intérêt du dosage pondéral de l'iso-enzyme MB de la créatine phosphokinase pour le diagnostic de l'infarctus du myocarde récent.

In 391 patients admitted 3.7 hours (h) (median) after experiencing infarct-like pain, kinetic monitoring of CK-MB "mass" (threshold: 7 micrograms/l), myoglobin (threshold: 90 micrograms/l) and total CK (threshold: 290 micrograms/l) was carried out at the time of admission and after 1.5, 3, 6, 9, 12, 24 and 48 h. When myocardial infarction (MI) was treated conventionally (102 patients). CK-MB peaked 11 h (median) after the onset of pain, later than myoglobin (9 h), but before total CK (12 h). The peak of the markers was higher in Q+ than in Q-MI (p < 0.05). When MI was treated by thrombolytic medications (44 patients), the increases in CK-MB, myoglobin and total CK were larger, and occurred sooner (peaks 9, 6 and 6 h, after the onset of pain respectively), but did not last as long. In 245 patients who had not had MI (including 123 with spontaneous angina), the levels of the three markers remained stable and well below the decision thresholds. The sensitivities of CK-MB, myoglobin and total CK were respectively 47.1, 51.8 and 34.8% at the time of admission, 67.3, 82.7 and 57.1% after 3 h and 83.1, 76.9 and 88.9% after 6 h. The combined determination of CK-MB and of myoglobin had a higher sensitivity (67.7% at the time of admission, 84.9% after 1.5% and 88.2% after 3 h: but most of this gain was due to myoglobin. The specificity of the three markers and their diagnostic accuracy are comparable. In the course of recent MI, the kinetics of CK-MB mass are thus slower than those of myoglobin, but a little faster than those of total CK. The choice of the most effective biochemical marker depends upon the interval between onset of chest pain and hospitalization of the patient. Repetition of the determinations improves the diagnostic situation.

[Myocardial infarction in the elderly. Comparison between 2 groups of patients over 75 and under 65 years of age]. / L'infarctus myocardique du sujet âgé. Comparaison de deux groupes de malades âgés de 75 ans et plus et de 65 ans et moins.

To define the clinical characteristics, prognosis and treatment of myocardial infarction (MI) in the elderly, we retrospectively compared the files of 101 patients aged > or = 75 years (mean: 82 +/- 4 years) and of 120 others aged < or = 65 years (mean: 55 +/- 4.7 years). The figures corresponding to younger patients are presented in brackets. The elderly group included 60.4% women (5%: p < 0.001), 58.9% hypertensive subjects (38.3%: p = 0.005); 30.4% diabetics (11.7%: p = 0.0013) and 12.6% smokers (66.1%: p < 0.001); 20.8% of the elderly had a history of MI (10%: p = 0.002), 15.8% of arteriopathy of the lower limbs (8.3%: p = 0.001) and 6.9% of cerebrovascular accident (1.7%: p = 0.02). Elderly patients were admitted after an average of 26.6 hours (10.4 hours: p < 0.001). Only 56.4% (79.2%) reported typical MI pain, 22.8% (7.5%) had a painless form, 31.8% (4.2%) an initial left ventricular failure, 21.8% (7.5%) a global cardiac dysfunction and 20.8% (4.2%) a cardiogenic shock (p < 0.001 for all comparisons). 63.4% had an anterior MI (40.8%: p < 0.001), 40.6% a Q-form (29.6%: p = NS) and 22.2% an atrial fibrillation (0.8%: p < 0.001). Serum myoglobin and total CK concentrations were significantly lower in elderly subjects. 20.8% of them received beta-blockers (86.7%), 43.6% aspirin (80%), 14.6% oral anticoagulant (56.7%), but 63.4% were given diuretics (25.2%) and 31.7% digitalis alkaloids and positive inotropic drugs (6.7%) (p < 0.001 for all these comparisons). Heparin, nitrates, calcium channel blockers, ACE inhibitors and antiarrhythmics were prescribed as often regardless of age. Only 10 elderly patients (9.9%) were treated with thrombolytics (77: 65%: p < 0.001); 6 (5.9%) underwent coronary angiography (43: 35.8%: p < 0.001), 2 (2%) angioplasty (11: 9.2%) and one (1%) coronary bypass surgery (12: 10%). 35 elderly patients (34.7%) died while in hospital (5: 4.2%), 22 suddenly, 10 in cardiogenic shock and 3 due to arrhythmias. 38 cases (37.8%) of heart failure (21: 17.5%), 21 (20.8%) recurrences of coronary insufficiency (8: 6.7%) and 11 (10.9%) mechanical complications of MI (4: 3.3%) were also observed (p < 0.001 for all these comparisons). Due to lack of sufficient data, we could not define the status of the surviving patients discharged from hospital. The wider use of thrombolytics, angiography and angioplasty (coronary bypass surgery still having a heavy mortality and morbidity) is probably the best way to improve the prognosis of MI in the elderly.

[Ilio-femoral vein thrombosis treated with tissue plasminogen activator in a pregnant woman]. / Thrombose veineuse ilio-fémorale traitée par l'activateur tissulaire du plasminogène chez une femme enceinte.

A 27-year-old woman, after 31 weeks of amenorrhoea during her second pregnancy, developed a left external iliac and femoral deep vein thrombosis, confirmed by venous ultrasonography and magnetic resonance imaging. The infusion of tissue plasminogen activator (rt-PA: 1.2 mg/kg, i.e. 80 mg over 3 hours), on the 2nd day, allowed revascularization of the femoral junction, while the external iliac vein remained occluded. The patient did not develop pulmonary embolism or haemorrhage, particularly obstetric haemorrhage. The subsequent pregnancy was uneventful until delivery, six weeks later, of a normal child. Three years later, the patient has no sequelae of her deep vein thrombosis. When required by the patient's condition, it seems that rt-PA can be used to treat severe deep vein thrombosis during pregnancy, either isolated or complicated by pulmonary embolism. Very rigorous cardiological, obstetric and laboratory surveillance is essential. A sufficient dosage, identical to that used in clinical settings other than pregnancy and a brief treatment duration (2 to 3 hours) are probably more effective and more reliable than lower doses continued for several days. However, the risk of haemorrhage remains difficult to predict and its prognosis, especially foetal, is often very poor. A larger series of cases is therefore necessary before this drug can unreservedly recommended in pregnant women.

Down syndrome with partial duplication and del (21) syndrome: study protocol and call for collaboration. Study I: Clinical assessment.

We report on the clinical and cytogenetic assessment of five cases of Down syndrome phenotype with either a partial duplication of chromosome 21 or a normal karyotype, and we quote a case of del (21q) syndrome. Down syndromes with a partial duplication of chromosome 21 (as well as cases of del (21q), which are partly the phenotypic countertype of trisomy 21) are of paramount importance in the understanding of genes involved in the phenotype of Down syndrome. The goal is to find the relevant genes implicated in the main traits of Down syndrome (i.e. mental retardation, Alzheimer disease, and serious visceral malformations). Such a goal, in our opinion, cannot be reached just by publishing the genotype and the phenotype of a small cohort of patients: 1. a sufficient number of accurate cases is needed, and 2. data have to be computerized for definite conclusions to be reached. The main aims of this report are to present our study protocol and to invite colleagues to participate in a collaborative study in order to collect a maximum of these (rare) cases.

Evidence of chromosomal instability in the lymphocytes of Gorlin basal-cell carcinoma patients.

The Gorlin syndrome, or naevoid basal-cell carcinoma syndrome (NBCS) is an autosomal dominant disease. It has been suspected for long that this cancer prone disease (multiple basal-cell carcinomas; other malignant or benign proliferations) is a chromosome instability syndrome. We previously reported a lengthening in the cell cycle of lymphocytes from two patients with NBCS. With a larger sample (n = 7), we confirm this disease to be a chromosome instability syndrome, although clearly, expression of this characteristic can vary between patients: (1) spontaneous chromatid breaks occurred more often in a subset of the patients; (2) spontaneous micronuclei were found more frequently in NBCS than in the controls; (3) we confirm the cell cycle to be affected in this disease. As these results were obtained on lymphocytes--a cell lineage not affected in NBCS manifestations--the chromosome instability we found would appear to be part of the general condition of this syndrome.