Ultrasound arterial anomalies in patients exposed to nilotinib therapy for chronic myeloid leukemia.

INTRODUCTION: Patients exposed to nilotinib for chronic myeloid leukemia (CML) appear to be at risk of arterial complication. The prevalence and aspect of ultrasound asymptomatic arterial lesions are unknown. OBJECTIVE: To describe prevalence and characteristics of ultrasound arterial anomalies in patients treated with nilotinib for CML. METHODS: Patients treated with nilotinib from 2006 to 2015 in the department of the Paoli-Calmettes Institute, Marseille, were included retrospectively. A vascular ultrasound screening was carried out from 2010. The arterial lesions at the first examination were described: plaque and its echogenicity, stenosis or occlusion. A vascular arterial anomaly (VAA) was defined by the presence of a clinical and/or ultrasound anomaly. Patients with or without VAA at initial vascular examination were compared using bivariate and multivariate analysis. RESULTS: 74 patients were included (51.4% men, mean age 54.5 years); 25 patients had ultrasound arterial anomalies (33.8%). Carotid bulb was the most involved territory (44%). Arterial anomalies were: 88% plaques, 44%>50% stenosis and 12% occlusion. 72.7% plaques were echolucent or hypoechogenic. A VAA was present in 25 patients with initial vascular evaluation (33.8%). Patients with VAA at baseline were significantly older (64.9 vs 49.3, P<0.001), older at nilotinib initiation (60.8 vs 46.5, P<0.001), with more arterial hypertension (40% vs 12.2%, P=0.01), with more cardiovascular risk factors (P=0.03). In patient with no cardiovascular risk factor 12.5% had VAA (n=24). CONCLUSION: Nilotinib seems to be associated to arterial lesions of unstable lipid-like appearance. The most involved arterial territory was the carotid bulb and the most common lesion was echolucent or hypoechogenic plaque. VAA can occur in patients without cardiovascular risk factors. This result encourages us to systematically screen and follow all patients exposed to nilotinib even those without cardiovascular risk factors.

[Revascularisation of unprotected left main coronary artery disease: an update]. / Revascularisation du tronc commun coronaire gauche non protégé.

Coronary bypass grafting is the reference treatment of unprotected left main coronary disease. Nevertheless, the experience of invasive cardiologists and the introduction of active stents make angioplasty possible in selected cases. Only the results of controlled clinical trials (SYNTAX trial currently under way) will enable physicians to choose the most appropriate method of revascularisation for their patients.

Recent studies on oculopharyngeal muscular dystrophy in Québec.

In 1990, we launched a major study to ascertain the clinical picture of OPMD in Québec and to identify large families for linkage analysis. In 14 patients, the chromosomes were karyotyped to eliminate any deletion or translocation. Relevant family information and clinical data were computerized and correlations were sought for the age of onset, the identification of the first symptom and the distribution of weakness. A simple test to detect dysphagia was validated. Twenty-one families have taken part in the study, which led to our localization of the gene in 1995 [Brais B, Xie Y-G, Sanson M, et al. Hum Mol Genet 1995; 4:429-434]. At least one case in each family underwent muscle biopsy to confirm the presence of the typical nuclear filaments found in OPMD. Electrodiagnostic and pathologic studies were also conducted to better understand the disease process. An illustrative case is presented.

Upper limb pain in chronic demyelinating polyneuropathy: electrophysiological correlates.

Pain is not usually considered a symptom of chronic relapsing demyelinating polyneuropathy. We report two patients with chronic demyelinating polyneuropathies in whom clinical and electrophysiological worsening was associated with the development of deep and distressing upper limb aching discomfort. One patient had a MGUS-associated hypertrophic demyelinating and prednisone-dependent polyneuropathy, followed over a course of two and a half years. His discomfort regularly predicted electrophysiological relapse of neuropathy, before more obvious clinical signs had emerged. Resolution of the discomfort also predicted subsequent clinical and electrophysiological improvement. Upper limb pain may be an important feature of early relapse in some patients with demyelinating polyneuropathies. Standardized serial electrophysiological testing in patients with chronic demyelinating polyneuropathy can be an important management tool in conjunction with clinical signs and symptoms.

Arterial isoflurane concentration and EEG burst suppression during cardiopulmonary bypass.

Isoflurane (1.5 to 3.0 vol% in oxygen) was used to control intraoperative hypertension in 10 patients undergoing hypothermic cardiopulmonary bypass surgery. Isoflurane was administered through the membrane oxygenator of the bypass pump and yielded plateau concentrations in arterial blood ranging from 36.6 to 84.4 micrograms/ml (0.5 and 1.16 vol%, respectively). Isoflurane dosing resulted in prolonged periods (21 to 63 minutes) of EEG burst suppression and isoelectric activity in nine patients. Burst suppression was not a result of hypothermia. There was a close temporal relationship between isoflurane concentration and the onset of burst suppression (mean onset time: 27.3 +/- 4.56 minutes after isoflurane begun). The mean arterial isoflurane concentration at the onset of burst suppression was 46.5 +/- 10.7 micrograms/ml; the nasopharyngeal temperature was 26.0 degrees +/- 0.61 degrees C. Isoflurane was eliminated rapidly from blood with a mean apparent t1/2 of 18.8 +/- 5.46 minutes.

[Long-term course after palliative surgery in children with a single ventricle]. / Evolution à long terme après chirurgie palliative des enfants atteints de ventricule unique.

Single ventricle or univentricular heart is a rare congenital malformation (1/10 000) which has a very poor prognosis. The cases of 68 children with single ventricles who had palliative surgery between January 1964 and December 1982 were studied. The average age of the patients at surgery was 4.41 +/- 5.8 years; 36% of the children were under 1 year of age. Surgery consisted in 48 systemico-pulmonary anastomoses and 20 pulmonary artery bandings. The global mortality was 22% (16% in the anastomosis group and 35% in the banding group). Age seemed to be an important factor: 25% mortality in children under the age of 1; 2.4% in children over 1 year (p less than 0.05). The 15 year survival rate was 43 +/- 23% overall; 56 +/- 28% in children with anastomoses and 20 +/- 28% at 8 years in children with banding. Although surgery did not appear to improve survival it probably did improve survival it probably did improve the quality of life; 73% of the survivors has satisfactory functional results and the efforts of everyday life were well tolerated. However, the evolution was complicated in 10% of cases by infection and neurological complications, and atrioventricular regurgitation was observed in 7 patients, requiring valve replacement in 2 cases. Our results are globally comparable with those of similar previously reported studies. The best published results of physiological correction of single ventricle (Fontan, septation) are relatively unsatisfactory with a mortality rate of about 35%. Therefore, palliative surgery still appears justified in these cases.

[Compression of the cervical spinal cord in achondroplasia]. / Compression de la moelle cervicale dans l'achondroplasie.

Two cases of tetraparesis due to cervico-occipital spinal cord compression in infants with achondroplasia are reported. Neurological disorders in achondroplasia are produced by structural anomalies of the cranium and spinal canal. They can be divided into two categories: hydrocephalus and radicular and spinal cord compression syndromes. Cervico-occipital compression is more frequent in childhood and may occur very early, in the first months of life. Early diagnosis of this complication and anatomic evaluation can now be obtained by computed tomography, which shows the exact dimensions of the foramen magnum, and myelography. Achondroplasia requires very close neurologic monitoring and, when findings are abnormal, radiologic investigations of the cervico-occipital zone. This allows for early surgical management which is the only means of obtaining significant therapeutic results.

Psoriatic arthritis: risk factors for patients with psoriasis - a study based on histocompatibility antigen frequencies.

Histocompatibility antigen frequencies were studied in a group of 100 patients with psoriatic arthritis (PSA) and were compared to a group of 80 patients whose psoriasis was restricted to skin lesions (PSC). The antigens B13, BW57 (17), CW6 were significantly increased in PSC while BW57 (17), BW39, CW6 and CW7 were increased in PSA. No DR or MT antigen was elevated in frequency when compared to normal controls. The significant information which results from this study includes: 1) a failure to confirm previous reports on HLA-DR antigen increased frequencies; 2) an association, in this population, of BW39 with PSA and not BW38; 3) finding of a closer link of PSC and PSA with HLA-C rather than HLA-B antigens; 4) an estimate of relative risk for patients with PSC to develop PSA.

[Azygography in pre-operative investigation of cancer of the esophagus : a report on 24 cases (author's transl)]. / L'azygographie : sa place dans le bilan pré-opératoire du cancer de l'oesophage. A propos de 24 cas.

The value of azygography for detecting extension of cancer of the middle third of the esophagus was assessed in 24 patients. The examination was conducted using the retrograde approach, and results were compared with those obtained by the esophagogram and on tracheobronchial fibroscopy. Compression of the azygos vein is a good indicator of the size of the tumor, which can also be evaluated from esophageal transit examinations. The vein can be invaded or even thrombosed due to severe regional spread but this does not mean that excision of the tumor is impossible. The results of azygography alone, therefore, are not a valid reason for contra-indicating esophagectomy. In case of doubt, tracheobronchial fibroscopy appears to be more suitable for deciding whether the tumor is operable, but azygography can assist in making this decision.