Résumé
BACKGROUND AND PURPOSE: Sickle cell disease (SCD) is strongly linked to stroke across all haplotypes in the pediatric population. Transcranial Doppler (TCD) ultrasound is known to identify the highest risk group in African-Americans who need to receive and stay on blood transfusions, but it is unclear if the same flow velocity cut-offs can be applied to the Iranian population. We aimed to evaluate baseline TCD findings in Iranian children with SCD and no prior strokes. METHODS: Children with genetically confirmed SCD (Arabian haplotype, homozygote) and without SCD (controls) were prospectively recruited from pediatric outpatient clinic over a period of 9 months. We performed TCD in both groups to determine flow velocities in the middle cerebral (MCA) and terminal internal carotid arteries (TICA). RESULTS: Of 74 screened children, 60 met the inclusion/exclusion criteria (62% female; mean age 10+/-4 years). Baseline characteristics did not differ between the cases and controls, except hemoglobin (Hb) which was significantly lower in the SCD group (P<0.001). The right MCA TAMM (Time Averaged Maximum Mean) was significantly higher than in controls (125+5.52 cm/s vs. 92.5+1.63 cm/s, P<0.001). Left MCA did not show differences. The TICA TAMM was also different between cases and controls (P<0.05). CONCLUSIONS: Among Iranian children with asymptomatic SCD and without receiving recent transfusion TCD velocities are higher as compared to healthy controls but appear much lower than those observed in STOP (Stroke Prevention Trial in Sickle Cell Anemia) studies. We hypothesize that some children at high risk may be present with velocities lower than 170-200 cm/s thresholds. A prospective validation of ethnicity-specific prognostic criteria is warranted.
Sujets)
Enfant , Femelle , Humains , Établissements de soins ambulatoires , Drépanocytose , Transfusion sanguine , Artère carotide interne , Haplotypes , Études prospectives , Accident vasculaire cérébral , ÉchographieRésumé
Carotid ultrasound appears to be useful in the assessment of cardiovascular risk. In this study, we have assessed the carotid intima-media thickness [CIMT] in a group of individuals without a history of cardiovascular events. A sample of 431subjects [189 [43.9%] males and 242 [56.1%] females] was obtained from an urban population using a stratified-cluster method in Mashhad stroke and heart atherosclerosis disorder study. None of the subjects had a history of the cardiovascular event. Carotid artery duplex ultrasound was used to determine the CIMT in all subjects, and to identify those with an abnormal value [CIMT [+]; i.e., CIMT >/= 0.8 mm]. Dietary intake of participants was assessed using a questionnaire for 24-h dietary recall. The relationship between anthropometric, biochemical and dietary data and CIMT were assessed. The mean age of subjects was 48.7 +/- 8.0 years. Of the 431 patients, 118 [27.4%] were found to be CIMT [+]. Of the cardiovascular parameters assessed, only age [odds ratio [OR] [95% confidence interval [CI]], 1.11 [0.56-4.34]; P < 0.01] and male gender [OR [95% CI], 1.14 [0.63-2.23]; P < 0.05] were significant independent predictors of ultrasound defined CIMT. Crude and total energy adjusted intake were not associated with the presence of CIMT [+]. It appears that within a relatively young Iranian population of individuals without a history of cardiovascular event, the presence of CIMT [+] defined by duplex ultrasound cut-off value of >/= 0.8 mm, did not associate with several modifiable cardiovascular risk factors or measures of dietary intake
Résumé
Muslim women tend to postpone their menstrual period using short course oral contraceptives [OC] during Ramadan, Muslims fasting month. Recently, many cases of stroke, particularly cerebral vein thrombosis [CVT], have been reported in Ramadan. This work studies the incidence of CVT and its relation to Ramadan. This is a cohort study conducted from October 2006 to October 2009 and included 4 consecutive Ramadan's. All patients with definite diagnosis of CVT who were referred to the neurology department of Ghaem Hospital, Mashhad, Iran in this four-year period were included in the study. During the study period, 70 cases with CVT [11 males and 59 females] were recruited. Twenty five cases were admitted during Ramadan months which was significantly higher than all the other 32 months [p=1.9×10[-7]]. The higher frequency of females [6 times more] among CVT cases probed by investigating possible risk factors and short term OC consumption was revealed as the major risk factor [p=0.00071]. Higher incidence of CVT in females during Ramadan suggests the presence of specific risk factors in this group. Our study revealed that short-term use of OCs may be a major risk factor for CVT
Résumé
Mobius Syndrome is rare, heterogeneous and nonprogressive congentital disorder that is mostly seen sporadically and has been reported few cases. The principle clinical features of the syndrome are bilateral abducens paresis with different degree, incomplete and bilateral facal nerve paresis, orofacial and limbs anomalies
The main cause of clinical syndrome is agenesis of cranial nerve nucleus in the ponse
Our patient is an 18-Year old man with history of poor sucking, lack of impressions during crawing and bilateral club foot at birth time. His parents noticed disorder of eye movements in the infancy period, these findings had stable, nonprogressive course
Sign that have been found in the clinical assessment are:
Bilateral, peripheral Type facial never paresis more on the left, bilateral club foot, complete inability of conjugated gaze to the right convegence of eyes ant left gaze, divergence of eyes at upward gaze and Goiter of grade two. Paraclinical studies such as brain MRI, Echocardiography, ECG, Liver and kideny fuction tests, thyroid hormones and audiometery were normal. NCV Showed axonal ytpe neuopathy of both facial nerves