Damien Bonnet

Université René Descartes - Paris 5 · M3C Reference center for Complex Congenital Cardiac defects
a
a
a
a
39.04

Topics (9) View all

Publications (161) View all

  • Article: Home point-of-care international normalised ratio monitoring sustained by a non-selective educational program in children.
    [show abstract] [hide abstract]
    ABSTRACT: Adverse events related to vitamin K antagonist (VKA) therapy might be reduced by point-of-care international normalised ratio (POC INR) monitoring supported by an education program (EP). Our aim was to evaluate the efficacy of a non-selective VKA paediatric EP (regardless of the social, economic, educational or linguistic levels) by analysing the time spent in the therapeutic range (TTR), VKA adverse events and compliance to treatment, and INR control prescriptions. The EP was modified from the pediatric EP previously described but improved by a specifically devised child-focused game. One hundred four consecutive children (median age 8 years) receiving VKA were included in a standardised EP. Patients were in self-testing, and dose adjustments were made by a single physician for three tolerance ranges according to the underlying disease: [2.5-4], [1.8-3.2], and [1.5-2.5]. The median follow-up was 481 days [70-1,001]. The overall TTR was 81.4% [36-100]. The TTR were 74%, 85.6% and 89% for the ranges [2.5-4], [1.8-3.2], and [1.5-2.5], respectively. These results were sustainable during the study period. Only one serious VKA adverse event was recorded. The median number of POC INR tests was 2.5 [1.6-5.7] INR per patient and month. Patients/families performed POC INR when requested in 86.9% of the cases. More than 90% of the families found the EP supportive and wished to follow a long-term reinforcement program. In conclusion, this non-selective child-focused EP for VKA therapy, strongly supported by our dedicated game, is useful in maintaining efficacy, safety and compliance to anticoagulation and its monitoring.
    Thrombosis and Haemostasis 09/2012; 108(4):710-8. · 5.04 Impact Factor
  • Article: [Neonatal atrial tachycardia: suggestive clinical sign of Costello syndrome].
    D Laux, F Bajolle, A Maltret, D Bonnet
    [show abstract] [hide abstract]
    ABSTRACT: Costello syndrome is a rare association of symptoms caused by de novo germline mutations of the HRAS oncogene interfering in the RAS/mitogen-activated protein kinase (MAPK) signal transduction pathway. Mutations in this pathway are also responsible for Noonan syndrome and the related cardiofaciocutaneous syndrome (CFC) as well as LEOPARD syndrome. The 4 syndromes share phenotypic resemblances concerning patients' morphology but also regarding associated cardiac disease, namely hypertrophic cardiomyopathy, pulmonary stenosis, and atrial septal defect. The electrocardiogram often shows an upper deviation of the QRS axis. Arrhythmias are rare but, if present, are particularly typical of CS. We describe herein two newborn infants with Costello syndrome revealed by atrial tachycardia associated with characteristic morphological and cardiac features of syndromes related to mutations in the RAS/MAPK pathway.
    Archives de Pédiatrie 08/2011; 18(10):1087-9. · 0.30 Impact Factor
  • Article: Prenatal diagnosis of cardiac rhabdomyomas: incidence of associated cerebral lesions of tuberous sclerosis complex.
    [show abstract] [hide abstract]
    ABSTRACT: To determine the prevalence of specific cerebral lesions of tuberous sclerosis complex (TSC) and neurological outcome in cases diagnosed prenatally with cardiac rhabdomyomas. We reviewed all fetuses diagnosed prenatally with cardiac rhabdomyomas which had undergone detailed ultrasound evaluation and cerebral magnetic resonance imaging (MRI) and which were recorded in the database of a single institution covering the period January 1992 to December 2005. Fifty-one fetuses were included in the study. MRI was performed at a mean +/- SD gestational age of 30 +/- 3 gestational weeks and showed specific lesions of TSC in 49% of cases. Termination of pregnancy was chosen by the parents in 26 cases. Neurological development was studied in 20 cases, follow-up lasting 4.8 +/- 2.9 years. Neurodevelopmental events occurred during the follow-up period in 45% of cases. Neurological complications occurred in 67% of patients who had cerebral lesions at MRI and in 33% of patients with normal MRI results. There was no significant difference between the two groups of patients (P = 0.2). In fetuses with cardiac rhabdomyomas detailed ultrasound examination and third-trimester cerebral MRI are able to diagnose most TSC cerebral lesions, but fail to determine neurological outcome.
    Ultrasound in Obstetrics and Gynecology 09/2009; 34(2):155-9. · 3.01 Impact Factor
  • Article: Conotruncal heart defects in three patients with congenital disorder of glycosylation type Ia (CDG Ia).
    Journal of Medical Genetics 05/2009; 46(4):287-8. · 6.36 Impact Factor
  • Article: Coronary myocardial bridging in Noonan syndrome: definitive diagnosis with high-resolution CT.
    [show abstract] [hide abstract]
    ABSTRACT: Noonan syndrome is an autosomal dominant disorder reported at an incidence of 1 in 1000-2500 live-born patients. Pulmonary stenosis with a dysplastic pulmonary valve and hypertrophic cardiomyopathy are often associated with this syndrome. We report on a 9-year-old patient affected by Noonan syndrome with left ventricle hypertrophy, in whom a sudden clinical presentation of chest pain after effort led to the clinical suspicion of associated coronary myocardial bridging, which was confirmed by high-resolution CT. We also review the literature on this topic.
    The British journal of radiology 02/2009; 82(973):e8-10. · 2.11 Impact Factor

Following (19) See all

Followers (53) See all