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1.
Diabetes Metab ; 40(4): 292-8, 2014 Sep.
Article in English | MEDLINE | ID: mdl-24814978

ABSTRACT

AIM: Type 1 diabetes (T1D) involves complex metabolic disturbances in cardiomyocytes leading to morphological and functional abnormalities of the myocardium. The relationship between T1D and cardiac structure and function in children is not well established. Our study investigated whether T1D is associated with early subclinical myocardial disturbances in children and adolescents, and whether the state of metabolic control and diabetes duration are influential factors. METHODS: Standard echocardiography, tissue Doppler imaging (TDI) and two-dimensional (2D) strain imaging were prospectively performed in 100 T1D children (age: 11.3 ± 3.6 years, 52 boys) and compared with 79 controls. RESULTS: The diabetic and control children were comparable with respect to age, gender, heart rate and blood pressure. There were no significant differences between the two groups in left ventricular (LV) ejection fraction, LV remodelling and TDI parameters. Conventional mitral Doppler demonstrated significantly fewer diastolic filling abnormalities with an early filling wave in the diabetes group. Global longitudinal strain (GLS) was also significantly lower in the T1D children, while circumferential strain and radial strain did not differ. GLS correlated with HbA1c (r=0.52; P<0.01), but there was no correlation with diabetes duration. CONCLUSION: Our results suggest that LV longitudinal myocardial deformation is decreased in young patients with T1D, and glycaemic control may be the main risk factor for these changes. Further follow-up is now necessary to precisely determine the clinical significance of these myocardial changes detected by 2D strain imaging in T1D children.


Subject(s)
Diabetes Mellitus, Type 1/physiopathology , Ventricular Dysfunction, Left/diagnostic imaging , Ventricular Dysfunction, Left/physiopathology , Adolescent , Biomarkers/blood , Case-Control Studies , Child , Diabetes Mellitus, Type 1/blood , Echocardiography, Doppler , Female , Glycated Hemoglobin/metabolism , Humans , Male , Prospective Studies , ROC Curve , Reproducibility of Results , Ventricular Dysfunction, Left/blood
3.
Arch Pediatr ; 15(1): 45-9, 2008 Jan.
Article in French | MEDLINE | ID: mdl-18162380

ABSTRACT

Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder due to alpha-L-iduronidase deficiency. Its severe prognosis has been significantly improved by enzyme replacement therapy using recombinant human alpha-L-iduronidase (laronidase). We report the case of a boy who was diagnosed at 19 months of age with Hurler's disease, the most severe form of MPS I, and received thereafter a treatment by laronidase, resulting in clinical and biological improvement. The aim of this case report is to draw physicians' attention on the presenting signs of Hurler's disease, in order to enable an earlier diagnosis, increasing the treatment's benefits.


Subject(s)
Iduronidase/therapeutic use , Mucopolysaccharidosis I/drug therapy , Child , Diagnosis, Differential , Follow-Up Studies , Humans , Infant , Mucopolysaccharidosis I/diagnosis , Treatment Outcome
4.
Arch Pediatr ; 13 Suppl 1: S48-50, 2006 Oct.
Article in French | MEDLINE | ID: mdl-17370396

ABSTRACT

Long-term low dose azithromycin treatment in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection is safe and reduces the decline in lung function, the number of acute exacerbations and improves nutritional status; underlying efficacy mechanisms are multiple and synergistic.


Subject(s)
Anti-Bacterial Agents/therapeutic use , Azithromycin/therapeutic use , Cystic Fibrosis/complications , Pseudomonas Infections/drug therapy , Child , Humans , Pseudomonas Infections/etiology
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