[Medical, cognitive and affective status of four-year-old very premature babies without severe disability. A case-control study]. / Devenir médical, cognitif et affectif à l'âge de quatre ans des prématurés indemnes de handicap sévère. Etude prospective cas-témoins.

AIM: To determine cognitive performances and affective development of a cohort of very premature babies at the age of 4 years. POPULATION AND METHODS: Longitudinal case-control study. Newborns less than 33 weeks gestation were included from 1992 to 1994 and were matched with two full-term newborns. At 4 years of age, they have been evaluated for medical status, cognitive functions with the K-ABC test and affective condition with the PSA and Conners' tests. RESULTS: The preterm group was significantly different from the controls for: a smaller height, (P < 0.01), a lower socioeconomic level (P = 0.027), a lower nursery school level (44% versus 27,6%, P = 0.05), the incapacity to draw "a bonhomme" (55,3% versus 93%, P < 0.001), lower performances for the K-ABC and PSA tests, a pathologic Conners score (20.4% versus 2.4%, P < 0.001). Multivariate analysis by logistic regression showed a similar neuropsychological results: a lower score of K-ABC (< 80, P = 0.0006, odds-ratio = 33.2), and a lower social competence score of PSA (< 45, P = 0.004, odds-ratio = 35.9). CONCLUSION: Follow-up of premature babies is required, and measures to prevent or minimize cognitive and social disabilities are mandatory.

[Severe infections in children with sickle cell disease: clinical aspects and prevention]. / Infections graves chez l'enfant drépanocytaire: aspects cliniques et prévention.

Sickle-cell disease (SCD) is associated with frequent and often severe infections as a result of immune function impairment and functional asplenia. Also, infection can trigger a vasoocclusive crisis. Pneumonococcal bacteremia and meningitis due to S. pneumoniae are often lethal and justify the penicillin prophylaxis, which has provided a dramatic decrease in early mortality bacterial pneumonia is common in patients younger than four years, with most cases being due to S. pneumoniae, H. influenzae, Mycoplasma pneumoniae, Chlamydia pneumoniae. Acute chest syndrome is both a difficult differential diagnosis and a common concomitant of bacterial pneumonia, because they are often intricated. Osteomyelitis is generally due to Salmonella, most often S. enteritidis. Multiple foci are common and treatment is difficult, with some patients developing chronic osteomyelitis with sequestration. Osteomyelitis is less frequent in developed countries and must been differentiated with bone infarction by use of bone scintigraphy. Parvovirus B19 infection causes acute erythroblastopenias. Malaria does not result in cerebral malaria, but can lead to severe anaemia or vasoocclusive crisis, and should therefore be effectively prevented. Antimicrobials are generally selected for efficacy against pneumococci (septicemia, meningitis), Salmonella (osteomyelitis, meningitis), and M. pneumoniae (pneumonia). Prophylactic therapy is of paramount importance and relies on long-term or lifelong penicillin therapy started at three months of age and no closely-spaced immunizations, most notably against peumococci, hepatitis B virus, S. typhi and H. influenzae. Resistant pneumococcal strains have not been reported to cause prophylactic treatment failures. New conjugated pneumococcal vaccines are effective in protecting very young infants and should therefore be used in sickle cell patients.

[Sickle cell disease: from childhood to adolescence. Management in 2001]. / La drépanocytose: de l'enfant à l'adolescent. Prise en charge en 2001.

Sickle cell anaemia is a chronic disease which evolves on the background of a basal state punctuated by critical periods, often with complications requiring overall treatment. The management of both periods must be well integrated into the treatment programme. In order to treat occlusive crisis, medical staff must be well trained and equipped for the complicated clinical diagnoses involved in drepanocytosis: bone infarction or osteomyelitis, pneumonia or acute chest syndrome, multifocal crisis or severe infectious disease. Neonatal diagnosis is the best way for early treatment by preventing severe pneumococcal diseases, ensuring sufficient hydration and appropriate surveillance, as well as providing family education. Much progress has been made over the last decade in the early treatment of retinitis, biliary lithiasis, and aseptic hip necrosis. It is also important to ensure accurate prescriptions for hydroxyurea. Specialised centres for drepanocytosis treatment are now a growing necessity and should be encouraged in order to provide treatment for dispersed patients against a relatively little known disease.

[Diagnostic value of physical examination and electrocardiogram in the initial evaluation of heart murmurs in children]. / Valeur diagnostique de l'examen clinique et de l'électrocardiogramme dans l'évaluation initiale d'un souffle cardiaque de l'enfant.

OBJECTIVE: To investigate the diagnostic value of the physical examination and electrocardiogram in the evaluation of heart murmur in new patients referred to the pediatric cardiologist. METHOD: From 1 April to 30 September 1998, all consecutive patients referred to the pediatric cardiology clinic for evaluation of heart murmur were included. They were prospectively categorized with no heart disease, possible heart disease or definite heart disease based on history and physical examination. They then underwent electrocardiogram with which the diagnosis was reevaluated by the pediatric cardiologist. Lastly, a doppler-echocardiography was systematically performed. RESULTS: In 120 children aged four days to 14 years (median: ten months), 72 (60%) showed abnormalities on doppler-echocardiography and 48 (40%) no heart disease. After physical examination, 52 patients were categorized with no heart disease: 45 patients had a normal doppler-echocardiography; in three of them, the diagnosis was incorrectly modified to possible heart disease on the basis of the electrocardiogram. In the other seven children, the electrocardiogram was normal and the doppler-echocardiography revealed minor (n = 5) or moderate (n = 2) heart defects. Nineteen patients were suspected of having possible heart disease, no diagnosis was modified after analysis of the electrocardiogram and six had normal doppler-echocardiography; 49 patients were correctly diagnosed as having definite heart disease. The sensibility of the physical examination was 90.3%, the specificity was 93.8%, the positive predictive value 95.6% and the negative predictive value 86.5%. CONCLUSION: The electrocardiogram is of no help in the discrimination between heart disease and no heart disease in children referred to the pediatric cardiologist for a cardiac murmur. The physical examination is able to differentiate children with or without heart disease in most of the cases.