Cystic kidney diseases in children.

Cystic kidney disease comprises a broad group of heterogeneous diseases, which differ greatly in age at onset, disease manifestation, systemic involvement, disease progression, and long-term prognosis. As our understanding of these diseases continues to evolve and new treatment strategies continue to emerge, correctly differentiating and diagnosing these diseases becomes increasingly important. In this review, we aim to highlight the key features of the most relevant cystic kidney diseases, underscore important diagnostic characteristics of each disease, and present specific management options if applicable.

[Autosomal dominant polycystic kidney disease : a pediatric perspective]. / Polykystose rénale autosomique dominante : perspective pédiatrique.

Polycystic kidney disease (PKD) is the most prevalent inherited kidney disease. The disease is usually asymptomatic until adulthood. End-stage renal disease occurs generally after the age of 55 years, with a large inter-individual variability. Renal cyst formation begins early in life, and animal models have shown that treatments able to prevent the cyst growth slow down the renal function decline. A treatment by tolvaptan is currently used in adults to decelerate PKD progression. Until now there is no consensus about the appropriate time to screen for PKD in children. However, these scientific progresses raise the interest of determining early (i.e. pediatric) predictive markers of renal function decline.

La polykystose rénale autosomique dominante (PRAD) est la maladie rénale génétique la plus fréquente. Le développement insidieusement progressif des kystes rénaux fait que la PRAD est, le plus souvent, asymptomatique jusqu'à l'âge adulte, mais la croissance kystique survient très précocement. L'insuffisance rénale terminale survient, généralement, après l'âge de 55 ans, avec, cependant, une grande variabilité interindividuelle. Les modèles animaux montrent que les traitements ralentissant la croissance du volume rénal freinent parallèlement le déclin de la fonction rénale. Par ailleurs, un traitement récemment utilisé chez l'adulte (le tolvaptan) permet de ralentir la progression de la PRAD. A ce jour, il n'y a pas de consensus sur l'âge de dépistage de la PRAD chez les enfants. Toutefois, les récentes avancées scientifiques suggèrent l'intérêt de déterminer des marqueurs prédictifs précoces, y compris pédiatriques, du déclin de la fonction rénale.

[How to explore...nosocomial infections in neonatology]. / Comment j'explore...Les infections nosocomiales en néonatologie.

Nosocomial infections (NI) remain a major problem of public health, giving rise to a serious morbi-mortality and significant costs. They represent a consequence of the medical progress and of the misuse of broad-spectrum antibiotics. Obviously, premature newborn of low birth weight present an increased risk of NI related to their degree of immaturity, as well as the invasive procedures use, and long-lasting hospitalizations.. Epidemiological data concerning NI in neonatology remain imprecise due to diagnostic difficulties. Responsible germs are related to the territory and its local ecology. Bacterial NI are the most frequent even if viral and Candida NI are widely underestimated and gradually gain ground.The medical and economic consequences of these infections justify prevention measures such as prematurity prevention and management of pregnancy taking into account the NI risk. Finally, simple measures such as a strict hygiene in newborn care and the limitation of invasive procedures in terms of frequency and duration have to be a priority.

[How to explore and treat ... a mucous otitis in the child]. / Comment j'explore ... une otite séromuqueuse chez l'enfant.

About nine children out of ten present at least one episode of otitis media with effusion (OME) before school age and near a third suffer from persistent or repetitive OME, which involves a higher risk of speech difficulties and of reduced learning abilities. The care of children with repeated or persistent OME remains a debated topic. The assessment of the risk of speech difficulties should go beyond the quantification of the hearing deficit. It should also take into account the context in which the disorder has developped and detect any comorbidity from which the child might suffer. Based on these various considerations, it will be necessary to assess the value of an immediate surgical treatment as opposed to the risks of a prolonged observation period.

[The Communauté française' eradication plan for measles, mumps and rubella]. / Le plan d'élimination de la Rougeole, de la Rubéole et des Oreillons de la Communauté francçaise.

The combined vaccine against measles, mumps and rubella provides an efficient tool for the elimination of these three diseases in a community. In the French Community of Belgium the MMR vaccine is commercially available since 1984 and was officially included in the immunization schedule in 1985. The present coverage rate is estimated at 60% of the children under 24 month. To improve the situation, the "Conseil Consultatif Communautaire de Prévention pour la Santé" of the French Community of Belgium has proposed a programme of MMR immunization promotion based on a coordinated mobilization of the various structures involved i.e. MCH organization, school medicine, general practitioners, pediatricians, Faculties of medicine and Health Administration. The programme has been adopted by the political authority and has started on February 1st 1989.

[Periarteritis nodosa in children. Diagnostic difficulties in 2 cases]. / Périartérite noueuse chez l'enfant. Difficultés diagnostiques dans 2 cas.

Periarteritis nodosa is a rare multisystemic disease whose diagnosis is difficult: diagnostic approach by biopsies in various involved organs is of poor efficacy; abdominal selective angiography yields a precise diagnosis in 50 to 60 of the studied cases as far as the celiac, mesenteric and renal circulations are investigated selectively. Digitalized intravenous angiography (DIVA) carried out in two patients of 9 and 13 years of age allowed to demonstrate aneurysm formations in hepatic, renal and cerebral circulations. Its true efficacy in the diagnosis of periarteritis nodosa of children remains to be determined.

Mortality in meningococcal disease in Belgium.

A survey of children admitted with meningococcal disease to 53 paediatric units in Belgium between 1975 and 1979 was made in order to assess the case mortality rate (CMR) and to identify risk factors associated with death. A total of 309 cases (226 bacteriologically confirmed and 83 unconfirmed) was recorded. The overall CMR was 6.1 per cent. It was 4.4 for bacteriologically confirmed cases and 10.8 for unconfirmed cases. The CMR was higher for septicaemia without meningitis (22.2 per cent) than for meningitis with or without signs of septicaemia (3.4 per cent). The risk of death was not related to the sex or nationality of the patients. Age was a major determinant of the CMR, independently of the clinical picture. The highest risk of death was in children under one year of age. Poor socio-economic conditions were a significant risk factor. Failure to recognise the severity of the disease by some poorly educated mothers, and the admission of the patient to a hospital lacking adequate facilities for managing severely affected children, were the two significant causes of delay of adequate treatment.

Meningococcal disease in Belgium. Secondary attack rate among household, day-care nursery and pre-elementary school contacts.

During a recrudescence of meningococcal disease mainly due to serogroup B, 1913 notified cases were investigated in Belgium from 1971 to 1976. From 1971 to 1973, 76 secondary cases were reported out of a total of 1455 cases (5.2 per cent); and from 1974 to 1976, nine secondary cases were reported of a total of 458 cases (2.0 per cent). Seventy per cent of the secondary cases occurred within seven days after the Index case. The 4.7 per cent fatality rate among secondary cases was lower than the 9.8 per cent fatality rate among primary cases. The estimated secondary attack rate was 685 per 100 000 among household contacts, 404 per 100 000 among day-care nursery contacts and 77 per 100 000 among pre-elementary school contacts. These attack rates were significantly higher (P less than 0.001) than the incidence rates in the corresponding age-groups in the general community, indicating the need for prophylaxis in these contacts.

[Pediatric home care services in Belgium]. / L'Hospitalisation pediatrique a domicile en belgique.

In 1970 a home care service for children was started by the committee for public assistance in Brussells with medical supervision from the department of paediatrics of the University. The main features are as follows: - It is exclusively paediatric and its aims are to shorten or avoid hospital admissions, or to provide medico-social support especially for the underpriveledged. - The medical supervision is undertaken by the family doctor (15% of cases) or by the physician in charge of the home care service with the help of specialists in the department of paediatrics in necessary. - Social workers, nurses and physiotherapists look after social problems, give nursing care and appropriate treatment. - The service has its own secretaries.

Concentration of gentamicin in bronchial secretions of children with cystic fibrosis of tracheostomy. (Comparison between the intramuscular route, the endotracheal instillation and aerosolization).

The levels of gentamicin in blood and in secretions of the tracheobronchial tree were measured in 14 children (8 with cystic fibrosis and 6 with tracheostomy) in a cross over fashion after the administration of 40 mg of gentamicin by aerosol or by endotracheal injection. High levels of gentamicin (greater than 20 mug/ml) within the bronchial secretions were observed in 7 children after aerosolization and in 11 children after endotracheal instillation. Corresponding blood levels were low (less than 3 mug/ml) in all patients and no detectable levels were found in 10 children after aerosolization and in 6 children after endotracheal instillation. No significant differences were observed between children with cystic fibrosis and those with tracheostomy. Since intramuscular injection of gentamicin (a single dose of 1.5 mg/kg) resulted in low levels of gentamicin within the bronchial secretions (less than 2 mug/ml in 10 patients, among whom 4 had undetectable levels); it is concluded that the administration of an antibiotic such as gentamicin, directly to the trachea by endotracheal injection or by aerosolization might prove to be helpful when the infection is confined mainly to the tracheo-bronchial tree.