Autosomal Dominant Polycystic Kidney Disease in Children and Adolescents: Assessing and Managing Risk of Progression.

The clinical management of autosomal dominant polycystic kidney disease (ADPKD) in adults has shifted from managing complications to delaying disease progression through newly emerging therapies. Regarding pediatric management of the disease, there are still specific hurdles related to the management of children and adolescents with ADPKD and, unlike adults, there are no specific therapies for pediatric ADPKD or stratification models to identify children and young adults at risk of rapid decline in kidney function. Therefore, early identification and management of factors that may modify disease progression, such as hypertension and obesity, are of most importance for young children with ADPKD.

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.

Low agreement between various eGFR formulae in pediatric and young adult ADPKD patients.

Background: Young autosomal dominant polycystic kidney disease (ADPKD) patients are becoming the new target population for the development of new treatment options. Determination of a reliable equation for estimated glomerular filtration rate (eGFR) from early stages is needed with the promising potential interventional therapies.

Methods: Prospective and longitudinal study on a cohort of 68 genotyped ADPKD patients (age range 0-23 years) with long-term follow-up.

Risk Severity Model for Pediatric Autosomal Dominant Polycystic Kidney Disease Using 3D Ultrasound Volumetry.

Background: Height-adjusted total kidney volume (htTKV) measured by imaging defined as Mayo Imaging Class (MIC) is a validated prognostic measure for autosomal dominant polycystic kidney disease (ADPKD) in adults to predict and stratify disease progression. However, no stratification tool is currently available in pediatric ADPKD. Because magnetic resonance imaging and computed tomography in children are difficult, we propose a novel 3D ultrasound-based pediatric Leuven Imaging Classification to complement the MIC.

Is autosomal dominant polycystic kidney disease an early sweet disease?

The clinical course of autosomal dominant polycystic kidney disease (ADPKD) starts in childhood. Evidence of the beneficial impact of early nephron-protective strategies and lifestyle modifications on ADPKD prognosis is accumulating. Recent studies have described the association of overweight and obesity with rapid disease progression in adults with ADPKD.

[Autosomal dominant polycystic kidney disease : a pediatric perspective].

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.

Long-term outcomes of peritoneal dialysis started in infants below 6 months of age: An experience from two tertiary centres.

Background: Little data are available for infants who started renal replacement therapy before 6 months of age. Because of extra-renal comorbidities and uncertain outcomes, whether renal replacement therapy in neonates is justified remains debatable.

Methods: We performed a retrospective analysis of all patients who began chronic peritoneal dialysis below 6 months between 2007 and 2017 in two tertiary centres.

In-depth phenotyping of a Donnai-Barrow patient helps clarify proximal tubule dysfunction.

Background: The megalin/cubilin/amnionless complex is essential for albumin and low molecular weight (LMW) protein reabsorption by renal proximal tubules (PT). Mutations of the LRP2 gene encoding megalin cause autosomal recessive Donnai-Barrow/facio-oculo-acoustico-renal syndrome (DB/FOAR), which is characterized by LMW proteinuria. The pathophysiology of DB/FOAR-associated PT dysfunction remains unclear.

[How to explore...nosocomial infections in neonatology].

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.

[How to explore and treat ... a mucous otitis in the child].

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.

[The Communauté française' eradication plan for measles, mumps and rubella].

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.

[Periarteritis nodosa in children. Diagnostic difficulties in 2 cases].

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.

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.

[Pediatric home care services in Belgium].

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.