Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study.

BACKGROUND: Pediatric ANCA vasculitis is a rare group of diseases with a scarcity of data in children. Annual incidence appeared to increase in the last several years, placing higher interest in the clinical and therapeutical outcomes of the disorder. Also, the growing use of rituximab questions the latest outcomes in these diseases. We therefore conducted a retrospective study to better understand the current characteristics, management, and the latest outcomes of the disorder. METHODS: We conducted a 9-year retrospective study of 46 children in 14 different centers across France to describe their clinical and laboratory presentations, therapeutic regimens, and kidney outcome. RESULTS: P-ANCA appeared to be a potential marker for higher relapse risk. Compared to adults, we found that ear-nose-throat presentations were frequent (45.7%) and more severe. Despite an evolution in the treatment management, kidney outcome remained poor with a substantial proportion of chronic kidney disease (54.8% at 1 year). Mortality stays low with 3 patients (6.5%) deceased at the end of our study. CONCLUSION: Clinical presentation was as previously described and time to diagnosis remains long. P-ANCA is a statistically significant marker for increased relapse risk. We observed a modification in the treatment regimens over the past several years with a growing use of rituximab and a decreasing use of cyclophosphamide. Despite these changes, kidney outcome remains poor and prospective studies should be conducted to assess the most appropriate therapeutic modality for each patient. A higher resolution version of the Graphical abstract is available as Supplementary information.

Hemodiafiltration: the addition of convective flow to hemodialysis.

Hemodiafiltration (HDF) combines both hemofiltration (HF) and hemodialysis in the same procedure. It was initially performed in adults in 1977, and later used in children in the early 1980s. The use of HDF allows a determined convective dialysis dose to be combined with the conventional urea dialysis dose. The dialysis session is better tolerated as a result of the effects of hemofiltration. On-line HDF, i.e., substitution fluid prepared from ultrafiltration of the ultrapure dialysate, can be performed safely due to recent advances in modern technology. However, despite interest and feasibility in children, the majority of pediatric dialysis units across the world still perform hemodialysis using highly permeable membranes, allowing back filtration in the filter and therefore a degree of convective flow, i.e., internal hemodiafiltration. In some countries, government restrictions prohibit the use of on-line hemodiafiltration, (such as the FDA recommendations in North America), and therefore it should not be used in these circumstances.

Dialysis strategies to improve growth in children with chronic kidney disease.

Despite major advances in the understanding and management of uremic growth failure, 35% to 50% of children with chronic kidney disease still grow up to become adults of small stature. The final adult height achieved is correlated with the height deficit recorded at the time of kidney transplantation. A degree of catch-up growth does occur after kidney transplantation in childhood, but it is often limited. Growth retardation in children with chronic kidney disease causes significant difficulties in their daily lives, often limiting psychosocial integration. Additionally, growth retardation is associated with a greater number of hospital admissions and an increased risk of mortality. Growth failure is the common endpoint of a variety of pathologies, including growth hormone resistance. In children on chronic dialysis, linear growth may be improved by ensuring that optimal clinical care is provided. This includes maximizing nutritional support (e.g., tube feeding in cases of anorexia) so as to prevent malnutrition. Further management options include the administration of recombinant human growth hormone (rhGH) treatment and the use of more frequent and intensive dialysis sessions, such as daily on-line hemodiafiltration, which combines increased dialysis convective flow with ultrapure dialysate, to limit cachexia.

Daily on line haemodiafiltration promotes catch-up growth in children on chronic dialysis.

BACKGROUND: In children, growth can be used as a measurable parameter of adequate nutrition and dialysis dose. Despite daily administration of recombinant human growth hormone (rhGH), growth retardation remains a frequent problem in children on chronic dialysis. Therefore, we performed an observational prospective non-randomized study of children on in-centre daily on line haemodiafiltration (D-OL-HDF) dialysis with the aim of promoting growth. PATIENTS AND METHODS: Mean age at the start of the study was 8 years and 3 months, and all children had been receiving rhGH treatment for >12 months before enrolment. Mean follow-up time on D-OL-HDF was 20.5 +/- 8 months (range, 11-39 months). Renal residual function was either <3 mL/min/1.73 m(2) or anuric. Vascular access was a fistula (13/15) or a central venous catheter (2/15). Dialysis was delivered daily, six days a week in 3 hourly sessions (18 h/week), in a predilution OL-HDF mode, allowing a high convective volume (18 to 27 L/m(2) body surface area per session), Kt/V(urea) on line measured at least 1.4 per session. RESULTS: Mean growth velocity increased from 3.8 +/- 1.1 cm/year at inclusion to 14.3 +/- 3.8 cm/year during the first year of D-OL-HDF, resulting in a change in height standard deviation score (SDS) over the follow-up period from -1.5 +/- 0.3 SDS to +0.2 +/- 1.1 SDS. Increase in body mass was also noted without impaired control of blood pressure. Time-average deviation for urea (TAD(urea)) was low at 2.5 +/- 0.4 as was TAD(bicarbonate) due to the normal pre and post dialysis bicarbonate levels, respectively, 23.6 +/- 0.5 mmol/L and 26.6 +/- 0.5 mmol/L. The absence of any dietary restrictions permitted a mean protein diet intake (PDI) of 2.5 +/- 0.2 g/kg/day (PDI measured from a 3-day diet survey), contrasting with a mean normalized protein nitrogen appearance (nPNA) of 1.53 +/- 0.12 g/kg/day (nPNA calculated from urea dialytic kinetic). A low C-reactive protein was noted in 13/15 children, and mean beta(2) microglobulin was low, 15.3 +/- 0.3.3 mg/L. CONCLUSIONS: Daily OL-HDF promotes catch-up growth in children despite on chronic dialysis. This catch-up growth if continued, should allow the children to reach their mid-parental target height in the future. It could be speculated that the improved response to rhGH is the result of several combined factors conducting to less malnutrition and to less cachexia.

Hemodialysis and nutritional status in children: malnutrition and cachexia.

Malnutrition is a common state in chronic hemodialyzed children. More than malnutrition, which infers that dietary supplementation would be curative, cachexia, which implies loss of protein stores, are combined factors of impaired linear growth and reduced muscle mass in uremic patients. Adequate diet to prevent malnutrition is of major importance. But to avoid cachexia in children on chronic hemodialysis, the management of acidosis, inflammation, abnormal metabolic rate, and endocrine disturbances should not, be forgotten. Daily hemodialysis regimen using convective flow therapy and ultrapure dialysate, i.e., on line hemodiafiltration together with growth hormone therapy, appears as a hopeful strategy for the chronic dialyzed child to achieve catch-up growth, a parameter of optimal nutrition.

[Children Lyme Borreliosis: retrospective study of 16 cases]. / Borréliose de Lyme chez l'enfant : étude rétrospective de 16 cas.

OBJECTIVES: Lyme Borreliosis exists in an endemic way for adult's in Alsace and the clinical demonstrations are well known. For children, there are no data in France. The objective of this study was to list the cases of Lyme Borreliosis seen in pediatric consultation in the Bas-Rhin and to study the clinical presentation. METHODS: We realized an investigation with 70 pediatricians of the Bas-Rhin between April 2006 and March 2008 which allowed us to count 16 cases of Lyme Borreliosis. RESULTS: It is about nine girls and seven boys, seven-years-old on average. Thirteen had an erythema migrans, two a neuroborreliosis and one arthritis of Lyme. Tick injection (notion established in 81 % of the cases) sat in upper limbs in the majority of the cases (39 %). The delay between the injection and the first signs was established in seven cases on 13 and varied from five to 30 days. The first signs of the disease appeared in spring or in summer for 81 % of the children. Amoxicilline was the the most used treatment for the erythema migrans; ceftriaxone for neuroborreliosis or Lyme arthritis. Those treatments were effective in every cases. CONCLUSION: Lyme Borreliosis in children exists in Alsace. Its main demonstration is the erythema migrans, but there are also neurological and articular demonstrations. With an appropriate treatment, its evolution is excellent.