A. J. Van Der Heijden

Bone marrow transplantation in children : consequences for renal function shortly after and 1 year post-BMT

The aim of this study was to investigate the effect of a bone marrow transplantation (BMT) on renal function in children. In a 5-year period, 142 children received a BMT at the Department of Pediatrics of the University Hospital Leiden. The study was performed retrospectively using the estimated glomerular filtration rate before and 1 year after BMT, and weekly measurements of serum creatinine during the first 3 months after BMT for assessment of renal function. Patient characteristics (sex, age, diagnosis), conditioning regimen, type of BMT, major complications (sepsis, veno-occlusive disease and graft-versus-host disease (GVHD)) and the use of nephrotoxic medication were listed. In the first 3 months after BMT 17 (12%) patients died, 13 from transplant-related complications other than renal failure and four from relapse of the disease. Forty-eight children (34%) had a period with acute renal insufficiency. A high pre-BMT serum creatinine, transplantation with either a non-HLA-identical related or a matched unrelated donor were risk factors for acute renal insufficiency after BMT. Sepsis and the use of intravenous vancomycin were risk factors for acute renal insufficiency only for patients with a high pre-BMT serum creatinine. GVHD seemed to have a beneficial effect on renal function of BMT recipients. One year after BMT a total of 35 (25%) patients had died, 16 from transplant-related complications and 19 from relapse of the disease; another 17 patients could not be evaluated. Twenty-five of 90 evaluable children (28%) had chronic renal insufficiency. Chronic renal insufficiency 1 year after BMT was correlated with a high serum creatinine in the first 3 months after BMT. None of the children of this retrospective study on renal function after BMT needed dialysis.

Long-term renal function after hemopoietic stem cell transplantation in children

Summary:Glomerular function of all long-term survivors who underwent hemopoietic stem cell transplantation (HSCT) from 1991 to 1998 (study I, n=121) was studied retrospectively. In addition, we prospectively analyzed glomerular and tubular function of all long-term surviving children who received an HSCT between 1998 and 2000 (study II, n=41). We found a lower prevalence of children with chronic renal failure (CRF) post-HSCT in our more recent cohort (study II: 10%) as compared to the older cohort (study I: 24%) 5.0 (0.7 s.d.) and 7.6 (2.4 s.d.) years post-HSCT, respectively. Furthermore, it seems that renal function may stabilize after 1-year post-HSCT. None of the patients required dialysis or antihypertensive medication at long-term follow-up. The sole predictor of CRF in our study was high serum creatinine pre-HSCT (P=0.007), while acute renal failure within 3 months after HSCT (P=0.08) only showed a trend towards predicting CRF. We could not confirm a relation of conditioning with irradiation with CRF post-HSCT, as was shown in several other pediatric and adult studies. Proximal and distal tubular dysfunction only occurred in a minority of long-time survivors of HSCT (3–12 and 9–13%, respectively) and had no clinical consequences.

Improvement of hepatitis B-associated glomerulonephritis after antiviral combination therapy

A 9-year-old boy with hepatitis B-associated glomerulonephritis and nephrotic syndrome underwent antiviral combination therapy including interferon and acyclovir. Pretreatment evaluation showed that active hepatitis B virus replication with HBsAg, HBeAg, HBV-DNA and DNA-polymerase had occurred for a period of at least 4 years. Signs of liver disease were minimal; serum amino transferases were normal and liver histology showed chronic persistent hepatitis with positive HBcAg, HBeAg and HBsAg immunofluorescence. A kidney biopsy revealed membranous glomerulonephritis with deposition of HBcAg, HBeAg, IgG, C3, C1q and, on electron microscopy, virus-like particles. After 8 weeks of therapy, active viral replication ceased, HBe seroconversion occurred and the nephrotic syndrome disappeared. One year after treatment, the boy was asymptomatic. No viral markers could be detected in the kidney, but low-grade membranous glomerulonephritis persisted with deposition of C1q, IgG and C3, but not HBeAg, HBsAg or HBcAg. Liver histology showed a minimal aspecific portal infiltrate with weak membrane-bound HBsAg immunofluorescence; no HBcAg could be detected. For patients with active viral replication and deposition of HBc, HBe immune complexes in the kidney, antiviral therapy can be beneficial, even in the absence of active liver disease.

Prevalentie van broek- en/of bedplassen bij kinderen met aandachtsproblemen

SummaryAim of the study was to assess the prevalence of daytime and/or night-time incontinence in children with a clinical score on Attention Problems. Of the population sample of 4970 children, 4480 participated (response 90.1%). Parents of 4171 children (2069 boys and 2102 girls) aged between 4 and 15 years filled out the Dutch version of the Child Behaviour Checklist (cbcl). Mean age was 9.3 years. For daytime and night-time incontinence, we used the items ‘daytime wetting’ and ‘bedwetting’ respectively of the cbcl. For Attention Problems we used the Attention Problems syndrome scale of the cbcl.The prevalence of daytime incontinence in this population-based study was 2.3%, of night-time incontinence 9.3% and of the combination of day- and night-time incontinence 1.2% (statistically significant higher in boys and in younger children). For children with a clinical score on the Attention Problems scale, parents reported daytime incontinence in 4.5% (2.4% of the boys and 8.0% of the girls), night-time incontinence in 12.1% (12.2% in boys and 12.0% in girls) and the combination of day- and night-time incontinence in 3.0% (0% of the boys and 8.0% of the girls). For children with a normal score, parents reported daytime incontinence in 2.2%, night-time incontinence in 9.2% and the combination of day- and night-time incontinence in 1.2%. The differences between children with a clinical score are not significant with the exception of girls with the combination of day- and night-time incontinence.There is no relation between day- or night-time incontinence separately and Attention Problems. These findings need to be confirmed in large studies with a clinical assessment of Attention-Deficit Hyperactivity Disorder.SamenvattingDoel van dit onderzoek was het bepalen van de prevalentie van broek- en/of bedplassen bij kinderen met aandachtsproblemen. Uit een populatieonderzoek onder 4970 kinderen namen 4480 kinderen deel (respons 90,1%). Ouders van 4171 kinderen (2069 jongens en 2102 meisjes) tussen de 4 en 15 jaar vulden de Nederlandse versie van de Child Behaviour Checklist (cbcl) in. De gemiddelde leeftijd was 9,3 jaar. We gebruikten de items ‘broekplassen’ en ‘bedplassen’ uit de cbcl. Voor aandachtsproblemen gebruikten we de syndroomschaal aandachtsproblemen van de cbcl.De prevalentie van broekplassen in dit populatieonderzoek was gemiddeld 2,3%, van bedplassen 9,3% en van de combinatie van broek- en bedplassen 1,2% (statistisch significant hoger bij jongens en bij jongere kinderen). Bij kinderen met een klinische score op de syndroomschaal aandachtsproblemen rapporteerden ouders broekplassen bij 4,5% van de kinderen (2,4% van de jongens en 8,0% van de meisjes), bedplassen bij 12,1% (12,2% van de jongens en 12,0% van de meisjes) en de combinatie van broek- en bedplassen bij 3,0% (0% van de jongens en 8,0% van de meisjes). Bij kinderen met een normale score had 2,2% broekplassen, 9,2% bedplassen en 1,2% de combinatie van broek- en bedplassen. Het verschil met kinderen met een klinische score is niet significant, met uitzondering voor meisje met de combinatie van broek- en bedplassen.Voor het vaak veronderstelde verband tussen adhd en broek- en/of bedplassen wordt in deze populatiestudie geen aanwijzing gevonden, met uitzondering van een kleine relatie bij meisjes met de combinatie van broek- en bedplassen. Deze resultaten dienen bevestigd te worden in grotere onderzoeken met een klinische diagnose van aandachtstekort en hyperactiviteitsstoornis (adhd).

Nefritis bij purpura van Henoch-Schönlein: een update

SummaryHenoch-Schönlein purpura nephritis (hspn) is one of the most frequent forms of glomerulonephritis in childhood and may lead to chronic renal insufficiency. The prognosis depends on the initial renal symptoms, the histological findings in the renal biopsy, the intensity of abdominal involvement, the age of the patient and the presence of a low factor XIII plasma activity. Important pathogenetic factors are a decrease in IgA mucosal response leading to increased antigenic load Regular with abnormal IgA glycosylation. hspn and IgA nephropathy are related diseases with biological and histological analogies, but also with striking differences. There is no consensus about therapy, although positive effects of Regular immunosuppressive therapy have been described. This article summarises the present knowledge on hspn.SamenvattingDe nefritis bij de purpura van Henoch- Schönlein (hspn) is één van de meest voorkomende vormen van glomerulonefritis bij kinderen en kan leiden tot chronische nierinsufficiëntie. De prognose hangt af van de initiële renale presentatie, de gevonden afwijkingen bij nierbiopsie, de intensiteit van de abdominale symptomen, de leeftijd waarop de ziekte optreedt en de aanwezigheid van een lage plasma-activiteit van factor XIII. Belangrijke pathogenetische factoren zijn verminderde mucosale respons van IgA, leidend tot verhoogde antigeenpenetratie en afwijkende IgA-glycosylering. hspn en IgA-nefropathie zijn gerelateerde ziekten met biologische en histologische overeenkomsten, maar ook met opmerkelijke verschillen. Er bestaat geen consensus over de behandeling van hspn, hoewel positieve effecten van gecombineerde immunosuppressie zijn gerapporteerd. In dit artikel wordt een overzicht gegeven van de huidige kennis over hspn.

Acute nierinsufficiëntie en nierfunctievervangende therapie na cardiopulmonale bypasschirurgie bij kinderen

SummaryIn a retrospective study renal function and renal replacement therapy after cardiopulmonary bypass surgery in children was investigated at the Department of Cardiothoracic Surgery at the Leiden University Medical Center and Academic Medical Center of Amsterdam. Patient characteristics (sex, age, diagnosis), operation type and death were listed. Serum creatinine level before and peak values after cardiopulmonary bypass surgery were used for assessment of renal function. For the children on renal replacement therapy, indication, efficacy, and complications were recorded. In a 5-year period (1994-1999), 1075 children had cardiopulmonary bypass surgery. Of the patients 180 (17%) developed acute renal insufficiency. Twenty-five (2,3%) patients required renal replacement therapy. Peritoneal dialysis is a safe and effective treatment for children post cardiopulmonary bypass surgery. However, 15 (60%) of the 25 children on renal replacement therapy died of non-renal causes. In 9 of the 10 surviving children renal function was normal at the time of discharge. In conclusion, acute renal insufficiency is a frequent complication after open-heart surgery, although renal replacement therapy was infrequently necessary. Peritoneal dialysis is a safe and effective therapeutic measure for children after cardiopulmonary bypass surgery.SamenvattingIn een retrospectieve studie werden het vóórkomen van acute nierinsufficiëntie en nierfunctievervangende therapie bij kinderen na cardiopulmonale bypasschirurgie onderzocht in het Centrum voor Aangeboren Hartafwijkingen Amsterdam/Leiden. Klinische gegevens van de patiënten (geslacht, leeftijd, diagnose), type cardiochirurgische interventie en mortaliteit werden vastgelegd. De nierfunctie werd geëvalueerd aan de hand van de serumcreatinineconcentratie preoperatief en de maximale serumcreatinineconcentratie postoperatief. Van de kinderen die nierfunctievervangende therapie ondergingen, werden indicatie, efficiëntie en complicaties bestudeerd. In een vijfjaarsperiode (1994-1999) ondergingen 1075 kinderen cardiopulmonale bypasschirurgie. Van de kinderen ontwikkelden 180 (17%) een acute nierinsufficiëntie. Vijfentwintig (2,3%) kinderen hadden nierfunctievervangende therapie nodig. Van de 25 kinderen met nierfunctievervangende therapie zijn 15 (60%) kinderen overleden aan niet-renale oorzaken. Negen van de tien overlevende kinderen met nierfunctievervangende therapie hadden een normale nierfunctie bij ontslag. Concluderend:acute nierinsufficiëntie is een frequente complicatie na cardiopulmonale bypasschirurgie, maar nierfunctievervangende therapie is zelden nodig. Peritoneale dialyse is een veilige en effectieve behandeling bij kinderen na cardiopulmonale bypasschirurgie.