Valerie Langlois

Eculizumab and Refractory Membranoproliferative Glomerulonephritis

This letter to the editor discusses the use of eculizumab in refractory membranoproliferative glomerulonephritis. The response appears to be dramatic.

Child and parental perspectives of multidimensional quality of life outcomes after kidney transplantation

Anthony SJ, Hebert D, Todd L, Korus M, Langlois V, Pool R, Robinson LA, Williams A, Pollock‐BarZiv SM. Child and parental perspectives of multidimensional quality of life outcomes after kidney transplantation.u2028Pediatr Transplantation 2010:14:249–256.

The prevalence of BK viremia and urinary viral shedding in a pediatric renal transplant population: A single‐center retrospective analysis

Abstract:u2002 Polyomavirus‐induced nephropathy has emerged as an important cause of renal graft dysfunction. Limited pediatric data are available for this disease. We therefore reviewed the results of the first year of polyomavirus screening in our pediatric renal transplant recipients to determine the prevalence of polyomavirus viremia and urinary shedding. Screening included detection of polyomavirus in plasma by polymerase chain reaction (PCR) and in urine by electron microscopy (EM). In patients with a positive screening test, an assessment of graft dysfunction was made. Fifty‐two patients met the inclusion criteria. Urinary EM was performed in 205 samples and polyomavirus was detected in 10 patients, representing 19% of the study population. PCR was performed on 222 samples and was positive for the BK virus in plasma from seven patients or 13.4% of the study population. Eight patients had a positive screening test and increased creatinine. All these patients underwent renal transplant biopsy. This revealed evidence of polyomavirus nephropathy in four patients. Our findings reveal a high prevalence of polyomavirus in both urine and plasma that is frequently associated with graft dysfunction. These findings support the routine screening of pediatric post‐renal transplant patients for polyomavirus replication.

Hemolytic uremic syndrome caused by Bordetella pertussis infection

We report the case of a 4-week-old infant with severe Bordetella pertussis infection resulting in hemolytic anemia, thrombocytopenia, and acute renal failure leading to a diagnosis of hemolytic uremic syndrome (HUS) associated with pertussis. In addition to antibiotic and supportive therapy, he was treated with plasma transfusions based on the possibility of underlying complement defect, and he improved. The association of B. pertussis infection and HUS has previously been described in a patient with a mutation in the gene encoding complement factor H (CFH). However, whereas a genetic workup for complement regulator mutations was performed, no mutation was found in our patient. This case demonstrates the possible association between pertussis infection and HUS and highlights the need for increased vigilance for renal complications in this diagnosis. Despite negative results in this case, in-depth workup of the complement system may be important to guide treatment efforts and strategies.

Outpatient pediatric renal transplant biopsy – Is it safe?

Abstract:u2002 The safe observation time following pediatric renal transplant biopsy is unknown. To predict the safety of day‐care pediatric renal transplant biopsy, we retrospectively evaluated the timing, incidence and severity of post‐biopsy complications in children observed overnight. Biopsies were performed under real time ultrasound guidance using an 18‐gauge Bard Biopty needle. Coagulation screen and platelet counts were measured preprocedure. Hemoglobin (Hb) was measured preprocedure, at 6u2003h and at 1‐day post‐procedure. Twenty‐eight of 45 children transplanted between January 2002 and May 2004 underwent 65 biopsies. There was gross hematuria following 8 (12%) biopsies; 2/8 occurred after 6u2003h. Hb fell by >15u2003g/L in six cases (9%) – three had Hb drop within 6u2003h post‐procedure and three had a steady decline over 24u2003h. No patient required blood transfusion. Oral analgesia post‐procedure was required in seven cases (11%). One of these had gross hematuria. No patient required surgical intervention or transfusion. Three complications were recorded >6u2003h post‐biopsy but none required intervention. Daycare renal transplant biopsy appears to be safe in selected patients.

Disease Beyond the Arch: A Systematic Review of Middle Aortic Syndrome in Childhood

BACKGROUNDnMiddle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options.nnnMETHODSnA systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes.nnnRESULTSnMost cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking.nnnCONCLUSIONSnMAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition.

Renal pyramid echogenicity in ureteropelvic junction obstruction: correlation between altered echogenicity and differential renal function

BackgroundImprovement in resolution and use of high-frequency transducers in US has enabled visualization of previously unreported changes in medullary pyramid echogenicity in children with obstructive hydronephrosis.ObjectiveTo determine whether these unreported changes in echogenicity and morphology of the renal pyramids in ureteropelvic junction (UPJ) obstruction correlate with differential renal function (DRF) of the kidney as determined by technetium-99m mercaptoacetyltriglycine (99mTc-MAG3) scan.Materials and methodsRenal sonograms in 60 children with UPJ obstruction were retrospectively reviewed. Children were divided into three groups based on the echogenicity of the pyramids: (1) normal echogenicity of the pyramids, (2) increased echogenicity of the pyramids with maintained corticomedullary differentiation (CMD), and (3) loss of CMD. DRF, as determined by 99mTc-MAG3 scan, of the obstructed kidney of ≥45% was considered normal and of ≤44% was considered abnormal based on a published study correlating histological changes with DRF. Fisher′s exact test was performed for assessing the association between DRF and altered echogenicity of the pyramids.ResultsIn group 1, which consisted of 13 patients with normal pyramids on US, DRF was normal in 11 and abnormal in two. In group 2, which consisted of 33 patients with echogenic pyramids and preserved CMD, DRF was normal in 15 and abnormal in 18. In group 3, which consisted of 14 patients with complete loss of CMD, DRF was normal in 2 and abnormal in 12. There was a strong correlation between abnormal pyramids and DRF (P=0.0009). The risk ratio (RR) of DRF becoming abnormal for those kidneys with abnormal echogenicity of the pyramids with preserved CMD (group 2) compared to normal pyramid echogenicity (group 1) was 1.56 (95% CI 1.088–2.236). The RR of DRF becoming abnormal for those kidneys with loss of CMD (group 3) compared to normal pyramid echogenicity (group 1) was 5.571 (95% CI 1.530–20.294).ConclusionWe observed that in obstructed kidneys the echogenicity of the pyramids may be abnormal. Increased echogenicity of the pyramids correlated weakly with abnormal DRF and does not necessarily indicate poor renal function. However, loss of CMD strongly correlated with poor renal function.

Behavioural abnormalities in children with nephrotic syndrome—an underappreciated complication of a standard treatment?

Behaviour and psychosocial adjustment are impaired in children with steroid-sensitive idiopathic nephrotic syndrome (SSNS). Both illness-related variables and family climate play a role. Steroid treatment-both short- and long-term-is an important contributor among other determinants. The exact mechanisms by which steroids lead to behavioural alterations in humans is unclear. Optimizing the benefit/risk ratio of steroid treatment in children with SSNS is a constant goal.

Ethnic Differences in Incidence and Outcomes of Childhood Nephrotic Syndrome

BACKGROUND AND OBJECTIVESnEthnic differences in outcomes among children with nephrotic syndrome are unknown.nnnDESIGN, SETTING, PARTICIPANTS, & MEASUREMENTSnWe conducted a longitudinal study at a single regional pediatric center comparing ethnic differences in incidence from 2001 to 2011 census data and longitudinal outcomes, including relapse rates, time to first relapse, frequently relapsing disease, and use of cyclophosphamide. Among 711 children, 24% were European, 33% were South Asian, 10% were East/Southeast Asian, and 33% were of other origins.nnnRESULTSnOver 10 years, the overall incidence increased from 1.99/100,000 to 4.71/100,000 among children ages 1-18 years old. In 2011, South Asians had a higher incidence rate ratio of 6.61 (95% confidence interval, 3.16 to 15.1) compared with Europeans. East/Southeast Asians had a similar incidence rate ratio (0.76; 95% confidence interval, 0.13 to 2.94) to Europeans. We determined outcomes in 455 children from the three largest ethnic groups with steroid-sensitive disease over a median of 4 years. South Asian and East/Southeast Asian children had significantly lower odds of frequently relapsing disease at 12 months (South Asian: adjusted odds ratio; 0.55; 95% confidence interval, 0.39 to 0.77; East/Southeast Asian: adjusted odds ratio; 0.42; 95% confidence interval, 0.34 to 0.51), fewer subsequent relapses (South Asian: adjusted odds ratio; 0.64; 95% confidence interval, 0.50 to 0.81; East/Southeast Asian: adjusted odds ratio; 0.47; 95% confidence interval, 0.24 to 0.91), lower risk of a first relapse (South Asian: adjusted hazard ratio, 0.74; 95% confidence interval, 0.67 to 0.83; East/Southeast Asian: adjusted hazard ratio, 0.65; 95% CI, 0.63 to 0.68), and lower use of cyclophosphamide (South Asian: adjusted hazard ratio, 0.82; 95% confidence interval, 0.53 to 1.28; East/Southeast Asian: adjusted hazard ratio, 0.54; 95% confidence interval, 0.41 to 0.71) compared with European children.nnnCONCLUSIONSnDespite the higher incidence among South Asians, South and East/Southeast Asian children have significantly less complicated clinical outcomes compared with Europeans.

Novel influenza (H1N1) infection in pediatric renal transplant recipients: a single center experience.

Frieling ML, Williams A, Al Shareef T, Kala G, Teh JC, Langlois V, Allen UD, Hebert D, Robinson LA. novel influenza (H1N1) infection in pediatric renal transplant recipients: A single center experience. u2028Pediatr Transplantation 2012: 16: 123–130.