D F Geary

SickKids, Toronto, Ontario, Canada

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Publications (80)228.33 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Chronic kidney disease is a persistent chronic health condition commonly seen in pediatric nephrology programs. Our study aims to evaluate the sensitivity of the Patient Reported Outcomes Measurement Information System (PROMIS) pediatric instrument to indicators of disease severity and activity in pediatric chronic kidney disease.
    Pediatric nephrology (Berlin, Germany). 06/2014;
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    ABSTRACT: There has been recent emphasis on increased arteriovenous fistula (AVF) use and decreased central venous catheter use in hemodialysis (HD) patients. The International Pediatric Fistula First Initiative was founded via collaborative effort with the Midwest Pediatric Nephrology Consortium to alert nephrologists, surgeons, and dialysis staff to consider fistulae as the best access in pediatric HD patients. A multidisciplinary educational DVD outlining expectations and strategies to increase AVF placement and usage in children was created. Participants were administered a survey previewing and postviewing to identify barriers to placement and usage of AVF in children. A total of 52 surveys were subdivided as either “dialysis staff” or “proceduralist” at five centers. Thirty-three percent of respondents were unaware if their practice was following published guidelines. Sixty-five percent of respondents stated they referred to a dedicated vascular access surgeon at their respective institutions. Methods used to monitor AVF function included physical exam, venous pressure monitoring, and ultrasound dilution. Vascular access was placed within 3 months in only 35% of patients. Interdisciplinary communication problems between surgeons, interventional radiologists, and nephrologists were identified as a major barrier. Lack of AVF usage was often due to maturation failure. Routine access rounds did not occur in any centers. Regarding monitoring, 74% of the respondents use physical exam, 26% use venous pressure monitoring, and 9% use ultrasound dilution. Ninety-three percent of dialysis staff stated they would change practice patterns following the intervention; however, 12% of surgeons stated they would alter practice patterns. To our knowledge, this is the first report to identify barriers to placement of AVF in children from the perspectives of multidisciplinary team members including pediatric nephrologists, surgeons, interventional radiologists, and multidisciplinary dialysis staff.
    Hemodialysis International 06/2014; · 1.44 Impact Factor
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    ABSTRACT: Deficiency of complement factor H-related (CFHR) proteins and CFH autoantibody-positive hemolytic uremic syndrome (DEAP-HUS) represents a unique subgroup of complement-mediated atypical HUS (aHUS). Autoantibodies to the C-terminus of CFH block CFH surface recognition and mimic mutations found in the genetic form of (CFH-mediated) aHUS. CFH autoantibodies are found in 10-15 % of aHUS patients and occur-so far unexplained-almost exclusively in the background of CFHR1 or CFHR3/CFHR1 deletions. As a well-defined role for eculizumab in the treatment of complement-mediated aHUS is becoming established, its role in DEAP-HUS is less conspicuous, where a B-cell-depleting and immunosuppressive treatment strategy is being proposed in the literature. We here show eculizumab to be safe and effective in maintaining a disease-free state, without recurrence, in a previously plasma-therapy-dependent DEAP-HUS patient, and in another patient in whom, although showing a good clinical response to plasma therapy, the therapy was hampered by allergic reactions to fresh frozen plasma and contend there is a rationale for the use of eculizumab in concert with an immunosuppressive strategy in the treatment of DEAP-HUS. Considering the high rate of early relapse, the possible coexistence and contribution of both known and unknown complement-gene mutations, the probable pathogenic role of CFHR1 as a complement alternative pathway (CAP) regulator, the experimental nature of measuring and using anti-CFH autoantibodies to guide management, and until the positive reports of immunosuppression in addition to plasma therapy are confirmed in prospective studies, we feel that a complement-directed therapy should not be neglected in DEAP-HUS. Serial CFH autoantibody titer testing may become a valuable tool to monitor treatment response, and weaning patients off eculizumab may become an option once CFH autoantibody levels are depleted. A prospective study of eculizumab treatment in a larger cohort of DEAP-HUS patients is required to validate the applicability of our positive experience.
    Pediatric Nephrology 11/2013; · 2.94 Impact Factor
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    ABSTRACT: Nephrotic syndrome (NS) represents a common disease in pediatric nephrology typified by a relapsing and remitting course and characterized by the presence of edema that can significantly affect the health-related quality of life in children and adolescents. The PROMIS pediatric measures were constructed to be publically available, efficient, precise, and valid across a variety of diseases to assess patient reports of symptoms and quality of life. This study was designed to evaluate the ability of children and adolescents with NS to complete the PROMIS assessment via computer and to initiate validity assessments of the short forms and full item banks in pediatric NS. Successful measurement of patient reported outcomes will contribute to our understanding of the impact of NS on children and adolescents. This cross-sectional study included 151 children and adolescents 8-17 years old with NS from 16 participating institutions in North America. The children completed the PROMIS pediatric depression, anxiety, social-peer relationships, pain interference, fatigue, mobility and upper extremity functioning measures using a web-based interface. Responses were compared between patients experiencing active NS (n = 53) defined by the presence of edema and patients with inactive NS (n = 96) defined by the absence of edema. All 151 children and adolescents were successfully able to complete the PROMIS assessment via computer. As hypothesized, the children and adolescents with active NS were significantly different on 4 self-reported measures (anxiety, pain interference, fatigue, and mobility). Depression, peer relationships, and upper extremity functioning were not different between children with active vs. inactive NS. Multivariate analysis showed that the PROMIS instruments remained sensitive to NS disease activity after adjusting for demographic characteristics. Children and adolescents with NS were able to successfully complete the PROMIS instrument using a web-based interface. The computer based pediatric PROMIS measurement effectively discriminated between children and adolescents with active and inactive NS. The domain scores found in this study are consistent with previous reports investigating the health-related quality of life in children and adolescents with NS. This study establishes known-group validity and feasibility for PROMIS pediatric measures in children and adolescents with NS.
    Health and Quality of Life Outcomes 01/2013; 11:30. · 2.27 Impact Factor
  • Gareth Morgan, Lee Benson, Denis Geary
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    ABSTRACT: Long segment narrowing of the thoracoabdominal aorta or so-called middle aortic syndrome (MAS), is an uncommon lesion presenting variably with physical signs of coarctation of the aorta, hypertension, renal insufficiency and or mesenteric ischemia. The etiologies are multiple, but Takayasu's arteritis is a leading cause. Variable involvement of diverse systemic arterial systems requires individualized management strategies. We report a case of a successful treatment algorithm for severe MAS using a strategy of percutaneous aortic endovascular stent implantation to rebuild the distal aorta and renal auto-implantation to address systemic hypertension.
    Catheterization and Cardiovascular Interventions 07/2011; 80(2):E5-8. · 2.51 Impact Factor
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    ABSTRACT: We have observed infants with renal dysplasia who developed sustained hypercalcemia, without vitamin D or calcium supplementation (idiopathic). This has not been previously described. 1) Define incidence, severity and duration of idiopathic hypercalcemia in infants with renal dysplasia below 12 months of age. 2) Evaluate phosphate, parathyroid hormone (PTH) and vitamin D levels in these infants. Methods: A retrospective study was conducted from June 2005 to June 2008. Patients receiving calcium-containing phosphate binders or daily supplemental vitamin D in excess of 400 IU were excluded. Hypercalcemia was defined as at least three corrected calcium values above normal lab values for age, in a one-week interval. 15 of 99 (15%) infants with renal dysplasia had hypercalcemia. All were males; 10/15 (67%) were below one month of age at presentation; 9/15 (60%) had posterior urethral valves (PUV). Mean hypercalcemia duration was 5.2 ± 6.0 months. Mean corrected calcium was 3.07 mmol/l (12.3 mg/dl). Only 3/10 infants had elevated PTH levels. None had elevated phosphate levels and only 1/8 patients who had 25-hydroxyvitamin D measured had an elevated level. Idiopathic hypercalcemia in infants with renal dysplasia is common. Neonates and those with PUVs are at greatest risk. Most have normal levels of 25-hydroxyvitamin D, phosphate and PTH.
    Clinical nephrology 05/2011; 75(5):466-71. · 1.29 Impact Factor
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    ABSTRACT: To characterize the attitudes of pediatric nephrologists caring for infants with end-stage renal disease (ESRD) compared with attitudes from a survey published in 1998. Nephrology nurses and social workers were included. An e-mail survey was distributed to pediatric nephrology teams in Canada, Germany, Japan, the United Kingdom, and the United States. Survey responders totaled 270. Renal replacement therapy (RRT) is offered by all nephrologists to some children 1-12 months, and by 98% to some less than 1 month of age (93% in 1998). Of responding nephrologists, 30% offer RRT to all children less than 1 month of age (41% in 1998), and 50%, to all children 1-12 months. Among respondents, 50% indicated that parents can never refuse RRT for children aged 1-12 months, compared with 27% for younger infants. The most influential factor in rejecting RRT for infants was the presence of a co-existing abnormality. Nurses were more likely to believe that parents have the right to refuse RRT for infants. Attitudes of pediatric nephrologists have changed since 1998. Also, nurses have opinions that are different from those of the nephrologists on some issues, and a consensus should be reached before speaking to families.
    Peritoneal dialysis international : journal of the International Society for Peritoneal Dialysis. 03/2011; 31(4):459-65.
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    ABSTRACT: Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.
    Pediatric Nephrology 03/2011; 26(6):979-85. · 2.94 Impact Factor
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    ABSTRACT: Outcome body size of gastrostomy tube (g-tube)-fed children with chronic kidney disease (CKD) was investigated. CKD patients, stages 2-5, who had a g-tube inserted and removed between 1985 and 2007 were retrospectively reviewed (n=20) for anthropometrics, lab values, and steroid use from insertion to latest date. CKD patients never having had a g-tube placed (n=82) acted as the comparison population with similar data collection at start and end of the latest 5-year period. Body mass index (BMI)-for-age, weight (Wt)-for-age, and height (Ht)-for-age z scores were calculated and compared between groups. Median age at insertion and duration of g-tube treatment was 1.7 years (range 0.9-15.6), and 2.9 years (range 0.9-11.8), respectively. There was a significant increase in Wt- (p<0.01), and BMI-for-age (p<0.03) z score, but not for Ht-for-age between insertion and removal for subjects. There were no significant differences in Ht-, Wt-, or BMI-for-age z scores, from removal to 5 years post-removal. In the comparison population, there were no significant differences in Ht-, Wt-, or BMI-for-age z scores over the 5-year period. Approximately 36% of the non-tube-fed comparison population and 50% of the tube-fed subjects were overweight or obese at the most recent evaluation. In both subjects and the comparison group, overweight and obesity is associated with transplant status and steroid use. G-tube feeding is an effective method for achieving catch-up weight and moderate height gain in pediatric CKD patients, and does not apparently predispose patients to obesity after removal; however, overweight and obesity may pose problems to children with CKD whether or not they are tube fed.
    Pediatric Nephrology 10/2010; 25(10):2115-21. · 2.94 Impact Factor
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    ABSTRACT: The aim of this study was to establish the efficacy and safety of rituximab in refractory nephrotic syndrome (NS). Members of the International Paediatric Nephrology Association were asked to retrospectively fill in a questionnaire with details on the use of rituximab in their centres. We divided the data into three groups: group 1, patients with steroid-dependent and frequently relapsing NS; group 2, with steroid-resistant NS; group 3, with post-transplant recurrence of NS. Seventy questionnaires from 25 centres described the outcome of 28, 27 and 15 patients in groups 1, 2 and 3, respectively. Of these, 82% of patients in group 1, 44% of patients in group 2 and 60% of patients in group 3 had a good initial response. Side effects were observed in 27% of the patients, and these were mostly acute reactions. We present a large multicentre series of children with refractory NS. Children in group 1 showed the best response. The good initial response in group 3 can be biased by the accompanying treatments that were administered at the same time as rituximab. Controlled prospective trials are required to establish the value of rituximab in idiopathic NS.
    Pediatric Nephrology 03/2010; 25(3):461-8. · 2.94 Impact Factor
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    ABSTRACT: Bone disease is common in children with chronic kidney disease (CKD) and when untreated may result in bone deformities, bone pain, fractures and reduced growth rates. To investigate the benefits and harms of interventions for preventing and treating bone disease in children with CKD. The Cochrane Renal Group's specialised register, the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, reference lists and abstracts were searched without language restriction. Randomised controlled trials (RCTs) comparing different interventions used to prevent or treat bone disease in children with CKD stages 2-5D compared with placebo, no treatment or other agents were included. Studies examining different routes or frequency of treatment were also included. Data were extracted by two authors. The random-effects model was used and results were reported as risk ratios or risk differences for dichotomous outcomes and mean differences for continuous outcomes with 95% confidence intervals. Fifteen RCTs (369 children) were identified. Compared with oral calcitriol, intraperitoneal calcitriol significantly reduced the level of serum parathyroid hormone (PTH) but there were no significant differences in bone histology or other biochemical measures (2 RCTs). There were no significant differences detected in growth, PTH, serum calcium or phosphorus between daily versus intermittent calcitriol (3 RCTs). Vitamin D therapy significantly reduced PTH levels compared with placebo or no treatment. The number of children with hypercalcaemia did not differ significantly between groups (4 RCTs). No significant differences were detected in growth rates, bone histology or biochemical parameters between calcitriol and either dihydrotachysterol or ergocalciferol (2 RCTs). Though fewer episodes of hypercalcaemia were reported with sevelamer, no significant differences were detected in serum calcium, phosphorus and PTH levels between calcium-containing phosphate binders and either aluminium hydroxide or sevelamer (4 RCTs). Bone disease, assessed by changes in PTH levels, is improved by all vitamin D preparations. However no consistent differences between routes of administration, frequencies of dosing or vitamin D preparations have been demonstrated. Though fewer episodes of high calcium levels occurred with the non calcium-containing binder, sevelamer, compared with calcium-containing binders, there were no differences in serum phosphorus and calcium overall and phosphorus values were reduced to similar extents. All RCTs were small with few data available on patient-centred outcomes (growth, bone deformities) and limited data on biochemical parameters resulting in considerable imprecision of results thus limiting the applicability to care of children with CKD.
    Cochrane database of systematic reviews (Online) 01/2010; · 5.70 Impact Factor
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    ABSTRACT: Atypical hemolytic uremic syndrome (aHUS) is associated with complement system dysregulation, and more than 25% of pediatric aHUS cases are linked to mutations in complement factor H (CFH) or CFH autoantibodies. The observation of thrombocytopenia and platelet-rich thrombi in the glomerular microvasculature indicates that platelets are intimately involved in aHUS pathogenesis. It has been reported that a releasable pool of platelet CFH originates from alpha-granules. We observed that platelet CFH can arise from endogenous synthesis in megakaryocytes and that platelets constitutively lacking alpha-granules contain CFH. Electron and high-resolution laser fluorescence confocal microscopy revealed that CFH was present throughout the cytoplasm and on the surface of normal resting platelets with no evident concentration in alpha-granules, lysosomes, or dense granules. Therapeutic plasma transfusion in a CFH-null aHUS patient revealed that circulating platelets take up CFH with similar persistence of CFH in platelets and plasma in vivo. Washed normal platelets were also observed to take up labeled CFH in vitro. Exposure of washed normal platelets to plasma of an aHUS patient with CFH autoantibodies produced partial platelet aggregation or agglutination, which was prevented by preincubation of platelets with purified CFH. This CFH-dependent response did not involve P-selectin mobilization, indicating a complement-induced platelet response distinct from alpha-granule secretion.
    Blood 08/2009; 114(20):4538-45. · 9.06 Impact Factor
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    ABSTRACT: The uremic state impairs compensatory responses to ultrafiltration (UF). Intradialytic symptoms and hypotension can result and lead to premature discontinuation of treatment and sub-optimal dialysis. We report the benefits of mannitol, sequential dialysis and midodrine in reducing dialysis failures in those children prone to intradialytic hypotension. Prophylactic mannitol halved the odds of intradialytic symptoms and hypotension and increased UF volumes. Sequential dialysis halved the odds of symptoms but hypotension persisted. In one patient with refractory hypotension, only intradialytic midodrine consistently maintained acceptable intradialytic blood pressures, reduced intradialytic symptoms and increased the UF potential.
    Pediatric Nephrology 04/2009; 24(8):1587-91. · 2.94 Impact Factor
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    ABSTRACT: A 4-month-old male infant was diagnosed with nephrogenic diabetes insipidus (NDI). Genetic testing of the arginine vasopressin receptor-2 (AVPR2) yielded a novel X-linked mutation, termed Q96H, in both the propositus and his mother; there was no family history. Protein sequence comparison between AVPR subtypes shows that Q96 is part of a highly conserved motif. Many other disease-causing mutations, confirmed with in vitro expression studies, map to surrounding residues. Molecular modelling studies showed that the equivalent residue in AVPR1 is likely critical for vasopressin binding. We posit that Q96 must be important for the integrity of AVPR2 function.
    NDT Plus 01/2009; 2(1):20-22.
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    ABSTRACT: The occurrence of acute hyponatremia associated with cerebral edema in hospitalized children has been increasingly recognized, with over 50 cases of neurological morbidity and mortality reported in the past decade. This condition most commonly occurs in previously healthy children where maintenance intravenous (IV) fluids have been prescribed in the form of hypotonic saline (e.g., 0.2 or 0.3 NaCl). In response to similar problems at The Hospital for Sick Children (six identified through hospital morbidity and mortality reviews and safety reports prior to fall 2007), an interdisciplinary clinician group from our institution developed a clinical practice guideline (CPG) to guide fluid and electrolyte administration for pediatric patients. This article reviews the evaluation of one patient safety improvement to change the prescribing practice for IV fluids in an acute care pediatric hospital, including the removal of the ability to prescribe hypotonic IV solutions with a sodium concentration of < 75 mmol/L. The evaluation of key components of the CPG included measuring practice and process changes pre- and post-implementation. The evaluation showed that the use of restricted IV fluids was significantly reduced across the organization. Success factors of this safety initiative included the CPG development, forcing functions, reminders, team engagement and support from the hospital leadership. A key learning was that a project leader with considerable dedicated time is required during the implementation to develop change concepts, organize and liaise with stakeholders and measure changes in practice. This project highlights the importance of active implementation for policy and guideline documents.
    Healthcare quarterly (Toronto, Ont.) 01/2009; 12 Spec No Patient:129-34.
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    ABSTRACT: A correlation between relative blood volume (RBV) reduction and adverse intradialytic events has been reported in children. Our aim was to determine whether the shape of the RBV curve and the RBV thresholds discriminated between haemodialysis treatments where patients developed complications and those where they did not. We retrospectively reviewed blood volume records (n = 74), using the Fresenius module. "Treatment complications" were defined as hypotension and/or intradialytic symptoms requiring nursing intervention. Treatments with complications were significantly associated with changes in heart rate (P = 0.05) and steeper decline of the RBV curve in the first hour (P = 0.01), but the final RBV measurement was not predictive (P = 0.27). When receiver operator characteristic (ROC) analyses were used, cut-off RBV thresholds of 88% at the end of the first hour, 84% at the end of the second hour, and 82% at the end of the third hour of dialysis, were able to discriminate between treatments with and without complications, but no RBV values at the end of the fourth hour of dialysis were discriminatory. The RBV curve differed in polyuric patients, such that complications occurred at lower RBV thresholds than in oliguric patients. Evolution of tachycardia and a steeper RBV fall in the first hour of dialysis predicted the occurrence of treatment complications.
    Pediatric Nephrology 06/2008; 23(5):813-20. · 2.94 Impact Factor
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    ABSTRACT: Adequate ultrafiltration (UF) is necessary for good health, but it can be hindered by the development of intradialytic symptoms and hypotension. To determine whether sodium ramping, UF profiles and mannitol could improve UF in children, we instituted a standardized prescription for chronic hemodialysis in our unit. We prospectively analyzed 506 treatments from ten patients. Ultrafiltration volumes up to 9.7% of dry weight were obtained with an overall mean of 5.4%. Mannitol reduced the risk of intradialytic symptoms by 64% (p < 0.05) with a mean UF volume of 6.2%. Step sodium ramping from 148-138 mmol/l reduced the odds of intradialytic symptoms (p = 0.1) and hypotension (p < 0.05) with no difference in the mean UF compared with linear profiles. All UF profiles were associated with an increased risk of intradialytic symptoms, but the effect was only statistically significant with profile 2 (stepwise UF reduction). Overall intradialytic morbidity occurred in 10% of the treatments. Notwithstanding the study limitations, UF volumes higher than traditional recommendations of 5% of the dry weight were achieved with the use of mannitol and 148-138 mmol/l sodium ramping. Despite this, the desired dry weight was not achieved in 66% of our treatments. No clear benefit was seen with UF profiles.
    Pediatric Nephrology 05/2008; 23(4):631-8. · 2.94 Impact Factor
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    ABSTRACT: Central venous lines (CVL) continue to be the most commonly used vascular access device for children on hemodialysis (HD). Despite their frequent use, little is known regarding the frequency of CVL-related intradialytic complications that could interfere with delivery of effective dialysis. To better assess this, we conducted a cross-sectional study of ten HD centers within the Midwest Pediatric Nephrology Consortium. Vascular access was provided by CVL in 61 of the 83 patients (73%) included. CVL dysfunction (defined as reduced blood flows, need for reversed lines, or frequent intradialytic alarms) occurred in 46% in the prior month. Treatment for suspected clots occurred in 16 patients. Intraluminal tissue plasminogen activator (tPA) was the preferred treatment for a suspected clot. The survey also inquired about the preferred treatment for documented clots, and intraluminal tPA was most preferred, followed by CVL stripping, CVL removal, CVL brushing, and systemic tPA. As for preventative strategies, most HD centers locked the CVL with intraluminal heparin in concentrations ranging from 1,000 to 5,000 U/ml. In conclusion, catheter usage rates and complications were highly prevalent in pediatric HD units in this study. As treatment strategies varied greatly, future prospective studies are needed to determine the effectiveness of each individual therapy.
    Pediatric Nephrology 03/2008; 23(2):291-5. · 2.94 Impact Factor
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    Susan M Koshy, Denis F Geary
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    ABSTRACT: Anemia is a common feature of chronic kidney disease, but the management of anemia in children is complex. Erythropoietin and supplemental iron are used to maintain hemoglobin levels. The National Kidney Foundation-Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) clinical practice guidelines for the management of anemia specifically in children were recently published. Pediatric nephrologists are encouraged to use current clinical practice guidelines and best evidence in conjunction with their clinical experience to optimally manage patients with anemia.
    Pediatric Nephrology 03/2008; 23(2):209-19. · 2.94 Impact Factor
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    ABSTRACT: Postinfectious glomerulonephritis (PIGN) most commonly follows streptococcal infection. Antineutrophil cytoplasmic antibodies (ANCA) are characteristically negative in PIGN. We report on five cases who had positive atypical pANCA at presentation. The outcome was typical of other cases of PIGN with complete resolution of the glomerulonephritis in all five patients. Atypical pANCA occurs in a number of inflammatory conditions and antigenic targets may include proteins other than myeloperoxidase or proteinase 3. The presence of atypical p-ANCA does not indicate a poor prognosis.
    Pediatric Nephrology 10/2007; 22(9):1383-6. · 2.94 Impact Factor

Publication Stats

858 Citations
228.33 Total Impact Points

Institutions

  • 1987–2014
    • SickKids
      • • Division of Nephrology
      • • Department of Paediatrics
      Toronto, Ontario, Canada
  • 1996–2013
    • University of Toronto
      • Hospital for Sick Children
      Toronto, Ontario, Canada
  • 2009
    • Great Ormond Street Hospital for Children NHS Foundation Trust
      Londinium, England, United Kingdom
  • 2003
    • The Children's Hospital of Philadelphia
      • Department of Pediatrics
      Philadelphia, PA, United States
  • 2000
    • University of Wales
      Cardiff, Wales, United Kingdom