Identification of a Locus on the X Chromosome Linked to Familial Membranous Nephropathy.

INTRODUCTION: Membranous nephropathy (MN) is the most common cause of nephrotic syndrome (NS) in adults and is a leading cause of end-stage renal disease due to glomerulonephritis. Primary MN has a strong male predominance, accounting for approximately 65% of cases; yet, currently associated genetic loci are all located on autosomes. Previous reports of familial MN have suggested the existence of a potential X-linked susceptibility locus. Identification of such risk locus may provide clues to the etiology of MN. METHODS: We identified 3 families with 8 members affected by primary MN. Genotyping was performed using single-nucleotide polymorphism microarrays, and serum was sent for anti-phospholipase A2 receptor (PLA2R) antibody testing. All affected members were male and connected through the maternal line, consistent with X-linked inheritance. Genome-wide multipoint parametric linkage analysis using a model of X-linked recessive inheritance was conducted, and genetic risk scores (GRSs) based on known MN-associated variants were determined. RESULTS: Anti-PLA2R testing was negative in all affected family members. Linkage analysis revealed a significant logarithm of the odds score (3.260) on the short arm of the X chromosome at a locus of approximately 11 megabases (Mb). Haplotype reconstruction further uncovered a shared haplotype spanning 2 Mb present in all affected individuals from the 3 families. GRSs in familial MN were significantly lower than in anti-PLA2R-associated MN and were not different from controls. CONCLUSIONS: Our study identifies linkage of familial membranous nephropathy to chromosome Xp11.3-11.22. Family members affected with MN have a significantly lower GRS than individuals with anti-PLA2R-associated MN, suggesting that X-linked familial MN represents a separate etiologic entity.

Management of Pediatric Kidney Transplant Patients During the COVID-19 Pandemic: Guidance From the Canadian Society of Transplantation Pediatric Group.

PURPOSE OF THE PROGRAM: To provide guidance on the management of pediatric kidney transplant patients during the COVID-19 pandemic. SOURCES OF INFORMATION: Program-specific documents, preexisting, and related to COVID-19; documents from provincial, national, and international kidney transplant societies/agencies and organ procurement agencies; national and international webinars, including webinars that we hosted for input and feedback; with additional information from formal and informal review of published academic literature. METHODS: Challenges in the care of pediatric kidney transplant patients during the COVID-19 pandemic were highlighted within the Canadian Society of Transplantation (CST) Pediatric Group. It identified pediatric kidney transplant nephrologists (including a pediatric nephrologist ethicist) across the country and formed a workgroup. The initial guidance document was drafted and members of the workgroup reviewed and discussed all suggestions in detail via e-mail and virtual meetings. Disagreements were resolved by consensus. The document was reviewed by the CST Kidney Transplant Working Group, by the Canadian Society of Nephrology (CSN) COVID-19 Rapid Response Team (RRT), and an infectious disease expert. The suggestions were presented at an interactive webinar sponsored by CSN in collaboration with the CST and Canadian Association of Pediatric Nephrologists (CAPN), and attended by pediatric kidney health care professionals for further peer input. Final revisions were made based on feedback received. CJKHD editors reviewed the parallel process peer review and edited the manuscript for clarity. KEY FINDINGS: We identified 8 key areas of pediatric kidney transplant care that may be affected by the COVID-19 pandemic: (1) transplant activity, (2) outpatient clinic activity, (3) monitoring, (4) multidisciplinary care, (5) medications (immunosuppression and others), (6) patient/family education/support, (7) school and employment, and (8) management of pediatric kidney transplant patients who are COVID-19 positive. We make specific suggestions for each of these areas. LIMITATIONS: A full systematic review of available literature was not undertaken for the sake of expediency in development of this guideline. There is a paucity of literature to support evidence-based recommendations at this time. Instead, these guidelines were formulated based on expert opinion derived from available knowledge/experience and are subject to the biases associated with this level of evidence. The parallel review process that was created to expedite the publication of this work may not be as robust as standard arms' length peer review processes. IMPLICATIONS: These recommendations are meant to serve as a guide to pediatric kidney transplant directors, clinicians, and administrators for providing the best patient care in the context of limited resources while protecting patients and health care providers wherever possible by limiting exposure to COVID-19. We recognize that recommendations may not be applicable to all provincial/local health authority practices and that they may not be delivered to all patients given the time and resource constraints affecting the individual provincial/local health jurisdiction.

Anterior Ischemic Optic Neuropathy in a Child Receiving Chronic Hemodialysis.

Anterior ischemic optic neuropathy (AION) occurs due to hypoperfusion of the optic nerve and is a rare complication in patients receiving maintenance dialysis. To date, AION has only been reported in 22 children, all of whom were receiving peritoneal dialysis. We report the first case of AION in a 2-year 11-month-old child receiving chronic hemodialysis secondary to polycystic kidney disease from a phosphomannomutase 2 gene mutation. This case highlights the consideration for frequent blood pressure monitoring and ophthalmic screening in a certain cohort of children receiving chronic dialysis.

Variation in estimated glomerular filtration rate at dialysis initiation in children.

BACKGROUND: Data guiding the timing of dialysis initiation in children are limited. We sought to determine current practice and secular trends in Canada with respect to the timing of dialysis initiation in children based on estimated glomerular filtration rate (eGFR). METHODS: This observational study included incident chronic dialysis patients aged ≤21 years identified from the Canadian Organ Replacement Register who started dialysis in Canada between January 2001 and December 2010 at any of the nine participating Canadian centers (n = 583). Youth were categorized utilizing CKiD Schwartz eGFR into ≥10.5 (higher) or <10.5 ml/min/1.73 m2 (lower) eGFR groups. Differences at dialysis initiation by facility and region were examined, and secular trends were determined. RESULTS: Median eGFR at dialysis initiation was 8.1 (interquartile range 5.4-11.0) ml/min/1.73 m2. Overall, 29 % of the patients started dialysis with an eGFR of ≥10.5 ml/min/1.73 m2. The proportion of children starting with higher eGFR increased from 27.3 % in 2001 to 35.4 % in 2010 (p = 0.04) and differed by treatment facility (12-70 %; p = 0.0001). Factors associated with higher eGFR at dialysis initiation in the adjusted regression model were female sex [odds ratio (OR) 1.48; 95 % confidence interval (CI) 1.02-2.14], genetic cause of end-stage kidney disease (OR 2.77; 95 % CI 1.37-5.58) and living ≥50 km from treatment facility (OR 1.47; 95 % CI 1.01-2.14). CONCLUSIONS: One-third of the children were found to have initiated dialysis with an eGFR ≥10.5 ml/min/1.73 m2, however significant practice variation exists with respect to timing of dialysis initiation by treatment facility. More data is required to evaluate the clinical implications of this practice variation.

Six of one, half a dozen of the other: A measure of multidisciplinary inter/intra-rater reliability of the society for fetal urology and urinary tract dilation grading systems for hydronephrosis.

INTRODUCTION: The urinary tract dilation (UTD) classification system was introduced to standardize terminology in the reporting of hydronephrosis (HN), and bridge a gap between pre- and postnatal classification such as the Society for Fetal Urology (SFU) grading system. Herein we compare the intra/inter-rater reliability of both grading systems. MATERIALS AND METHODS: SFU (I-IV) and UTD (I-III) grades were independently assigned by 13 raters (9 pediatric urology staff, 2 nephrologists, 2 radiologists), twice, 3 weeks apart, to 50 sagittal postnatal ultrasonographic views of hydronephrotic kidneys. Data regarding ureteral measurements and bladder abnormalities were included to allow proper UTD categorization. Ten images were repeated to assess intra-rater reliability. Krippendorff's alpha coefficient was used to measure overall and by grade intra/inter-rater reliability. Reliability between specialties and training levels were also analyzed. RESULTS: Overall inter-rater reliability was slightly higher for SFU (α = 0.842, 95% CI 0.812-0.879, in session 1; and α = 0.808, 95% CI 0.775-0.839, in session 2) than for UTD (α = 0.774, 95% CI 0.715-0.827, in session 1; and α = 0.679, 95% CI 0.605-0.750, in session 2). Reliability for intermediate grades (SFU II/III and UTD 2) of HN was poor regardless of the system. Reliabilities for SFU and UTD classifications among Urology, Nephrology, and Radiology, as well as between training levels were not significantly different. DISCUSSION: Despite the introduction of HN grading systems to standardize the interpretation and reporting of renal ultrasound in infants with HN, none have been proven superior in allowing clinicians to distinguish between "moderate" grades. While this study demonstrated high reliability in distinguishing between "mild" (SFU I/II and UTD 1) and "severe" (SFU IV and UTD 3) grades of HN, the overall reliability between specialties was poor. This is in keeping with a previous report of modest inter-rater reliability of the SFU system. This drawback is likely explained by the subjective interpretation required to assign grades, which can be impacted by experience, image quality, and scanning technique. As shown in the figure, which demonstrates SFU II (a) and SFU III (b), as assigned by a radiologist, it is possible to make an argument that either of these images can be classified into both categories that were observed during the grading sessions of this study. CONCLUSION: Although both systems have acceptable reliability, the SFU grading system showed higher overall intra/inter-rater reliability regardless of rater specialty than the UTD classification. Inter-rater reliability for SFU grades II/III and UTD 2 was low, highlighting the limitations of both classifications in regards to properly segregating moderate HN grades.

Perceptions of pediatric nephrologists regarding timing of dialysis initiation in children in Canada.

BACKGROUND: Significant practice variation exists in Canada with respect to timing of dialysis initiation in children. In the absence of evidence to guide practice, physicians' perceptions may significantly influence decision-making. OBJECTIVE: The objectives of this study are to (1) evaluate Canadian pediatric nephrologists' perceptions regarding dialysis initiation in children with chronic kidney disease (CKD) and (2) determine the factors guiding practice that may contribute to practice variation across Canada. DESIGN: This study was a cross-sectional online survey. SETTING: This study was done in academic pediatric nephrology centers in Canada. PARTICIPANTS: The participants of this study are pediatric nephrologists. MEASUREMENTS AND METHODS: An anonymous web-based survey was administered to pediatric nephrologists in Canada to evaluate perspectives and practice patterns regarding timing of dialysis initiation. We also explored the importance of estimated glomerular filtration rate (eGFR) vs. symptoms and the role of patient and provider factors influencing decisions. RESULTS: Thirty-five nephrologists (59 %) completed the survey. Most respondents care for advanced CKD patients in a multidisciplinary clinic (86 %) and no centers have a formal policy on timing of dialysis initiation. Seventy-five percent of centers follow <20 stage 4-5 CKD patients, and 9 % follow >30 patients. Discussions about dialysis initiation are generally informal (75 %) and the decision to start is made by the nephrologist (37 %) or a team (57 %). Fifty percent agreed GFR was important when deciding when to initiate dialysis, 41 % were neutral, and 9 % disagreed. Variability exists in the threshold that nephrologists considered early (vs. late) dialysis initiation: >20 (21 %), >15 (38 %), >12 (26 %), and >10 ml/min/1.73 m(2) (12 %). Practitioners however typically start dialysis in asymptomatic patients at eGFRs of 7-9 (9 %), 10-11 (41 %), 12-14 (38 %), and 15-19 (6 %) ml/min/1.73 m(2). Patient factors important in the decision to start dialysis for >90 % of nephrologists were fatigue, >10 % weight loss, nausea, increasing missed school, and awaiting a pre-emptive transplant. Age was only a factor for 56 %. LIMITATIONS: This study has a 59 % response rate. CONCLUSIONS: Variability exists in Canada regarding the importance and threshold of eGFR guiding the decision as to when to start dialysis in children, whereas patient symptoms are almost universally important to pediatric nephrologists' decision-making. Additional studies evaluating outcomes of children starting dialysis earlier vs. later are needed to standardize decision-making and care for children with kidney failure.

MISE EN CONTEXTE: Des différences significatives existent dans la pratique au Canada quant au moment où des traitements de dialyse devraient être entrepris chez les enfants souffrant d'insuffisance rénale. En absence de données probantes pour guider leur pratique, les différentes approches des néphrologues exerçant auprès de cette population jouent probablement un rôle majeur dans leur prise de décision. OBJECTIFS DE L'ÉTUDE: Dans un premier temps, l'étude visait à évaluer la perception des néphrologues canadiens en regard de l'amorce de traitements de dialyse chez les enfants atteints d'insuffisance rénale chronique (IRC). Ensuite, on a voulu déterminer les facteurs qui guident la pratique et qui font en sorte que des variations subsistent à cet égard dans la pratique en néphrologie pédiatrique à travers le Canada. CADRE ET TYPE D'ÉTUDE: Il s'agit d'un sondage transversal mené en ligne auprès des néphrologues pratiquant dans les unités pédiatriques des centres hospitaliers universitaires dans tout le Canada. MÉTHODOLOGIE: Un sondage accessible par le web, auquel les participants répondaient de façon anonyme, a été distribué aux spécialistes canadiens pratiquant en néphrologie pédiatrique. Ce sondage avait pour objectif d'évaluer les perceptions et les schémas de pratique relativement au moment le plus propice pour amorcer la dialyse. Le sondage explorait aussi l'importance du débit de filtration glomérulaire estimé (DFGe) par rapport aux symptômes ressentis par le patient dans la prise de décision. Finalement, nous avons tenté d'identifier les facteurs pouvant influencer le patient et son médecin traitant au moment de décider d'entreprendre des traitements de dialyse. RÉSULTATS: Tous les pédiatres-néphrologues pratiquant au Canada ont reçu le questionnaire. Toutefois, seulement 35 d'entre eux, soit un peu plus de la moitié (59 %), l'ont complété et renvoyé. La grande majorité des répondants (86 %) exerçaient au sein de cliniques multidisciplinaires, auprès de cas sévères d'IRC. Aucun des centres de soins où ces spécialistes pratiquent ne possédait de politique formelle quant au moment d'entreprendre des traitements de dialyse chez les patients suivis en néphrologie pédiatrique. Les trois quarts des centres de soins cités (75 %) suivaient moins de 20 cas d'IRC de stade 4 ou 5, alors que 9 % en suivaient plus de 30. Les répondants ont indiqué dans une proportion de 75 % que la discussion concernant l'amorce de la dialyse se déroulait de façon informelle. De plus, le sondage révèle que la décision d'amorcer la dialyse est prise par le néphrologue soignant seulement (37 % des cas) ou par une équipe (57 % des cas). La moitié des répondants (50 %) s'accordait pour dire que le DFGe était important dans leur prise de décision d'amorcer des traitements de dialyse chez leurs patients, 41 % avaient une opinion neutre à ce sujet alors que 9 % ne jugeaient pas cet élément important. Les pédiatres-néphrologues répondants sont partagés quant à la valeur seuil de DFGe qu'ils considèrent comme une amorce « hâtive ¼ de dialyse. Pour 21 % des répondants, cette valeur se situe à 20 ml/min/1,73 m2; pour 38 % elle se situe plutôt à 15 ml/min/1,73 m2; 26 % l'établissent à 12 ml/min/1,73 m2; alors que 12 % des répondants jugent l'amorce d'une dialyse dite « hâtive ¼ à un DFGe de 10 ml/min/1,73 m2. De façon générale, chez les patients asymptomatiques, la dialyse est amorcée lorsque le DFGe se situe entre 7 et 9 ml/min/1,73 m2 (9 % des cas), entre 10 et 11 ml/min/1,73 m2 (41 % des cas), entre 12 et 14 ml/min/1,73 m2 (38 % des cas) ou entre 15 et 19 ml/min/1,73 m2 (6 % des cas). Enfin, parmi les facteurs motivant les patients à entreprendre des traitements de dialyse, tels que rapportés par les répondants dans une proportion de plus de 90 %, on trouve : une sensation de fatigue, une perte de poids de plus de 10 %, des nausées, les absences répétées à l'école et l'attente d'une greffe rénale préventive. L'âge n'a été mentionné comme facteur que dans 56 % des cas. LIMITES DE L'ÉTUDE: Le taux de réponse au sondage relativement faible (59 %) limite la portée des résultats. CONCLUSIONS: En néphrologie pédiatrique, alors que les symptômes ressentis par les patients sont considérés de façon universelle dans la décision d'entreprendre une dialyse, les pédiatres-néphrologues canadiens ont des perceptions très différentes quant à la valeur seuil de DFGe qui devrait guider cette décision et à l'importance du rôle que ce paramètre devrait y jouer. Des études supplémentaires comparant les résultats chez les patients qui amorcent une dialyse dite hâtive par rapport à ceux de patients l'amorçant plus tard s'avèrent nécessaires pour établir des pratiques standardisées en matière de soins pour les enfants atteints d'insuffisance rénale.

Fanconi Syndrome Secondary to Deferasirox in Diamond-Blackfan Anemia: Case Series and Recommendations for Early Diagnosis.

Deferasirox is an oral iron chelator used to treat patients with transfusion-related iron overload. We report, from two institutions, two children with Diamond-Blackfan anemia who developed Fanconi syndrome secondary to deferasirox administration, along with a review of the literature. The current recommendation for the laboratory monitoring of patients receiving deferasirox does not include serum electrolytes or urine analysis. Thus, despite routine clinic visits and bloodwork, these two patients presented with life-threatening electrolyte abnormalities requiring hospitalization. Hence, we propose the inclusion of serum electrolytes and urine analysis as part of routine monitoring to facilitate the early diagnosis of Fanconi syndrome in the context of high doses of deferasirox therapy.

Effects of acute exercise on markers of inflammation in pediatric chronic kidney disease: a pilot study.

BACKGROUND: Children and adolescents with chronic kidney disease (CKD) are chronically exposed to high levels of inflammation, placing them at an increased risk of secondary health complications. Regular exercise may represent an effective therapy to reduce inflammation. The aims of this pilot study were to determine the effects of acute exercise on inflammation and immune cell counts in CKD. METHODS: Nine children and adolescents (4 males) with CKD stages III-V performed a graded exercise test to determine peak oxygen uptake (VO2peak). Following a 10-min break, participants cycled for 20 min at 50 % of VO2peak. Blood samples were collected before and after the exercise period for the determination of complete blood counts, natural killer cells (NK(bright), NK(dim)) and circulating progenitor cell (CPC) counts, as well as interleukin-6 (IL-6), and tumor necrosis factor-α (TNF-α) concentrations. RESULTS: Complete blood counts and NK(dim) cell and CPC counts were unchanged with exercise. Following exercise, NK(bright) cell counts increased (7.4 ± 4.3 vs. 12.2 ± 8.3 × 10(6) cells/L; p = 0.02), while trends were observed for an increase in IL-6 (2.1 ± 2.2 vs. 2.7 ± 2.6 pg/mL; p = 0.08), decrease in TNF-α (4.5 ± 1.2 vs. 4.2 ± 1.0 pg/mL; p = 0.08) and an increase in the IL-6:TNF-α ratio (0.6 ± 0.7 vs. 0.8 ± 0.8; p = 0.07). CONCLUSIONS: Our findings suggest that acute exercise may create an anti-inflammatory environment in children and adolescents with CKD stages III-V.

The Canadian Childhood Nephrotic Syndrome (CHILDNEPH) Project: overview of design and methods.

BACKGROUND: Nephrotic syndrome is a commonly acquired kidney disease in children that causes significant morbidity due to recurrent episodes of heavy proteinuria. The management of childhood nephrotic syndrome is known to be highly variable among physicians and care centres. OBJECTIVES: The primary objective of the study is to determine centre-, physician-, and patient-level characteristics associated with steroid exposure and length of steroid treatment. We will also determine the association of dose and duration of steroid treatment and time to first relapse as a secondary aim. An embedded qualitative study utilizing focus groups with health care providers will enrich the quantitative results by providing an understanding of the attitudes, beliefs and local contextual factors driving variation in care. DESIGN: Mixed-methods study; prospective observational cohort (quantitative component), with additional semi-structured focus groups of healthcare professionals (qualitative component). SETTING: National study, comprised of all 13 Canadian pediatric nephrology clinics. PATIENTS: 400 patients under 18 years of age to be recruited over 2.5 years. MEASUREMENTS: Steroid doses for all episodes (first presentation, first and subsequent relapses) tracked over course of the study. Physician and centre-level characteristics catalogued, with reasons for treatment preferences documented during focus groups. METHODS: All patients tracked prospectively over the course of the study, with data comprising a prospective registry. One focus group at each site to enrich understanding of variation in care. LIMITATIONS: Contamination of treatment protocols between physicians may occur as a result of concurrent focus groups. CONCLUSIONS: Quantitative and qualitative results will be integrated at end of study and will collectively inform strategies for the development and implementation of standardized evidence-based protocols across centres.

CONTEXTE: Le syndrome néphrotique est une néphropathie fréquente chez l'enfant, qui cause une morbidité considérable en raison de la récurrence d'épisodes de protéinurie importante. La gestion du syndrome néphrotique de l'enfant varie énormément d'un médecin et d'un centre de soins à l'autre. OBJECTIFS: L'objectif principal de cette étude est de déterminer les caractéristiques associées à l'exposition et la durée du traitement aux stéroïdes, liées au centre, au médecin et au patient. En deuxième lieu, nous déterminerons la corrélation entre la dose et la durée du traitement aux stéroïdes, puis la durée avant la première rechute. À cette étude s'ajoutera une étude qualitative avec des groupes de discussion composés de professionnels de la santé qui viendra enrichir les résultats quantitatifs en favorisant une meilleure compréhension des attitudes, des croyances et des facteurs contextuels locaux qui entraînent des variations dans les soins. TYPE D'ÉTUDE: Une étude méthodologique mixte; étude d'observation de cohorte prospective (composante quantitative), combinée avec des groupes de discussion semi-structurés composés de professionnels de la santé (composante qualitative). CONTEXTE/ÉCHANTILLON: Étude nationale, constituée des 13 cliniques canadiennes de néphrologie pédiatrique. PARTICIPANTS: 400 patients âgés de moins de 18 ans, à recruter sur une période de 2,5 années. MESURES: Suivi des doses de stéroïdes pour chacun des épisodes (première présentation, première rechute et suivantes), tout au long de l'étude. Catalogage des caractéristiques liées au médecin ou au centre, et attestation des raisons justifiant les préférences de traitement au cours des séances avec les groupes de discussion. MÉTHODES: Tous les patients suivis de manière prospective dans le cadre de l'étude, dont les données constituent un registre prospectif. Un groupe de discussion à chaque endroit, afin d'enrichir la compréhension des variations dans les soins. LIMITES DE L'ÉTUDE: La contamination des protocoles de traitement entre les médecins peut se produire en raison de la tenue simultanée de groupes de discussions. CONCLUSIONS: Les résultats quantitatifs et qualitatifs seront intégrés à la fin de l'étude et permettront de mettre en place des stratégies de développement et de mise en œuvre de protocoles normalisés et fondés sur des données probantes.

Substantial practice variation exists in the management of childhood nephrotic syndrome.

BACKGROUND: Practice variation is common for nephrotic syndrome (NS) treatment. METHODS: A cross-sectional, web-based survey on NS treatment was administered to 58 Canadian pediatric nephrologists with the aim to document existing practice variation and compare practice with the recommendations of the Kidney Disease Improving Global Outcomes Clinical Practice Guideline for NS. RESULTS: Of the 58 nephrologists asked to participate in the survey, 40 (69 %) responded. Among these, 62 % prescribed initial daily glucocorticoid (GC) therapy for 6 weeks, 26 % for 4 weeks by 26 %, and 10 % prescribed 'other'. Alternate-day GC was continued for 6 weeks by 63 % of respondents and for >6 and <6 weeks by 32 and 6 %, respectively. For biopsy-confirmed minimal change disease, 65 and 46 % of respondents chose oral cyclophosphamide for frequently relapsing and steroid-dependent phenotypes, respectively; calcineurin inhibitors or mycophenolate were the second most popular choices. Kidney biopsy was 'always' performed by 16, 39, and 97 % of respondents for frequently relapsing, steroid-dependent, and steroid-resistant patients, respectively. Rituximab had been administered by 60 % of respondents; 22, 56, and 72 % reported that they would consider rituximab for frequently relapsing, steroid-dependent, and steroid-resistant patients, respectively. Most notable differences between practice and Guideline recommendations were first presentation GC duration, GC-sparing agent choices in frequently relapsing and steroid-dependent patients, and biopsy practices. CONCLUSIONS: There is substantial Canadian practice variation in NS treatment. Assessment of factors driving variation and strategies to implement Guideline recommendations are needed.

Prevalence of sleep disturbances in children and adolescents with chronic kidney disease.

Although sleep disorders are common in adults with chronic kidney disease, little is known about the prevalence of sleep problems in children and adolescents with chronic kidney disease and their relationship to health-related quality of life measurements. We performed a clinic-based survey of sleep habits and common symptoms of sleep disturbances in 159 school-aged patients with chronic kidney disease. Three patient groups of chronic kidney disease were assessed: group 1, those not on dialysis and not transplanted; group 2, those on dialysis; and group 3, those with a functioning renal allograft. Four symptom domains for sleep disorders were assessed: excessive daytime sleepiness; sleep disordered breathing; restless legs syndrome symptoms; and insufficient sleep. Patients and the parent-proxy also completed the Pediatric Quality of Life Inventory Version 4.0 Generic Core Scales questionnaire. Ninety-three (93) patients (58.5%) had symptoms of a sleep disturbance. The presence of a sleep disturbance correlated with a decrease in health-related quality of life scores that was independent of the chronic kidney disease study group or estimated glomerular filtration rate. We conclude that sleep disturbances are common throughout the spectrum of chronic kidney disease in children and adolescents and are associated with diminished health-related quality of life scores.