Publications by authors named "Sarah Swartz"

26 Publications

  • Page 1 of 1

Continued reduction in peritonitis rates in pediatric dialysis centers: results of the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative.

Pediatr Nephrol 2021 Mar 1. Epub 2021 Mar 1.

Children's Mercy Kansas City, Kansas City, MO, USA.

Background: In its first 3 years, the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative demonstrated a statistically significant increase in the likelihood of compliance with a standardized follow-up care bundle and a significant reduction in peritonitis. We sought to determine if compliance with care bundles and low peritonitis rates could be sustained in centers continuously participating for 84 months.

Methods: Centers that participated from collaborative launch through the 84-month study period and provided pre-launch peritonitis rates were included. Children on maintenance peritoneal dialysis were eligible for enrollment. Changes in bundle compliance were assessed using a logistic regression model or a generalized linear mixed model (GLMM). Changes in average annualized peritonitis rates over time were modeled using GLMMs.

Results: Nineteen centers contributed 1055 patients with 1268 catheters and 17,247 follow-up encounters. The likelihood of follow-up compliance increased significantly over the study period (OR 1.05 95% confidence interval (CI) 1.03, 1.07; p < 0.001). Centers achieved ≥ 80% follow-up bundle compliance by 28 months and maintained a mean compliance of 84% between 28 and 84 months post-launch. Average monthly peritonitis rates decreased from 0.53 (95% CI 0.37, 0.70) infections per patient-year pre-launch to 0.30 (95% CI 0.23, 0.43) at 84 months post-launch, p < 0.001.

Conclusions: Centers participating in the SCOPE Collaborative for 84 months achieved and maintained a high level of compliance with a standardized follow-up care bundle and demonstrated a significant and continued reduction in average monthly peritonitis rates.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-021-04924-0DOI Listing
March 2021

Correlation of Venous Oxygen Saturations from Noninvasive Hematocrit Monitoring Using Blood Gas Measured Oximetry in Chronic Pediatric Hemodialysis Patients.

Blood Purif 2020 8;49(6):665-669. Epub 2020 Jul 8.

Department of Pediatrics, Renal Section, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas, USA.

Introduction: Noninvasive hematocrit monitoring (NIVHM) during pediatric hemodialysis (pedHD) provides data in real time regarding changes in hematocrit and blood volume and also provides venous oxygen saturations. The latter has been proposed to indicate changes in tissue oxygen consumption. It is not known how well NIVHM oxygen saturations (O2sat) approximate blood gas measured oximetry saturation (mO2sat) in the course of pedHD. We aimed to assess the validity and reliability of NIVHM O2sat compared to mO2sat.

Methods: This is a prospective study in 15 patients <21 years old with >90 days on hemodialysis (HD) without congenital heart disease. HD access was fistula (AVF) in 4 patients and tunneled catheters in the remainder. Pulse oximetry (spO2) was continuously monitored; mO2sat was measured via oximetry in a blood gas analyzer and NIVHM O2sat values collected at the start, middle, and end of HD treatment.

Results: A total of 45 dyad measurements were obtained. NIVHM O2sat correlated well with mO2sat (R = 0.89, p < 0.0001); the same was seen at pre, mid, and post HD time points (R = 0.86-0.95, p < 0.001). NIVHM O2sat was lower than mO2sat; with catheter as access, the difference was 9.3 ± 8.6 (CI: 12.3-6.22, p < 0.0001) and with AVF was 2.1 ± 0.78 (CI: 2.6-1.7, p < 0.0001). Bland-Altman analysis demonstrated the difference but did not show any systematic bias. Continuous monitor of spO2 showed no hypoxia.

Discussion/conclusion: Intradialytic NIVHM O2sat correlates well with mO2sat but yield lower values. Future studies can include NIVHM O2sat changes as a surrogate for central venous O2 saturation changes and potentially yield useful information regarding tissue oxygen consumption in pedHD patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1159/000508059DOI Listing
July 2020

Elevated Serum Creatinine: But Is It Renal Failure?

Pediatrics 2020 07 17;146(1). Epub 2020 Jun 17.

Texas Children's Hospital, Houston, Texas; and.

Serum creatinine is typically used to evaluate kidney function. Yet, it is a marker that can only provide estimations of kidney function because it can be influenced by other factors, such as dietary intake. The expanding field of infant formula selection in recent history has given many options for parents who are unable to provide breastmilk. Standard infant formulas and breastmilk generally fall within a select range of creatine content. With greater accessibility to internet-based medical advice (licensed or unlicensed), parents and families have more chances to be exposed to opportunistic websites and opinions that may provide harmful information. In this report, we describe the case of excessive dietary creatine intake in an infant who presented with elevated creatinine while otherwise appearing healthy and having normal cystatin C. After in-depth evaluation of nutritional intake, there was a suspicion for high creatine load of the infant's homemade formula, which was composed of beef liver and various unregulated nutritional powders. Within 12 hours of stopping the infant's homemade formula and providing intravenous fluids, the infant's creatinine normalized. We highlight the importance of in-depth nutrition assessments and education on the health risks associated with improper formula selection.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2019-2828DOI Listing
July 2020

Surgical correction of hydrothorax complicating pediatric peritoneal dialysis.

Perit Dial Int 2021 Jan 20;41(1):122-124. Epub 2020 May 20.

Renal Section, Department of Pediatrics, 3989Baylor College of Medicine/Texas Children's Hospital, Houston, USA.

Hydrothorax complicating continuous cycling peritoneal dialysis (CCPD) is an uncommon event. Its presentation may occur shortly after or years after initiation of dialysis. Surgical intervention offers the advantage of direct visualization and repair of the diaphragmatic defect. Video assisted thoracoscopy surgery (VATS) has been increasingly used in identifying these defects to facilitate this repair. We present 2 pediatric cases who underwent successful direct surgical repair of diaphragmatic defects using VATS with return to CCPD. Initial approach with VATS should be strongly considered in patients in whom a lifetime change in modality has significant repercussions.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0896860820923458DOI Listing
January 2021

Characteristics and Clinical Outcomes of Prolonged Continuous Renal Replacement Therapy in Critically Ill Pediatric Patients.

Pediatr Crit Care Med 2020 06;21(6):571-577

Department of Pediatrics, Renal Section, Texas Children's Hospital, Baylor College of Medicine, Houston, TX.

Objectives: Paralleling improved outcomes in critically ill patients, survival for pediatric acute kidney injury has improved. Continuous renal replacement therapy is the preferred modality to optimize fluid and electrolyte management as well as nutritional support for children developing acute kidney injury in the PICU. However, some patients remain too fragile for transition to intermittent renal replacement therapies and require continuous renal replacement therapy for a prolonged period. Characteristics of this cohort and factors impacting outcomes are not well known. We aimed to describe the characteristics of pediatric patients requiring prolonged continuous renal replacement therapy and evaluate the factors impacting hospital survival.

Design: Retrospective chart review.

Setting: Tertiary PICU.

Patients: Children requiring prolonged continuous renal replacement therapy. Prolonged continuous renal replacement therapy was defined as continuous renal replacement therapy dependence greater than or equal to 28 days. Primary outcome was hospital mortality.

Interventions: None.

Measurements And Main Results: From 2013 to 2016, 344 patients received continuous renal replacement therapy, 36 (10%) received continuous renal replacement therapy for greater than or equal to 28 days. Seventeen patients (47%) were female. Overall mortality was 44% (16/36); 69% (11/16) of nonsurvivors died of sepsis. Pediatric Logistic Organ Dysfunction score was significantly higher in nonsurvivors. Mortality rate was significantly higher in patients who were neutropenic at continuous renal replacement therapy start. Neutropenia (defined as absolute neutrophil count < 1,500/mm) at continuous renal replacement therapy start was the only independent predictor of mortality. One in four survivors did not recover renal function and remained dialysis dependent.

Conclusions: Prolonged continuous renal replacement therapy patients are at high risk of nonrecovery of renal function and require close monitoring. The majority of nonsurvivors in the study group died from sepsis. Neutropenia at continuous renal replacement therapy initiation was associated with increased risk of mortality. Progression of underlying disease process could explain the higher death rate in patients with neutropenia; however, inadequate treatment of infectious complications could be another explanation to explore further in future studies.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/PCC.0000000000002290DOI Listing
June 2020

Tunneled hemodialysis catheter care practices and blood stream infection rate in children: results from the SCOPE collaborative.

Pediatr Nephrol 2020 01 25;35(1):135-143. Epub 2019 Oct 25.

Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Objective: The Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) collaborative seeks to reduce hemodialysis (HD) catheter-associated blood stream infections (CA-BSI) by increasing implementation of standardized HD catheter care bundles. We report HD catheter care practices and HD CA-BSI rates from SCOPE.

Methods: Catheter care practices and infection events were collected prospectively during the study period, from collaborative implementation in June 2013 through May 2017. For comparative purposes, historical data, including patient demographics and HD CA-BSI events, were collected from the 12 months prior to implementation. Catheter care bundle compliance in 5 care bundle categories was monitored across the post-implementation reporting period at each center via monthly care observation forms. CA-BSI rates were calculated monthly, and reported as number of infections per 100 patient months. Changes in CA-BSI rates were assessed using generalized linear mixed model (GLMM) techniques.

Results: Three hundred twenty-five patients with tunneled HD catheters [median (IQR) age 12 years (6, 16), M 53%, F 47%] at 15 centers were included. A total of 3996 catheter care observations over 4170 patient months were submitted with a median (IQR) 5 (2, 14) observations per patient. Overall bundle compliance was high at 87.6%, with a significant and progressive increase (p < 0.001) in compliance for 4/5 bundle categories over the 48-month study period. The adjusted CA-BSI rate significantly decreased over time from 3.3/100 patient months prior to implementation of the care bundles to 0.8/100 patient months 48 months after care bundle implementation (p < 0.001).

Conclusions: Using quality improvement methodology, SCOPE has demonstrated a significant increase in compliance with a majority of HD catheter care practices and a significant reduction in the rate of CA-BSI among children maintained on HD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-019-04384-7DOI Listing
January 2020

Cinacalcet for secondary hyperparathyroidism in end-stage renal disease patients below age 5 years
.

Clin Nephrol 2019 Dec;92(6):279-286

Background: Management of chronic kidney disease mineral bone disorder (CKD-MBD) in pediatric patients with end-stage renal disease (ESRD) is challenging. While the use of calcimimetics is well-studied in adults on dialysis, few studies have been performed in pediatrics. Little is known about the use of cinacalcet in young children with ESRD. The aim of this study was to report the efficacy and safety of cinacalcet for treatment of secondary hyperparathyroidism in chronic dialysis patients younger than 5 years.

Materials And Methods: We included children aged < 5 years on chronic dialysis, either hemodialysis (HD) or peritoneal dialysis (PD), who were prescribed cinacalcet for more than 1 month. Retrospective chart review was performed to gather demographics, dialysis prescription, relevant mineral imbalance laboratory parameters, and medications. Data was collected for 6 consecutive months.

Result: 18 patients (9 male), mean age at initiation of cinacalcet was 2.3 years; 13 PD and 5 HD. Average starting dose of cinacalcet: 6.2 mg daily, 0.55 mg/kg/day. Average time on dialysis was 14.4 months. Parathyroid hormone significantly decreased over the 1 month following initiation of cinacalcet from 929 (IQR 572 - 1,056) to 427 (IQR 256 - 778) pg/mL, p = 0.009. Three patients developed asymptomatic hypocalcemia (Ca < 9.4 mg/dL). Serum phosphorous decreased after initiation, and this was persistent at 6 months. Significant improvement in linear growth was observed while on cinacalcet and growth hormone (GH).

Conclusion: Cinacalcet can be effectively used in young children on dialysis with minimal side effects. Good linear growth was seen in patients on cinacalcet and GH therapy. Long-term large scale data is necessary to confirm. Institution-based management algorithm incorporating cinacalcet would be helpful to maintain uniformity in role of cinacalcet for management of CKD-MBD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5414/CN109871DOI Listing
December 2019

Successful kidney transplantation in a small child with end-stage renal disease due to angiotensin receptor blocker fetopathy and atretic inferior vena cava.

Pediatr Transplant 2019 09 18;23(6):e13497. Epub 2019 Jun 18.

Division of Abdominal Transplantation, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.

Kidney transplantation is the treatment of choice in pediatric patients with end-stage renal disease. This population presents technical challenges particularly in those less than 20 kg due to anomalous anatomy, vascular access issues prior to transplantation, and a generally small size for age. Standard allograft outflow is usually achieved utilizing the iliac veins or IVC. When use of the iliocaval system is not feasible, alternative anastomosis must be considered. Herein, we report a case of a pediatric kidney transplantation where successful allograft outflow was achieved using the SMV when he was found to have an atretic IVC intraoperatively. In this setting, use of the portal system was required to achieve adequate allograft outflow. We created a donor iliac graft for added length to anastomose the renal vein with the SMV. In the setting of IVC occlusion with poor drainage, we utilized a patent vessel with larger caliber for outflow to reduce the risk of high venous pressures, allograft failure, venous rotation, and thrombosis. We conclude that the SMV may serve as an alternative outflow tract in the small pediatric patient and provides the vessel caliber needed to reduce the risks of complications.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/petr.13497DOI Listing
September 2019

Ambulatory blood pressures and central blood pressures are associated with cardiovascular morbidity in adolescent and young adult patients receiving chronic hemodialysis.

Pediatr Nephrol 2019 07 18;34(7):1261-1268. Epub 2019 Feb 18.

Renal Section, Department of Pediatrics, Baylor College of Medicine, 1102 Bates St, Suite 245, Houston, TX, 77030, USA.

Background: Left ventricular hypertrophy (LVH) and arterial stiffness measured by pulse wave velocity (PWV) are independent predictors of cardiovascular (CV) mortality in adults receiving chronic dialysis. Hypertension strongly associates with elevated left ventricular mass index (LVMI) and PWV, with ambulatory blood pressure monitoring (ABPM), and central blood pressure (CBP) superior to office blood pressures (BP) in predicting CV morbidity. Few studies have described associations of office BP, ABPM, and CBP with LVMI and PWV in adolescent and young adult patients receiving hemodialysis (HD).

Methods: Cross-sectional study of 22 adolescents and young adults receiving chronic HD. Pre- and post-dialysis office BP and CBP using applanation tonometry were obtained. Twenty-four-hour ABPM was obtained midweek post-dialysis. Pre- and post-dialysis carotid-brachial PWV were obtained same day as BP measurements. Annual echocardiograms for standard care were reviewed for LVH.

Results: Pre-dialysis CBP index correlated with LVMI (r = 0.3, p = 0.04) and PWV (r = 0.48, p = 0.02). Hypertensive patients identified by ABPM had worse LVMI; daytime ABPM systolic BP index correlated with LVMI (r = 0.5, p = 0.02). Office BP was not associated with LVMI; only office diastolic BP was associated with PWV (r = 0.46, p = 0.02). There was no correlation of LVMI or PWV with bone health parameters, anemia, interdialytic weight gain, or residual renal function.

Conclusions: Ambulatory blood pressure monitoring is superior to casual office BP obtained at time of dialysis in delineating cardiovascular morbidity in adolescent and young adult HD patients. CBP is easily performed and correlates with LVMI and PWV in adolescent and young adult HD patients; however, large-scale normative data is needed.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-019-04208-8DOI Listing
July 2019

Peritoneal dialysis catheter outcomes in infants initiating peritoneal dialysis for end-stage renal disease.

BMC Nephrol 2018 09 14;19(1):231. Epub 2018 Sep 14.

Renal Section, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, 1102 Bates Avenue, Suite 245, Houston, TX, 77030, USA.

Background: End-stage renal disease (ESRD) although rare among infants presents many management challenges. We sought to evaluate factors associated with PD catheter failure among infants initiated on chronic PD.

Methods: A retrospective chart review of all children under two years of age who had PD catheters placed for initiation of chronic PD from 2002 to 2015. Data was extracted for catheter related events occurring within 12 months of catheter placement. Cox and Poisson regression models were used to delineate factors associated catheter complications.

Results: Twenty-five infants with median age 18 days had PD catheters placed for chronic dialysis. Common complications included leakage around the exit site (31%), blockage (26%), migration or malposition (23%), catheter-related infections (18%), and other complications (2%). Predictors of initial PD catheter failure were age less than one month at catheter placement (hazard ratio (HR) 7.77, 95% CI, 1.70-35.39, p = 0.008), use of catheter within three days of placement (HR 5.67, 95% CI, 1.39-23.10, p = 0.015) and presence of a hernia (HR 8.64, 95% CI, 1.19-62.36, p = 0.033). In an adjusted Poisson regression model, PD catheter use within three days of placement was the only predictor of any catheter complication over the12 months of follow up.

Conclusions: Use of PD catheters within three days of placement was associated with catheter failure. We recommend that when possible, catheters should be allowed to heal for at least three days prior to use to reduce risk of complications and improve catheter survival.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12882-018-1015-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6137733PMC
September 2018

Exit site and tunnel infections in children on chronic peritoneal dialysis: findings from the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative.

Pediatr Nephrol 2018 06 26;33(6):1029-1035. Epub 2018 Feb 26.

Division of Nephrology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA.

Background: The Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative is a quality improvement initiative to reduce dialysis-associated infections. The frequency of peritoneal dialysis (PD) catheter exit site infection (ESI) and variables influencing its development and end result are unclear. We sought to determine ESI rates, to elucidate the epidemiology, risk factors, and outcomes for ESI, and to assess for association between provider compliance with care bundles and ESI risk.

Methods: We reviewed demographic, dialysis and ESI data, and care bundle adherence and outcomes for SCOPE enrollees from October 2011 to September 2014. ESI involved only the exit site, only the subcutaneous catheter tunnel, or both.

Results: A total of 857 catheter insertions occurred in 734 children over 10,110 cumulative months of PD provided to these children. During this period 207 ESIs arose in 124 children or 0.25 ESIs per dialysis year. Median time to ESI was 392 days, with 69% of ESIs involving exit site only, 23% involving the tunnel only, and 8% involving both sites. Peritonitis developed in 6%. ESI incidence was associated with age (p = 0.003), being the lowest in children aged < 2 years and highest in those aged 6-12 years, and with no documented review of site care or an exit site score  of > 0 at prior month's visit (p < 0.001). Gender, race, end stage renal disease etiology, exit site orientation, catheter cuff number or mobilization, and presence of G-tube, stoma, or vesicostomy were unassociated with ESI incidence. Of the ESIs reported, 71% resolved with treatment, 24% required hospitalization, and 9% required catheter removal, generally secondary to tunnel infection.

Conclusions: Exit site infections occur at an annualized rate of 0.25, typically well into the dialysis course. Younger patient age and documented review of site care are associated with lower ESI rates. Although most ESIs resolve, hospitalization is frequent, and tunnel involvement/catheter loss complicate outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-018-3889-3DOI Listing
June 2018

Thrombocytopenia-associated multi-organ failure secondary to hyperglycemic, hyperosmolar non-ketotic syndrome: A case report.

Pediatr Diabetes 2018 05 22;19(3):574-577. Epub 2017 Nov 22.

Pediatric Nephrology, Baylor College of Medicine, Houston, Texas.

Thrombocytopenia associated multi-organ failure (TAMOF) is a clinical syndrome with features of new onset thrombocytopenia, increased lactate dehydrogenase, and multi-organ failure in critically ill patients. TAMOF can be the initial presentation of an underlying disease process or can develop during the course of illness either during the hospital stay. TAMOF has a high mortality rate if not treated; therefore, early detection is critical. TAMOF has been rarely reported in diabetic ketoacidosis. We are describing the first case of a patient diagnosed with hyperglycemic, hyperosmolar non-ketotic syndrome who developed TAMOF on the third day of his hospital course. In addition to supportive care in the intensive care unit the patient received serial therapeutic plasma exchanges and improved quickly after treatment. Early diagnosis and treatment of TAMOF decreases morbidity and mortality.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/pedi.12608DOI Listing
May 2018

Efficacy and safety of sevelamer carbonate in hyperphosphatemic pediatric patients with chronic kidney disease.

Pediatr Nephrol 2018 02 12;33(2):325-333. Epub 2017 Sep 12.

Children's Mercy Kansas City, 2401 Gilham Road, Kansas City, MO, 64108, USA.

Background: Treatment for hyperphosphatemia in chronic kidney disease (CKD) involves dietary control of phosphorus intake, dialysis, and treatment with oral phosphate binders, none of which were approved by the Federal Food and Drug Administration in pediatric patients at the time of this study.

Methods: This was a phase 2, multicenter study (NCT01574326) with a 2-week, randomized, placebo-controlled, fixed-dose period (FDP) followed by a 6-month, single-arm, open-label, dose-titration period (DTP), with the aim to evaluate the safety and efficacy of sevelamer carbonate (SC) in hyperphosphatemic pediatric patients with CKD. Following a 2-4 week screening phase, pediatric patients with a serum phosphorus level higher than age-appropriate levels were randomized to receive either SC or placebo as powder/tablets in 0.4-1.6 g doses, based on body surface area. The primary efficacy outcome was the change in serum phosphorus from baseline to end of the FDP in the SC versus placebo arms (analysis of covariance). The secondary outcome was mean change in serum phosphorus from baseline to end of DTP by treatment group and overall. Treatment-emergent/serious adverse events (AEs) were recorded.

Results: Of 101 enrolled patients (29 centers), 66 completed the study. The majority of patients were adolescents (74%; mean age 14.1 years) and on dialysis (77%). Renal transplant was the main reason for discontinuation. SC significantly reduced serum phosphorus from baseline levels (7.16 mg/dL) during the FDP compared to placebo (least square mean difference - 0.90 mg/dL, p = 0.001) and during the DTP (- 1.18 mg/dL, p < 0.0001). The safety and tolerability of SC and placebo were similar during the FDP, with patients in both groups reporting mild/moderate gastrointestinal AEs during the DTP.

Conclusions: Sevelamer carbonate significantly lowered serum phosphorus levels in hyperphosphatemic children with CKD, with no serious safety concerns identified.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-017-3787-0DOI Listing
February 2018

Intra-procedural continuous dialysis to facilitate interventional catheterization in pediatric patients with severe renal failure.

Catheter Cardiovasc Interv 2017 Nov 12;90(5):784-789. Epub 2017 Jul 12.

Lillie Frank Abercrombie Section of Cardiology, Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas.

Background: Interventional catheterization procedures may be needed for patients with severe renal failure who are dependent on dialysis. To avoid the risk of fluid overload and electrolyte derangement during complex procedures in this oliguric/anuric patient population, we performed intra-procedural dialysis, either continuous renal replacement therapy (CRRT) or continous cycling peritoneal dialysis (CCPD).

Methods: We performed a retrospective review of a cohort of pediatric patients, ages 0-18 years, with dialysis-dependent renal failure who received CRRT or CCPD during catheterization procedures from January 2013 to March 2016.

Results: Eight patients underwent a total of nine interventional catheterization procedures while receiving intra-procedural dialysis. Median age was 4.5 years (range 8 months to 17 years) and weight, 11.6 kg (11.2-62.6 kg). Six patients had end-stage renal disease (ESRD) and two patients had acute kidney injury (AKI), one due to hepatorenal syndrome and one due to multifactorial causes associated with congenital heart disease. The most common reason for catheterization was occlusive venous thrombosis requiring recanalization. CRRT was used during five cases and CCPD during four cases. Median procedure time was 337 min (95-651 min) and median contrast dose 4.2 mL kg (1.2-8.2 mL kg ). Euvolemia was maintained based on pre- and post-catheterizations weights, and no significant electrolyte abnormalities occurred based on lab monitoring during and post-procedure.

Conclusions: Intra-procedural dialysis using CRRT or CCPD enables even small pediatric patients with severe renal failure to undergo long and complex interventional catheterizations by reducing the risk of fluid overload and electrolyte abnormalities. Collaboration between nephrology, cardiology, and dialysis teams is necessary for successful management of this challenging patient population.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/ccd.27188DOI Listing
November 2017

Milky appearance of peritoneal fluid in a neonate on peritoneal dialysis due to end-stage renal disease: Answers.

Pediatr Nephrol 2018 01 10;33(1):73-76. Epub 2017 Mar 10.

Renal Section, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, 1102 Bates Avenue, Suite 245, Houston, TX, 77030, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-017-3633-4DOI Listing
January 2018

Milky appearance of peritoneal fluid in a neonate on peritoneal dialysis due to end-stage renal disease: Questions.

Pediatr Nephrol 2018 01 10;33(1):71-72. Epub 2017 Mar 10.

Renal Section, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, 1102 Bates Avenue, Suite 245, Houston, TX, 77030, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-017-3632-5DOI Listing
January 2018

Dialysis in Children and Adolescents: The Pediatric Nephrology Perspective.

Am J Kidney Dis 2017 Feb 7;69(2):278-286. Epub 2016 Dec 7.

Division of Nephrology, Boston Children's Hospital, Boston, MA.

The care of children with end-stage renal disease (ESRD) is highly specialized and often poorly understood by nonpediatric providers and facility/institution administrators. As such, this position paper has been created to offer provider, facility, and institutional guidance regarding the components of care necessary for children receiving dialysis. Key differences between adult and pediatric dialysis units are highlighted. Responsibilities and expectations of the members of the interdisciplinary dialysis team are outlined as they pertain specifically to the care of pediatric dialysis patients. Physical and staffing requirements of the dialysis facility are reviewed, again focusing on unique needs and challenges faced by the pediatric dialysis care team. Among these, vascular access options and proper planning of ESRD care are underscored. Pediatric quality-of-life metrics differ significantly from adult quality variables, and proper tools for assessment must be used. Endorsed by the Council of the American Society of Pediatric Nephrology (ASPN), this position paper serves as a reference tool for the provision of care to pediatric patients with ESRD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.ajkd.2016.09.023DOI Listing
February 2017

Whole-exome sequencing in the molecular diagnosis of individuals with congenital anomalies of the kidney and urinary tract and identification of a new causative gene.

Genet Med 2017 04 22;19(4):412-420. Epub 2016 Sep 22.

Center for Reproductive Medicine, Baylor College of Medicine, Houston, Texas, USA.

Purpose: To investigate the utility of whole-exome sequencing (WES) to define a molecular diagnosis for patients clinically diagnosed with congenital anomalies of kidney and urinary tract (CAKUT).

Methods: WES was performed in 62 families with CAKUT. WES data were analyzed for single-nucleotide variants (SNVs) in 35 known CAKUT genes, putatively deleterious sequence changes in new candidate genes, and potentially disease-associated copy-number variants (CNVs).

Results: In approximately 5% of families, pathogenic SNVs were identified in PAX2, HNF1B, and EYA1. Observed phenotypes in these families expand the current understanding about the role of these genes in CAKUT. Four pathogenic CNVs were also identified using two CNV detection tools. In addition, we found one deleterious de novo SNV in FOXP1 among the 62 families with CAKUT. The clinical database of the Baylor Miraca Genetics laboratory was queried and seven additional unrelated individuals with novel de novo SNVs in FOXP1 were identified. Six of these eight individuals with FOXP1 SNVs have syndromic urinary tract defects, implicating this gene in urinary tract development.

Conclusion: We conclude that WES can be used to identify molecular etiology (SNVs, CNVs) in a subset of individuals with CAKUT. WES can also help identify novel CAKUT genes.Genet Med 19 4, 412-420.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1038/gim.2016.131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5362362PMC
April 2017

Outcomes after peritoneal dialysis catheter placement.

J Pediatr Surg 2016 May 11;51(5):730-3. Epub 2016 Feb 11.

Division of Pediatric Surgery, Department of Surgery, Texas Children's Hospital, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX. Electronic address:

Background: The purpose of this study was to review surgical outcomes after elective placement of peritoneal dialysis (PD) catheters in children with end-stage renal disease.

Methods: Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (>30days post-op) complications (catheter malfunction, catheter malposition, infection), and re-operation rates. Comparison groups included laparoscopic versus open placement, age<2, and weight<10kg. Univariate and multivariate analysis were performed.

Results: One hundred sixteen patients had 173 catheters placed (122 open, 51 laparoscopic) with an average patient age of 9.7±6.3years. Mean catheter life was similar in the laparoscopic and open groups (581±539days versus 574±487days, p=0.938). The late complication rate was higher for open procedures (57% versus 37%, p=0.013). Children age<2 or weight<10kg had higher re-operation rates (64% versus 42%, p=0.014 and 73% versus 40%, p=0.001, respectively). Adjusted for age and weight, open technique remained a risk factor for late complications (OR 2.44, 95% CI 1.20-4.95) but not re-operation.

Discussion: Laparoscopic placement appears to reduce the rate of late complications in children who require PD dialysis catheters. Children <2years age or <10kg remain at risk for complications regardless of technique.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2016.02.011DOI Listing
May 2016

Nocturnal Hypertension and Attenuated Nocturnal Blood Pressure Dipping is Common in Pediatric Lupus.

F1000Res 2015 23;4:164. Epub 2015 Jun 23.

Department of Pediatrics, Renal Section, Baylor College of Medicine, Houston, TX, 77030, USA.

Hypertension is an important manifestation of systemic lupus erythematosus (SLE) but reports of prevalence vary between 20-70% in published reports of adult and pediatric patients. For both children and adults with SLE, the clinical diagnosis and management of hypertension has traditionally been based on guidelines developed for the general population. In clinical trials, the criteria used for defining participants with hypertension are mostly undefined. As a first step towards formally assessing the blood pressure (BP) patterns of children diagnosed with SLE, 24-hr ambulatory BP monitoring data was analyzed on clinic patients who presented with prehypertension or stage I hypertension. In this pediatric SLE cohort (n=10), 20% met daytime criteria for a diagnosis of hypertension. Patterns of BP elevation varied widely with white coat, masked, isolated systolic, and diastolic nocturnal hypertension all identified. Nocturnal hypertension was detected in 60% and attenuated nocturnal BP dipping in 90% of both hypertensive and normotensive SLE patients. In SLE patients, the median nighttime systolic and diastolic loads were 25% and 15.5% compared with median daily loads of 12.5% and 11.5%. Daytime and nighttime systolic and diastolic BP load and nocturnal dipping was compared to a control population consisting of 85 non-SLE patients under 21 years old with prehypertension or stage 1 hypertension presenting to hypertension clinic. Median systolic BP dipped 5.3 mmHg in SLE patients compared to 11.9 mmHg in non-lupus ( p-value = 0.001). Median diastolic BP dipped 12.9 mmHg versus 18.5 mmHg in non-lupus ( p-value = 0.003). Patterns of BP dysregulation in pediatric SLE merit further exploration. Children with or without SLE displaying prehypertensive or stage 1 casual BP measurements had similar rates of hypertension by ambulatory BP monitoring. However, regardless of BP diagnosis, and independent of kidney involvement, there was an increased proportion with attenuated nocturnal dipping and nocturnal hypertension in SLE patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4654458PMC
http://dx.doi.org/10.12688/f1000research.6532.2DOI Listing
December 2015

Interferon Gamma Release Assays to Diagnose Latent Tuberculosis Infection in Pediatric Dialysis Patients.

J Pediatric Infect Dis Soc 2015 Mar 1;4(1):84-6. Epub 2014 Dec 1.

Section of Nephrology, Department of Pediatrics, Baylor College of Medicine and Dialysis Unit Section of Texas Children's Hospital, Houston, Texas.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/jpids/piu113DOI Listing
March 2015

High-dose continuous renal replacement therapy for neonatal hyperammonemia.

Pediatr Nephrol 2013 Jun 8;28(6):983-6. Epub 2013 Mar 8.

Division of Nephrology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA.

Background: Infants with hyperammonemia can present with nonspecific findings so ordering an ammonia level requires a high index of suspicion. Renal replacement therapy (RRT) should be considered for ammonia concentrations of >400 μmol/L since medical therapy will not rapidly clear ammonia. However, the optimal RRT prescription for neonatal hyperammonemia remains unknown. Hemodialysis and continuous renal replacement therapy (CRRT) are both effective, with differing risks and benefits.

Case-diagnosis/treatment: We present the cases of two neonates with hyperammonemia who were later diagnosed with ornithine transcarbamylase deficiency and received high-dose CRRT. Using dialysis/replacement flow rates of 8,000 mL/h/1.73 m(2) (1,000 mL/h or fourfold higher than the typical rate used for acute kidney injury) the ammonia decreased to <400 μmol/L within 3 h of initiating CRRT and to <100 μmol/L within 10 h.

Conclusions: We propose a CRRT treatment algorithm to rapidly decrease the ammonia level using collaboration between the emergency department and departments of genetics, critical care, surgery/interventional radiology, and nephrology.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-013-2441-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3633740PMC
June 2013

Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome.

Pediatr Nephrol 2009 Jun 14;24(6):1187-92. Epub 2009 Feb 14.

Renal Division, Department of Pediatrics, Baylor College of Medicine, 6621 Fannin St., MC3-2482, Houston, TX 77030, USA.

Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children's Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-009-1130-0DOI Listing
June 2009

Cost-effectiveness of ambulatory blood pressure monitoring in the initial evaluation of hypertension in children.

Pediatrics 2008 Dec;122(6):1177-81

Department of Pediatrics, Renal Section, Baylor College of Medicine, 6621 Fannin St, MC3-2482, Houston, TX 77030, USA.

Objective: The goal was to determine the cost-effectiveness of ambulatory blood pressure monitoring in the initial evaluation of stage 1 hypertension.

Methods: Retrospective chart review of data for children referred to Texas Children's Hospital hypertension clinic between January 2005 and August 2006 was performed. We compared the costs of standard evaluations versus the initial use of ambulatory blood pressure monitoring for children with clinic blood pressure measurements suggesting stage 1 hypertension. Charges for clinic visits, laboratory tests, and imaging were obtained from the Texas Children's Hospital billing department.

Results: A total of 267 children were referred. One hundred thirty-nine children did not receive ambulatory blood pressure monitoring; 54 met clinical indications for ambulatory blood pressure monitoring but did not receive it because it was not a covered expense (44 children) or the family refused the study (10 children). One hundred twenty-six children received clinically indicated ambulatory blood pressure monitoring, paid for either through insurance or by the family. Fifty-eight children (46%) had confirmed white-coat hypertension, 62 (49%) stage 1 hypertension, and 6 (5%) stage 2 hypertension. With the observed prevalence of white-coat hypertension, initial ambulatory blood pressure monitoring use yielded net savings after evaluation of 3 patients, with projected savings of $2.4 million per 1000 patients.

Conclusions: Ambulatory blood pressure monitoring in the initial evaluation of suspected childhood hypertension is highly cost-effective. Awareness of cost saving potential may increase the availability of ambulatory blood pressure monitoring for evaluation of new-onset hypertension.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2007-3432DOI Listing
December 2008