Publications by authors named "Poyyapakkam R Srivaths"

22 Publications

  • Page 1 of 1

BK Virus Epidemiology, Risk Factors, and Clinical Outcomes: An Analysis of Hematopoietic Stem Cell Transplant Patients at Texas Children's Hospital.

J Pediatric Infect Dis Soc 2021 Jan 8. Epub 2021 Jan 8.

Department of Pediatrics, Section of Infectious Diseases, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas, USA.

Background: BK virus-associated hemorrhagic cystitis (BKV-HC) is a serious complication after hematopoietic stem cell transplantation (HSCT).

Methods: A retrospective review was performed to determine the frequency of BKV-HC and identify risk factors and renal morbidity associated with BKV-HC in pediatric HSCT recipients at our institution.

Results: A total of 314 pediatric recipients underwent allogeneic HSCT for either malignant (173, 55.1%) or nonmalignant disorders (141, 44.9%) from January 1, 2011, to December 31, 2015, with a minimum follow-up of 5 years post-HSCT. Severe BKV-HC (grades 3 and 4) was prevalent in 46 out of 67 (68.7%) recipients. Timing to presentation of severe BKV-HC (grades 3 and 4) occurred at a median of 37 days (26, 74; IQ1, IQ3) post-HSCT, with the duration of macroscopic hematuria lasting a median of 37.5 days (18, 71; IQ1, IQ3). In the first 60 days post-HSCT, peak acute kidney injury (AKI) stages 2 and 3 were seen more frequently in HSCT recipients who developed BKV-HC than those without (P = .004). Similarly, during post-HSCT days 61 to 100, peak AKI stage 3 was also more frequently seen in HSCT recipients who already developed BKV-HC prior to or during this time period than those without BKV-HC (P = .0002). Recipients who developed BKV-HC within 1 year of HSCT had more frequent mild to moderate chronic kidney disease (CKD stages 2-3) than those without BKV-HC (P = .002 and .007, respectively). On multivariate analysis, BKV-HC was associated with all-cause mortality (hazard ratio [HR]: 2.22; 95% confidence interval [CI]: 1.35-3.65). The following clinical variables were associated with time to development of HC on multivariate analysis: age (subdistribution HR [sHR] 1.11; 95% CI: 1.06-1.16) and myeloabalative conditioning regimen (sHR 4.2; 95% CI: 2.12-8.34).

Conclusions: Pediatric HSCT patients with BKV-HC experience significant morbidity and mortality. Renal morbidity, including AKI and CKD, is associated with BKV-HC.
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http://dx.doi.org/10.1093/jpids/piaa147DOI Listing
January 2021

Therapeutic plasma exchange: single-center experience in children with kidney disorders.

Pediatr Nephrol 2021 Mar 19;36(3):621-629. Epub 2020 Sep 19.

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

Background: Therapeutic plasma exchange (TPE) is used in kidney diseases as an adjunct treatment. Little has been described as to its effectiveness in kidney disorders in children. This study aimed to assess the safety, efficacy, and outcomes of patients who underwent TPE for kidney indications.

Methods: Retrospective chart review of patients receiving TPE from 2010 to 2018 for kidney indications, such as antibody-mediated rejection, bone marrow transplant-associated thrombotic microangiopathy (TA-TMA), atypical hemolytic uremic syndrome, transplant recurrence of focal segmental glomerulosclerosis, and glomerulonephritis. Outcomes assessed were trends in kidney function, mortality, and progression to stage 5 chronic kidney disease (CKD 5). Significant hypocalcemia was defined as ionized calcium < 1 mmol/L.

Results: A total of 641 TPE procedures were performed on 47 patients (25 male). Average age was 12.8 ± 5.9 years. Median glomerular filtration rate (GFR) improved from baseline to end of TPE treatments (pre 44.9 (19.8, 79), end 56.1 (23, 98) [p = 0.02]). Ten out of 47 children developed CKD 5. Seven out of 47 patients died; 5 had TA-TMA. Initial 7 consecutive sessions were reviewed for complications. Among 335 procedures, 41 episodes of significant hypocalcemia were noted (12.2%); only 1 was symptomatic. Of the 26 episodes (7.7%) of allergic reactions, all were associated with the use of FFP; 5 were anaphylactic. No TPE-associated mortality was noted.

Conclusions: TPE is a relatively well-tolerated useful adjunct therapy in children with kidney indications. The benefit of TPE has to be balanced with risks such as hypocalcemia and allergic reactions which can occur more frequently with FFP. Graphical abstract.
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http://dx.doi.org/10.1007/s00467-020-04744-8DOI 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.
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http://dx.doi.org/10.1159/000508059DOI Listing
July 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.
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http://dx.doi.org/10.5414/CN109871DOI Listing
December 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.
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http://dx.doi.org/10.1007/s00467-019-04208-8DOI Listing
July 2019

Malnutrition Risk in Hospitalized Children: A Descriptive Study of Malnutrition-Related Characteristics and Development of a Pilot Pediatric Risk-Assessment Tool.

Nutr Clin Pract 2019 Jun 7;34(3):406-413. Epub 2018 Oct 7.

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

Background: Underrecognition of pediatric malnutrition may affect nutrition interventions and outcomes. Pediatric malnutrition uses more specific etiology-based criteria but lacks clarity in implementation guidelines. Study goals were to identify malnutrition and risk among hospitalized patients, characterize malnutrition risk factors, and assess reliability of criteria against outcome measures.

Materials And Methods: All children 44 weeks postmenstrual age-18 years, admitted for 48 hours during a 16-day period, were included (n = 528). Trained dietitians assessed patients in physical assessments (PA), growth, energy intake, increased nutrient losses (IL), altered absorption of nutrients (AA), hypermetabolism and inflammation, laboratory information, micronutrient deficiency, and functional status. Outcome data assessed were length of stay (LOS), intensive care unit (ICU) LOS, ventilation days, nutrition support, and dietitian intervention.

Results: Malnutrition prevalence upon admission was 19.7%. Weight/length or BMI/age z-score (ZS) had no effect on LOS. AA and IL upon admission were independently associated with malnutrition (both, P<.01). Wasting and hypermetabolism were independently associated with longer LOS (P<.01). Other factors associated with longer LOS included IL and inflammation (P < .05). Those with hypermetabolism had significant ZS improvements if followed by a dietitian (P < .05). Wasting via PA was the only factor associated with longer ICU LOS (P < .05).

Conclusions: Identification of risk factors (wasting, hypermetabolism, AA, IL) beyond anthropometrics to define malnutrition and risk is important in prioritizing care in a tertiary pediatric facility. Of great significance is the ability of dietitian-based PA to predict LOS and need for intervention.
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http://dx.doi.org/10.1002/ncp.10200DOI Listing
June 2019

An 8-year-old with genu valgum: Answers.

Pediatr Nephrol 2019 04 26;34(4):621-624. Epub 2018 Sep 26.

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

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http://dx.doi.org/10.1007/s00467-018-4090-4DOI Listing
April 2019

An 8-year-old with genu valgum: Questions.

Pediatr Nephrol 2019 04 26;34(4):619-620. Epub 2018 Sep 26.

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

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http://dx.doi.org/10.1007/s00467-018-4086-0DOI Listing
April 2019

ABO-incompatible deceased donor pediatric liver transplantation: Novel titer-based management protocol and outcomes.

Pediatr Transplant 2018 11 2;22(7):e13263. Epub 2018 Aug 2.

Section of Pediatric Allergy & Immunology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.

ABO-ILT have re-emerged as an alternate option for select patients awaiting transplant. However, treatment protocols for children undergoing deceased donor ABO-ILT are not standardized. We implemented a novel IS protocol for children undergoing deceased donor ABO-ILT based on pretransplant IH titers. Children with high pretransplant IH titers (≥1:32) underwent an enhanced IS protocol including plasmapheresis, rituximab, IVIG, and mycophenolate, while children with IH titers ≤1:16 received steroids and tacrolimus. We retrospectively assessed our outcomes of ABO-ILT with ABO-compatible recipients of similar age and diagnosis over a 2-year period. Ten children with median age of 8.9 months underwent ABO-ILT, 4 of 10 patients underwent enhanced IS due to high IH titers. Rates of complications (rejection, infections, biliary, and vascular) at both 1 year and up to 3 years post-transplant were comparable between the groups. Patients with ABO-ILT had good graft function with 100% survival at a median follow-up of 3.3 years. In conclusion, IS tailored to pretransplant IH titers in pediatric deceased donor ABO-ILT is feasible and can achieve outcomes similar to ABO-CLT at 1 and 3 years post-transplantation.
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http://dx.doi.org/10.1111/petr.13263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6197909PMC
November 2018

Regional citrate anticoagulation for continuous renal replacement therapy in pediatric patients with liver failure.

PLoS One 2017 8;12(8):e0182134. Epub 2017 Aug 8.

Baylor College of Medicine, Department of Pediatrics, Renal Section, Houston, Texas, United States of America.

Pediatric liver failure patients frequently develop multiple organ failure and require continuous renal replacement therapy (CRRT) as part of supportive therapy in the pediatric intensive care unit. While many centers employ no anticoagulation for fear of bleeding complications, balanced coagulation disturbance predisposes these patients to clotting as well as bleeding, making maintenance of longer circuit life to deliver adequate dialysis clearance challenging. Regional citrate anticoagulation (RCA) is an attractive option as it avoids systemic anticoagulation, but since citrate metabolism is impaired in liver failure, concerns about toxicity has limited its use. Pediatric data on RCA with liver failure is very scarce. We aimed to establish safety and efficacy of RCA in pediatric liver failure patients on CRRT. Retrospective review of pediatric patients with liver failure receiving CRRT over 30 months. Demographic data and CRRT related data were collected by chart review. Citrate accumulation (CA) was defined as total calcium (mg/dl) /ionized calcium (mmol/L) ratio >2.5 for > 48 hours. Efficacy was assessed by filter life. Safety was assessed by frequency of adverse events ((AEs) defined as bleeding, hemodynamic instability, arrhythmias). Fifty-one patients (median age 3.5 (IQR 0.75-14.2) years) received 861 CRRT days; 70% experienced at least one episode of CA, only 37% were recorded as such in the medical record. AE rate was 93/1000 CRRT days and did not differ between CA days and others. Median filter life was 66 hours (IQR 29-74); 63% filters lasted longer than 48 hrs. Though common, CA was not associated with increased AEs on in pediatric liver failure patients on CRRT receiving RCA. Filter life was adequate. RCA appears an effective anticoagulation for CRRT in pediatric liver failure. Application of a structured definition would increase recognition of CA to allow timely intervention.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0182134PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5549692PMC
October 2017

Air Displacement Plethysmography Versus Bioelectrical Impedance to Determine Body Composition in Pediatric Hemodialysis Patients.

J Ren Nutr 2017 11;27(6):439-444

Department of Pediatrics, Renal Section, Baylor College of Medicine, Houston, Texas.

Objective: Protein energy wasting is difficult to assess in pediatric hemodialysis (HD) populations because of fluid shifts. Body composition (BC) analysis aids in monitoring nutrition therapy's effects on fat and lean mass. Air displacement plethysmography (ADP) is a "practical" gold standard for fat mass (FM) measurements. We aim to compare BC measures in a pediatric HD population via anthropometry, ADP, and bioelectrical impedance (BIA).

Design: The study was prospective cohort study and the setting was 1 pediatric HD unit in Houston, Texas.

Subjects: A total of 15 pediatric and young adult patients receiving maintenance HD.

Intervention: ADP, BIA, and anthropometry were obtained mid-week post HD for 3 consecutive months.

Main Outcome Measure: The primary outcome was the difference in FM as defined by various assessment methods. Secondary outcomes included correlations with anthropometry.

Results: ADP demonstrated a strong positive correlation to body mass index, mid-upper arm circumference, and triceps skin fold (all P < .0001). No differences in FM assessment using ADP or BIA were found except in males with advanced tanner (P = .0004). BIA underestimated FM in obese subjects (P = .005). Analysis of malnutrition status by mid-upper arm circumference revealed an overestimation of FM in BIA measures (P = .02).

Conclusions: This is the first pediatric dialysis study using ADP to assess BC. Estimation of FM in pediatric HD patients via ADP and BIA has significant error rates in 2 populations: (1) males tanner ≥4: BIA underestimates FM and (2) weight extremes: BIA underestimates FM for obese and overestimates FM for malnourished. BIA should be used with caution in these populations when estimating fluid content and leads to false estimates of dry weight. ADP is a useful adjunct in assessment of nutritional status via BC in pediatric HD patients.
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http://dx.doi.org/10.1053/j.jrn.2017.04.007DOI Listing
November 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.
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http://dx.doi.org/10.1038/gim.2016.131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5362362PMC
April 2017

Perceived appetite and clinical outcomes in children with chronic kidney disease.

Pediatr Nephrol 2016 07 8;31(7):1121-7. Epub 2016 Feb 8.

Emory University and Children's Healthcare of Atlanta, Atlanta, GA, USA.

Background: Children with chronic kidney disease (CKD) may have impaired caloric intake through a variety of mechanisms, with decreased appetite as a putative contributor. In adult CKD, decreased appetite has been associated with poor clinical outcomes. There is limited information about this relationship in pediatric CKD.

Methods: A total of 879 participants of the Chronic Kidney Disease in Children (CKiD) study were studied. Self-reported appetite was assessed annually and categorized as very good, good, fair, or poor/very poor. The relationship between appetite and iohexol or estimated glomerular filtration rate (ieGFR), annual changes in anthropometrics z-scores, hospitalizations, emergency room visits, and quality of life were assessed.

Results: An ieGFR < 30 ml/min per 1.73 m(2) was associated with a 4.46 greater odds (95 % confidence interval: 2.80, 7.09) of having a worse appetite than those with ieGFR >90. Appetite did not predict changes in height, weight, or BMI z-scores. Patients not reporting a very good appetite had more hospitalizations over the next year than those with a very good appetite. Worse appetite was significantly associated with lower parental and patient reported quality of life.

Conclusions: Self-reported appetite in children with CKD worsens with lower ieGFR and is correlated with clinical outcomes, including hospitalizations and quality of life.
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http://dx.doi.org/10.1007/s00467-016-3321-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5627603PMC
July 2016

Using a non-invasive method in chronic hemodialysis pediatric patients to estimate hemoglobin.

Pediatr Nephrol 2015 Apr 17;30(4):661-4. Epub 2014 Oct 17.

Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA,

Background: Maintaining hemoglobin (Hgb) levels within a target range is difficult. Non-invasive hematocrit monitoring (NIVH) continuously monitors both the hematocrit and percent change in intravascular blood volume in real time. Based on the data reported here, NIVH can be utilized as a tool for anemia management in pediatric hemodialysis patients.

Methods: Monthly, mid-week pre-dialysis, Hgb levels were obtained for 12 consecutive months. Concurrent with monthly Hgb, hematocrit was recorded at the start of the dialysis treatment using NIVH. Hgb (oHgb) was calculated using the adult equation Hgb = 0.3112*HCT + 0.71, and a linear regression model was used to derive a pediatric specific equation (pHgb = 0.28*CRIT Hct + 2.5).

Results: A total of 310 observations were obtained from 47 patients. The mean actual hemoglobin (mHgb) was 11.14 ± 1.4, and the mean derived hemoglobin from the adult equation, oHgb, was 10.3 ± 1.3 (p = 0.0001). For the target hemoglobin of 10-12 gm/dl, the adult equation was 72 % sensitive and 63 % specific, whereas the pediatric equation was 93 % sensitive and 70 % specific.

Conclusions: The newly derived pediatric equation (pHgb = 0.28*CRIT Hct + 2.5) improved the prediction capability compared to the standard equation with lower false-negative and false-positive rates.
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http://dx.doi.org/10.1007/s00467-014-2967-4DOI Listing
April 2015

High serum phosphorus and FGF 23 levels are associated with progression of coronary calcifications.

Pediatr Nephrol 2014 Jan 8;29(1):103-9. Epub 2013 Aug 8.

Texas Children's Hospital-Pediatric Nephrology, Baylor College of Medicine, 6621 Fannin Street, MC 3-2482, Houston, TX, 77030, USA,

Background: Coronary calcifications (CC) portend increased mortality in adults receiving hemodialysis (HD), however the risk factors associated with CC progression are not well known in pediatric patients. Our previous cross-sectional studies demonstrated high CC prevalence (31 %) in pediatric patients, which were significantly associated with high serum phosphorus (P), fibroblast growth factor 23 (FGF) levels, dialysis vintage, and low cholesterol. The current study was undertaken to determine and elucidate CC progression in pediatric HD patients.

Methods: A 1-year prospective longitudinal study of 16 pediatric patients (ten male; mean age, 16.9 ± 3 years; range, 10.1-20.4 years) receiving chronic HD was conducted.

Results: CC were observed in five of 16 (31.3 %) patients on baseline computed tomogram (CT) scan; 14/16 patients underwent 1-year CT. All patients with initial CC who completed CT at 1 year (3/5) progressed; one patient had new CC and none of the patients had resolved CC. Mean Agatston score increased from 23.4 ± 18.06 HU (baseline) to 169 ± 298.9 HU. Patients with CC progression had higher mean serum P (8.6 ± 1.8 mg/dl vs. 6.3 ± 1.1 mg/dl, p = 0.015) and FGF 23 levels (3,994 ± 860.5 pg/ml vs. 2,327 ± 1,206.4 pg/ml, p = 0.028). Serum P and FGF 23 levels were positively correlated with final Agatston scores (R = 0.65, p = 0.01 for serum P and R = 0.54, p = 0.045 for FGF 23) and change in Agatston scores (R = 0.65, p = 0.01 for serum P and R = 0.52, p = 0.048 for FGF 23).

Conclusions: Our study shows that CC is progressive in pediatric patients receiving HD and that increased serum P and FGF 23 levels are associated with this progression.
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http://dx.doi.org/10.1007/s00467-013-2575-8DOI Listing
January 2014

Two-point normalized protein catabolic rate overestimates nPCR in pediatric hemodialysis patients.

Pediatr Nephrol 2013 May 2;28(5):797-801. Epub 2012 Dec 2.

Renal Section, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, TX 77030, USA.

Background: Normalized protein catabolic rate (nPCR) calculation depends on estimating the urea generation between consecutive hemodialysis (HD) treatments. Two-point nPCR using blood urea nitrogen (BUN) before and after the same HD treatment has not been validated in pediatric patients, who typically receive a more intense HD dose than adults. This study aimed to compare nPCR calculated with a two-point vs. a three-point nPCR model in pediatric HD patients.

Methods: Pediatric patients receiving HD at 2 units were enrolled. Three BUN measurements were obtained around a midweek HD treatment: one prior to HD (preBUN1), one 30 s after HD (30sBUN), and one prior to the subsequent HD (preBUN2). The two-point nPCR model was calculated using preBUN1 and 30sBUN and the three-point nPCR model was calculated using preBUN2 and 30sBUN.

Results: Seventy-six BUN sets from 35 patients were analyzed. Mean age was 16.4 ± 3.5 years. Mean dry weight was 51.4 ± 17.1 kg. Mean spKt/V was 1.54 ± 0.23. Mean preBUN2 was significantly lower than mean preBUN1 (60.2 ± 18.6 vs. 64.0 ± 18.9 mg/dl, p = 0.0001). nPCR obtained from the three-point model was significantly lower than nPCR obtained from the two-point model (1.07 ± 0.31 vs. 1.17 ± 0.31 g/kg/day, p = 0.00001). Seven of 76 (9.2 %) paired comparisons yielded three-point nPCR <1 vs. two-point nPCR >1.

Conclusions: Our data show that in pediatric patients receiving HD, the ((1) two-point and three-point models lead to significantly different nPCRs, and (2) inaccurate protein intake assessment may result from reliance on a two-point model for nPCR estimates.
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http://dx.doi.org/10.1007/s00467-012-2371-xDOI Listing
May 2013

Elevated FGF 23 and phosphorus are associated with coronary calcification in hemodialysis patients.

Pediatr Nephrol 2011 Jun 27;26(6):945-51. Epub 2011 Feb 27.

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

Increased mortality of adult chronic hemodialysis (HD) patients is associated with coronary calcifications (CC), increased serum phosphorus (P), use of calcium (Ca)-containing P-binders, and vitamin D deficiency. Serum concentration of fibroblast growth factor 23 (FGF 23) is markedly elevated in adults receiving dialysis and is independently associated with increased mortality. Although coronary calcifications have been described in pediatric and adult HD patients, no significant association between serum FGF 23 and CC has been reported. In our study, 5/16 patients had CC. Patients with CC were older, had longer dialysis vintage and higher serum P. Serum Ca, total PTH, elemental Ca intake, and calcitriol doses were not different for CC patients. Serum FGF 23 levels were markedly elevated in all patients (mean 4,024, range 874-8,253), but significantly higher in patients with CC (4,247 ± 10,35 vs 2,427 ± 11,92, p = 0.01) and positively correlated with Agatston calcification score (r = 0.69, p = 0.003) and serum P (r = 0.49, p = 0.05). Using multivariate analysis, serum FGF 23 and serum P remained the most significant factors associated with Agatston score. This study confirms the occurrence of CC in pediatric HD patients and is the first to show a significant association between CC and elevated serum FGF 23 in children.
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http://dx.doi.org/10.1007/s00467-011-1822-0DOI Listing
June 2011

Malnutrition-inflammation-coronary calcification in pediatric patients receiving chronic hemodialysis.

Hemodial Int 2010 Jul 17;14(3):263-9. Epub 2009 Nov 17.

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

Malnutrition, inflammation, and renal osteodystrophy parameters with resultant coronary calcification (CC) are associated with increased cardiovascular mortality in adults. Previous pediatric studies demonstrated CC in children but none assessed for an association between inflammation, malnutrition, renal osteodystrophy, and CC. To assess CC, ultrafast computerized tomogram was obtained for 16 pediatric patients (6 females; median age 17.2 years; range 9.1-21.2 years) receiving hemodialysis for >/=2 months. Inflammation was assessed by serum IL-6, IL-8, and C-reactive protein levels on the day of the computerized tomogram scan; nutrition parameters included serum albumin, cholesterol, the body mass index standard deviation score, and normalized protein catabolic rate. Renal osteodystrophy parameters included time-averaged serum calcium, phosphorus, total PTH, and calcitriol/calcium dose. Patients received hemodialysis thrice-weekly; mean single pool Kt/V 1.48+/-0.13; and mean normalized protein catabolic rate 1.27+/-0.17 g/kg/day. Five of 16 patients had CC. Patients with CC were older (19.1+/-2.1 vs. 15.4+/-3.1 months; P=0.03), had longer dialysis vintage (49.4+/-15.3 vs. 17.2+/-10.5 months, P=0.0002), lower serum cholesterol (122+/-17.7 vs. 160.4+/-10.6 mg/dL, P=0.02), and higher phosphorus (9.05+/-1.2 vs. 6.1+/-0.96 mg/dL, P=0.0001). Mean serum albumin and normalized protein catabolic rate did not differ for patients with CC. All patients had elevated IL-6 and IL-8 levels compared with healthy norms; the mean IL-6, IL-8, and C-reactive protein levels were not different in patients with CC. Coronary calcification was prevalent in older children receiving maintenance hemodialysis with a longer dialysis vintage. Worse renal osteodystrophy control and malnutrition (low cholesterol) may contribute to CC development.
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http://dx.doi.org/10.1111/j.1542-4758.2010.00442.xDOI Listing
July 2010

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

Nutrition aspects in children receiving maintenance hemodialysis: impact on outcome.

Pediatr Nephrol 2009 May 22;24(5):951-7. Epub 2008 Feb 22.

Department of Pediatrics, Baylor College of Medicine and Renal Services, Texas Children's Hospital, Houston, TX 77030, USA.

Children with end-stage renal disease (ESRD) have rates of mortality estimated to be 30-times higher than expected for age compared with those of healthy children. Physical manifestations of under-nutrition, such as body mass index (BMI) and low height standard deviation score (SDS), have been associated with increased risk of mortality. Traditional measures, such as height, weight and serum albumin concentration, may not be accurate indicators to assess the nutritional status of children receiving maintenance hemodialysis. Normalized protein catabolic rate (nPCR) has emerged as a better marker of nutritional status of such children. Meeting the special nutritional needs of these children often requires nutritional supplementation, by either the enteral or the parenteral route. Recently, in children receiving maintenance hemodialysis who are malnourished, intradialytic parenteral nutrition (IDPN) has been utilized as a means to provide additional protein and calories. This article is a state-of-the-art review of malnutrition in children receiving maintenance hemodialysis, with special focus on outcome, nPCR and IDPN.
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http://dx.doi.org/10.1007/s00467-007-0728-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2772959PMC
May 2009

Bacillus cereus central line infection in an immunocompetent child with hemophilia.

J Pediatr Hematol Oncol 2004 Mar;26(3):194-6

Tulane University School of Medicine, Huey P. Long Medical Center, Department of Pediatrics, New Orleans, Louisiana, USA.

Bacillus species are increasingly recognized as pathogens in immunocompromised patients. The authors report a case of Bacillus cereus infection of a central line in an immunocompetent patient with hemophilia, which required line removal for complete cure.
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http://dx.doi.org/10.1097/00043426-200403000-00010DOI Listing
March 2004