Improving Pediatric Hypertension Screening in an Academic Primary Care Setting.

Introduction: Adherence to the American Academy of Pediatrics clinical practice guidelines for screening and managing high blood pressure (BP) is low. This team sought to improve recognition and documentation of relevant diagnoses in patients aged 13-20 years who presented to general pediatric clinics. Methods: The primary outcome measure was the proportion of office visits for patients ages 13-20 with a BP ≥ 120/80 with a visit or problem list diagnosis of hypertension or elevated BP. Secondary measures included (1) the proportion of patients who had their BP measured in the right arm, (2) the proportion of patients who had a mid-arm circumference measurement recorded, and (3) the proportion of patients who had a second BP reading measured at the visit. Interventions addressed key drivers for evidence-based high BP screening: standard BP measurement, electronic health record clinical decision support, and clinical pathway adoption. Data were collected over a twenty-seven-month period and plotted using the Laney p' chart. Results: Provider documentation of elevated BP or hypertension improved from a baseline mean of 24% in April 2020 through January 2022 to 41% in February 2021 through June 2022. All secondary outcome measures also demonstrated significant improvement. Conclusions: This project demonstrates the feasibility of improving adherence to best practices of BP measurement in primary care clinics through education, acquisition of resources, and implementation of electronic health record flags for abnormal values.

Streptococcus pneumoniae associated hemolytic uremic syndrome in children.

Introduction: Previous small-scale, single-center investigations of Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) have shown increased disease severity among SpHUS relative to non-SpHUS patients. Our study compares the impact of S. pneumoniae on patient outcomes between SpHUS cases and non-SpHUS controls using the national, multicenter retrospective Pediatric Health Information Systems (PHIS) Database. Methods: Children <18 years of age with a diagnosis of HUS were included. Univariate analyses and multivariable linear and logistic regressions were utilized to assess the impact of S. pneumoniae on mortality, length of stay (LOS), intensive care unit admission (ICU), and mechanical ventilation use. Models were adjusted for demographic and clinical characteristics, including cardiac, neurologic, pulmonary, gastrointestinal, immunologic and renal clinical complications. Results: Of 3,952 index HUS hospitalizations, 231 (5.8%) were due to SpHUS. SpHUS patients had worse outcomes, including longer hospital stays, increased rate of ICU admission, and increased use of mechanical ventilation (p < 0.001 for all). There was a strong positive relationship between clinical complications and adverse outcomes. After adjusting for covariates, SpHUS was associated with an increase in hospital LOS by 3.47 days (p = 0.009) and overall ICU-LOS by 4.21 days (p < 0.001). SpHUS was also associated with increased likelihood of mechanical ventilation (OR: 3.08; p < 0.001), with no increase in ICU admission (p = 0.070) and in-hospital mortality (p = 0.3874). Discussion: Our study highlights that SpHUS patients are at increased risk of multiple adverse outcomes likely due to the summative impact of pneumococcal infection and HUS as well as more frequent clinical complications.

Cardiac Manifestation among Children with Hemolytic Uremic Syndrome.

OBJECTIVE: The primary objectives of the study were to describe the association between cardiac manifestations and in-hospital mortality among children with hemolytic uremic syndrome. STUDY DESIGN: Using the Pediatric Health Information System database, this retrospective, multicenter, cohort study identified the first hemolytic uremic syndrome-related inpatient visit among children ≤18 years (years 2004-2018). The frequency of selected cardiac manifestations and mortality rates were calculated. Multivariate analysis identified the association of specific cardiac manifestations and the risk of in-hospital mortality. RESULTS: Among 3915 patients in the analysis, 238 (6.1%) had cardiac manifestations. A majority of patients (82.8%; n = 197) had 1 cardiac condition and 17.2% (n = 41) had ≥2 cardiac conditions. The most common cardiac conditions was pericardial disease (n = 102), followed by congestive heart failure (n = 46) and cardiomyopathy/myocarditis (n = 34). The percent mortality for patients with 0, 1, or ≥2 cardiac conditions was 2.1%, 17.3%, and 19.5%, respectively. Patients with any cardiac condition had an increased odds of mortality (OR, 9.74; P = .0001). In additional models, the presence of ≥2 cardiac conditions (OR, 9.90; P < .001), cardiac arrest (OR, 38.25; P < .001), or extracorporeal membrane oxygenation deployment (OR, 11.61; P < .001) were associated with increased risk of in-hospital mortality. CONCLUSIONS: This study identified differences in in-hospital mortality based on the type of cardiac manifestations, with increased risk observed for patients with multiple cardiac involvement, cardiac arrest, and extracorporeal membrane oxygenation deployments.

Targeting Mitochondria during Cold Storage to Maintain Proteasome Function and Improve Renal Outcome after Transplantation.

Kidney transplantation is the preferred treatment for end-stage kidney disease (ESKD). Compared to maintenance dialysis, kidney transplantation results in improved patient survival and quality of life. Kidneys from living donors perform best; however, many patients with ESKD depend on kidneys from deceased donors. After procurement, donor kidneys are placed in a cold-storage solution until a suitable recipient is located. Sadly, prolonged cold storage times are associated with inferior transplant outcomes; therefore, in most situations when considering donor kidneys, long cold-storage times are avoided. The identification of novel mechanisms of cold-storage-related renal damage will lead to the development of new therapeutic strategies for preserving donor kidneys; to date, these mechanisms remain poorly understood. In this review, we discuss the importance of mitochondrial and proteasome function, protein homeostasis, and renal recovery during stress from cold storage plus transplantation. Additionally, we discuss novel targets for therapeutic intervention to improve renal outcomes.

Risk factors for early onset peritonitis: the SCOPE collaborative.

BACKGROUND: Peritoneal dialysis (PD) is the preferred chronic dialysis modality amongst pediatric patients. Peritonitis is a devastating complication of PD. Adult data demonstrates early onset peritonitis (EP) is associated with higher rates of subsequent peritonitis and technique failure. Limited data exists regarding EP in the pediatric population, here defined as peritonitis occurring within 60 days of catheter insertion. METHODS: PD catheter insertion practices and EP episodes were examined from the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) collaborative database. RESULTS: There were 98 episodes of EP amongst 1106 PD catheters inserted. Multivariable analysis demonstrated a significant association between early use of the PD catheter and EP (P = 0.001). Age less than 1 year at the time of catheter insertion (P < 0.001), first catheter placed (P < 0.001) for the patient, use of a plastic adapter (P = 0.003), placement of sutures at the exit site (ES) (P = 0.032), and dressing change prior to 7 days post-operatively (P < 0.001) were all significantly associated with early PD catheter use. Concurrent placement of a hemodialysis catheter was associated with a decreased risk for early PD catheter use (P = 0.010). CONCLUSIONS: In this large cohort of pediatric PD recipients, 8.4% of PD catheters were associated with the development of EP. The finding of an association between early use of the PD catheter and EP represents a potentially modifiable risk factor to reduce infection rates within this patient population.

Acute kidney injury is associated with increased in-hospital mortality in mechanically ventilated children with trauma.

BACKGROUND: Acute kidney injury (AKI) is associated with significant morbidity and mortality in patients with critical illness; however, its impact on children with trauma is not fully unexplored. We hypothesized that AKI is associated with increased in-hospital mortality. METHODS: A retrospective review of consecutive mechanically ventilated patients aged 0 years to 20 years from 2004 to 2007 with trauma hospitalized at our institution was performed. Univariate and multivariate analyses were performed to identify whether AKI was a risk factor for hospital mortality. RESULTS: Eighty-eight patients met inclusion/exclusion criteria. The study cohort included 58 (66%) males with mean (SD) age of 11.6 (5.5) years (median, 13.25; range, 0.083-19.42 years) and mean (SD) Pediatric Expanded Logical Organ Dysfunction score of 24 (11) (median, 22; range 2-51). Mean pediatric intensive care unit length of stay (median, 11; range, 4-43) and duration of mechanical ventilation (median, 9; range, 3-34), was 13.5 (8.2) days and 11.2 (7.2) days, respectively. The mean (SD) Injury Severity Score for the cohort was 28 (14). Pediatric RIFLE identified those at risk (R), those with injury (I), or those with failure (F) in 30 (51%), 10 (17%), and 12 (21%) patients, respectively. There was a 10% (3 of 30 patients) mortality rate in those at risk, 30% (3 of 10 patients) in those with injury, and 33% (4 of 12 patients) in those with failure. AKI (injury and failure groups) was significantly associated with increased in-hospital mortality. CONCLUSION: Development of AKI (injury or failure) is a significant risk factor associated with in-hospital mortality. Our study highlights the need to consider both urine output as well as creatinine-based components of the pRIFLE criteria to define AKI. LEVEL OF EVIDENCE: Prognostic and epidemiological study, level II.

Cystatin C and neutrophil gelatinase-associated lipocalin as markers of renal function in pediatric heart transplant recipients.

We hypothesized that use of Schwartz formula underestimates the prevalence of CKD in PHT recipients. This study determined the prevalence and risk factors for CKD in PHT using novel methods-serum cystatin C, CKiD formula, Revised Schwartz formula, s- and u-NGAL. Serum BUN, creatinine, cystatin C and s- and u-NGAL were measured after prospective enrollment. Schwartz formula GFR was compared with novel methods. CKD was defined as CKiD GFR < 90 mL/min/1.73 m(2) . The s- and u-NGAL were compared between those with and without CKD. Potential risk factors for CKD were analyzed. Seventy-nine patients (46 male children or boys), mean age 9.9 ± 5.8 yr formed the study cohort. The prevalence of mild and moderate CKD was 2- to 3-fold higher using novel methods compared to Schwartz formula. u-NGAL and u-NGAL/Cr were significantly higher in patients with CKD. u- and s-NGAL had negative correlation with estimates of GFR. Women were at a higher risk for CKD (odds ratio 8.7) as was longer duration since transplant (p = 0.009). In conclusion, use of novel methods of GFR estimation unmasked 2- to 3-fold increased prevalence of CKD in PHT. Women and those with longer duration since transplant are at higher risk for CKD.

Bone mineral status in pediatric heart transplant recipients: a retrospective observational study of an "at risk" cohort.

There is a paucity of literature assessing the burden of bone loss in PHT recipients. We sought to describe the bone mineral status in PHT recipients by doing a retrospective medical record review of those who underwent evaluation of BMD when clinically indicated. Data collected included patient demographics, BMD evaluations, serum calcium, phosphorus, alkaline phosphatase, cumulative steroid dose, osseous complications and their management. Of 149 PHT recipients, 26 underwent BMD evaluation. This evaluation was done at a median of 3.4 yrs after PHT. There total serum calcium, phosphorus and alkaline phosphatase were similar at transplant and BMD study. The median BMD Z-scores were: whole body -0.09 (1.5 to -5.13) and lumbar spine -1.1 (1.5 to -5.16). Bone loss (Z-score <-1) was present in 14 (53.8%). Three patients had spinal fractures and/or avascular necrosis of various bones. Treatment included calcitrol and bisphosphonates; and vertebroplasty for spinal fracture. Bone loss was present in a significant proportion of PHT recipients and may be associated with fractures and avascular necrosis. More than half of our "at risk" cohort had bone loss. Careful surveillance of these patients should be performed to prevent morbidity.

Incorporating the ACGME educational competencies into morbidity and mortality review conferences.

BACKGROUND: The Accreditation Council for Graduate Medical Education (ACGME) mandates that residents be trained in six core educational competencies. Practice-based learning and improvement (PBLI), one of the six competencies, is defined as the investigation and evaluation of one's own patient care. Morbidity and Mortality Conference, a frequently used venue to review the clinical outcome of hospitalized patients, provides the opportunity to teach and assess PBLI. DESCRIPTION: We report an approach to Morbidity and Mortality Conference that includes a systematic analysis of the ACGME core competencies and their application to a clinical case, a regular review of the factors that defines high-quality patient care, and a focused discussion of the PBLI competency. EVALUATION: Preliminary data indicate that our residents preferred this revised method for conducting Morbidity and Mortality Conference. CONCLUSION: Our adaptation to Morbidity and Mortality Conference provides a systematic review of the core competencies and their relevance to clinical decision making, with the ultimate goal of improving patient care.

The development of a competency-based assessment rubric to measure resident milestones.

BACKGROUND: The outcomes-based assessment rubric is a novel systematic instrument for documenting improvement in clinical learning. APPROACH: This article describes the development of a rubric aimed at introducing specific performance indicators to measure the Accreditation Council for Graduate Medical Education competencies. RESULTS: The potential benefits and implications for medical education include specifying performance indicators and outcomes, ensuring that assessment is coherent and consistent for all residents, measuring resident outcomes based on real-life criteria, providing opportunities for residents to demonstrate proficiency in a specific competency and outcome level, and improving the quality of assessment.

Laparoscopic Tenckhoff catheter placement in children using a securing suture in the pelvis: comparison to the open approach.

BACKGROUND/PURPOSE: Secure placement of peritoneal dialysis (PD) catheters in the pelvis has been described by various techniques. We describe minimally invasive placement using an Endo Close device, securing the catheter in the pelvis, and compare this method with standard open technique in children. METHODS: A retrospective institutional review was conducted for children requiring PD access from 2001 to 2007. Patients were grouped into laparoscopic with secure placement (SP) and open placement (OP) groups. Groups were cohort-matched based on age, paying particular attention to the number of catheter migrations. RESULTS: Twenty-seven patients underwent 36 procedures in SP, whereas 23 patients in OP had 32 catheter-related procedures. Exit site infections were decreased in SP (0.57 vs 1.33 episodes per patient-year). There was no difference in the number of catheter migrations (3 vs 5); however, time to migration was statistically longer in the SP group (9 vs. 2.4 months, P < .05). CONCLUSIONS: Laparoscopic placement of PD catheters using a securing suture in the pelvis is a more durable technique when compared to open placement. Extending the catheter migration time is important in children when PD is used as a bridge to renal transplantation.

Factors related to long-term renal transplant function in children.

Short-term renal allograft survival in children has improved. It is therefore important to determine the factors leading to long-term graft function. To this end, we evaluated patients in the NAPTRCS registry who were <12 years old when they received their renal transplant between 1987 and 1993. Children with 10 years of post-transplant follow-up were compared to those in whom the transplant failed within 10 years. Children with a failed transplant within 10 years of the surgery tended to be older, female, and non-Caucasian; they also manifested obstructive uropathy less often and had focal segmental glomerulosclerosis more often, and they received more deceased donor kidneys. Children with a failed renal transplant had fewer HLA donor and recipient matches, received pre-transplant dialysis compared to a preemptive transplant, required dialysis in the first week post-transplant, and required more antihypertensives the first month post-transplant. Allograft function was examined at 10 years. Patients with continued allograft function and a serum creatinine

Defining family-centered rounds.

BACKGROUND: Physicians are required to provide safe, effective, and high-quality care that is patient-centered. Continuing to meet the educational needs of residents and medical students in the setting of patient-centered care will require developing new models for hospital "work rounds." Family-centered rounds is a model of communicating and learning between the patient, family, medical professionals, and students on an academic, inpatient ward setting. Unfortunately, in the medical literature, there is no consensus on the definition of family-centered rounds. SUMMARY: Despite the increased utilization of hospitalists and the recognition that bedside teaching has many benefits, bedside rounds are underutilized. In this article, we present a description of family-centered rounds that is supported by a review of the literature on bedside teaching, family-centered care, and interdisciplinary care. The key difference between family-centered rounds and traditional bedside teaching is the active participation of the patient and family in the discussion. Interdisciplinary care implies that professionals from a variety of disciplines work collaboratively to develop a unified care plan. Family-centered rounding provides an interface between families and medical professionals that allows education of medical students and residents as well as the development of a unified care plan. CONCLUSIONS: Family-centered rounds hold potential to create a patient-centered environment, enhance medical education, and improve patient outcomes. The model is a planned, purposeful interaction that requires the permission of patients and families as well as the cooperation of physicians, nurses, and ancillary staff.

Determinants of renal function in pediatric heart transplant recipients: long-term follow-up study.

BACKGROUND: Renal insufficiency (RI) is a known complication in heart transplant recipients. We sought to determine the prevalence and risk factors for RI in pediatric heart transplant recipients over a long-term follow-up period. METHODS: The study cohort included 77 pediatric heart transplant recipients (35 girls, 18 African Americans) who had a minimum follow-up of 1 year. Data were obtained from pre-transplant evaluations and at 1, 6 and 12 months post-transplant and annually thereafter. Factors evaluated for their influence on renal function included duration of listing, age at transplant, gender, race, cardiac diagnosis, use of assist devices, inotropic support, rejection episodes and use of calcineurin inhibitors. RESULTS: The median age at transplant was 2 years, with a median follow-up duration of 5.1 years. RI was prevalent in 33% pre-transplant, and in 17%, 21% and 25.9% at 1, 3 and 5 years post-transplant, respectively. Two patients developed end-stage renal disease requiring long-term dialysis, with 1 eventually receiving a renal transplant. Significant risk factors for RI were African-American race (p = 0.04), younger age at transplant (p = 0.007), duration of listing (p < 0.0001) and calcineurin inhibitor level (p = 0.003). RI at 6 months post-transplant predicted chronic kidney disease at 5 years (odds ratio = 9). CONCLUSIONS: The prevalence of RI increased during a median follow-up of 5 years in this pediatric heart transplant cohort. African-American race, younger age at transplant, longer duration of listing, high level of calcineurin inhibitors and RI at 6 months were important determinants of RI. These patients should be followed-up carefully with early referral to a pediatric nephrologist if they develop chronic kidney disease.

Disposition of ceftazidime after intraperitoneal administration in adolescent patients receiving continuous cycling peritoneal dialysis.

BACKGROUND: Peritonitis is the single most common complication in children maintained on continuous cycling peritoneal dialysis (CCPD) and a significant cause of morbidity. Recent consensus guidelines for the treatment of peritonitis in children receiving peritoneal dialysis recommend the combined intraperitoneal administration of ceftazidime with either a first-generation cephalosporin or vancomycin. The objective of this study is to determine whether a continuous maintenance dose of intraperitoneal ceftazidime in the absence of a loading dose would maintain adequate serum and dialysate concentrations to be effective in the treatment of peritonitis. METHODS: Five ambulatory patients on CCPD therapy were studied with continuous intraperitoneal administration of ceftazidime (125 mg/L). Blood, dialysate, and urine samples were collected at specified intervals during a 24-hour period. Ceftazidime concentrations were measured by using a high-performance liquid chromatography assay. RESULTS: Mean serum concentrations at completion of the short rapid cycles and at 24 hours were 28.92 +/- 13.64 and 23.92 +/- 11.93 microg/mL, respectively. Serum bioavailability at 24 hours was 74% +/- 6%. Mean dialysate concentrations at completion of the short rapid cycles and at 24 hours were 87.43 +/- 19.18 and 32.06 +/- 6.27 microg/mL, respectively. All 5 patients achieved serum and dialysate ceftazidime concentrations greater than the mean inhibitory concentration within 4 hours. CONCLUSION: In adolescent patients on CCPD therapy, a continuous maintenance dose of intraperitoneal ceftazidime in the absence of a loading dose achieves serum and dialysate levels greater than the mean inhibitory concentration of sensitive organisms within 4 hours that persist for 24 hours.

Adequacy of peritoneal dialysis in children following cardiopulmonary bypass surgery.

Acute renal failure requiring renal replacement therapy can complicate cardiopulmonary bypass in children. Peritoneal dialysis has been shown to stabilize electrolytes and improve fluid status in these patients. To assess dialysis adequacy in this setting, we prospectively measured Kt/V and creatinine clearance in five patients (6-839 days of age) requiring renal replacement therapy at our institution. Median dialysis creatinine clearance was 74.25 L/week/1.73m(2) (range 28.28-96.63 L/week/1.73m(2)). Residual renal function provided additional solute clearance as total creatinine clearance was 215.97 L/week/1.73m(2) (range 108.04-323.25 L/week/1.73m(2)). Dialysis Kt/V of >2.1 (median 4.84 [range 2.12-5.59]) was achieved in all patients. No dialysis-associated complications were observed. We conclude that peritoneal dialysis is a safe, simple method of providing adequate clearance in children who develop acute renal failure following exposure to cardiopulmonary bypass.

Hyperphosphatemia in children receiving peritoneal dialysis--an educational program.

Hyperphosphatemia has been associated with the development of secondary hyperparathyroidism, renal osteodystrophy, cardiovascular calcification and increased risk of death. Using a one group, pretest-posttest study design, we sought to evaluate changes in serum phosphorus, calcium, parathyroid hormone and subject knowledge in response to a 3-month educational intervention. Mean serum phosphorus levels did not differ significantly between the three evaluation periods: pretreatment (5.69 mg/dl), treatment (5.84 mg/dl) and posttreatment (6.17 mg/dl). Mean serum calcium, calcium-phosphorus product and PTH values also did not differ significantly between the treatment periods. We observed no difference between the subject test scores between the two testing periods. Calcium-phosphorus product, serum phosphorus and PTH values on average, despite the education program, remained outside the K/DOQI target guidelines 44, 56 and 81% of the time, respectively. The results of this study suggest that an aggressive 3-month patient education program targeting dietary phosphorus knowledge, phosphate binder name and dosing, and knowledge of medical consequences associated with non-compliance had no effect on the serum phosphorus, calcium, PTH or phosphate binder need.

PKD1 intron 21: triplex DNA formation and effect on replication.

Although autosomal dominant polycystic kidney disease is transmitted in an autosomal dominant fashion, there is evidence that the pathophysiology of cystogenesis involves a second hit somatic mutation superimposed upon the inherited germline mutation within the renal tubule cells. The polypurine.polypyrimidine (Pu.Py) tract of PKD1 intron 21 may play a role in promoting somatic mutations. To better characterize this tract and to evaluate its potential to participate in mutagenesis, we investigated the thermodynamics of intramolecular triplex formation by 15 Pu.Py mirror repeat tracts from PKD1 intron 21 by 2D gel electrophoresis. We demonstrate that intramolecular triplexes form with modest superhelical tensions for all the tracts examined. Primer extension studies demonstrated significant polymerase arrest within the Pu.Py tracts in one direction of replication only. We found correlation between polymerization arrest and both the potential length of the triplex and superhelical tension of intramolecular triplex formation. The presence of a Pu.Py tract also led to a replication blockade and double-strand breakage using an SV40 in vitro replication assay with HeLa cell extracts. During DNA replication, the G-rich template of the PKD1 Pu.Py tracts may form a triplex structure with the nascent strand, thereby blocking replication and potentially leading to recombination and mutation.

Use of appendix for complete transplant ureteral necrosis.

A 3-yr-old boy with posterior urethral valves underwent cadaveric renal transplant. On the ninth day after transplantation the patient developed a urinary leak, with complete ureteral necrosis. There was insufficient length of undamaged ureter to permit ureteroneocystostomy, unavailability of a native ureter to permit ureteroureterostomy, and an inability to mobilize the transplant kidney or bladder sufficiently to permit direct pyelovesicostomy. As the kidney was otherwise functioning perfectly, we decided to create an appendiceal conduit in the hope of salvaging the patient's renal allograft. At present, 7 months post-transplant, the child is clinically well with a serum creatinine of 0.7 mg/dL. Complete ureteral necrosis is an infrequent but devastating complication following renal transplantation. We report a novel method that allowed an otherwise normally functioning cadaveric graft to be salvaged.