Barbara Bush Children’s Hospital

BACKGROUND: Doxorubicin chemotherapy is very effective in children with acute lymphoblastic leukemia (ALL) but also injures myocardial cells. Dexrazoxane, a free-radical scavenger, may protect the heart from doxorubicin-associated damage. METHODS: To determine whether dexrazoxane decreases doxorubicin-associated injury of cardiomyocytes, we randomly assigned 101 children with ALL to receive doxorubicin alone (30 mg per square meter of body-surface area every three weeks for 10 doses) and 105 to receive dexrazoxane (300 mg per square meter) followed immediately by doxorubicin. Serial measurements of serum cardiac troponin T were obtained in 76 of 101 patients in the doxorubicin group and 82 of 105 patients in the group given dexrazoxane and doxorubicin. A total of 2377 serum samples (mean, 15.1 samples per patient) were obtained before, during, and after treatment with doxorubicin. Troponin T levels were evaluated in a blinded fashion to determine whether they were elevated (>0.01 ng per milliliter)--the primary end point--or extremely elevated (>0.025 ng per milliliter). RESULTS: Elevations of troponin T occurred in 35 percent of the patients (55 of 158). Patients treated with doxorubicin alone were more likely than those who received dexrazoxane and doxorubicin to have elevated troponin T levels (50 percent vs. 21 percent, P<0.001) and extremely elevated troponin T levels (32 percent vs. 10 percent, P<0.001). The median follow-up was 2.7 years. The rate of event-free survival at 2.5 years was 83 percent in both groups (P=0.87 by the log-rank test). CONCLUSIONS: Dexrazoxane prevents or reduces cardiac injury, as reflected by elevations in troponin T, that is associated with the use of doxorubicin for childhood ALL without compromising the antileukemic efficacy of doxorubicin. Longer follow-up will be necessary to determine the influence of dexrazoxane on echocardiographic findings at four years and on event-free survival.

Child life programs are an important component of pediatric hospital-based care; they address the psychosocial concerns that accompany hospitalization and other health care experiences. Child life specialists focus on the optimal development and well-being of infants, children, adolescents, and young adults while promoting coping skills and minimizing the adverse effects of hospitalization, health care encounters, and/or other potentially stressful experiences. In collaboration with the entire health care team and family, child life specialists provide interventions that include therapeutic play, expressive modalities, and psychological preparation to facilitate coping and normalization at times and under circumstances that might otherwise prove overwhelming for the child. Play and developmentally appropriate communication are used to (1) promote optimal development, (2) educate children and families about health conditions, (3) prepare children and partner with families for medical events or procedures, (4) plan and rehearse useful coping and pain-management strategies with patients and families, (5) help children work through feelings about past or impending experiences, and (6) partner with families to establish therapeutic relationships between patients, siblings, and caregivers. Child life specialists collaborate with the entire interdisciplinary team to promote coping and enhance the overall health care experience for patients and families.

UNLABELLED: There is significant variation in diagnostic testing and treatment for inflammatory bowel disease. Quality improvement science methods can help address unwarranted variations in care and outcomes. METHODS: The ImproveCareNow Network was established under the sponsorship of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the American Board of Pediatrics as a prototype for a model of improving subspecialty care that included three components: 1) creating enduring multicenter collaborative networks of pediatric subspecialists, 2) sharing of performance data collected in patient registries, and 3) training in quality improvement. The network began with a focus on improving initial diagnostic testing and evaluation, the classification of the severity and extent of disease, the detection and treatment of inadequate nutrition and growth, and the appropriate dosing of immunomodulator medications. Changes are based on an evidence-based model of chronic illness care involving the use of patient registries for population management, previsit planning, decision support, promoting self-management, and auditing of care processes. RESULTS: Currently, patients are being enrolled at 23 sites. Through 2009, data have been analyzed on over 2500 patients from over 7500 visits. Initial results suggest improvements in both care processes (e.g., appropriate medication dosing and completion of a classification bundle that includes the patient's diagnosis, disease activity, distribution and phenotype, growth status, and nutrition status) and outcomes (e.g., the percentage of patients in remission). CONCLUSIONS: These improvements suggest that practice sites are learning how to apply quality improvement methods to improve the care of patients.

The incidence and predictors of premature physeal closure (PPC) after pediatric distal tibial fractures were investigated. PPC was defined as evidence of growth plate disturbance on the injured side compared with the uninjured side. Ninety-two fractures were reviewed with at least 1 year of follow-up, or until physiologic closure of the growth plates. Twenty-five fractures (27.2%) were complicated by PPC, as confirmed by CT scan in most cases. Salter-Harris III and IV (medial malleolar type) fractures resulted in the highest percentage of PPC by fracture type (38%). Salter-Harris I and II fractures resulted in PPC in 36% of cases, followed by triplane fractures (21%) and Tillaux fractures (0%). Initial displacement, number of reduction attempts, or treatment method did not significantly affect the incidence of PPC. More anatomic reductions resulted in a statistically significant decrease in PPC rates. Residual physeal gap (>3 mm) following reduction was determined from radiographs in Salter-Harris I and II fractures. If a residual gap was seen on the radiograph, the incidence of PPC increased to 60%; if no gap was present, the incidence decreased to 17%. Open reduction was performed in five Salter-Harris II fractures that had a residual gap. Periosteum was entrapped in the physis in all of these cases. Residual gaps in the physis following closed reduction may represent entrapped periosteum in Salter-Harris I and II fractures. This can lead to a higher incidence of PPC, suggesting that open reduction and removal of the entrapped periosteum may be beneficial.

PURPOSE: The cognitive sequelae of treatment for childhood acute lymphoblastic leukemia (ALL) were compared in a group of patients who received dexamethasone during the intensification and maintenance phases of therapy with those in a historical control group for whom antileukemia therapy was similar, except that the corticosteroid component of therapy was prednisone. METHODS: Patients treated for ALL on Dana-Farber Cancer Institute protocols 87-01 (n = 44) and 91-01 (n = 23) were evaluated by standard cognitive and achievement tests. Corticosteroid therapy was delivered in 5-day pulses given every 3 weeks during intensification and continuation phases of therapy for a total of 2 years. RESULTS: Children treated on protocol 87-01 received prednisone at a dose of 40 mg/m2/d (standard risk, SR) or 120 mg/ m2/d (high risk, HR); those treated on protocol 91-01 received dexamethasone at a dose of 6 mg/m2 per day (SR) or 18 mg/m2 per day (HR). Children treated on protocol 91-01 performed less well on cognitive testing. Subsample analysis indicated that cranial radiation therapy and methotrexate dose did not account for differences in cognitive outcomes. CONCLUSIONS: The findings of this preliminary study are consistent with the hypothesis that dexamethasone therapy can increase risk for neurocognitive late effects in children treated for ALL and indicate that further investigation of this question is warranted.

BACKGROUND: The authors report the occurrence of fatal or near-fatal sepsis in 16 of 38 children with newly diagnosed acute lymphoblastic leukemia (ALL) treated with a new induction regimen that differed from its predecessor by the substitution of dexamethasone for prednisone. METHODS: The frequency of septic deaths among 38 children who received multiagent remission induction therapy, including dexamethasone (6 mg/m(2)) daily for 28 days (pilot protocol 91-01P), was compared with the frequency of septic deaths among children previously treated (protocol 87-01) and subsequently treated (protocol 91-01) in consecutive Dana-Farber Cancer Institute (DFCI) ALL trials with induction therapy that included 21 and 28 days of prednisone (40 mg/m(2)), respectively. Except for dexamethasone in protocol 91-01P, the remission induction agents used were identical in substance to those used in protocol 87-01. Protocol 91-01, the successor 91-01P, was also similar, with the exception of the deletion of a single dose of L-asparaginase. RESULTS: Sixteen of the 38 children (42%) treated on the DFCI 91-01P had documented gram positive or gram negative sepsis (17 episodes) during remission induction, including 4 toxic deaths (11%). In contrast, there were 4 induction deaths among 369 children (1%) treated on protocol 87-01 (P = 0.0035) and 1 induction death among 377 children (<1%) treated on protocol 91-01 (P = 0.0003). CONCLUSIONS: Substitution of dexamethasone for prednisone or methylprednisolone in an otherwise intensive conventional induction regimen for previously untreated children with ALL resulted in an alarmingly high incidence of septic episodes and toxic deaths. Awareness of this complication, considering that the substitution has no apparent benefit in the efficacy of remission induction, argues against its routine use in intensive induction regimens for children with ALL.

PURPOSE: To evaluate outcome and assess complications in children and adolescents with low-risk Hodgkin's disease treated with vinblastine, doxorubicin, methotrexate, and prednisone (VAMP) chemotherapy and low-dose, involved-field radiation therapy (IFRT). PATIENTS AND METHODS: One hundred ten children with low-risk Hodgkin's disease were treated with four cycles of VAMP and 15 Gy IFRT for those who achieved a complete response (CR) or 25.5 Gy for those with a partial response after two cycles of VAMP. RESULTS: With median follow-up of 9.6 years (range, 1.7 to 15.0), 5- and 10-year overall survival were 99.1% and 96.1%, respectively, and 5-and 10-year event-free survival (EFS) were 92.7% and 89.4%. Factors contributing to 10-year EFS were: early CR (P = .02), absence of B symptoms (P = .01), lymphocyte predominant histologic subtype (P = .04), and less than three initial sites of disease (P = .02). Organ toxicity has been limited to correctable hypothyroidism in 42% of irradiated patients, and one case of cardiac dysfunction. Seventeen healthy babies have been born to 106 survivors. There have been two malignant tumors: one thyroid cancer within the radiation therapy field and one Ewing's sarcoma outside the radiation therapy field. CONCLUSION: Risk-adapted, combined-modality therapy using VAMP chemotherapy with radiation is effective and well tolerated. Pediatric patients with low-risk Hodgkin's disease can be cured with therapy without an alkylating agent, bleomycin, etoposide, or high-dose, extended-field radiotherapy. Thus, these children are expected to retain normal fertility, organ function, and be at low risk of a second malignant tumor.

A range of phenotypes including Greig cephalopolysyndactyly and Pallister-Hall syndromes (GCPS, PHS) are caused by pathogenic mutation of the GLI3 gene. To characterize the clinical variability of GLI3 mutations, we present a subset of a cohort of 174 probands referred for GLI3 analysis. Eighty-one probands with typical GCPS or PHS were previously reported, and we report the remaining 93 probands here. This includes 19 probands (12 mutations) who fulfilled clinical criteria for GCPS or PHS, 48 probands (16 mutations) with features of GCPS or PHS but who did not meet the clinical criteria (sub-GCPS and sub-PHS), 21 probands (6 mutations) with features of PHS or GCPS and oral-facial-digital syndrome, and 5 probands (1 mutation) with nonsyndromic polydactyly. These data support previously identified genotype-phenotype correlations and demonstrate a more variable degree of severity than previously recognized. The finding of GLI3 mutations in patients with features of oral-facial-digital syndrome supports the observation that GLI3 interacts with cilia. We conclude that the phenotypic spectrum of GLI3 mutations is broader than that encompassed by the clinical diagnostic criteria, but the genotype-phenotype correlation persists. Individuals with features of either GCPS or PHS should be screened for mutations in GLI3 even if they do not fulfill clinical criteria.

OBJECTIVE: To test the efficacy and safety of combining intravenous iron in amounts approximating the in utero iron accretion rate and the postnatal iron loss with erythropoietin (EPO) in very low birth weight (VLBW) infants. METHODS: A prospective, controlled, randomized, unmasked trial lasting 21 days was performed in 29 clinically stable VLBW infants <31 weeks' gestation and <1300 g birth weight not treated with red blood cell transfusions during the study period. Mean (+/- standard error of the mean) age at study entry was 23 +/- 2.9 days. After a 3-day run-in baseline period in which all participants received oral supplements of 9 mg/kg/day of iron polymaltose complex (IPC), participants were randomized to receive 18 days of treatment with: 1) oral IPC alone (oral iron group); 2) 300 U of recombinant human EPO (r-HuEPO) kg/day and daily oral IPC (EPO + oral iron group); 3) 2 mg/kg/day of intravenous iron sucrose, r-HuEPO, and oral iron (intravenous iron + EPO group). To assess efficacy of the 3 treatments, serial blood samples were analyzed for hemoglobin (Hb), hematocrit (Hct), reticulocyte count, red blood cell indices and plasma levels of transferrin, transferrin receptor (TfR), ferritin, and iron. Oxidant injury was assessed before and after treatment by plasma and urine levels of malondialdehyde (MDA) and o-tyrosine. RESULTS: At the end of treatment, Hb, Hct, reticulocyte count, and plasma TfR were markedly higher in both of the EPO-treated groups, compared with the oral iron group. At study exit a trend toward increasing Hb and Hct levels and significantly higher reticulocyte counts were observed in the intravenous iron + EPO group, compared with the EPO + oral iron group. During treatment, plasma ferritin levels increased significantly in the intravenous iron + EPO group and decreased significantly in the other 2 groups. By the end of treatment, ferritin levels were significantly higher in the intravenous iron + EPO group compared with the other 2 groups. Although plasma and urine MDA or o-tyrosine did not differ among the 3 groups, plasma MDA was significantly greater in the subgroup of intravenous iron + EPO participants sampled at the end of the 2-hour parenteral iron infusion, compared with values observed immediately before and after parenteral iron-dosing. CONCLUSIONS: In stable VLBW infants receiving EPO treatment, parenteral supplementation with 2 mg/kg/day of iron sucrose results in a small, but significant, augmentation of erythropoiesis beyond that of r-HuEPO and enteral iron alone. However, to reduce the potential adverse effects of parenteral iron/kg/day on increasing plasma ferritin levels and on causing oxidative injury, we suggest that the parenteral iron dose used should be reduced and/or the time of infusion extended to maintain a serum iron concentration below the total iron-binding capacity.

OBJECTIVE: Document the impact of Let's Go!, a multisetting community-based childhood obesity prevention program on participants in 12 communities in Maine. METHODS: The study used repeated random telephone surveys with 800 parents of children to measure awareness of messages and child behaviors. Surveys were conducted in schools, child care programs, and afterschool programs to track changes in policies and environments. RESULTS: Findings show improvements from 2007 to 2011: Children consuming fruits and vegetables increased from 18%, 95% CI [15, 21], to 26% [23, 30] (p < .001); children limiting sugary drinks increased from 63% [59, 67] to 69% [65, 73] (p = .011); and parent awareness of the program grew from 10% [7, 12] to 47% [43, 51] (p < .001). Participating sites implemented widespread changes to promote healthy behaviors. CONCLUSIONS: A multisetting, community-based intervention with a consistent message can positively impact behaviors that lead to childhood obesity.

BACKGROUND: Acute viral bronchiolitis is the most common diagnosis resulting in hospital admission in pediatrics. Utilization of non-evidence-based therapies and testing remains common despite a large volume of evidence to guide quality improvement efforts. OBJECTIVE: Our objective was to reduce utilization of unnecessary therapies in the inpatient care of bronchiolitis across a diverse network of clinical sites. METHODS: We formed a voluntary quality improvement collaborative of pediatric hospitalists for the purpose of benchmarking the use of bronchodilators, steroids, chest radiography, chest physiotherapy, and viral testing in bronchiolitis using hospital administrative data. We shared resources within the network, including protocols, scores, order sets, and key bibliographies, and established group norms for decreasing utilization. RESULTS: Aggregate data on 11,568 hospitalizations for bronchiolitis from 17 centers was analyzed for this report. The network was organized in 2008. By 2010, we saw a 46% reduction in overall volume of bronchodilators used, a 3.4 dose per patient absolute decrease in utilization (95% confidence interval [CI] 1.4-5.8). Overall exposure to any dose of bronchodilator decreased by 12 percentage points as well (95% CI 5%-25%). There was also a statistically significant decline in chest physiotherapy usage, but not for steroids, chest radiography, or viral testing. CONCLUSIONS: Benchmarking within a voluntary pediatric hospitalist collaborative facilitated decreased utilization of bronchodilators and chest physiotherapy in bronchiolitis.

OBJECTIVE: To evaluate the effect of a pediatric primary care-based intervention, on improved clinical decision support and family management of risk behaviors for childhood overweight. METHODS: An experimental field trial was conducted with 12 intervention sites in urban and rural areas of Maine and nonrandomized control sites. Change was assessed by using clinical and parent measures from 9 intervention and 10 control sites before and during the Maine Youth Overweight Collaborative intervention. Longitudinal information was collected from chart audits of patients aged 5-18 years (n = 600), systematic samples of parents collected before (n = 346) and during (n = 386) the intervention in 12 sites, and systematic samples of parents in 9 intervention (n = 235) and 10 control (n = 304) sites collected during the intervention. Surveys of health care providers (n = 14 and 17) before and during the intervention were also collected. Teams worked over 18 months to implement improvements in clinical decision support, including tracking BMI percentiles, identification of overweight patients, appropriate laboratory tests, counseling of families and patients use of a behavioral screening tool, and other improvements following the chronic-care model targeting patients aged 5 to 18 and their families. RESULTS: Large changes occurred in clinical practice from before to during the Maine Youth Overweight Collaborative: increases in assessment of BMI (38%-94%), BMI percentile for age and gender (25%-89%), use of the 5-2-1-0 behavioral screening tool (0%-82%), and weight classification (19%-75%). Parent surveys indicated improvements in providers' behavior and rates of counseling. Intervention providers reported improvements in knowledge, attitudes, self-efficacy, and practice. CONCLUSIONS: The Maine Youth Overweight Collaborative intervention improved clinical decision support and family management of risk behaviors, indicating a promising primary care-based approach to address overweight risk among children and youth.

Since the addition of tandem mass spectrometry (MS/MS) to the North Carolina Newborn Screening Program, 20 infants with two consecutive elevated 3-hydroxyisovalerylcarnitine (C5OH) levels have been evaluated for evidence of inborn errors of metabolism associated with this metabolite. Ten of these 20 infants had significant concentrations of both 3-hydroxyisovaleric acid and 3-methylcrotonylglycine in their urine, suggestive of 3-methylcrotonyl-CoA carboxylase (3-MCC) deficiency. Four of these 10 were infants whose abnormal metabolites were found to be of maternal origin. Of 8 patients with probable 3-MCC deficiency, 7 have been tested and found to have the enzyme deficiency confirmed in lymphoblasts or cultured fibroblasts; one of these 7 infants had only marginally decreased 3-MCC activity in lymphocytes but deficient 3-MCC in fibroblasts. We estimate the incidence of 3-MCC deficiency at 1:64000 live births in North Carolina. We conclude that MS/MS newborn screening will detect additional inborn errors of metabolism, such as 3-MCC deficiency, not traditionally associated with newborn screening. The evaluation of newborns with two abnormally elevated C5OH levels on MS/MS newborn screening should include, at least, urine organic acid analysis by capillary GC-MS and a plasma acylcarnitine profile by MS/MS. Long-term follow-up is needed to determine the outcome of presymptomatically diagnosed patients with 3-MCC deficiency by MS/MS newborn screening.

PURPOSE: To evaluate the efficacy of vinblastine, doxorubicin, methotrexate, and prednisone (VAMP) and cyclophosphamide, vincristine, and procarbazine (COP) chemotherapy and response-based, involved-field radiation, a combined-modality regimen that limits doses of alkylating agents, anthracyclines, and radiation, in children with advanced and unfavorable Hodgkin's disease. PATIENTS AND METHODS: From 1993 to 2000, 159 children and adolescents with unfavorable Hodgkin's disease received three alternating cycles (total of six cycles) of VAMP/COP chemotherapy followed by response-based, involved-field radiation therapy: 15 Gy was administered to patients achieving a complete response, and 25.5 Gy was administered to those achieving a partial response after the first two cycles of chemotherapy and to all sites of bulky lymphadenopathy. Unfavorable disease was defined as clinical stage I and II with bulky peripheral nodal disease greater than 6 cm, initial bulky mediastinal mass 33% or more of the intrathoracic diameter, and/or "B" symptoms and all stage III and IV. RESULTS: Study enrollment was closed after an interim analysis estimated a 5-year event-free survival (EFS) rate below a predefined level. Disease presentation was localized (stage I/II) in 77 patients (48.4%) and advanced (stage III/IV) in 82 patients (51.6%). At a median follow-up of 5.8 years (range, 1.3 to 10.0 years), 38 patients had events, including relapse/progression (n = 35), second malignancy (n = 2), and accidental death (n = 1); nine relapses (25.7%) occurred greater than 4 years from diagnosis. Five-year survival and EFS estimates are 92.7% +/- 2.5% and 75.6% +/- 4.1%, respectively. CONCLUSION: Risk-adapted combined-modality therapy with VAMP/COP and response-based, involved-field radiation therapy results in an unsatisfactory outcome for pediatric patients with unfavorable presentations of Hodgkin's disease.

PURPOSE: Haploinsufficiency of USP7, located at chromosome 16p13.2, has recently been reported in seven individuals with neurodevelopmental phenotypes, including developmental delay/intellectual disability (DD/ID), autism spectrum disorder (ASD), seizures, and hypogonadism. Further, USP7 was identified to critically incorporate into the MAGEL2-USP7-TRIM27 (MUST), such that pathogenic variants in USP7 lead to altered endosomal F-actin polymerization and dysregulated protein recycling. METHODS: We report 16 newly identified individuals with heterozygous USP7 variants, identified by genome or exome sequencing or by chromosome microarray analysis. Clinical features were evaluated by review of medical records. Additional clinical information was obtained on the seven previously reported individuals to fully elucidate the phenotypic expression associated with USP7 haploinsufficiency. RESULTS: The clinical manifestations of these 23 individuals suggest a syndrome characterized by DD/ID, hypotonia, eye anomalies,feeding difficulties, GERD, behavioral anomalies, and ASD, and more specific phenotypes of speech delays including a nonverbal phenotype and abnormal brain magnetic resonance image findings including white matter changes based on neuroradiologic examination. CONCLUSION: The consistency of clinical features among all individuals presented regardless of de novo USP7 variant type supports haploinsufficiency as a mechanism for pathogenesis and refines the clinical impact faced by affected individuals and caregivers.

There is little research on psychosocial factors and insulin pump use in adolescents. The purpose of the present study was to use qualitative and quantitative methods to explore psychosocial issues related to insulin pump use [continuous subcutaneous insulin infusion (CSII)] in youth. Eighteen early adolescents and their parents were interviewed about the experience of using an insulin pump, and transcripts were content-analyzed. In general, interviewees reported modest improvements in glycemic control with initiation of CSII. Teens and parents did, however, report high levels of satisfaction with pump therapy and increased adolescent responsibility for the diabetes regimen. In addition, pump users reported few issues related to body image, appearance, or social aspects of pump dependency. Chief concerns were related to the demands of initiating pump therapy, pump alarms and malfunctions, potential for regimen non-compliance with CSII, and school-related issues. Pump use is not associated with social difficulties, and, in general, youths and their parents report high satisfaction with CSII.

SATB2-associated syndrome (SAS) is an autosomal dominant disorder characterized by significant neurodevelopmental disabilities with limited to absent speech, behavioral issues, and craniofacial anomalies. Previous studies have largely been restricted to case reports and small series without in-depth phenotypic characterization or genotype-phenotype correlations. Seventy two study participants were identified as part of the SAS clinical registry. Individuals with a molecularly confirmed diagnosis of SAS were referred after clinical diagnostic testing. In this series we present the most comprehensive phenotypic and genotypic characterization of SAS to date, including prevalence of each clinical feature, neurodevelopmental milestones, and when available, patient management. We confirm that the most distinctive features are neurodevelopmental delay with invariably severely limited speech, abnormalities of the palate (cleft or high-arched), dental anomalies (crowding, macrodontia, abnormal shape), and behavioral issues with or without bone or brain anomalies. This comprehensive clinical characterization will help clinicians with the diagnosis, counseling and management of SAS and help provide families with anticipatory guidance.

Journal of Hospital MedicineVolume 15, Issue 8 p. 507-509 Perspectives in Hospital Medicine Collateral Damage: How COVID-19 Is Adversely Impacting Women Physicians Yemisi Jones MD, Corresponding Author Yemisi Jones MD [email protected] Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio Cincinnati Children's Hospital Medical Center, Cincinnati, OhioCorresponding Author: Yemisi Jones, MD; Email: [email protected]; Telephone: 412-965-9630; Twitter: @YJonesMD.Search for more papers by this authorVanessa Durand DO, Vanessa Durand DO Department of Pediatrics, Drexel University College of Medicine, Philadelphia, Pennsylvania Section of Hospital Medicine, St. Christopher's Hospital for Children, Philadelphia, PennsylvaniaSearch for more papers by this authorKayce Morton DO, Kayce Morton DO Department of Pediatrics, University of Missouri School of Medicine, Columbia, MissouriSearch for more papers by this authorMary Ottolini MD, MPH, MEd, Mary Ottolini MD, MPH, MEd Department of Pediatrics, Tufts University School of Medicine, Boston, Massachusetts Department of Pediatrics, The Barbara Bush Children's Hospital, Maine Medical Center, Portland, MaineSearch for more papers by this authorErin Shaughnessy MD, MSHCM, Erin Shaughnessy MD, MSHCM Department of Pediatrics, University of Arizona College of Medicine–Phoenix, Phoenix, Arizona Division of Hospital Medicine, Phoenix Children's Hospital, Phoenix, ArizonaSearch for more papers by this authorNancy D Spector MD, Nancy D Spector MD Department of Pediatrics, Drexel University College of Medicine, Philadelphia, Pennsylvania Faculty Development, Drexel University College of Medicine, Philadelphia, PennsylvaniaSearch for more papers by this authorJennifer O'Toole MD, MEd, Jennifer O'Toole MD, MEd Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio University of Cincinnati Medical Center, Cincinnati, OhioSearch for more papers by this authoron behalf of the ADVANCE PHM Steering Committee, on behalf of the ADVANCE PHM Steering CommitteeSearch for more papers by this author Yemisi Jones MD, Corresponding Author Yemisi Jones MD [email protected] Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio Cincinnati Children's Hospital Medical Center, Cincinnati, OhioCorresponding Author: Yemisi Jones, MD; Email: [email protected]; Telephone: 412-965-9630; Twitter: @YJonesMD.Search for more papers by this authorVanessa Durand DO, Vanessa Durand DO Department of Pediatrics, Drexel University College of Medicine, Philadelphia, Pennsylvania Section of Hospital Medicine, St. Christopher's Hospital for Children, Philadelphia, PennsylvaniaSearch for more papers by this authorKayce Morton DO, Kayce Morton DO Department of Pediatrics, University of Missouri School of Medicine, Columbia, MissouriSearch for more papers by this authorMary Ottolini MD, MPH, MEd, Mary Ottolini MD, MPH, MEd Department of Pediatrics, Tufts University School of Medicine, Boston, Massachusetts Department of Pediatrics, The Barbara Bush Children's Hospital, Maine Medical Center, Portland, MaineSearch for more papers by this authorErin Shaughnessy MD, MSHCM, Erin Shaughnessy MD, MSHCM Department of Pediatrics, University of Arizona College of Medicine–Phoenix, Phoenix, Arizona Division of Hospital Medicine, Phoenix Children's Hospital, Phoenix, ArizonaSearch for more papers by this authorNancy D Spector MD, Nancy D Spector MD Department of Pediatrics, Drexel University College of Medicine, Philadelphia, Pennsylvania Faculty Development, Drexel University College of Medicine, Philadelphia, PennsylvaniaSearch for more papers by this authorJennifer O'Toole MD, MEd, Jennifer O'Toole MD, MEd Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio University of Cincinnati Medical Center, Cincinnati, OhioSearch for more papers by this authoron behalf of the ADVANCE PHM Steering Committee, on behalf of the ADVANCE PHM Steering CommitteeSearch for more papers by this author First published: 20 July 2020 https://doi.org/10.12788/jhm.3470Citations: 38Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. 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1. Karl S. Roth, MD* 2. Barbara H. Amaker, MD* 3. James C.M. Chan, MD† 1. *Departments of Pediatrics and Pathology, Medical College of Virginia Campus, Virginia Commonwealth University, Richmond, VA 2. †Department of Pediatrics, The Barbara Bush Children’s Hospital at Maine Medical Center, Portland, ME After completing this article, readers should be able to: 1. Describe the signs and symptoms of minimal-change nephrotic syndrome. 2. Characterize the laboratory findings in children who have minimal-change nephrotic syndrome. 3. Plan a treatment program for a young child who has an initial episode of minimal-change nephrosis. 4. Recognize the major complications of minimal-change nephrotic syndrome. The estimated annual incidence of nephrotic syndrome in healthy children is 2 to 7 new cases per 100,000 children younger than 18 years of age, making it a relatively common major disease in pediatrics. The peak age of onset occurs at 2 to 3 years except for the rare, congenital type of nephrosis. Approximately 50% of affected children are ages 1 to 4 years; 75% are younger than age 10 years. In addition, even the most benign form of the nephrotic syndrome is, by nature, a recurrent disorder, so each new-onset case likely will continue to manifest disease for some time. Nephrotic syndrome is one of the most frequent reasons for referral to a pediatric nephrologist for evaluation, although its insidious onset frequently causes delay in diagnosis. Careful examination of the anatomy of a nephron permits characterization of the glomerular basement membrane as the barrier between the circulation and the external environment. Thus, the glomerular membrane, which permits passage in an adult of approximately 180 L/d of fluid, is the final determinant of how much of the solute originally contained in this volume enters the tubular lumen. The normal glomerular membrane is remarkably selective for protein compared with other solutes (Table 1⇓). Once this selectivity is lost, the ensuing proteinuria defines not only the diagnosis of nephrotic syndrome, but many pathophysiologic consequences as well. It is the purpose of this article to discuss the definition, causes, pathophysiologic consequences, and management of nephrotic syndrome. The emphasis is …

AIM: To evaluate currently available paediatric falls assessments instruments and to build a predictive fall model while also evaluating injury risk as a predictor of fall likelihood within the paediatric inpatient population. BACKGROUND: There is lack of paediatric-specific fall assessment instruments and little information on the exploration of injury risk as related to falls in hospitalized children. METHOD: An ambispective, matched case-control design conducted in a sample of 100 inpatient paediatric patients. Results Two out of five instruments performed well to classify children at risk of falls. Longer length of stay, bleeding cautions/blood disorders and temperament/behaviour issues were significant predictors of fall likelihood. Cognitive impairment or neurological disease was not related to an increased likelihood of fall or injury risk for this sample. CONCLUSIONS: More research is required to institute and standardize paediatric fall and injury risk assessments for everyday use. The explicit approach of using predictive modelling is critical in creating a universal, baseline reference for the most reliable and valid measure of assessment in children. IMPLICATIONS FOR NURSING MANAGEMENT: Findings of the present study increase awareness of nursing managers and leaders as to the necessity for fall and injury risk assessment as a safety and quality measure for inpatient paediatric populations.