Children's Hospital Of Pittsburgh Research Articles

Improving Access to Comprehensive Care for Pediatric Sickle Cell Patients through the Creation of Multispecialty Clinics

There are many barriers to health care for those in underserved populations such as the pediatric sickle cell population. Due to limitations of transportation, clinical availability, and lack of care coordination, pediatric sickle cell patients are at an increased risk of not receiving the extensive multi-subspecialty health care recommended to prevent potential sickle cell related complications that affect every organ system. To improve access to this multidisciplinary care, UPMC Children's Hospital of Pittsburgh (CHP) pediatric sickle cell program developed a multi-subspecialty collaborative clinic model to offer coordinated sickle cell and pulmonary care, which is critical for sickle cell patients due to the increased risk of pulmonary complications associated with sickle cell disease. By considering health care barriers experienced by our pediatric sickle cell population, we proposed that this coordinated subspecialty clinic model will increase compliance by offering patients and families the opportunity to receive synchronized care from two essential subspecialty groups in just one medical center trip. This increased access to preventative health care for the pediatric sickle cell population should contribute to decreased acute medical events that require critical care and other extensive resources within the health care system. Methods: Two adjacent clinic appointments within the sickle cell and pulmonology clinics were designated for sickle cell patients also requiring preventative pulmonary care to be seen consecutively by both clinical teams during the same half day (one in sickle cell clinic and one in pulmonary clinic). The effect of improving completed referrals using this multi-specialty clinic model was assessed by retrospective electronic medical record review comparing the number of pulmonology referrals made by SCD team and pulmonary visits completed pre- and post- coordinated multi-subspecialty clinic initiative. The studied time period prior to the multispecialty clinic creation was chosen to be calendar year 2019 in order to eliminate the variable of the COVID-19 pandemic affecting outpatient clinic participation. Post-intervention data were collected during calendar year 2022 soon after the creation of this multi-subspecialty initiative. Prior to the creation of this multi-subspecialty clinic model, coordinating CHP sickle cell and pulmonology care, only 24% of pediatric sickle cell patients referred for outpatient pulmonology consultation completed the clinical evaluation. Our program referred 53 of 168 total families during calendar year 2019, with only 17 patients completing their visit. Since beginning this multi-subspecialty clinic initiative, pulmonology evaluations completed by referred sickle cell patients increased to 55%. Thus, CHP's multispecialty clinic offering coordinated sickle cell and pulmonology care increased completed pulmonology referrals by 129%. Using Pearson's chi-square test to compare the relationship between the two categorical variables of prior- and post- multispecialty clinic formation with a null hypothesis of multispecialty clinics will not increase completed referrals, we found a value of 10.76, which is greater than the applied critical value of 9.21 (p=0.01). Therefore, these results are significant, and the null hypothesis was rejected. In summary, by establishing this multi-subspecialty coordinated care at CHP for our pediatric sickle cell population, our goal of improving compliance with critical preventive care was achieved. We hope that this type of intervention will enhance the quality of life for children with sickle cell disease currently, as well as contribute to better health in the patients' futures. We also predict that this improved preventive care will also contribute to decreased emergency care and extensive treatments required for patients cared by CHP. This multi-subspecialty care model also has the potential to benefit other pediatric patients with chronic diseases from underserved populations who experience similar barriers, both in CHP's Western Pennsylvania region as well as underserved populations and health care organizations beyond our region. By applying an approach to care focused on reducing health care barriers to underserved populations, impactful improvements may be seen not only in the pediatric sickle cell population, but in the health care system at large.

Epilepsy panel testing criteria: A clinical assessment.

Epilepsy is a common, and often genetic, neurological disorder. Few guidelines exist to help medical providers or insurance companies decide when to order or cover epilepsy panels for patients with epilepsy. The most recent guidelines were published by NSGC after this study's data collection. Since 2017, the Genetic Testing Stewardship Program (GTSP) at UPMC Children's Hospital of Pittsburgh (CHP) has been utilizing a set of internally developed epilepsy panel (EP) testing criteria to facilitate appropriate EP ordering practices. The purpose of this study was to assess these testing criteria by determining their sensitivities and positive predictive values (PPV). Retrospective chart review of the electronic medical record (EMR) was performed for 1242 CHP Neurology patients that were evaluated for a primary diagnosis of epilepsy between 2016 and 2018. One hundred and nine patients had EPs at various testing laboratories. Of the patients that met criteria, 17 had diagnostic EPs and 54 had negative EPs. Criteria were organized into category groupings (C1-C4), and analyzed alone for C1, in pairs for C2, etc. The highest sensitivity and PPV results in each category grouping were: C1 (64.7%, 60%); C2, (88%, 30.3%); C3, (94.1%, 27.1%); C4, (94.1%, 25.4%). Family history was crucial to increasing sensitivity. Confidence intervals (CIs) narrowed as category grouping level increased, though this was not statistically significant due to the considerable CI overlap across category groupings. The PPV from C4 was applied to the untested population cohort and predicted 121 patients with unidentified positive EPs. This study presents data supporting the predictive capabilities of EP testing criteria and suggests the addition of a family history criterion. This study impacts public health by encouraging the adoption of evidence-driven insurance policies and by suggesting guidelines to ease EP ordering and coverage decisions, which could potentially improve patient access to EP testing.

Interval Assessment of Beta-Lactam Antibiotic Allergy Status and De-labeling Process of Pediatric Patients in Children's Hospital of Pittsburgh between 2020 – 2022.

Beta-lactam allergy is associated with suboptimal medical care and increased healthcare expenses. We hypothesize that introducing a multidisciplinary institutional beta-lactam de-labeling protocol at Children's Hospital of Pittsburgh (CHP) would lead to increased percentage of de-labeling encounters.

Long-term liver transplant outcomes for progressive familial intrahepatic cholestasis type 1: The Pittsburgh experience.

Progressive familial intrahepatic cholestasis type 1 (PFIC1) arises from biallelic variants in the ATP8B1gene that annul FIC1 activity, resulting in progressive liver disease. Liver transplant (LT) is indicated in refractory disease; however, post-LT complications including worsening diarrhea and steatohepatitis progressing to fibrosis with graft loss have been reported. We aim to describe long-term outcomes of PFIC1 LT recipients at our center, focusing on the histological changes of the allografts. We assessed 7 PFIC1 patients post-LT at the Children's Hospital of Pittsburgh (CHP). All pre-transplant, explant, and sequential post-transplant pathology samples were reviewed. Continuous data are presented as the mean±SD. We compared the pre- and post-transplant height and weight z-scores using Wilcoxon signed-rank test. Seven (29% male) patients with PFIC1 received a LT (n=6) or had post-LT care (n=1) at CHP. Six had confirmed or suspected identical genetic. At a mean follow-up of 10.9years, both patient survival and graft survival were 100%. Diarrhea persisted (n=3) or newly developed (n=4) in all patients after LT contributing to ongoing growth failure, with mean z-scores -2.63 (weight) and -2.98 (height) at follow-up. Histologically, allograft steatosis was common but was not accompanied by significant inflammation, ballooning, or fibrosis. We show that extrahepatic disease persists and near-universal allograft steatosis occurs. However, at a mean follow-up period of over 10years, no patients developed steatohepatitis or significant fibrosis, and both patient survival and graft survival are excellent.

5. DO WELLNESS INTERVENTIONS IN A LARGE PEDIATRIC RESIDENCY PROGRAM IMPACT RESIDENT BURNOUT?

BackgroundThe ACGME has called upon programs to more directly address resident physician well-being. In recent years, the pediatric residency program at Children's Hospital of Pittsburgh (CHP) has implemented multiple interventions aimed at improving resident wellness. Examples include the addition of an elective to the intern year, incorporation of wellness weeks, and visits for interns with a clinical therapist. The program monitors individual resident burnout biannually using the Maslach Burnout Inventory (MBI), a validated survey which evaluates three domains: emotional exhaustion, depersonalization and personal accomplishment. The aim of this study was to evaluate the prevalence of burnout in pediatric residents, and to examine changes in burnout rates over time as the program has implemented wellness interventions.MethodsRetrospective analysis of MBIs completed by pediatric residents at CHP between July 2015 and July 2018 was performed. Burnout was defined as a high score in either emotional exhaustion or depersonalization. Data were analyzed using descriptive statistics.Results92 pediatric residents were included. Residents were excluded from sub-analyses if they did not complete MBIs for all the terms being analyzed. During the 2017-2018 academic year, 42% of residents experienced burnout. Following a cohort from the 2015 PGY-1 class (N=16), 80% of residents who were burned out during their intern year had burnout at later points in residency. Between 2015-2018, there was a decrease in PGY-1 burnout rates. Prevalence of burnout in PGY1s during the fall and spring terms was 40% and 45% in 2015/2016 (N=20), 27% and 40% in 2016/2017 (N=30), 22% and 28% in 2017/2018 (N=32).ConclusionsThe prevalence of burnout in pediatric residents at CHP was comparable to rates reported in the literature. When following a cohort of individuals through their residency, burnout seemed to persist over time. Burnout rates were higher for interns in the spring compared to the fall. With an increase in wellness interventions the program has seen a decrease in intern burnout over the past three years. The ACGME has called upon programs to more directly address resident physician well-being. In recent years, the pediatric residency program at Children's Hospital of Pittsburgh (CHP) has implemented multiple interventions aimed at improving resident wellness. Examples include the addition of an elective to the intern year, incorporation of wellness weeks, and visits for interns with a clinical therapist. The program monitors individual resident burnout biannually using the Maslach Burnout Inventory (MBI), a validated survey which evaluates three domains: emotional exhaustion, depersonalization and personal accomplishment. The aim of this study was to evaluate the prevalence of burnout in pediatric residents, and to examine changes in burnout rates over time as the program has implemented wellness interventions. Retrospective analysis of MBIs completed by pediatric residents at CHP between July 2015 and July 2018 was performed. Burnout was defined as a high score in either emotional exhaustion or depersonalization. Data were analyzed using descriptive statistics. 92 pediatric residents were included. Residents were excluded from sub-analyses if they did not complete MBIs for all the terms being analyzed. During the 2017-2018 academic year, 42% of residents experienced burnout. Following a cohort from the 2015 PGY-1 class (N=16), 80% of residents who were burned out during their intern year had burnout at later points in residency. Between 2015-2018, there was a decrease in PGY-1 burnout rates. Prevalence of burnout in PGY1s during the fall and spring terms was 40% and 45% in 2015/2016 (N=20), 27% and 40% in 2016/2017 (N=30), 22% and 28% in 2017/2018 (N=32). The prevalence of burnout in pediatric residents at CHP was comparable to rates reported in the literature. When following a cohort of individuals through their residency, burnout seemed to persist over time. Burnout rates were higher for interns in the spring compared to the fall. With an increase in wellness interventions the program has seen a decrease in intern burnout over the past three years.

Abstract A42: Prenatal role of medical radiation in children diagnosed with a blood cancer: Single-institution study, 2006-2016

Abstract Background: Prenatal exposure to medical radiation is an identified risk for childhood cancers, but is difficult to document. The author developed a family questionnaire and interview form to identify possible exposures in children diagnosed from 2006-2016 with a blood cancer at a single institution. The Children's Hospital of Pittsburgh historically in this ten-year period has seen 180-200 new cases of a pediatric cancer each year with approximately 35-40% of these cancers being a blood cancer, notably an acute leukemia, chronic leukemia, Hodgkins's lymphoma, or a non-Hodgkin's lymphoma. Method: Each converted family (approximately 30% of the cohort) has been interviewed at hospital, clinic, or through phone conversation who were diagnosed with a type of acute or chronic leukemia or lymphoma. All children were diagnosed/treated at the Children's Hospital of Pittsburgh (CHP). CHP is the tristate regional referral center which sees about 180-200 new cases of a pediatric cancer each year. Medical staff and psychosocial staff referred patient families for interview with the author. Results: Among the families interviewed, at least one medical radiation prenatally has been identified in over one-third of families. These exposures prominently included sinus, chest or abdominal x-rays, or CT in either patient. Conclusion: Exposures to medical radiation for a child later diagnosed with cancer may occur pre-conception, in utero, or in early childhood. These exposures may be part of the “perfect storm” which eventually results in a blood or other cancer in children. Documenting these exposures may possibly in future lead to more preventive measures pre-conception similar to the “image gently” initiative to limit the number of diagnostic procedures and radiation doses in CT imaging of children today. Citation Format: Robin L. Rohrer. Prenatal role of medical radiation in children diagnosed with a blood cancer: Single-institution study, 2006-2016 [abstract]. In: Proceedings of the AACR International Conference held in cooperation with the Latin American Cooperative Oncology Group (LACOG) on Translational Cancer Medicine; May 4-6, 2017; São Paulo, Brazil. Philadelphia (PA): AACR; Clin Cancer Res 2018;24(1_Suppl):Abstract nr A42.

No improvement in clinical trial enrollment for adolescents and young adults with cancer at a children's hospital.

We have previously published data from 2001 to 2006 showing that adolescent and young adult (AYA) oncology patients have significantly lower therapeutic clinical trial enrollment rates than younger patients. Our objective was to determine if the enrollment of AYA patients on therapeutic studies at the same institution has improved in recent years with the greater focus on this population locally and nationally. We retrospectively analyzed cancer registry data at the Children's Hospital of Pittsburgh (CHP) for all new oncologic diagnoses between January 2010 and December 2014. These data included age, gender, diagnosis, race and whether the patient was enrolled on an open treatment study. Univariate analyses were carried out to compare demographic data between AYA patients (aged 15-22) who enrolled on study and those who did not. Eight hundred sixty-five new oncology patients were seen at CHP during this time, 23% of whom were 15 years or older; 33% of all patients were treated on a clinical trial, including 34% of younger patients and 24% of older patients (P = 0.0017). The differences between these rates and those from prior years in both age groups (38% and 27%, respectively) were not statistically significant (P = 0.15, 0.53). The most common reason for the low enrollment rates was again the lack of an open therapeutic trial. Despite initiatives at CHP and on the national level to enroll more AYA patients on clinical trials, our most recent data show no improvement. This is a potentially remediable factor that needs to continue to be prioritized nationally.

Neurocutaneous melanosis is associated with tethered spinal cord.

Neurocutaneous melanosis (NCM) is a rare congenital disorder occurring in children born with multiple or large congenital melanocytic nevi (CMN) in association with melanocytic deposits in the leptomeninges. Multiple associations between NCM and other syndromes or neurologic abnormalities have been reported. Of note, there exists a possible association between NCM and tethered cord (TC). We retrospectively reviewed charts and films of all patients with the diagnosis of NCM at the Children's Hospital of Pittsburgh (CHP) from August 2002 to present. Five children met the criteria for NCM at our institution over a 12-year period. Apart from the melanocytic deposits, one or more additional spinal abnormalities were identified in all children. Three children had radiographic evidence of a low-lying conus medullaris, two of which also demonstrated lipomatous infiltration of the filum terminale, consistent with a tethered cord (TC). Clinical features of NCM include dermatologic and neurologic manifestations. To date, this is the first series to note an association between NCM and TC. While nearly all recent series of NCM patients advocate early MRI of the neuroaxis, we recommend screening imaging of the spine on children with possible NCM regardless of the locations of CMN.

Impact of individualized pain plan on the emergency management of children with sickle cell disease

Vaso-occlusive crisis (VOC) the hallmark of sickle cell disease (SCD) is often treated inadequately in the emergency department (ED). We hypothesized that pain management plans individualized for each patient can improve pain management and lead to high levels of patient satisfaction. Starting in 2002, we treated all patients with SCD reporting to Children's Hospital of Pittsburgh (CHP) ED with VOC using a structured algorithm. We recorded regimens used successfully for each patient as an "individualized pain plan" and implemented it during subsequent VOC visits and adjusted it to patient response. We compared rates of hospitalization following an ED visit with VOC and readmission within 1 week after discharge for CHP with that of four comparable hospitals from Pediatric Health Information (PHIS) database. Patients and parents completed surveys of satisfaction with pain management and with care. Between 2002 and 2008 there was a greater decline in the rate of admission of patients presenting to the ED at CHP (78% to 52%) as compared to PHIS (71% to 68%), (P < 0.05) and readmission rates at CHP (7.3% to 3.2%) as compared to PHIS (6.5% to 5.1%) (P < 0.05). Improvement in pain score during ED management was 2.0 or more on a Wong Baker scale of 0-5 (P < 0.01). Participants on average, rated quality of pain management as very good or higher. Individualized pain management plans in the ED are effective in delivering high quality management of VOC and are associated with a high level of patient satisfaction and decreased avoidable hospitalizations.

Improved collaboration and clinical trial enrollment in adolescent and young adult (AYA) oncology patients after the establishment of an AYA program.

9558 Background: Over the past thirty years there has been significant improvement in the survival rates of pediatric and older cancer patients, but adolescent and young adult (AYA) patients aged 15-40 years old have not seen the same improvement. The reasons are multifactorial but data has shown a direct correlation between improving cure rates and clinical trial enrollment. We have published data showing inferior clinical trial enrollment when these patients are treated at an adult versus an affilaited pediatric oncology center. To address this deficit, we at the Children's Hospital of Pittsburgh (CHP) and University of Pittsburgh Cancer Institute (UPCI) established a joint AYA Oncology Program. The primary goal of this program was and is to improve outcomes by increasing clinical trial accrual in this population. Methods: We retrospectively analyzed patients referred to our AYA program from July, 2006 through December, 2009 to establish if referrals were improving and clinical trial enrollment was increasing versus historical controls from 2003-2006 at the same institutions. Patients outside of the AYA age range could be evaluated if they were diagnosed with a malignancy more common in this age group. Results: 46 patients were referred to our program from 9 different hospitals. In the year prior to program (2005), only 2 patients were referred which increased to 15 in 2006, followed by 11 in 2007, 13 in 2008 and 14 projected for 2009. The median age of all patients evaluated was 22 years-old (range 13-75 years). 18 of these patients were treated or followed at CHP with 5 (27%) enrolled on a clinical trial. 21 were treated at the UPCI with 5 (24%) on a clinical trial. There was no difference in enrollment rates in those patients treated at CHP after the program was established but there was superior trial participation compared to the previous three years at the UPCI (p<0.001). Conclusions: Our data demonstrate that establishing a unified AYA Oncology Program both leads to increased referrals and improved clinical trial enrollment in this population. It is too soon to conclude if more trial participation for these patients will translate into improved disease-free survival. No significant financial relationships to disclose.

Consultative pediatrics in the new millenium

The pediatric hospitalist program at the Children's Hospital of Pittsburgh (CHP)-the Diagnostic Referral Service (DRS)-was first described in the pediatric literature in 1988. At that time, the group consisted of 5 members with a variety of inpatient and outpatient responsibilities. Since then, there has been a significant nationwide growth in pediatric hospital medicine. In the same time frame, the DRS has also grown significantly, with new and enhanced responsibilities in both the inpatient and outpatient settings. This work reflects on the recent trends in pediatrics that resulted in the growth of specialists in hospital medicine and in the evolution of the DRS responsibilities. A detailed description of the unique changes in the DRS is provided as a model for effective care of children in the modern era.

Disparities in the medical examination of children in the home of a child with suspected physical abuse

Identifies factors associated with medical examination of a contact child after a report of suspected physical abuse in an index child. Reviews all index children reported for suspected physical abuse from the Children's Hospital of Pittsburgh (CHP) and all contact children identified and examined in the course of the evaluation of the index child. Contact children under 2 years of age had the highest odds of undergoing medical examination following a possible abuse exposure, with black children over-represented. Disparities in suspicion of child abuse based on racial and social factors was not hypothesised, but is consistent with prior research. Surprisingly, the results found no association between hospitalization of the index child and medical examination of contact children.

Different Rates of Clinical Trial Enrollment Between Adolescents and Young Adults Aged 15 to 22 Years Old and Children Under 15 Years Old With Cancer at a Children's Hospital

Over the last 30 years significant strides have been made in cure rates for children with cancer, but these improvements have not been seen in adolescents and young adults. The reasons for this lack of progress are multifactorial, but it is clear greater cure rates are correlated with controlled clinical trials. Our objective was to see if pediatric patients over the age of 15 had a lower rate of clinical trial enrollment, and if so, why. We retrospectively analyzed the clinical data on all patients with new oncology diagnoses at Children's Hospital of Pittsburgh (CHP) diagnosed over a 5-year period from July 2001 to June 2006. Six hundred and forty new oncology patients were seen at CHP over this time, 501 under 15 years old and 139 patients aged 15 to 22. Thirty-six percent of all patients were treated on a clinical trial, including 38% of the younger patients and 27% of the older patients (P=0.03). Fifty-seven percent in the older group were not enrolled on a clinical trial because one was not available versus 41% in the younger group (P=0.04). There were no significant differences between the age groups when other reasons were analyzed. A significantly lower proportion of adolescents and young adults patients (aged 15 to 22) were placed on a treatment trial than younger patients. The lack of an open clinical trial was the main reason for this deficit. Interventions to address this discrepancy need to be instituted on a national level.

Towards the Development of a Pediatric Ventricular Assist Device

The very limited options available to treat ventricular failure in children with congenital and acquired heart diseases have motivated the development of a pediatric ventricular assist device at the University of Pittsburgh (UoP) and University of Pittsburgh Medical Center (UPMC). Our effort involves a consortium consisting of UoP, Children's Hospital of Pittsburgh (CHP), Carnegie Mellon University, World Heart Corporation, and LaunchPoint Technologies, Inc. The overall aim of our program is to develop a highly reliable, biocompatible ventricular assist device (VAD) for chronic support (6 months) of the unique and high-risk population of children between 3 and 15 kg (patients from birth to 2 years of age). The innovative pediatric ventricular assist device we are developing is based on a miniature mixed flow turbodynamic pump featuring magnetic levitation, to assure minimal blood trauma and risk of thrombosis. This review article discusses the limitations of current pediatric cardiac assist treatment options and the work to date by our consortium toward the development of a pediatric VAD.

Assessment of Adenoidal Obstruction in Children: Clinical SignsVersus Roentgenographic Findings

1. Paradise JL, 2. Bernard BS, 3. Colborn DK, 4. Janosky JE (1998) Pediatrics. 101:979–986. [OpenUrl][1][Abstract/FREE Full Text][2] #### Purpose of the Study To investigate the reliability and correlation of standardized clinical assessments and x-ray assessments of adenoidal obstruction. ##### Study Population Children were studied as part of a comprehensive, prospective study of the indications for tonsillectomy and adenoidectomy that was carried out at the Children's Hospital of Pittsburgh between 1971 and 1991. ##### Methods The patient's degree of mouth breathing and speech hyponasality were rated on a 4-point … [1]: {openurl}?query=rft.jtitle%253DPediatrics%26rft.stitle%253DPediatrics%26rft.issn%253D0031-4005%26rft.aulast%253DParadise%26rft.auinit1%253DJ.%2BL.%26rft.volume%253D101%26rft.issue%253D6%26rft.spage%253D979%26rft.epage%253D986%26rft.atitle%253DAssessment%2Bof%2BAdenoidal%2BObstruction%2Bin%2BChildren%253A%2BClinical%2BSigns%2BVersus%2BRoentgenographic%2BFindings%26rft_id%253Dinfo%253Adoi%252F10.1542%252Fpeds.101.6.979%26rft_id%253Dinfo%253Apmid%252F9606223%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [2]: /lookup/ijlink?linkType=ABST&journalCode=pediatrics&resid=101/6/979&atom=%2Fpediatrics%2F104%2FSupplement_2%2F370.2.atom

Progressive varicella presenting with pain and minimal skin involvement in children with acute lymphoblastic leukemia.

Here we report the experience at the Children's Hospital of Pittsburgh (CHP) with varicella zoster virus (VZV) in children with acute lymphoblastic leukemia (ALL). This record review was prompted by a patient with ALL who died suddenly of varicella hepatitis within 24 hours of presentation with a single skin lesion. We reviewed the medical records of children diagnosed with ALL at the CHP from January 1984 through December 1993, who subsequently developed VZV infection. Of 294 patients aged 0 to 15 years, 41 (14%) were identified as having had 42 episodes of VZV infection. Twenty patients (49%) had received prophylaxis with varicella zoster immunoglobulin (VZIG), and all 39 patients in whom the diagnosis was made premortem were treated with acyclovir. Twenty-nine of the 42 cases (70%) had disease limited to the skin. Thirteen cases (30%) had extracutaneous involvement, and five of these episodes (12% of all cases) ended in death. Risk factors for progressive varicella included age greater than 6 years and intensive immunosuppressive therapy at the time of exposure. Six of eight patients with progressive varicella, including two who died, had received VZIG. The clinical presentation in 10 of 13 patients with progressive disease and in four of five patients who died was dominated by severe abdominal and/or back pain. In seven cases, these symptoms preceded the development of skin lesions by several days, and in six patients were associated with extensive involvement of the spleen by varicella, as demonstrated histopathologically by the presence of Howell-Jolly bodies on peripheral-blood smear or radiographically. No patient with uncomplicated varicella was reported to have had premonitory pain. Recognition of these prodromes and suspicion of varicella even in the absence of skin lesions and even in children with a history of prior disease or VZIG administration should prompt early diagnostic and therapeutic measures.

PEDIATRIC EMERGENCY DEPARTMENT COMPLAINTS

Study objective: To identify the incidence and major causes of patient and parental complaints in a pediatric emergency department. Design: Retrospective analysis of complaints received regarding patients seen between January 1987 and December 1989. Setting: ED of Children's Hospital of Pittsburgh. Participants: All complaints received during the three-year period. Interventions: Complaints were reviewed for reason, validity, and location at which patient was seen. Measurements and results: One hundred seventy-six complaints from a total of 154,648 ED visits yielded a frequency of 1.1 complaints per 1,000 patient visits. Main reasons for dissatisfaction were misdiagnosis, billing, and inadequate treatment; 49% of complaints were judged valid. There were 0.69 complaints per 1,000 patient visits in the nonurgent medical portion of the ED. Patients seen emergently (critical care and trauma) had a significantly lower complaint frequency of 0.08 per 1,000 patient visits ( P χ 2 analysis). Conclusion: Assessment of ED complaints is useful to highlight areas of patient dissatisfaction and develop plans for improving patient care.

A Retrospective Review of Undifferentiated Malignancy in Childhood

We reviewed the experience of children with "undifferentiated" cancer at The Children's Hospital of Pittsburgh (CHP) from 1971 through 1987. Of 2,095 patients 0-18 years old with diagnoses during that time of any cancer, the initial CHP pathology report rendered a diagnosis in 22 children (1.1%) of "undifferentiated malignancy" or "malignant tumor" (15 cases), "undifferentiated carcinoma" (two cases) or "anaplastic tumor or carcinoma" not otherwise specified (five cases). A review of pathologic findings using current methods and immunostaining led to the assignment of a specific diagnosis in 15 of the 20 cases so studied. Thus, the incidence of undifferentiated cancer by current criteria was reduced to no more than 0.23%. Two of the five tumors for which an alternative diagnosis could not be established were described as "rhabdoid," but because it is not clear that these tumors fit into a single category they were still considered to be undifferentiated. Clinical features and management of the 22 cases including the five persistently diagnosed as undifferentiated malignancy were heterogeneous. In the face of reassigned diagnoses, a number of patients would likely have received different chemotherapy as well as radiation. Nonetheless, seven patients with malignancy show no evidence of disease (NED), including several whose therapy, given the current best diagnosis, would not be considered to have been optimal. We recommend that for patients undergoing biopsy of a tumor, sufficient material be obtained for extensive pathologic evaluation. In this way, the diagnosis of undifferentiated malignancy in children can be almost eliminated.(ABSTRACT TRUNCATED AT 250 WORDS)

Usefulness of a Gentamicin Pharmacokinetics Service in Selected High-Risk Pediatric Patients

A pharmacokinetic consultation service was initiated in 1985 at Children's Hospital ofPittsburgh for individualizing aminoglycoside therapy. Charts of all patients receiving these antibiotics were reviewed for clinical and laboratory data, and pharmacokinetic (PK) profiles were generated. PK data from oncology and appendectomy patients receiving gentamicin were analyzed to document the usefulness of the service. One hundred eighty-six PK profiles were obtained for 79 oncology patients. On initial determination, 57% (45) had therapeutic levels, 39% (31) had subtherapeutic peaks, and 4% (3) had toxic levels. Subsequent PK data in 47 patients showed that therapeutic levels were achieved in 85% (40/47) with recommended dosage changes. In three of seven patients with persistent subtherapeutic levels, recommended dosage changes were not followed. In the remaining four patients, changes in the volume of distribution made readjustment necessary. In the appendectomy group, 105 profiles were generated for 84 patients. On initial evaluation, 60% (51) had therapeutic levels and 40% (33) had subtherapeutic levels; no follow-up was available in 23 patients in the latter group. Eighty percent (8/10) of patients with subtherapeutic levels on initial evaluation achieved therapeuticlevels with recommended dosages; dosage recommendations were not followed in two patients who remained subtherapeutic. In both groups 20% of the levels obtained were unusable due to poor collection technique or inaccurate time of collection or dosing; use of these levels would have led to incorrect dosage changes. Individualizing gentamicin regimens based on pharmacokinetic evaluations optimizes dosing regimens to achieve therapeutic concentrations in these high-risk populations.

Quality assessment in the pediatric trauma care system.

The goal of the pediatric trauma care system is to prevent death, disability, and suffering of injured children. Quality assessment (QA), the evaluation of clinical performance and quality, is essential not only for formal accreditation processes, but also for day-to-day trauma center operation. QA involves three basic types of performance measures: input, process, and outcome. Input measures are inventories of the resources of a given institution, such as the availability of a surgeon on a 24-hour-a-day in-house basis. They give baseline descriptions of facilities and do not monitor performance directly. Process measures attempt to verify that the system is using its resources appropriately in response to demands, which at Children's Hospital of Pittsburgh (CHP), involves tracking all admitted patients from injury to discharge with the assistance of a system of audit screens to help identify problem cases. The methodology of the Major Trauma Outcome Study (MTOS) provides basic outcome data by identifying unexpected survivors and deaths. However, the few cases identified (four of 316 patients submitted to MTOS; 1.3%) limit conclusions regarding trauma center performance. Performance measures, when applied to admitted trauma patients, allow timely recognition of individual complications and problem trends. QA provides necessary data for important clinical decisions and resource allocations that affect trauma center operation.