Papers by Sandra Friedman
Journal of Pediatric Surgery, 2001
In 1990, the authors began a multidisciplinary follow-up clinic for congenital diaphragmatic hern... more In 1990, the authors began a multidisciplinary follow-up clinic for congenital diaphragmatic hernia (CDH) patients. Although the nonpulmonary complications associated with CDH have been reported previously from this clinic, the purpose of this report is to detail the pulmonary outcome in survivors of CDH with severe pulmonary hypoplasia. Methods: Between 1990 and 1999, one hundred patients were seen in the clinic. Before hospital discharge, all patients had baseline tests performed, which were repeated per protocol at clinic during follow-up. The data were analyzed by regression analysis to identify and determine the impact of factors on outcomes associated with the long-term pulmonary morbidity. Results: The average birth weight was 3.16 kg (+/-0.7) with a mean Apgar score of 7 (+/-2) at 5 minutes. Forty-one patients had an antenatal diagnosis performed. Extracorporeal membrane oxygenation (ECMO) was utilized in 29 patients, and a patch repair was required in 32, whereas 16 patients received both. Average time to extubation was 20.7 (+/-20) days and mean time to discharge was 59.7 (+/-61) days. Regression analysis showed that both the need for ECMO and a patch repair were independent predictors of delay in extubation (P < .001, R 2 = 36%), and delay in discharge from the hospital (P = .001, R 2 = 29%). ECMO also was significantly correlated with the need for diuretics at discharge (P < .001, R 2 = 18%), and with the presence of left-right mismatch (P = .009, R 2 = 9%) and V/Q mismatch (P = .005, R 2 = 11%) on subsequent pulmonary ventilationperfusion examinations. Sixteen patients required 02 at discharge, and diuretics were necessary in 43 patients. Seventeen patients at discharge required bronchodilators, and during the first year an additional 36 required at least transient therapy. Similarly, 6 patients at discharge required steroids, and an additional 35 patients required at least transient therapy during the first year. Chest x-rays, although frequently abnormal, had little correlation with clinical outcome, but did influence medical therapy. V/Q scans had limited utility in patient management, and the presence of V/Q mismatch was not highly specific for future obstructive airway disease. Nevertheless, V/Q mismatch was sensitive for obstructive airway disease assessed by spirometry. Twenty-five patients over 5 years of age performed pulmonary function tests (PFTs), which showed 72% normal PFT results and 28% with evidence of obstructive airway disease. Before January 1997, 2 of 8 patients who required urgent treatment in the emergency department (ED) were admitted to the intensive care unit (ICU) secondary to acute respiratory distress. After the implementation of respiratory syncytial viral prophylaxis in January 1997, 8 patients were treated in the ED for acute respiratory distress, but none required admission to the ICU. Conclusions: Pulmonary problems continue to be a source of morbidity for survivors of CDH long after discharge. The need for ECMO and the presence of a patch repair are both predictive of more significant morbidity, but the data clearly show that non-ECMO CDH survivors also require frequent attention to pulmonary issues beyond the neonatal period. These data show the need for long-term follow-up of CDH patients preferably with a multidisciplinary team approach.
Journal of Pediatric Surgery, Jun 1, 2008
Infants who survive congenital diaphragmatic hernia (CDH) repair may have ongoing medical and neu... more Infants who survive congenital diaphragmatic hernia (CDH) repair may have ongoing medical and neurodevelopmental morbidity after hospital discharge. We evaluated the relationship between medical and neurodevelopmental outcomes of CDH survivors seen in a multidisciplinary clinic at ages 1 and/or 3. Methods: From January 1997 to December 2004, 69 (61%) of 112 CDH survivors were followed in our CDH clinic at ages 1 and/or 3. Medical issues (cardiac, pulmonary, gastrointestinal) were tabulated at hospital discharge and at follow-up. Neurodevelopmental data were obtained from clinic assessments by a neurodevelopmental pediatrician. Descriptive results were summarized for each cohort. Multivariate analyses were performed to identify predictors of motor problems at age 1. Results: Of the 69 study participants, 64% were male, 75% had left-sided CDH, 17% had cardiac anomalies, and 25% had other congenital malformations. Nearly all required ventilator management (99%) with a median ventilator time of 14 days (range, 1-54 days); 30% required extracorporeal membrane oxygenation. While 87% of patients had medical issues at hospital discharge, 61% and 67% had medical issues at ages 1 and 3, respectively. Pulmonary problems were noted in 34% and 33% of the ages 1 and 3 cohorts, respectively. Motor and language problems were detected in 60% and 18% of the age 1 cohort and 73% and 60% of the age 3 cohort, respectively. Multivariate analysis found ventilator time as the only independent predictor of motor problems at age 1 (odds ratio, 1.12 per day; 95% confidence interval, 1.05-1.20; P b .01). Conclusions: Young CDH survivors continue to have ongoing medical problems and a high incidence of motor and language problems. Duration of neonatal ventilatory support was a significant predictor of motor problems at age 1. Prospective studies are needed to confirm these findings.
The Journal of Pediatrics, Jun 1, 2023
JAMA Pediatrics, Dec 1, 2022
ImportanceAutism spectrum disorder (ASD) affects 1 in 44 children. The Autism Diagnostic Observat... more ImportanceAutism spectrum disorder (ASD) affects 1 in 44 children. The Autism Diagnostic Observation Schedule (ADOS) is a semi-structured observation developed for use in research but is considered a component of gold standard clinical diagnosis. The ADOS adds time and cost to diagnostic assessments.ObjectiveTo evaluate consistency between clinical diagnosis (index ASD diagnosis) and diagnosis incorporating the ADOS (reference standard ASD diagnosis) and to examine clinician and child factors that predict consistency between index diagnoses and reference standard diagnoses.Design, Setting, and ParticipantsThis prospective diagnostic study was conducted between May 2019 and February 2020. Developmental-behavioral pediatricians (DBPs) made a diagnosis based on clinical assessment (index ASD diagnosis). The ADOS was then administered, after which the DBP made a second diagnosis (reference standard ASD diagnosis). DBPs self-reported diagnostic certainty at the time of the index diagnoses and reference standard diagnoses. The study took place at 8 sites (7 US and 1 European) that provided subspecialty assessments for children with concerns for ASD. Participants included children aged 18 months to 5 years, 11 months, without a prior ASD diagnosis, consecutively referred for possible ASD. Among 648 eligible children, 23 refused, 376 enrolled, and 349 completed the study. All 40 eligible DBPs participated.ExposuresADOS administered to all child participants.Main Outcomes and MeasuresIndex diagnoses and reference standard diagnoses of ASD (yes/no).ResultsAmong the 349 children (279 [79.7%] male; mean [SD] age, 39.9 [13.4] months), index diagnoses and reference standard diagnoses were consistent for 314 (90%) (ASD = 250; not ASD = 64) and changed for 35. Clinician diagnostic certainty was the most sensitive and specific predictor of diagnostic consistency (area under curve = 0.860; P &amp;lt; .001). In a multilevel logistic regression, no child or clinician factors improved prediction of diagnostic consistency based solely on clinician diagnostic certainty at time of index diagnosis.Conclusions and RelevanceIn this prospective diagnostic study, clinical diagnoses of ASD by DBPs with vs without the ADOS were consistent in 90.0% of cases. Clinician diagnostic certainty predicted consistency of index diagnoses and reference standard diagnoses. This study suggests that the ADOS is generally not required for diagnosis of ASD in young children by DBPs and that DBPs can identify children for whom the ADOS may be needed.
John Wiley & Sons, Ltd eBooks, Aug 8, 2008
International journal of forensic sciences, 2022
A neglected yet foundational diagnosis of Autism Spectrum Disorder (ASD) is added to the differen... more A neglected yet foundational diagnosis of Autism Spectrum Disorder (ASD) is added to the differential diagnosis of the many medical and psychiatric diagnoses that have been utilized to explain the unique and unusual persona of Vincent van Gogh. From his earliest years, Van Gogh exhibited the salient features of ASD (formerly Asperger Disorder), which resulted in volatile and alienating relationships with family, friends, art colleagues, and residents of the communities in which he resided. This diagnosis provides a framework for deepening our understanding of the multi-dimensional medical and psychiatric struggles that Van Gogh endured with resiliency and heroic effort. His profound loneliness caused by his inability to display empathy and social reciprocity did not ultimately stop him from pursuing family reconciliation, enduring friendships, love and ultimately marriage. We know that autistic adults have similar desires as their neurotypical peers to lead fulfilling lives, which includes romantic and other meaningful relationships.
Intellectual and Developmental Disabilities, Apr 1, 2007
American Academy of Pediatrics eBooks, Apr 17, 2017
Read more and get great! That's what the book enPDFd end of life care for children and adults... more Read more and get great! That's what the book enPDFd end of life care for children and adults with intellectual and developmental disabilities will give for every reader to read this book. This is an on-line book provided in this website. Even this book becomes a choice of someone to read, many in the world also loves it so much. As what we talk, when you read more every page of this end of life care for children and adults with intellectual and developmental disabilities, what you will obtain is something great.
American journal on intellectual and developmental disabilities, Nov 1, 2012
Intellectual and Developmental Disabilities, Feb 1, 2018
Youth with intellectual and developmental disabilities (IDD) often experience difficulties with s... more Youth with intellectual and developmental disabilities (IDD) often experience difficulties with successful transition from pediatric to adult healthcare. A consultative Transition Clinic for youth with IDD was piloted as a quality improvement project, and assessed the engagement of primary care providers (PCPs) for transition planning after patients were seen in clinic. Although many PCPs found the clinic and resources useful, individual and systemic barriers often prohibited them from participating in transition planning for this patient population. These findings highlight systemic barriers that need to be addressed to ensure successful transition, as well as the need for a specialized Transition Clinic with involvement of specialists with expertise in IDD, such as Developmental-Behavioral Pediatrics, to assist throughout transition process.
Carolina Digital Repository (University of North Carolina at Chapel Hill), 2022
The association of autism spectrum disorder (ASD) with self-reported maternal cannabis use from 3... more The association of autism spectrum disorder (ASD) with self-reported maternal cannabis use from 3 months pre-conception to delivery ("peri-pregnancy") was assessed in children aged 30-68 months, born 2003 to 2011. Children with ASD (N = 1428) were compared to children with other developmental delays/disorders (DD, N = 1198) and population controls (POP, N = 1628). Peri-pregnancy cannabis use was reported for 5.2% of ASD, 3.2% of DD and 4.4% of POP children. Adjusted odds of peri-pregnancy cannabis use did not differ significantly between ASD cases and DD or POP controls. Results were similar for any use during pregnancy. However, given potential risks suggested by underlying neurobiology and animal models, further studies in more recent cohorts, in which cannabis use and perception may have changed, are needed.
Journal of Developmental & Behavioral Pediatrics
: Objectives: This study aimed to determine whether parent ratings of attention-deficit/hyperacti... more : Objectives: This study aimed to determine whether parent ratings of attention-deficit/hyperactivity disorder (ADHD) symptom severity or externalizing symptoms (EXT) or internalizing symptoms (INT) moderate response to stimulants (STIM) and alpha-2 adrenergic agonists (A2As) in preschool ADHD. Methods: Health records for children treated with medication for ADHD and with parent rating scale data available (N = 309; age &lt;72 months) were reviewed at 7 Developmental-Behavioral Pediatric Research Network sites. Severity of ADHD was defined as the number of ADHD symptoms occurring often or very often on DSM-IV–based parent rating scales. EXT or INT from standardized rating scales were categorized as T score &lt;60, 60 to &lt;70, or ≥70. Ordinal logistic regression models predicting response to medication were calculated. Results: The median (interquartile range) age at ADHD diagnosis was 59 (54–65) months. One hundred eighty-three participants had ADHD symptom severity, and 195 had EXT or INT data. ADHD severity was not associated with medication response. Both EXT and INT were associated with medication response but with significant medication class by EXT/INT interactions. Children with higher EXT were less likely to respond to STIM, with percentage of nonresponders for T-score categories &lt;60, 60 to &lt;70, and ≥70 being 3.6%, 25.7%, and 33.3% (p = 0.016) and, for A2As, being 60%, 50%, and 33.3% (p = 0.55), respectively. A similar pattern was observed for INT categories: STIM 19.4%, 22.5%, and 50.0% (p = 0.002) and A2As 42.3%, 30%, and 42.3% (p = 0.48), respectively. Conclusion: For preschool ADHD, low ratings of EXT or INT are associated with a high likelihood of response to STIM. By contrast, response rates to STIM and A2As are more similar for children with high levels of EXT or INT.
Neuron, Jan 7, 2018
The Simons Foundation Autism Research Initiative (SFARI) has launched SPARKForAutism.org, a dynam... more The Simons Foundation Autism Research Initiative (SFARI) has launched SPARKForAutism.org, a dynamic platform that is engaging thousands of individuals with autism spectrum disorder (ASD) and connecting them to researchers. By making all data accessible, SPARK seeks to increase our understanding of ASD and accelerate new supports and treatments for ASD.
Health Care for People with Intellectual and Developmental Disabilities across the Lifespan, 2016
With the advent of the modern hospice movement in mid-twentieth century, longer life expectancy r... more With the advent of the modern hospice movement in mid-twentieth century, longer life expectancy reflective of medical advances, growing attention to ethical issues, and development of palliative medicine, increased attention has been paid to palliative and end-of-life care in general. Similarly, interest in issues pertaining to end-of-life care for people with intellectual and developmental disabilities (IDD) also has gained more attention in the professional and lay media. Despite these developments, there continues to be many misconceptions issues specific to people with IDD at end of life. Current medical, social, ethical, and practical issues, as well as challenges faced by children and adults with IDD, reflect the gains that have been made and the ongoing need for further refinement of systems and supports.
Health Care for People with Intellectual and Developmental Disabilities across the Lifespan, 2016
More people with intellectual and developmental disabilities (IDD) experience better health and l... more More people with intellectual and developmental disabilities (IDD) experience better health and longer lives than in previous years. Although fewer individuals reside in congregate care settings, there remains a subset of children, youth, and adults, primarily those with severe IDD and medical complexity, who require intensive nursing and medical supports that cannot be provided in the home setting for a variety of reasons and are not appropriate for acute care settings. This chapter reviews the evolution of nursing home care for people with IDD, the characteristics of people residing in these settings, reasons for placement, and type of care provided. Challenges and controversies pertaining to nursing home care are also discussed.
BMC Medical Education
Background Entrustable Professional Activities (EPA) and competencies represent components of a c... more Background Entrustable Professional Activities (EPA) and competencies represent components of a competency-based education framework. EPAs are assessed based on the level of supervision (LOS) necessary to perform the activity safely and effectively. The broad competencies, broken down into narrower subcompetencies, are assessed using milestones, observable behaviors of one’s abilities along a developmental spectrum. Integration of the two methods, accomplished by mapping the most relevant subcompetencies to each EPA, may provide a cross check between the two forms of assessment and uncover those subcompetencies that have the greatest influence on the EPA assessment. Objectives We hypothesized that 1) there would be a strong correlation between EPA LOS ratings with the milestone levels for the subcompetencies mapped to the EPA; 2) some subcompetencies would be more critical in determining entrustment decisions than others, and 3) the correlation would be weaker if the analysis includ...
Pediatrics, May 1, 2012
Children and youth with complex medical issues, especially those with technology dependencies, ex... more Children and youth with complex medical issues, especially those with technology dependencies, experience frequent and often lengthy hospitalizations. Hospital discharges for these children can be a complicated process that requires a deliberate, multistep approach. In addition to successful discharges to home, it is essential that pediatric providers develop and implement an interdisciplinary and coordinated plan of care that addresses the child' s ongoing health care needs. The goal is to ensure that each child remains healthy, thrives, and obtains optimal medical home and developmental supports that promote ongoing care at home and minimize recurrent hospitalizations. This clinical report presents an approach to discharging the child with complex medical needs with technology dependencies from hospital to home and then continually addressing the needs of the child and family in the home environment.
Journal of Pediatric Surgery, Jun 1, 2007
Background: Infants with congenital diaphragmatic hernia require complex surgical care and may ha... more Background: Infants with congenital diaphragmatic hernia require complex surgical care and may have neurodevelopmental morbidity. We examined the performance of reports of motor functioning in 25 congenital diaphragmatic hernia survivors using the parent-completed Developmental Profile-II and a clinical evaluation by a neurodevelopmental pediatrician (MD) measured against the Bayley motor scale. Methods: Bayley motor scores were dichotomized as normal or abnormal. Sensitivity and specificity were calculated for each test. Results: The median age at assessment was 25 months. Bayley motor scores were abnormal in 77% of infants tested (10/13). The MD examinations detected motor problems in 92% (12/13). Sensitivity and specificity of the MD examination were 1.0 and 0.33, respectively. Developmental Profile-II physical scores were abnormal in 15% (2/13); sensitivity and specificity were 0.2 and 1.0, respectively. Conclusions: The high rate of abnormal motor findings in this study supports the need for ongoing screening and evaluation. The sensitivity of MD examinations was excellent, but hypotonia findings were not universally corroborated by the Bayley. Although specificity of parent-reported motor findings was high, parents underreported abnormal motor findings. Parental reports of neurodevelopmental problems should be heeded, and physicians should perform screening motor examinations. Bayley assessments may be warranted to determine the functional implications of observed abnormalities.
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Papers by Sandra Friedman