To evaluate genetic testing use in infants with congenital diaphragmatic hernia (CDH) over the past decade to better inform future practices and individualize prognostication and management.
A retrospective cohort study was performed of all infants with CDH enrolled in the Pulmonary Hypoplasia Program at Children\'s Hospital of Philadelphia, born between January 2011 and February 2021. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed.
The charts of 411 infants were analyzed. Overall, 22% (n = 89) were complex/syndromic and 78% (n = 322) were isolated/nonsyndromic. Mortality was significantly higher in complex/syndromic infants (P < .001) and in infants with diagnostic genetic testing (P < .001). Microarray was diagnostic in 9% (n = 34/399) and exome sequencing was diagnostic in 38% (n = 15/39). Genetic testing was diagnostic in 57% (n = 51/89) of complex/syndromic infants, but in only 2% of isolated/nonsyndromic infants (n = 8/322). Overall, genetic testing was diagnostic in 14% (n = 56).
The high diagnostic rate in this cohort highlights the utility of comprehensive genetic testing in infants with CDH. However, 43% of complex/syndromic and 98% of isolated/nonsyndromic infants do not have a genetic etiology identified. This finding underscores the need for additional genetic and genomic studies (eg, whole genome, RNA sequencing) to identify novel genes and mutational mechanisms (single genes, regulatory elements, complex traits) that will allow for improved diagnostic rates and ultimately individualized management of infants with CDH.

The Ending the HIV Epidemic in the U.S. initiative considers cluster and outbreak response essential. This article describes the design, implementation, and early findings of a Philadelphia-based project to systematically assess sentinel cases among priority populations for improving public health infrastructure and preventing future outbreaks.
Sentinel HIV cases (i.e., early-stage or acute infection or molecular cluster cases) were identified among priority populations (Black and Hispanic/Latino men who have sex with men, youth aged 18-24 years, and transgender people who have sex with men). Chart abstraction and structured interview data were reviewed to determine themes and service gaps and to identify, prioritize, and implement recommendations. Interdisciplinary review teams included individuals with lived experience, frontline staff, and local agency leadership.
Data were collected during July 2019-December 2020 and analyzed for 53 of 126 sentinel cases of HIV diagnosed since July 1, 2018. The majority were men who have sex with men (79.3%), those aged 18-24 years (67.9%), and non-Hispanic Black (67.9%). More than half received sexually transmitted infection and HIV testing ≤3 years preceding HIV diagnosis (56.6% and 54.7%, respectively), had a healthcare visit within 12 months before diagnosis (64.2%), and had no evidence of pre-exposure prophylaxis awareness (58.5%). Project recommendations effectuated actions to improve pre-exposure prophylaxis provision, integrate sexually transmitted infection and HIV testing, and educate primary care providers.
HIV sentinel case review is a model for health departments to rapidly respond to recent transmission, identify missed HIV prevention opportunities, strengthen community partnerships, and implement programmatic and policy changes. Such efforts may prevent outbreaks and inform longer-term strategies.

OBJECTIVE: The aim of this article is to assess a large tertiary care medical center\'s emergency radiology response after the 2015 Amtrak Philadelphia train derailment.
METHODS: A total of 55 patients with 308 total CTs and radiographs ordered within 12 hours of arrival to Temple University Health System (combining Temple University Hospital and Episcopal Hospital) emergency departments on May 12 to 13, 2015, were included in this study. A retrospective PACS and electronic medical record chart review of emergency department imaging turnaround times (TAT) during this event was completed and compared with emergency department radiology operations for the same 12-hour period throughout the preceding year. Wilcoxon\'s rank-sum test analysis was performed.
RESULTS: A total of 308 CTs and radiographs were performed, and 91 radiologically evident injuries were observed in a total of 30 patients, with fractures (n = 51) as the most common type of injury. There were no significant differences in time from patient arrival to beginning of radiological examination (26 min; interquartile range [IQR], 11-58 min) compared with annual median (28 min; IQR, 10-131 min; P = .232). Examination completion TATs were significantly increased (35 min; IQR, 17-112 min) compared with annual median (10 min; IQR, 5-15 min; P < .001), and time required from viewing of the examination by the radiologist to the examination being marked as read was significantly decreased (17 min; IQR, 6-45 min) compared with annual median (248 min; IQR, 126-441 min; P < .001).
CONCLUSIONS: The analysis highlights areas of efficiency in our response but also indicates areas for process improvement in future potential mass casualty events.

OBJECTIVE: Cutaneous lesions are often the first marker of invasive mold infection, which can cause substantial morbidity in immunocompromised children. The purpose of this study was to describe the evaluation and outcomes of immunocompromised children who presented with findings requiring skin biopsy because of concern about invasive infection. In children who were biopsied, we sought to determine the factors predictive of invasive mold infection.
METHODS: A retrospective review was conducted at the Children\'s Hospital of Philadelphia. Patients included in the study were immunocompromised individuals younger than 26 years old who underwent skin biopsy by the inpatient dermatology consultation team between January 1, 2003, and March 15, 2015, because of development of new cutaneous lesions that were suspected of being invasive infection.
RESULTS: One hundred five encounters met the inclusion criteria. Fifty (47.6%) biopsied individuals had an infectious pathogen identified on histopathology or culture. Mold was the most common (36%) pathogen, followed by bacteria (32%) and yeast (26%). The presence of a single lesion (P = .001) and prior occlusion at the site of the lesion (P < .001) were associated with mold on biopsy. The combination of a single lesion, history of occlusion, and tissue necrosis on examination was highly predictive for invasive mold infection (86.3% [95% confidence interval 55.1-97.0%]). Of the 18 individuals with confirmed invasive mold infection, 13 (72%) underwent surgical resection, of whom 12 (92%) survived the 30-day follow-up period.
CONCLUSIONS: Skin biopsy enabled the detection of a pathogen that informed directed therapeutic interventions in nearly half of participants. Institutions caring for immunocompromised children should ensure adequate staffing of clinical personnel approved to perform skin biopsies.

OBJECTIVE: To develop an interdisciplinary, interactive, skills review program to improve team responses during a postpartum hemorrhage (PPH).
METHODS: Online didactic modules in combination with an interdisciplinary skills program consisting of seven hemorrhage-related stations.
UNASSIGNED: The project was conducted in the Women\'s Health Department in a quaternary-care Magnet- and Baby Friendly-designated academic medical center in Philadelphia, Pennsylvania. Women cared for at this center have comorbidities that place them at greater risk for PPH. A need was identified to implement a multidisciplinary and comprehensive program to assess hemorrhage risk and appropriately recognize and intervene with all PPHs in this setting.
METHODS: The 276 participants, including registered nurses, obstetric and family medicine attending physicians and residents, advanced practice nurses, and ancillary staff in the hospital\'s Women\'s Health Department, completed the initial obstetric hemorrhage program.
UNASSIGNED: The program included online didactic modules, seven interdisciplinary skills stations led by trained nurses and providers, and an in situ simulation. Successful completion of the online modules was a prerequisite for participation in the skills stations. All participants completed a written program evaluation at the conclusion of the program.
RESULTS: Results of the postassessment survey indicated that participants rated the program 3.94 of 4.00 for overall effectiveness to improve interdisciplinary team responses to PPH. Comments were overwhelmingly positive, and participants expressed increased confidence and knowledge related to PPH after completion of the program.
CONCLUSIONS: An interdisciplinary program that included online didactic modules, interactive skills stations, and simulation improved team confidence and responses to PPH.

OBJECTIVE: Patient fall prevention begins with accurate risk assessment. However, sustained improvements in prevention and quality of care include use of validated fall risk assessment tools (FRATs). The goal of FRATs is to identify patients at highest risk. Adult FRATs are often borrowed from to create tools for pediatric patients. Though factors associated with pediatric falls in the hospital setting are similar to those in adults, such as mobility, medication use, and cognitive impairment, adult FRATs and the factors associated with them do not adequately assess risk in children.
METHODS: Articles were limited to English language, ages 0-21years, and publish date 2006-2015.
METHODS: The search yielded 22 articles. Ten were excluded as the population was primarily adult or lacked discussion of a FRAT. Critical appraisal and findings were synthesized using the Johns Hopkins Nursing evidence appraisal system.
RESULTS: Twelve articles relevant to fall prevention in the pediatric hospital setting that discussed fall risk assessment and use of a FRAT were reviewed. Comparison between and accuracy of FRATs is challenged when different classifications, definitions, risk stratification, and inclusion criteria are used.
CONCLUSIONS: Though there are several pediatric FRATs published in the literature, none have been found to be reliable and valid across institutions and diverse populations.
CONCLUSIONS: This integrative review highlights the importance of choosing a FRAT based on an institution\'s identified risk factors and validating the tool for one\'s own patient population as well as using the tool in conjunction with nursing clinical judgment to guide interventions.

OBJECTIVE: Loose anagen syndrome (LAS) is a disorder of abnormal anchorage of the hair to the scalp. Its symptoms include an inability to grow hair long and hair that is easily pulled out.
METHODS: We conducted a retrospective chart review of patients with LAS over the last 10 years at the Children\'s Hospital of Philadelphia and found 37 cases.
RESULTS: LAS was more common in females and was found in all hair colors and skin types.
CONCLUSIONS: Diagnosis of LAS should be made based on history, microscopic findings, and hair bulb and shaft features.

OBJECT Sagittal craniosynostosis has been treated using both cranial remodeling techniques and modification of the sagittal strip craniectomy. A more recent technique is to implant springs in conjunction with a suturectomy to transversely expand the parietal bones to accommodate the growing brain. In this paper the authors describe and evaluate several modifications to the spring-mediated cranioplasty (SMC) technique, most notably use of an ultrasonic scalpel to limit dural dissection and maximize opening of the stenosed suture by placement of multiple spring devices. In addition, the literature is reviewed comparing SMC to other surgical treatments of sagittal synostosis. METHODS The authors retrospectively reviewed patients who presented to the Children\'s Hospital of Philadelphia with a diagnosis of sagittal synostosis from August 2011 to November 2014. A pooled data set was created to compare our institutional data to previously published work. A comprehensive literature review was performed of all previous studies describing the SMC technique, as well as other techniques for sagittal synostosis correction. RESULTS Twenty-two patients underwent SMC at our institution during the study period. Patients were 4.2 months of age on average, had a mean blood loss of 56.3 ml, and average intensive care unit and total hospital stays of 29.5 hours and 2.2 days, respectively. The cranial index was corrected to an average of 73.7 (SD 5.2) for patients who received long-term radiological follow-up. When comparing the authors\' institutional data to pooled SMC data, blood loss and length of stay were both significantly less (p = 0.005 and p < 0.001, respectively), but the preoperative cranial index was significantly larger (p = 0.01). A review of the SMC technique compared with other techniques to actively expand the skull of patients with sagittal synostosis demonstrated that SMC can be performed at a significantly earlier age compared with cranial vault reconstruction (CVR). CONCLUSIONS The authors found that their institutional modifications of the SMC technique were safe and effective in correcting the cranial index. In addition, this technique can be performed at a younger age than CVRs. SMC, therefore, has the potential to maximize the cognitive benefits of early intervention, with lower morbidity than the traditional CVR.

Individuals with type 1 diabetes are at increased risk for depression, anxiety disorder, and eating disorder diagnoses. People with type 1 diabetes are also at risk for subclinical levels of diabetes distress and anxiety. These mental/behavioral health comorbidities of diabetes are associated with poor adherence to treatment and poor glycemic control, thus increasing the risk for serious short- and long-term physical complications, which can result in blindness, amputations, stroke, cognitive decline, decreased quality of life, as well as premature death. When mental health comorbidities of diabetes are not diagnosed and treated, the financial cost to society and health care systems is catastrophic, and the human suffering that results is profound. This review summarizes state-of-the-art presentations and working group scholarly reports from the Mental Health Issues of Diabetes Conference (7-8 October 2013, Philadelphia, PA), which included stakeholders from the National Institutes of Health, people living with type 1 diabetes and their families, diabetes consumer advocacy groups, the insurance industry, as well as psychologists, psychiatrists, endocrinologists, and nurse practitioners who are all nationally and internationally recognized experts in type 1 diabetes research and care. At this landmark conference current evidence for the incidence and the consequences of mental health problems in type 1 diabetes was presented, supporting the integration of mental health screening and mental health care into routine diabetes medical care. Future research directions were recommended to establish the efficacy and cost-effectiveness of paradigms of diabetes care in which physical and mental health care are both priorities.

OBJECTIVE: To Assess changes in infective endocarditis (IE) epidemiology over the last 5 decades.
RESULTS: We searched the published literature using PubMed, MEDLINE, and EMBASE from inception until December 2011.
UNASSIGNED: Einstein Medical Center, Philadelphia, PA were also included. Criteria for inclusion in this systematic review included studies with reported IE microbiology, IE definition, description of population studied, and time frame. Two authors independently extracted data and assessed manuscript quality. One hundred sixty studies (27,083 patients) met inclusion criteria. Among hospital-based studies (n=142; 23,606 patients) staphylococcal IE percentage increased over time, with coagulase-negative staphylococcus (CNS) increasing over each of the last 5 decades (p<0.001) and Staphylococcus aureus (SA) in the last decade (21% to 30%; p<0.05). Streptococcus viridans (SV) and culture negative (CN) IE frequency decreased over time (p<0.001), while enterococcal IE increased in the last decade (p<0.01). Patient age and male predominance increased over time as well. In subgroup analysis, SA frequency increased in North America, but not the rest of the world. This was due, in part, to an increase in intravenous drug abuse IE in North America (p<0.001). Among population-based studies (n=18; 3,477 patients) no significant changes were found.
CONCLUSIONS: Important changes occurred in IE epidemiology over the last half-century, especially in the last decade. Staphylococcal and enterococcal IE percentage increased while SV and CN IE decreased. Moreover, mean age at diagnosis increased together with male:female ratio. These changes should be considered at the time of decision-making in treatment of and prophylaxis for IE.