Hidradenitis Suppurativa and Maternal and Offspring Outcomes.

Importance: Hidradenitis suppurativa (HS) is associated with morbidity in persons of reproductive age, but the effect on maternal and offspring outcomes is understudied. Objective: To determine the association of HS with pregnancy outcomes and maternal and child morbidity in the long term. Design, Setting, and Participants: This population-based longitudinal cohort study with up to 16 years of follow-up took place between 2006 and 2022 in Quebec, Canada. . Exposure: Maternal HS. Main Outcomes and Measures: Outcomes included hypertensive disorders of pregnancy, gestational diabetes, and other birth outcomes as well as the long-term risk of hospitalization up to 16 years after delivery. The study used adjusted log-binomial and Cox proportional hazards regression models to estimate the association between maternal HS and pregnancy outcomes or hospitalization following pregnancy. Outcomes in both mothers and offspring were assessed. Results: There were 1 324 488 deliveries during the study, including 1332 (0.1%) among mothers with HS. Compared with patients without HS, patients with HS had a greater risk of hypertensive disorders of pregnancy (risk ratio [RR], 1.55 [95% CI, 1.29-1.87]), gestational diabetes (RR, 1.61 [95% CI, 1.40-1.85]), and severe maternal morbidity (RR, 1.38 [95% CI, 1.03-1.84]). In neonates, maternal HS was associated with risk of preterm birth (RR, 1.28 [95% CI, 1.07-1.53]) and birth defects (RR, 1.29 [95% CI, 1.07-1.56]). In the long term, HS was associated with 2.29 times the risk of maternal hospitalization (95% CI, 2.07-2.55) and 1.31 times the risk of childhood hospitalization (95% CI, 1.18-1.45), including hospitalization for respiratory, metabolic, psychiatric, and immune-related morbidity over time. Conclusions and Relevance: This cohort study found that HS is associated with adverse maternal and offspring outcomes in the peripartum period and in the long term. Early detection and management of HS may help mitigate these outcomes.

BDNF-dependent nano-organization of Neogenin and the WAVE regulatory complex promotes actin remodeling in dendritic spines.

Synaptic structural plasticity, the expansion of dendritic spines in response to synaptic stimulation, is essential for experience-dependent plasticity and is driven by branched actin polymerization. The WAVE regulatory complex (WRC) is confined to nanodomains at the postsynaptic membrane where it catalyzes actin polymerization. As the netrin/RGM receptor Neogenin is a critical regulator of the WRC, its nanoscale organization may be an important determinant of WRC nanoarchitecture and function. Using super-resolution microscopy, we reveal that Neogenin is highly organized on the spine membrane at the nanoscale level. We show that Neogenin binding to the WRC promotes co-clustering into nanodomains in response to brain-derived neurotrophic factor (BDNF), indicating that nanoclustering occurs in response to synaptic stimulation. Disruption of Neogenin/WRC binding not only prevents BDNF-mediated actin remodeling but also inhibits BDNF-induced calcium signaling. We conclude that the assembly of Neogenin/WRC nanodomains is a prerequisite for BDNF-mediated structural and synaptic plasticity.

Feasibility of biliary atresia newborn screening in an integrated health network.

Diagnostic delay remains a barrier to improving biliary atresia (BA) outcomes. We tested the implementation feasibility of a two stage BA newborn screening program in an integrated healthcare system. METHODS: Under a waiver of consent, we measured direct bilirubin (DB) levels in well newborns undergoing standard of care hyperbilirubinemia screening at four hospitals. Initial DB was measured by modifying nursery admission electronic medical record (EMR) order sets. Second-stage DB was obtained at ~2 weeks of age under parental permission/informed consent (PP/IC). Implementation measures included the proportions of (1) eligible newborns that were screened before nursery discharge, (2) newborns undergoing second stage screening at ~2 weeks of age, and (3) newborns that underwent clinical evaluation for persistently elevated DB. RESULTS: A total of 12,276 newborns met eligibility criteria for screening, of which 12,055 (98.2%) underwent first-stage screening in the newborn nursery. Ninety-four (0.78%) had elevated positive initial screens. Ninety newborns (95.7%) underwent second-stage screening (n = 20) or contact was made with the primary care provider to recommend second-stage screening (n = 70). Among all screened newborns, 15 (0.12%) had abnormal second screens. All had follow-up clinical evaluation for potential cholestatic liver disease. No BA cases were identified through screening, though two infants who met exclusion criteria (admission to the newborn intensive care unit) were subsequently diagnosed with BA during the screening period. CONCLUSIONS: BA newborn screening is feasible in an integrated health network. Low consent rates have implications for future studies. Program infrastructure is required for implementation success and sustainability.

Interpretation of immediately released health information: Informing patient medical education in breast oncology.

BACKGROUND: The Cures Act mandated immediately released health information. In this study, we investigated patient comprehension of mammography reports and the utility of online resources to aid report interpretation. METHODS: Patients who received a normal mammogram from February to April 2022 were invited to complete semi-structured interviews paired with health literacy questionnaires to assess patient's report comprehension before and after internet search. RESULTS: Thirteen selected patients via purposeful sampling completed interviews. Most patients described their initial understanding of the mammography report as "good" and improved to between "good" and "very good" after an internet search. Patients suggested "a little column on the side" for medical terminology, "an extra prompt" for making an appointment, or a recommendation for "good sites" to improve mammography reports. CONCLUSION: Patients varied in their ability to independently interpret medical reports and seek additional resources. While online resources marginally improved patient understanding, actionable and clear resources are needed.

Access to general obstetrics and gynecology care among Medicaid beneficiaries and the privately insured: a nationwide mystery caller study in the USA.

BACKGROUND: The mean wait time for new patient appointments has been growing across specialties, including obstetrics and gynecology, in recent years. This study aimed to assess the impact of insurance type (Medicaid versus commercial insurance) on new patient appointment wait times in general obstetrics and gynecology practices. METHODS: A cross-sectional study used covert mystery calls to general obstetrician gynecologists. Physicians were selected from the American College of Obstetricians and Gynecologists directory and stratified by districts to ensure nationwide representation. Wait times for new patient appointments were collected and analyzed. RESULTS: Regardless of insurance type, the mean wait time for all obstetrician gynecologists was 29.9 business days. Medicaid patients experienced a marginally longer wait time of 4.8% (Ratio: 1.048). While no statistically significant difference in wait times based on insurance type was observed (P=0.39), the data revealed other impactful factors. Younger physicians and those in university-based practices had longer wait times. The gender of the physician also influenced wait times, with female physicians having a mean wait time of 34.7 days compared to 22.7 days for male physicians (P=0.03). Additionally, geographical variations were noted, with physicians in American College of Obstetricians and Gynecologists District I (Atlantic Provinces, CT, ME, MA, NH, RI, VT) having the longest mean wait times and those in District III (DE, NJ, PA) the shortest. CONCLUSIONS: While the type of insurance did not significantly influence the wait times for general obstetrics and gynecology appointments, physician demographic and geographic factors did.

Comparison of Suprainguinal Fascia Iliaca Nerve Block and Epidural Analgesia in Patients Undergoing Periacetabular Osteotomy.

INTRODUCTION: Adequate pain control and early mobilization are critical in the postoperative period after periacetabular osteotomy (PAO). Regional anesthesia can reduce postoperative pain, but certain techniques can increase the risk of postoperative motor block and delayed functional recovery. Continuous lumbar epidurals can provide excellent analgesia but also create challenges with early ambulation. Recently, suprainguinal fascia iliaca (SIFI) single-shot blocks have been shown to provide effective analgesia in PAO patients. The goal of this study was to compare opioid use, time to achieve inpatient physical therapy (PT) goals, and length of stay (LOS) between a cohort of patients receiving SIFI blocks and a cohort of patients receiving epidural analgesia (EA). METHODS: This retrospective single-surgeon comparative cohort study included all patients who underwent a PAO between 2012 and 2022. Regional anesthetic technique (SIFI vs EA), length of hospital stay, intraoperative and postoperative opioid use, pain scores, and time to achievement of PT milestones before discharge were recorded. Patients were excluded if they had any preexisting neuromuscular syndrome or neurosensory deficit. All opioid use was converted to morphine-milligram equivalents using standard conversions. RESULTS: Two hundred four surgeries were done over the study period; 164 patients received EA, and 40 received a SIFI block. The average age of our cohort was 19.5 years (±6 yrs). The SIFI cohort had a significantly shorter mean LOS than the EA cohort (2.9 vs 4.1 days, P < 0.001). Patients in the SIFI cohort achieved all PT ambulation goals significantly sooner than the lumbar epidural group (P < 0.001), with lower total opioid use on each postoperative day. No notable differences were observed in sex, race, BMI, or pain scores at inpatient PT visits before discharge. CONCLUSION: After PAO surgery, the SIFI block is associated with shorter hospital LOS, reduced postoperative opioid use, and earlier mobilization when compared with those who were managed with an epidural.

Neonatal and Obstetrical Outcomes of Pregnancies Complicated by Alloimmunization.

BACKGROUND AND OBJECTIVES: Despite advances in the prevention of rhesus (Rh)(D) alloimmunization, alloantibodies to Rh(D) and non-Rh(D) red blood cell antigens continue to be detected in ∼4% of US pregnancies and can result in hemolytic disease of the fetus and newborn (HDFN). Recent reports on HDFN lack granularity and are unable to provide antibody-specific outcomes. The objective of this study was to calculate the frequency of alloimmunization in our large hospital system and summarize the outcomes based on antibody specificity, titer, and other clinical factors. METHODS: We identified all births in a 6-year period after a positive red blood cell antibody screen result during pregnancy and summarized their characteristics and outcomes. RESULTS: A total of 707 neonates were born after a positive maternal antibody screen result (3.0/1000 live births). In 31 (4%), the positive screen result was due to rhesus immune globulin alone. Of the 676 neonates exposed to alloantibodies, the direct antibody test (DAT) result was positive, showing antigen-positivity and evidence of HDFN in 37% of those tested. Neonatal disease was most severe with DAT-positive anti-Rh antibodies (c, C, D, e, E). All neonatal red blood cell transfusions (15) and exchange transfusions (6) were due to anti-Rh alloimmunization. No neonates born to mothers with anti-M, anti-S, anti-Duffy, anti-Kidd A, or anti-Lewis required NICU admission for hyperbilirubinemia or transfusion. CONCLUSIONS: Alloimmunization to Rh-group antibodies continues to cause a majority of the severe HDFN cases in our hospital system. In neonates born to alloimmunized mothers, a positive DAT result revealing antigen-positivity is the best predictor of anemia and hyperbilirubinemia.

Perioperative management and outcomes for posterior spinal fusion in patients with Friedreich ataxia: A single-center, retrospective study.

BACKGROUND: Friedreich ataxia is a rare genetic disorder associated with progressive mitochondrial dysfunction leading to widespread sequelae including ataxia, muscle weakness, hypertrophic cardiomyopathy, diabetes mellitus, and neuromuscular scoliosis. Children with Friedreich ataxia are at high risk for periprocedural complications during posterior spinal fusion due to their comorbidities. AIM: To describe our single-center perioperative management of patients with Friedreich ataxia undergoing posterior spinal fusion. METHODS: Adolescent patients with Friedreich ataxia presenting for spinal deformity surgery between 2007 and 2023 were included in this retrospective case series performed at the Children's Hospital of Philadelphia. Perioperative outcomes were reviewed along with preoperative characteristics, intraoperative anesthetic management, and postoperative medical management. RESULTS: Seventeen patients were included in the final analysis. The mean age was 15 ± 2 years old and 47% were female. Preoperatively, 35% were wheelchair dependent, 100% had mild-to-moderate hypertrophic cardiomyopathy with preserved systolic function and no left ventricular outflow tract obstruction, 29% were on cardiac medications, and 29% were on pain medications. Intraoperatively, 53% had transesophageal echocardiography monitoring; 12% had changes in volume status on echo but no changes in function. Numerous combinations of total intravenous anesthetic agents were used, most commonly propofol, remifentanil, and ketamine. Baseline neuromonitoring signals were poor in four patients and one patient lost signals, resulting in 4 (24%) wake-up tests. The majority (75%) were extubated in the operating room. Postoperative complications were high (88%) and ranged from minor complications like nausea/vomiting (18%) to major complications like hypotension/tachycardia (29%) and need for extracorporeal membrane oxygenation support in one patient (6%). CONCLUSIONS: Patients with Friedreich ataxia are at high risk for perioperative complications when undergoing posterior spinal fusion and coordinated multidisciplinary care is required at each stage. Future research should focus on the utility of intraoperative echocardiography, optimal anesthetic agent selection, and targeted fluid management to reduce postoperative cardiac complications.

The state of point-of-care ultrasound training in pediatric anesthesia fellowship programs in the United States: A survey assessment.

BACKGROUND: Point-of-care ultrasound is an invaluable bedside tool for anesthesiologists and has been integrated into anesthesiology residency training and board certification in the United States. Little is known about point-of-care ultrasound training practices in pediatric anesthesia fellowship programs. AIMS: To describe the current state of point-of-care ultrasound education in pediatric anesthesia fellowship programs in the United States. METHODS: We conducted a cross-sectional survey study distributed to 60 American Accreditation Council for Graduate Medical Education-accredited pediatric anesthesia fellowship programs. Two programs were in their initial accreditation period and were excluded due to lack of historical data. Program directors or associate program directors were invited to complete this 23-item survey. RESULTS: Thirty-three of fifty-eight programs (57%) completed the survey. Of those, 15 programs (45%) reported having a point-of-care ultrasound curriculum. Programs with ≤3 fellows per year were less likely to have an ultrasound curriculum compared to programs with ≥4 fellows per year (30% programs 0-3 fellows/year vs. 69% programs ≥4 fellows/year, odds ratio 0.19 [95% confidence intervals 0.04-0.87]; p = .03). Program directors and associate program directors rated point-of-care ultrasound training as highly valuable to fellows' education. Barriers to use most commonly included lack of experience (64%), lack of oversight/interpretive guidance (58%), and lack of time (45%). Programs without point-of-care ultrasound training had significantly higher odds of listing lack of ultrasound access as a primary barrier (50% programs without vs. 13% programs with, odds ratio 6.5, [95% confidence intervals 1.3-50]; p = .04). CONCLUSIONS: This observational survey-based study suggests that fewer than half of pediatric anesthesia training programs in the United States offer point-of-care ultrasound education. Additional research is needed to optimize this education and training in pediatric anesthesia fellowship programs.

Identification of Achalasia Within Absent Contractility Phenotypes on High-Resolution Manometry: Prevalence, Predictive Factors, and Treatment Outcome.

INTRODUCTION: Absent contractility on high-resolution manometry (HRM) defines severe hypomotility but needs distinction from achalasia. We retrospectively identified achalasia within absent contractility using HRM provocative maneuvers, barium esophagography, and functional lumen imaging probe (FLIP). METHODS: Adult patients with absent contractility on HRM during the 4-year study period were eligible for inclusion. Inadequate studies, achalasia after therapy, or prior foregut surgery were exclusions. Upright integrated relaxation pressure (IRP) >12 mm Hg, panesophageal pressurization, and/or elevated IRP on multiple rapid swallows and rapid drink challenge (RDC) were considered abnormal. Esophageal barium retention and abnormal esophagogastric junction distensibility index (<2.0 mm 2 /mm Hg) on FLIP defined achalasia. Clinical, endoscopic, and motor characteristics of patients with achalasia were compared with absent contractility without obstruction. RESULTS: Of 164 patients, 20 (12.2%) had achalasia (17.9% of 112 patients with adjunctive testing), while 92 did not, and 52 did not undergo adjunctive tests. Achalasia was diagnosed regardless of IRP value, but the median supine IRP was higher (odds ratio 1.196, 95% confidence interval 1.041-1.375, P = 0.012). Patients with achalasia were more likely to present with dysphagia (80.0% vs 35.9%, P < 0.001), with obstructive features on HRM maneuvers (83.3% vs 48.9%, P = 0.039), but lower likelihood of GERD evidence (20.0% vs 47.3%, P = 0.027) or large hiatus hernia (15.0% vs 43.8%, P = 0.002). On multivariable analysis, dysphagia presentation ( P = 0.006) and pressurization on RDC ( P = 0.027) predicted achalasia, while reflux and presurgical evaluations and lack of RDC obstruction predicted absent contractility without obstruction. DISCUSSION: Despite HRM diagnosis of absent contractility, achalasia is identified in more than 1 in 10 patients regardless of IRP value.

Perioperative Management and Outcomes in Patients With Autism Spectrum Disorder: A Retrospective Cohort Study.

BACKGROUND: Autism spectrum disorder (ASD) is a neurocognitive disorder characterized by impairments in communication and socialization. There are little data comparing the differences in perioperative outcomes in children with and without ASD. We hypothesized that children with ASD would have higher postoperative pain scores than those without ASD. METHODS: Pediatric patients undergoing ambulatory tonsillectomy/adenoidectomy, ophthalmological surgery, general surgery, and urologic procedures between 2016 and 2021 were included in this retrospective cohort study. ASD patients, defined by International Classification of Diseases-9/10 codes, were compared to controls utilizing inverse probability of treatment weighting based on surgical category/duration, age, sex, race and ethnicity, anesthetizing location, American Society of Anesthesiology physical status, intraoperative opioid dose, and intraoperative dexmedetomidine dose. The primary outcome was the maximum postanesthesia care unit (PACU) pain score, and secondary outcomes included premedication administration, behavior at induction, PACU opioid administration, postoperative vomiting, emergence delirium, and PACU length of stay. RESULTS: Three hundred thirty-five children with ASD and 11,551 non-ASD controls were included. Maximum PACU pain scores in the ASD group were not significantly higher than controls (median, 5; interquartile range [IQR], 0-8; ASD versus median, 5; IQR, 0-8 controls; median difference [95% confidence interval {CI}] of 0 [-1.1 to 1.1]; P = .66). There was no significant difference in the use of premedication (96% ASD versus 95% controls; odds ratio [OR], 1.5; [95% CI, 0.9-2.7]; P = .12), but the ASD cohort had significantly higher odds of receiving an intranasal premedication (4.2% ASD versus 1.2% controls; OR, 3.5 [95% CI, 1.8-6.8]; P < .001) and received ketamine significantly more frequently (0.3% ASD versus <0.1% controls; P < .001). Children with ASD were more likely to have parental (4.9% ASD versus 1.0% controls; OR, 5 [95% CI, 2.1-12]; P < .001) and child life specialist (1.3% ASD versus 0.1% controls; OR, 9.9 [95% CI, 2.3-43]; P < .001) presence at induction, but were more likely to have a difficult induction (11% ASD versus 3.4% controls; OR, 3.42 [95% CI, 1.7-6.7]; P < .001). There were no significant differences in postoperative opioid administration, emergence delirium, vomiting, or PACU length of stay between cohorts. CONCLUSIONS: We found no difference in maximum PACU pain scores in children with ASD compared to a similarly weighted cohort without ASD. Children with ASD had higher odds of a difficult induction despite similar rates of premedication administration, and significantly higher parental and child life specialist presence at induction. These findings highlight the need for future research to develop evidence-based interventions to optimize the perioperative care of this population.

Information transparency with immediate release: Oncology clinician and patient perceptions.

INTRODUCTION: As part of the 21st Century Cures Act (April 2021), electronic health information (EHI) must be immediately released to patients. In this study, we sought to evaluate clinician and patient perceptions regarding this immediate release. METHODS: After surveying 33 clinicians and 30 patients, semi-structured interviews were conducted with a subset of the initial sample, comprising 8 clinicians and 12 patients. Open-ended questions explored clinicians' and patients' perceptions of immediate release of EHI and how they adjusted to this change. RESULTS: Ten themes were identified: Interpreting Results, Strategies for Patient Interaction, Patient Experiences, Communication Strategies, Provider Limitations, Provider Experiences, Health Information Interfaces, Barriers to Patient Understanding, Types of Results, and Changes due to Immediate Release. Interviews demonstrated differences in perceived patient distress and comprehension, emphasizing the impersonal nature of electronic release and necessity for therapeutic clinician-patient communication. CONCLUSIONS: Clinicians and patients have unique insights on the role of immediate release. Understanding these perspectives will help improve communication and develop patient-centered tools (glossaries, summary pages, additional resources) to aid patient understanding of complex medical information.

Teledermatology in remote Indigenous populations: Lessons learned and paths to explore, an experience from Canada (Québec) and Australia.

Objective: Recent introduction of a provincially funded and administered teledermatology platform in Quebec presents a major opportunity to improve healthcare delivery to rural Indigenous communities where healthcare is suboptimal. In this study, we assessed approaches, challenges, solutions, and outcomes in implementing teledermatology in rural Indigenous communities of Australia and Canada. Methods: A narrative review was performed using journal articles and grey literatures to assess challenges encountered in Canadian and Australian teledermatology programs in rural Indigenous communities. We then conducted a focused search to identify solutions and outcomes to these challenges. We identified four main areas of focus for implementing teledermatology: financial, cultural, legal, and provider competency. Results: Main financial concerns included identifying the cost-to-benefit ratio of teledermatology and financial benefits of the store-and-forward system compared to videoconferencing. Delivery of teledermatology through culturally considerate services is crucial to mend the mistrust felt by Indigenous people toward mainstream health services. From a legal standpoint, patient confidentiality and physician liability must be considered. A uniform teledermatology platform and physician competency in both telemedicine and dermatology are needed to ensure standard of care. Conclusion: Teledermatology initiatives represent great opportunities to improve healthcare services to rural Indigenous populations.

RGMa and Neogenin control dendritic spine morphogenesis via WAVE Regulatory Complex-mediated actin remodeling.

Structural plasticity, the ability of dendritic spines to change their volume in response to synaptic stimulation, is an essential determinant of synaptic strength and long-term potentiation (LTP), the proposed cellular substrate for learning and memory. Branched actin polymerization is a major force driving spine enlargement and sustains structural plasticity. The WAVE Regulatory Complex (WRC), a pivotal branched actin regulator, controls spine morphology and therefore structural plasticity. However, the molecular mechanisms that govern WRC activation during spine enlargement are largely unknown. Here we identify a critical role for Neogenin and its ligand RGMa (Repulsive Guidance Molecule a) in promoting spine enlargement through the activation of WRC-mediated branched actin remodeling. We demonstrate that Neogenin regulates WRC activity by binding to the highly conserved Cyfip/Abi binding pocket within the WRC. We find that after Neogenin or RGMa depletion, the proportions of filopodia and immature thin spines are dramatically increased, and the number of mature mushroom spines concomitantly decreased. Wildtype Neogenin, but not Neogenin bearing mutations in the Cyfip/Abi binding motif, is able to rescue the spine enlargement defect. Furthermore, Neogenin depletion inhibits actin polymerization in the spine head, an effect that is not restored by the mutant. We conclude that RGMa and Neogenin are critical modulators of WRC-mediated branched actin polymerization promoting spine enlargement. This study also provides mechanistic insight into Neogenin's emerging role in LTP induction.

The Role of Sex and Gender in Dermatology - From Pathogenesis to Clinical Implications.

BACKGROUND: Sex and gender have increasingly been recognized as significant risk factors for many diseases, including dermatological conditions. Historically, sex and gender have often been grouped together as a single risk factor in the scientific literature. However, both may have a distinct impact on disease incidence, prevalence, clinical presentation, severity, therapeutic response, and associated psychological distress. OBJECTIVES AND PROJECT DESCRIPTION: The mechanisms that underlie differences in skin diseases between males, females, men, and women remain largely unknown. The specific objectives of this review paper are:To highlight the biological differences between males and females (sex), as well as the sociocultural differences between men and women (gender) and how they impact the integumentary system.To perform a literature review to identify important sex- and gender-related epidemiological and clinical differences for various skin conditions belonging to a range of disease categories and to discuss possible biological and sociocultural factors that could explain the observed differences.To discuss dermatological skin conditions and gender-affirming treatments within the transgender community, a population of individuals who have a gender identity which is different than the gender identity they were assigned at birth. FUTURE IMPACT: With the rising number of individuals that identify as non-binary or transgender within our increasingly diverse communities, it is imperative to recognize gender identity, gender, and sex as distinct entities. By doing so, clinicians will be able to better risk-stratify their patients and select treatments that are most aligned with their values. To our knowledge, very few studies have separated sex and gender as two distinct risk factors within the dermatology literature. Our article also has the potential to help guide future prevention strategies that are patient-tailored rather than using a universal approach.

Analysis of an Expanded Targeted Early Cytomegalovirus Testing Program.

OBJECTIVE: Determine the detection rate from an expanded targeted early cytomegalovirus (CMV) testing program implemented from a large healthcare system (Intermountain Healthcare, IHC). STUDY DESIGN: Retrospective review. SETTING: Tertiary medical center. METHODS: An electronic system was modified to include indications for testing whenever a provider placed an order for CMV testing. A retrospective analysis of this database was performed. RESULTS: From March 1, 2021 to August 31, 2022, there were 3450 (8.8%) patients who underwent CMV testing out of 39,245 total live births within the IHC system. Since the formal implementation of this program in 2019, annual CMV testing has increased almost 10-fold: 2668 CMV tests were performed in 2021 compared to 289 CMV tests in 2015. The most frequent indication for congenital CMV (cCMV) testing was small for gestational age (SGA) (68.2%), followed by macrocephaly (13.5%), an abnormal hearing test (5.0%), and microcephaly (4.4%). Fourteen cCMV-infected infants were diagnosed all of them meeting the criteria for symptomatic cCMV. The most common indication resulting in a positive diagnosis was those who presented with SGA (n = 10 patients). The positivity rate would result in a prevalence of 35.7 symptomatic cCMV cases diagnosed per 100,000 live births, numbers comparable to those expected for universal cCMV screening. CONCLUSION: An expanded targeted early cCMV testing program may improve detection rates of symptomatic cCMV cases and should be considered as a feasible alternative approach to universal or hearing-targeted early CMV testing.

Fractionated Bilirubin Among 252 892 Utah Newborns with and Without Biliary Atresia: A 15-year Historical Birth Cohort Study.

OBJECTIVES: To determine whether neonatal conjugated or direct bilirubin levels were elevated in infants with biliary atresia (BA) and to estimate the number of newborns who would have positive screens in the nursery necessitating repeat testing after discharge. STUDY DESIGN: We used administrative data from a large integrated healthcare network in Utah to identify newborns who had a fractionated bilirubin recorded during birth admission from 2005 through 2019. Elevated conjugated bilirubin was defined as greater than 0.2 mg/dL and direct bilirubin was defined as greater than 0.5 mg/dL (>97.5th percentile for the assays). We performed simulations to estimate the anticipated number of false-positive screens. RESULTS: There were 32 cases of BA and 468 161 live births during the study period (1/14 700). There were 252 892 newborns with fractionated bilirubin assessed, including 26 of those subsequently confirmed to have BA. Conjugated or direct bilirubin was elevated in all 26 infants with BA and an additional 3246 newborns (1.3%) without BA. Simulated data suggest 9-21 per 1000 screened newborns will have an elevated conjugated or direct bilirubin using laboratory-based thresholds for a positive screen. Screening characteristics improved with higher thresholds without increasing false-negative tests. CONCLUSIONS: This study validates the previous findings that conjugated or direct bilirubin are elevated in the newborn period in patients with BA. A higher threshold for conjugated bilirubin improved screening performance. Future studies are warranted to determine the optimal screening test for BA and to assess the effectiveness and cost-effectiveness of implementing such a program.

Skin disorders and interstitial lung disease: Part II-The spectrum of cutaneous diseases with lung disease association.

Part 2 of this 2-part CME introduces dermatologists to noninfectious inflammatory skin diseases associated with pulmonary involvement. In many cases, dermatologists may be the first physicians recognizing respiratory complications associated with these diagnoses. Because pulmonary involvement is often the leading cause of morbidity and mortality, dermatologists should be comfortable screening and monitoring for lung disease in high-risk patients, recognizing cutaneous stigmata of lung disease in these patients and referring to pulmonary specialists, when appropriate, for prompt treatment initiation. Some treatments used for skin disease may not be appropriate in the context of lung disease and hence, choosing a holistic approach is important. Interstitial lung disease and pulmonary hypertension are the most common pulmonary complications and a significant cause of mortality in autoimmune connective tissue diseases, especially systemic sclerosis, dermatomyositis, and mixed connective tissue disease. Pulmonary complications, notably interstitial lung disease, are also common and life-threatening in sarcoidosis and vasculitis, while they are variable in neutrophilic and autoimmune blistering diseases.

Practical Guidelines for Managing Patients With Hidradenitis Suppurativa: An Update.

Hidradenitis suppurativa (HS) is a chronic, inflammatory skin disease that is characterized by the formation of comedones, papules, nodules, abscesses and sinus tracts in the axillary, inframammary, groin, and gluteal areas. Up to 3.8% of the Canadian population has HS, though due to a lack of awareness of HS, many patients are initially misdiagnosed and do not receive adequate treatment early on in the disease course. Once a diagnosis of HS is made, developing an effective management plan can be a dilemma for many providers. There is significant variability in response to any given therapy within the HS patient population and many HS patients have other medical comorbidities which must be taken into consideration. The aim of this review is to provide a practical approach for all healthcare providers to diagnose and manage HS and its associated comorbidities. A sample electronic medical record template for HS management was developed by the Canadian Hidradenitis Suppurativa Foundation Executive Board and is intended for use in clinical settings. This will help to increase collaboration between primary healthcare providers, dermatologists, and other medical specialists and ultimately improve the quality of care that HS patients receive.