Updated Guidelines For Management Research Articles

Blood pressure classification using the Japanese Society of Hypertension Guidelines for the Management of Hypertension and cardiovascular events among young to middle-aged working adults.

The Japanese Society of Hypertension updated guidelines for hypertension management (JSH2019), changing the blood pressure (BP) classification. However, evidence is sparse regarding the association of the classification with cardiovascular disease (CVD) events among young to middle-aged workers in Japan. We examined this issue using longitudinal data from Japan Epidemiology Collaboration on Occupational Health Study with a prospective cohort design. Participants were 81,876 workers (aged 20-64 years) without taking antihypertensive medication at baseline. BP in 2011 or 2010 was used as exposure. CVD events that occurred from 2012 to 2021 were retrieved from a within-study registry. Cox regression was used to calculate multivariable-adjusted hazard ratios of CVD events. During 0.5 million person-years of follow-up, 334 cardiovascular events, 75 cardiovascular deaths, and 322 all-cause deaths were documented. Compared with normal BP (systolic BP [SBP] < 120 mmHg and diastolic BP [DBP] < 80 mmHg), multivariable-adjusted hazard ratios (95% confidence intervals) of cardiovascular events were 1.98 (1.49-2.65), 2.10 (1.58-2.77), 3.48 (2.33-5.19), 4.12 (2.22-7.64), and 7.81 (3.99-15.30) for high normal BP (SBP120-129 mmHg and DBP < 80 mmHg), elevated BP (SBP130-139 mmHg and/or DBP80-89 mmHg), stage 1 hypertension (SBP140-159 mmHg and DBP90-99 mmHg), stage 2 hypertension (SBP160-179 mmHg and/or DBP100-109 mmHg), and stage 3 hypertension (SBP ≥ 180 mmHg and/or DBP ≥ 110 mmHg), respectively. The highest population attributable fraction was observed in elevated BP (17.8%), followed by stage 1 hypertension (14.1%). The present data suggest that JSH2019 may help identify Japanese workers at a higher cardiovascular risk.

Lessons From the Pandemic for Hand Surgery in Wales.

Aims In March 2020 the World Health Organisation (WHO) declared the COVID-19 virus a global pandemic. The United Kingdom's National Health Service (NHS) was placed under unprecedented pressure and hospitals were forced to adapt their working practices to continue offering world-leading healthcare. This project aims to highlight the lessons learnt within hand surgical departments throughout Wales. Using this knowledge, we can consider how these lessons can be implemented in both emergency and elective hand practice. Methods A qualitative questionnaire was distributed to hand consultants working across Health Boards within Wales during the pandemic. The questionnaire encompasses the impact of the pandemic on usual practices and what local departmental changes have been implemented in response to patient needs. Results Across the Welsh Health Boards, we received 12 of 19 consultant responses achieving a 63% response rate and captured data from five of seven (71%) major health boards. The questionnaire revealed that 100% of respondents changed their routine management of elective cases whilst 83% changed their management of hand trauma. 50% reported the need to issue updated management guidelines to junior doctors. The major highlighted lessons were the importance of a dedicated hand fracture clinic, coupled with a ring-fenced day-surgical unit (offering regional anaesthetic support) to manage trauma and elective patients independently from general trauma. Conclusion This qualitative research demonstrates that the pandemic drove the restructuring of many hand departments enabling us to find new, efficient ways of working. We must take these lessons forward to tackle the ever-growing waiting list, increased patient expectations and increasingly complex workloads.

Year in Review in Axial Spondyloarthritis Clinical Research and Guidelines: SPARTAN 2023 Annual Meeting Proceedings.

To highlight high impact clinical publications in spondyloarthritis from May 2022 to April 2023 that were summarized and presented at the SPARTAN annual meeting in May 2023. Publications included updated guidelines on management of axial spondyloarthritis (axSpA) by ASAS-EULAR and development of a modified Juvenile Spondyloarthritis Disease Activity Index (JSpADA). Definitions were published for MRI lesions of the spine in axSpA and active and structural sacroiliac (SI) joint lesions in juvenile spondyloarthritis (JSpA). Anatomic variants of the sacroiliac joint were commonly detected using synthetic CT derived from pelvis MRI images. Anti-CD74 antibodies hold promise as a diagnostic biomarker for axSpA; however, the mechanism of such antibody development seems unrelated to gastrointestinal inflammation. High impact clinical publications in spondyloarthritis addressed lab-based and imaging biomarkers, outcome measures, and updated management guidelines.

State of the Art in Pediatric Anesthesia: A Narrative Review about the Use of Preoperative Time.

This review delves into the challenge of pediatric anesthesia, underscoring the necessity for tailored perioperative approaches due to children's distinctive anatomical and physiological characteristics. Because of the vulnerability of pediatric patients to critical incidents during anesthesia, provider skills are of primary importance. Yet, almost equal importance must be granted to the adoption of a careful preanesthetic mindset toward patients and their families that recognizes the interwoven relationship between children and parents. In this paper, the preoperative evaluation process is thoroughly examined, from the first interaction with the child to the operating day. This evaluation process includes a detailed exploration of the medical history of the patient, physical examination, optimization of preoperative therapy, and adherence to updated fasting management guidelines. This process extends to considering pharmacological or drug-free premedication, focusing on the importance of preanesthesia re-evaluation. Structural resources play a critical role in pediatric anesthesia; components of this role include emphasizing the creation of child-friendly environments and ensuring appropriate support facilities. The results of this paper support the need for standardized protocols and guidelines and encourage the centralization of practices to enhance clinical efficacy.

452 Evaluating updated guidelines for delivery management of severe fetal growth restriction

452 Evaluating updated guidelines for delivery management of severe fetal growth restriction

Adherence to international guidelines in neurocritical care of cervical traumatic spinal cord injury-a retrospective study

IntroductionThe American Association of Neurologic Surgeons guidelines on the management of traumatic spinal cord injury (SCI), updated in 2013, focus on spinal cord perfusion, early decompressive surgery, and venous thromboembolism (VTE) prophylaxis to improve neurological outcome. Research questionHow neurocritical care and initial management have changed with the implementation of updated management guidelines, focusing on guidelines adherence and neurological outcome. Material and methodsSystemic physiological variables, time to neurosurgical treatment and VTE prophylaxis, and neurological outcome, were retrospectively collected from adult patients treated for cervical SCI 2001–2021. ResultsFifty-two patients were included. Mean arterial blood pressure (MAP) was significantly higher after 2013 (86±9.9 mmHg) when compared to before 2013 (79±9.9 mmHg), p = 0.041. Median time to surgery was 41 h before, and 20 h after 2013 (p = 0.029). Time to VTE prophylaxis was six days before and four days after 2013. Most neurocritical care complications were less commonly observed after 2013. Despite improved adherence to treatment goals, 44 % of MAP levels were below target, and 33% of patients were operated beyond 24h post-injury. Neurological outcome was not improved after implementation of the revised guidelines. Discussion and conclusionWhile implementation of the revised 2013 guidelines improved most aspects of the acute SCI management, many guideline targets were not met in a large subset of patients. Strict adherence to the acute neurocritical management goals, and early surgical treatment, is likely crucial when aiming to improve SCI outcome.

Clinical Surveillance vs Anticoagulation Therapy for Isolated Subsegmental Pulmonary Embolism: A Systematic Review of Clinical Outcomes.

This systematic review aims to evaluate and compare differences in clinical outcomes for adult patients diagnosed with ISSPE who were managed with anticoagulation vs clinical surveillance. PubMed, Embase, ProQuest, Cochrane, and Google Scholar were searched to identify studies evaluating the use of anticoagulation and/or clinical surveillance in patients diagnosed with ISSPE. The search included studies published up to August 3, 2023. Outcomes of interest included 90-day recurrent venous thromboembolism (VTE), major bleeding, and all-cause mortality rates. Ten studies were included with a total of 1224 patients. Of these patients, 791 were treated with anticoagulation and 433 underwent surveillance. Studies found no difference in recurrent VTE rates, with the majority of studies reporting no recurrence. Of the studies that reported VTE recurrence, rates were .5% to 1.4% for the anticoagulation groups and 3.1% to 3.2% for the surveillance groups. Major bleeding rates were also similar. In anticoagulated patients, major bleeding rates ranged from 1% to 10%. In clinical surveillance patients, the majority found no rate of major bleeding, with 2 studies reporting rates of .8% to 3.2%. Mortality rates ranged widely with no significant differences reported. Clinical surveillance appears to be a safe and effective alternative to anticoagulation in patients with ISSPE. Ninety-day rates of recurrent VTE, major bleeding, and mortality were comparable between groups. These findings highlight the need for updated practice management guidelines to improve patient outcomes.

Updated Clinical Practice Guidelines for Management of Hyperbilirubinemia in Infants Born at 35 Weeks or More Gestation.

Given the prevalence of jaundice in newborns, and the consequences of untreated hyperbilirubinemia, the long-awaited revised clinical practice guidelines for hyperbilirubinemia were finally released in August 2022 by the American Academy of Pediatrics as an update to the 2004 guidelines on the same topic. As new evidence and data become available, it is important for pediatricians and neonatologists to re-assess their clinical decision-making over time to ensure that patients are receiving the best care possible. With improvements in medical equipment and medical technology, and growing concerns about the overtreatment of hyperbilirubinemia, the newest clinical practice guidelines attempt to tackle the prevention, risk assessment, monitoring, and treatment of hyperbilirubinemia with these things in mind. [Pediatr Ann. 2023;52(12):e436-e439.].

Perioperative Optimization and Management of the Oral and Maxillofacial Surgical Patient: A Narrative Review on Updates in Anticoagulation, Hypertension and Diabetes Medications

PurposeThe preoperative management guidelines of surgical patients are constantly evolving as newer evidence-based research is published. Oral and maxillofacial surgeons need to be current with the increasingly more complex new drug therapies and updated national association(s) guidelines. This narrative review provides a synopsis with important reference tables for updated preoperative optimization guidelines for anticoagulation, antiplatelet therapy, antihypertensive management, and glycemic control in the preoperative period for the oral and maxillofacial surgery patient. It also includes the most current anesthesia guidelines on glucagon-like peptide (GLP-1) receptor agonists. MethodsThe search strategy utilized pubmed.gov to identify the most recent national society guidelines and review articles pertinent to perioperative anticoagulation, antiplatelet therapy, antihypertensive management, and glycemic control. ResultsThe search identified 75 articles from the American College of Surgeons, American Heart Association, American Society of Anesthesiologists, American College of Cardiologists, in addition to recent reviews discussing the standard of care for optimization of patients in the perioperative period. ConclusionMedical optimization prior to surgery is important for safe and efficient surgical practice and has been shown to improve overall mortality. This narrative review provides a summary of the current data with recommendations focusing on four key points.

Posttraumatic hydrocephalus: Recent advances and new therapeutic strategies.

Hydrocephalus or ventriculomegaly is a condition brought on by an overabundance of cerebrospinal fluid (CSF) in the ventricular system. The major contributor to posttraumatic hydrocephalus (PTH) is traumatic brain injuries (TBIs), especially in individuals with occupations set in industrial settings. A variety of criteria have been employed for the diagnosis of PTH, including the combination of neurological symptoms like nerve deficits and headache, as well as an initial improvement followed by a worsened relapse of altered consciousness and neurological deterioration, which is detected by computed tomography-brain imaging that reveals gradual ventriculomegaly. In this article, we discuss and summarize briefly the current understandings and advancements in the management of PTH. The available literature for this review was searched on various bibliographic databases using an individually verified, prespecified approach. The level of evidence of the included studies was considered as per the Centre for Evidence-Based Medicine recommendations. The commonly practiced current treatment modality involves shunting CSF but is often associated with complications and recurrence. The lack of a definitive management strategy for PTH warrants the utilization of novel and innovative modalities such as stem cell transplantations and antioxidative stress therapies. One of the worst complications of a TBI is PTH, which has a high morbidity and mortality rate. Even though there hasn't been a successful method in stopping PTH from happening, hemorrhage-derived blood, and its metabolic by-products, like iron, hemoglobin, free radicals, thrombin, and red blood cells, may be potential targets for PTH hindrance and management. Also, using stem cell transplantations in animal models and antioxidative stress therapies in future studies can lower PTH occurrence and improve its outcome. Moreover, the integration of clinical trials and theoretical knowledge should be encouraged in future research projects to establish effective and updated management guidelines for PTH.

SAT234 Variations In Clinical Practice Patterns For Hypoparathyroidism

Abstract Disclosure: K.L. Deering: None. Q. Harshaw: None. D.M. Mitchell: None. N.J. Larsen: None. B. Weiss: None. P. Loustau: None. S. Allas: None. Objectives: Emerging evidence regarding optimal treatment for patients with chronic hypoparathyroidism (cHP) has led to the development of updated evaluation and management guidelines. The goal of this study was to determine how current practice patterns for individuals with cHP compare with these guidelines. Methods: We extracted data from a large US claims database (130 million patients) from Oct 2014 to Dec 2019. cHP was defined as an HP diagnosis claim ≥6 months following neck surgery or ≥2 claims ≥6 months apart. Patient data were extracted from 1 year prior to surgery (if applicable) and up to 2 years following their qualifying HP claim. Additionally, a panel comprised of 103 endocrinologists and nephrologists (US=40, UK=21, France=21, Germany=21) was assembled and conducted chart reviews with each participant contributing data for 5 patients with cHP in their practice. Results: 5,302 patients met criteria for cHP from the claims database, while the physician panel chart review included 515 cHP patients. In the claims database, 33.0% saw an endocrinologist and 9.4% or less saw a nephrologist during the 1-year follow-up. Panel physicians reported being the decision maker for 93% of their cHP patients and 90% of the patients were seen at least twice per year. Guidelines recommend all new and established cHP patients be monitored for key laboratory values every 3-12 months. Overall, while neither study found monitoring practices consistent with guidelines, there was less laboratory monitoring in the database study than in the physician panel: serum magnesium (36.6%; 57%), serum phosphorous (36.8%; 90%), urine calcium (10.0%; 57%), and renal imaging (20.9%; 34%). Additionally, patients were monitored for bone health in the database study and physician panel (bone mineral density: 10.7%; 44%; bone markers: 5.2%; 25%). Conclusion: Real-world practice patterns vary from guideline recommendations; however, the practice patterns from the physician panel study align more closely with recommendations than the claims database study. This discrepancy may be due to patients less frequently utilizing physicians specialized in cHP in the database study. Our data underscore challenges with optimizing care for patients with this rare disorder. In addition, they suggest that ongoing efforts to educate both patients and physicians about management guidelines may improve care and ultimately enhance health and quality of life in cHP. Presentation: Saturday, June 17, 2023

An updated review for hyperuricemia and gout management; special focus on the available drug delivery systems and clinical trials.

Hyperuricemia belongs to metabolic syndromes where increased uric acid levels are identified in the blood serum. Such a syndrome could be responsible for kidney stone formation, gout, hypertension, and chronic kidney diseases. It has been reported that cardiovascular risks have been linked with hyperuricemia. Gout is of the most frequent manifestations due to hyperuricemia; its management involves various pharmacological available options and dietary changes. Throughout the literature, various dosage forms are studied as alternative options to the present drug delivery systems. To update and summarize the current information for gout and hyperuricemia management Methods: Authors have performed a thorough literature research from 2010-2023 using keywords such as hyperuricemia, gout, diagnosis, guidelines, drug delivery and clinical trials. The databases used were PubMed, ScienceDirect. According to our inclusion criteria, all studies which include the previous terms, as well as drugs or other molecules that can be applied for gout and/or hyperuricemia management, were added. In this article, authors have summarized the pathogenesis, diagnosis and updated guidelines for gout and hyperuricemia management. Moreover, the authors have reviewed and discussed current drug delivery systems found in the literature, including drugs targeting the above disorders. Finally, the available clinical trials assessing the efficacy of newer drugs or combinations of the past ones, are being discussed. The available drugs and dosage forms are limited, and therefore, scientific society should focus on the development of more efficient drug delivery systems for hyperuricemia and gout management.

Morbidity and Mortality in Adult Congenital Heart Surgery: Physiologic Component Augments Risk Prediction

BackgroundWe sought to evaluate whether the anatomic and physiologic stratification system (ACAP score), released as part of the American College of Cardiology/American Heart Association updated guidelines for management of adult congenital heart disease (ACHD) in 2018, better estimated mortality and morbidity after cardiac operations for ACHD. MethodsThe ACAP score was determined for 318 patients (age ≥18 years) with ACHD undergoing heart surgery at our institution between December 2001 and August 2019. The primary end point was perioperative mortality. The secondary aim was to evaluate the performance of the ACAP, The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories, and ACHS mortality scores/categories at predicting a composite adverse outcome of perioperative mortality, prolonged ventilation, and renal failure requiring replacement therapy. Logistic regression models were built to estimate mortality and the composite outcome using anatomic and physiologic components independently and together. Receiver operating characteristic curves were created, and area under the curves were compared using the Delong test. ResultsThe median age was 37 years (interquartile range, 26.3-50.0 years). There were 9 perioperative mortalities (2.8%). With respect to perioperative mortality, the area under the curve using the anatomic component only was 0.74, which improved to 0.81 after including physiologic severity (P = .05). When physiologic severity was added to the model for the composite outcome, the discriminatory abilities of the ACHS mortality score and the STAT categories increased significantly to 0.83 (95% CI, 0.75-0.91; P = .02) and 0.82 (95% CI, 0.73-0.90; P = .04), comparable to the predictive power of ACAP. ConclusionsPhysiologic severity augments ability to predict mortality and morbidity after cardiac surgery for ACHD. There is need for more robust ACHD-specific risk models.

Lithium related thyroid and parathyroid disease: Updated clinical practice guidelines are needed

ObjectiveThis study aims to estimate the prevalence of and determine physician approaches to the screening and management of lithium-associated thyroid and parathyroid disorders in British Columbia, Canada. MethodsSerum lithium and thyroid/parathyroid laboratory data were collected retrospectively for patients with lithium levels measured at seven BC hospitals between 2012 and 2021. A mail-out survey about screening and management of thyroid/parathyroid disorders in patients on lithium was sent to the ordering physicians of patients with abnormal results. Three months after, a follow-up questionnaire was sent to respondents, and the original survey was re-sent to non-responders. ResultsOf 4917 patients, 1.9 % had PTH (mean 22.33 ± 23.00 pmol/L) and 77.1 % had TSH (mean 3.61 ± 6.69 pmol/L) measured. Of 222 hypercalcemic patients (defined as any serum calcium or ionized calcium above the laboratory reference), 17.6 % had a PTH level measured. From 294 surveys sent to 214 physicians, the overall response rate was 31.6 % (n = 93) with twelve fully completed surveys. All twelve respondents monitored TSH levels every 6–12 months, and eight physicians monitored PTH and/or calcium at variable intervals. Two physicians routinely ordered both thyroid and parathyroid screening laboratory tests. Of the 80 non-respondents, limited patient contact was the most common reason for opting out (n = 27). ConclusionsOur results suggest biochemical screening for lithium-associated parathyroid disorders is less common than for thyroid disorders. There is insufficient data to determine the true prevalence of lithium-associated thyroid and parathyroid disorders. This highlights the need for updated clinical guidelines for management of lithium-associated thyroid and parathyroid disorders.

Updated consensus guidelines on the management of Phelan-McDermid syndrome.

Phelan-McDermid syndrome (PMS) is a genetic condition caused by SHANK3 haploinsufficiency and characterized by a wide range of neurodevelopmental and systemic manifestations. The first practice parameters for assessment and monitoring in individuals with PMS were published in 2014; recently, knowledge about PMS has grown significantly based on data from longitudinal phenotyping studies and large-scale genotype-phenotype investigations. The objective of these updated clinical management guidelines was to: (1) reflect the latest in knowledge in PMS and (2) provide guidance for clinicians, researchers, and the general community. A taskforce was established with clinical experts in PMS and representatives from the parent community. Experts joined subgroups based on their areas of specialty, including genetics, neurology, neurodevelopment, gastroenterology, primary care, physiatry, nephrology, endocrinology, cardiology, gynecology, and dentistry. Taskforce members convened regularly between 2021 and 2022 and produced specialty-specific guidelines based on iterative feedback and discussion. Taskforce leaders then established consensus within their respective specialty group and harmonized the guidelines. The knowledge gained over the past decade allows for improved guidelines to assess and monitor individuals with PMS. Since there is limited evidence specific to PMS, intervention mostly follows general guidelines for treating individuals with developmental disorders. Significant evidence has been amassed to guide the management of comorbid neuropsychiatric conditions in PMS, albeit mainly from caregiver report and the experience of clinical experts. These updated consensus guidelines on the management of PMS represent an advance for the field and will improve care in the community. Several areas for future research are also highlighted and will contribute to subsequent updates with more refined and specific recommendations as new knowledge accumulates.

A phase 1 study of the SYK inhibitor fostamatinib and weekly paclitaxel for recurrent platinum-resistant ovarian cancer.

5574 Background: Response to chemotherapy in patients (pts) with recurrent platinum-resistant ovarian cancer (PROC) is generally &lt; 15%. Spleen tyrosine kinase (SYK) is overexpressed in PROC and is a key mediator of paclitaxel resistance in ovarian cancer cells. Fostamatinib (Fos), an orally available inhibitor of SYK, is currently FDA approved for the treatment of idiopathic thrombocytopenic purpura. The active form of Fos (R406) was shown to synergistically enhance taxane-mediated cytotoxicity in preclinical ovarian cancer models. This phase 1 study aimed to identify the maximum tolerated dose (MTD) and recommended phase 2 dose (RP2D) of Fos when combined with standard weekly paclitaxel (wPac) for pts with PROC. Methods: Pts with PROC, defined as progression ≤6 months of last platinum, were eligible. A modified toxicity probability interval (mTPI) dose escalation design was used to determine the MTD/RP2D and response was assessed using RECISTv1.1 criteria. All pts received paclitaxel (wPac) 80mg/m2 intravenously on Days 1, 8, and 15, of a 28-day cycle. Fos was administered orally twice a day (BID) on a continuous schedule. Three dose levels (DL) were planned: DL1 - 100mg, DL2 - 150mg, and DL3 - 200mg. MTD was determined based on cycle 1 dose-limiting toxicities (DLTs) and responses were assessed every 2 cycles. A dose expansion cohort to further assess adverse events (AEs) and tolerability was performed. Results: 27 eligible pts, median age 62 years (range 35-79 years) with high-grade serous (n = 18), clear cell (n = 4), carcinosarcoma (n = 3), or other (n = 2) histology were treated at 3 centers. Twelve pts were treated in the dose escalation cohort (DL1 = 6, DL2 = 3, DL3 = 3). Common AEs at least possibly attributed to Fos, wPac, or both included diarrhea (70%), fatigue (52%), anemia (44%), neutropenia (33%), nausea (33%), hypertension (30%), and dysgeusia (30%). Treatment-emergent grade 3-4 AEs were neutropenia (37%), anemia (26%), and thromboembolic event (22%). During DL1, updated toxicity management guidelines not consistent with the original protocol were released and 2 of the 3 pts treated prior to the amendment were not considered evaluable for DLT. Thus 3 more pts were enrolled to DL1 after the amendment. Of the 10 pts evaluable for DLT in dose escalation, no DLTs were reported on any of the 3 DLs (DL1 = 4, DL2 = 3, DL3 = 3). DL3 was selected for dose expansion. One of 15 pts in the dose expansion cohort had a DLT (neutropenia) and one patient had a grade 5 infection. Of 18 pts treated at DL3, 7 (39%) had partial or complete response (95% CI: 17.3, 64.3%). Overall, 27 patients received a median of 3 cycles (range 0-10). Analysis of pharmacokinetic and correlative molecular studies of target expression are ongoing. Conclusions: The RP2D of Fos will be 200 mg orally BID when combined with wPac. AE profile of the combination was as expected and the combination demonstrated promising efficacy in pts with recurrent PROC. Clinical trial information: NCT03246074 .

Neonatal hyperbilirubinemia: Assessing variation in knowledge and practice.

Neonatal hyperbilirubinemia (NH) is commonly diagnosed and managed by pediatricians in various clinical settings. The 2004 American Academy of Pediatrics (AAP) Clinical Practice Guideline on NH is widely cited, but literature examining variation across pediatric specialties is limited. This study aimed to assess baseline knowledge and practice habits regarding NH among pediatric providers across various specialties immediately prior to the release of the 2022 NH clinical practice guideline. A non-probability, convenience, self-selected sampling survey was electronically distributed to 311 subjects across five specialties within one pediatric healthcare institution. The survey included eight multiple choice knowledge-based questions with confidence assessments and five management-based questions assessing respondent agreement on a 5-point scale. To compare groups, the Kruskal-Wallis and Mann-Whitney tests were used for continuous variables, and the chi-square and Fisher's exact tests were used for categorical variables. The overall survey response rate is 46%. There were significant differences between specialties' knowledge regarding NH (p<0.05). There were also significant differences between specialties' confidence ratings, independent of choosing the correct response (p<0.05). For select management-based questions, there were also significant differences between specialties (p<0.05). A majority of respondents (56%) indicated phototherapy treatment thresholds should remain the same in updated management guidelines. Significant variations in knowledge and management of NH were identified among pediatric specialties. This suggests dissemination of new guidelines must be cognizant of different constraints impacting knowledge and practice across specialties.

Updated Management Guidelines for Adenosine Deaminase Deficiency

Inherited defects in the adenosine deaminase (ADA) gene typically cause severe combined immunodeficiency. In addition to infections, ADA-deficient patients can present with neurodevelopmental, behavioral, hearing, skeletal, lung, heart, skin, kidney, urogenital, and liver abnormalities. Some patients also suffer from autoimmunity and malignancies. In recent years, there have been remarkable advances in the management of ADA deficiency. Most ADA-deficient patients can be identified by newborn screening for severe combined immunodeficiency, which facilitates early diagnosis and treatment of asymptomatic infants. Most patients benefit from enzyme replacement therapy (ERT). Allogeneic hematopoietic cell transplantation from an HLA-matched sibling donor or HLA-matched family member donor with no conditioning is currently the preferable treatment. When matched sibling donor or matched family member donor is not available, autologous ADA gene therapy with nonmyeloablative conditioning and ERT withdrawal, which is reported in recent studies to result in 100% overall survival and 90% to 95% engraftment, should be pursued. If gene therapy is not immediately available, ERT can be continued for a few years, although its excessive cost might be prohibitive. The recent improved outcome of hematopoietic cell transplantation using HLA-mismatched family-related donors or HLA-matched unrelated donors, after reduced-intensity conditioning, suggests that such procedures might also be considered rather than continuing ERT for prolonged periods. Long-term follow-up will further assist in determining the optimal treatment approach for ADA-deficient patients.

Congenital Syphilis Epidemiology, Prevention, and Management in the United States: A 2022 Update.

Congenital syphilis (CS) has dramatically increased in the United States (US) in the past decade despite the widespread availability of penicillin.Once considered an infection on the verge of elimination, CS has re-emerged as a familiar neonatal pathogen in US hospitals. This rise in cases has prompted the evaluation of potential causes and updates in prevention and management guidelines.Following a structured narrative approach, we reviewed CS data reports, peer-reviewed research articles, and updated management guidelines from state health departmentsover the past two decades. Our main search criteria centered on the treatment and prevention of CS, with a focus on prenatal health disparities. We identified geographical regions reporting disproportionate rates of CS, examined state laws regarding maternal syphilis testing, and evaluated potential reasons for the recent rise in cases. This article examines the current epidemiology, screening, and management recommendations for perinatal and CS in the US.It also reviews pathogenesis and clinical features in perinatal and pediatric populations. Finally, it highlights the likely contributing factors to increased CS rates and identifies areas for future research. Dramatically rising CS cases in certain regions and racial groups reflect gaps in the prevention, timely diagnosis, treatment, and management of perinatal syphilis and CS. Healthcare providers attending to mothers and children should recognize the re-emergence of this pathogen and be familiar with new screening and management guidelines.Increased federal funding for targeted interventions and research that address vulnerable populations is critical to curbing the re-emergence of this infection.

Updated Treatment Guidelines for Chronic Hepatitis B

Chronic hepatitis B virus (HBV) infection is a major cause of liver cirrhosis and hepatocellular carcinoma in Korea. While the prevalence of HBV infection is gradually declining in Korea, 3 of the population still suffers from HBV-related chronic liver diseases. In this review, we summarize the updated clinical guidelines for management of chronic HBV, as revised by the Korean Association for the Study of the Liver in 2022.