Saint Luke's Hospital (NY, USA)

Sickle cell disease (SCD) predominantly affects individuals of African descent and is characterized by frequent painful vaso-occlusive crises, often requiring opioid management. With the opioid epidemic raising concerns about opioid overdose, this study examines in-hospital outcomes among SCD and non-SCD patients hospitalized for opioid overdose. Using the National Inpatient Sample (NIS) from 2016 to 2021, we analyzed 479,175 opioid overdose hospitalizations, including 1,315 (0.3%) with a concomitant diagnosis of SCD. Propensity score matching was used to balance demographics, comorbidities, and hospital characteristics. SCD patients were younger (45.3% aged 30–49 vs. 33.1%, p < 0.001), predominantly of African descent (92.1% vs. 14.1%, p < 0.001), and more often from lower-income households (58.4% vs. 35.2%, p < 0.001). SCD patients had lower rates of mechanical ventilation (aOR: 0.7, 95% CI: 0.6–0.9) and anoxic brain injury (aOR: 0.5, 95% CI: 0.4–0.8) but experienced longer hospital stays and higher costs. No significant differences in in-hospital mortality were observed (aOR: 0.89, 95% CI: 0.7–1.1, p = 0.34). These findings emphasize the need for a nuanced approach to managing SCD patients during opioid overdose hospitalizations, focusing on mitigating complications, addressing prolonged hospital stays and higher costs, and reducing healthcare disparities through tailored strategies informed by the unique needs of this population.

Pan-genomics and genome-editing technologies are revolutionizing breeding of global crops1,2. A transformative opportunity lies in exchanging genotype-to-phenotype knowledge between major crops (that is, those cultivated globally) and indigenous crops (that is, those locally cultivated within a circumscribed area)3, 4–5 to enhance our food system. However, species-specific genetic variants and their interactions with desirable natural or engineered mutations pose barriers to achieving predictable phenotypic effects, even between related crops6,7. Here, by establishing a pan-genome of the crop-rich genus Solanum⁸ and integrating functional genomics and pan-genetics, we show that gene duplication and subsequent paralogue diversification are major obstacles to genotype-to-phenotype predictability. Despite broad conservation of gene macrosynteny among chromosome-scale references for 22 species, including 13 indigenous crops, thousands of gene duplications, particularly within key domestication gene families, exhibited dynamic trajectories in sequence, expression and function. By augmenting our pan-genome with African eggplant cultivars⁹ and applying quantitative genetics and genome editing, we dissected an intricate history of paralogue evolution affecting fruit size. The loss of a redundant paralogue of the classical fruit size regulator CLAVATA3 (CLV3)10,11 was compensated by a lineage-specific tandem duplication. Subsequent pseudogenization of the derived copy, followed by a large cultivar-specific deletion, created a single fused CLV3 allele that modulates fruit organ number alongside an enzymatic gene controlling the same trait. Our findings demonstrate that paralogue diversifications over short timescales are underexplored contingencies in trait evolvability. Exposing and navigating these contingencies is crucial for translating genotype-to-phenotype relationships across species.

Objective To investigate maternal morbidities and their implications on term neonates in a socioeconomically underprivileged population. Methods In a retrospective population-based study, maternal morbidities were associated with neonatal birth weight (BW), gestational age (GA), Apgar scores, respiratory distress (RD), admission to Neonatal intensive care nursery (NICU), feeding difficulties, and the length of hospital stay (LOS) in infants admitted to the level 1 nursery via multivariate regression analyses. Results In the 508 mother-infant pairs, 61.4% were Hispanic, 18.4% were African American, 9.6% were non-Hispanic white, and 10.6 % were Asian/Pacific islanders. 8.7% of the neonates [GA 38.63 (1.3) weeks and BW 3196.3 (574.6) g] had Apgar scores <7 at 1 and 2.2% at 5 minutes of life. The prevalence of maternal hypertension (8.5%), Preeclampsia-eclampsia (10.1%), Apgar scores <7 (8.7, 2.2 % at 1 and 5 minutes), NICU admission (20.4%), and feeding difficulty (7.5%) were higher than reported. Mean LOS at 4.29 days was higher than documented in term infants. Apgar scores were associated with eclampsia/preeclampsia, C-section, and antepartum hemorrhage; NICU admission with chorioamnionitis, C-section, and substance abuse; feeding difficulties with maternal alcohol consumption; and longer LOS with preeclampsia/eclampsia, chorioamnionitis, C-section, and tobacco abuse. Maternal hematocrit had positive associations with BW and GA and negative associations with LOS, feeding difficulties, and NICU admission rates. Conclusions In the underprivileged multiethnic maternal-neonatal population, term neonates exhibited low-intensity complications attributable to adverse maternal conditions. Optimizing maternal hematocrit may improve neonatal birth weight and maturational status and decrease adverse outcomes.

A BSTRACT Introduction In breast cancer care, chemotherapy is an important modality, and this study evaluated the cost-minimization analysis (CMA) considering the price of the most and the least expensive branded drugs by taking body surface area (BSA) relevant to the Indian context. Objectives The objective of this study was to conduct CMA comparing the costliest and cheapest cytotoxic drugs used to treat breast cancer in India by focusing on the direct comparison of cost. Materials and Methods The current index of medical stores was used to identify the most and least expensive brand-name medications. Based on this, the cost difference, cost ratio, and percentage variation in cost were calculated for both individual drugs and treatment regimens. The cost of the drugs is presented in both Indian rupees and American dollars. Results The anthracycline–taxane chemotherapy regimen with four cycles of adriamycin–cyclophosphamide + four cycles of paclitaxel had the biggest cost variation. Using a less expensive brand of trastuzumab instead of the most costly version resulted in an annual cost savings of approximately ₹715,065 ($9,608.51). Similar observations were seen for other regimens. Conclusions A huge difference in the cost was observed between the costliest and the cheapest brands suggesting a judicious selection can reduce financial toxicity for breast cancer patients.

Background Psoriasis, a chronic inflammatory skin disorder, is associated with an elevated risk of cardiovascular diseases due to shared inflammatory pathways. This meta‐analysis evaluates the efficacy of statins, known for their lipid‐lowering and anti‐inflammatory properties, in managing psoriasis severity. Methods A systematic search was conducted following Preferred Reporting Items for Systematic reviews and meta‐analysis guidelines across PubMed, Cochrane, Web of Science, Scopus, EMBASE, and CINAHL databases up to October 2024. Randomized clinical trials comparing statins with placebo or alternative treatments in adult psoriasis patients were included. The primary outcome was the Psoriasis Area and Severity Index (PASI) score or symptom improvement. Results Out of 11,894 identified articles, 10 randomized clinical trials were included in the final analysis. Data from eight studies with 638 observations revealed a standardized mean difference (SMD) of −0.36 (95% confidence intervals [CI]: −0.72 to 0.00; p = 0.05; I ² = 52.0% [95% CI: 0.0% to 79.5%]) for PASI scores, indicating a beneficial effect of statins on psoriasis severity, although not statistically significant. Subgroup analysis demonstrated significant effects for topical administration (SMD = −0.82; 95% CI: −1.47 to −0.16; I ² = 0%). Secondary outcomes, measured using the Dermatology Life Quality Index (DLQI), were assessed in three studies (232 observations) and showed an SMD of 0.24 (95% CI: −0.09 to 0.57; p = 0.1; I ² = 0%), indicating no significant improvement in DLQI scores. Analysis of high‐sensitivity C‐reactive protein (hsCRP) from two studies (164 observations) revealed an SMD of −0.12 (95% CI: −0.42 to 0.18; p = 0.44; I ² = 0%), indicating no significant reduction in systemic inflammation. Conclusions While statins may reduce psoriasis severity, the meta‐analysis did not show statistically significant improvements in PASI scores, except for topical application, and found no significant benefits in DLQI or hsCRP levels. Variability across studies and small sample sizes are notable limitations. Future research with larger cohorts and extended follow‐ups is warranted to clarify the potential role of statins in psoriasis management.

Marriage, couple, and family counselors and therapists are encouraged to understand adoption-related developmental considerations when working with international adult adoptees. One specific subgroup, Chinese adoptees, are entering adulthood and are well-served by adoption-informed mental health providers. This practice brief reviews demographic information about the Chinese adoptee diaspora and describes adoption-informed practices.

Background There are limited studies of juvenile dermatomyositis (JDM) in low and middle-income countries (LMIC). Many demonstrate delays to care, high prevalence of severe manifestations, and high mortality. Given the disease-associated damage with JDM, understanding JDM in Africa further is critical. Our objectives are to understand the burden of JDM in Africa and provider access to diagnostic tools and therapy through survey methodology. Methods A survey (available in English and French) was distributed via WhatsApp to 363 total members of the African League of Associations for Rheumatology (AFLAR; n = 233) and Paediatric Society of the African League Against Rheumatism (PAFLAR; n = 130) from November 2022-January 2023. Topics included respondent specialty, number of JDM patients followed, severe manifestations, and available diagnostic tools and medications (with and without considering cost). Results Forty-three (12%) of the 363 providers who received the survey started it. Among the 43 who started the survey, 37 (86%) provided consent and manage JDM patients; of these 37 providers, 4 (11%) partially and 16 (43%) fully completed the survey. Most were adult and/or pediatric rheumatologists (n = 19; 95%). Respondents represented all 5 African regions and described 216 children with JDM within the last 10 years. There was high prevalence of calcinosis (as high as 100%) and interstitial lung disease (ILD) (as high as 32%); mortality rates in Kenya (6/42; 14%) and Zambia (2/7; 29%) exceeded the 1–3% mortality reported in studies of high-income countries. Thirteen of 27 diagnostic tools and medications were accessible to ≤ 50% of respondents after considering cost, mostly in Northern or Southern Africa (9/13; 69%). Despite being cost-free, disease assessment tools and physical exam to assess calcinosis were not reported as universally available or accessible. Conclusions This is the first study to explore experiences of providers caring for children with JDM in Africa. Respondents identified 216 children with JDM seen within the last 10 years, exceeding the 196 children with JDM reported within the last 25 years but likely still underestimating prevalence. Our findings align with reports of severe manifestations and poor outcomes in African children with JDM. Access to many diagnostics and medications is limited, and differences in accessibility parallel regional healthcare disparities. The potential differences in JDM severity warrant systematic study and highlight the need to include patients and providers from LMIC in collaborative research efforts.

Unique to pediatrics is the physician–patient–parent relationship. Medical decisions are best made keeping the rights and obligations of each of these individuals in mind while of course taking into account principles of medical ethics that are widely accepted. Recognizing the difficulty sometimes inherent in balancing the best interests of the patient with parental rights, pediatricians have recourse to multidisciplinary team meetings. If no resolution is found there, they can call on institutional ethics committees, as outlined by the American Academy of Pediatrics, for the resolution of ethically challenging cases.

This chapter gives a brief description of the most common pediatric musculoskeletal disorders (congenital, developmental, and acquired) and injuries. Clinical pictures and radiographs are provided to help explain the pathology of these conditions.

Introduction Premature advanced subclinical coronary atherosclerosis among young adults is an under-recognized and unique disease phenotype that has not been well characterized. Methods We used data from 44,047 participants with no prior CVD history (59.8% male) from the Coronary Artery Calcium (CAC) Consortium. We defined advanced disease as CAC ≥90th percentile for age, sex, and race, and compared risk factor profile of persons with advanced disease to those without CAC and those with CAC <90th percentile. Using multivariable-adjusted Cox proportional hazard and competing risks regression, we assessed the association of premature advanced disease with all-cause, cardiovascular, and CHD mortality. Results Of 44,047 participants, 18,561 (42.2%) had CAC. Among those with CAC, 6,680 (36.0%) had CAC ≥90th percentile. Notably, 76.4% of those with CAC ≥90th percentile had multivessel CAC compared to 40.6% of those with CAC <90th percentile. After a mean follow-up of 12.5±3.6 years, the incidence per 1,000 person-years of all-cause (2.93 vs 1.85 vs 1.11), cardiovascular (1.11 vs 0.39 vs 0.21), and CHD mortality (0.65 vs 0.19 vs 0.08) was highest in the advanced disease group compared to CAC <90th percentile and the no CAC group. Persons with CAC ≥90th percentile had a higher multivariable-adjusted risk of all-cause (HR:2.17[1.83–2.57]), cardiovascular (SHR:3.89[2.78–5.44]), and CHD mortality (SHR:5.45[3.38–8.78]), compared to those without CAC. In the subgroup analysis, there was no difference in mortality between men and women with advanced CAC. Conclusions Premature advanced atherosclerosis is a distinct clinical phenotype that strongly predicts all-cause and cause-specific mortality. Among persons with CAC at young age, those with scores ≥ 90th percentile have the highest risk of early death and should be identified in future guidelines as a focus for aggressive clinical prevention.

Objective We aimed to study the disease course, outcomes, and predictors of outcome in pediatric‐onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) affecting the kidneys. Methods Patients eligible for this study had a diagnosis of granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or ANCA‐positive pauci‐immune glomerulonephritis, were 18 years or younger at diagnosis, had renal disease defined by biopsy or dialysis dependence, and had clinical data at diagnosis and at either 12 or 24 months. Ambispective data from A Registry for Children with Vasculitis/Pediatric Vasculitis Initiative Registry was used. The primary outcome was inactive renal disease (pediatric vasculitis activity score = 0 or 1) at 12 months. Secondary outcomes included rates of improved renal function and damage within 24 months. Renal function, defined by estimated glomerular filtration rate, was categorized into Kidney Disease Improving Global Outcomes (KDIGO) stages at diagnosis and tested as a predictor of outcome using a proportional‐odds logistic regression model. Results A total of 145 patients were included: 68% were female, and 78% had GPA. At 12 months, 83% of patients achieved inactive renal disease; however, 42% had evidence of permanent renal damage. Compared with patients with normal renal function at diagnosis, patients with moderate to severely reduced renal function, or kidney failure at diagnosis, had an odds ratio of 8.62 (P = 0.002; 95% confidence interval [CI] 2.31–32.1) and 26.3 (P < 0.001; 95% CI 6.32–109), respectively, for being in a non‐normal KDIGO category at 12 months. Conclusion The majority of patients with pediatric AAV achieve inactive renal disease by 12 months; however, almost half have evidence of damage. Renal function at diagnosis is a strong predictor of renal function at 12 months.

Purpose of Review This review aims to explain the causes, diagnosis, and treatment of malnutrition in hospitalized patients with inflammatory bowel disease (IBD), focusing on both adults and children. Recent Findings Malnutrition is common among IBD patients, affecting up to 85% of individuals, and is linked to higher rates of illness, death, and longer hospital stays. Recent studies highlight the importance of early detection using tools like the Subjective Global Assessment (SGA) and handgrip strength tests. Nutritional interventions—such as exclusive enteral nutrition (EEN) and parenteral nutrition (PN)—have proven effective in inducing remission and improving patient outcomes, especially in pediatric Crohn’s disease. New evidence suggests that optimizing nutrition before and after surgery, as well as using immunonutrition, may reduce postoperative complications. Summary Early identification and management of malnutrition in hospitalized IBD patients may be important for improving clinical outcomes. Using appropriate nutritional screening tools and creating personalized nutrition plans can help with recovery, decrease hospital stays, and improve quality of life. Further research is needed to develop standard protocols for nutritional assessment and treatment in this patient population.

Axillary web syndrome (AWS), also known as Mondor Disease, is a self-limiting condition characterized by axillary pain radiating down the anteromedial upper extremity with an associated decrease in shoulder abduction and overlying palpable cords. AWS is commonly seen in female patients with a history of axillary clearance (AC), though it is also associated with patients who have undergone sentinel lymph node biopsy (SLNB), mastectomy, or radiation therapy to the axilla or breast. AWS is a significant cause of morbidity in such patients. While the exact pathophysiology is not entirely understood, AWS is thought to arise from a complex interplay of disruptions in lymphatic flow and connective tissue fibrosis following the interventions described. We present a unique case of AWS with concurrent thrombophlebitis in a 25-year-old male who presented to the ED with a two-day history of right axillary pain and no past medical or surgical history. On physical examination, the patient showed decreased range of motion (ROM) in abduction of the right upper extremity (RUE) with an overlying palpable cord-like structure at the proximal medial RUE. Following a Doppler ultrasound (US), a diagnosis of AWS with concurrent superficial thrombophlebitis was made. This case highlights the importance of considering AWS in patients without a suggestive medical history, along with the potential utility of US in approaching a diagnosis.

The Clever Hans effect occurs when machine learning models rely on spurious correlations instead of clinically relevant features and poses significant challenges to the development of reliable artificial intelligence (AI) systems in medical imaging. This scoping review provides an overview of methods for identifying and addressing the Clever Hans effect in medical imaging AI algorithms. A total of 173 papers published between 2010 and 2024 were reviewed, and 37 articles were selected for detailed analysis, with classification into two categories: detection and mitigation approaches. Detection methods include model-centric, data-centric, and uncertainty and bias-based approaches, while mitigation strategies encompass data manipulation techniques, feature disentanglement and suppression, and domain knowledge-driven approaches. Despite the progress in detecting and mitigating the Clever Hans effect, the majority of current machine learning studies in medical imaging do not report or test for shortcut learning, highlighting the need for more rigorous validation and transparency in AI research. Future research should focus on creating standardized benchmarks, developing automated detection tools, and exploring the integration of detection and mitigation strategies to comprehensively address shortcut learning. Establishing community-driven best practices and leveraging interdisciplinary collaboration will be crucial for ensuring more reliable, generalizable, and equitable AI systems in healthcare.

Epiglottitis is an inflammatory condition involving the epiglottis and surrounding tissues. While it can develop at any age, it was traditionally more common in children, largely due to infections with Haemophilus influenzae type B (Hib). Since the introduction of the Hib vaccine, the incidence in children has significantly decreased, while cases in adults have become more prevalent. We present here the case of a 44-year-old male who presented to the emergency department with a one-day history of throat pain. He reported throat discomfort and pain exacerbated by swallowing and phonation that began one day prior after taking a dose of azithromycin. X-ray and CT imaging revealed inflammation and swelling of the epiglottis (thumbprint sign) with near-complete airway obstruction. Despite the absence of severe respiratory distress, the patient was intubated, differing from recommendations in previous studies. This case emphasizes the importance of developing standardized treatment protocols for acute epiglottitis in adults, particularly regarding airway management and minimizing unnecessary imaging.

Inguinal hernias are the most prevalent type of abdominal wall hernia. While many cases are uncomplicated, some variant forms can pose a heightened risk of severe complications. We report the case of a 46-year-old male who arrived at the emergency department with a two-day history of diffuse abdominal pain, with an otherwise negative review of systems, an unremarkable medical and surgical history, and normal laboratory results. The physical examination revealed a palpable, non-reducible mass in the right groin, raising suspicion of an inguinal hernia. A CT scan of the abdomen and pelvis was conducted, confirming Amyand's hernia, characterized by the presence of the vermiform appendix within the hernia sac in the dilated right inguinal canal. Amyand's hernia is a rare and clinically challenging condition to diagnose because its symptoms are nonspecific and often resemble those of other inguinal hernias. Delayed diagnosis can heighten the risk of complications, including inflammation, infection, perforation, and acute appendicitis. Mortality rates for Amyand's hernias have been reported to be between 14% and 30%, primarily due to complications associated with infections, especially peritoneal sepsis. Prompt diagnosis and treatment of Amyand's hernia, usually involving surgical hernia repair and an appendectomy if appendicitis is present, are highly effective in preventing potentially life-threatening complications. Despite their rarity, the difficult nature of the diagnosis and the associated high mortality rate underscore the importance of considering Amyand's hernia as a serious differential diagnosis.