Azathioprine-induced hypersensitivity reaction mimicking sepsis in a patient with systemic lupus erythematosus.

A 21-year-old woman with a history of systemic lupus erythematosus presented to the emergency department with acute-onset nausea, vomiting, and fevers. Two weeks prior, she was started on azathioprine 50 mg daily by her outpatient rheumatologist; the dose was up-titrated to 100 mg when repeat blood work showed no drug toxicity. The morning after increasing her dose, she was awoken by recurrent emesis. At presentation, she was febrile, tachycardic, and hypotensive. Her exam showed mild, generalised abdominal tenderness but was otherwise unremarkable. Lab work demonstrated elevated inflammatory markers, elevated liver transaminases, and stable hypocomplementemia. Chest X-ray and computed tomography abdomen/pelvis were unrevealing. She was given intravenous fluids and broad-spectrum antibiotics, and azathioprine was held. A thorough infectious workup returned negative. A flare of her systemic lupus erythematosus was considered but deemed an unlikely explanation of her systemic inflammatory response syndrome. With azathioprine discontinuation, she made a rapid, near-complete recovery within 24 h of admission, suggesting a diagnosis of azathioprine hypersensitivity syndrome. This case exemplifies the difficulty in distinguishing azathioprine hypersensitivity from mimickers such as infection and underlying autoimmune disease flare. Prompt recognition of hypersensitivity can lead to appropriate discontinuation of the drug and prevent future morbidity.

Confluent and reticulated papillomatosis in pediatric patients at an urban tertiary care center.

Confluent and reticulated papillomatosis (CARP) is a dermatosis that often presents during adolescence. Prior studies have linked CARP to metabolic syndrome and comorbidities associated with insulin resistance, such as acanthosis nigricans and type 2 diabetes. Despite this, few studies have evaluated the clinical relationship between glucose dysmetabolism and CARP. In this report, we describe the characteristics of a large cohort of pediatric patients with CARP to further evaluate the potential relationship between CARP and metabolic syndrome in children.

Evaluation of the Prevalence and Incidence of Pediatric Alopecia Areata Using Electronic Health Record Data.

Importance: Pediatric alopecia areata (AA) prevalence and incidence data are key to understanding the natural history of this medical disease. Objective: To determine the prevalence and incidence of AA in a pediatric population across time, age, sex, race and ethnicity, and geographic areas within the US. Design, Setting, and Participants: In this multicenter cohort study conducted among 5 children's hospitals, data (January 2009 to November 2020) were collected from a standardized electronic health record (PEDSnet database, version 4.0) to evaluate the incidence and prevalence of pediatric AA. The study cohort included patients younger than 18 years with at least 2 physician visits during which a diagnosis code for AA was recorded, or 1 dermatologist specialty visit for which AA was recorded. Main Outcomes and Measures: The prevalence denominator population comprised 5 409 919 patients. The incidence denominator population was 2 896 241. We identified 5801 children for inclusion in the AA cohort, and 2398 (41.3%) had 12 months or more of follow-up and were included in the incidence analysis. Results: Of 5801 patients in the AA cohort, the mean (SD) age was 9.0 (4.5) years, 3259 (56.2%) were female, 359 (6.2) were Asian, 1094 (18.9%) were Black, 1348 (23.2%) were Hispanic, and 2362 (40.7%) were White. The overall prevalence of pediatric AA was 0.11%, and the participants in the AA cohort were more often older, female, and members of a racial and ethnic minority group than the full PEDSnet population. The 11-year overall incidence rate of pediatric AA between 2009 and 2020 was 13.6 cases per 100 000 person-years (95% CI, 13.1-14.2). The incidence rate by age was normally distributed and peaked at age 6 years. Rates were 22.8% higher in female patients than male patients (15.1 cases per 100 000 person-years for females vs 12.3 cases per 100 000 person-years for males). Additionally, incidence rates were highest among Hispanic children (31.5 cases per 100 000 person-years). Conclusions and Relevance: This cohort study examined the prevalence and incidence rates of pediatric AA in the US across time, age, sex, race and ethnicity, and region from 2009 to 2020, finding a prevalence of 0.11% (doubling during the last decade) and incidence rate of 13.6 cases per 100 000 person-years. Additionally, the results identified Asian and Hispanic children as high-risk demographic subgroups who were shown to be 2 and 3 times more likely, respectively, to receive a diagnosis of AA.

Experience using dupilumab for pediatric atopic dermatitis at a tertiary care c enter: Inadequate response and adverse events.

BACKGROUND/OBJECTIVE: Dupilumab is highly effective in treating atopic dermatitis (AD). However, some patients experience difficulties with dupilumab therapy, such as inadequate clinical response, failure to achieve long-term disease control, or adverse events (AEs). Our objective is to assess inadequate response and AEs occurring in children on dupilumab therapy for AD. METHODS: This is a retrospective cohort study of children on dupilumab for AD. Collected variables included patient demographics, medical histories, and dupilumab therapy characteristics. Response analysis was conducted in those with ≥3 months of dupilumab therapy: primary poor responders were defined as those whose EASI scores did not decrease by >50%, and secondary poor responders were those who initially responded but had significant AD flares while on therapy. RESULTS: We included 200 patients on dupilumab for AD in our cohort; 192 received ≥3 months of therapy and were included in our response analysis. Twelve children experienced inadequate primary response, and 4 were secondary poor responders. Four of these 16 children discontinued therapy due to inadequate response. The most common dupilumab-associated AEs were facial erythema (n = 24, 12.0%) and injection-site reactions (n = 24, 12.0%). Female sex was significantly associated with experiencing injection-site reactions, and prior hospitalization was significantly associated with HSV infection on dupilumab. Eight patients discontinued therapy due to an AE. CONCLUSION: A small but significant number of patients experienced treatment difficulties while on dupilumab. The risk of inadequate response to dupilumab and dupilumab-associated AEs should be discussed thoroughly with patients and their families prior to initiation.

Localized hair loss in infancy: a review.

PURPOSE OF REVIEW: The current review will address the different causes of localized hair loss in infancy. The data presented here will provide clinicians with the latest understanding of different disorders leading to localized hair loss and will provide recommendations for further management of infants who present with alopecia. RECENT FINDINGS: Localized hair loss in infancy is common, but its underlying causes vary greatly. Alopecia in infants can be categorized into congenital, genetic, inflammatory, mechanical, and physiologic causes. Decisions regarding further management are complex, as they often involve not only cosmetic concerns, but also work-up of possible systemic medical issues related to hair loss. SUMMARY: Clinicians must be able to distinguish between the different causes of infantile hair loss so that appropriate work-up and further management can be pursued. Factors such as physical appearance, timing of presentation, dermoscopic exam, histopathology, and associated systemic features can help lead clinicians to the correct diagnosis in the case of an infant with localized alopecia.

Retraining Convolutional Neural Networks for Specialized Cardiovascular Imaging Tasks: Lessons from Tetralogy of Fallot.

Ventricular contouring of cardiac magnetic resonance imaging is the gold standard for volumetric analysis for repaired tetralogy of Fallot (rTOF), but can be time-consuming and subject to variability. A convolutional neural network (CNN) ventricular contouring algorithm was developed to generate contours for mostly structural normal hearts. We aimed to improve this algorithm for use in rTOF and propose a more comprehensive method of evaluating algorithm performance. We evaluated the performance of a ventricular contouring CNN, that was trained on mostly structurally normal hearts, on rTOF patients. We then created an updated CNN by adding rTOF training cases and evaluated the new algorithm's performance generating contours for both the left and right ventricles (LV and RV) on new testing data. Algorithm performance was evaluated with spatial metrics (Dice Similarity Coefficient (DSC), Hausdorff distance, and average Hausdorff distance) and volumetric comparisons (e.g., differences in RV volumes). The original Mostly Structurally Normal (MSN) algorithm was better at contouring the LV than the RV in patients with rTOF. After retraining the algorithm, the new MSN + rTOF algorithm showed improvements for LV epicardial and RV endocardial contours on testing data to which it was naïve (N = 30; e.g., DSC 0.883 vs. 0.905 for LV epicardium at end diastole, p < 0.0001) and improvements in RV end-diastolic volumetrics (median %error 8.1 vs 11.4, p = 0.0022). Even with a small number of cases, CNN-based contouring for rTOF can be improved. This work should be extended to other forms of congenital heart disease with more extreme structural abnormalities. Aspects of this work have already been implemented in clinical practice, representing rapid clinical translation. The combined use of both spatial and volumetric comparisons yielded insights into algorithm errors.