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Interconception Care and Safe Sleep: Adapting the IMPLICIT Toolkit for Pediatrics

Lun, 01/11/2021 - 09:01
BACKGROUND

A woman’s health in the interconception period has an impact on birth outcomes. Pediatric visits offer a unique opportunity to provide interconception care (ICC). Our aim was to screen and provide interconception and safe sleep screening, counseling, and interventions for 50% of caregivers of children <2 years of age in a pediatric medical setting.

METHODS

Two pediatric clinics implemented the March of Dimes’ Interventions to Minimize Preterm and Low Birth Weight Infants Through Continuous Improvement Techniques (IMPLICIT) toolkit, in addition to standardized safe sleep assessments. A quality improvement learning collaborative was formed with a local "infant mortality champion" leading quality improvement efforts. Monthly webinars with the clinic teams reviewed project successes and challenges. Framework for Reporting Adaptations and Modifications was used to document adaptations.

RESULTS

For each individual IMPLICIT domain, clinics screened and provided needed interventions for ICC and safe sleep in >50% of eligible encounters. Over the course of the quality improvement learning collaborative, the number of caregivers screened for at least 4 of the 5 IMPLICIT domains increased from 0% to 95%.

CONCLUSIONS

To successfully implement the IMPLICIT toolkit in pediatrics, adaptations were made to the existing model, which had previously been used in family medicine clinics. Pediatricians should consider providing ICC as an innovative way to impact infant mortality rates in their community. Framework for Reporting Adaptations and Modifications can be used to systematically describe the adaptations needed to improve the fit of IMPLICIT in the pediatric clinic, understand the process of change and potential application to local context.

An Intervention to Improve Chlamydia and Gonorrhea Testing Among Adolescents in Primary Care

Lun, 01/11/2021 - 09:01
BACKGROUND AND OBJECTIVES

Rates of chlamydia and gonorrhea among adolescents continue to rise. We aimed to evaluate if a universal testing program for chlamydia and gonorrhea improved testing rates in an urban general pediatric clinic and an urban family medicine clinic within a system of federally qualified health care centers and evaluated the feasibility, cost, and logistic challenges of expanding implementation across 28 primary care clinics within a federally qualified health care centers system.

METHODS

A universal testing quality improvement program for male and female patient 14 to 18 years old was implemented in a general pediatrics and family medicine clinic in Denver, Colorado. The intervention was evaluated by using a controlled pre-post quasi-experimental design. The difference in testing rates due to the intervention was assessed by using a difference-in-differences regression model weighted with the inverse probability of treatment.

RESULTS

In total, 15 541 pediatric encounters and 5420 family medicine encounters were included in the analyses. In pediatrics, the unadjusted testing rates increased from 32.0% to 66.7% in the intervention group and from 20.9% to 28.9% in the comparison group. For family medicine, the rates increased from 38.5% to 49.9% in the intervention group and decreased from 26.3% to 24.8% in the comparison group. The intervention resulted in an adjusted increase in screening rates of 25.2% (P < .01) in pediatrics and 11.8% (P < .01) in family medicine. The intervention was well received and cost neutral to the clinic.

CONCLUSIONS

Universal testing for chlamydia and gonorrhea in primary care pediatrics and family medicine is a feasible approach to improving testing rates .

Constraint-Induced Movement Therapy for Cerebral Palsy: A Randomized Trial

Lun, 01/11/2021 - 09:01
OBJECTIVES

With the Children with Hemiparesis Arm and Hand Movement Project (CHAMP) multisite factorial randomized controlled trial, we compared 2 doses and 2 constraint types of constraint-induced movement therapy (CIMT) to usual customary treatment (UCT).

METHODS

CHAMP randomly assigned 118 2- to 8-year-olds with hemiparetic cerebral palsy to one of 5 treatments with assessments at baseline, end of treatment, and 6 months posttreatment. Primary blinded outcomes were the assisting hand assessment; Peabody Motor Development Scales, Second Edition, Visual Motor Integration; and Quality of Upper Extremity Skills Test Dissociated Movement. Parents rated functioning on the Pediatric Evaluation of Disabilities Inventory-Computer Adaptive Test Daily Activities and Child Motor Activity Log How Often scale. Analyses were focused on blinded and parent-report outcomes and rank-order gains across all measures.

RESULTS

Findings varied in statistical significance when analyzing individual blinded outcomes. parent reports, and rank-order gains. Consistently, high-dose CIMT, regardless of constraint type, produced a pattern of greatest short- and long-term gains (1.7% probability of occurring by chance alone) and significant gains on visual motor integration and dissociated movement at 6 months. O’Brien’s rank-order analyses revealed high-dose CIMT produced significantly greater improvement than a moderate dose or UCT. All CIMT groups improved significantly more in parent-reported functioning, compared with that of UCT. Children with UCT also revealed objective gains (eg, 48% exceeded the smallest-detectable assisting hand assessment change, compared with 71% high-dose CIMT at the end of treatment).

CONCLUSIONS

CHAMP provides novel albeit complex findings: although most individual blinded outcomes fell below statistical significance for group differences, high-dose CIMT consistently produced the largest improvements at both time points. An unexpected finding concerns shifts in UCT toward higher dosages, with improved outcomes compared with previous reports.

Corticosteroids in the Treatment of Pediatric Retropharyngeal and Parapharyngeal Abscesses

Lun, 01/11/2021 - 09:01
BACKGROUND AND OBJECTIVES

Treatment of retropharyngeal abscesses (RPAs) and parapharyngeal abscesses (PPAs) includes antibiotics, with possible surgical drainage. Although corticosteroids may decrease inflammation, their role in the management of RPAs and PPAs is unclear. We evaluated the association of corticosteroid administration as part of initial medical management on drainage rates and length of stay for children admitted with RPAs and PPAs.

METHODS

We conducted a retrospective study using administrative data of children aged 2 months to 8 years discharged with RPAs and PPAs from 2016 to 2019. Exposure was defined as systemic corticosteroids administered as part of initial management. Primary outcome was surgical drainage. Bivariate comparisons were made between patients in the corticosteroid and noncorticosteroid groups by using Wilcoxon rank or 2 tests. Outcomes were modeled by using generalized linear mixed-effects models.

RESULTS

Of the 2259 patients with RPAs and PPAs, 1677 (74.2%) were in the noncorticosteroid group and 582 (25.8%) were in the corticosteroid group. There were no significant differences in age, sex, or insurance status. There was a lower rate of drainage in the corticosteroid cohort (odds ratio: 0.28; confidence interval: 0.22–0.36). Patients in this group were more likely to have repeat computed tomography imaging performed, had lower hospital costs, and were less likely to have opioid medications administered. The corticosteroid cohort had a higher 7-day emergency department revisit rate, but there was no difference in length of stay (rate ratio 0.97; confidence interval: 0.92–1.02).

CONCLUSIONS

Corticosteroids were associated with lower odds of surgical drainage among children with RPAs and PPAs.

Land Rights and Health Outcomes in American Indian/Alaska Native Children

Lun, 01/11/2021 - 09:01

American Indian and Alaska Native (AI/AN) land rights, sovereignty conflicts, and health outcomes have been significantly influenced by settler colonialism. This principle has driven the numerous relocations and forced assimilation of AI/AN children as well as the claiming of AI/AN lands across the United States. As tribes across the country begin to reclaim these lands and others continue to struggle for sovereignty, it is imperative to recognize that land rights are a determinant of health in AI/AN children. Aside from the demonstrated biological risks of environmental health injustices including exposure to air pollution, heavy metals, and lack of running water, AI/AN children must also face the challenges of historical trauma, the Missing and Murdered Indigenous Peoples crisis, and health care inequity based on land allocation. Although there is an undeniable relationship between land rights and the health of AI/AN children, there is a need for extensive research into the impacts of land rights and recognition of sovereignty on the health of AI/AN children. In this article we aim to summarize existing evidence describing the impact of these factors on the health of AI/AN children and provide strateg ies that can help pediatricians care and advocate for this population.

Reducing Benzodiazepine Exposure by Instituting a Guideline for Dexmedetomidine Usage in the NICU

Lun, 01/11/2021 - 09:01
BACKGROUND

Midazolam is a benzodiazepine sedative used in NICUs. Because benzodiazepine’s effects include respiratory depression and potential detrimental developmental effects, minimizing exposure could benefit neonates. Dexmedetomidine is routinely used for sedation in older pediatric populations. We implemented a quality improvement initiative with the aim of decreasing midazolam infusions by 20% through use of dexmedetomidine.

METHODS

A multidisciplinary committee created a sedation guideline that included standardized dexmedetomidine dosing escalation and weaning. Baseline data collection occurred from January 2015 to February 2018, with intervention from March 2018 to December 2019. Percentage of sedation episodes with dexmedetomidine initiated was followed as a process measure. Outcomes measures were percentage of eligible infants receiving midazolam infusions and midazolam-free days per sedation episode. Bradycardia with dexmedetomidine, unplanned extubation rates, and morphine dosage were monitored as balancing measures.

RESULTS

Our study included 434 episodes of sedation in 386 patients. Dexmedetomidine initiation increased from 18% to 49%. The intervention was associated with a significant reduction in midazolam initiation by 30%, from 95% to 65%, with special cause variation on statistical process control chart analysis. Midazolam-free days per sedation episode increased from 0.3 to 2.2 days, and patients receiving dexmedetomidine had lower midazolam doses (1.3 mg/kg per day versus 2.2 mg/kg per day, P = 5.97 x 10–04). Bradycardia requiring discontinuation of dexmedetomidine, unplanned extubation rates, and morphine doses were unchanged.

CONCLUSIONS

Implementation of a quality improvement initiative was successful in reducing the percentage of patients receiving midazolam infusions and increased midazolam-free days per sedation episode, revealing an overall reduction in benzodiazepine exposure while maintaining adequate sedation.

Neonatal MIS-C: Managing the Cytokine Storm

Lun, 01/11/2021 - 09:01

A term infant girl with uneventful antenatal history had an erythematous rash followed by fever from day 8. She was diagnosed with late-onset sepsis and was treated accordingly. She received immunoglobulin for persistent thrombocytopenia, after which there was transient improvement. The patient was transferred to our hospital on day 25 after recurrence of fever, watery diarrhea, and a generalized maculopapular rash. On admission, she had tachycardia, tachypnoea, anemia, thrombocytopenia, hypoalbuminemia, and generalized edema. Reverse transcriptase–polymerase chain reaction results for coronavirus disease 2019 (COVID-19) was positive. Within 12 hours of admission, she developed cardiogenic shock with pulmonary edema and needed invasive ventilation. Echocardiography revealed ejection fraction of 40% with mild pericardial effusion. N-terminal pro–brain natriuretic peptide was 33000 g/L, D-dimer 16500 µg/L, and ferritin 16000 ng/mL. Methylprednisolone, immunoglobulin, and enoxaparin was started, with a diagnosis of multisystem inflammatory syndrome in children, associated with COVID-19. She developed seizures, pulmonary hemorrhage, and cardiac arrest the following day, along with acute kidney injury. She was extubated after 5 days. Steroid was stopped after 5 days because she developed hypertension and echocardiography had normalized. Five days after extubation, she again developed respiratory distress and was ventilated again for 2 days. Echocardiography revealed moderate left ventricular dysfunction, along with secondary elevation of ferritin. Methylprednisolone was restarted and continued for 5 days followed by tapering dose of oral prednisolone, on which she was finally discharged. Although mild myocarditis with COVID-19 has been reported, multisystem inflammatory syndrome in children in a newborn with refractory myocarditis, along with gastrointestinal and renal manifestations, is a rare entity. Dermatologic manifestation of neonatal COVID-19 is also unique.

Transgender Youth Referred to Clinics for Gender-Affirming Medical Care in Canada

Lun, 01/11/2021 - 09:01
BACKGROUND AND OBJECTIVES

Referrals of transgender and gender-diverse (trans) youth to medical clinics for gender-affirming care have increased. We described characteristics of trans youth in Canada at first referral visit.

METHODS

Baseline clinical and survey data (2017–2019) were collected for Trans Youth CAN!, a 10-clinic prospective cohort of n = 174 pubertal and postpubertal youth <16 years with gender dysphoria, referred for hormonal suppression or hormone therapy, and 160 linked parent-participants. Measures assessed health, demographics, and visit outcome.

RESULTS

Of youth, 137 were transmasculine (assigned female) and 37 transfeminine (assigned male); 69.0% were aged 14 to 15, 18.8% Indigenous, 6.6% visible minorities, 25.7% from immigrant families, and 27.1% low income. Most (66.0%) were gender-aware before age 12. Only 58.1% of transfeminine youth lived in their gender full-time versus 90.1% of transmasculine (P < .001). Although transmasculine youth were more likely than transfeminine youth to report depressive symptoms (21.2% vs 10.8%; P = .03) and anxiety (66.1% vs 33.3%; P < .001), suicidality was similarly high overall (past-year ideation: 34.5%, attempts: 16.8%). All were in school; 62.0% reported strong parental gender support, with parents the most common support persons (91.9%). Two-thirds of families reported external gender-related stressors. Youth had met with a range of providers (68.5% with a family physician). At clinic visit, 62.4% were prescribed hormonal suppression or hormone therapy, most commonly depot leuprolide acetate.

CONCLUSIONS

Trans youth in Canada attending clinics for hormonal suppression or gender-affirming hormones were generally healthy but with depression, anxiety, and support needs.

Next-Generation Sequencing as an Auxiliary Tool in Pediatric Laryngeal Lymphoma Diagnosis

Lun, 01/11/2021 - 09:01

Lymphomatous involvement of the larynx is a rare entity. We present a case of atypical laryngotracheitis as the initial manifestation of non-Hodgkin’s lymphoma in a pediatric patient. The diagnosis was aided through the use of microbial cell-free DNA (mcfDNA) testing, which detected the presence of Epstein-Barr virus in the patient’s plasma. This enabled the consideration of an Epstein-Barr virus–related lymphoproliferative process, leading to additional workup and the final diagnosis of lymphoma. To our knowledge, this is the first case of mcfDNA testing leading not simply to an infectious organism, but further to a new oncologic diagnosis. Plasma mcfDNA testing has the potential to inform clinical practice beyond classic infectious disease manifestations. In this article, we review both the possible future applications and the areas of further investigation that remain.

Addressing Myths and Vaccine Hesitancy: A Randomized Trial

Lun, 01/11/2021 - 09:01
OBJECTIVES

Evidence on repeating vaccination misinformation or "myths" in debunking text is inconclusive; repeating myths may unintentionally increase agreement with myths or help discredit myths. In this study we aimed to compare the effect of repeating vaccination myths and other text-based debunking strategies on parents’ agreement with myths and their intention to vaccinate their children.

METHODS

For this online experiment we recruited 788 parents of children aged 0 to 5 years; 454 (58%) completed the study. We compared 3 text-based debunking strategies (repeating myths, posing questions, or making factual statements) and a control. We measured changes in agreement with myths and intention to vaccinate immediately after the intervention and at least 1 week later. The primary analysis compared the change in agreement with vaccination myths from baseline, between groups, at each time point after the intervention.

RESULTS

There was no evidence that repeating myths increased agreement with myths compared with the other debunking strategies or the control. Posing questions significantly decreased agreement with myths immediately after the intervention compared with the control (difference: –0.30 points, 99.17% confidence interval: –0.58 to –0.02, P = .004, d = 0.39). There was no evidence of a difference between other debunking strategies or the control at either time point, or on intention to vaccinate.

CONCLUSIONS

Debunking strategies that repeat vaccination myths do not appear to be inferior to strategies that do not repeat myths.

Pediatric Fatalities Associated With Over-the-Counter Cough and Cold Medications

Lun, 01/11/2021 - 09:01
BACKGROUND AND OBJECTIVES

In 2008, over-the-counter cough and cold medications (CCMs) underwent labeling changes in response to safety concerns, including fatalities, reported in children exposed to CCMs. The objective of this study is to describe fatalities associated with exposures to CCMs in children <12 years old that were detected by a safety surveillance system from 2008 to 2016.

METHODS

Fatalities in children <12 years old that occurred between 2008 and 2016 associated with oral exposure to one or more CCMs were identified by the Pediatric Cough and Cold Safety Surveillance System. An expert panel reviewed all cases to determine the causal relationship between the exposure and death, if the intent of exposure was therapeutic, and if the dose was supratherapeutic. Other contributing factors related to the child’s death were also identified as part of a root cause analysis.

RESULTS

Of the 180 eligible fatalities captured during the study period, 40 were judged by the expert panel to be either related or potentially related to the CCM. Of these, the majority (n = 24; 60.0%) occurred in children <2 years old and involved nontherapeutic intent (n = 22; 55.0%). The most frequently involved index ingredient was diphenhydramine (n = 28; 70.0%). In 6 cases (n = 6; 15.0%), the CCM was administered to murder the child. In another 7 cases (n = 7; 17.5%), death followed the intentional use of the CCM to sedate the child.

CONCLUSIONS

Pediatric fatalities associated with CCMs occurred primarily in young children after deliberate medication administration with nontherapeutic intent by a caregiver.

Novel Device for Endoluminal Esophageal Atresia Repair: First-in-Human Experience

Lun, 01/11/2021 - 09:01

Thoracoscopic esophageal atresia (EA) repair affords many benefits to the patient; however, intracorporeal suturing of the anastomosis is technically challenging. Esophageal magnetic compression anastomosis (EMCA) is a compelling option for endoluminal EA repair, but available EMCA devices have prohibitive rates of recalcitrant stricture. Connect-EA is a new endoluminal EMCA device system that employs 2 magnetic anchors with a unique mating geometry designed to reliably create a robust anastomosis and decrease rates of leak and stricture. We describe our first-in-human experience with this novel endoluminal device for staged EA repair in 3 patients (Gross type A, B, and C) at high risk for conventional surgical repair. First, the esophageal pouches were approximated thoracoscopically. After acute tension subsided, the device anchors were endoscopically placed in the esophageal pouches and mated. Anchors were spontaneously excreted in 2 cases. Endoscopic repositioning and retrieval of the anchors were required in 1 patient because of narrowed esophageal anatomy. There were no perioperative complications. Patients were managed for 14 to 18 months. The strictures that developed in the patients were membranous and responded well to dilation alone, resolving after 4 to 5 outpatient dilations. Gastrostomies were closed between 6 and 11 months and all patients are tolerating full oral nutrition. Early experience with this new endoluminal EMCA device system is highly favorable. The device offers considerable benefit over conventional handsewn esophageal anastomosis and anastomotic outcomes are superior to available EMCA devices.

Changing Pediatric Hospice and Palliative Care Through Medicaid Partnerships

Lun, 01/11/2021 - 09:01

Since its inception in 2010, the Concurrent Care for Children Provision of the Affordable Care Act has enabled seriously ill pediatric patients and their families to access comprehensive, supportive hospice services while simultaneously receiving ongoing treatment-directed therapies. Although this groundbreaking federal legislation has resulted in improvements in care for vulnerable pediatric patients, the implementation of the law has varied from state to state through Medicaid programming. The pediatric professional community is called to consider how Medicaid services can more effectively be delivered by leveraging legislative mandates and collaborative relationships between clinicians, Medicaid administrators, and policy makers. In this article, we examine ways concurrent care has been executed in 3 different states and how key stakeholders in care for children with serious illness advocated to ensure effective implementation of the legislation. The lessons learned in working with state Medicaid programs are applicable to any advocacy issue impacting children and families .

Scalp Nodules and Facial Droop in an Infant

Lun, 01/11/2021 - 09:01

A previously healthy, term, 5-week-old girl initially presented to her primary care physician with a solitary, enlarging scalp nodule. The infant was otherwise well without additional signs or symptoms of illness. Over the next several weeks, the nodule continued to grow, and additional lesions appeared on her scalp. An ultrasound of the primary nodule revealed a hypoechoic structure favored to represent a serosanguinous fluid collection. After evaluation by general surgery and dermatology, she underwent a scalp biopsy of the largest lesion. While biopsy specimen results were pending, her parents noted that she was developing increased irritability, difficulty closing her right eye, and facial weakness. She was referred to the emergency department where a right-sided facial droop involving the brow and forehead was noted. The skin biopsy specimen results, along with subsequent laboratory studies and imaging, led to the final diagnosis.

Cross-Sector Approach Expands Screening and Addresses Health-Related Social Needs in Primary Care

Lun, 01/11/2021 - 09:01
OBJECTIVES

During infancy, the American Academy of Pediatrics Bright Futures fourth edition health supervision guidelines recommend frequent well-child visits (WCVs) in which providers are expected to screen for and address maternal depression, intimate partner violence (IPV), and health-related social needs (HRSN). We spread an evidence-based approach that implements these recommendations (Developmental Understanding and Legal Collaboration for Everyone; DULCE) with 3 aims for 6-month-old infants and their families: 75% receive all WCVs on time, 95% are screened for 7 HRSNs, and 90% of families with concrete supports needs and 75% of families with maternal depression or IPV receive support.

METHODS

Between January 2017 and July 2018, five DULCE teams (including a community health worker, early childhood system representative, legal partner, clinic administrator, pediatric and behavioral health clinicians) from 3 communities in 2 states participated in a learning collaborative. Teams adapted DULCE using Plan-Do-Study-Act cycles, reported data, and shared learning monthly. Run charts were used to study measures. The main outcome was the percent of infants that received all WCVs on time.

RESULTS

The percentage of families who completed all WCVs on time increased from 46% to 65%. More than 95% of families were screened for HRSNs, 70% had ≥1 positive screen, and 86% and 71% of those received resource information for concrete supports and maternal depression and IPV, respectively.

CONCLUSIONS

Quality improvement–supported DULCE expansion increased by 50% the proportion of infants receiving all WCVs on time and reliably identified and addressed families’ HRSNs, via integration of existing resources.

HLH-Like Syndrome and Rhabdomyolysis in an Adolescent Patient

Lun, 01/11/2021 - 09:01

Hemophagocytic lymphohistiocytosis (HLH) is a rare heterogeneous group of disorders characterized by immune overactivation. It can occur because of primary genetic mutations or secondary to almost any inflammatory or infectious process. The clinical manifestations of this syndrome are varied and life-threatening and resemble those of many malignancies, infections, sepsis, and multisystem inflammatory syndrome in children. Laboratory abnormalities often are not diagnostic for HLH until late in the disease course, and the laboratory studies are send-out tests at most institutions. Thus, quickly and accurately diagnosing pediatric patients with HLH presents significant challenges to the clinician. Furthermore, there has been recent discussion in the literature regarding the use of diagnostic criteria for HLH. In this case report, we detail an adolescent male individual who developed persistent unexplained fever, rhabdomyolysis, and regional ischemic immune myopathy. To our knowledge, there is no previous report of a pediatric patient with this rare myopathy or HLH presenting with persistent rhabdomyolysis in the literature. The patient was hospitalized for a total of 61 days, with multiple treatments attempted throughout during his course of illness. In this report, we highlight the importance of using diagnostic flexibility when HLH is suspected in pediatric patients and provide insight into the unique challenges of identifying this condition.

Achieving Entrustable Professional Activities During Fellowship

Lun, 01/11/2021 - 09:01
BACKGROUND AND OBJECTIVES

Entrustable Professional Activities (EPAs) were developed to assess pediatric fellows. We previously showed that fellowship program directors (FPDs) may graduate fellows who still require supervision. How this compares with their expectations for entrustment of practicing subspecialists is unknown.

METHODS

We surveyed US FPDs in 14 pediatric subspecialties through the Subspecialty Pediatrics Investigator Network between April and August 2017. For each of 7 common pediatric subspecialty EPAs, we compared the minimum level of supervision that FPDs required for graduation with the level they expected of subspecialists for safe and effective practice using the Friedman rank sum test and paired t test. We compared differences between subspecialties using linear regression.

RESULTS

We collected data from 660 FPDs (response rate 82%). For all EPAs, FPDs did not require fellows to reach the level of entrustment for graduation that they expected of subspecialists to practice (P < .001). FPDs expected the least amount of supervision for the EPAs consultation and handovers. Mean differences between supervision levels for graduation and practice were smaller for clinical EPAs (consultation, handovers, lead a team) when compared with nonclinical EPAs (quality improvement, management, lead the profession and scholarship; P = .001) and were similar across nearly all subspecialties.

CONCLUSIONS

Fellowship graduates may need continued development of clinical and nonclinical skills in their early practice period, underscoring a need for continued assessment and mentoring. Graduation readiness must be based on clear requirements, with alignment of FPD expectations and regulatory standards, to ensure quality care for patients.

Sexuality Among Adolescents With Intellectual Disability: Balancing Autonomy and Protection

Lun, 01/11/2021 - 09:01

Adolescents and young adults living with intellectual disability (ID) have made significant advancements integrating into multiple aspects of western society, but there has been less progress with regards to sexual health. While advocating for individuals with ID to live life to the fullest, pediatricians have practical concerns regarding the ability to consent to sex as well as avoid coercion and manipulation in sexual encounters. This has led to tension between supporting the autonomy of a patient with ID while protecting them from harm. We present a case of a young adult with moderate ID who is engaging in a sexual relationship with her boyfriend without parental knowledge. The pediatrician must decide the most appropriate course of action to support the patient’s autonomy but also ensure that the patient is a willing participant and understands the risks of engaging in sexual activity. This case highlights 4 main themes: (1) practical concerns when approaching sexual health in the adolescent with ID, (2) advocating for the rights of those with ID to live life to the fullest, (3) the critical inclusion of individuals with ID in decisions directly affecting them and their peer group, and (4) decision-making capacity and respect for autonomy in individuals with ID. This case highlights the delicate balance providers face when providing care to adolescents and young adults with ID: supporting autonomy to make decisions while reducing harm to a vulnerable population.

Multisystem Autoimmune Inflammatory Disease, Including Colitis, Due to Inborn Error of Immunity

Lun, 01/11/2021 - 09:01

Our understanding of inflammatory bowel disease is changing as we identify genetic variants associated with immune dysregulation. Inflammatory bowel disease undetermined, even when diagnosed in older children and adolescents, in the setting of multiple inflammatory and infectious diseases should raise the suspicion of complex immune dysregulation with a monogenic basis. We report a case of inflammatory bowel disease undetermined triggered by exposure to a nonsteroidal antiinflammatory drug in a 16-year-old girl with a background history of juvenile idiopathic arthritis, cytopenias, recurrent respiratory tract and middle ear infections, and esophageal candidiasis. Immunologic assessment included measurement of immunoglobulin levels, lymphocyte immunophenotyping, B-cell functional tests, and whole-exome sequencing. Laboratory investigation revealed defects of humoral immunity, including mild persistent hypogammaglobulinemia affecting all 3 isotypes and absent isohemagglutinins. Whole exome sequencing revealed a heterozygous TNFRSF13B (Tumor Necrosis Factor Receptor Superfamily Member 13B, or Transmembrane Activator and Calcium-modulating cyclophilin ligand Interactor, TACI) gene variant, which is associated with common variable immunodeficiency and the development of autoimmune diseases. In conclusion, a clinical history of recurrent infections, atypical histologic features of inflammatory bowel disease, additional autoimmune manifestations, and an inadequate response to conventional therapy should prompt the physician to refer to an immunologist with the query of inborn error of immunity. We report how extensive immune evaluation and genetic diagnosis can individualize care and facilitate a multidisciplinary team approach.

Corticosteroids for Acute Orbital Cellulitis

Lun, 01/11/2021 - 09:01
OBJECTIVES

Standard treatment of children hospitalized for acute orbital cellulitis includes systemic antibiotics. Recent data from single-center studies suggest the addition of systemic corticosteroids may hasten clinical improvement and reduce hospital length of stay (LOS). We investigate the potential relationship between corticosteroid exposure and duration of hospitalization for pediatric orbital cellulitis.

METHODS

Using Pediatric Health Information System registry data from 51 children’s facilities, we performed a retrospective cohort study of children hospitalized for orbital cellulitis <18 years of age from 2007 to 2018. The primary study outcome was hospital LOS. Secondary outcomes included frequency of surgical interventions, PICU admission, and 30-day related-cause readmission.

RESULTS

Of the 5645 children included for study, 1347 (24%) were prescribed corticosteroids within 2 days of admission. Corticosteroid prescription was not associated with LOS in analyses adjusted for age; presence of meningitis, abscess, or vision issues; and operative episode and PICU admission within 2 days (eβ = 1.01, 95% confidence interval [CI]: 0.97–1.06). Corticosteroid exposure was associated with operative episodes after 2 days of hospitalization (odds ratio = 2.05, 95% CI: 1.29–3.27) and 30-day readmission (odds ratio = 2.40, 95% CI: 1.52–3.78) among patients with a primary diagnosis of orbital cellulitis.

CONCLUSIONS

In this database query, we were not able to detect a reduction in LOS associated with corticosteroid exposure during hospitalization for orbital cellulitis. Corticosteroid prescription was associated with PICU admission and operative episodes after 2 days of hospitalization. Before the adoption of routine corticosteroid use, prospective, randomized control trials are needed.

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