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We describe 2 children with persistent fever and profuse diarrhea who developed signs of mucocutaneous involvement (conjunctivitis, fissured lips, skin rash, erythema, and edema of the hands and feet). Blood tests revealed elevated markers of inflammation, lymphopenia, thrombocytopenia, and complement consumption. Afterward, diffuse edema with hypoalbuminemia appeared in the context of a capillary leak syndrome. In both patients, repeated nasal swabs were negative for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), but each patient had high titers of immunoglobulin G and immunoglobulin M against the SARS-CoV-2 virus. The negative PCR results in the presence of immunoglobulin M and immunoglobulin G suggested that the inflammatory response developed in the late phase of viral infection, when SARS-CoV-2 was not detectable in the upper airway. In this report, we describe patients with what we propose to name as SARS-CoV-2–induced Kawasaki-like hyperinflammatory syndrome. SARS-CoV-2–induced Kawasaki-like hyperinflammatory syndrome seems to be caused by a delayed response to SARS-CoV-2. It resembles Kawasaki disease complicated by macrophage activation syndrome, although it has peculiar features, such as prodromal diarrhea, capillary leak syndrome, and myocardial dysfunction. Intravenous corticosteroid treatment appears to be helpful.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome classified into primary HLH and secondary HLH. Secondary HLH is always caused by autoimmune disease, infections, or cancer. The first-line therapy for secondary HLH is the HLH 2004 protocol, including dexamethasone, etoposide, and supportive therapy. However, up to 30% of patients, especially pediatric patients, remain unresponsive to first-line treatment, and the mortality rate reaches 50% in children with HLH. Furthermore, some children who have special conditions, such as an active virus infection, are not suitable for immunosuppressants treatment. Recently, several HLH-promoting cytokines have been identified, including interferon-, interleukin-2, and interleukin-6. Janus kinase 1 and 2 control the signaling of many cytokines, notably interferon-, interleukin-2, and interleukin-6. Janus kinase 1 and 2 inhibitors, such as ruxolitinib, have been successfully used to treat HLH in mice. Here, we report that a boy, diagnosed with HLH and high titer of hepatitis B virus–DNA copies, improved quickly, and the cytokine storm of HLH was alleviated after receiving ruxolitinib. Five days after ruxolitinib treatment, entecavir was introduced and serum titer results of hepatitis B virus–DNA returned negative. With 3 months of ruxolitinib treatment and following-up 1 year, the boy’s situation maintained sustained remission. In this study, it is suggested that ruxolitinib might be a first-line drug, which could alleviate the cytokine storm of HLH. This treatment may be ushering in the age of glucocorticosteroid-free HLH treatment, which is particularly meaningful for children because it avoids the side effects of glucocorticosteroid.
Hereditary hemorrhagic telangiectasia (HHT) can be clinically diagnosed, but children often lack characteristic features. We report a family with homozygous growth differentiation factor 2 (GDF2)–related HHT diagnosed by genetic testing. A boy aged 5 years and 2 months presented with isolated hypoxemia. He was the product of a consanguineous marriage; his parents were second cousins. Physical examination revealed cyanosis of nail beds and clubbed fingers. Pulse oxygen saturation was 84% to 89%. Lung function, contrast-enhanced lung computed tomography, and noncontrast echocardiography were normal. A pulmonary perfusion scan revealed radioactivity in the brain and bilateral kidney, suggesting the existence of a intrapulmonary shunt. Whole-exome sequencing revealed a homozygous variant [c.1060_1062delinsAG (p.Tyr354ArgfsTer15)] in GDF2, which was found to be inherited from his heterozygous parents. At the age of 8 years, he developed epistaxis, and an angiogram revealed diffuse pulmonary arteriovenous malformations. At the age of 9 years, he was treated with sirolimus, and his condition improved significantly. However, his now 7-year-old sister with the same homozygous variant currently has no symptoms. Physical examinations revealed 1 pinpoint-sized telangiectasia on the chest of his mother and a vascular lesion on the forehead of his sister. Additionally, the patient’s father and great-uncle had a history of mild to moderate epistaxis. Mutation in GDF2 is a rare cause of HHT. Ours is the first report of homozygous GDF2-related HHT; in addition, this variant has not been reported previously. In our report, we also confirm variable expressivity, even with the same pathogenic variant in GDF2-related HHT.
We evaluated 4 diagnostic strategies to predict the presence of inflammatory bowel disease (IBD) in children who present with chronic nonbloody diarrhea and abdominal pain.METHODS:
We conducted a prospective cohort study including 193 patients aged 6 to 18 years who underwent a standardized diagnostic workup in secondary or tertiary care hospitals. Each patient was assessed for symptoms, C-reactive protein (>10 mg/L), hemoglobin (<–2 SD for age and sex), and fecal calprotectin (≥250 μg/g). Patients with rectal bleeding or perianal disease were excluded because the presence of these findings prompted endoscopy regardless of their biomarkers. Primary outcome was IBD confirmed by endoscopy or IBD ruled out by endoscopy or uneventful clinical follow-up for 6 months.RESULTS:
Twenty-two of 193 (11%) children had IBD. The basic prediction model was based on symptoms only. Adding blood or stool markers increased the AUC from 0.718 (95% confidence interval [CI]: 0.604–0.832) to 0.930 (95% CI: 0.884–0.977) and 0.967 (95% CI: 0.945–0.990). Combining symptoms with blood and stool markers outperformed all other strategies (AUC 0.997 [95% CI: 0.993–1.000]). Triaging with a strategy that involves symptoms, blood markers, and calprotectin will result in 14 of 100 patients being exposed to endoscopy. Three of them will not have IBD, and no IBD-affected child will be missed.CONCLUSIONS:
Evaluating symptoms plus blood and stool markers in patients with nonbloody diarrhea is the optimal test strategy that allows pediatricians to reserve a diagnostic endoscopy for children at high risk for IBD.
To describe the proportion of children screened by the Modified Checklist for Autism in Toddlers (M-CHAT), identify characteristics associated with screen completion, and examine associations between autism spectrum disorder (ASD) screening and later ASD diagnosis.METHODS:
We examined data from children attending 18- and 24-month visits between 2013 and 2016 from 20 clinics within a health care system for evidence of screening with the M-CHAT and subsequent coding of ASD diagnosis at age >4.75 years. We interviewed providers for information about usual methods of M-CHAT scoring and ASD referral.RESULTS:
Of 36 233 toddlers, 73% were screened and 1.4% were later diagnosed with ASD. Hispanic children were less likely to be screened (adjusted prevalence ratio [APR]: 0.95, 95% confidence interval [CI]: 0.92–0.98), and family physicians were less likely to screen (APR: 0.12, 95% CI: 0.09–0.15). Compared with unscreened children, screen-positive children were more likely to be diagnosed with ASD (APR: 10.3, 95% CI: 7.6–14.1) and were diagnosed younger (38.5 vs 48.5 months, P < .001). The M-CHAT’s sensitivity for ASD diagnosis was 33.1%, and the positive predictive value was 17.8%. Providers routinely omitted the M-CHAT follow-up interview and had uneven referral patterns.CONCLUSIONS:
A majority of children were screened for ASD, but disparities exist among those screened. Benefits for screen-positive children are improved detection and younger age of diagnosis. Performance of the M-CHAT can be improved in real-world health care settings by administering screens with fidelity and facilitating timely ASD evaluations for screen-positive children. Providers should continue to monitor for signs of ASD in screen-negative children.
Home nursing is essential for children with medical complexity (CMC), but provision varies substantially across states. Our objectives were to quantify state-to-state variability in distribution of posthospitalization home nursing to commercially insured CMC and to rank-order states.METHODS:
Retrospective cohort study of hospitalized commercially insured children with ≥1 complex chronic condition from birth to 18 years of age in the Truven MarketScan database. Cohort eligibility criteria were hospital discharge between January 2013 and November 2016 and at least 30 days of follow-up after discharge. Two primary outcome measures were used: receipt of any home nursing within 30 days of hospital discharge (yes or no) and number of days of posthospitalization home nursing (1–30 days). A composite metric encompassing both receipt and quantity was created by evaluating the 95th percentile of days of home nursing (0–30 days).RESULTS:
Overall, 9.9% of the sample received home nursing. After we adjusted for patient characteristics, the probability of receiving home nursing varied across states, ranging from 3.4% to 19.2%. Among home nursing recipients, the adjusted median home nursing days across states ranged from 6.6 to 24.5 days. The adjusted 95th percentile of days of home nursing (across the entire of sample, including recipients and nonrecipients of home nursing) ranged from 6.8 to 22.6 days.CONCLUSIONS:
We observed striking state-to-state variability in receipt of home nursing and mean number of days of posthospitalization home nursing among commercially insured CMC after adjustment for demographic and clinical differences. This suggests opportunities for state-level improvement.
Cannabinoids, the psychoactive compounds in marijuana, are one of the most commonly used substances in the United States. In this review, we summarize the impact of marijuana on child and adolescent health and discuss the implications of marijuana use for pediatric practice. We review the changing epidemiology of cannabis use and provide an update on medical use, routes of administration, synthetic marijuana and other novel products, the effect of cannabis on the developing brain, other health and social consequences of use, and issues related to marijuana legalization.
To explore the hypothesis that obesity is associated with increased mortality and worse outcomes in children who are critically ill.METHODS:
Secondary analysis of the Assessment of Worldwide Acute Kidney Injury, Renal Angina, and Epidemiology study, a prospective, multinational observational study. Patients between 3 months and 25 years across Asia, Australia, Europe, and North America were recruited for 3 consecutive months. Patients were divided into 4 groups (underweight, normal weight, overweight, and obese) on the basis of their BMI percentile for age and sex.RESULTS:
A total of 3719 patients were evaluated, of whom 542 (14%) had a primary diagnosis of sepsis. One thousand fifty-nine patients (29%) were underweight, 1649 (44%) were normal weight, 423 (11%) were overweight, and 588 (16%) were obese. The 28-day mortality rate was 3.6% for the overall cohort and 9.1% for the sepsis subcohort and differed significantly by weight status (5.8%, 3.1%, 2.2%, and 1.8% for subjects with underweight, normal weight, overweight, and obesity, respectively, in the overall cohort [P < .001] and 15.4%, 6.6%, 3.6%, and 4.7% in the sepsis subcohort, respectively [P = .003]). In a fully adjusted model, 28-day mortality risk was 1.8-fold higher in the underweight group versus the normal weight group in the overall cohort and 2.9-fold higher in the sepsis subcohort. Patients who were overweight and obese did not demonstrate increased risk in their respective cohorts. Patients who were underweight had a longer ICU length of stay, increased need for mechanical ventilation support, and a higher frequency of fluid overload.CONCLUSIONS:
Patients who are underweight make up a significant proportion of all patients in the PICU, have a higher short-term mortality rate, and have a more complicated ICU course.
Vancomycin remains one of the most commonly prescribed antibiotics in NICUs despite recommendations to limit its use for known resistant infections. Baseline data revealing substantially higher vancomycin use in our NICU compared to peer institutions informed our quality improvement initiative. Our aim was to reduce the vancomycin prescribing rate in neonates hospitalized in our NICU by 50% within 1 year and sustain for 1 year.METHODS:
In the 60-bed level IV NICU of an academic referral center, we used a quality improvement framework to develop key drivers and interventions including (1) physician education with benchmarking antibiotic prescribing rates; (2) pharmacy-initiated 48-hour antibiotic time-outs on rounds; (3) development of clinical pathways to standardize empirical antibiotic choices for early-onset sepsis, late-onset sepsis, and necrotizing enterocolitis; coupled with (4) daily prospective audit with feedback from the antimicrobial stewardship program.RESULTS:
We used statistical process u-charts to show vancomycin use declined from 112 to 38 days of therapy per 1000 patient-days. After education, pharmacy-initiated 48-hour time-outs, and development of clinical pathways, vancomycin use declined by 29%, and by an additional 52% after implementation of prospective audit with feedback. Vancomycin-associated acute kidney injury also declined from 1.4 to 0.1 events per 1000 patient-days.CONCLUSIONS:
Through a sequential implementation approach of education, standardization of care with clinical pathways, pharmacist-initiated 48-hour time-outs, and prospective audit with feedback, vancomycin days of therapy declined by 66% over a 1-year period and has been sustained for 1 year.
The Centers for Disease Control and Prevention recommend testing for Chlamydia trachomatis in sexually active female patients <25 years old using nucleic-acid amplification tests (NAAT) from a vaginal swab. Our providers were typically testing using the less sensitive urine NAATs. We aimed to increase the percentage of urogenital C trachomatis NAATs performed by using vaginal swabs in adolescent female patients ages 10 through 20 years from 1.4% to 25%.METHODS:
We implemented 3 interventions at 3 pediatric practices over 12 months including education, process standardization, and cross-training. We used statistical process control to analyze the effect of interventions on our primary outcome: the percentage of urogenital C trachomatis tests performed with a vaginal swab. Our balance measure was the total number of urogenital C trachomatis tests.RESULTS:
There were 818 urogenital C trachomatis tests performed: 289 before and 529 after the first intervention. Of urogenital C trachomatis tests in the preintervention time period, 1.4% were performed by using vaginal swabs. We surpassed our aim of 25% 6 weeks after the first intervention. We noted sustained improvement after the second intervention, with an average of 68.3% of tests performed by using vaginal swabs for the remaining postintervention period. There was no difference in the overall number of urogenital C trachomatis tests pre- and postintervention.CONCLUSIONS:
Using quality improvement methodology and implementing easily replicable interventions, we significantly and sustainably increased use of vaginal swabs. The interventions standardizing processes were associated with a higher impact than the educational intervention.
Developing Content for Pediatric Hospital Medicine Certification Examination Using Practice Analysis
The American Board of Pediatrics (ABP) and the Pediatric Hospital Medicine (PHM) subboard developed a content outline to serve as a blueprint for the inaugural certification examination through practice analysis. The systematic approach of practice analyses process is described in the study.METHODS:
A diverse, representative panel of 12 pediatric hospitalists developed the draft content outline using multiple resources (publications, textbooks, PHM Core Competencies, PHM fellow’s curriculum, etc). The panel categorized practice knowledge into 13 domains and 202 subdomains. By using the ABP database self-defined practicing pediatric hospitalists were identified. Participants rated the frequency and criticality of content domains and subdomains along with providing open-ended comments.RESULTS:
In total, 1449 (12.1%) generalists in the ABP database self-identified as pediatric hospitalists, and 800 full-time pediatric hospitalists responded. The content domains that were rated as highly critical and frequently required in practice were weighted more heavily (ie, the percentage of examination questions associated with a domain) than the less critical and less frequently rated. Both community and noncommunity pediatric hospitalists rated domains similarly (P = .943). Subdomain and preliminary weights were rated with similar means and SDs in the majority of topics.CONCLUSIONS:
There was concordance in the rating of domain and universal tasks among both community and noncommunity hospitalists. The areas of significant differences, although minor, could be explained by difference in practice settings. The practice analysis approach was structured, engaged the PHM community, reflected the breadth and depth of knowledge required for PHM practice, and used an iterative process to refine the final product.
Establishing the diagnosis of hereditary fructose intolerance (HFI) remains difficult despite the availability of specific molecular genetic testing of the ALDOB gene. This is attributable, at least in part, to the lack of a specific and practical biomarker. We report the incidental diagnosis of HFI as a consequence of nontargeted genetic testing ordered for alternative indications in 5 patients, including 3 children and 2 adults. Two of the children were diagnosed with HFI after extensive evaluations that ultimately involved clinical or research exome sequencing. The third child was diagnosed with HFI during subsequent genetic testing of at-risk family members. Both adults learned to avoid fructose and remained asymptomatic of HFI before diagnosis. One was diagnosed with HFI during preconception, nontargeted expanded carrier screening. For the other, concern for HFI was initially raised by indeterminate direct-to-consumer genetic testing results. None of these patients presented with infantile acute liver failure or other acute decompensation. Our findings suggest that the emphasis of classic teaching on infantile liver failure after first exposure to fructose may be inadvertently increasing the likelihood of missing cases of HFI characterized by other manifestations. HFI is likely underdiagnosed and should be considered for patients with nonspecific findings as well as for individuals with significant aversion to sweets.
Cardiorespiratory and pulse oximetry monitoring in children who are hospitalized should balance benefits of detecting deterioration with potential harms of alarm fatigue. We developed recommendations for monitoring outside the ICU on the basis of available evidence and expert opinion.METHODS:
We conducted a comprehensive literature search for studies addressing the utility of cardiorespiratory and pulse oximetry monitoring in common pediatric conditions and drafted candidate monitoring recommendations based on our findings. We convened a panel of nominees from national professional organizations with diverse expertise: nursing, medicine, respiratory therapy, biomedical engineering, and family advocacy. Using the RAND/University of California, Los Angeles Appropriateness Method, panelists rated recommendations for appropriateness and necessity in 3 sequential rating sessions and a moderated meeting.RESULTS:
The panel evaluated 56 recommendations for intermittent and continuous monitoring for children hospitalized outside the ICU with 7 common conditions (eg, asthma, croup) and/or receiving common therapies (eg, supplemental oxygen, intravenous opioids). The panel reached agreement on the appropriateness of monitoring recommendations for 55 of 56 indications and on necessity of monitoring for 52. For mild or moderate asthma, croup, pneumonia, and bronchiolitis, the panel recommended intermittent vital sign or oximetry measurement only. The panel recommended continuous monitoring for severe disease in each respiratory condition as well as for a new or increased dose of intravenous opiate or benzodiazepine.CONCLUSIONS:
Expert panel members agreed that intermittent vital sign assessment, rather than continuous monitoring, is appropriate management for a set of specific conditions of mild or moderate severity that require hospitalization.