Six Month Follow-up of Patients With Multi-System Inflammatory Syndrome in Children.

BACKGROUND AND OBJECTIVES: Myocardial dysfunction and coronary abnormalities are prominent features of multisystem inflammatory syndrome in children (MIS-C). In this study we aim to evaluate the early and midterm outcomes of MIS-C. METHODS: This is a longitudinal 6-month cohort study of all children admitted and treated for MIS-C from April 17 to June 20, 2020. Patients were followed ∼2 weeks, 8 weeks, and 6 months postadmission, with those with coronary aneurysms evaluated more frequently. RESULTS: Acutely, 31 (62%) patients required intensive care with vasoactive support, 26 (52%) had left ventricular (LV) systolic dysfunction, 16 (32%) had LV diastolic dysfunction, 8 (16%) had coronary aneurysms (z score ≥2.5), and 4 (8%) had coronary dilation (z score <2.5). A total of 48 patients (96%) received immunomodulatory treatment. At 2 weeks, there was persistent mild LV systolic dysfunction in 1 patient, coronary aneurysms in 2, and dilated coronary artery in 1. By 8 weeks through 6 months, all patients returned to functional baseline with normal LV systolic function and resolution of coronary abnormalities. Cardiac MRI performed during recovery in select patients revealed no myocardial edema or fibrosis. Some patients demonstrated persistent diastolic dysfunction at 2 weeks (5, 11%), 8 weeks (4, 9%), and 6 months (1, 4%). CONCLUSIONS: Children with MIS-C treated with immunomodulators have favorable early outcomes with no mortality, normalization of LV systolic function, recovery of coronary abnormalities, and no inflammation or scarring on cardiac MRI. Persistence of diastolic dysfunction is of uncertain significance and indicates need for larger studies to improve understanding of MIS-C. These findings may help guide clinical management, outpatient monitoring, and considerations for sports clearance.

Structured Neonatal Point-of-Care Ultrasound Training Program.

OBJECTIVE: Point-of-care ultrasound (POC US) has been increasingly used by intensive care physicians. Growing use of POC US necessitates defining distinct clinical indications for its application, as well as structured POC US training programs. Homogeneous approach to POC US education combined with rigorous quality assurance should further enable POC US to become standard-of-care clinical tool. This study aimed to present the first, innovative, and structured POC US program in neonatal-perinatal medicine field. In addition, we reviewed the availability of the POC US training programs across different medical specialties. STUDY DESIGN: Available English-language publications on POC US training programs in general and neonatal-perinatal medicine were reviewed in this study. DISCUSSION: Mounting body of evidence suggests improved procedural completion rates, as well as clinical decision making with the use of POC US. However, limited research supported the existence of structured, comprehensive POC US programs. It was recognized that medical institutions need to develop syllabuses, teach, and credential increasing number of health care professionals in the use of POC US. We defined intuitive educational strategy that encompasses POC US clinical indications, educational curriculum, scanning protocols, competence evaluation, and finally credentialing process. In addition, we offered description of the imaging quality assurance, as well as POC US coding, and reimbursement. CONCLUSION: Future efforts need to be dedicated to the ongoing development of neonatal POC US as a clinical instrument. It should allow for eventual paradigm change and improved effectiveness in management of critically ill neonates.

Non-invasive assessment of endothelial function in children with obesity and lipid disorders.

BACKGROUND: Digital tonometry is designed to non-invasively screen for endothelial dysfunction by the detection of impaired flow-induced reactive hyperaemia in the fingertip. We determined whether digital reactive hyperaemia correlated with risk factors for atherosclerosis in two groups of children at increased risk for endothelial dysfunction. METHODS: A total of 15 obese children and 23 non-obese, dyslipidaemic children, 8-21 years of age, were enrolled, and their medical histories, anthropometric measurements, carotid wall thickness by means of ultrasonography, and fasting blood samples for cardiovascular risk factors were obtained. The standard endoPAT index of digital reactive hyperaemia was modified to reflect the true peak response or the integrated response of the entire post-occlusion period. In each group, age, sex, pubertal status, carotid wall thickness, and multiple cardiovascular risk factors were tested as predictors of endothelial dysfunction. RESULTS: In the non-obese, dyslipidaemic group, but not in the obese group, both indices strongly correlated with height (r=0.55, p=0.007, by peak response) followed by weight, waist circumference, and age. In both groups, neither index of reactive hyperaemia significantly correlated with any other cardiovascular risk factor. CONCLUSIONS: Contrary to the known age-related increase in atherosclerosis, digital reactive hyperaemia increased with age and its correlates in non-obese, dyslipidaemic children and was not related to other cardiovascular risk factors in either group. The reason for the lack of this relationship with age in obese children is unknown. The age-dependent physiology of digital microvascular reactivity and the endothelium-independent factors controlling the peak hyperaemic response need further study in children with a wide age range.

Libman-Sacks endocarditis as the first manifestation of systemic lupus erythematosus in an adolescent, with a review of the literature.

Libman-Sacks endocarditis is rare in children and adolescents, more so as a first manifestation of systemic lupus erythematosus. Currently, sterile verrucous lesions of Libman-Sacks endocarditis are recognised as a cardiac manifestation of both systemic lupus erythematosus and antiphospholipid syndrome. They are clinically silent in a majority of the cases. The presence of antiphospholipid antibodies in systemic lupus erythematosus is associated with three times higher prevalence of mitral valve nodules and significant mitral regurgitation. We present the case of isolated mitral regurgitation with abnormal looking mitral valve, detected in early childhood, which deteriorated to a severe degree in the next decade and was diagnosed as Libman-Sacks endocarditis after surgical repair from histopathology. The full-blown clinical spectrum of systemic lupus erythematosus with antiphospholipid antibodies was observed several weeks after cardiac surgery. We discuss the atypical course of Libman-Sacks endocarditis with follow-up for 10 years, along with a review of the literature.

Psychiatric disorders in youth with medically unexplained chest pain versus innocent heart murmur.

OBJECTIVE: To examine the prevalence of Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition psychiatric disorders in youth with chest pain compared with a control sample with innocent heart murmur. STUDY DESIGN: We assessed youth ages 8 to 17 years who were examined in cardiology settings for medically unexplained chest pain (n=100) or innocent heart murmur (n=80). We conducted semi-structured interviews and assessed medical history, quality of life, and disability. RESULTS: Youth with chest pain had a higher prevalence of psychiatric disorders compared with youth with murmur (74% versus 47%, χ(2)=13.3; P<.001). Anxiety disorders predominated, although major depression was also more common in the chest pain group (9% versus 0%; Fisher exact tests; P<.01). Onset of psychiatric disorders generally preceded chest pain. Patterns were similar for boys and girls and for children and adolescents. Chest pain was associated with poorer quality of life and with pain-related disability for youth with co-morbid psychiatric disorder. CONCLUSIONS: In childhood and adolescence, medically unexplained chest pain is associated with a high prevalence of psychiatric disorders. Systematic mental health screening may improve detection and enhance treatment of these patients.

Atherosclerosis in survivors of Kawasaki disease.

OBJECTIVES: To test the hypothesis that long-term survivors of low-risk Kawasaki disease (KD) have ongoing vascular inflammation and dysfunction and a higher risk of accelerated atherosclerosis than healthy control subjects. STUDY DESIGN: Twenty-eight patients with KD (7-20 years after acute illness) and 27 age-matched healthy control subjects were examined for medical and dietary history, serum markers of atherosclerotic risk and inflammation, carotid intimal-medial thickness (CIMT) with vascular ultrasound scanning and arterial stiffness with applanation tonometry. RESULTS: Patients and control subjects were similar in age, sex, body mass index, waist-to-hip ratio, blood pressure, cigarette smoking, family history, diet, high-density lipoprotein cholesterol level, lipoprotein (a) level, homocysteine level, glucose level, insulin level, CIMT, arterial stiffness, C-reactive protein level, and inflammatory cytokine level. Levels of total cholesterol and apolipoprotein B were significantly higher in patients with KD than in control subjects. CONCLUSIONS: There was no evidence of increased atherosclerosis. Small but significant differences in cholesterol and apolipoprotein B levels could suggest increased future risk for atherosclerosis and warrant further study.

Decreased levels of cystatin C, an inhibitor of the elastolytic enzyme cysteine protease, in acute and subacute phases of kawasaki disease.

Elevation of tissue-destructive proteases has been reported in acute Kawasaki disease. Cystatin C is a naturally occurring inhibitor of elastolytic cysteine protease in humans. Serum cystatin C deficiency in human beings has been linked to atherosclerosis and aortic aneurysms. We investigated the serum levels of cystatin C during acute Kawasaki disease. Serum samples from 17 acute Kawasaki disease patients were collected before and after immunoglobulin therapy and also at a median of 17 days after the therapy. Eight adults and 10 children without intercurrent infections served as control patients. Children with Kawasaki disease prior to therapy had significantly lower levels of cystatin C compared to adults (p = 0.002) and control children (p = 0.001). The low levels persisted 1-106 days after the therapy. Compared to control children and adults, children with Kawasaki disease had significantly lower serum levels of cystatin C in the acute stage before immunoglobulin therapy and in the subacute phase after the immunoglobulin therapy.