Left atrial appendage aneurysm in newborns: a report of two cases.

Left atrial appendage aneurysm is an extremely rare cardiac anomaly with serious complications. It is rarely diagnosed in childhood. Herein, we reported two newborns diagnosed with left atrial appendage aneurysm.

Cardiac involvement in multisystem inflammatory syndrome in children: single-centre experience.

BACKGROUND: This study aimed to evaluate electrocardiographic and echocardiographic findings, Holter recordings of the multisystem inflammatory syndrome in children, and to identify prognostic factors for cardiac involvement. METHODS: We retrospectively reviewed demographic characteristics, medical data, laboratory findings, electrocardiogram and echocardiographic findings, 24-hour Holter recordings, need for an ICU, and extracorporeal membrane oxygenation in multisystem inflammatory syndrome in children. Acute left ventricular systolic dysfunction was defined as left ventricular ejection fraction (EF) ≤%55 on echocardiography. RESULTS: Sixty-seven children were included in the study. 24-hour Holters were recorded in 61.2% of the patients and 49.2% were normal. On echocardiographic examination, 14.9% of the patients had systolic dysfunction (EF ≤ 55%). While 32.8% of patients had mild mitral regurgitation, 3% had moderate mitral regurgitation, and 6% had mild aortic regurgitation. There was no statistically significant difference in EF values between the group with arrhythmia in Holter and the group with normal Holter results (p ≥ 0.05). B-type natriuretic peptide was positively correlated with C-reactive protein, ferritin, and fibrinogen. Significant effectivity of the B-type natriuretic peptide value was observed in the differentiation of those with EF ≤ and > 55%. Extracorporeal membrane oxygenation support was needed for three (4.5%) patients. One patient who died had systemic juvenile idiopathic arthritis. CONCLUSIONS: Neutrophil/lymphocyte ratio, C-reactive protein, D-dimer, ferritin, troponin, and B-type natriuretic peptide were found to be significantly higher in patients with systolic dysfunction. Also, the cut-off value of 1700 pg/ml for B-type natriuretic peptide was significantly effective. These parameters may indicate the severity of the disease but should be supported by prospective studies.

Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study.

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.

Health-related quality of life and parental depression in children with pulmonary arterial hypertension.

BACKGROUND: Impaired health-related quality of life (HRQoL) is a common problem in pulmonary arterial hypertension (PAH), but there is limited data on HRQoL in children with PAH. We aimed to investigate the QoL, determine the potential risk factors for poor HRQoL in children with PAH, and assess the depression and anxiety of their families. METHODS: We performed a prospective cross-sectional study of children with PAH, healthy peers, and their parents. HRQoL was measured by the self-reported and age-adapted KINDL questionnaire. Beck Depression Inventory (BDI) and hospital anxiety and depression scale (HADS) were used to assess the depression and anxiety of parents. RESULTS: Children with PAH had statistically lower total HRQoL scores than healthy peers (p < 0.001). There was no correlation between HRQoL and duration of disease, World Health Organization functional class, pro-B-type natriuretic peptide, 6-min walk test, and combined or single treatment. BDI and HADS scores were significantly higher in the parents of patients (p < 0.001, p = 0.023, p < 0.001, respectively). There was a negative correlation between HRQoL and BDI in patients (p = 0.016), while there was no significant correlation between HRQoL and HADS (p > 0.05). CONCLUSION: We demonstrated impairment of HRQoL of children with PAH. In addition, there was a correlation between the depression of the families and the QoL of the children.

Effect of vitamin D on myocardial remodeling and inflammatory status in children with congenital heart disease.

BACKGROUND: Vitamin D insufficiency is a common public health problem that is often unrecognized in children with congenital heart disease, and is not generally evaluated at congenital heart disease (CHD) follow-up. Recent studies have suggested that inadequate vitamin D status may have an adverse effect on cardiovascular health. This study investigates the relationship between vitamin D levels and hemodynamic parameters in children with CHD. METHODS: Included in the study 40 patients (25 females, 15 males) with CHD, who were evaluated for Ross heart failure score, vitamin D, parathyroid hormone (PTH), calcium, phosphorus, alkaline phosphatase (ALP), whole blood count (WBC) and echocardiographic measurements, and all measurements were repeated in the third month of the therapy. RESULTS: The mean vitamin D level was 16.4 ± 6.6 ug/L before and 27.5 ± 9.9 µg/L in the third month of therapy, while the mean PTH level was 53.3 ± 34.9 pg/mL before and 43.8 ± 21.4 pg/mL in the third month of therapy. The mean WBC was 8084 ± 2324/µL before and 7378±1893/µL in the third month of the therapy, and the mean platelet (PLT) count was 280,897 ± 80,119/µL before and 307,179 ± 60,202/µL in the third month of the therapy. The mean ejection fraction (EF) was 64% ± 7.2% before and 66.7% ± 6.2% in the third month of therapy, while the right ventricle (RV) myocardial performance index (MPI) was 32.1% ± 6.7% before and 28.9% ± 6.5% in the third month of the therapy. IL10 level was increased in four patients in the third month of therapy. A statistically significant decrease in PTH level and WBC, and an increase in PLT number and IL-10 level were detected by the therapy. Furthermore, echocardiographic findings revealed a statistically significant increase in EF and a decrease in RVMPI attributable to the therapy. DISCUSSION: The decreased levels of PTH, which is a proinflammatory marker, the increases in IL-10, which is an antiinflammatory cytokine, and the decreases in the number of WBC resulting from vitamin D treatment demonstrate the antiinflammatory effects of vitamin D. An improvement in EF means improvement in left ventricular contractility, while a decrease in RV MPI has been shown to improve the systolic and diastolic function of the right ventricle. These results suggest that vitamin D therapy has a positive effect on the heart, and so vitamin D levels should be evaluated during the routine follow-up of congenital heart disease.

The clinical course and short-term health outcomes of multisystem inflammatory syndrome in children in the single pediatric rheumatology center.

OBJECTIVES: Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition resulting in excessive response of the immune system after SARS-CoV-2 infection. We report a single-center cohort of children with MIS-C, describing the spectrum of presentation, therapies, clinical course, and short-term outcomes. METHODS: This is a prospective observational study from to a tertiary pediatric rheumatology center including patients (aged 1 month to 21 years) diagnosed with MIS-C between April 2020-April 2021. Demographic, clinical, laboratory results and follow-up data were collected through the electronic patient record system and analyzed. RESULTS: A total of 67 patients with MIS-C were included in the study. Fever was detected in all patients; gastrointestinal system symptoms were found in 67.2% of the patients, rash in 38.8%, conjunctivitis in 31.3%, hypotension in 26.9% myocarditis, and/or pericarditis in 22.4%, respectively. Respiratory symptoms were only in five patients (7.5%). Kawasaki Disease like presentation was found 37.3% of the patients. The mean duration of hospitalization was 11.8 7.07 days. Fifty-seven patients (85%) received intravenous immunoglobulin (IVIG), 45 (67%) received corticosteroids, 17 (25.3%) received anakinra, and one (1.5%) received tocilizumab. Seven of the patients (10.4%) underwent therapeutic plasma exchange (TPE). In 21 (31.3%) patients, a pediatric intensive care unit (PICU) was required in a median of 2 days. The first finding to improve was fever, while the first parameter to decrease was ferritin (median 6.5 days (IQR, 4-11.2 days)). Sixty-five patients were discharged home with a median duration of hospital stay of 10 days (IQR, 7-15 days). CONCLUSION: Patients with MIS-C may have severe cardiac findings and intensive care requirements in admission and hospital follow-up. The vast majority of these findings improve with effective treatment without any sequelae until discharge and in a short time in follow-up. Although the pathogenesis and treatment plan of the disease are partially elucidated, follow-up studies are needed in terms of long-term prognosis and relapse probabilities.

Prognostic value of oxygen saturation and heart rate during a six-minute walk test in pediatric pulmonary hypertension

Background/aim: To evaluate the pre and post-treatment heart rate and oxygen (O2) saturation values measured before, during and after 6MWT in children who received PAH-specific treatment and to determine the effect of these variables on prognosis. Materials and methods: This retrospective study included 29 patients who were diagnosed as PAH and treated. The transcutaneous O2 saturation and heart rate levels were recorded before the test: baseline (Sat0, HR0), at the end of the test: exercise (Sat1, HR1) and 5 min after the test: recovery (Sat2, HR2). The increase in heart rate was defined as HR1-HR0 and the decrease in saturation as Sat0-Sat1. The results obtained before and after the PAH-specific treatment were analyzed and their effect on survival was assessed. Results: Fifteen of 29 patients were female (51.7%). The mean age was 127.2 ± 44.8 months and the median follow-up time was 60 (12­156) months. Only pre-treatment HR1 was found associated with survival. The mean HR1 value of survivors was 122.8 ± 18.4 and that of deceased 94.3 ± 19.1 (p = 0.03). Post-treatment 6MWT was associated with survival. It was 453.3 ± 96.5 m for survivors and 250 ± 135.2 m for deceased (p = 0.02). Conclusion: Post-treatment 6MWT, FC and proBNP had prognostic value in pediatric patients with PAH. The decrease in saturation and increase in heart rate were not found to have a prognostic value. Pre-treatment HR1 was associated with survival. Identification of these prognostic factors at the beginning and throughout the treatment may be a guide for detecting the severity of the disease and follow-up.

A retrospective study on children with pulmonary arterial hypertension: A single-center experience.

OBJECTIVE: The aim of this study was to evaluate children with pulmonary arterial hypertension (PAH) regarding epidemiological characteristics, clinical status with respect to the WHO functional class (WHO-FC), prognostic factors, and efficacy of medical treatment. METHODS: A retrospective evaluation of 41 patients with PAH was made in the Pediatric Cardiology Unit, Gazi University Medical Faculty, between February 2006 and October 2015. RESULTS: Of the 41 patients included in this study, 51.2% were female. The median age was 60 months at first evaluation. The median follow-up was 60 months. At the start of the treatment, 43.9% patients were receiving combined drug therapy, and this rate increased to 60.9% by the last evaluation. The median time of adding a new medication to the therapy was 20 months. The 1- and 5-year survival rates were 94% and 86%, respectively. At the time of diagnosis, only pro-brain natriuretic peptide (proBNP) levels were associated with mortality (p=0.004), but at the last evaluation, 6-min walking test, proBNP and uric acid levels, and WHO-FC were also associated with survival (p=0.02, p=0.001, p=0.002, and p=0.05, respectively). CONCLUSION: With current treatment choices in experienced centers, positive results are obtained with respect to the functional status and survival rates of patients with PAH. At the time of diagnosis, only proBNP had a prognostic value, whereas at the last evaluation, WHO-FC, 6-min walking test, proBNP, and uric acid were reported prognostic factors. For preventing rapid progression, determination of factors that have an effect on prognosis, in particular, is extremely important.

Immunomodulator effect of topical zinc oxide application in tuberculin skin test.

OBJECTIVE: We aimed to evaluate the sensitivity of tuberculin skintest (purified protein derivative-PPD) by topical zinc application on test site to improve diagnostic reliability. METHODS: We performed this study in 100 children aged 6-14 years, and plasma zinc levels were analyzed after 10-12 hours fasting. After PPD, we applied 40% zinc oxide cream on one forearm and placebo on the other forearm. PPD indurations were measured 72 hours later. RESULTS: In this study, 26% of the children showed increases in PPD induration following local zinc applications. There was no correlation between indurations size and serum zinc levels. CONCLUSION: We concluded that topical zinc cream application can enhance sensitivity of tuberculin reactivityin the diagnosis of tuberculosis.

Surgical and transcatheter management alternatives in refractory pulmonary hypertension: Potts shunt.

Despite advances in the medical treatment of children with pulmonary arterial hypertension that have resulted in improved health quality and life expectancy, the progression of the disease is still the main problem for some patients. Because of this undesirable condition, the search for new treatment strategies continues for pediatric cardiologists. At this point, the Eisenmenger physiology is the main target because of the long-life expectancy and more stable hemodynamics of patients with Eisenmenger syndrome. Therefore, some invasive procedures may be used for conversion to Eisenmenger physiology with the aim of decompressing the right ventricle.

Association of glycosylated hemoglobin (HbA1c) levels with Iinsulin resistance in obese children.

OBJECTIVES: We investigated the relationship between insulin resistance reflected by homeostasis model assessment (HOMA-IR) index and serum HbA1c levels of obese children. MATERIAL AND METHODS: This study included 70 obese and 60 normal weight healthy children between the ages of 3 and 15. Anthropometric measures and biochemical tests (fasting glucose, fasting insulin, HbA1c) were performed on all subjects. Plasma glucose levels were measured by the glucose oxidase method. Plasma insulin concentrations were measured by radioimmunoassay (RIA). HOMA-IR index was used to estimate insulin resistance. A cut-off HOMA-IR level of ≥2.5 was accepted. The HbA1c analysis was performed using high-pressure liquid chromatography. The statistical analysis was performed using SPSS 5. Student's unpaired t-test and the Mann-Whitney U test were used to determine statistical significance. RESULTS: Gender distribution did not reveal significant difference among the obese (F: 48.6%, M: 51.4%) and the non-obese (F: 46.7%, M: 53.3%) groups. The mean age value was significantly higher in the obese group (10.09 ± 3.09) (p > 0.005) than the non-obese group (8.31 ± 3.14) (p < 0.05). The mean value of body mass index (BMI) was 25.55 ± 4.3 in the obese group and 16.63 ± 2.3 in the non-obese group. The mean HOMA-IR values of obese group (2.84 ± 1.77) was significantly higher than the non-obese group (1.50 ± 0.95) (p < 0.005). Insulin resistance was significantly higher in the obese group. Subjects with HOMA-IR ≥2.5 levels in the obese group had significantly higher HbA1c values than those with HOMA-IR <2.5 levels. CONCLUSIONS: High HbA1c levels in obese children can be used as a screening tool to detect insulin sensitivity and resistance at an early stage.