Comparison of MicroRNA Levels of 18-60-month-old Autistic Children with Those of Their Siblings and Controls.

Objective: The present study aims to compare the levels of 7 microRNAs (mi-RNAs) (mi-RNA-125b, mi-RNA-23a-3p, mi-RNA-146a-5p, mi-RNA-106a, mi-RNA-151a-3p, mi-RNA-28, mi-RNA-125a) in the blood of the preschool children with autism and those of their siblings with healthy controls, and to investigate the association between these mi-RNAs and the severity of autism, behavioral problems, and siblings' autistic traits. Methods: A total of 35 children diagnosed with autism spectrum disorder (ASD) at the ages of 18-60 months (patient group), 35 non-affected siblings of the ASD group (sibling group), and 30 control subjects (control group) were involved in the study. The severity of ASD was measured using the Childhood Autism Rating Scale and the Autism Behavior Checklist (ABC). The behavioral problems of the children with ASD were assessed with the Aberrant Behavior Checklist, and the autistic traits of the siblings were assessed using the Autism spectrum screening scale for children. Results: mi-RNA-106a-5p, mi-RNA-151a-3p, and mi-RNA-28-3p were found to be expressed significantly lower in the patient group compared to the control group. There was a significant positive correlation between mi-RNA-23a and the sensory subscale of the ABC. mi-RNA-151a was significantly associated with sound sensitivity and mi-RNA-28 with echolalia. After controlling for age and sex, the differences between groups were disappeared. Conclusion: The present study examined mi-RNAs that have been reported as biomarkers in the literature. Although several symptom clusters are found to be related to certain mi-RNA expression levels, they were not found to be significant in discriminating the patient and healthy groups.

Multisystem inflammatory syndrome in children associated with COVID-19 in 101 cases from Turkey (Turk-MISC study).

AIM: Multisystem inflammatory syndrome in children (MIS-C) may cause shock and even death in children. The aim of this study is to describe the clinical features, laboratory characteristics and outcome of children diagnosed with MIS-C in 25 different hospitals in Turkey. METHODS: The retrospective study was conducted between 8 April and 28 October 2020 in 25 different hospitals from 17 cities. Data were collected from patients' medical records using a standardised form. Clinical and laboratory characteristics and outcomes according to different age groups, gender and body mass index percentiles were compared using multivariate logistic regression analysis. RESULTS: The study comprised 101 patients, median age 7 years (interquartile range (IQR) 4.6-9.3); 51 (50.5%) were boys. Reverse-transcriptase polymerase chain reaction (PCR) assay was positive in 21/100 (21%) patients; 62/83 (74.6%) patients had positive serology for SARS-CoV-2. The predominant complaints were fever (100%), fatigue (n = 90, 89.1%), and gastrointestinal symptoms (n = 81, 80.2%). Serum C-reactive protein (in 101 patients, median 165 mg/L; range 112-228), erythrocyte sedimentation rate (73/84, median 53 mm/s; IQR 30-84) and procalcitonin levels (86/89, median 5 µg/L; IQR 0.58-20.2) were elevated. Thirty-eight patients (37.6%) required admission to intensive care. Kawasaki disease (KD) was diagnosed in 70 (69.3%) patients, 40 of whom had classical KD. Most patients were treated with intravenous immunoglobulin (n = 92, 91%) and glucocorticoids (n = 59, 58.4%). Seven patients (6.9%) died. CONCLUSION: The clinical spectrum of MIS-C is broad, but clinicians should consider MIS-C in the differential diagnosis when persistent fever, fatigue and gastrointestinal symptoms are prominent. Most patients diagnosed with MIS-C were previously healthy. Immunomodulatory treatment and supportive intensive care are important in the management of cases with MIS-C. Glucocorticoids and intravenous immunoglobulins are the most common immunomodulatory treatment options for MIS-C. Prompt diagnosis and prompt treatment are essential for optimal management.

COVID-19 associated multisystemic inflammatory syndrome in 614 children with and without overlap with Kawasaki disease-Turk MIS-C study group.

Multisystemic inflammatory syndrome (MIS-C) diagnosis remains difficult because the clinical features overlap with Kawasaki disease (KD). The study aims to highlight the clinical and laboratory features and outcomes of patients with MISC whose clinical manifestations overlap with or without KD. This study is a retrospective analysis of a case series designed for patients aged 1 month to 18 years in 28 hospitals between November 1, 2020, and June 9, 2021. Patient demographics, complaints, laboratory results, echocardiographic results, system involvement, and outcomes were recorded. A total of 614 patients were enrolled; the median age was 7.4 years (interquartile range (IQR) 3.9-12 years). A total of 277 (45.1%) patients with MIS-C had manifestations that overlapped with KD, including 92 (33.3%) patients with complete KD and 185 (66.7%) with incomplete KD. Lymphocyte and platelet counts were significantly lower in patients with MISC, overlapped with KD (lymphocyte count 1080 vs. 1280 cells × µL, p = 0.028; platelet count 166 vs. 216 cells × 103/µL, p < 0.001). The median serum procalcitonin levels were statistically higher in patients overlapped with KD (3.18 vs. 1.68 µg/L, p = 0.001). Coronary artery dilatation was statistically significant in patients with overlap with KD (13.4% vs. 6.8%, p = 0.007), while myocarditis was significantly more common in patients without overlap with KD features (2.6% vs 7.4%, p = 0.009). The association between clinical and laboratory findings and overlap with KD was investigated. Age > 12 years reduced the risk of overlap with KD by 66% (p < 0.001, 95% CI 0.217-0.550), lethargy increased the risk of overlap with KD by 2.6-fold (p = 0.011, 95% CI 1.244-5.439), and each unit more albumin (g/dl) reduced the risk of overlap with KD by 60% (p < 0.001, 95% CI 0.298-0.559). CONCLUSION: Almost half of the patients with MISC had clinical features that overlapped with KD; in particular, incomplete KD was present. The median age was lower in patients with KD-like features. Lymphocyte and platelet counts were lower, and ferritin and procalcitonin levels were significantly higher in patients with overlap with KD. WHAT IS KNOWN: • In some cases of MIS-C, the clinical symptoms overlap with Kawasaki disease. • Compared to Kawasaki disease, lymphopenia was an independent predictor of MIS-C. WHAT IS NEW: • Half of the patients had clinical features that overlapped with Kawasaki disease. • In patients whose clinical features overlapped with KD, procalcitonin levels were almost 15 times higher than normal. • Lethargy increased the risk of overlap with KD by 2.6-fold in MIS-C patients. • Transient bradycardia was noted in approximately 10% of our patients after initiation of treatment.

Altered kynurenine pathway metabolite levels in toddlers and preschool children with autism spectrum disorder.

AIM: There are increasing reports about the potential role of kynurenine pathway metabolites in autism spectrum disorder (ASD). Early childhood is a very crucial period of time for the etiopathogenesis of ASD and previous studies reported an age-dependent alteration in kynurenine metabolism. However, no study specifically examined kynurenine metabolites in very young children with ASD. This study aimed to investigate kynurenine pathway metabolite levels, kynurenine pathway enzyme activities and neuroprotective index (kynurenic acid/3-hydroxykynurenine ratio) in toddlers and preschool children with ASD. MATERIALS AND METHODS: A total of 68 children with ASD and 44 healthy controls aged between 18 and 60 months were included in this study. Serum levels of kynurenine pathway metabolites were determined by liquid chromatography-mass spectrometry/mass spectrometry system. RESULTS: Serum 3-hydroxykynurenine and kynurenic acid concentrations were significantly higher in the ASD group than in the control group, whereas serum 3-hydroxyanthranilic acid concentrations were significantly lower. CONCLUSIONS: These findings showed that the kynurenine pathway may play a role in the etiopathogenesis of ASD in early childhood.

First Evaluation of P Dispersion and Tp-e Parameters in Electrocardiograms of Children with Diabetic Ketoacidosis

Objective: Diabetic ketoacidosis (DKA) is an important complication of type 1 diabetes mellitus. We aimed to evaluate the effect of metabolic disorders of DKA on electrocardiography (ECG) parameters in children. Methods: This study was performed between December 2018 and March 2020 and included 39 children with DKA and 40 healthy children. Three ECGs (one before and two after treatment) were obtained from the patient group. P-wave dispersion (Pd), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e intervals, and the ratios of Tp-e/QT and Tp-e/QTc were measured electrocardiographically. ECG parameters from children with DKA and healthy controls were compared statistically. Results: The mean age of the patient group was 10.50±4.12 years. There was no significant difference in terms of age, gender, weight, height and body mass index between patients and controls. In the patient group, a statistically significant increase was found in Pd, QTd and QTcd in the initial ECG compared to the second and third ECGs. Also, when the first and third ECGs were compared, a significant increase in Tp-e and Tp-e/QT was evident in the first ECG. There was a significant difference in the values of Pd, QTd, QTcd, Tp-e and Tp-e/QT in the first ECGs, obtained before DKA treatment, and those values obtained from the control group. Conclusion: This is the first article evaluating Pd and Tp-e parameters in children with DKA. Cardiac arrhythmia risk markers were increased in children with DKA compared to controls. Therefore, clinicians should be aware of the possibility of developing new arrhythmias during DKA treatment.

Is there a relationship between causative microorganisms and hearing loss in neonatal sepsis?

BACKGROUND: The aim of the present study was to determine the possible relationship between cultured microorganisms and hearing loss in infants admitted to the neonatal intensive care unit (NICU) who could not pass a standard hearing test. METHOD: The medical records of infants treated at the NICU were retrospectively evaluated. The patients were first divided into two groups, and group 1 was divided into two subgroups: Group 1 included patients with hearing loss accompanied by proven sepsis caused by either gram-negative (group 1A) or gram-positive (group 1B) bacteria, and group 2 included patients with clinical sepsis. The groups were compared with potential risk factors related to hearing loss. RESULTS: Between January 2014 and January 2019, the cases of 3,800 infants admitted to the NICU were reviewed. Of 3,548 living babies, the Auditory Brainstem Response (ABR) test showed that 35 infants (0.98%) were diagnosed with hearing loss. In 12 infants with hearing loss, microbial growth in the blood cultures was detected, whereas in the remaining 23, the blood cultures were negative. Of the cases with microbial growth, five were gram negative and seven were gram positive. In the comparison of groups 1A, 1B, and 2, there were statistically significant differences in terms of risk factors such as low birth weight (p = .048), neonatal hospitalization time (p = .001), free oxygen support (p = .001), intraventricular bleeding (p = .001), loop diuretic use (p = .001), and blood transfusion (p = .048). CONCLUSION: The relationship between hearing loss and microorganisms causing sepsis could not be determined in this research.

Evaluation of Appetite-Regulating Hormones in Young Children with Autism Spectrum Disorder.

This study aimed to investigate the role of leptin, ghrelin, neuropeptide Y, and nesfatin-1 in young children with autism spectrum disorder (ASD). A total of 44 children with ASD and 44 healthy controls aged 18-60 months were included. Plasma levels of hormones were measured using commercial enzyme-linked immunosorbent assay kits. Plasma leptin and ghrelin levels were significantly higher in the ASD group than in the control group. However, no significant difference for plasma neuropeptide Y and nesfatin-1 levels was detected between the groups. No relation was found between the severity of ASD symptoms, severity of eating problems, and plasma levels of hormones. Leptin and ghrelin may play a potential role in the pathogenesis of ASD.

Investigation of the effect of epicardial adipose tissue thickness on cardiac conduction system in children with type 1 diabetes mellitus.

Objectives Investigation of the association between epicardial adipose tissue thickness (EATT) and P-wave dispersion (Pd), QT dispersion (QTd), corrected QT dispersion (QTcd) and Tp-e interval in children with Type 1 Diabetes Mellitus (T1DM) was aimed. Methods Forty-one children with T1DM and 41 age- and gender-matched healthy children were included in the study. Demographical characteristics of all cases were examined. In echocardiography; in addition to conventional echocardiographic measurements, end-systolic EATT was measured from right ventricular free wall. In electrocardiogram; Pd, QTd, QTcd and Tp-e interval durations, as well as Tp-e/QT and Tp-e/QTc ratios were calculated. Correlation values between EATT and electrocardiographic parameters were also noted. Results Mean age of the patient group was determined to be 12.43 ± 3.04 years and that of the control group was determined to be 12.08 ± 2.56 years. There was no significant difference between the groups in regard to age, gender, body weight, height and body mass index. In the patient group; EATT, Pd, QTd, QTcd and Tp-e interval were determined to be significantly higher compared to the control group. In the patient group, no significant correlation was determined between EATT and Pd, QTd, QTcd and Tp-e. However, when both patient and control groups were evaluated together, a statistically significant positive correlation was determined between EATT and Pd, QTd, QTcd and Tp-e. Conclusions In children with T1DM, an increase in epicardial adipose tissue thickness and in risk of cardiac arrhythmias has been demonstrated. To reveal the possible unfavorable effects of EATT on cardiac conduction system in T1DM patients needs further studies.

Mosaic trisomy 14 and aorta-pulmonary window association: A case report.

Trisomy 14 mosaicism is a rare chromosomal abnormality with distinct and recognizable clinical features. Congenital heart anomalies can accompany in this syndrome. To the best of our knowledge, this is the first case of mosaic trisomy 14 with an aortopulmonary window to be described in the literature.

Assessment of 17 Pediatric Cases With Colchicine Poisoning in a 2-Year Period.

AIM: The aim of the study is to discuss clinical effects, treatments, and outcomes of pediatric colchicine poisoning. METHOD: This study was designed as an observational case series study. The medical records of children aged between 0 and 18 years, who were hospitalized for colchicine poisoning at the Department of Pediatric Intensive Care Unit, Cumhuriyet University Faculty of Medicine, between January 2010 and January 2012, were retrospectively evaluated. RESULTS: We presented 17 children with colchicine poisoning. The mean (SD, range) age of patients was 71.5 (69.19, 18-204) months. The period to apply to the hospital after taking the medications was 7.3 hours (7.97, 30 minutes-26 hours) on average. The use of colchicine was due to diagnosis of Familial Mediterranean fever (FMF) in the families of 8 patients, diagnosis of Behçet disease in 1 patient's father, diagnosis of Behçet disease in 1 patient herself, and diagnosis of FMF in 6 patients themselves. Thirteen patients had taken colchicine at the dose of less than 0.5 mg/kg known as subtoxic and 1 patient had taken colchicine at the dose of greater than 0.8 mg/kg, and doses taken by 3 patients were not known. Fourteen patients (82.4%) had involuntary drug intake. Fifty percent of them were symptomatic at the moment of application and all had gastrointestinal complaints. All patients were observed in intensive care unit upon first admission and received supportive care. One of patients showed total alopecia, one showed leucocytosis, and another one showed acute abdomen picture. None of the patients showed mortality. CONCLUSIONS: Mortality of colchicine toxicity is high and quick assessment is absolutely required. In regions where FMF is common and the use of colchicine is high, clinicians should pay attention to symptoms and findings related to colchicine intoxication and keep them in mind in differential diagnosis.

Identification of SLC22A5 Gene Mutation in a Family with Carnitine Uptake Defect.

Primary systemic carnitine deficiency is caused by homozygous or compound heterozygous mutation in the SLC22A5 gene on chromosome 5q31. The most common presentations are in infancy and early childhood with either metabolic decompensation or cardiac and myopathic manifestations. We report a case of 9-year-old boy with dysmorphic appearance and hypertrophic cardiomyopathy. Tandem MS spectrometry analysis was compatible with carnitine uptake defect (CUD). His sister had died due to sudden infant death at 19 months. His second 4-year-old sister's echocardiographic examination revealed hypertrophic cardiomyopathy, also suffering from easy fatigability. Her tandem MS spectrometry analyses resulted in CUD. We sequenced all the exons of the SLC22A5 gene encoding the high affinity carnitine transporter OCTN2 in the DNA. And one new mutation (c.1427T>G → p.Leu476Arg) was found in the boy and his sister in homozygous form, leading to the synthesis of an altered protein which causes CUD. The parent's molecular diagnosis supported the carrier status. In order to explore the genetic background of the patient's dysmorphic appearance, an array-CGH analysis was performed that revealed nine copy number variations only. Here we report a novel SLC22A5 mutation with the novel hallmark of its association with dysmorphologic feature.

Effect of obesity on left ventricular longitudinal myocardial strain by speckle tracking echocardiography in children and adolescents.

BACKGROUND: Impaired subclinical ventricular function may contribute to the risk of cardiovascular disease in obesity. AIMS: The aim of this study was to determine the influence of obesity on left ventricular (LV) longitudinal myocardial function in normotensive obese children using two-dimensional (2D) speckle tracking echocardiography (STE). STUDY DESIGN: Case-control study. METHODS: Sixty normotensive obese children aged 10-16 years (mean age, 13.9±2.3 years) were compared with 50 normal-weight controls. Obese participants had a body mass index (BMI)≥95(th) percentile. Regional strain/strain rate (SR) values were compared with left ventricular (LV) parameters. The correlation was studied by linear regression analysis. RESULTS: Obese subjects exhibited a significantly higher LV end-diastolic diameter, left atrium/aortic diameter ratio, and LV mass/index when compared to controls (p<0.001). Left ventricular ejection fraction and regional systolic myocardial velocities were similar in the obese and control groups. By 2D STE, regional strain of both the septal wall (average strain: -16.0±3.9% vs -21.9±2.4%, p<0.001) and lateral wall (average strain: -15.6±2.3% vs -22.9±3.5%, p<0.001); regional SR of both the septal wall (average SRsys: -0.7±0.22 s(-1) vs -1.3±0.32 s(-1), p<0.001) and lateral wall (average SRsys: -0.67±0.19 s(-1) vs -1.33±0.31 s(-1), p<0.001); regional SRE/A of both the septal wall (average SRE/A: 1.8±0.83 vs. 2.2±0.91, p: 0.004) and lateral wall (average SRE/A: 1.4±0.43 vs. 2.4±1.21, p<0.001); and global strain (-14.6±7.34% vs -20.9±3.24%, p<0.001) were lower in the obese group compared with the controls. These strain imaging parameters appear to be related to the severity of obesity and can contribute to increased BMI. Left ventricular mass was found to be correlated with a decrease in global LV strain. CONCLUSION: Our study showed that childhood obesity is associated with an alteration in the longitudinal LV function. Segmental analysis of the LV can provide subtle markers for the emergence of future obesity-related cardiac disease.

Neutrophil-lymphocyte ratio in children with familial Mediterranean fever: Original article.

OBJECTIVE: The aim of present study was (a) to evaluate the relationship between the neutrophil/lymphocyte (N/L) ratio and mutation types of familial Mediterranean fever (FMF) in children and (b) to evaluate the relationship between the N/L ratio and age. MATERIAL AND METHODS: Three hundred forty-three children with familial Mediterranean fever in the attack-free period and 283 healthy control children were included in the study. Patients were divided into subgroups according to mutation types. Neutrophil and lymphocyte counts were retrieved from medical records of patients and the N/L ratio was calculated from these parameters. RESULTS: The N/L ratio of patients was found to be significantly higher than that of controls (p<0.001). Among 343 patients, homozygous, heterozygous, and compound mutations were observed in 39, 253, and 51 patients, respectively. The differences in the N/L ratio among patients with homozygous, heterozygous, and compound mutations were not statistically significant. The most common mutations were M694V (n=126), E148Q (n=70), M680I, (n=33), and V726A (n=28). Significant differences were not observed among these mutations in terms of the N/L ratio (p>0.05). In all subjects, there was a weak but significant relationship between age and the N/L ratio (r: 0.215, p<0.001). CONCLUSION: The N/L ratio, which can be determined by simple methods in routine blood tests, may be used for the follow-up monitoring of chronic inflammation in patients. In addition, the N/L ratio may give an idea to clinicians regarding the early initiation of treatment in patients with typical clinical findings of FMF.

Value of ABO blood group in predicting the severity of children with Crimean-Congo hemorrhagic fever.

PURPOSE: The aim of this study was to assess the role of ABO blood groups in predicting disease severity and bleeding potential in children with Crimean-Congo hemorrhagic fever (CCHF). METHODS: One hundred fifty-one hospitalized patients with CCHF were enrolled in this retrospective study. The patients were divided according to O- and non-O- (A, B and AB) blood groups (n=91 and n=60, respectively). They were also classified into two groups (severe and non-severe) based on disease severity (n=29 and n=122, respectively). Demographic characteristics, clinical findings, and hematologic and biochemical parameters of all patients were recorded on admission and discharge. RESULTS: Although, in all cases, compared to the non-O blood group, the ratio of the blood group O was considerably higher (60% vs. 40%) and similarly so in severe cases (58.6% vs. 41.4%), this difference was not statistically significant (p>0.05). The aPTT at discharge and fever duration of the O-blood group were significantly higher than those of the non-O-blood group (p=0.042, p=0.034, respectively). The factor VIII level of the O-blood group was significantly lower than that of the non-O-blood group (p=0.040). Although the ratios of bleeding and severity were higher in the O-blood group compared to the other group, statistical significance was not reached (p>0.05). CONCLUSIONS: Consideration of the ABO blood group is important during diagnostic follow-up to assess the severity of CCHF. In clinical practice, pediatric CCHF patients with the O blood group need to be followed closely for tendency to bleed.

P-wave and QT interval dispersion analysis in children with Eisenmenger syndrome.

OBJECTIVES: P-wave and QT dispersion are increased and associated with atrial and ventricular arrhythmia and an increase in sudden death in a variety of diseases. This study aimed to investigate P-wave and QT dispersion in children with Eisenmenger syndrome (ES). STUDY DESIGN: The study group included 27 children (15 females, 12 males) with both congenital heart disease (CHD) and ES. The control group consisted of 30 children with CHD without pulmonary arterial hypertension. Electrocardiographic records were used to determine P-wave, QT, and corrected QT (QTc) dispersions. 24-hour (h) rhythm Holter was fitted in all patients. Atrial volumes, ventricular dimensions and tricuspid annular plane systolic excursion (TAPSE) were measured by echocardiography. RESULTS: There was no difference between groups with regard to age, sex, weight, and body surface area (p>0.05). Right atrial volume was significantly larger in the ES group than in the control group. P-wave, QT and QTc dispersions were higher in the patients with ES (50.10±11.12 vs. 26.32±8.90, p<0.001; 57.40±24.21 vs. 38.20±8.92 ms, p<0.001; and 78.20±16.02 vs. 56.52±13.92 ms, p<0.001, respectively). Ventricular and supraventricular ectopy were significantly more frequent in the ES group. Four patients (14.8%) in the study group had tachyarrhythmias during 24-h Holter monitoring. CONCLUSION: In our study, P-wave and QT dispersion were found to be greater in children with ES than in the healthy control subjects.

The effects of pre-pregnancy obesity on fetal cardiac functions.

Obesity is a substantial public health problem with a rapidly increasing prevalence in numerous industrialized nations. The objective of this study was to evaluate the effects of maternal pre-pregnancy obesity on fetal cardiac functions. We studied 55 fetuses of obese mothers and 44 fetuses of healthy mothers at 26-38 weeks of gestation. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. The two groups were similar in terms of maternal age, gravidity, parity, gestational age, estimated birth weight, serum lipids, and systolic-diastolic blood pressure. Fetal heart rate, diameters of the aortic and pulmonary valve annulus, aortic and pulmonary peak systolic velocities, ventricular systolic function, and cardiothoracic ratio were similar in the two groups. Pulsed-wave Doppler-derived E/A ratios in the mitral and tricuspid valves were similar in the two groups. The deceleration time of early mitral inflow was prolonged in the fetuses of the obese mothers. In the interventricular septum, left ventricle posterior wall, and right ventricle free wall, the E a and A a were higher, and E a/A a ratios were significantly lower in the study group than in the control group. The E/E a ratio was higher in the obese group than in the control group. The isovolumic relaxation time and the right and left ventricle myocardial performance indices were higher in the fetuses of the obese mothers than in the fetuses of the healthy mothers. We believe that maternal obesity has an important influence on fetal cardiac diastolic functions.

Evaluation of the serum levels of soluble IL-2 receptor and endothelin-1 in children with Crimean-Congo hemorrhagic fever.

We aimed to assess the association between serum levels of soluble IL-2 receptor (sIL-2r) and endothelin-1 and severe infection in children with Crimean-Congo hemorrhagic fever (CCHF). Fifty-two patients under 18 years of age with a laboratory- confirmed diagnosis of CCHF and 38 healthy controls were enrolled in the study. Patients were classified into two groups based on disease severity (severe group and non-severe group). The sIL-2r and endothelin-1 levels were observed to be significantly higher in patients with severe CCHF compared with those with non-severe CCHF and the control group (p < 0.05). In addition, those with non-severe CCHF were also found to have a significantly higher sIL-2r level relative to the control group (p < 0.001). Although there was a positive correlation between sIL-2r and endothelin-1 levels, serum levels of both sIL-2r and endothelin-1 were negatively correlated with the platelets count. In children with CCHF, serum levels of sIL-2r and endothelin-1 were increased, and this increase is related to the severity of the disease. In this study, we concluded through prognosis that serum levels of sIL-2r and endothelin-1 might be related, and that hemophagocytic lymphohistiocytosis and endothelial injury might contribute to a pathogenesis of the disease.

Noninvasive evaluation of cardiac autonomic modulation in children with primary Raynaud's phenomenon: a controlled study.

This study aimed to objectively evaluate autonomic nervous function in children with primary Raynaud's phenomenon (PRP). Thirty-two children with PRP and 30 healthy subjects were included in the study. We analyzed heart rate variability (HRV) in the time domain by the following six standard time-domain measures: standard deviation of all normal R-R intervals during 24 h (SDNN), standard deviation of all normal R-R intervals for all 5-min segments (SDNNi), standard deviation of the average normal R-R intervals for all 5-min segments (SDANN), root mean square of the successive normal R-R interval difference, percentage of successive normal R-R intervals longer than 50 ms, and triangular index (integral of the density distribution of NN intervals divided by the maximum of the density distribution). The mean heart rate throughout 24 h was significantly higher in the PRP group than in the control group (p = 0.001). Although heart rate during the activity period was not significantly different from that during the night period, it was higher in the PRP group than in the control group (p = 0.002). In children with PRP, HRV analysis showed significantly lower values of SDNN (p = 0.01), SDNNi (p = 0.005), SDANN (p = 0.02), and HRV triangular index (p = 0.02) compared with the control group. HRV analysis for sympathovagal balance demonstrated a preponderance for the sympathetic component in patients with PRP. We conclude that all time-domain parameters evaluated in HRV analysis are significantly lower in children with PRP than in healthy subjects.

Assessment of cardiac functions in fetuses of gestational diabetic mothers.

We investigated cardiac function in 67 fetuses of gestational diabetic mothers (FGDMs) and 122 fetuses of healthy mothers between 24 and 36 weeks of gestation. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. Fetal echocardiograms were performed at 24, 28, 32, and 36 weeks of gestation. Glycated hemoglobin (HbA1c) levels were obtained from all pregnant women at 24 weeks of gestation. The mean age of diabetic pregnant women was significantly greater than that of controls. Serum HbA1c values of both groups were within normal limits, but they were significantly greater in the diabetic group (p = 0.003). The increase in peak aortic and pulmonary artery velocities were greater in FGDM (p < 0.001). No pathological interventricular septal (IVS) hypertrophy was observed. There was a significant increase in IVS thickness in FGDM compared with controls, which was more prominent at the end of the third trimester (p < 0.001). During the course of pregnancy, mitral E-wave (p < 0.001), A-wave (p = 0.007), tricuspid E-wave (p < 0.001) and A-wave (p = 0.002) velocities were greater in FGDM. The increases in mitral E/A and tricuspid E/A ratios were lower in FGDM with advancing gestation. The E a-wave (p = 0.02), A a-wave (p = 0.04), and S a-wave (p < 0.001) velocities of the right-ventricular (RV) free wall and the E a (p = 0.02) and A a (p = 0.01) velocities of the left-ventricle (LV) posterior wall were greater in FGDM during the course of pregnancy. The E a/A a ratio of the RV posterior wall was greater in FGDM with advancing gestation (p < 0.03). LV and RV E/E a ratios were similar in both groups. The LV myocardial performance index measured by pulsed-wave Doppler was greater in FGDM (p < 0.001). We detected diastolic dysfunction in FGDM. The data suggest that gestational diabetes mellitus may impair ventricular diastolic functions without causing pathological fetal myocardial hypertrophy. We detected subclinical diastolic dysfunction using both pulsed-wave and tissue Doppler imaging in FGDM.