Cardiac Inflammatory Myofibroblastic Tumor Causing Pulmonary Artery Obstruction: A Rare Case Report.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor of the heart. In the literature, cardiac IMT is often described as an endocardial-based cavitary mass originating from the right side of the heart in infants and adolescents. In this article, we present a 5-year-old boy with a rare cardiac IMT who had no complaints and was diagnosed with murmur during his routine examination. Transthoracic echocardiography showed a homogeneous polypoid mass originating from the pulmonary valve, extending into the main pulmonary artery during systole and causing obstruction of the pulmonary artery and right ventricular outflow tract. Surgical resection of the tumor was performed successfully. There was no tumor recurrence in the control echocardiography at the postoperative first month.

Evaluation of left ventricular functions by speckle-tracking echocardiography in coarctation patients.

BACKGROUND/AIM: Two-dimensional speckle-tracking echocardiography (2D-STE) is a novel method that allows the assessment of regional myocardial function. The aim of our study was to use 2D-STE to assess left ventricular deformation in patients with coarctation of the aorta (CoA). METHODS: In this prospective study, patients with CoA (n = 42) and healthy controls (n = 39) were recruited. Children with CoA who visited the outpatient clinic between 2013 and 2014 were included. The data were compared with those obtained from the sex- and age-matched controls. RESULTS: The mean age of the patients was 5.8 ± 4.5 years. Global longitudinal strain based on all three apical views and total global strain values did not appear to be different between the patient and the control groups (P = .59, P = .51, P = .15, P = .38). Hypertension was detected in 14 (33.3%) patients with CoA. There were significant differences between the global longitudinal strain values of the normotensive CoA subgroup and the hypertensive CoA subgroup (P < .05). CONCLUSIONS: In our study, we found that 2D-STE total strain analysis of patients with CoA was not different from comparative healthy controls. However, we determined that 2D-STE parameters were lower in the hypertensive CoA subgroup compared to the normotensive CoA subgroup.

Assessments of left ventricular systolic and diastolic functions with tissue Doppler imaging and myocardial performance index in children with chronic kidney disease.

BACKGROUND: This study aimed to document early left ventricular (LV) dysfunction in chronic kidney disease (CKD) using methods such as tissue Doppler imaging and the myocardial performance index (MPI). METHODS: A total of 40 patients diagnosed with CKD (mean age, 10.1 ± 4.1 years) and 40 sex- and age-matched healthy controls (mean age, 9.6 ± 4.3 years) were examined. In the patient group, 20 patients had early stage (Stage 2-3) CKD and 20 patients had late-stage (stage 4-5) CKD, and 18 patients had hypertension. RESULTS: The pulmonary artery systolic pressure (PAPs) and LV mass index (LVMI) were significantly higher in the patient group (P < 0.05). The LV septal and lateral margins of the mitral annulus E'/A' ratio, E/E' ratio and MPI results were significantly different between the groups (P < 0.05). The MPI scores were higher in late-stage CKD than in early stage CKD (P < 0.05). The E'/A' ratio was lower and the MPI was higher in the hypertensive CKD group compared with the normotensive CKD group (P < 0.05). The E/E' ratio was correlated positively with the LVMI, and the PAPs, and negatively with glomerular filtration rate, S' value, E'/A' ratio. The MPI was correlated positively with blood pressure, LVMI, PAPs, and the S value, and negatively with the E'/A' ratio. CONCLUSIONS: The E'/A' ratio, the E/E' ratio, and the isovolumetric relaxation time measured by tissue Doppler imaging is highly accurate and easily applicable for detecting diastolic LV function, and the MPI is suitable for detecting both systolic and diastolic LV dysfunction. Their routine use may be useful in evaluating LV functions in children with CKD.

Evaluation of left ventricular systolic functions in two-dimensional speckle-tracking echocardiography in children with chronic renal failure.

BACKGROUND AND AIM: Chronic kidney disease may lead to left ventricular dysfunction. Early detection of cardiovascular disease in children with chronic kidney disease is essential to prevent cardiovascular morbidity and mortality in early adulthood. This study aimed to document the dysfunction using methods such as two-dimensional speckle-tracking echocardiography in the early stage. METHODS: A total of 34 patients diagnosed with chronic kidney disease (mean age ± standard deviation, 10.5 ± 4.1 years) and 37 sex- and age-matched (mean age 9.8 ± 4.2 years) healthy controls were studied. The results of the two groups were compared along with those of the published studies. RESULTS: The echocardiography measurements had no significant difference in the end-diastolic and end-systolic diameter values of left ventricular, ejection fraction, shortening fraction, mitral E value, mitral A value, and E/A ratio between the groups. Pulmonary artery systolic and diastolic pressure and left ventricular mass index were significantly higher in the patient group (p < 0.01). The longitudinal global strain values in the apical four-chamber, three-chamber, and two-chamber views and the total global strain values were significantly lower in the patients (p < 0.01). The circumferential global strain values in the apical, mid, basal, and total global strain were lower in the patient group, but this difference was statistically significant in the apical global and total global strain values (p < 0.05). CONCLUSIONS: Speckle-tracking echocardiography might help identify subclinical left ventricular dysfunction in patients with chronic kidney disease with unremarkable conventional echocardiography.

Tissue Doppler and speckle tracking echocardiography assessment of left ventricular function in children with cystic fibrosis.

BACKGROUND: Cystic fibrosis may lead to left ventricular (LV) dysfunction. This dysfunction can be documented by methods such as tissue Doppler echocardiographic (TDI) imaging and two-dimensional speckle tracking echocardiography (STE) in early stage. STUDY DESIGN: This was prospective cohort study. METHODS: A total of 34 patients diagnosed with cystic fibrosis (mean age and SD 9.9 ± 4.9 years) and 37 healthy control subjects with a comparable gender and age distribution (mean age 9.8 ± 4.3) were studied. The results for the two groups were compared along with the results of published reports. RESULTS: No significant relationship was found between the groups in terms of systolic and diastolic measurements of the interventricular septum and posterior left ventricular wall, and ejection fraction (P > .05). Myocardial performance indexes of left ventricular free wall and interventricular septum increased in the patient group compared with the controls (P < .05). As measured by STE, seven segments in the LV myocardial longitudinal strain and three segments in the LV myocardial circumferential strain showed significant reductions in patients with cystic fibrosis compared with controls (P < .05). The longitudinal global, circumferential global, and total global strain values had no significant difference between the groups (P > .05). Longitudinal strain rates and circumferential strain rates were both lower in five segments in the patient group (P < .05). CONCLUSIONS: Tissue Doppler echocardiographic imaging and STE may help identifying subclinical LV dysfunction in cystic fibrosis patients with unremarkable conventional echocardiography. They may be considered for the routine follow-up of cystic fibrosis patients.

Symptom Frequency in Children with Congenital Heart Disease and Parental Care Burden in Predicting the Quality of Life of Parents in Turkey.

PURPOSE: This study was conducted to investigate the symptom frequency and parental care burden of the children with congenital heart disease in predicting the quality of life of parents in Turkey. DESIGN AND METHOD: The study subjects for the present study included 124 parents whose children were suffering from heart disease. The data for the study were collected from these parents using parent information form, the questionnaire of the frequency of heart disease-related symptoms of the child, the caregiver burden scale, and the quality of life scale. The level by which the heart disease-related symptoms of the child and caregiver burden predicted the quality of life was analyzed using the regression analysis. RESULTS: The most common symptoms in children were found to be anorexia, difficulty in activities, palpitations, shortness of breath, weakness, and fatigue. The number of symptoms observed in children and the care burden of the parents were determined to explain 27.1% of the psychological health sub-dimension, 14.4% of the social relations sub-dimension, and 34.9% of the environment sub-dimension. When the variables were examined separately, the number of symptoms was found to significantly predict only social relations sub-dimensions. CONCLUSION: Increased number of symptoms increased parental care burden. Increased number of symptoms and care burden of parents reduced the quality of life of parents. PRACTICE IMPLICATIONS: Symptom management is one of the important responsibilities of nurses in improving care burden and quality of life for parents of children with heart disease.

Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

Both an excess of iron and iron deficiency (ID) may lead to significant cardiac problems. Parameters that represent ventricular repolarization heterogeneity, like QT dispersion (QTd), corrected QT dispersion (QTcd), the interval between the peak and the end of the T wave (Tp-e), and Tp-e dispersion, have not been evaluated in otherwise healthy children with low iron levels before. Here we assessed the effects of low iron storage on P wave dispersion (PWd), QTd, Tp-e intervals, and Tp-e dispersion in otherwise healthy children. We prospectively reviewed 283 patients who were referred to pediatric cardiology department for cardiac evaluation due to murmurs and who were found to have no structural heart disease. The patients were divided into three groups according to their ferritin levels: Group 1: ferritin <15 ng/mL (n = 58); Group 2: ferritin 15-25 ng/mL (n = 80); Group 3: ferritin >25 ng/mL (n = 145). P wave duration (PW), QT and Tp-e intervals, and PW, QT, corrected QT (QTc), and Tp-e dispersions were significantly higher in patients whose ferritin level was <15 ng/mL. A negative correlation was found between ferritin level and QT and QTc intervals, and QT, QTc, and Tp-e dispersions. Our results showed that a low serum ferritin level is associated with changes in some ECG parameters such as prolonged PWd, Tp-e interval, QT, QTc, and Tp-e dispersions in otherwise healthy children, and studies of other populations indicated that these parameters may predict arrhythmias in selected patients. These patients may be considered at some risk of developing arrhythmias. Therefore, careful evaluation of these ECG parameters is necessary in otherwise healthy children with low iron stores.

Can ambulatory blood pressure monitoring detect early diastolic dysfunction in children with type 1 diabetes mellitus: correlations with B-type natriuretic peptide and tissue Doppler findings.

AIM: To evaluate the relationship between 24-h blood pressure (BP) measurements and diastolic heart function evaluated by Doppler tissue imaging and B-type natriuretic peptide (BNP) levels in children with type 1 diabetes mellitus (DM). PATIENTS AND METHODS: A total of 32 diabetic and 18 healthy children were enrolled. Spectral Doppler analysis and tissue Doppler measurements were performed by conventional echocardiography. The 24-h ambulatory BP and serum BNP levels were measured. RESULTS: Analysis of ambulatory blood pressure monitoring (ABPM) recordings showed that median daytime diastolic BP load were significantly higher in diabetic patients compared to controls [12.35 (4.23-27.23) vs. 2.5 (0-8.7), p = 0.007]. Patients with elevated daytime systolic and diastolic BP loads had significantly higher BNP values compared to patients with normal BP load (31.4 ± 24.36 vs. 11.84 ± 11.25 pg/mL, p = 0.03 and 23.21 ± 15.12 vs. 12.12 ± 14.65 pg/mL, p = 0.03, respectively). Isovolemic contraction time (47.43 ± 7.84 vs. 42.27 ± 7.47, p = 0.045), isovolemic relaxation time (68.84 ± 10.43 vs. 58.77 ± 10.02, p = 0.02), and myocardial performance index (0.45 ± 0.10 vs. 0.37 ± 0.09, p = 0.02) as determined by tissue Doppler echocardiography were significantly high in diabetic patients compared to that of control cases. Ratio of mitral peak early diastolic flow velocity (E) to peak early diastolic myocardial velocities by tissue Doppler echocardiography (E') was also higher in patients with elevated daytime systolic BP load (E/E', 6.71 ± 1.97 vs. 4.91 ± 1.02, p = 0.04). CONCLUSION: Elevated BP loads detected by 24-h ambulatory BP measurements in children with type 1 diabetes are associated with increased BNP levels and abnormal tissue Doppler echocardiography indices, indicating early stage cardiac dysfunction.

Assessment of Microvolt T Wave Alternans in Children with Repaired Tetralogy of Fallot during 24-Hour Holter Electrocardiography.

BACKGROUND: We aimed to examine microvolt T wave alternans (MTWA) in 24-hour Holter electrocardiography (ECG) of children with repaired tetralogy of Fallot (TOF) to assess associations of MTWA with ventricular arrhythmias, ECG parameters, and echocardiographic findings. METHODS: Holter ECG records and archive files of 56 repaired TOF patients (62.5% male) who were analyzed retrospectively. Subjects' ECG parameters and MTWA values were compared with age-sex-matched control group. T wave changes were analyzed by time-domain-modified moving average method from the three channels of 24-hour Holter ECG. RESULTS: Mean age was 123.4 ± 48.3 months. Median MTWA value was 55.5 µV in the control group, whereas 95.5 µV in patients group (P < 0.001). A significant weak positive correlation was found between the presence of ventricular extrasystoles and tricuspid regurgitation. There was no correlation between ECG parameters, echocardiographic findings, and MTWA. CONCLUSIONS: MTWA was increased in children with repaired TOF as reported before. To our knowledge, this is the first study analyzing MTWA with 24-hour Holter ECG in repaired TOF patients.

Prolonged Tp-e Interval and Tp-e/QT Ratio in Children with Mitral Valve Prolapse.

Although it is considered to be a benign condition, previous studies have shown that a subset of patients with mitral valve prolapse (MVP) may be at risk of ventricular arrhythmia and sudden cardiac death (SCD). Previous studies have suggested that the interval between the peak and the end of the T wave (Tp-e) can be used as a marker for the transmural dispersion of repolarization. Increased Tp-e interval and Tp-e/QT ratio are associated with ventricular arrhythmias and SCD. The aim of this study was to assess alterations in ventricular repolarization by using the Tp-e interval and Tp-e/QT ratio in children with MVP and to investigate their relationships with the degree of valvular regurgitation. This study prospectively investigated 110 children with MVP and 107 age- and sex-matched healthy control subjects. Tp-e interval, Tp-e/QT ratio, and QT and QTc dispersions were measured from a 12-lead electrocardiogram and compared between groups. QT and QTc dispersions, Tp-e interval, and Tp-e/QTc ratio were found to be significantly higher in patients with MVP. A positive correlation was found between Tp-e/QTc ratio and increase in the degree of mitral regurgitation (MR) (p < 0.05; r = 0.2). However, the degree of MR was not associated with QT, QTc, or Tp-e intervals; QT, QTc, or Tp-e dispersions; or Tp-e/QT ratio (all p values >0.05). Individuals with MVP may be more prone to ventricular arrhythmias due to prolonged QTd, QTcd, and Tp-e interval and increased Tp-e/QT and Tp-e/QTc ratios. Therefore, due to their longer life expectancy, children with MVP should be followed up on regarding life-threatening arrhythmias.

Different haemodynamic patterns in head-up tilt test on 400 paediatric cases with unexplained syncope.

OBJECTIVE: To assess haemodynamic patterns in head-up tilt testing on 400 paediatric cases with unexplained syncope. METHODS: Medical records of 520 children who underwent head-up tilt testing in the preceding year were retrospectively evaluated, and 400 children, 264 (66%) girls and 136 (34%) boys, aged 12.6±2.6 years (median 13; range 5-18), with unexplained syncope were enrolled in the study. Age, sex, baseline heart rate, baseline blood pressure, frequency of symptoms, and/or fainting attacks were recorded. The test protocol consisted of 25 minutes of supine resting followed by 20 minutes of 70° upright positioning. Subjects were divided into nine groups according to their differing haemodynamic patterns. RESULTS: There were no statistically significant differences between the groups with regard to age, gender, baseline blood pressure, and frequency of syncope (p>0.05). The response was compatible with orthostatic intolerance in 28 cases (7.0%), postural orthostatic tachycardia syndrome in 24 cases (6.0%), asymptomatic postural orthostatic tachycardia syndrome in 26 cases (6.5%), orthostatic hypotension in seven cases (1.7%), vasovagal syncope in 38 cases (9.5%), and negative in 274 cases (69.2%). Vasovagal syncope response patterns were of type 3 in nine cases (2.2%), type 2A in 10 cases (2.5%), type 2B in two cases (0.5%), and type 1 (mixed) in 17 cases (4.25%). CONCLUSIONS: In the 400 paediatric cases with unexplained syncope, nine different haemodynamic response patterns to head-up tilt testing were discerned. Children with orthostatic intolerance syndromes are increasingly referred to hospitals because of difficulty in daily activities. Therefore, there is need for further clinical trials in these patient groups.

Revisiting subclavian flap repair for neonates and small infants.

OBJECTIVE: We have utilized subclavian flap angioplasty (SFA) frequently in infants with coarctation particularly in patients with arch hypoplasia which is quite frequent. We have followed these patients with serial echocardiography and have analyzed our results in this study to determine recoartation rates, recurrent hypertension and left arm development. METHODS: Thirty eight infants less than 3 months age (22 boys and 16 girls, mean age was 28±22.6 days) operated at Dokuz Eylul University Hospital between August 2007 - December 2013. Twelve (32%) patients with pulmonary banding due to accompanying VSD or AVSD were included to the study, those infants with complex pathologies such as transposition of great arteries or single ventricle, while the patients less than 1000 gram in weight were excluded. RESULTS: The mean follow-up time was 21 months (1-76 months). Twelve (32%) patients had aortic arch hypoplasia proximal to the left subclavian artery. Operative mortality was found 7.7% for isolated coarctation, 16% for coarctation repair with pulmonary banding. In 5 patients, a residual gradient was detected and re intervention was required in 7.8% patients with balloon angioplasty. CONCLUSION: Subclavian flap angioplasty is a safe repair technique in small infants and neonates. High gradients and intervention more likely depends on the anatomy of the aortic arch rather than the subclavian flap angioplasty technique.

Are mean platelet volume and platelet distribution width useful parameters in children with acute rheumatic carditis?

Rheumatic fever (RF) is an inflammatory disease caused by autoimmune response to a preceding group A streptococcal infection. Mean platelet volume (MPV) reflects the platelet size and the rate of platelet production in bone marrow, and it may be used as an indicator of platelet activation and severity of inflammation. Fifty-three consecutive patients diagnosed with acute rheumatic carditis and 53 control subjects were enrolled into this study. Leukocyte and platelet counts were significantly higher in patients with acute carditis before treatment compared with controls, whereas MPV and platelet distribution width (PDW) values were not significantly different between groups. Platelet counts, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) values were decreased significantly in patients with RF after treatment. There was not a significant difference in terms of platelet count between the controls and the patient group after treatment. ESR was found to be correlated with CRP in patients before and after treatment. In conclusion, the results of our study showed that MPV and PDW levels do not change during acute rheumatic carditis before and after treatment.

Increased P-wave and QT dispersions necessitate long-term follow-up evaluation of Down syndrome patients with congenitally normal hearts.

Reports state that Down syndrome (DS) patients with congenitally normal hearts might experience the development of cardiac abnormalities such as cardiac autonomic dysfunction, valvular lesions, bradycardia, and atrioventricular block. However, the presence of any difference in terms of P-wave dispersion (PWd) and QT dispersion (QTd) was not evaluated previously. This study prospectively investigated 100 DS patients with structurally normal hearts and 100 age- and sex-matched healthy control subjects. Standard 12-lead electrocardiograms were used to assess and compare P-wave and QT durations together with PWd and QTd. The median age of the DS patients and control subjects was 48 months. Heart rates and P-wave and QT dispersions were significantly greater in the DS group than in the control group (113 ± 22.9 vs 98.8 ± 16.6 bpm, p < 0.001; 31.3 ± 9.5 vs 24 ± 8.6 ms, p < 0.001; and 46.6 ± 15.9 vs 26 ± 9.1 ms, p < 0.001, respectively). A positive correlation was found between PWd and age in the DS patients (p < 0.05; r = 0.2). All children with DS should be followed up carefully with electrocardiography in terms of increased P-wave and QT dispersions even in the absence of concomitant congenital heart disease for management of susceptibility to arryhthmias.

A novel missense mutation of the paired box 3 gene in a Turkish family with Waardenburg syndrome type 1.

PURPOSE: Screening of mutations in the paired box 3 (PAX3) gene in three generations of a Turkish family with Waardenburg syndrome type 1 (WS1). METHODS: WS1 was diagnosed in a 13-month-old girl according to the WS Consortium criteria. Detailed family history of the proband revealed eight affected members in three generations. Routine clinical and audiological examination and ophthalmologic evaluation were performed on eight affected and five healthy members of the study family. Dystopia canthorum was detected in all affected patients; however, a brilliant blue iris was present in five patients who also had mild retinal hypopigmentation. Genomic DNA was extracted from the peripheral blood of affected and unaffected individuals in the family as well as 50 unrelated healthy volunteers. All coding exons and adjacent intronic regions of PAX3 were sequenced directly. RESULTS: A novel missense heterozygous c.788T>G mutation was identified in eight patients. This nucleotide alteration was not found in unaffected members of the study family or in the 50 unrelated control subjects. The mutation causes V263G amino-acid substitution in the homeodomain of the PAX3 protein, which represents the 45(th) residue of helix 3. CONCLUSIONS: We identified a novel missense c.788T>G mutation in PAX3 in a family with Waardenburg syndrome with intrafamilial phenotypic heterogeneity.

Clinical accuracy of tympanic thermometer and noncontact infrared skin thermometer in pediatric practice: an alternative for axillary digital thermometer.

INTRODUCTION: The aim of this study was to compare the body temperature measurements of infrared tympanic and forehead noncontact thermometers with the axillary digital thermometer. METHODS: Randomly selected 50 pediatric patients who were hospitalized in Dr Behcet Uz Children's Training and Research Hospital, Pediatric Infectious Disease Unit, between March 2012 and September 2012 were included in the study. Body temperature measurements were performed using an axillary thermometer (Microlife MT 3001), a tympanic thermometer (Microlife Ear Thermometer IR 100), and a noncontact thermometer (ThermoFlash LX-26). RESULTS: Fifty patients participated in this study. We performed 1639 temperature readings for every method. The average difference between the mean (SD) of both axillary and tympanic temperatures was -0.20°C (0.61°C) (95% confidence interval, -1.41°C to 1.00°C). The average difference between the mean (SD) of both axillary and forehead temperatures was -0.38 (0.55°C) (95% confidence interval, -1.47°C to 0.70°C). The Bland-Altman plot showed that most of the data points were tightly clustered around the zero line of the difference between the 2 temperature readings. With the use of the axillary method as the criterion standard, positive likelihood ratios were 17.9 and 16.5 and negative likelihood ratios were 0.2 and 0.4 for tympanic and forehead measurements, respectively. DISCUSSION: The results demonstrated that the infrared tympanic thermometer could be a good option in the measurement of fever in the pediatric population. The noncontact infrared thermometer is very useful for the screening of fever in the pediatric population, but it must be used with caution because it has a high value of bias.

Transcatheter Patent Ductus Arteriosus Closure in Children With Different Devices and Long-Term Results.

INTRODUCTION: With the development of transcatheter interventional techniques and the introduction of next-generation duct occluder devices, transcatheter closure has become the first treatment option for patent ductus arteriosus (PDA) in pediatric patients. In this study, we compared the effectiveness and safety of different devices for transcatheter PDA closure in pediatric patients, focusing on long-term outcomes. METHODS: A total of 235 patients aged 0-18 years who underwent transcatheter PDA closure at a tertiary care center between January 2005 and February 2020 were included. The medical records of the cases were retrospectively evaluated. RESULTS: The median age of the patients was 2.3 years (range: 3.5 months to 17 years), with a mean weight of 12.8 kg (range: 5.7-43.2 kg). The mean PDA diameter at its narrowest point was 2.9 mm (range: 2.2-5.1 mm). Ductal anatomy was as follows: Type A in 98 (41.7%) patients, Type E in 36 (15.6%) patients, Type C in 32 (13.5%) patients, Type F in 27 (11.4%) patients, Type D in 23 (9.7%) patients, and Type B in 19 (8.1%) patients. Arterial access was used in 138 (57.1%) patients, venous + arterial access in 58 (24.6%) patients, and venous access only in 39 (16.5%) patients. Closure was performed with Amplatzer Duct Occluder (ADO; AGA Medical Corp., Golden Valley, MN, USA) II in 151 (64.2%) cases, ADO I in 43 (18.2%) cases, and coils in 41 (17.4%) cases. The mean fluoroscopy time and mean procedural time were 10.3 ± 4.2 minutes and 41 ± 7.2 minutes, respectively. The mean radiation dose was 1364 ± 497 cGy/min. The early closure rate after the procedure was 92%, while residual shunting on the first day post-procedure was observed in 1.8% of cases, decreasing to 0.1% at the one-month follow-up. The overall procedural success rate for all cases was 96.0%. The mean follow-up duration was 9.7 years (range: 2.9-13.8 years). CONCLUSION: For percutaneous PDA closure, ADO I devices are preferred for larger defects, whereas ADO II devices are prioritized for small- to medium-sized defects instead of coils.

Risk factors for coronary arterial involvement in Turkish children with Kawasaki disease: a multicenter retrospective study.

BACKGROUND: Coronary arterial lesions (CALs) are the major component of Kawasaki disease (KD), associated with significant morbidity, which affect a substantial proportion of patients despite proper treatment. The aim of this study was to define the risk factors for CALs in Turkish children with KD. METHODS: Medical records of 399 KD patients from five pediatric rheumatology centers in Turkey were reviewed retrospectively. Demographic, clinical (including duration of fever before intravenous immunoglobulin [IVIG] and resistance to IVIG), laboratory and echocardiographic data were noted. RESULTS: The patients with CALs were younger, had a higher male ratio and a longer duration of fever before IVIG. They also had higher lymphocyte and lower hemoglobin values before the initial treatment. Multiple logistic regression analyses defined the following three criteria as independent risk factors for predicting CALs in Turkish children with KD: age ≤12 months, male gender and duration of fever before IVIG ≥9.5 days. High sensitivity rates of elevated risk of CALs up to 94.5% were calculated despite specificity values falling to 16.5%, depending on which of these three parameters are taken into account. CONCLUSIONS: Based on the demographic and clinical features, we established an easily applicable risk-scoring system for predicting CALs in Turkish children with KD. This may be useful for choosing appropriate treatment and follow-up for KD to prevent coronary artery involvement. Further studies will show whether these risk factors can be used in other Caucasian populations as well.

Clinical and epidemiological features of Turkish children with 2009 pandemic influenza A (H1N1) infection: experience from multiple tertiary paediatric centres in Turkey.

BACKGROUND: In April 2009 a novel strain of human influenza A, identified as H1N1 virus, rapidly spread worldwide, and in early June 2009 the World Health Organization raised the pandemic alert level to phase 6. Herein we present the largest series of children who were hospitalized due to pandemic H1N1 infection in Turkey. METHODS: We conducted a retrospective multicentre analysis of case records involving children hospitalized with influenza-like illness, in whom 2009 H1N1 influenza was diagnosed by reverse-transcriptase polymerase chain reaction assay, at 17 different tertiary hospitals. RESULTS: A total of 821 children with 2009 pandemic H1N1 were hospitalized. The majority of admitted children (56.9%) were younger than 5 y of age. Three hundred and seventy-six children (45.8%) had 1 or more pre-existing conditions. Respiratory complications including wheezing, pneumonia, pneumothorax, pneumomediastinum, and hypoxemia were seen in 272 (33.2%) children. Ninety of the patients (11.0%) were admitted or transferred to the paediatric intensive care units (PICU) and 52 (6.3%) received mechanical ventilation. Thirty-five children (4.3%) died. The mortality rate did not differ between age groups. Of the patients who died, 25.7% were healthy before the H1N1 virus infection. However, the death rate was significantly higher in patients with malignancy, chronic neurological disease, immunosuppressive therapy, at least 1 pre-existing condition, and respiratory complications. The most common causes of mortality were pneumonia and sepsis. CONCLUSIONS: In Turkey, 2009 H1N1 infection caused high mortality and PICU admission due to severe respiratory illness and complications, especially in children with an underlying condition.

Four ectopia cordis cases surgically managed with different strategies.

Ectopia cordis is a rare congenital disorder in which the heart is partially or completely located outside the chest cavity. In this article, we present four cases of ectopia cordis accompanied by cardiac abnormalities with either thoracic and/or abdominal placed heart, managed with strategies ranging from follow-up without any intervention to complete surgical closure.