From cyanosis to diagnosis: A case report on right atrial fibroma in a pediatric patient.

A 3-year-old female patient presented with symptoms of cyanosis and intermittent eyelid edema, leading to the discovery of a lobulated mass in the right atrium, obstructing the superior vena cava. Despite the inability to entirely remove the mass due to its origins in the right atrium myocardium and its extension towards the sinoatrial node, successful surgical intervention and subsequent histopathological evaluation identified the mass as a fibroma, and postoperative symptoms were significantly alleviated.

Assessment of Cardiovascular Surgery Requirements in Children and Adolescents Admitted With Chest Pain To A Pediatric Emergency Clinic.

INTRODUCTION: Chest pain in children and adolescents is an important symptom in the pediatric emergency clinic. The aim of this study is to assess the characteristics of chest pain and cardiovascular surgery in the pediatric population. MATERIALS AND METHODS: There were 352 children who presented with chest pain to the pediatric emergency department between December 2007 and February 2017. These children were included in this study, which is a retrospective observational study. RESULTS: Among the 352 patients, six patients (1.7%) underwent cardiovascular surgery. Forty-eight patients (13.6%) were diagnosed with cardiac disease, and the most common cardiac causes were myocarditis and pericarditis. CONCLUSION: Only six patients (1.7%) who were admitted to the emergency department with chest pain needed cardiovascular surgery. The chest pain in children required less surgery, and mortality and morbidity were lower compared with adult cardiac chest pain.

Subclinical anthracycline-induced cardiotoxicity in long-term follow-up of asymptomatic childhood cancer survivors: Assessment by speckle tracking echocardiography.

OBJECTIVE: Survivors of childhood cancer treated with anthracyclines carry the risk for developing late-onset cardiotoxicity. The purpose of this study was to evaluate left ventricular (LV) function in this patient group and compare it with healthy controls by means of conventional and speckle tracking echocardiography (STE) after exposure to chemotherapy. MATERIAL AND METHODS: Conventional and STE were performed in 45 childhood cancer survivors (mean age 11 ± 4.6; 26 male) treated with anthracyclines (median cumulative dosage 240 mg/m2 ; range, 100-460) and compared with age, gender and body surface area matched healthy controls. Follow-up period after chemotherapy was 21.9 ± 17.8 months. Blood samples were taken from survivors and controls to determine brain natriuretic peptide (BNP). RESULTS: Following anthracycline exposure, pediatric cancer survivors had lower longitudinal, radial anteroseptal, and radial anterior strain values compared to controls (P < .05). The calculated global longitudinal and global radial strain values were lower compared to the control group (P < .05). Both groups had normal ejection fraction (EF) and fractional shortening (FS). Brain natriuretic peptide (BNP) levels of both groups were in the normal range. CONCLUSION: Despite normal EF and FS, children exposed to anthracycline therapy may have late-onset subtle changes of LV strain values measured by STE. Whether these changes of strain can predict future risk of developing heart failure needs to be explored in further studies.

Left ventricular mechanics are affected in children with celiac disease: A study based on two-dimensional speckle tracking echocardiography.

BACKGROUND: An association between cardiomyopathy and celiac disease has been reported, but not frequently. We examined the effects on cardiac mechanics in children with celiac disease (CD) by two-dimensional speckle tracking echocardiography (2DSTE). METHODS: Eighty-one children with CD were compared with a control group comprising 51 healthy children by echocardiographic examination. Children with CD who had been on gluten-free diet for more than 6 months were divided into two different groups: group 1 was positive for serum antitissue transglutaminase antibody (n=48), and group 2 was negative for serum antitissue transglutaminase antibody (n=33). Cardiac functions were evaluated in all children using conventional echocardiography, tissue Doppler imaging (TDI), and 2DSTE methods. RESULTS: For children with CD, the mean age and male:female ratio were 10.1±4.0 years and 26/55 (67% female), respectively, which did not differ from the controls. Patients were diagnosed at a mean age of 7.9±4.1 years, and the mean follow-up time was 2.37±2.98 years. Conventional echocardiography and TDI measurements did not differ between groups. The left ventricular longitudinal and radial strains, and strain rate values were significantly lower in patients with CD, particularly in those with positive antitissue transglutaminase antibody compared with the control group. CONCLUSIONS: Our results suggest that 2DSTE is superior to conventional and TDI echocardiography for evaluating subclinical carditis in children with CD.

Effect of high-dose oral cholecalciferol on cardiac mechanics in children with chronic kidney disease.

Cardiovascular factors are an important cause of mortality in chronic kidney disease, and vitamin-D deficiency is common in this patient population. Therefore, we aimed to investigate the effect of oral cholecalciferol on cardiac mechanics in children with chronic kidney disease. A total of 41 children with chronic kidney disease - the patient group - and 24 healthy subjects - the control group - free of any underlying cardiac or renal disease with low 25-hydroxyvitamin-D3 levels were evaluated by conventional tissue Doppler imaging and two-dimensional speckle-tracking echocardiography, both at baseline and following Stoss vitamin-D supplementation. Left ventricular strain and strain rate values were compared between the study groups. Initial longitudinal and radial strain as well as strain rate values of the left ventricle were significantly lower in patients. After vitamin-D supplementation, these improved significantly in patients, whereas no significant change was observed in the control group. Our study showed that, although conventional and tissue Doppler imaging methods could not determine any effect, two-dimensional speckle-tracking echocardiography revealed the favourable effects of high-dose cholecalciferol on cardiac mechanics, implying the importance of vitamin-D supplementation in children with chronic kidney disease.

Effect of cholecalciferol on local arterial stiffness and endothelial dysfunction in children with chronic kidney disease.

BACKGROUND: As cardiovascular factors are the leading cause of mortality in chronic kidney disease (CKD) and as vitamin D deficiency is prevalent in this population, we aimed to examine the effect of oral cholecalciferol on cardiac parameters and biomarkers for endothelial cell activation in children with CKD. METHODS: Forty-one children with CKD and 24 healthy subjects free of any underlying cardiac or renal disease with low 25-hydroxyvitamin D3 (25OHD) levels were evaluated using echocardiography basally and following Stoss vitamin D supplementation. The local vascular stiffness and endothelial dysfunction markers were compared among the groups. RESULTS: Initial flow-mediated dilatation (FMD) measurements were lower and local arterial stiffness was significantly higher in patients. After vitamin D supplementation, these improved significantly in patients, while no significant change was observed for the healthy group. Homocysteine showed inverse correlation with baseline vitamin D level in CKD children and von Willebrand factor emerged as an independent risk factor for FMD impairment. CONCLUSIONS: Our interventional study revealed the favorable effects of high-dose cholecalciferol on cardiovascular and endothelial parameters, implying the importance of vitamin D supplementation in children with CKD.

Early signs that predict later haemodynamically significant patent ductus arteriosus.

OBJECTIVE: Our aim was to determine the optimal cut-off values, sensitivity, specificity, and diagnostic power of 12 echocardiographic parameters on the second day of life to predict subsequent ductal patency. METHODS: We evaluated preterm infants, born at ⩽32 weeks of gestation, starting on their second day of life, and they were evaluated every other day until ductal closure or until there were clinical signs of re-opening. We measured transductal diameter; pulmonary arterial diastolic flow; retrograde aortic diastolic flow; pulsatility index of the left pulmonary artery and descending aorta; left atrium and ventricle/aortic root ratio; left ventricular output; left ventricular flow velocity time integral; mitral early/late diastolic flow; and superior caval vein diameter and flow as well as performed receiver operating curve analysis. RESULTS: Transductal diameter (>1.5 mm); pulmonary arterial diastolic flow (>25.6 cm/second); presence of retrograde aortic diastolic flow; ductal diameter by body weight (>1.07 mm/kg); left pulmonary arterial pulsatility index (⩽0.71); and left ventricle to aortic root ratio (>2.2) displayed high sensitivity and specificity (p0.9). Parameters with moderate sensitivity and specificity were as follows: left atrial to aortic root ratio; left ventricular output; left ventricular flow velocity time integral; and mitral early/late diastolic flow ratio (p0.05) had low diagnostic value. CONCLUSION: Left pulmonary arterial pulsatility index, left ventricle/aortic root ratio, and ductal diameter by body weight are useful adjuncts offering a broader outlook for predicting ductal patency.

Cardiovascular functional and structural changes in children with primary hypertension.

BACKGROUND: Primary hypertension in children is not a benign condition because of end-organ damage. Early investigations of cardiovascular effects rely on vascular structural and functional changes. We aimed to determine presence of early cardiovascular involvement in children with primary hypertension. For this purposes, we investigated functional and morphological changes in vascular system. METHODS: Seventy-five consecutive patients with primary hypertension and 35 healthy children comparable for age and gender were studied. Patients were classified according to the stages of blood pressure. Intima-media thickness of the carotid arteries (cIMT), carotid-femoral pulse wave velocity (PWVcf) and Pulse Wave Augmentation Index (AIx) and Left Ventricular Mass Index (LVMi) were investigated. RESULTS: PWVcf and AIx were higher in patients (5.87±0.87 m/s vs. 5.29±0.67 m/s, P=0.02; 9.41±8.54% vs. 8.36±3.59%, P=0.04, respectively) than in controls. cIMT was evaluated significantly higher in study group than controls (0.46±0.06 mm vs. 0.35±0.12 mm, respectively, P=0.01). The mean LVMi was higher in patients (32.9±11.5 vs. 28.8±1.55, P=0.01). CONCLUSION: Our results demonstrated that vascular findings were more sensitive for target organ changes than the more frequently utilized LVMi in patients with pre-hypertension.

The relationship between cytotoxin-associated gene A positive Helicobacter pylori infection and autoimmune thyroid disease.

OBJECTIVE: The aim of this study was to determine whether there is an association between cagA [cytotoxin-associated gene A] positivity and thyroid autoimmunity and thyroid volume. METHODS: This prospective study included 78 Helicobacter pylori-positive (H. pylori) dyspeptic patients in the study group, and 50 age-, gender-, and body mass index-matched H. pylori-negative dyspeptic patients in the control group. All the controls were evaluated via upper gastrointestinal endoscopic biopsy or breath test, and were found as H. pylori negative. Gastric biopsy specimens were obtained via endoscopy and histological examination was performed for documentation of H. pylori. RESULTS: In all, 55.1% (n = 43) of the H. pylori-positive patients were cagA positive. There was no significant difference in metabolic syndrome parameters or thyroid function test results between the study and control groups. The frequency of anti-TPO and Hashimoto's thyroiditis positivity was significantly higher in the study group than in the control group. Thyroid volume was higher and severe parenchymal heterogeneity was more common in the H. pylori-positive patients. CONCLUSIONS: H. pylori infection might be a risk factor for autoimmune thyroid disease and high thyroid volume in patients diagnosed with histological evaluation. However, cagA positivity has no additional effect on these parameters.

Prevalence of overweight and obesity among patients with congenital and acquired heart disease in Kocaeli, Turkey.

BACKGROUND: Childhood obesity has increased in the last half of the century. The aim of this study was to evaluate the frequency of obesity in the children with congenital or acquired heart disease. METHODS: A total of 1410 children were assessed in this study. The study population was composed of 518 children (289 boys, 229 girls) as control group and 892 children (477 boys, 415 girls) as heart disease group. Patients were grouped into four categories: (I) "Clinic control subjects"; (II) "mild heart disease" that has not been treated with either surgical or catheter intervention; (III) congenital heart disease treated with surgical and/or catheter intervention; and (IV) "arrhythmias". A body mass index ⩾85th percentile was defined as overweight, ⩾95th percentile as obese, and <5th percentile was defined as underweight. RESULTS: We did not detect any association between heart disease and obesity. There was no difference in the rates of overweight, obesity, and underweight between the healthy control subjects and patients with heart disease (8.1%, 13.3%, and 5.0%; 9.0%, 10.7%, and 4.7%, respectively, p=0.145). All subgroups had a similar prevalence of underweight, overweight, and obesity as the healthy control population. Within the heart disease population, the overall prevalence rates for overweight, obesity, and underweight were similar between the boys and girls. CONCLUSION: Obesity is a common problem in children with heart disease, at least in general population. It is an important additional risk factor for long-term cardiovascular morbidity and mortality in children with heart disease. Precautions to prevent obesity should be a part of paediatric cardiologist's examination.

The early cardiovascular changes in pediatric patients with systemic lupus erythematosus.

BACKGROUND: In patients with systemic lupus erythematous (SLE) late-onset deaths are due to morbid cardiovascular changes (CVCs). Inflammatory and immune-mediated mechanisms are involved in promoting atherosclerosis development in SLE that is reflected in both functional and morphological changes in the cardiovascular system. The aim of our study was to determine the presence of these changes in pediatric SLE patients. METHODS: Fifty-one consecutive patients (13 male, 38 female) with SLE and 25 healthy controls were included in the study. Arterial stiffness was assessed by carotid-femoral pulse wave velocity (PWVcf) and augmentation index (AIx), as measured by the Vicorder. Carotid intima-media thickness (cIMT) and the left ventricular mass index (LVMi) were also determined. RESULTS: Patients with SLE, despite equivalent exposure to "traditional" cardiovascular risk factors, presented a higher mean PWVcf and AIx than controls (6.56 ± 1.45 vs. 5.29 ± 0.67 m/s, P =0.01 and 14.7 ± 8.1 vs. 9.36 ± 3.59 %, P = 0.02, respectively). SLE patients had greater values of cIMT and LVMi than controls (0.54 ± 0.06 vs. 0.35 ± 0.12 mm, P = 0.00 and 32.4 ± 10.8 vs 28.8 ± 1.5, P = 0.01, respectively). Nine patients had left ventricular hypertrophy (LVMi >38 g/m(2.7)). There was no significant difference in PWV, AIx, cIMT and LVMi values between presence of hypertension or nephritis within SLE patients. We found significant correlations between all parameters and activity scores. CONCLUSIONS: Our results demonstrate that functional and morphological CVCs are independent of traditional risk factors in pediatric SLE patients and correlate with SLE disease activity scores in the early disease stages.

Adding pineapple juice to a polyethylene glycol-based bowel cleansing regime improved the quality of colon cleaning.

BACKGROUND/AIMS: An unsuccessful colonoscopy procedure is often related to inadequate bowel cleansing. It is difficult for patients to finish the whole 4 liters of polyethylene glycol-electrolyte lavage (PEG-EL) because of its salty taste and the large quantity. Pineapple juice has been shown to be an effective agent in the dissolution of undigested food in the stomach. This study assessed the effectiveness of both 2 and 4 liters of PEG-EL in precolonoscopic bowel cleansing and the quality of colonoscopic cleaning by adding 1 liter of pineapple juice to a reduced-volume PEG-based regime. METHODS: The patients were chosen from those undergoing a colonoscopic procedure. A total of 126 patients were randomized into 3 groups receiving 3 different PEG-EL (Golytely®) regimes, i.e. 4 liters of PEG-EL (group 1, n = 44), 2 liters of PEG-EL (group 2, n = 39) or 2 liters of PEG-EL with 1 liter of pineapple juice (Dimes® 100%; group 3, n = 43). RESULTS: Both the 4- and 2-liter PEG-EL regimes resulted in similar bowel cleansing scores in all parts of the colonic segments. However, adding 1 liter of pineapple juice to the reduced-volume PEG-EL regime improved the quality of the cleansing on the right side of the colon and in the transverse colon. Adequate bowel cleansing was achieved in 68.1% of the patients in group 1, 63.9% in group 2 and 80% in group 3 (the lowest score in one of the segments). On the other hand, the tolerability of the regimes was similar in all 3 groups (p = 0.509). CONCLUSIONS: Reduced PEG-EL (2 rather than 4 liters) may be sufficient for precolonoscopic bowel cleansing in the Turkish population. Administration of pineapple juice in the reduced-dose preparation regime may improve the quality of the bowel cleaning.

Cerebellar metastasis of colon cancer at diagnosis: A very rare case.

Colorectal cancer (CRC) is one of the leading causes of cancer deaths worldwide. Besides, brain metastasis from CRC is relatively rare. A 68-year-old male referred to the emergency clinic with headache, vertigo, nausea, and vomiting. A cerebellar mass lesion was determined and totally excised. Pathology revealed a metastatic adenocarcinoma. Colonoscopic biopsy obtained from the lesion in the colon was reported as adenocarcinoma. A cerebellar recurrent lesion occurred, and radiotherapy was initiated. After radiotherapy, FOLFOX-bevacizumab combination regimen was initiated as the first-line treatment of the patient with metastatic colon cancer. Significant improvements in the clinical outcomes of CRC were achieved in recent years. However, brain metastasis from CRC has a poor prognosis. We aimed to report a rare presentation of CRC with synchronous brain metastasis.

Biruni Index as a Novel Diagnostic Tool for the Early Prediction of Mortality in Critical Patients With COVID-19: A Cohort Study.

BACKGROUND: The aim of this study was to find out the potential risk factors associated with mortality in severe coronavirus disease 2019 (COVID-19) patients hospitalized due to viral bronchopneumonia, and to establish a novel COVID-19 mortality index for daily use. METHODS: The study included 431 quantitative real-time polymerase chain reaction (qRT-PCR)-confirmed COVID-19-positive patients admitted to the intensive care unit in a tertiary care hospital. Patients were divided into training and validation cohorts at random (n= 285 and n= 130, respectively). Biruni Index was developed by multivariate logistic regression analysis for predicting COVID-19-related mortality. RESULTS: In univariate logistic regression analysis, age, systolic and diastolic blood pressures, respiratory and pulse rates per minute, D-dimer, pH, urea, ferritin, and lactate dehydrogenase levels at first admission were statistically significant factors for the prediction of mortality in the training cohort. By using multivariate logistic regression analysis, all of these statistically significant parameters were used to produce Biruni Index. Statistically significant differences in Biruni Index were observed between ex and non-ex groups in both training and validation cohorts (P < 0.001 for both comparisons). Areas under receiver operating characteristic (ROC) curve for Biruni Index were 0.901 (95CI%: 0.864-0.938, P < 0.001) and 0.860 (95CI%: 0.795-0.926, P < 0.001) in training and validation cohorts, respectively. CONCLUSION: As a pioneering clinical study, Biruni Index may be a useful diagnostic tool for clinicians to predict the mortality in critically ill patients with COVID-19 hospitalized due to severe viral bronchopneumonia. However, Biruni Index should be validated with larger series of multicenter prospective clinical studies.

Comprehensive Genetic Analysis of RASopathy in the Era of Next-Generation Sequencing and Definition of a Novel Likely Pathogenic >KRAS Variation.

Introduction: Germline pathogenic variations of the genes encoding the components of the Ras-MAPK pathway are found to be responsible for RASopathies, a clinically and genetically heterogeneous group of diseases. In this study, we aimed to present the results of patients genetically investigated for RASopathy-related mutations in our Genetic Diagnosis Center. Methods: The results of 51 unrelated probands with RASopathy and 4 affected relatives (31 male, 24 female; mean age: 9.327 ± 8.214) were included in this study. Mutation screening was performed on DNA samples from peripheral blood of the patients either by Sanger sequencing of PTPN11 hotspot regions (10/51 probands), or by a targeted amplicon next-generation sequencing panel (41/51 probands) covering the exonic regions of BRAF, CBL, HRAS, KRAS, LZTR1, MAP2K1, MAP2K2, NF1, NRAS, PTPN11, RAF1, RASA2, RIT1, SHOC2, SOS1, SOS2, SPRED1, and KAT6B genes. Results: Pathogenic/likely pathogenic variations found in 22 out of 51 probands (43.13%) and their 4 affected family members were located in PTPN11, BRAF, KRAS, NF1, RAF1, SOS1, and SHOC2 genes. The c.148A>C (p.Thr50Pro) variation in the KRAS gene was a novel variant detected in a sibling in our patient cohort. We found supportive evidence for the pathogenicity of the NF1 gene c.5606G>T (p.Gly1869Val) variation which we defined in an affected boy who inherited the mutation from his affected father. Conclusion: Although PTPN11 is the most frequently mutated gene in our patient cohort, as in most previous reports, different mutation distribution among the other genes studied motivates the use of a next-generation sequencing gene panel including the possible responsible genes.

Is there myocardial involvement in children with long-term follow-up for Kawasaki disease? A study based on two-dimensional speckle tracking echocardiography.

OBJECTIVE: This study aimed to determine the possibility of subclinical myocardial dysfunction detected by strain echocardiography in the late period of children with Kawasaki disease. MATERIAL AND METHODS: The study enrolled 30 patients with Kawasaki disease with a follow-up period of at least 12 months and 30 healthy age- and gender-matched children. During the follow-up period, standard echocardiography, pulsed and tissue Doppler, and strain echocardiography were recorded for both groups. RESULTS: The mean age at the time of the diagnosis was 2.6±2.3 years (2 months-11 years). The mean follow-up period after the diagnosis was 3.55±2.20 years. Conventional echocardiography, M mode, pulsed and tissue Doppler values, and myocard performance index did not reveal significant differences. Left ventricle strain and strain rate parameters obtained by apical four-, three-, and two-chamber views did not show statistical differences between patients and controls. There was a positive correlation between the duration of follow-up and global four- and three-chamber longitudinal strain and global longitudinal strain values (r=0.465, p=0.010; r=0.414, p=0.023; r=0.492, p=0.006, respectively), whereas global radial strain showed negative correlation (r=-0.517, p=0.003). CONCLUSION: The analysis of systolic strain and strain rate did not detect a subclinical myocardial dysfunction in the long-term follow-up of Kawasaki disease. However, strain values showed variability with the follow-up periods, which indicates that Kawasaki disease might cause left ventricular dysfunction in the later phases. Therefore, a follow-up of children with a diagnosis of Kawasaki disease is of capital importance.

Two Novel Pathogenic FBN1 Variations and Their Phenotypic Relationship of Marfan Syndrome.

Marfan syndrome is an autosomal dominant disease affecting connective tissue involving the ocular, skeletal systems with a prevalence of 1/5,000 to 1/10,000 cases. Especially cardiovascular system disorders (aortic root dilatation and enlargement of the pulmonary artery) may be life-threatening. We report here the genetic analysis results of three unrelated cases clinically diagnosed as Marfan syndrome. Deoxyribonucleic acid (DNA) was isolated from EDTA (ethylenediaminetetraacetic acid)-blood samples of the patients. A next-generation sequencing panel containing 15 genes including FBN1 was used to determine the underlying pathogenic variants of Marfan syndrome. Three different variations, NM_000138.4( FBN1 ):c.229G > A(p.Gly77Arg), NM_000138.4( FBN1 ):c.165-2A > G (novel), NM_000138.4( FBN1 ):c.399delC (p.Cys134ValfsTer8) (novel) were determined in our three cases referred with a prediagnosis of Marfan syndrome. Our study has confirmed the utility of molecular testing in Marfan syndrome to support clinical diagnosis. With an accurate diagnosis and genetic counseling for prognosis of patients and family testing, the prenatal diagnosis will be possible.

A case report with type II pleuropulmonary blastoma: successful treatment with surgery and chemotherapy.

Pleuropulmonary blastoma (PPB) is a very rare intrathoracic malignancy in childhood. It is an aggressive embryonal or blastemic neoplasm usually occurring in children younger than five years of age. PPB is treated with aggressive multimodal therapies consisting of surgery and chemotherapy. We present a case with PPB type II successfully treated with complete surgical resection following neoadjuvant chemotherapy. She has been free of disease for 33 months of follow-up. Complete surgical resection of the tumor at the time of diagnosis is the cornerstone of PPB management, but in the majority of patients, initial surgery is incomplete because a large tumor may involve vital structures. For this reason, patients with initially unresectable tumors should be treated with neoadjuvant chemotherapy to reduce the lesion to the point that it becomes resectable.

A Study of Mortality in Cardiac Patients in a Pediatric Intensive Care Unit.

OBJECTIVE: One of the major causes of mortality in the pediatric intensive care unit (PICU) is heart disease. This study aimed to determine the causes of mortality in children with pre-existing cardiac abnormalities who were admitted into the PICU. METHODS: Data were collected through patient profile assessment and outcome and heart diseases affecting prognosis were analyzed. Medical records of children were reviewed retrospectively. The updated Pediatric Index of Mortality 2 (PIM2) scores were used. Exploratory data analysis was performed using descriptive measures. Kolmogorov-Smirnov tests were used to test the normality of data distribution. RESULTS: Out of 566 admissions into PICU, 76 (13.4%) had cardiac abnormalities. Median and range of PICU stay were 5.50 and 417.88 days. The mean PIM2 score on admission was found to be 31.05. The most common admission was due to atrioventricular septal defect (AVSD) (15.7%), cardiomyopathy (13.1%), ventricular septal defect (VSD) (11.8%), tetralogy of Fallot (10.5%) and others (48.9%). There were multiple cardiac anomalies in 3.9% of patients. The most important cause of cardiac mortality in PICU was septic shock (26.0%) followed by cardiogenic shock (20.6%), and cardiac failure (13.7%). The nosocomial infection rate of cardiac patients in PICU was 10.5%. CONCLUSIONS: Our study reconfirmed that the PIM2 score is a good indicator of cardiac diseases. Infections, nosocomial infections, pneumonia, and septic shock were the leading causes of mortality in cardiac patients. Better infection control in the PICU may have a significant impact on decreasing mortality rates.

Relation between microalbuminuria and gene mutations in familial Mediterranean fever.

We aimed to investigate the urinary microalbumin level, which is a sensitive marker of glomerular function for establishing probable renal involvement in early stages of the disease in patients with familial Mediterranean fever (FMF), and to determine the relation between gene mutations of these cases and urinary microalbumin levels. Fifty patients with FMF who were admitted to our department and had been followed up in the pediatric rheumatology outpatient clinic for five years were included in the study. Diagnosis was based on Tel-Hashomer criteria. Gene mutations (M694V, V726A, M680I) and acute phase reactants were determined as supportive findings. Routine renal function tests with 24 hour urinary microalbumin levels and urinary microalbumin/creatinine ratios were evaluated. There was a statistically significant difference between the study and control groups in terms of microalbumin/creatinine ratios, whereas no difference was observed with respect to the other parameters. Comparison of subgroups (gene mutations) in terms of all parameters (age, age at diagnosis, duration of delay in treatment, glomerular filtration rate, tubular reabsorption of phosphorus, and microalbumin/creatinine ratios) showed no difference. We suggest measurement of urinary microalbumin levels at regular intervals in order to establish renal injury early and decrease related complications.