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Objective: We aimed to investigate the clinical, and laboratory factors that may effect the left ventricular mass index in children with stage predialysis chronic renal disease. Method: Children with predialysis chronic renal disease consisted the study group. The weight, height, renal diagnosis and follow-up duration were retrieved from medical records. Instantaneous and ambulatory blood pressure measurements were performed. Laboratory tests were performed. The left ventricular mass index was calculated based on the echocardiographic measurements. Frequency of hypertension was calculated, and the correlation between the clinical and laboratory parameters and the left ventricular mass index was investigated. Results: Mean left ventricular mass index was 48.8±12.74 g/m2.7, and left ventricular hypertrophy was detected in 16 (76.1%) patients. Hypertension was detected in 5 (23.8%) patients by instantaneous measurements. However, ambulatory measurements showed systolic and diastolic hypertension in 6 (28.6%) and only diastolic hypertension in 2 (9.5%) patients. A positive and significant correlation was detected between left ventricular mass index and relative body mass index, mean night diastolic blood pressure, night diastolic load and cholesterol level (p<0.05). Also a negative significant correlation between albumin and hemoglobin was detected (p<0.05). Conclusion: The results of our study indicate that in predialysis children; ambulatory blood pressure measurement is superior to instantaneous measurements in prediction of left ventricular hypertrophy, and the left ventricular mass index significantly correlates with hemoglobin, cholesterol and albumin levels in addition to blood pressure parameters. [ABSTRACT FROM AUTHOR]

Aim: Upper respiratory tract obstruction can cause cardiac and pulmonary dysfunction in children, which are largely reversible. We assessed the impact of upper respiratory tract obstruction on cardiac function in children scheduled for surgery for adenoidal and/or tonsillar hypertrophy. Methods: In total, 30 patients who were admitted to the ear nose and throat outpatient clinic and were scheduled for surgery for adenoidal and/or tonsillar hypertrophy and 30 control subjects were included into study. Peak nasal inspiratory flow (PNIF) was measured in all subjects, and echocardiographic and electrocardiographic examinations were performed. Results: The mean age of the patients was 9.43±2.51 years. The mean PNIF was significantly lower in patients (110.0 ± 43.9 L/min) than in the control group (140.7±39.4 L/min; p=0.006), and the mean P wave amplitude was significantly higher in patients than in controls (p<0.001). The mean pulmonary ejection time (ET) was significantly shorter in patients than in controls. Conclusion: Lower mean PNIF values in the patient group indicated increased airway resistance, suggesting that PNIF values below 110 L/min may affect right heart function. PNIF can be used by the pediatrician for screening to guide surgeons in children with adenoidal and/or tonsillar hypertrophy. [ABSTRACT FROM AUTHOR]

Objective: The nasal cavity is anatomically and functionally communicates with eyes, paranasal sinuses, nasopharynx, middle ear and lower airways. Therefore, comorbid diseases such as asthma, chronic sinusitis, adenoid hypertrophy allergic conjunctivitis are common in patients with allergic rhinitis. In our study, we aimed to evaluate the frequency of comorbid diseases, clinical features of patients who referred to our outpatient clinic with allergic rhinitis. Method: The study included children aged between 3-17 years who were diagnosed with moderate to severe allergic rhinitis between September 2015 and March 2016 in our pediatric allergy outpatient clinic. Patient files were retrospectively reviewed; comorbid diseases, family history, eosinophil percentage, total IgE level and allergy test results and the number of drugs used were recorded. In the first month of the treatment, visual analog scale was used to score the symptoms. P<0.05 was considered significant in the statistical analysis. Results: A total of 106 patients (33 female, 73 male) with respective median ages of 7.25 and 5.13 years [range: 3.0-17.0, years] were included in the study. In 84.9% of our patients, skin test was positive, and in 69.8% of the patients the symptoms persisted for more than 2 years. The most common comorbid diseases were asthma (52.8%) and adenoid hypertrophy (30.2%). In our study, the incidence of adenoid hypertrophy was higher and total IgE levels were lower in the skin test-negative group (p=0.019, p=0.027). Conclusion: In our study, the most common comorbid diseases in patients with allergic rhinitis were asthma and adenoid hypertrophy, and in the skin test-negative group adenoid hypertrophy was more frequent. [ABSTRACT FROM AUTHOR]

Introduction: Costello syndrome is probably an autosomal dominant inherited disorder that is characterized by postnatal growth retardation, developmental delay, coarse facies, loose skin, nonprogressive cardiomyopathy and friendly behavior. The aim of this study was to evaluate a variety of clinical findings and cardiac involvement of six patients diagnosed as Costello syndrome. Materials and Methods: Six cases that had a typical features of Costello syndrome admitted to pediatric cardiology department due to cardiac murmur between 2002-2013 years were evaluated. Their complaints, clinical findings, treatments and clinical courses were examined. Results: Three of the patients were male and the mean age of patients was 34±12 months (7 months to 11 years). All of the cases had coarse faces, deep palmar and plantar creases, hypertelorism, epicanthal folds, depressed nasal bridges and cardiac murmur at auscultation. Congenital heart disease was found in all patients. Five patients had pulmonary stenosis and balloon pulmonary valvuloplasty was performed in 3 of these patients. Atrial septal defect was detected in 3 patients and 1 of the patient had hypertrophic cardiomyopathy. Mental retardation was found in 2 patients. Conclusions: Costello syndrome must be keep in mind in all patients with psychomotor retardation, coarse faces, sparse hairs, deep palmar and plantar creases, phenotypical features resembling a storage disease especially in association with pulmonary stenosis and hypertrophic cardiomyopathy. [ABSTRACT FROM AUTHOR]

Myopathy and pseudohypertrophy of muscles accompanied by acquired or congenital hypothyroidism is called Kocher-Debre-Semelaigne syndrome. Kocher-Debre-Seimelaigne syndrome is a rare disorder which is often presented by symmetrical proximal muscle weakness, accompanied by polymyositis, pseudohypertrophy in muscles, muscle rigidity, involuntary muscle contractions, exercise intolerance, myxedema, failure to thrive and neuromuscular findings like cretinism. In thus case report, we present a 5-year-old girl with delayed diagnosis of hypothyroidism and poor compliance to treatment. We suspected Kocher-Debre-Semelaigne syndrome when she developed difficulty in climbing stairs and walking. She had bilateral pseudohypertrophy of gastrocnemius and quadriceps muscles of the lower extremities, elevation in serum creating kinase levels and physical examination findings relevant with myopathy as hypoactive deep tendon reflexes. It was emphasized that this syndrome should not to be overlooked in cases with pseudohypertrophy due to hypothyroidism. [ABSTRACT FROM AUTHOR]

Objective: Respiratory disorders related to sleeping problems are examined in a wide spectrum ranging from simple snoring to obstructive sleep apnea syndrome. Hypertension and the risk for cardiovascular morbidity because of obstructive sleep apnea syndrome have been demonstrated in many previous studies. But, there are limited number of studies demonstrating the influential blood pressure changes during the rime interval from primary snoring to obstructive sleep apnea syndrome. Methods: Twenty children who had no obstructive sleep apnea symptoms with tonsillar hypert- rophy (grade 3-4) and twenty children as control subjects (range: 6-13 years) were included in this study. Healthy children consisting of equal number of boys and girls who were in the same age group with the patient group with no complaints were chosen as a control group. All partici- pants were evaluated by twenty 24-ambulatory blood pressure monitorization, and the data obtained were compared. Results: There was no significant difference between the groups as for average blood pressure measurements obtained during the whole day, daytime and at night Still blood pressure load, blood pressure index and blood pressure variability of both groups did not differ statistically significantly. The number of non-dippers in the patient group was greater than the those in the control group. Although intergroup difference did not reach a statistical significance. Conclusion: This study showed that cardiovascular complications like systemic hypertension did not become manifest in children with tonsillar hypertrophy but without any sign of clinical obs- tructive sleep apnea. Higher number of non-dippers in the patient group, reveal the requirement for larger population-based studies. [ABSTRACT FROM AUTHOR]

Asthma is a chronic lung disorder marked by recurring episodes of airway obstruction accompanied especially by wheezing and coughing and by a sense of constriction in the chest, and triggered by hyperreactivity to various stimuli. Its pathophysiology involves mucus hypersecretion, bronchial hyperresponsiveness, smooth muscle hypertrophy and airway obstruction. Current therapeutic modalities of asthma have not been proved to be successful in reversing the already established chronic changes in airways. Ultimately, an important part of the treatment will aim at immunomodulation and preventing the chronic irreversible changes in asthma. For this reason interventions including dietary changes before the sensitization and remodelling period would be beneficial in terms of preventing those changes. Very long chain fatty acids are the substrates for cyclooxygenase and lipoxygenase and give rise to mediators that often have different biological actions or potencies than those formed from arachidonic acid. This paper provides a short synopsis of the immunological pathology of asthma and discusses potential targets for omega-3 fatty acids. [ABSTRACT FROM AUTHOR]

Aim: In our study we compared the structure and functions of the heart between predialysis and hemodialysis patients and investigated the related factors. Methods: This study included a total of 105 patients (mean age: 18-75 years): 51 on continuous hemodialysis and 54 predialysis subjects followed by the Nephrology Clinic and not yet involved in dialysis program. Laboratory data showing calcium-phosphorus metabolism disorders and transthoracic echocardiography results were evaluated. Results: Serum parathormone, phosphorus, calcium x phosphorus, alkaline phosphatase levels were significantly higher in the dialysis group compared to the predialysis group (p<0.001). No statistical difference was found between the groups with regard to median values of ejection fraction, mean values of left ventricular hypertrophy index and frequencies of diastolic dysfunction, and valve calcifications. Conclusion: Since predialysis group had higher smoking rate (29.6%), low-density lipoprotein cholesterol levels [122 (100-148) mg/dL] and body mass index (27.6±3.8 kg/m2) and had been receiving systemic drugs in different ratios, the two groups were not similar regarding the traditional risk factors; thus, this may be the explanation for finding no statistical difference in echocardiographic parameters. Besides investigating all the factors involved in the pathogenesis of disease, echocardiographic evaluation should be done periodically in both predialysis and dialysis patients. [ABSTRACT FROM AUTHOR]

Long standing hypothyroidism without therapy leads to myopathy which is associated with proximal muscle weakness, fatigue, exertional pain, pseudohypertrophy due to myoedema, diminished deep tendon reflexes, and stiffness in muscles. Kocher-Debre-Semelaigne syndrome is a specific, rare form of hypothyroid myopathy, which causes hypertrophy of muscles. In this article, we emphasized hypothyroid myopathy by reporting a-13 4/12-year old girl with short stature who was diagnosed with hypothyroidism due to thyroid aplasia that caused pseudohypertrophy of muscles. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy, is unexplained reasons and characterized with left ventricular hypertrophy, is an inherited chronic disease of the heart muscle. Hypertrophic cardiomyopathy is seen rarely but it is very important because of genetic transmission and high risk of sudden cardiac death. Echocardiography is basic application for diagnosis left ventricular hypertrophy. We defined a rare case in literature which has massive left ventricular hypertrophy. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot (FT) is the most common cyanotic congenital heart disease. FT is mainly composed of ventricular septal defect, right ventricular outflow tract obstruction, pulmonary stenosis and right ventricular hypertrophy, but also can include dextrapositioned aorta (overriding aorta), additional malformations such as atrial septal defect, and coronary artery anomalies. In the first week of life patients undergo palliative anastamosis between a pulmonary artery and subclavian artery and after they become eligible for major surgery total correction isperformed. With neurological involvement ischemic or venous stroke, syncope and seizures can occur. Generally, clinical presentation is cerebral venous thrombosis. IÝschemic stroke in children is associated with hyperviscosity and microstasis, and in adults with phlebotomy, microcytosis and traditional stroke risc factors such as hypertension, diabetes mellitus and cardiac rhythm disorders. Patients with uncorrected FT rarely survive adulthood. In this study, we present a case without total correction, who lived up to the age of 39 years and presented with arterial stroke instead of expected venous thrombosis, along with stroke mechanisms in the literature. [ABSTRACT FROM AUTHOR]

B-cell prolymphocytic leukemia (B-PLL) comprises 1% of chronic lymphocytic leukemias. CD5 positivity is seen in 1/3rd of cases which generally arise from pre existing CLL. They have longer median survival compared with de novo B-PLL which are commonly CD5 negative and are more aggressive with an older age of presentation. Herewith, we describe a 48-year-old male of de-novo CD5+ B-PLL presenting with minimal lymphadenopathy and massive splenomegaly with 90% atypical lymphoid cells in the peripheral smear and bone marrow. Immunophenotyping was strongly positive for CD5, CD45, CD19, CD22, FMC-7, S-Ig and CD38, moderately positive for CD 11c, weakly positive for CD23 and negative for CD-103 and ZAP 70. The patient responded well to fludarabine and cyclophosphamide and had an uneventful hospital course. Our case illustrates a de-novo B-PLL with aberrant CD5 positivity who had a short duration of illness, younger age at presentation and favourable treatment outcome. [ABSTRACT FROM AUTHOR]