Gülendam Koçak

Brain MR spectroscopy in children with a history of rheumatic fever with a special emphasis on neuropsychiatric complications

PURPOSE To investigate whether there are metabolite changes in basal ganglia of children with complete healing of rheumatic fever (RF), history of Syndenham chorea (SC) and obsessive compulsive-tic disorder (OCTD) developed after RF when compared with healthy controls and each other. MATERIAL AND METHODS A total of 49 children with history of RF and 31 healthy controls were included into the study. All patients and control group underwent a detailed neuropsychiatric evaluation. Children with the history of RF were classified into three groups as; group 1: with history of RF without neuropsychiatric complications (NCRF), group 2: only with history of SC (HSC), group 3: with HSC and OCTD (OCTD). After MR imaging, single voxel MR spectroscopy was performed in all subjects. Voxels (15 x 15 x 15 mm) were placed in basal ganglia. N-acetyl aspartate (NAA)/creatin (Cr), and choline (Cho)/Cr ratios were calculated. RESULTS OCTD were detected in 13 children with HSC. NAA/Cr ratio was found to be decreased in these children when compared with NCRF (n:29), HSC without OCTD (n:7) and control groups (n:31). No significant difference was found in metabolite ratios of children with HSC without OCTD when compared with NCRF and control groups. There were no significant differences in Cho/Cr ratio between patient and control groups. CONCLUSION Although MR imaging findings was normal, MR spectroscopy findings (decreased NAA/Cr ratio) in our study support the neuronal loss in basal ganglia of children with OCTD and could indicate the development of permanent damage.

Association of cardiac changes with serum adiponectin and resistin levels in obese and overweight children.

Objectives To investigate serum adiponectin and resistin levels in childhood obesity and their relationship with cardiac changes and insulin resistance. Methods Seventy-one obese and 24 overweight children and 40 healthy children and adolescents were selected for the study. Height and weight measurements, BMI values and BMI SD score values were obtained for each individual. After blood pressure measurement, left ventricular wall thickness, left ventricular mass, stroke volume, cardiac output, systolic and diastolic functions of the left ventricle were measured using an M-mode, two dimensional color-coded echocardiography device. Blood samples of the individuals were obtained for fasting blood sugar, total blood cholesterol, triglyceride, low-density lipoprotein (LDL) cholesterol, very low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, insulin, C-peptide, adiponectin and resistin values. Results Cholesterol and LDL values, homeostasis model assessment of insulin resistance, fasting insulin and fasting C-peptide values of the obese and overweight groups were higher (P < 0.01). Adiponectin level (P < 0.001) and resistin level (P < 0.05) of the obese and overweight groups were lower than those of the control group (P < 0.05). Echocardiographic evaluation showed diastolic dysfunction in addition to increased left ventricular wall thickness and left ventricle mass values in the obese and overweight children. We also detected a significant positive correlation among left ventricular mass, interventricular septum systolic diameter and resistin in obese children. Among the factors, resistin level was determined as an independent predictor of left ventricular mass in obese children. Conclusion In this study, even in asymptomatic obese and overweight children, cardiac structural and functional changes, such as increased left ventricular mass and diastolic dysfunction, were demonstrated. Although decreased adiponectin level was not related to cardiac changes, it was shown that decreased serum resistin levels in the obese cases lead to left ventricle hypertrophy.

Brucella myocarditis in a 3-month-old: probable transplacental transmission

Abstract A 3-month-old girl presented with myocarditis owing to brucellosis. Her mother had been diagnosed with brucellosis at 28 weeks gestation but did not receive treatment until after delivery. The infant had intrauterine retardation and had gained little weight since birth. It is considered likely that the brucellosis was transmitted transplacentally.

Prevalence and Outcome of Congenital Heart Disease in Patients With Neural Tube Defect

A prospective clinical study was designed to establish the risk factors, the prevalence, and the progress of congenital heart defects in children with neural tube defects. Study included 90 children with a mean age of 13.5 ± 30.4 months. There were 53 (59%) patients with spina bifida occulta and 37 (41%) patients with spina bifida aperta. The overall prevalence of congenital heart disease was 27.8% (40.5% in spina bifida aperta and 18.9% in spina bifida occulta; P = .024). There was no statistically significant difference for maternal age, usage of periconceptional folate, and maternal diabetes between the patient and control groups. The authors conclude that congenital heart defects are more common than reported in neural tube defects, and screening echocardiograms are warranted. This should be kept in mind especially in patients requiring minor or major surgical procedures. Furthermore, routine obstetric examination and therefore the use of periconceptional folic acid during pregnancy is still lacking in our country.

Prenatal Diagnosis of a Patient With Tetralogy of Fallot and an Absent Pulmonary Valve

A 34-week-gestation fetus of a gravid 3 mother 29 years of age was referred to our hospital due to an abnormal vascular image on obstetric ultrasonographic examination. The family history of congenital heart disease was negative. The mother had been using valproate due to epilepsy. Fetal echocardiography showed aneurysm of the pulmonary artery and rudimentary pulmonary valve and pulmonary regurgitation (Figs. 1, 2, movie 1). Tetralogy of Fallot and an absent pulmonary valve was diagnosed (Figs. 3, 4, 5). At birth, the patient was symptomatic with severe respiratory failure. Postnatal echocardiography confirmed the diagnosis of dextroversion due to right lung atelectasia in addition to tetralogy of Fallot and an absent pulmonary valve. The aneurysm of the pulmonary artery was measured as 3.5 cm. Clinical examination showed no extra cardiac malformations or dysmorphic findings. Genetic testing for 22q11.2 deletion was not possible. The chest tomography showed a right lung compression due to an aneurysmatically dilated pulmonary artery. The patient’s condition deteriorated while on the ventilator due to bilateral pneumothorax. He died on the day 6 after the delivery due to severe respiratory distress while awaiting cardiac surgery. Tetralogy of Fallot and an absent pulmonary valve are a rare congenital malformation characterized by an absent or rudimentary valve, typically both stenotic and regurgitant,

Possible association between fluconazole administration and acute hyperkalemia in a critically ill cyanotic infant

Fluconazole, an azole antifungal agent, inhibits the fungal cytochrome P450 enzyme. Although in vitro data show that fluconazole is not a potent inhibitor of mammalian P450 enzymes, hyperkalemia secondary to adrenal insufficiency associated with fluconazole has been described rarely [1–3]. However, life-threatening severe hyperkalemia related to administration of intravenous fluconazole without adrenal suppression has not been reported before. A 2.5-month-old male infant diagnosed with tetralogy of Fallot with pulmonary atresia and multiple aorto-pulmonary collateral vessels was transferred to a pediatric intensive care unit due to septicemia. On physical examination, he was tachypneic and tachycardic with decreased oxygen saturation (62%) and a temperature of 38.8°C. Laboratory studies included hemoglobin, 12.4 g/dl; white blood count, 23.2 × 109/l; C-reactive protein, 38.3 mg/l (0–3.2); and serum potassium, 3.8 mEq/l (3.5–5.1 mEq/l). Other biochemical parameters and renal function tests were in the normal range. Blood cultures were positive for coagulase negative Staphylococcus. He was started on teicoplanin and meropenem intravenously. As fever persisted after 48 h with new infiltrates on chest radiography, and tracheal colonization with Candida spp., fluconazole 5 mg/kg intravenously once a day was given. After 24 h of treatment with fluconazole, bradycardia developed in the patient. Electrocardiography showed a prolonged PR interval and widening of the QRS complex with bradycardia (Figure 1). His simultaneous laboratory testing demonstrated a serum potassium level of 8 mEq/l. He was treated with repetitive administration of sodium bicarbonate, insulin/glucose, as well as calcium gluconate. Repeat blood results showed potassium 11 mEq/l. The results of renal and liver function tests and the levels of other electrolytes were within normal limits. There were no signs of rhabdomyolysis, hemolysis or severe acidosis. No medication errors could be identified. Infusion of fluconazole was stopped, and he was treated with repetitive administration of bicarbonate, insulin/glucose, and albuterol by nebulizer. In the meantime peritoneal dialysis was administered in the patient. The serum potassium level gradually returned to a normal level (4 mEq/l), and the findings of ECG abnormalities were improved. Figure 1 ECG shows widened QRS and prolonged PR interval Prophylaxis with fluconazole has been shown to reduce the occurrence of candidiasis in intensive care units [4, 5]. Even though fluconazole in standard doses is thought to be safe, there are rare reports of adrenal suppression associated with fluconazole administration [2, 3]. In our patient, severe hyperkalemia was observed after 24 h of treatment with fluconazole. Our patients renal functions remained normal and evidently did not impair potassium excretion. No evidence of rhabdomyolysis, severe acidosis, hemolysis or adrenal suppression was detected. Although the causative mechanism is unclear, severe hyperkalemia was associated with fluconazole in our patient. All other possible causes of hyperkalemia were excluded. Fluconazole may have unknown life-threatening side-effects, despite having been tested prior to its clinical use. To our knowledge, this is the first report of a life-threatening complication of fluconazole without adrenal insufficiency. We speculate that because of chronic hypoxia of our patient due to his cyanotic heart disease, the drug side effect may have been triggered, and it may be prudent to monitor electrolytes especially in critically ill cyanotic patients.

Combined effect of aerosolized iloprost and oxygen on assessment of pulmonary vasoreactivity in children with pulmonary hypertension.

OBJECTIVE The evaluation of pulmonary vascular reactivity plays a significant role in the management of patients with pulmonary hypertension. Inhaled nitric oxide in combination with oxygen (O2) has become widely used as an agent for pulmonary vasodilator testing. However, inhaled nitric oxide is not available in many developing countries. Recently, aerosolized iloprost was suggested as an alternative to nitric oxide for this purpose. In the present study, aerosolized iloprost was used together with O2 in the pulmonary vasoreactivity test of children with severe pulmonary hypertension. Thus, the synergistic effect of both vasodilators was utilized without extending the duration of cardiac catheterization. METHODS The prospective cohort study registered a total of 16 children with severe pulmonary hypertension whose median age was 4.5 years. Hemodynamic parameters were quantified before and after the vasoreactivity test. Increased left-to-right shunt, pulmonary vascular resistance of <6 Woods units (WU)/m(2) and a pulmonary-systemic resistance ratio of <0.3, as well as a decrease >10% in the pulmonary vascular resistance and pulmonary-systemic vascular resistance ratio after the vasoreactivity test were accepted as a positive response. The data were analyzed using Wilcoxon signed-rank and the Mann-Whitney U tests. RESULTS Eleven children gave a positive response to the vasoreactivity test, while 5 children did not respond. Pulmonary vascular resistance dropped from 9.98 ± 1.39 WU/m(2) to 5.08 ± 1.05 WU/m(2) (p=0.013) and the pulmonary-systemic vascular resistance ratio fell from 0.68 ± 0.08 to 0.32 ± 0.05 (p=0.003) in the children who were responsive. No side effects were observed related to iloprost administration. CONCLUSION Administration of inhaled iloprost in combination with O2 for pulmonary vasoreactivity testing can be useful for correctly identifying pulmonary vasoreactivity without extending the duration of cardiac catheterization.

Presence of a D8/17 B lymphocyte marker and HLA-DR subgroups in patients with rheumatic heart disease.

OBJECTIVE The aim of our study was to investigate the association of HLA antigens and a non-HLA protein D8/17 with rheumatic heart disease and its pattern of cardiac involvement. METHODS This cross- sectional observational study included 35 children and 12 adult patients who have rheumatic heart disease and 35 healthy children and 12 healthy adult controls. After physical examination, all patients and control group members were evaluated with 2D and color-coded echocardiography. B- lymphocyte D8/17 expression was tested by a flow cytometry assay. HLA genotyping was performed using polymerase chain reaction sequence-specific primers. In statistical analysis, Chi-square, unpaired t and Mann-Whitney U tests were used for comparison groups. RESULTS The percentage of the D8/17-expressing B lymphocytes of the patient group was significantly higher than of the control group (77.3±15.6% vs. 67.7±20.0%, p=0.013). When compared with the control group, the HLA DRB5 (38.6% vs. 13.6%, p=0.007) and HLA DRB1*15 (31.8% vs. 9.0%, p=0.008) expression levels of the patient group were significantly higher and the DRB4 expression of the patient group was significantly lower (29.5% vs. 50.0%, p=0.049). CONCLUSION Our findings support the association between HLA Class 2 subgroups and rheumatic heart disease, and an association between D8/17 expression and rheumatic heart disease. Further studies including higher number of patients and control group members should be performed for the confirmation of our results.