Role of Hemodialysis in the Management of Cyanide Intoxication From Apricot Kernels in a 3-Year-Old Child.

Cyanide (CN) is one among the most potent and rapidly acting lethal poisons, and it may cause death unless immediately diagnosed and treated. We report an unusual case of pediatric CN poisoning after ingestion of apricot kernels containing amygdalin, who survived with antidotal therapy and hemodialysis. A 3-year-old girl presented with respiratory distress and coma following tonic-clonic convulsions after ingestion of 3 apricot kernels. She had severe metabolic acidosis (pH 6.91, bicarbonate [HCO3] 5.6 mEq/L, base excess -26.0 mEq/L). Her blood CN level was measured 3.15 mg/L, 3 hours after ingestion. Hydroxocobalamin could not be administered immediately because it had to be brought from a medical center 4 hours apart. Therefore, a 3-hour hemodialysis session was carried out, following which she showed some clinical improvement. In addition, when hydroxocobalamin was obtained, it was then administered. During follow-up, she was completely asymptomatic with blood pressure, and other hemodynamic parameters normalized. This case presents hemodialysis as a way to correct metabolic derangements from CN poisoning and suggests that it may have a role in select cases of pediatric CN poisoning, especially when CN-scavenging antidotes may be unavailable.

The report of sequence analysis on familial Mediterranean fever gene (MEFV) in South-eastern Mediterranean region (Kahramanmaras) of Turkey.

Familial Mediterranean fever (FMF) is defined as an inherited and autosomal recessive disease. Many researches have been done about this subject, and we believe that it should be necessary to focus on phenotype-genotype correlation, especially novel mutation types. We aim to announce the results of FMF sequence analysis in Kahramanmaras/Turkey. The number of participants is 380 males and 451 females who clinically diagnosed as FMF subjects of different age groups. Genomic sequences of exons 2 and 10 and in some cases exon 3 of the MEFV gene were scanned for mutations by sequence analyzer. The most common mutation identified in 230 (57.07 %) patients is heterozygous. The frequencies of mutation types in heterozygous subjects are R202Q (39.13 %), E148Q (18.70 %), M680I (16.52 %), M694V (13.91 %), and V726A (4.78 %), respectively. The most striking point among the compound heterozygous subjects is R202Q/M694V mutation type found at the highest rate (32 subjects). Fever and peritonitis are the most frequent signs of homozygous M694V and combine heterozygous mutations. Interestingly, the rate of homozygous mutation types (M694V/M694V+ R202Q/R202Q) is 96.70 % among all compound homozygous mutation types. The most frequent rate of homozygous patients is M680I mutation types (68.42 % in all homozygous mutation types). Two novel mutations were found in this study: N206K (p.Asn206Lys) and S208T (p.Ser208Tyr). Our findings in this study on the FMF sequence analysis are different from the results obtained from the other regions of Turkey.

Evaluation of angiogenesis with vascular endothelial growth factor in patients with thalassemia major.

BACKGROUND: Thalassemia major (TM) is an important cause of severe anemia that necessitates regular blood transfusion to prevent the profound weakness and cardiac decompensation caused by the anemia. However, iron overloading is an inevitable consequence of prolonged transfusion therapy. In addition, extramedullary hematopoiesis and hemosiderosis cause spleen, liver and marrow enlargement. In recent years the role of angiogenesis has been investigated in physiological and pathological conditions. However, it is known that angiogenetic factors, especially the vascular endothelial growth factor (VEGF), cause differentiation of the hemangioblast. METHODS: The effect of angiogenesis hasn't been investigated in TM patients yet, and in this study, angiogenesis was researched in 43 thalassemic patients by serum VEGF measurement. RESULTS: VEGF levels were not affected by hemoglobin levels, ferritin levels, or chelation type (P > 0.05). However, VEGF was positively affected by chelation starting age and negatively affected by yearly transfusion requirement of TM patients (P < 0.05). In addition, VEGF of patients who underwent splenectomy were higher than those who didn't undergo splenectomy (P < 0.05). CONCLUSION: Early chelating age will negatively influence the VEGF level, which increases angiogenesis, however, early starting transfusion age and regular blood transfusion will positively influence the VEGF level, which decreases angiogenesis in thalassemic patients.

Cardiac hydatid disease: CT and MRI findings.

OBJECTIVE: The purpose of this article is to review the CT and MRI findings of cardiac hydatid disease. CONCLUSIONS: CT and MRI are helpful for localizing and defining the morphologic features of hydatid cysts. Specific signs include calcification of the cyst wall, presence of daughter cysts, and membrane detachment. CT best shows wall calcification, whereas MRI depicts the exact anatomic location and nature of the internal and external structures.

Oxidative stress and antioxidant status in neonatal hyperbilirubinemia.

OBJECTIVE: To investigate the possible relationships between plasma bilirubin levels and concentrations of nitric oxide (NO), malondialdehyde (MDA), and erythrocyte antioxidant enzyme activities in newborn infants with hyperbilirubinemia. METHODS: Thirty term (gestational age > or = 37 weeks) newborn infants with indirect hyperbilirubinemia aged less than 10 days were prospectively recruited in the Kahramanmaras Sutcu Imam University Neonatal Unit, Kahramanmaras, Turkey, between January and July 2007. Thirty randomly selected healthy newborns who had similar age and without clinical jaundice comprised the control group. Erythrocyte catalase, superoxide dismutase, glutathione peroxidase (GPx), glucose-6-phosphate dehydrogenase, and plasma MDA and NO concentrations were measured. RESULTS: Both MDA and NO concentrations were higher in the study group than the controls (p=0.000). The mean activities of erythrocyte antioxidant enzymes were found to be lower in the study group compared with the controls (p=0.000). Furthermore, plasma bilirubin showed significant negative correlations with antioxidant enzyme activities but positive correlations with MDA and NO. CONCLUSION: In this sample, infants with significant hyperbilirubinemia had elevated oxidative stress and disturbed antioxidant enzyme activity. Since these states have been shown to cause cellular injury in neonatal patients with indirect hyperbilirubinemia, such patients should be followed-up and undergo therapy to prevent the harmful effects of hyperbilirubinemia. Further studies are needed to investigate possible benefits of antioxidants in hyperbilirubinemia.

Fish oil supplementation improves left ventricular function in children with idiopathic dilated cardiomyopathy.

Fish oil has a cardioprotective effect in adults with ischemic heart disease. The authors examined the effects of fish oil in children with idiopathic dilated cardiomyopathy (DCM). Eighteen DCM patients (group I) and 12 healthy children (group III) were given fish oil (10 mL/d). Their cardiac findings were compared with those of 11 patients with DCM who did not receive fish oil (group II). After 6.62+/-1.70 months, left ventricular ejection fraction had increased by 8.44%+/-3.80% (P<.05), in group I; 2.48%+/-3.85% (not statistically significant) in group II; and 0.84%+/-2.34% (not statistically significant) in group III. Left ventricular internal diastolic diameter (mm) was reduced by 4.36+/-4.86 (P=.001) in group I and 1.92+/-5.37 (P=.263) in group II, but increased by 0.22+/-2.54 (not statistically significant) in group III. The results suggest that fish oil leads to accelerated improvement of left ventricular function. The authors believe that if these results are confirmed in larger studies, fish oil should be added to the standard anticongestive therapy of children with DCM.

Recurrent hemiparesis due to anterior mitral leaflet myxomas.

Strokes, cardiac or noncardiac, generally affect the elderly and only occasionally occur in children. A tendency to stroke occurs in cyanotic congenital heart disease; however, this report describes two female patients, aged 6 years and 4 years, who had no cyanotic disease. The first patient was admitted to the hospital after the first stroke but suffered a second one after admission. Her initial cardiac examination was normal. The other patient was admitted after a second right-sided hemiparesis. A mild pansystolic ejection murmur was observed at the lower left sternal border on auscultation; slight late diastolic murmurs with opening snap were found in the mitral valve area, and the second heart sound was increased. Transthoracic echocardiography revealed a mass in the anterior mitral valve in both patients. These masses were completely removed surgically and pathology revealed myxomas. Hemiparesis completely resolved after surgical and medical treatment. The clinical manifestations of cardiac tumors primarily depend on their number, size, location, and histology. Strokes and anterior mitral valve masses are extremely rare in children. In the two cases reported here, anterior mitral valve myxomas caused systemic embolization as a result of their location. Echocardiography should be performed early in patients presenting with recurrent strokes to exclude such pathology.

Different clinical presentations of Naxos disease and Carvajal syndrome: Case series from a single tertiary center and review of the literature.

OBJECTIVE: Naxos disease is an autosomal recessive, inherited, cardiocutaneous disorder, characterized by arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. Carvajal syndrome is characterized by palmoplantar keratoderma, curly hair, dilated cardiomyopathy, especially on the left ventricle side, and early morbidity. The aim of this study was to evaluate the cutaneous and cardiac findings and genotype-phenotype relationship of six patients diagnosed with Naxos/Carvajal syndrome. METHODS: A retrospective review of six cases diagnosed with Naxos/Carvajal syndrome at our institution from 2002 to 2012 was performed. Demographic data; presenting complaints; cutaneous and cardiac findings; electrocardiography, echocardiography, and genetic analysis results; and treatment data were obtained from patient files. RESULTS: The patient group was composed of 4 males and 2 females, ranging from 1.5 to 13 years, with a mean age 6.4 years. Typical cutaneous and hair findings were present in all patients. Two cases presented with ventricular tachycardia attack, and 2 cases presented with severe heart failure. Two cases had only cutaneous findings without cardiac involvement at diagnosis. An implantable cardioverter-defibrillator was implanted in one case due to ongoing recurrent ventricular tachycardia attacks despite various antiarrhythmic treatments. Three of the 6 patients died during the follow-up. CONCLUSION: For cases with woolly hair and palmoplantar keratoderma, the physician should provide a cardiac assessment, considering Naxos/Carvajal disease associated with cardiomyopathy. When an early diagnosis is made, the life expectancy may be increased by treatment of heart failure and arrhythmias; also, genetic counseling should be performed.

Anaesthesia in Naxos disease: first case report.

Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. The hair phenotype is unique, characterized by congenital woolly, curly, rough, and light-colored scalp hair and sparse eyebrows. However, arrhythmias and severe cardiomyopathies are causes of severe life threatened intracardiac thrombus. Thrombus therapy needs private care and sedative, operative processes need to give close attention to these patients. In this case report, sedation given a patient who having severe congestive heart failure with huge mural thrombus in left ventriculus has been presented.

Efficacy of a land-based home exercise programme for patients with juvenile idiopathic arthritis: a randomized, controlled, single-blind study.

OBJECTIVE: To investigate the effects of a land-based home exercise programme on pain, functional ability and quality of life in patients with juvenile idiopathic arthritis. DESIGN: A randomized, controlled, single-blind study. PATIENTS: Eighty-one patients with juvenile idiopathic arthritis participated in this study. METHODS: Functional ability, pain, and quality of life were assessed with a 6-minute walk test, Childhood Health Assessment Questionnaire, visual analogue scale, and the Pediatric Quality of Life Inventory. The patients were randomly assigned to an exercise or control group. The exercise group (n = 43) completed a 12-week individually planned land-based home exercise programme once a week at the hospital for 4 days per week. The control group (n = 38) was placed on the waiting list until the end of the study. RESULTS: Statistically significant improvements (p < 0.001) were found in all the outcome measures in the exercise group. The visual analogue scale score decreased significantly (p < 0.01) in the control group after 12 weeks. Other than the visual analogue scale score (P > 0.05), the changes in the other outcome measures (P < 0.001) were significant in favour of the exercise group. CONCLUSION: The study demonstrated that participating in a 12-week individually planned land-based home exercise programme may result in improved physical function and quality of life in patients with juvenile idiopathic arthritis.

Effects of fluorosis on QT dispersion, heart rate variability and echocardiographic parameters in children.

OBJECTIVE: Chronic fluoride poisoning is called fluorosis. The aim of the study was to investigate effects of fluorosis on cardiovascular system in children by measuring QT dispersion (QTd), corrected QT dispersion (QTcd), heart rate variability (HRV) and echocardiography findings. METHODS: Thirty-five children with dental fluorosis and 26 children as control group were included in this cross-sectional study. Dean index was used for the clinical diagnosis. The fluoride levels of subjects measured by ion electrode method in spot urine higher than 0.6 ppm were included in the study. Serum electrolytes and thyroid function tests were analyzed. Electrocardiography (ECG), echocardiography and 24-hour ambulatory Holter monitorizations were applied, and all the data were analyzed for measuring HRV, and calculation of QTd and QTcd intervals. Corrected QT (QTc) intervals were determined with the Bazzett formula. Difference between the longest and shortest intervals was considered as dispersion. Statistical analysis was performed Kruskal-Wallis test and Pearson correlation test. RESULTS: Low free thyroxine hormone (FT4) (Control Group, Group 2 1.11 (0.85-1.64) ng/dL, 0.96 (0.85-1.11) ng/dL, p<0.05), calcium (Control Group, Group 1, 2, 9.80 (9.30-10.70) mg/dL, 9.60 (8.90-10.70) mg/dL, 9.50 (8.90-10.10) mg/dL, p<0.05) and high serum sodium levels (Control Group, Group 2 139 (136-142) mEq/L, 141 (138-148) mEq/L, p<0.01), increased QT (Control Group, Group 2 329.8 (300.0-363.5) msec, 351.8 (318.0-372.0) msec, p<0.05) and QTc intervals (Control Group, Group I2 390.6 (309.0-418.5) msec, 366.8 (318.2-468.5) msec, p<0.05) were found in subjects with fluorosis. No significant difference was found with respect to echocardiography and HRV variables. CONCLUSION: Endemic fluorosis is a risk factor for decrease in calcium and FT4 levels, increase in sodium levels and QT prolongation. These findings might be related with some cardiovascular system dysfunctions such as arrhythmias or syncope. Subjects with fluorosis should be monitored in terms of long QT and QTc intervals.

Electrocardiographic and echocardiographic findings in street children known to be substance abusers.

Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05).

Shunt operations improved thrombocytopenia in a patient with congenital cyanotic heart disease.

Cardiac and vascular intervention in thrombocytopenic congenitally cyanotic patients is more dangerous. Thrombocytopenia in these patients is related to immune thrombocytopenia, polycythemia, hyperviscosity, pseudothrombocytopenia, and drugs. Herein we report on a thrombocytopenic 8-year-old girl with tricuspid valve atresia and pulmonary valve stenosis admitted for catheterization. Thrombocytopenia (21,000/mm3) and shunt occlusion was noticed. Thrombocytopenia did not recover after intravenous immunoglobulin (IVIG) and phlebotomy therapies. During preparation for surgery, she suffered cardiopulmonary arrest. A Gore-tex graft was placed in the right pulmonary artery and truncus brachiocephalicus. After surgery, her platelet count spontaneously increased to within the normal range (178,000/mm3 to 250,000/mm3). After resuscitation, she had right-sided hemiplegia sequelae, though there were no hemorrhagic findings on cranial magnetic resonance imaging (MRI) or computed tomography (CT) scans. Two months after surgery, the Blalock-Taussig (BT) shunt blood flow decreased, thrombocyte count dropped, and peripheral cyanosis reappeared. A Fontan operation was performed without hemorrhagic events, and after surgery the thrombocyte count reached 330,000/mm3. We suggest that if a patient with cyanotic heart disease has thrombocytopenia and there is no apparent cause, hypoxia-related thrombocytopenia must be considered. After reoxygenation by shunt or corrective surgeries, thrombocyte count and functions will recover.

Signal-averaged electrocardiogram may be a beneficial prognostic procedure in the postoperative follow-up tetralogy of fallot patients to determine the risk of ventricular arrhythmias.

Early detection of arrhythmias after congenital heart disease surgery is important because it can help decrease morbidity and mortality. Standard electrocardiograms (ECGs) contain frequencies between 0.05 and 100 Hz, but higher frequencies are also present. Using high-resolution technology, the highest amplitudes of these high-frequency components within the QRS complex can be recorded and analyzed. We studied the relationship between ventricular late potentials, ventricular arrhythmias and right ventricular systolic pressure in 22 patients who underwent tetralogy of Fallot repair (mean follow-up, 40.1 +/- 33.5 months). Holter ECG monitoring and signal-averaged electrocardiograms (SAECGs) were performed. SAECG parameters studied included the duration of the filtered QRS, the duration of terminal QRS below 40 muV, and the root mean square amplitude of the terminal 40 msec. Cardiac catheterization was performed on 19 patients. Eighteen healthy volunteers were studied as a control. Ventricular arrhythmias were found in 13 patients; right ventricular systolic hypertension was found in 1 patient. No significant residual ventricular septal defects were detected. Eight patients had ventricular late potentials. Right ventricular systolic pressure did not differ significantly between patients with or without late potentials. There were significant differences between patients with ventricular arrhythmias and healthy volunteers; filtered QRS duration was significantly longer in patients with ventricular arrhythmias. SAECG may be beneficial in determining ventricular arrhythmia risk in tetralogy of Fallot patients postoperatively.

Does red-man reaction stimulate macrophage activation syndrome in children with systemic juvenile idiopathic arthritis?

Macrophage activation syndrome (MAS) is a major cause of death in patients with systemic juvenile idiopathic arthritis (JIA). We describe 4 patients who developed MAS during or after vancomycin treatment. Vancomycin should be used with great care in patients with systemic JIA.

Novel evidence suggesting an anti-oxidant property for erythropoietin on vancomycin-induced nephrotoxicity in a rat model.

1. The aim of the present study was to investigate the role of oxidative stress in renal injury and to determine whether erythropoietin (EPO) acts as an anti-oxidant in vancomycin (VCM)-induced renal impairment. 2. Twenty-four rats were divided into three groups as follows: (i) control (Group 1); (ii) VCM treated (Group 2); and (iii) VCM + EPO treated (Group 3). Vancomycin (200 mg/kg, i.p.) was administered to Groups 2 and 3 for 7 days. Erythropoietin (150 IU/kg, i.p.) treatment was started 24 h before VCM and lasted for 7 days. On Day 8, renal tissues were excised and blood samples were collected. Serum creatinine and blood urea nitrogen were measured, along with renal malondialdehyde (MDA) levels, superoxide dismutase (SOD) and catalase (CAT) activity and tissue VCM levels. The kidneys were examined for any histopathological changes. 3. Renal MDA levels were found to be increased, whereas SOD and CAT activity was decreased, in the VCM-treated group compared with the control group. There was a marked decrease in MDA levels and an increase in SOD activity, but not CAT activity, after VCM + EPO treatment. Marked histopathological alterations, including interstitial oedema, tubular dilatation, tubular epithelial cell desquamation and vacuolization, were observed in VCM-treated rats. Histopathological changes were significantly improved after EPO administration. 4. In conclusion, the present data suggest that oxidative stress plays an important role in VCM-induced nephrotoxicity. Erythropoietin seems to act as an anti-oxidant, diminishing the toxic oxidative effects of VCM on renal tissues.

Can renal leukemic infiltration cause hypertension in children?

Out of 334 children with acute lymphoblastic leukemia who were treated with St Jude Total XI and Total XIII chemotherapy protocols were investigated and 21 (6.3%) were hypertensive. The incidence of tumor lysis syndrome was higher in the hypertensive group than in the nonhypertensive group (28.6% vs. 11.5%) (P = 0.035). There were no differences between patients treated with high-dose methylprednisolone and prednisolone St Jude Total XI and Total XIII, St Jude Total XIII LR and St Jude Total XIII HR groups in respect of the above-mentioned parameters. Central nervous system involvement, skeletal system involvement, abdominal lymphadenopathy, elevated lactate dehydrogenase and leukocyte count, French-American-British types and immunophenotypes were not found to be statistically significant to the development of hypertension (P > 0.05). We found that renal leukemic infiltration is a risk factor in hypertension development (P = 0.04) and hypertension is a risk factor for renal parenchymal disorder in the follow-up period (P = 0.0001). Six patients presenting with hypertension in the first week of disease therapy were evaluated for renal parenchymal disorder and glomerular filtration rate abnormality in the follow-up period. Glomerular filtration rate abnormality was found in 1 and renal scintigraphic dimercaptosuccinic acid abnormalities (reduced uptake and dilated hypoactivity) were found in 4 patients. Hypertension was also found to be a risk factor for renal parenchymal disorder in the follow-up period.

Methotrexate-induced renal oxidative stress in rats: the role of a novel antioxidant caffeic acid phenethyl ester.

The exact mechanisms of methotrexate-induced renal toxicity have not yet been determined. However, several hypotheses have been put forward, including oxidative stress. The aim of this study was to investigate the role of caffeic acid phenethyl ester (Caffeic Ester), a novel antioxidant, on methotrexate-induced renal oxidative stress in rats. Nineteen adult male rats were equally divided into three experimental groups as follows: control group, methotrexate-treated group, and methotrexate+Caffeic Ester-treated group. A single dose of methotrexate (20 mg/kg) was administered intraperitoneally (ip). Caffeic Ester (10 micromol/kg) was administered ip, once daily for seven days. Malondialdehyde (MDA) levels (an index of lipid peroxidation) were used as a marker of oxidative stress-induced renal injury. Similarly, the activities of superoxide dismutase (SOD), catalase (CAT) and glutathione peroxidase (GSH-Px) were determined to evaluate the changes of antioxidant status in renal tissue. Methotrexate administration to control rats increased MDA levels (P<0.0001), but decreased SOD, CAT and GSH-Px activities in renal tissue (P<0.0001). Caffeic Ester+ methotrexate treatment caused a significant decrease in MDA levels (P<0.001), and caused an increase in SOD, CAT and GSH-Px activities when compared with methotrexate treatment alone (P<0.001, <0.05, <0.0001, respectively). In conclusion, methotrexate leads to a reduction in antioxidant enzymatic defense capacity and causes lipid peroxidation in renal tissue. Similarly, Caffeic Ester exhibits protective effects on methotrexate-induced renal oxidative impairment in rats.

Protective role of erdosteine on vancomycin-induced oxidative stress in rat liver.

Drug-induced liver toxicity is a common cause of liver injury. This study was designed to elucidate whether high dose vancomycin (VCM) induces oxidative stress in liver and to investigate the protective effects of erdosteine, an expectorant agent. Twenty-two young Wistar rats were divided into three groups as follows: control group, VCM, and VCM plus erdosteine. VCM was administered intraperitoneally in the dosage of 200 mg/kg twice daily for 7 days. Erdosteine was administered orally administered once a day at a dose of 10 mg/kg body weight. The activities of antioxidant enzymes such as superoxide dismutase and catalase as well as the concentration of malondialdehyde, as an indicator of lipid peroxidation, were measured to evaluate oxidative stress in homogenates of the liver. VCM administration increased malondialdehyde levels (p < 0.001), superoxide dismutase (p < 0.01) and catalase (p < 0.001) activities. Erdosteine co-administration with VCM injections caused significantly decreased malondialdehyde levels (p < 0.001), superoxide dismutase (p < 0.01) and catalase (p < 0.001) activities in liver tissue when compared with VCM alone. It can be concluded that erdosteine may prevent VCM-induced oxidative changes in liver by reducing reactive oxygen species.