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.

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.

Parry-Romberg syndrome (PRS) is a rarely seen connective tissue disorder and is characterized by chronic inflammation of the face. Anti-nuclear antibodies, anti-extractable nuclear antigens and skin biopsy are helpful for diagnosis. In this study, we determined that the external carotid artery blood flow rate was faster on the affected side than on the unaffected side. We believe that patients suffering from PRS who also have central nervous system symptoms must be examined for vascular structure by Doppler ultrasonography, magnetic resonance imaging or conventional angiography.

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.

Background and aims: Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiologic entity characterized by headache, visual disturbances, seizures, and the presence of edema on MRI scan, predominantly in the posterior white matter. We wanted to refer to a patient diagnosed PRES. Aims: We wanted to refer to a patient diagnosed PRES. Methods: 15 years old female patient. FMF, FMF depending on the amyloidosis and amyloidosis patients with renal failure receiving hemodialysis was started three months ago. Admitted to hospital before nausea, vomiting, headache going on 6–8 hours after the onset of complaints in the eyes blinking, twitching at the corners of the mouth in the form of 1–2 minutes duration was admitted to our emergency department patients with seizures. GCS: 12 that fluctuations in consciousness, arterial pressure 177/150 mmHg in patients as measured by cranial CT scans were obtained. Right parieto, parietosuperi and parietoposteri observed hypodense areas in the region to press the patients with a diagnosis of acute encephalopathy was admitted to the intensive care pediatrics. Emergency and after hemodialysis patients held antihypertensive therapy, cranial MR were taken. Results: As a result of the etiological processes that many PRESS heterogeneous disease. Conclusions: In this report: We wanted to emphasize in a patient with amyloidosis associated kidney failure PRES development.

Gram negatif mikroorganizmalarin etken oldugu enfektif endokardit nadir gorulmesine ragmen morbidite ve mortaliteye yol acmaktadir. Bu etkenlerden birisi olan Pseudomonas suslarinin neden oldugu enfektif endokarditler oldukca nadir gorulmektedir. Inlet tipi genis ventrikuler septal defekt ve pulmoner stenozu olan 3 yasindaki kiz hasta, ventrikuler septal defektin yama ile kapatilmasindan iki gun sonra gelisen ates, tasipne ve tasikardi, hepatomegali bulgulariyla enfektif endokardit on tanisi ile yatirildi. Kan kulturunde Pseudomonas aeruginosa ureyen hastanin konjestif kalp yetersizligi tablosunda olmasi, uygun kombine antibiyotik tedavisine karsin uc aydan fazla surede enfeksiyonun kontrol altina alinamamasi nedeniyle operasyon karari alindi. Operasyon sirasinda, enfekte olan ventrikuler septal defekt yamasi cikartildi, trikuspit kapak tamiri yapildi ve pulmoner kapaktan vejetasyon cikartildi. P.aeruginosa’nin etken oldugu sag kalp kaynakli endokarditte uygun antibiyoterapi ile iyilesme saglanmasina ragmen bakteriyeminin persiste ettigi hastalarda, geciktirilmeden uygulanan cerrahi tedavinin tedavi basarisini artirabilecegi dusunulmustur. (J Pediatr Inf 2014; 8: 196-9)

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.

Objective: To investigate the relationships between urinary tract infection (UTI), and activity of antioxidant enzymes, xanthine oxidase (XO) and myeloperoxidase (MPO) in urine of children with UTI Design: Prospective observational study Setting: Department of Pediatrics, Kahramanmaras Sutcu Imam University Faculty of Medicine, Turkey Subjects and Methods: A total of 115 random children admitted to our hospital for urinary symptoms, 61 girls and 54 boys, aged between 2 and 15 years (average 10 years) were included. Study subjects were divided into four groups: Group 1 consisted of 29 pyuria positive and urine culture negative children; Group 2 included 30 children with pyuria and positive urine culture; Group 3 included 26 pyuria negative and urine culture positive children and Group 4 included 30 with pyuria and negative urine culture. Measurement of urine XO and MPO activity were performed spectrophotometrically. Intervension: Urine samples Main Outcome Measures: Antioxidant enzyme activity levels in urine were examined in children with urinary tract infection. Results: Urinary XO activities in Group 2 were significantly higher compared with other groups (p < 0.001, p = 0.001 and p < 0.001, respectively). Significantly different MPO activities were found between Group 1 and Group 2 (p = 0.007). However, no significant difference was found between Group 3 and 4. Conclusion: Significantly increased MPO and XO enzyme activity was found in children with UTI. Measurement of MPO and XO activity may be useful in children with urinary symptoms to diagnose UTI, before obtaining a positive urine culture.

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.

Aim: To evaluate malnutrition, anemia and related deficiency of vitamin B12, iron and folic acid in children with neurological disease and related nutritional problems.Material and Method: In this study, our aim was to investigate anemia and possible reasons such as iron deficiency, folic acid deficiency and vitamin B12 deficiency among patients with nutritional problems who were admitted to Kahramanmaras Sutcu Imam University, Faculty of Medicine, Outpatient and Service of Pediatric Neurology between June 2010 and December 2010. The study involved a total of 50 patients (30 male and 20 female). A control group including 30 boys and 20 girls was included in the study as well. Complete blood count, iron, folic acid and vitamin B12 values were studied.Results: A total of 39 patients (78%) had malnutrition; among malnutrition patients 16 (32%) had severe malnutrition, 18 (36%) had moderate malnutrition and five (10%) had mild malnutrition. A total of 22 patients (44%) had anemia, iron deficiency, folic acid deficiency or vitamin B12 deficiency alone or in combination. Nine of 22 patients (18%) had iron deficiency anemia, four (8%) had iron deficiency, five (10%) had folic acid deficiency, one (2%) had folic acid deficiency macrocytic anemia, six (12%) had anemia of chronic disease, two (4%) had B12 deficiency, four (8%) had both iron deficiency anemia and folic acid deficiency.Conclusions: In our patients with neurological disease, nutritional deficiencies and anemia were found to be quite frequent. These patients should get a special care for nutrition and should be checked periodically for anemia and nutritinal parameters. (Turk Arch Ped 2012; 47: 199-203)

Sum mary Aim: To evaluate malnutrition, anemia and related deficiency of vitamin B12, iron and folic acid in children with neurological disease and related nutritional problems. Material and Method: In this study, our aim was to investigate anemia and possible reasons such as iron deficiency, folic acid deficiency and vitamin B12 deficiency among patients with nutritional problems who were admitted to Kahramanmaraş Sütçü İmam University, Faculty of Medicine, Outpatient and Service of Pediatric Neurology between June 2010 and December 2010. The study involved a total of 50 patients (30 male and 20 female). A control group including 30 boys and 20 girls was included in the study as well. Complete blood count, iron, folic acid and vitamin B12 values were studied. Results: A total of 39 patients (78%) had malnutrition; among malnutrition patients 16 (32%) had severe malnutrition, 18 (36%) had moderate malnutrition and five (10%) had mild malnutrition. A total of 22 patients (44%) had anemia, iron deficiency, folic acid deficiency or vitamin B12 deficiency alone or in combination. Nine of 22 patients (18%) had iron deficiency anemia, four (8%) had iron deficiency, five (10%) had folic acid deficiency, one (2%) had folic acid deficiency macrocytic anemia, six (12%) had anemia of chronic disease, two (4%) had B12 deficiency, four (8%) had both iron deficiency anemia and folic acid deficiency. Conclusions: In our patients with neurological disease, nutritional deficiencies and anemia were found to be quite frequent. These patients should get a special care for nutrition and should be checked periodically for anemia and nutritinal parameters. (Turk Arch Ped 2012; 47: 197-201)

Benzalkonium chloride (BAC) is a frequently used disinfectant and its most well-known side effect is contact dermatitis. in this report, two children who had vesicular dermatitis, headache, lethargy, fever and encephalopathy mimicking Herpes zoster encephalitis were presented. Their consciousness level improved on the second day. From the medical history it was understood that the mother had applied 20% BAC solution to the scalps of two children. The aim of the presentation of this report is to draw attention to the fact that BAC application to the scalp for treating pediculosis capitis may resemble the herpes encephalitis clinical picture.

Yellow nail syndrome, usually autosomal dominant transition and occurs with yellow nails, lymph edema and pleural effusion triad. In this article, a girl, 13 months, who was diagnosed as yellow nail syndrome and followed with pericardial effusion and lymph edema from her birth, has been presented. Yellow nail syndrome has been diagnosed lately due to the occurrence of clinical symptoms which are often more obvious after the puberty. However, neonatal or infant period of pericardial effusion in patients with lymphedema and yellow nail syndrome should be kept in mind. OZET

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.

Yellow nail syndrome, usually autosomal dominant transition and occurs with yellow nails, lymph edema and pleural effusion triad. In this article, a girl, 13 months, who was diagnosed as yellow nail syndrome and followed with pericardial effusion and lymph edema from her birth, has been presented. Yellow nail syndrome has been diagnosed lately due to the occurrence of clinical symptoms which are often more obvious after the puberty. However, neonatal or infant period of pericardial effusion in patients with lymphedema and yellow nail syndrome should be kept in mind.

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.

SuleymanDemirel Universitesi TIP FAKULTESI DERGISI: 2006 Aralik; 13(4) Cok dusuk dogum agirlikli bebeklerde total parenteral beslenme iliskili kolestaz ve ursodeoksikolik asit tedavisinin etkinligi Hasan Cetin, Sevgi Kosaci Akdeniz, Seref Olgar, Faruk Oktem, Bumin Dundar, Ali Ayata Ozet Cok dusuk dogum agirlikli bebeklerde uzun sureli parenteral beslenme gereklidir ve kolestaz riski de fazladir. Calismanin amaci cok dusuk dogum agirligi olan bebeklerde kolestaz oranini, enteral beslenmeyle olan iliskisini belirlemek, ursodeoksikolik asid tedavisinin etkisini degerlendirmektir. Yenidogan Yogun Bakim Unitesinde cok dusuk dogum agirlikli bebeklere ait bes yillik veriler degerlendirildi. Calismanin ikinci yarisinda gastrointestinal sistem beslenmeye elverisli hale gelince 15-20 ml/kg/gun olacak sekilde minimal enteral beslenme baslandi. Ikinci donemde total parenteral beslenme suresi uzun olmasina ragmen minimal enteral beslemeye baslama zamani anlamli olarak kisa idi(p<0.05). Total kolestaz orani %12 saptandi. Kolestaz oraninin ikinci donemde belirgin olarak %20.8.den %5.e geriledigi gozlendi(p<0.05). Ursodeoksikolik asid tedavisiyle kolestaz suresinin belirgin oranda kisaldigi gozlendi. Sonuc olarak enteral beslenme olabildigince cabuk baslanmalidir. total parenteral beslenme iliskili kolestaz tedavisinde ursodeoksikolik asid kullanimi faydali olabilir. Anahtar kelimeler: Kolestaz, total parenteral beslenme, enteral beslenme, cok dusuk dogum agirlikli bebek. Abstract Total parenteral nutrition-associated cholestasis and the effect of ursodeoxycholic acid in very low birth weight infants Prolonged parenteral nutrition are necessary and there is high risk of cholestasis in very low birth weight infants. The aim of this study was to determine the rate of cholestasis, correlation with enteral feeding and the effect of ursodeoxycholic acid in very low birth weight infants. The data of five years in Neonatal Intensive Care Unit belong to very low birth weight infants was analysed. In the second half of the performed study, minimal enteral feeding was started by the dose of 15-20 ml/kg/day when the gastointestinal system was ready to feeding. Although total parenteral nutrition days was longer, the age of minimal enteral feeding significantly shorter in second period than first period(p<0.05). Total cholestasis rate was detected as 12%. The rate of cholestasis significantly reduced from 20.8% to 5% in second period(p<0.05). Ursodeoxycholic acid treatment shortened the period of cholestasis significantly. In conclusion enteral feeding should be begin quickly. The use of ursodeoxycholic acid treatment could be beneficial in the treatment of total parenteral nutrition associated cholestasis. Key words: Cholestasis, total parenteral nutrition, very low birth weight infants, enteral feeding

SuleymanDemirel Universitesi TIP FAKULTESI DERGISI: 2004 Haziran; 11(2) Uriner sistem enfeksiyonu olan cocuklarda reaktif trombositoz Faruk Oktem, Seref Olgar, Hasan Cetin Ozet Amac: Bu calismada uriner sistem infeksiyonu olan cocuklarda reaktif trombositozun degerlendirilmesi amac- landi. Gerec ve Yontem: Trombositoz ve uriner sistem infeksiyonunun duzeyi arasindaki iliski, yas ortala- malari 3.9 yil olan (21 erkek ve 36 kiz) 57 olguda retrospektif olarak degerlendirildi. Bulgular: Uriner sistem infeksiyonu olan olgularin % 36' sinda, baslangic doneminde reaktif trombositoz belirlendi. Ortalama trom- bosit sayimi ust uriner sistem enfeksiyonu olan olgularda daha yuksekti (445.950 /mm3 'a karsilik 339.260 /mm3) (p 0.05). Sonuc: Reaktif trombositoz pediatrik uriner sistem enfeksiyonlarinin seyri sirasinda gorulebilen bir bulgudur. Bunula beraber, ozellikle baslangic fazinda sadece bobrek parankim infeksiyonlarinda bulunabilen bir ozellik degildir. Anahtar kelimeler: trombositoz, uriner sistem infeksiyonu Abstract Reactive thrombocytosis in children with urinary tract infections Objectives: This study aimed to evaluate reactive thrombocytosis in pediatric cases with urinary tract infec- tions. Material and Methods: The relationship between thrombocytosis and the level of urinary tract infections was studied retrospectively in (21 boys, 36 girls) 57 children, mean age 3.9 years. Results: Reactive thrombo- cytosis was noticed in 36 % of children with urinary tract infections during the initial phase. The mean platelet counts were significantly higher in upper urinary tract infections (445.950 /mm3 vs 339.260 /mm3). But thrombocytosis determined was similar in upper and lower urinary tract infections, respectively 43 % vs 20 % (p>0.05). Conclusion: Reactive thrombocytosis appears as a finding to appear during the course of pediatric urinary system infections. However, it was not found exclusively in the renal parenchmal infections particu- larly during the initial phase. Keywords: Thrombocytosis, urinary tract infections

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.