Meki Bilici

Pamidronate treatment in acute vitamin D intoxication.

Management with glucocorticoid, high iv fluid saline intake, furosemide and calcitonin may not result in a favorable reduction of hypercalcemia and may cause several side effects in infants with acute vitamin D intoxication. The bisphosphonate pamidronate, a specific inhibitor of bone resorption through osteoclast mediation was successfully used in a 6-month old infant with acute vitamin D intoxication managed in the Pediatric Emergency and Intensive Care Unit, after an ineffective trial of hydration, furosemide, calcitonin and prednisolone. After a double infusion of pamidronate on two consecutive days (1 mg/kg/day), an early and safe correction of hypercalcemia/hypercalciuria was supplied. Pamidronate therapy may be considered in patients with hypercalcemia secondary to acute vitamin D poisoning.

Polymorphisms of the angiotensin converting enzyme and angiotensin II type 1 receptor genes and renal scarring in non-uropathic children with recurrent urinary tract infection

Aim:  The aim of this study was to investigate whether the angiotensin converting enzyme (ACE) and angiotensin II type 1 receptor (A1166C) gene polymorphisms were associated with the renal scar formation secondary to recurrent urinary tract infection in children without uropathy.

Anti-oxidant status in relation to lipoproteins, leptin and pro-inflammatory cytokines in children with steroid-sensitive nephrotic syndrome.

Background:  Reactive oxygen species and cytokines are reported to play a role in the proteinuria of nephrotic syndrome. The aim of this study was to investigate indirect evidence of oxidant activity together with leptin, lipoproteins and pro‐inflammatory cytokines in children with steroid‐sensitive nephrotic syndrome.

Stent implantation in the arterial duct of the newborn with duct-dependent pulmonary circulation: single centre experience from Turkey

OBJECTIVES Implantation of stents into the ductus arteriosus is an alternative treatment to palliative or corrective cardiac surgery in newborns with duct-dependent pulmonary circulation, although the use of this technique for congenital heart disease is limited. METHODS Between April 2010 and June 2011, 13 patients underwent patent ductus arteriosus stenting after full assessment by echocardiogram and angiogram, two of patients had pulmonary atresia (PA) and ventricular septal defect (VSD), six patients had PA with intact ventricular septum (IVS), four patients had critical pulmonary stenosis with IVS and one single ventricle physiology with PA and four patients had radiofrequency-assisted perforation of the pulmonary valve at the same time. All procedures were retrograde through the femoral artery, except one, which was by the femoral vein approach. RESULTS The mean age and weight during intervention were 10.5±5.7 days and 3.1±0.4 kg, respectively. The mean of procedure and scopy time, time of stay in intensive care, total out-of-hospital and total follow-up time were 138.88±67.11 min; 40.32±25.86 min; 4.88±6.07 days; 11.00±6.89 days and 86.40±73.21 days, respectively. The mean of the radiation amount was 1054.27±1106.91 cGy/cm2. The mean of saturation before and after intervention were 64.44±5.83; 81.88±6.95%, respectively. Procedure-related deaths were observed in two patients. The causes of death were pulmonary haemorrhage (n=1) and retroperitoneal haemorrhage (n=1). Two patients also died after discharge before surgery due to sepsis (n=1) and aspiration pneumonia (n=1). Eight of 13 patients achieved stent patency during 6 months of follow up and re-stenosis developed in one patient (1/8; 12.5%) who had undergone a Glenn operation at 4.5 months of age. CONCLUSIONS Ductal stenting is a practicable, effective, safer and less invasive method compared palliative or corrective surgery. Patients with ductal stenting have growth of the pulmonary artery which provides additional time for surgical repair. Our data suggested that ductal stenting should be considered as a first treatment step in newborns with duct-depended pulmonary circulation. However, long-term palliation without stent re-stenosismight still be a concern especially in patients with hypoplastic pulmonary arteries.

Diabetic Ketoasidosis is Associated with Prothrombotic Tendency in Children

Children and adolescents with type I diabetes mellitus (DM) may present with diabetic ketoacidosis (DKA), which is associated with significant morbidity and mortality. This study aimed to evaluate the hematological parameters at diagnosis (0th hour) and 96th hour after the initiation of treatment in children with DKA. Twenty-six children with DKA treated in Dicle University Faculty of Medicine between September 2002 and August 2003 were included in this study. General characteristics of the patients and hematological parameters (platelet count, white blood cell count, prothrombin time, partial thromboplastin time (PTT), bleeding time, coagulation time, protein C, protein S, antithrombin III, fibrinogen, D-dimer, factor VIII, factor IX, and factor X levels) at diagnosis (0th hour) and 96th hour after the initiation of treatment were determined. The mean age of the children (10 girls and 16 boys) was 9.15 ± 3.85 years (range: 4–15 years). DKA developed for the first time in 58.3% of these children and they had recently been diagnosed as DM. After hematological parameters at 0th hour were evaluated, increased platelet count, decreased PTT, low protein C, and high factor VIII levels were determined at diagnosis, indicating prothrombotic tendency. If the hematological parameters at 0th hour were compared with those at 96th hour; platelet count decreased, PTT increased, protein C and factor VIII levels turned to be normal at 96th hour. When all the results are considered together, children with DKA appeared to have a prothrombotic tendency. Although this tendency was not reflected in clinical findings in this study, it should be kept in mind that children with DKA are prone to the development of thrombosis and they need to be investigated for the possibility of thrombosis.

Spontaneous Closure of a Symptomatic Coronary Artery Fistula Just within a Few Days of Newborn Period

We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14-day-old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent flow of a large coronary artery fistula (CAF) between the left coronary artery and the RV. Tachypnea was regressed and repeat echocardiogram showed no CAF related to cardiac chambers after 4 days of admission. The spontaneous closure of CAF was found to be more likely in cases younger than 2 years with small-sized fistulas opening into the right-sided structures, especially into the RV. Nevertheless, the spontaneous closure is very rare in cases with significant shunt. To the best of our knowledge, this is the first case with documented spontaneous closure of CAF just within the newborn period.

Steroid Responsiveness of Children with Idiopathic Nephrotic Syndrome in Southeastern Region of Turkey

Background. Our aim was to determine the prognostic factors effective in the response to steroid treatment and relapse frequency. Patients and Methods. In this study, we evaluated 84 children with idiopathic nephrotic syndrome followed-up from 1997–2002. The variables were analyzed with respect to medical history, physical examination, laboratory findings, response to treatment, and factors associated with remissions and relapses. Our study group consisted of 62 children with minimal change nephrotic syndrome (MCNS), 11 children with focal segmental glomerulosclerosis (FSGS), and 11 children with diffuse mesangial proliferation (DMP). Results. According to response to steroids; 57.1% were steroid-sensitive with infrequent relapses, 22.6% were steroid-dependent with frequent relapses, and 20.2% were steroid-non-responders. Significantly high non-responder ratios to steroids were found in children with initial hypertension and hematuria (p < 0.05). Although patients older than six years were found to be associated with steroid non-response (p < 0.05), the number of relapses were found to be increased with an increasing number of infections (p < 0.05). The time period for the first relapse was found to be statistically correlated with relapse numbers of the first 6 (p = 0.001) and 12 (p = 0.01) months. Conclusion. The time span between initial presentation and remission and the number of infections were significant for relapse frequency. The existence of hematuria and hypertension and age greater than 6 years at initial presentation were associated with steroid non-responsiveness. The likelihood of developing resistance to the treatment should be emphasized early to the parents of patients bearing these risk factors, and hence the possible disappointment in the family should be prevented.

Short-Term Outcomes of Patent Ductus Arteriosus Closure With New Occlutech® Duct Occluder: A Multicenter Study

AIM Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. METHODS We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. RESULTS The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. CONCLUSION Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

Radiofrequency ablation of fast ventricular tachycardia causing an ICD storm in an infant with hypertrophic cardiomyopathy: ERGUL et al.

An implantable cardioverter defibrillator (ICD) storm involves very frequent arrhythmia episodes and ICD shocks, and it is associated with poor short‐term and long‐term prognosis. Radiofrequency catheter ablation can be used as an effective rescue treatment for patients with an ICD storm. To our knowledge, this is the first report of an infant with hypertrophic cardiomyopathy presenting with an ICD storm and undergoing successful radiofrequency catheter ablation salvage treatment for the fast left posterior fascicular ventricular tachycardia.

Cardiac Tamponade may be the First Symptom of Leukemia

Acute myeloblastic leukemia (AML) is originating from hematopoietic precursor cells. CD7 is expressed in 30% of AML cases and is associated with poor prognosis in myeloid malignancies [1,2]. Pericardial effusion complicated by cardiac tamponade is a life-threatening condition is quite rare as the initial symptom in cases with AMLM4 [3,4]. Pericardial effusion can be observed during the course of the leukemiawhile it can also develop secondary to infectious, hemorrhagic causes as well as leukemic infiltration. It is especially associated with chemotherapeutics such as bleomycin, all-trans-retinoic acid, and cyclophosphamide [5]. In this report, it was aimed to increase the awareness of the fact that pericardial tamponade may sometimes be the first sign of leukemia. A 7-year-old boy in toxic appearance presented with fatigue, respiratory difficulty for 1 month. He was hypotensive, tachypneic, and tachycardic. His growth and development were consistent with his age. Respiratory examination revealed fine crackles at the base of the lungs. His heart sounds became muffled and hepatosplenomegaly was detected. Chest X-ray demonstrated increased cardiothoracic index. Transthoracic echocardiography showed pericardial effusion and diastolic right atrial collapse (Figure 1). Pericardiocentesis was performed. Direct examination of the exudative fluid revealed leukemic cells. Complete blood count revealed, hemoglobin level 8 g/dL, platelet count 35 × 106 per mm3, leukocyte count 7,7 × 10 mc/L. Serum lactate dehydrogenase: 1995U/L (normal 125–243U/L), other biochemical tests were normal. According to immunophenotyping study and bone marrow aspiration; the patient was diagnosed with CD7+ AMLM4 (French-American-British classification). Based on AML-BFM-2004 protocol, induction chemotherapy was begun. At the tenth day of the therapy, pericardial fluid was dramatically decreased.The patient now is at thefirst year ofmaintenance therapyof theAML-BFM2004 treatment protocol and healthy. Inchildhood,AMLsexpressionof lymphoid-associated surfaceantigens is amarker of poor prognosis [1]. AML, especially subtypeM4, associatedwith CD7 has a particular worse prognosis with a lower complete remission rate [6]. When complications of leukostasis known as a severemedical emergency necessitating rapid treatment, such