Filiz Şenocak

Hepatic artery aneurysm in a 10-year-old boy as a complication of infective endocarditis

Mycotic aneurysms occur from septic emboli in patients with infective endocarditis and may involve any artery, but frequently they are not detected before autopsy. The most common sites are the brain, abdominal aorta, sinus of Valsalva, ligated ductus arteriosus, and superior mesenteric, splenic, coronary, and pulmonary arteries. The authors report on a 10-year-old boy who had a mycotic aneurysm of the common hepatic artery, which developed during the course of infective endocarditis of the mitral valve and was cured successively using a platinum coil embolization technique.

Thiamine-responsive megaloblastic anemia syndrome with atrial standstill: a case report.

Thiamine-responsive megaloblastic anemia (TRMA) syndrome is an uncommon autosomal recessive disorder. The disease is caused by mutations in the gene, SLC19A2, encoding a high-affinity thiamine transporter, which disturbs the active thiamine uptake into cells. Major features include megaloblastic anemia, diabetes mellitus, and sensorineural deafness. Cardiac malformations with conduction defects and/or dysrhythmias, have also been described in some patients. To our knowledge, only 13 TRMA patients with cardiac defects have been reported. Here, we describe the first case of TRMA syndrome with atrial standstill, probably caused by a 2 base-pair deletion in exon 4 (1147delGT) of the gene SLC19A2.

Tolmetin and salicylate therapy in acute rheumatic fever: Comparison of clinical efficacy and side-effects

Abstract Background : The arthritis of rheumatic fever is very responsive to treatment with salicylates, but there are many adverse reactions, especially hepatotoxicity, due to aspirin (acetylsalicylic acid) therapy. These side‐effects change the course and duration of rheumatic fever. Other non‐steroidal anti‐inflammatory drugs may be equally effective, although no reports are available.

An evaluation of heart rate variability and its modifying factors in children with type 1 diabetes

OBJECTIVE To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. METHODS This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. RESULTS The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = -0.263 and -0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = -0.751 and -0.644) and between very low frequency and diabetes duration. CONCLUSION A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Kalbin Sol Tarafinin Multipl Obstrüktif Doğumsal Lezyonlari

Congenital multiple obstructive lesions of the left heart Objective: To investigate the localization, type, associations, other accompanying abnormalities and treatment methods. Material and Methods: The study included 24 cases presenting to the Pediatric Cardiology Unit of our Hospital with more than one left heart lesion, evaluated with two-dimensional color Doppler echocardiography (Vivid 7 Pro); 18 underwent cardiac catheterization and angiography. Complaints at admission, lesion site, type and associations, additional abnormalities and treatment were evaluated. Results: Multiple obstructive left heart lesions were more frequent (79%) in males, at an average age of 6.3. Most frequent symptoms at admission were dyspnea, fatigability and cyanosis. The most frequent associations of obstructive abnormalities were: mitral and aortic stenosis in 29%, mitral stenosis with aortic coarctation in 17% and aortic valve stenosis with aortic coarctation in 37%. Mitral stenosis associated to both aortic valve stenosis and aortic coarctation was seen in 17%. The most frequent associated non-obstructive abnormality was a bicuspid aortic valve (62%). Following diagnosis, 5 of the patients did not show up for follow-up, while the decision for one patient was observation. The remaining 18 cases underwent cardiac catheterization and angiography. Six patients had successful coarctation angioplasty and one angioplasty plus aortic valvuloplasty. We lost 2 of the patients who had undergone coarctation angioplasty. Conclusion: It was observed that the lesion most significantly contributing to mortality due to congenital obstructive lesions of left heart outflow are mitral valve obstruction and pulmonary hypertension, and that early invasive intervention is life-saving.

Transcatheter occlusion of tortuous feeding vessel with new vascular plug in an infant with pulmonary sequestration with Scimitar syndrome

We report the case of a 9-month-old girl with a Scimitar syndrome which was feeding via an anomalous vessel dividing into three tortuous branches and supplying blood to a localized region of the right lower lobe which was successfully occluded using an Amplatzer® Vascular Plug 4 (AGA Medical, Golden Valley, MN, USA). The percutaneous procedure performed via femoral artery, provided an immediate closure of the vessel, excluding the need for surgical repair. This new device is found to be safe and effective for occlusion of the collaterals, especially large ones in pediatric ages.

Malformations due to warfarin: A case report

We report a 5-month-old infant with severe growth retardation who was exposed to in utero warfarin for the first 4 months. She had a dysmorphic face with depressed nasal hypoplasia and low-set ears. Cranial computerized tomography revealed bifrontal atrophy, agenesis of corpus callosum, and dilation of lateral ventricles. In addition she had patent ductus arteriosus with pulmonary hypertension, which had to be ligated.