Taliha Oner

Postural Orthostatic Tachycardia Syndrome (POTS) and Vitamin B12 Deficiency in Adolescents

OBJECTIVE: Vitamin B12 is involved in the production of adrenaline from noradrenaline. It is the cofactor involved in catecholamine degradation and plays a role in myelin synthesis. The current study aimed to investigate the association between vitamin B12 levels and postural orthostatic tachycardia syndrome (POTS) during adolescence when accelerated myelin synthesis increases the vitamin B12 need. METHODS: One hundred twenty-five patients (mean age 11.1 ± 2.3 years; 60% female) reporting short-term loss of consciousness and diagnosed with vasovagal syncope based on anamnesis with a normal distribution and 50 control subjects (mean age 10.94 ± 2.5 years, 62% female) were included in this study. Serum vitamin B12, folic acid, and ferritin levels were measured prospectively in addition to other tests. We defined vitamin B12 deficiency as a serum level <300 pg/mL.1–4 RESULTS: Vitamin B12 levels were significantly lower in the patient group compared with the control group (47.2% vs 18%, P < .001). In the patient group, children with the POTS pattern had significantly lower vitamin B12 levels compared with children without the POTS response (P = .03). CONCLUSIONS: Vitamin B12 deficiency in patients with POTS may lead to sympathetic nervous system baroreceptor dysfunction.

Incomplete (atypical) Kawasaki disease in a young infant with remarkable paucity of signs

We read with great interest the case report by Thapa et al. [1] entitled “Atypical Kawasaki disease with remarkable paucity of signs and symptoms” Kawasaki disease (KD) is an acute febrile vasculitis that was seen predominantly in infants and young children. It is the predominant cause of pediatric acquired heart disease in the developing world. The etiology remains unknown, but clinical and epidemiological features strongly suggest an infectious cause or trigger factor plus a genetic predisposition [2, 3]. The conventional diagnostic criteria should be viewed as guidelines that are particularly useful in diagnosis of complete form but may result in failure to recognize incomplete forms of illness. Unlike KD, in cases of incomplete Kawasaki disease (iKD), coronary artery disease was present in children with fever lacking the suYcient number of criteria to fulWll the epidemiologic case deWnition [3]. Thus, iKD diagnosis often depends on echocardiographic Wndings of coronary artery abnormalities (CAA). But according to the Japanese guidelines [4], iKD deWned as the presence of four or fewer of the principal Wndings of KD regardless of the presence or absence of CAA. No matter which diagnostic criteria was preferred, it complications were as severe as KD. In this letter, an 8-month-old infant referred to our pediatric cardiology clinic with high-grade fever for 19 days and lacking associated symptoms was diagnosed as iKD, since she had saccular aneurysm on her echocardiography is described. Her physical examination revealed no speciWc Wndings except fever (39.5°C). There was no history of conjunctivitis, rash, erythema of the lips, extremity changes or cervical lymphadenopathy. Until her reference to our clinic, patient had a history of multiple antibiotic usage with diVerent clinical diagnosis. Laboratory tests revealed the following: hemoglobin, 9.2 gm/dl; leukocyte count, 12,100/mm; (peripheral smear revealed neutrophils, 30%; lymphocytes, 60%; reactive lymphocytes, 8%; eosinophils, 2%); platelets, 988,000/mm. On admission erythrocyte sedimentation rate was 125 mm/h and C-reactive protein level was 22.3 mg/dl (normal range 0–0.5), respectively. Since the patient had persistent fever for 19 days despite antimicrobial therapy, echocardiography was performed. Twodimensional echocardiography revealed saccular aneurysm at left main coronary artery with a diameter of 6 mm (Fig. 1). Intravenous immunoglobulin (IVIG) (2 g/kg per total dose) and high-dose (100 mg/kg per day, divided into 4 doses/day) acetylsalicylic acid was applied to the patient with diagnosis of iKD and her fever recovered on the fourth hour of IVIG administration. Periungual desquamation of the Wnger tips emerged 1 day after the IVIG therapy. He was discharged from the hospital with low-dose acetylsalicylic M. M. Yilmazer (&) · T. Mese · S. Demirpençe · B. Guven · T. Öner Department of Pediatric Cardiology, Behcet Uz Children’s Hospital, Alsancak, 35210 Izmir, Turkey e-mail: drmuratmuhtar@hotmail.com

An observational study on peripheral blood eosinophilia in incomplete Kawasaki disease

OBJECTIVE To investigate the peripheral blood eosinophilia (PBE) in the acute stage of incomplete Kawasaki disease (iKD). METHODS Twenty-four patients with iKD (median age; 31.5 months, range; 7-88 months) and 25 with complete Kawasaki disease (cKD) (median age; 37 months, range; 9-140 months) were evaluated between 2004 and 2010 from İzmir Dr. Behçet Uz Childrens Hospital records retrospectively. We determined the eosinophil counts and rates from the complete blood count in two study groups before the IVIG treatment and 30 febrile age-matched controls and 30 control cases with congenital heart disease (control Group 1 and 2 respectively). Kruskal-Wallis test was performed in detecting the differences of eosinophil rates and counts between four subgroups. RESULTS In iKD group, the mean value of eosinophil rates and median value of eosinophil counts were 4.39±2.5% and 377 cells/mm(3), respectively, which did not significantly different with cKD group (mean eosinophil rates; 5.47±4.8% and median eosinophil counts 525 cells/mm(3)) (p>0.05). The median values of eosinophil cell counts and mean value of eosinophil rates were 220 cell/mm(3) and 2.83±2.65% in the control group 1 and 165 cell/mm(3) and 1.63±1.43% in the control Group 2 respectively, which were statistically significant lower compared to both study groups (p< 0.001). CONCLUSION The rate of PBE was found significantly higher in iKD patients compared to the controls. Since the diagnosis of iKD is difficult, unexplained eosinophilia may be helpful in the presence of suggestive clinical findings of KD.

Predictive Factors for Determining the Clinical Severity of Pediatric Scorpion Envenomation Cases in Southeastern Turkey

OBJECTIVE The aim of this study was to define the epidemiological, clinical, and laboratory manifestations of scorpion envenomation and to identify factors that are predictive of severe cases. METHODS The medical files of 41 scorpion envenomation cases were reviewed retrospectively. The cases were classified as mild-moderate or severe. The epidemiological, clinical, and laboratory findings of patients were recorded. RESULTS There were 27 patients (65.9%) in the mild-moderate group and 14 patients (34.1%) in the severe group. The median age of all patients was 48 months. The most common systemic finding was cold extremities (41.5%). In all patients, the most commonly observed dysrhythmia was sinus tachycardia (34.1%). Two patients (4.9%) had pulseless ventricular tachycardia and died. Pulmonary edema and myocarditis were observed in 9 patients (22%). Median values of leukocyte and glucose levels were markedly increased in the severe group. Additionally, the mean thrombocyte level (540,857 ± 115,261 cells/mm(3)) in the severe group was significantly increased compared with the mild-moderate group (391,365 ± 150,017 cells/mm(3)). Thrombocyte levels exhibited a positive correlation with leukocyte and glucose values and a negative correlation with patient left ventricular ejection fraction. Multivariate analysis of laboratory parameters indicated that the most predictive factor for clinical severity is thrombocytosis (odds ratio 23.9; 95% CI: 1.6-353.5, P = .021). CONCLUSIONS Although our results share some similarities with those of other reports, thrombocytosis was markedly increased in the severe group and served as the most predictive laboratory factor of clinical severity.

Mid-term assessment of cardiac autonomic functions in children with transposition of the great arteries after arterial switch operation.

OBJECTIVE It has been documented that impaired heart rate variability (HRV) is related to life threatening arrhythmias in children with surgically repaired congenital heart disease. We aimed to analyze the balance of the cardiac autonomic functions by assessing HRV in children with arterial switch operation (ASO). METHODS In this observational cohort study, HRV analysis using 24-h Holter electrocardiography recordings was examined in 22 patients (mean age: 59.5±38.7 months, 18 male, 4 female) who had undergone ASO during the newborn period and 22 healthy children (mean age: 65.1±39.4 months, 18 male, 4 female). After Kolmogorov-Smirnov testing for normality, Student t-test and Mann-Whitney U test were used when appropriate. Chi-square was used for categorical data. RESULTS In 24-h HRV analysis showed that SDANN and VLF were significantly higher in patient group. Awake SDNN, rMSSD, pNN50, TP and VLF levels of patient group were significantly higher than those of control subjects. Awake LF/HF ratio in patient group was significantly higher than their counterpart in asleep group. In the patient group, awake rMSSD, pNN50, TP, LF and HF were significantly lower than their counterpart in the asleep group. CONCLUSION Children with transposition of the great arteries (TGA) following ASO have not decreased levels of time and frequency HRV parameters in the mid-term follow-up period. All HRV parameters reflecting vagal tone were increased in the patient group. It is suggested that vagal tone is more predominant than sympathetic tone for children with ASO.

Arterial function and anatomy in obese children with cardiovascular risk factors

Limited data are available related to the effects of cardiovascular risk factors on abdominal arterial stiffness using carotid and brachial artery indices. Therefore, we aimed to determine arterial changes in obese children and investigate any relation with cardiovascular risk factors.

Infracardiac total anomalous pulmonary venous drainage with unusual presentation

Total anomalous pulmonary venous drainage (TAPVD) is a rare entity which forms approximately 0.4 to 2% of all congenital heart disease. The infracardiac type usually involve obstructions on pulmonary venous connections and comprising a quarter of all TAPVD cases. The clinical findings in patients with obstructed infracardiac TAPVD could mimic respiratuary distress of several different etiologies during first hours of life. In this article, we present a case of a neonate with infracardiac type of TAPVD presented with only distinct subcutaneous veins of abdominal and thoracic wall.

Multifocal atrial tachycardia caused by risperidone.

Risperidone, an atypical antipsychotic drug, is one of the most frequently used atypical neuroleptic drugs for the treatment of symptoms of behavioral disorders seen in autism. Although various cardiovascular side effects have been reported with risperidone, to our knowledge, it has not yet been reported that it can also result in multifocal atrial tachycardia. Based on the case reported herein, our aim is to bring awareness that risperidone may cause multifocal atrial tachycardia.

Cardiac Function in Newborns with Congenital Hypothyroidism: Association with Thyroid-Stimulating Hormone Levels.

Objective: The aims of this study were to demonstrate ventricular function changes in patients with congenital hypothyroidism and to investigate whether there is an association between any such changes and thyroid-stimulating hormone (TSH) levels using M-mode and Doppler electrocardiography (ECG) and tissue Doppler imaging (TDI). Methods: Twenty-seven patients 5-30 days of age with congenital hypothyroidism who were scheduled to receive L-thyroxine treatment and 20 healthy newborns were included in this study. Twelve-lead ECG and M-mode TDI recordings of the patient and healthy groups were obtained. The patient group was divided into two subgroups according to TSH level (>100 uIU/mL or <100 uIU/mL), which were then compared on all parameters. Results: Decreases were observed in the ejection fraction (EF), shortening fraction (SF), and mitral lateral annulus, mitral septal annulus, and tricuspid lateral annulus systolic velocity (Sa) on TDI, whereas left ventricular end-systolic diameter (LVESd) and corrected QT interval (QTc) dispersion were significantly increased in the patient group compared with the control group. No significant differences between the groups were found in left ventricular end-diastolic diameter (LVEDd) or heart rate. When the two patient subgroups (TSH >100 uIU/mL and <100 uIU/mL) were compared, TDI septal annulus Sa wave length and heart rate were significantly lower in the TSH >100 group. Conclusion: Impairment in left ventricular systolic function and increased risk of arrhythmia were observed in newborn infants with congenital hypothyroidism. TSH level was associated with heart rate and interventricular septum velocity.

Low iron storage in children with tilt positive neurally mediated syncope

BackgroundThe mechanisms under neurally mediated syncope (NMS) are not fully understood. This study aimed to assess the level of storage iron in children with different hemodynamic patterns in head-up tilt test.MethodsAltogether 210 children (11.31±2.49 years) with syncope or pre-syncope treated between May 2008 and September 2010 were studied prospectively. Following history taking and physical examination, their levels of hemoglobin (Hb), hematocrit (Hct) and serum ferritin were measured.ResultsIn the 210 children, 162 (77.1%) had NMS and 48 (22.9%) had syncope due to other causes. In the 162 children with NMS, 98 children were subjected to positive tilt test. The level of serum ferritin was significantly lower in the 98 children with NMS (P<0.001). The comparison of levels of Hb, Hct and mean cell volume (MCV) displayed no significant difference between the two groups. Reduced iron storage (serum ferritin <25 ng/mL) was found to be more prevalent in children with NMS (63% vs. 20%, P<0.001). Prevalence of iron deficiency was also significantly higher in children with NMS than in children with syncope due to other causes (27% vs. 6%, P=0.003).ConclusionsIn head-up tilt test positive children with NMS, the level of serum ferritin should be evaluated. Low storage iron may be one of the underlying mechanisms of NMS.