Effects of vagus nerve stimulation on heart rate variability in children with epilepsy.

PURPOSE: The aim of this study was to evaluate the effects of vagus nerve stimulation (VNS) on heart rate variability (HRV) in children with epilepsy. METHODS: The subgroups of HRV, namely time domain (Standard deviation of NN interval (SDNN), SDNN index, Standard deviation of the averages of NN intervals (SDANN), Root mean square of successive differences (RMMSD), Adjacent NN intervals differing by more than 50 ms in the entire recording divided by the total number of all NN intervals (PNN50), triangular index) and frequency domain (Low-frequency (LF), High-frequency (HF), LF/HF), were investigated in 20 pediatric patients before and after 6 and 12months of VNS treatment during day and night by comparing their data with those of 20 control subjects. In addition, subgroups of age, epilepsy duration and localization, and antiepileptic drugs (AEDs) were also evaluated if they had further effects on basal HRV levels. RESULTS: Increased heart rates (HRs); decreased SDNN, SDANN, RMMSD, and PNN50; and increased LF/HF ratios were identified before VNS therapy (p<0.05). Even though remarkable improvement was seen after 6months of VNS treatment (p<0.05), no further changes were observed in 12-month compared with 6-month levels (p>0.05) in all parameters, still even significantly lower than those of controls (p<0.05). Longer duration of epilepsy and localization of epileptic focus, such as in the temporal lobe, were also found to further contribute to diminished basal HRV levels (p<0.05). CONCLUSION: The cardiovascular system is under deep sympathetic influence in children with epilepsy. Although VNS seems to provide a substantial improvement by achieving increased parasympathetic effects in short-term therapy, the levels were still lower than those of healthy children after either short- or long-term therapy. Therefore, impaired cardiovascular autonomic regulation may be associated with the epileptic process itself as well as with the contribution of some additional factors. Overall, different aspects such as age, epilepsy duration, epileptic focus, seizure frequency, and AEDs should also be considered for their further possible effects on HRV during VNS therapy.

Focal Nodulary Hyperplasia of the Liver Due to Congenital Portosystemic Shunt: A Rare Condition Mimicking Hepatocellulary Carcinoma.

Congenital portosystemic shunts are rare vascular malformations that lead to several complications including liver tumors, pulmonary hypertension, and metabolic encephalopathy. We describe a rare case of a 17-year-old girl with an extrahepatic portosystemic shunt presenting recurrent syncope episodes and a liver mass mimicking hepatocellulary carcinoma.

Evaluation of cardiotoxicity by tissue Doppler imaging in childhood leukemia survivors treated with low-dose anthracycline.

Improvement in long-term survival in patients with acute childhood leukemia has led to the need for monitorization of chemotherapy-related morbidity and mortality. This study included 60 patients with acute lymphoblastic leukemia that were in remission for at least 2 years and 30 healthy controls. Systolic and diastolic function of myocardium was evaluated using conventional echocardiography and tissue Doppler imaging of the left ventricle, interventricular septum and right ventricle. Median age of patients was 11.7 years (range 10-14.9 years), and the median duration of remission was 4 years (range 2.5-5 years). All patients were treated with a low cumulative dose of adriamycin (100 mg/m(2)) according to the St. Jude Total-XIIIA protocol. The ejection fraction (EF) and fractional shortening were normal in the patient and control groups, even though EF values were significantly lower in the patients (69.5 ± 2.3 vs. 72.7 ± 3 %, P < 0.01). Myocardial systole (S m), early diastole (E m) and late diastole (A m) velocities in all segments of the myocardium were significantly lower in the patient group (P < 0.01 for all segments). Cardiotoxicity was noted in all segments of the myocardium in the patient group, despite the fact that they were all treated with a low cumulative dose of adriamycin. Based on these findings, we think that there is no safe dose for anthracyclines and periodic echocardiographic evaluation of both the left and right ventricles must be performed in all patients treated with anthracyclines, even at low doses.

Three-dimensional echocardiographic evaluation of a patient with double-orifice mitral valve, bicuspid aortic valve, and coarctation of aorta.

A 16-year-old male patient was diagnosed with bicuspid aortic valve and coarctation of aorta, and had a history of a subclavian patch plasty operation at 1 year of age. In addition, we detected double-orifice mitral valve (DOMV) at a routine evaluation and demonstrated by both two-dimensional and three-dimensional (3D) echocardiographic examinations. DOMV should be kept in mind in cases with left ventricular obstructive lesions and 3D echocardiography may provide a more detailed assessment of mitral valve and subvalvular apparatus.

Native aortic valve endocarditis complicated by pseudoaneurysm of mitral-aortic intervalvular fibrosa.

Pseudoaneurysm of mitral-aortic intervalvular fibrosa (PA-MAIVF) is a rare complication of native aortic valve endocarditis. This region is a relatively avascular area and prone to infection during endocarditis and subsequent aneurysm formation. The rupture into the pericardial cavity and left atrium or aorta, systemic embolism, myocardial infarction secondary to left coronary compression, and sudden death are the reported complications. Herein, we present a 9-year-old boy who was diagnosed with bicuspid aortic valve endocarditis complicated by PA-MAIVF, cerebral embolism, and hemorrhage. PA-MAIVF was visualized by both two- and three-dimensional transthoracic echocardiography and ruptured into pericardial space causing a fatal outcome.

Evaluation of cardiac functions and atrial electromechanical delay in children with adenotonsillar hypertrophy.

Deterioration of the right ventricular (RV) functions and the increase in the pulmonary arterial pressure (PAP) of children with moderate to severe adenotonsillar hypertrophy (ATH) have been well described. In addition to these complications, this study aimed to investigate the influence of ATH on the conduction system. The study investigated 46 patients with a diagnosis of ATH and 46 healthy control subjects. Conventional echocardiography, P-wave dispersion (Pd), tissue Doppler imaging (TDI) findings, and atrial electromechanical delay (AED) were compared between the patients and the control subjects before and after adenotonsillectomy. The maximum P-wave duration and Pd were significantly higher in the patients than in the control subjects (p < 0.001). The patient group showed significantly greater RV end-diastolic dimension (p = 0.01), right atrial area (p < 0.001), and mean PAP (p = 0.03) but lower E/A ratios for the mitral (p = 0.04) and tricuspid (p = 0.01) valves and a shorter pulmonary flow trace acceleration time (p = 0.03). The tricuspid annular-plane systolic excursion was similar between these groups (p = 0.21). In the patient group, TDI studies showed significantly lower E'/A' ratios for the tricuspid lateral (p = 0.006) and mitral septal (p = 0.003) segments than in the control group. The myocardial performance index was lower for the mitral lateral, mitral septal, and tricuspid lateral segments in patient group (p < 0.001). Similarly, AED was prolonged in the patient group at all three segments (p < 0.001). Also, the patient group showed a significantly longer interatrial (p = 0.03) and intraatrial (p = 0.04) electromechanical delay. However, all the electro- and echocardiographic parameters were similar between the patients and the control subjects after adenotonsillectomy (p > 0.05). The prolongations in P-wave duration, Pd, and inter- and intraatrial electromechanical delays were first shown in this population. The cardiac changes induced by ATH-associated hypoxia may facilitate arrhythmias during the long term.

The effect of recombinant human growth hormone therapy on left-ventricular chamber size and function in children with growth hormone deficiency.

This study was designed to assess the effect of recombinant human growth hormone (rhGH) therapy on left-ventricular (LV) chamber size and function in children with idiopathic isolated growth hormone deficiency (GHD) using conventional echocardiography and tissue Doppler imaging (TDI). Thirty patients (19 boys and 11 girls) with idiopathic isolated GHD were followed-up for 12 months. Mean age of patients was 11.0 ± 2.6 years (range 6.3-15.5). At baseline and at 3, 6, and 12 months of treatment, the structure of the left ventricle was assessed by conventional echocardiography and myocardial rates and time intervals by TDI. There was a significant increase in LV mass (LVM) compared with pretreatment values. Like LVM, relative wall thickness (RWT) was also increased significantly. The significant increase in LVM indexed to body surface area and RWT became apparent at month 3 of treatment with a significant increase in LVM indexed to height(2.7) at treatment month 6. Normalized LVM increased as early month 3 of treatment, and a steady increase was observed until month 12. However, no patient had LVM > +2 standard deviation scores at month 12 of treatment. No significant differences were observed in functional parameters of the left ventricle and the interventricular septum. The results of this study showed that rhGH therapy causes an increase in myocardial mass without changing the geometry or function of the myocardium. Therefore, the increase in myocardial mass appears to be concentric, thus causing remodeling instead of hypertrophy.

All-transretinoic acid (ATRA) treatment-related pancarditis and severe pulmonary edema in a child with acute promyelocytic leukemia.

Use of all-transretinoic acid (ATRA) with other chemotherapeutic agents in the treatment of acute promyelocytic leukemia (APL) has been shown to cause the differentiation of abnormally granulated specific blast cells into mature granulocytes by acting on the t(15; 17) fusion gene product. The complete remission rate is increased and survival time is prolonged in APL patients who receive chemotherapy plus ATRA, whereas ATRA syndrome and other ATRA-related adverse effects including pseudo tumor cerebri, headache, severe bone pain, mucosal and skin dryness, hypercholesterolemia, and cheilitis may be observed especially during induction phase of the treatment. In this paper, we report a 9-year-old girl with APL who developed pancarditis while receiving the APL-93 treatment protocol. In our patient, endocarditis and myocarditis were initially determined after ATRA treatment during the induction part of the protocol. All findings disappeared after ATRA was discontinued. When ATRA was readministered in the maintenance part of the treatment protocol, she developed pancarditis and severe pulmonary edema. As her symptoms decreased dramatically with the discontinuation of ATRA and the initiation of steroid treatment, the clinical picture strongly suggested the ATRA treatment as the causative factor. To the best of our knowledge, this clinical picture of pancarditis secondary to ATRA treatment has not been reported earlier in the English literature.

Anti-neutrophil cytoplasmic antibodies (ANCA) in serum and bronchoalveolar lavage fluids of cystic fibrosis patients and patients with idiopathic bronchiectasis.

We investigated the presence of anti-neutrophil cytoplasmic antibodies (ANCA) in the serum and bronchoalveolar lavage fluid (BALF) of 21 cystic fibrosis (CF), 7 idiopathic bronchiectasis (IBR), and 11 control children and the relation between ANCA and any bacteria grown in BALF. Six of the CFs, but none of the IBRs or controls had positive serum cytoplasmic or perinuclear-ANCA (c-ANCA, p-ANCA). Serum autoantibodies against bactericidal/permeability increasing protein (BPI-ANCA) were positive in 2 CFs, 1 IBR and 1 control. While none of the CFs, IBRs or controls had positive BALF (c- or p-ANCA), 1 CF, 1 IBR and none of the controls had positive BALF BPI-ANCA. Pseudomonas aeruginosa was not grown in the specimens of any of the subjects. As the number of the patients in our study was very limited, further longitudinal and well-designed studies are necessary to show whether or not the presence of ANCA in serum or BALF relates to the presence of P. aeruginosa infection in the airways of CF and IBR patients.

Figulla ASD occluder versus Amplatzer Septal Occluder: a comparative study on validation of a novel device for percutaneous closure of atrial septal defects.

OBJECTIVES: Occlutech Figulla ASD Occluder (FSO) is an alternative device to Amplatzer Septal Occluder (ASO) with some structural innovations including increased flexibility, minimizing the amount of material implanted, and absence of the left atrial clamp. We aimed to report our experiences with FSO and compare the outcomes of this novel device versus ASO. INTERVENTIONS: Between December 2005 and February 2009, 75 patients diagnosed with secundum atrial septal defects underwent transcatheter closure. The FSO device was used in 33 patients, and the ASO was used in 42. RESULTS: Patient characteristics, stretch size of the defect, device left disc size, procedure, and fluoroscopy time were similar between the groups. However, the difference between device waist size and stretched diameter of the defect was significantly higher, and device delivery sheath was significantly larger in FSO group and device left disc size was significantly lower in the FSO group. In all subjects, the residual shunt was small to trivial during follow-up and the reduction in prevalence of residual shunt with time was similar in both groups (P = 0.68). We found no differences in complication rate between the two devices; however, device embolization to the pulmonary bifurcation in one patient was recorded as major complication in FSO device group. CONCLUSIONS: Both devices are clinically safe and effective in ASD closure. FSO device has similar outcomes when compared to ASO device. Difficulties in selecting the correct device size in larger defects and larger venous sheath requirement need to be evaluated in further studies.

Common trunks of the subclavian and the vertebral arteries: presentation of a new aortic arch anomaly.

Information about the variations of the origins of subclavian and vertebral arteries and their course is of great importance for head and neck surgery. The normal vertebral arteries arise as the first branches of the corresponding subclavian arteries. In a minority of cases, the left vertebral artery could arise directly from the aortic arch between the left common carotid and the left subclavian arteries. An aberrant right subclavian artery arising as the last branch of the aortic arch is also a frequently seen aortic arch anomaly. Here, we present a case with common trunks of the subclavian and vertebral arteries, demonstrated by cardiac catheterization.

Evaluation of right ventricular function by using tissue Doppler imaging in patients after repair of tetralogy of fallot.

BACKGROUND: The aim of this study was to assess the relation between plasma B-type natriuretic peptide (BNP) levels and right ventricular function evaluated by tissue Doppler imaging (TDI) in patients after repair of tetralogy of Fallot (ToF). METHODS: Twenty-five patients with a mean age of 14.1 +/- 4.4 years who underwent repair of ToF at a mean age of 4.9 +/- 5.1 years enrolled in this study. The control group consisted of 29 healthy children at a mean age of 13.1 +/- 2.8 years. The right ventricle and pulmonary regurgitation (PR) were assessed by two-dimensional echocardiography and color Doppler. Blood samples for BNP levels were taken and TDI was performed at rest. RESULTS: Plasma BNP levels were significantly higher in patients than in controls (28.3 +/- 24.1 vs. 7.4 +/- 2.3 pg/mL, P = 0.0001). The myocardial performance index (MPI) (1.08 +/- 0.35 vs. 0.58 +/- 0.11, P = 0.0001) was higher and isovolumic acceleration (IVA) (3.1 +/- 0.7 vs. 5.4 +/- 1.0 m/s(2), P = 0.0001) was lower in patients. The correlations were also significant between the degree of PR and MPI (r = 0.7, P = 0.0001) and also IVA (r =-0.7, P = 0.0001). The correlations were also significant between the BNP level and MPI (r = 0.6, P = 0.0001), IVA (r =-0.4, P = 0.002) and the degree of PR (r = 0.6, P = 0.0001). CONCLUSION: As a result, plasma BNP level increases in patients with ToF and both MPI and IVA from the right ventricular basal segments might be used to assess the right ventricular function.

Anomalous pulmonary venous connection to the azygous vein: surgical approach of a case with 3D computed tomography findings.

Echocardiographic evaluation of anomalous pulmonary venous connection (APVC) may be difficult especially in patients with complex congenital heart diseases. Angiography may also be hazardous in sick patients. For this reason, sometimes we need noninvasive and highly demonstrative procedures for the diagnosis and management of APVC. We have presented a patient, operated with the diagnosis of partial APVC to the azygous vein. In this patient the diagnosis was suspected by echocardiography and confirmed by using three-dimensional computed tomography with angiography.

Evaluation of right ventricular functions and B-type natriuretic peptide levels by cardiopulmonary exercise test in patients with pulmonary regurgitation after repair of tetralogy of Fallot.

Impairment of right ventricular functions, especially due to chronic pulmonary regurgitation (PR), is a well-known entity in patients with tetralogy of Fallot (ToF) after repair. The aim of this study was to examine the relation between B-type natriuretic peptide (BNP) levels and right ventricular dysfunction by cardiopulmonary exercise test (ET) in patients after repair of ToF. Twenty-five patients with a mean age of 14.1 +/- 4.4 years at follow-up who underwent repair of ToF at a mean age of 4.9 +/- 5.1 years and 29 age- and sex-matched healthy children at a mean age of 13.1 +/- 2.8 years were enrolled in this study. Plasma BNP levels were measured at baseline and at maximal exercise. The volume of right ventricle (RV) and the degree of PR were assessed by two-dimensional echocardiography and color Doppler. Plasma BNP levels were significantly higher in patients with ToF than in controls (28.3 +/- 24.1 vs 7.4 +/- 2.3 pg/mL, p = 0.0001). Exercise was associated with increased plasma BNP levels in both groups. A greater increase in BNP was noted in patients with ToF than in controls (37.6 +/- 27.5 vs 11.3 +/- 4.5 pg/mL, p = 0.0001). Forced vital capacity (FVC%) (84.9 +/- 16.9 vs 98.4 +/- 18.2, p = 0.01) and forced expiratory volume during the 1st second (FEV1%) (91.5 +/- 19.3 vs 103.8 +/- 16.1, p = 0.02) were decreased, exercise duration (ED) (10.1 +/- 1.9 vs 11.4 +/- 1.7 min, p = 0.02), maximum heart rate (HRmax) (171.2 +/- 18.9 vs 186.4 +/- 13.9 /min, p = 0.004), and maximum oxygen uptake (VO(2)max) (1.56 +/- 0.53 vs 2.1 +/- 0.6 L/min, p = 0.007) were lower in patients with ToF. There were significant correlations between the degree of PR and ED (r =-0.3, p = 0.009), HRmax (r =-0.4, p = 0.001), and VO(2)max (r =-0.4, p = 0.001). The correlations were significant both before and after exercise, being more pronounced after exercise between BNP level and the degree of PR (r = 0.6, p = 0.0001). As a result, the severity of PR has a negative influence on right ventricular functions and there is significant relation between right ventricular functions and exercise capacity after repair of tetralogy of Fallot.

[Midterm results of total knee arthroplasty in degenerative knee joint diseases with severe deformity]. / Ileri derecede deformiteye yol açan dejeneratif diz eklemi hastaliklarinda total diz artroplastisinin orta dönem sonuçlari.

OBJECTIVES: We evaluated the midterm results of total knee arthroplasty in degenerative knee joint diseases with severe deformity. METHODS: Total knee arthroplasty was performed in 125 knees of 86 patients (75 women, 11 men; mean age 69 years; range 39 to 85 years) with degenerative knee joint diseases accompanied by severe deformity. Almost all the patients had osteoarthritis (100 knees, 80%) or rheumatoid arthritis (22 knees, 17.6%). Thirty-nine patients with bilateral involvement underwent single-stage (n=20) or two-stage (n=19) surgery. During surgery, the posterior cruciate ligament was sacrificed in 108 knees and retained in 17 knees. Pre- and postoperative assessments were made according to the Knee Society clinical scoring system. The mean follow-up was 53 months (range 24 to 96 months). RESULTS: The results were excellent or good in 80.2% of the knees. Nine knees (7.2%) required revision surgery, of which preoperative diagnosis was rheumatoid arthritis in five. The reasons for revision were infection-related problems (n=5), aseptic loosening (n=3), and periprosthetic fracture (n=1). Compared with preoperative values, the mean knee score increased by 60.9 (from 26.2 to 87.1), and the mean functional score increased by 42.6 (from 33.2 to 75.8) (p<0.05), with a mean range of knee joint motion of 93.2 degrees . The mean preoperative and postoperative alignments were 17.8 degrees of varus and 4.1 degrees of valgus (range 0 degrees to 6 degrees ), respectively. The number of knees with a flexion contracture decreased from 68 (mean 28.8 degrees ) to 8 (mean 8.4 degrees ), all of which had a flexion contracture angle below 10 degrees . CONCLUSION: Total knee arthroplasty performed in degenerative knee joint diseases with severe deformity significantly improves patients' complaints and functional results.

A rare cause of facial nerve palsy in a young infant: Kawasaki disease.

Orgun A, Karagöl C, Pamuk U, Gürsu HA, Çetin I. A rare cause of facial nerve palsy in a young infant: Kawasaki disease. Turk J Pediatr 2018; 60: 433-435. Kawasaki disease (KD) is a vasculitis in which the most common complication is development of coronary aneurysms. Neurological complications rarely occur in KD patients such as facial nerve palsy (FNP). FNP associated with KD may indicate increased risk of coronary artery aneurysm. Infants with facial nerve paralysis and unexplained-prolonged febrile period should be evaluated with echocardiography. Here in, we present a 4-month-old female with FNP and unexplained fever who was diagnosed KD due to echocardiographic findings.

A rare cause of fatal cardiac arrhythmia: Inhalation of butane gas.

Pamuk U, Gürsu HA, Emeksiz S, Özdemir-Sahan Y, Çetin I. A rare cause of fatal cardiac arrhythmia: Inhalation of butane gas. Turk J Pediatr 2018; 60: 755-756. Butane gas, especially available in lighters, is commonly misused among adolescents and its side effects are rarely observed but serious. A 14-yearold male was brought to our emergency department. The electrocardiographic (ECG) examination showed biphasic T waves in leads V4-V6, and long QTc at 481ms. Echocardiographic study showed left ventricular systolic dysfunction. Troponin I level was found to be high at 9.1 ng/ml. Taking into consideration the patient`s history, clinical and laboratory findings, ventricular fibrillation and myocardial injury resulting from butane gas inhalation was diagnosed.

Assessment of tumors in children with tuberous sclerosis: a single centre's experience.

AIM: As a result of mutations in TSC1 (9q34) and TSC2 (16p13.3) tumor supressor genes, the mammalian target of the rapamycin (mTor) signaling pathway is overactivated in patients with tuberous sclerosis. Abnormal cell proliferation and differentiation is responsible for the growth several different tumors. The aim of this study was to review tumors in our patients with tuberous sclerosis. MATERIAL AND METHODS: Thirty-six patients with tuberous sclerosis were reviewed retrospectively in terms of age, sex, family history, clinical findings, presence of tumors, and treatments. RESULTS: Our study included 36 patients (18/18:M/F) aged between two days and 17 years with a median age of 6 years. There were hypopigmented spots in 30 patients, seizures in 28 patients, and a family history in 11 patients. Tumors related to tuberous sclerosis were renal angiomyolipomas in 21 patients, cardiac rhabdomyomas in 11, subependymal giant cell astrocytomas in seven, and non renal hamartoma in one patient. Everolimus treatment was used in only two patients because of hemodynamic instability. CONCLUSIONS: Tuberous sclerosis is a multisystemic disease characterized by the presence of various benign tumors and neurologic disorders. Renal angiomyolipomas, cardiac rhabdomyomas, and subependymal giant cell astrocytomas are commonly observed in patients with tuberous sclerosis. mTOR inhibitors such as everolimus and sirolimus have been increasingly used in the treatment of these tumors. However, the duration and optimal dose of mTOR inhibitors is still controversial and should be used in selected cases.

[Midterm results of total hip replacement in osteonecrosis of the hip joint]. / Kalça osteonekrozunda total kalça artroplastisinin orta dönem sonuçlari.

OBJECTIVES: We evaluated the midterm results of total hip arthroplasty (THA) for femoral neck osteonecrosis. METHODS: The study included 59 patients (23 females, 36 males; mean age 45.6 years; range 24 to 66 years) who underwent THA in 72 hips. Osteonecrosis was secondary to fracture in the femoral neck in 11 hips (15.3%), was associated with steroid use in 23 hips (31.9%), and was idiopathic in 38 hips (52.8%). Functional evaluations were made with the hip scoring system of D'Aubigne and Postel. Bone-implant relations were assessed radiographically using the Gruen's seven zones in the femur and Charnley's three zones in the acetabulum. The mean follow-up was 4.1 years (range 2 to 7 years). RESULTS: The mean D'Aubigne-Postel hip score was 11.8 (range 7 to 16), with good or excellent results in all hips. While no significant difference was found between cementless and hybrid systems (p>0.05), the mean hip score was significantly higher with press-fit acetabular systems compared to expansion cups (p<0.001). The effects of the following were found insignificant on the clinical outcome (p>0.05): etiology, metal-on-metal or metal-on-polyethylene surfaces, and development of stress shielding in the proximal femur or of varus-valgus angulation in the femoral stem. However, the results were significantly less favorable in cases in which complications arose (p<0.001). Intraoperative complications were fissure in five patients and isolated trochanteric fracture in five patients, all of which occurred in cementless THA. Heterotopic ossification was seen in six hips. At the end of 6.5 years, mild polyethylene wear was detected in eight hips, two of which also had focal osteolysis in Gruen zone 1. CONCLUSION: Thanks to improvements in implant technology and surgical techniques, the results of both cementless and hybrid systems are satisfactory in hip osteonecrosis, regardless of the etiology.