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.

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.

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.

The successful non-invasive management of pulmonary thromboembolism in a child with acute lymphoblastic leukemia.

In childhood, pulmonary thromboembolism (PTE) is an uncommonbut potentially life-threatening disease. The greater numbers of venous thromboembolism (VTE) are complications of underlying risk factors such as malignancies, chemotherapy (L-asparaginase), and central venous catheter. We report a patient with acute lymphoblastic leukemia and PTE, who presented with near-syncope, and was successfully treated with low molecular weight heparin and calcium channel blockers.

Éxito en el manejo no invasivo de la tromboembolia pulmonar en un niño con leucemia linfocítica aguda / The successful non-invasive management of pulmonary thromboembolism in a child with acute lymphoblastic leukemia

Durante la niñez, la tromboembolia pulmonar (TEP) es una afección poco frecuente, aunque potencialmente mortal. El mayor número de episodios de tromboemblia venosa (TEV) es resultado de complicaciones de factores de riesgo subyacentes, tales como tumores malignos, quimioterapia (L-asparaginasa) y colocación de un catéter venoso central. Presentamos el caso de un paciente con leucemia linfocítica aguda y TEP que tuvo un presíncope y fue tratado satisfactoriamente con heparina de bajo peso molecular y antagonistas del calcio.

In childhood, pulmonary thromboembolism (PTE) is an uncommonbut potentially life-threatening disease. The greater numbers of venous thromboembolism (VTE) are complications of underlying risk factors such as malignancies, chemotherapy (L-asparaginase), and central venous catheter. We report a patient with acute lymphoblastic leukemia and PTE, who presented with near-syncope, and was successfully treated with low molecular weight heparin and calcium channel blockers.

Cementless total hip arthroplasty in developmental dysplasia of the hip with end stage osteoarthritis: 2-7 years' clinical results.

Between 2006 and 2011, 102 hips of 78 patients with end-stage osteoarthritis secondary to developmental dysplasia of the hip (DDH) underwent cementless total hip arthroplasty (THA). According to the Crowe's classification, 22 hips (21%) were type 1, 19 hips (18%) were type 2, 22 hips (21%) were type 3 and 39 hips (38%) were type 4 respectively. Functional and clinical analyses were performed by Harris Hip Scores (HHS). There were 73 (71%) excellent or good results according to HHS. The postoperative HHS was significantly lower in patients who underwent femoral shortening (p<0.01). We observed 25 (24.5%) complications in total, 15 (14.7%) of which required revision surgery. The authors concluded that THA for DDH is a safe and a reliable procedure with good clinical outcomes.

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.

Outcomes for revision total knee replacement after unicompartmental knee replacement.

OBJECTIVE: The aim of this retrospective, observational study was to describe the outcomes of total knee replacement (TKR) after failed Oxford phase 3 medial unicompartmental knee replacement (UKR). METHODS: The study included 24 revision TKRs (20 females, 4 males; mean age: 61 years) performed following failed aseptic UKR. Outcomes were assessed using the Knee Society Score (KSS). RESULTS: The most common causes for revision were mobile bearing dislocation and unexplained pain. Mean preoperative KSS was 50.3 (range: 37 to 66) and 82.2 (range: 58 to 97) after TKR. There were 17 excellent, 4 good, 2 fair and 1 poor results. CONCLUSION: The type of UKR performed (cemented versus uncemented) had no effect on TKR success. Revision for failed UKR with TKR appears to be a technically straightforward procedure with satisfactory early clinical results.

Bradycardia related to ribavirin in four pediatric patients with Crimean-Congo hemorrhagic fever.

Crimean-Congo hemorrhagic fever (CCHF) is a potentially fatal fever due to infection with the CCHF virus, which belongs to the genus Nairovirus in the Bunyaviridae family and causes severe disease in humans, with reported mortality rates of 15-70% (Centers for Disease Control and Prevention 1983 ). Humans become infected through the bites of ticks, by contact with blood or tissue from viremic livestock, or by contact with a patient with CCHF during the acute phase of infection (Khan et al. 1997 ). Uysal et al. ( 2012 ) reported bradycardia in a patient with CCHF related to ribavirin treatment. We have observed the same side effect in four pediatric patients that also resolved with discontinuation of ribavirin.

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.

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.

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.

Cemented versus Uncemented Oxford Unicompartmental Knee Arthroplasty: Is There a Difference?

Purpose. The use of uncemented unicompartmental knee prostheses has recently increased. However, few studies on the outcomes of uncemented unicompartmental knee prostheses have been performed. The purpose of this study was to compare the outcomes of cemented and uncemented Oxford unicompartmental knee arthroplasty. Materials and Methods. This retrospective observational study evaluated the clinical and radiological outcomes of 263 medial Oxford unicompartmental prostheses (141 cemented, 122 uncemented) implanted in 235 patients. The mean follow-up was 42 months in the cemented group and 30 months in the uncemented group. Results. At the last follow-up, there were no significant differences in the clinical results or survival rates between the two groups. However, the operation time in the uncemented unicompartmental knee arthroplasty group was shorter than that in the cemented unicompartmental knee arthroplasty group. In addition, the cost of uncemented arthroplasty was greater. Conclusion. Despite the successful midterm results in the uncemented unicompartmental knee arthroplasty group, a longer follow-up period is required to determine the best fixation mode.

The assessment of time-dependent myocardial changes in infants with perinatal hypoxia.

OBJECTIVE: The aim of the study was to assess myocardial damage in infants due to perinatal hypoxia. METHODS: The findings of 29 infants with perinatal hypoxia and 20 healthy infants were compared. Blood gas analysis, serum lactate, cardiac troponin I (cTnI), troponin T (cTnT), creatine kinase-MB (CK-MB) and B-type natriuretic peptide (BNP) were evaluated. Echocardiography together with tissue Doppler imaging was performed. RESULTS: cTnT, CK-MB and BNP were higher in patients at the first day. There were positive correlations between the left ventricular (LV) myocardial performance index (MPI) and cTnT at first day and also at first month. LV ejection fraction and fractional shortening were lower at first day and at first month in patients. Myocardial systolic (Sm) and diastolic (Em and Am) velocities at all segments were lower at first day, and interventricular septum Sm, LV Sm, LV Em, right ventricular Em and LV Am were still lower at first month in patients. Isovolumic relaxation time at all segments together with LV MPI was higher at first day, ejection time values were lower and MPI values were higher at all segments at first month in patients. CONCLUSIONS: These findings demonstrated that the signs of myocardial damage due to perinatal hypoxia still present at first month.

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.