Aida Koka

The frequency of pulmonary hypertension in patients with juvenile scleroderma

Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

A 17-year experience with ALL-BFM protocol in acute lymphoblastic leukemia: prognostic predictors and interruptions during protocol.

Acute lymphoblastic leukemia (ALL) is the most common childhood cancer and despite the intense combination chemotherapy, cure rates are less than 90%. Several prognostic parameters, including nonneoplastic hematologic cell counts during induction phase, are suggested to predict outcome in ALL. We analyzed 242 ALL patients treated in our center to investigate individual prognostic parameters and the impact of delays on disease outcome. Age at diagnosis, risk groups, extramedullary involvement, t(9;22), prednisone response, bone marrow response at days 15 and 33, day 15 platelet count, day 33 lymphocyte, monocyte, and platelet counts, treatment delay, sepsis, and omission of day 64 cyclophosphamide were valuable predictors of survival in univariate analysis. However only the age, CNS involvement, omission of cyclophosphamide, and total delay during treatment were associated with survival in multivariate analysis. Omission of second cyclophosphamide dose had no impact on survival of standard risk group patients, but adversely affected the long term survival of medium risk group (MRG) patients. The second dose might be given with the first dose on day 36 to MRG patients to prevent delays. Day 15 and 33 platelet counts are promising predictors of survival in low income countries where assessment of minimal residual disease is difficult, but this data needs further consolidation.

Our experience in the diagnosis and treatment of postural orthostatic tachycardia syndrome, vasovagal syncope, and inappropriate sinus tachycardia in children

OBJECTIVES The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded. Tilt table test, 24-hour Holter rhythm monitoring, and exercise test were performed, as required. Patients were diagnosed as vasovagal syncope, postural orthostatic tachycardia syndrome (POTS), or inappropriate sinus tachycardia based on these findings. Treatment of the patients was evaluated. RESULTS Thirty patients were diagnosed as vasovagal syncope, 7 patients as POTS, and 2 as inappropriate sinus tachycardia. POTS accompanied Raynauds phenomenon in 1 patient, hypertrophic cardiomyopathy in 1 patient, and homocystinuria in another patient. Complaints of patients with vasovagal syncope improved with non-medical therapy. Medical treatment was administered to the patients with diagnosis of POTS and inappropriate sinus tachycardia. CONCLUSION In patients with complaints of syncope, pre-syncope, dizziness, and palpitations without structural heart disease or non-rhythm problems, cardiovascular autonomic disorders, such as POTS and inappropriate sinus tachycardia should be kept in mind, as well as vasovagal syncope.

Erratum to: Evaluation of cardiac functions in juvenile systemic lupus erythematosus with two-dimensional speckle tracking echocardiography

The aim of this study was to investigate subclinical systolic and diastolic dysfunction in juvenile-onset systemic lupus erythematosus (j-SLE) patients with speckle tracking echocardiography (STE) and the effects of disease activity on left ventricular (LV) regional functions. Thirty-five patients with j-SLE and 30 healthy children (control group) were evaluated between January and August 2015. STE was performed on all patients and controls. Medical records, including diagnosis criteria, age at diagnosis, and duration of disease, were evaluated. SLE disease activity was assessed using the SLE Disease Activity Index (SLEDAI). j-SLE patients had lower ejection fraction than did control subjects but still within normal range. LV end-diastolic and end-systolic dimensions were significantly larger in j-SLE patients (32.43 ± 3.2 vs 28.3 ± 3.1 and 21.1 ± 1.9 vs 18.9.0 ± 2.2, respectively; p = 0.001). There was a significant reduction in longitudinal strain of LV segments in the j-SLE patients compared with controls. J-SLE patients were further divided into subgroups. Group 1 comprised patients having SLEDAI scores >8 at the onset of disease but who improved with therapy during follow-up. Group 2 included j-SLE patients with SLEDAI scores >8 at diagnosis and persistently >4 at the end of follow-up. In the LV mid-inferior and mid-inferolateral segments, STE strain measurements of group 2 were significantly lower than those of group 1 (15.9 ± 6.4 vs 20.0 ± 4.4, 17.9 ± 7.2 vs 23.2 ± 3.8; p = 0.075, p = 0.055, respectively). Simple and non-invasive STE would be helpful in predicting cardiovascular prognosis with new therapeutic medications/interventions or in objectively comparing the effects of immunosuppressive drugs in comparison with preceding STE evaluation.

Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (jSLE)

Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (mPAP) in these patients. A total of 38 jSLE patients and 40 sex- and age-matched controls were retrospectively analyzed. All patients underwent combined M-mode, cross-sectional echo, and Doppler Echo examination. The RV function was significantly impaired in jSLE patients compared to controls. PASP and mPAP were normal in 37 out of 38 patients (97.37%), however, the mean values of PASP and mPAP were significantly higher in jSLE patients compared to controls (26.90 mmHg versus 21.71 mmHg and 12.63 mmHg versus 9.89 mmHg, respectively) [p < 0.05]. Only one patient (2.6 %) had elevated mPAP (60 mmHg). The right cardiac catheterization confirmed PAH in this patient. Although PAH was detected only in one patient, there was a marked increase of PAP in our jSLE patients. Overall, PASP and mPAP were significantly higher in jSLE patients compared to healthy controls. Prospective studies with ethnically diverse cohorts could give more insight on the relevance of PAP and PHT in patients with jSLE.

A case with right atrial appendage aneurysm and hydrops foetalis

Right atrial appendage aneurysm is a very rare malformation that may be congenital or acquired (Gulati et al. 2011). Congenital-type atrial appendage aneurysms are thought to develop due to dysplasia of muscular structure in the atrium wall, while the acquired type generally develops as a post-traumatic lesion (Tejero-Hernandez et al. 2012). Although it may be diagnosed during the neonatal period, most cases are diagnosed in the third or fourth decades of life. Right atrial appendage aneurysm can be asymptomatic or can cause supraventricular arrhythmia and thromboembolic events (Ishii et al. 2012). This study presents a patient with a right atrial appendage aneurysm diagnosed via foetal echocardiography that was referred due to hydrops foetalis at the 20th week of gestation.

SAT0254 The Iceberg in Juvenile Onset Systemic Lupus Erythematosus: Subclinical Deterioration of Cardiac Functions Assessed with Two-Dimensional Speckle Tracking Echocardiography and Contributing Factors of Systolic Dysfunction

Background Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by vasculitis and inflammation in various organs. Cardiovascular involvement, although not frequent in juvenile-onset SLE (j-SLE), if present, is a significant cause of morbidity and mortality (1). Particularly, involvement of the myocardium layer may lead to ventricular dysfunction since its progression is insidious (1,2). Speckle tracking echocardiography (STE) could demonstrate subclinical myocardial deformations (strain) that globally seems normal in conventional echocardiography (2). Objectives The aim of this study is early detection of subclinical systolic dysfunctions in j-SLE with STE and if present, then to investigate whether this is disease-related or it is a result of other predisposing conditions. Methods 35 patients with j-SLE and 30 healthy children as a control group were evaluated between January and August 2015 at outpatient clinics of Cerrahpasa Medical Faculty. STE was performed on all patients and controls. Medical records that are including age at diagnosis, duration of the disease, diagnostic criteria, laboratory tests and cumulative clinical manifestations were evaluated. SLE disease activity was assessed using the SLE Disease Activity Index (SLEDAI). A SLEDAI score >4 was arbitrarily designated as a sign of moderate/severe disease activity. Results j-SLE patients had lower EF values than control subjects. Left ventricular end diastolic dimension (LVEDD) and left ventricular end systolic dimension (LVESD) were significantly greater in j-SLE patients (42,278±4,530 vs. 37,314±5,535; 28,108±3,344 vs. 24,055±3,290 p=0.001, respectively) than in the control group. There was a significant reduction in systolic parameters of longitudinal strain in the j-SLE group (p<0.05) at all segments compared to control patients. SLE patients were divided into two subgroups. Group 1 included patients having SLEDAI scores >8 at the beginning of the disease but who improved with therapy during follow up, with resulting SLEDAI scores less than or equal to 4 points. Group 2 included j-SLE patients with SLEDAI scores >8 at diagnosis but with SLEDAI scores still greater than 4 at the end of follow up. In comparisons of two groups, mid inferior and mid inferolateral LV segment STE strain measurements of Group 2 were significantly lower than those in Group 1, (15.9000±6.47130 vs. 20.0714±4.49725 mid inferior; 17.9000±7.23341 vs. 23.2308±3.87629 mid inferolateral, p=0.075, 0.055, respectively). Conclusions We can say that prevention of long-term cardiovascular complications in j-SLE begins with noticing the iceberg early, before irreversible changes take place. In this regard, STE is an accurate method for detecting subclinical systolic dysfunction. References Apte M, McGwin G Jr, Vilá LM, Kaslow RA, Alarcόn GS, Reveille JD. Associated factors and impact of myocarditis in patients with SLE from LUMINA, a multiethnic US cohort. Rheumatology (Oxford) 2008;47: 362–367. Huang BT, Yao HM, Huang H. Left ventricular remodeling and dysfunction in systemic lupus erythematosus: A three-dimensional speckle tracking study. Echocardiography 2014; 31: 1085–1094. Disclosure of Interest None declared

THU0224 Pulmonary Arterial Hypertension in Patients with Juvenile Lupus Erythematosus

Background Juvenile systemic lupus erythematosus (jSLE) is a chronic autoimmune disease characterized with multisystemic involvement. Vital organ involvement is the most important morbidity and mortality factor. Early diagnosis of cardio-vascular and pulmonary involvement is shown to be relevant in term of reducing mortality. Objectives The aim of this study is to explore the frequency of pulmonary hypertension in patients with jSLE, using non-invasive methods (Doppler echocardiography). Methods Patients with diagnosis of jSLE followed up at our department were included in the study. Doppler echocardiography was performed at all included patients. Pulmonary arterial pressure was determineted by three different methods. Systolic pulmonary arterial pressure (SPAP) was estimated by measurement the maximal velocity of tricuspid insufficiency (TI). Diastolic pulmonary arterial pressure (DPAP) was estimated by velocity of pulmonary insufficiency at the end of diastole (PI). Mean pulmonary arterial pressure (MPAP) was determinated by pulmonary insufficiency acceleration time (AT) and the ration of AT to ejection time (ET). Systolic pulmonary arterial pressure was calculated by modified Bernoulli equation. Right atrial pressure was evaluated by measurement the right atrial volume, the degree of tricuspid regurgitation and contraction of vena cava inferior during inspiration Results A total of 38 jSLE patients were included in the study. Mean age of patient was 16±2,59 year, mean age at diagnosis was 10,6±3,5 year and mean disease duration was 57±33,6 month. Velocity of pulmonary insufficiency at the end of diastole (PI) was 1,469±0,295 m/sec and 1,214±0,128 m/sec in patients and control group, respectively. Maximal velocity of tricuspid insufficiency (TI) was 2,340±0,277 m/sec and 2,044±0,411 m/sec in patients and control group, respectively.The ration of pulmonary insufficiency acceleration time (AT) to ejection time (ET) was normal in both patients and in control group. Although estimated pulmonary arterial pressure was found to be in normal ranges (<25 mmHg) in all studies patients, it was statistical significantly higher comparing to control group. According to those results, there were no findings of pulmonary hypertension among patients with jSLE. Conclusions This study confirms that pulmonary hypertension is uncommon among patients with jSLE. Early diagnosis, regular follow up and proper therapy are thought to be important in reducing the risk of pulmonary hypertension. References Kasparian A, Floros A, Gialafos E, Kanakis M, Tassiopoulos S, Kafasi N, et al. Raynauds phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus. Lupus 2007;16:505–8. Prabu A, Gordon C. Pulmonary arterial hypertension in SLE: what do we know? Lupus 2013;22:1274–85. Min HK, Lee JH, Jung SM, Lee J, Kang KY, Kwok SK, et al. Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival. Korean J Intern Med 2015;30:232–41. Kamel SR, Omar GM, Darwish AF, Asklany HT, Ellabban AS, et al. Asymptomatic pulmonary hypertension in systemic lupus erythematosus. Clin Med Insights Arthritis Musculoskelet Disord. 2011;4:77–86. Disclosure of Interest None declared

THU0223 Demographic and Clinical Characteristics of Patients with Juvenile Scleroderma-A Single Center Experience

Background Juvenile scleroderma (JS) is a rarely seen chronic connective tissue disorder. According to organ involvement, the disease is divided into two main forms: systemic and localized. Localized scleroderma (JLS) is characterized with sclerosis of the skin but internal organs involvement is not expected. Juvenile systemic sclerosis (JSS) is characterized by both cutaneous and internal organ involvement. Clinical features are insidious and it can take years until complete clinical picture develops. Objectives The aim of the study was to investigate main demographic and clinical characteristics of patients with JS, followed up at our department. Additionally, we aim to share our experience of this rare condition. Methods Patients with JS were included in cross-sectional study. Demographic data were taken from disease history of patients. Clinical features, laboratory results and treatment options were evaluated at last clinical visit. Results A totally of 46 patients were included in the study: 40 (86,9%) female, 6 (13%) male. The mean age of patients was 14±3 years, mean age at disease onset was 9,1±4,23 years and the mean age at diagnosis was 10,2±3,4 years. Twenty six patients (56,5%) were diagnosed with JSS and 20 (43,4%) had JLS. Skin involvement and peripheral vasculopathy were the most common clinical features. Musculoskeletal involvement takes a second place. Gastro-intestinal (GIS) involvement was present only in systemic sclerosis group. Six patients (13%) had lung fibrosis. Pulmonary hypertension was found in 6,5% of all JS patients but its percentage was higher among patients with JSS (11,5%). None of patients had neurological and renal involvement. All JSS patients were positive while 40% of JLS patients were negative for ANA. Anti Scl 70 antibody was positive only among patients with JSS. Combination of MTX+corticosteroids was used in 15 patients (32,6%). Vasoactive agent was added in therapy of 20 patients (43,5%) which all belong to JSS group. While 31 patients were in remission (67,4%), 15 (32,6%) had active disease in last visit. All patients with active disease belonged to systemic group. Conclusions Juvenile scleroderma is rarely seen multisystemic disease. Unlike adults, cardiovascular and pulmonary involvement is uncommon among children. Early diagnosis, regular follow up and appropriate treatment are of high importance in clinical course and disease prognosis. References Zulian F, Cuffaro G, Sperotto F. Scleroderma in children: an update. Curr Opin Rheumatol 2013;25:643–50. Foeldvari I. New developments in juvenile systemic and localized scleroderma. Rheum Dis Clin N Am 2013;39: 905–20. Hedrich CM, Fiebig B, Hahn G, Suttorp M, Gahr M. Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease. Clin Pediatr 2011;50: 604–14. Adrovic A, Oztunc F,Barut K, Koka A, Gojak R, Sahin S, et al. The frequency of pulmonary hypertension in patients with juvenile scleroderma. Bosn J Basic Med Sci. 2015;15:30–5. Disclosure of Interest None declared

The frequency of pulmonary hypertension in juvenile scleroderma

Juvenile scleroderma (JS), represents a rarely seen group of connective tissue disease with multiple organ involvement. Although quite rare in childhood, cardio-vascular and pulmonary involvements are the most important mortality and morbidity factors. Pulmonary arterial hypertension (PAH), the most important sequelae of pulmonary involvement, could be determined by echocardiographic examinations. Early cardio-vascular and pulmonary involvement determination is extremely important in reducing mortality of patients.