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© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians)Aim: To evaluate the incidence and clinical features of acute rheumatic fever (ARF) in Turkey, following the revised Jones criteria in 2015. Methods: This multicentre study was designed by the Acquired Heart Diseases Working Group of the Turkish Pediatric Cardiology and Pediatric Cardiac Surgery Association in 2016. The data during the first attack of 1103 ARF patients were collected from the paediatric cardiologists between 1 January 2016 and 31 December 2016. Results: Turkey National Institute of Statistics records of 2016 were used for the determination of ARF incidence with regard to various cities and regions separately. The estimated incidence rate of ARF was 8.84/100 000 in Turkey. The ARF incidence varied considerably among different regions. The highest incidence was found in the Eastern Anatolia Region as 14.4/100 000, and the lowest incidence was found in the Black Sea Region as 3.3/100 000 (P < 0.05). Clinical carditis was the most common finding. The incidence of clinical carditis, subclinical carditis, polyarthritis, aseptic monoarthritis, polyarthralgia and Sydenham's Chorea was 53.5%, 29.1%, 52.8%, 10.3%, 18.6% and 7.9%, respectively. The incidences of clinical carditis, subclinical carditis, polyarthritis and polyarthralgia were found to be significantly different among different regions (P < 0.05). Conclusion: The findings of this nationwide screening of ARF suggest that Turkey should be included in the moderate-risk group.

OBJECTIVE: In this study, it was aimed to assess the neurological evaluation of children diagnosed with sydenham chorea.MATERIAL AND METHODS: Children diagnosed with Sydenham's chorea were retrospectively screened and evaluated for demographic, clinical, laboratory, neuroimaging, echocardiographic findings (ECHO), presenting complaints, and response to the treatment.RESULTS: Ten patients with Sydenham chorea were included in the study. Eight of the patients were female (80%), two were male (20%), the female / male ratio was 4 and the mean age at diagnosis was 12.05 ± 2.84 years. Carditis was detected in all cases (100%) at the admission. ECHO examination revealed mitral insufficiency in six cases (60%), mitral insufficiency and aortic insufficiency in four cases (40%). When the cases were evaluated in terms of presenting complaints, seven cases (70%) had choreiform movements; one case choreiform movements and gait disturbance; one patient presented with hemiparesis, dysarthria, and choreiform movements and one patient presented with emotional instability and gait disturbance. Generalized chorea was observed in four cases (40%) and hemicorea in six cases (60%). Four (66.6%) of the cases hadright hemicorea and two had left hemicorea (33.3%). Valproate treatment (10-15 mg / kg / day) was administered to all patients. The duration of the disappearance of choreiform movements ranged from 20 to 90 days (mean 42.50 ± 22.39 days). No side effects were observed in any of the patients. Only one patient had a positive ANA. When the neuroimaging was examined, brain magnetic resonance imaging (MRI) was normal in eight cases (80%) and pathology was observed in two cases (20%). Brain MRI revealed glioticlesions in the periventricular white matter in one case and in the bilateral frontal lobe deep white matter in the other case.CONCLUSIONS: Although the neurological examination is important in the diagnosis of Sydenham's chorea, it should be kept in mind that patients may present with behavioral symptoms before choreiform movements. It can be suggested that valproate is an effective and safe treatment option and non-specific hyperintense lesions seen on brain MRI are the result of inflammation and vasculitis mechanisms., AMAÇ: Bu çalışmada Sydenham koresi tanısı alan çocukların nörolojik açıdan değerlendirilmesi amaçlanmıştır.GEREÇ VE YÖNTEM: Tanı alan hastalar retrospektif olarak taranıp demografik, klinik, laboratuar, nörogörüntüleme, ekokardiyografi (EKO), başvuru yakınmaları ve uygulanan tedaviye yanıtları bakımından değerlendirilmiştir.BULGULAR: Çalışmaya 10 olgu dahil edildi. Hastaların sekizi kız (%80), ikisi erkek (%20), kız/erkek oranı 4, ortalama tanı yaşı 12,05±2,84 yıl olarak saptandı. Başvuru anında olguların hepsinde (%100) kardit tesbit edildi. EKO incelemesinde altı olguda (%60) sadece mitral yetmezlik, dört olguda (%40) ise mitral yetmezlik ve aort yetmezliği mevcuttu. Başvuru yakınmaları açısından değerlendirildiğinde yedi olgu (%70) koreiform hareketler; bir olgu koreiform hareketler ve yürüyüş bozukluğu; bir olgu hemiparezi, dizartri ve koreiform hareketler ve bir olgu da emosyonel instabilite ve yürüyüş bozukluğu nedeniyle başvurdu. Jeneralize kore dört olguda (%40), hemikore altı olguda (%60) izlendi. Olguların dördünde sağ (% 66,6) ve ikisinde sol (%33,3) hemikore bulgusu saptandı. Hastaların tümüne valproat tedavisi (10-15 mg/kg/gün) başlandı. Tedavi ile koreiform hareketlerin kaybolma süresi 20-90 gün (ortalama 42,50±22,39 gün) arasında değişti. Hastaların hiç birinde yan etki gözlenmedi. Yalnızca bir hastanın ANA değeri pozitif olarak saptandı. Olguların sekizinin (%80) beyin manyetik rezonans görüntüleme (MRG) tetkiki normal olup iki olguda (%20) patoloji izlendi. Beyin MRG’de bir olguda periventriküler beyaz cevherde bir olguda ise bilateral frontal lob derin beyaz cevherde gliotik lezyonlar saptandı.SONUÇ: Tanıda nörolojik muayene önemli olmakla beraber hastaların koreiform hareketlerden önce davranışsal semptomlar ile de başvurabileceği akılda tutulmalıdır. Valproatın etkin ve güvenilir bir tedavi seçeneği olduğu ve beyin MRG’de görülen spesifik olmayan hiperintens lezyonların inflamasyon ve vaskulit mekanizmalarının sonucu ortaya çıktığı ileri sürülebilir.

ObjectiveThe aim of this study is to determine the relationship between the cardiac catheterization findings and pulsed-wave (PW) Doppler and Doppler tissue imaging (DTI) in pulmonary arterial hypertension patients with congenital heart disease with intracardiac shunts.

WOS: 000298589000046 PubMed: 21810152 Background: The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying PLSVC. Methods: The present study is based on a retrospective review of 1205 children who consecutively underwent cardiac catheterization from 2000 to 2007. In order to determine the existence of PLSVC, all the subjects routinely underwent superior vena cava (SVC) injection during angiography at the catheter laboratory of the study center. Results: The prevalence of PLSVC was computed to be 6.1% for the present study population. Transthoracic echocardiography was able to detect PLSVC in 32 children (2.6%) whereas angiography diagnosed PLSVC in 74 children (6.1%). The mean age of the patients with PLSVC was 40.09 +/- 50.21 months. A communication between the right and left SVC was determined in 27% of the children who were diagnosed with PLSVC after angiography was performed (20 out of 74). A statistically significant association was present between PLSVC and other congenital cardiac anomalies, including ventricular septal defect (n = 42, 56.8%), atrial septal defect (n = 31, 41.9%), pulmonary stenosis (n = 19, 25.7%), atrioventricular septal defect (n = 10, 13.5%), patent ductus arteriosus (n = 6, 8.1%) and cor triatriatum (n = 3, 4.1%). Conclusion: Transthoracic echocardiography usually visualizes dilated coronary sinus in association with PLSVC. However, SVC injection should be performed in patients undergoing angiography so that morbidity and mortality related with persistent left superior vena cava can be avoided during cardiovascular surgery.