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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.

AMAÇ: Çölyak hastalıklı çocuklarda glutensiz diyetin hematolojik parametreler üzerindeki etkilerini değerlendirmek amaçlanmıştır. GEREÇ VE YÖNTEM: Öncelikle kliniğimizde çölyak hastalığı tanısı almış olan çocuk hastalar belirlenmiştir. Daha sonra retrospektif olarak bu hastalar arasında glutensiz diyeti doğru şekilde uygulayabilmiş hastalar belirlenmiştir. Bu hastaların tanı sırasında ve glutensiz diyetten 6 ay sonra hematolojik parametreleri karşılaştırılmıştır. BULGULAR: Çölyak hastalığı konulmuş 97 çocuk hastadan 66’sı çalışmaya dahil edilmiştir. Hastaların 44’ü kız (%66.7), 22’si erkek (%33.3) olup tanı yaşları ortalama 7.1 ± 4.2 yıl olarak saptanmıştır. Hastalarda en sık saptanan hematolojik bozukluklar, sırasıyla 38 hastada (%57.5) trombositoz ve 28 hastada (%42.2) anemi olmuştur. Anemilerin 24’ü (%36.6) demir eksikliği anemisi, 2’si (%3) folat eksikliği anemisi, 1’i (%1.5) vitamin B12 eksikliği anemisi olarak değerlendirilmiştir. Altı ay devam eden glutensiz diyetten sonra anemi sıklığı 8 hastaya (%13) düşmüştür. Glutensiz diyet sonrasında trombosit sayısı 351.2 ± 11.0 (103/μL)’den 310.5 ± 82.0 (103/μL) değerine, lökosit sayısı 8.82 ± 3.04 (103/μL)’den 8.04 ± 2.56 (103/μL)’ye, ortalama platelet hacmi 10.1 ±1.1 fl’den 9.5 ± 1.2 fl değerlerine düşmüştür. Buna karşılık; hemoglobin, ortalama korpuskuler volüm ve eritrosit dağılım genişliği değerlerinde ise anlamlı derecede artış olduğu görülmüştür. SONUÇ: Çölyak hastası çocuklarda en sık saptanan hematolojik bulgular, trombositoz ve anemi olmuştur. Glutensiz diyet sonrasında anemide düzelme olduğu görülmektedir. Bundan başka, hemostazda etkili birçok parametrenin de glutensiz diyetle birlikte düzeldiği gözlenmiştir. OBJECTIVE: This study aims to evaluate the effects on hematological parameters of the gluten-free diet in celiac disease children. MATERIAL AND METHODS: Firstly, children with celiac disease were identified in our clinic. Then, retrospectively, among these patients who were able to perform gluten-free diet correctly were specified. Hematological parameters of these patients were compared during the diagnosis and 6 months after the gluten-free diet. RESULTS: Sixty-seven of 97 children with celiac disease were included in the study. Forty-four patients were female (66.7%), 22 patients were male (33.3%) and the mean age of diagnosis was 7.1 ± 4.2 years. The most common hematologic disorders in patients were thrombocytosis in 38 patients (57.5%) and anemia in 28 patients (42.2%). Twenty-four patients with anemia (36.6%) had iron deficiency anemia, two patients with anemia (3%) had folate deficiency and one patient with anemia (1.5%) had vitamin B12 deficiency. After 6 months of gluten-free diet, only 8 patients (13%) had anemia. After the gluten-free diet, platelet counts decreased from 351.2 ± 11.0 (103 / μL) to 310.5 ± 82.0 (103 / μL), leukocyte count decreased from 8.82 ± 3.04 (103 / μL) to 8.04 ± 2.56 and mean platelet volume decreased from 10.08 ± 1.07 fl to 9.5 ± 1.19 fl. It was determined that there was a significant increase in hemoglobin, mean corpuscular volume and red cell distribution width values. CONCLUSIONS: The most common hematological findings in children with celiac disease are thrombocytosis and anemia. An improvement has been observed in anemia after gluten-free diet. In addition, many hemostasis parameters have been improved with the gluten-free diet.

Short QT sydrome (SQT) is one of the rarely seen arythmia causes related to sudden cardiac death. An important part of the patients are diagnosed during the examination due to cardiac arrest. Therefore, diagnosing asymptomatic patients is lifesaving. In this case report, we presented a five-year old asymptomatic female patient how to put the SQT diagnosis. There was no complaint of the patient who had been directed to pediatric cardiology polyclinic due to murmur. In her electrocardiography (EKG), QTc duration was determined in lower limits. However, when the patient's family history was examined in a detailed way, suspicious deaths were recognized. In the patient's 24-hour holter EKG; SQT was diagnosed by establishing average QTc duration as 340 ms together with sharp, narrow T waves. Satolol treatment was initiated to the patient. To diagnose asymptomatic SQT patients, a decent family history must be obtained. Spot EKG is insufficient to rule out the diagnosis. All patients with suspicious death histories in their familieis must be directed to pediatric cardiologists.

Siyanotik doğuştan kalp hastalarında arteriyel oksijen saturasyonunun düşük olması nedeniyle sekonder eritrositoz görülmektedir; hastaların Hb, Hct düzeyleri yaş gurubuna göre beklenenden yüksek değerlerdedir. Sekonder eritrositozun doğal bir sonucu olarak hastalarda demir tüketimi artmıştır. Siyanotik doğuştan kalp hastalarında demir eksikliği sık görülen bir durum olup hastaların izleminde demir tedavisi sıklıkla önerilmektedir. Ancak demir tedavisi ile birlikte hastalarda Hb, Hct düzeyleri de artmaktadır. Siyanotik doğuştan kalp hastalarında en önemli klinik sorunlardan biri olan hiperviskozite yüksek Hb ve Hct düzeyleri ile doğrudan ilişkilidir. Bu çalışma siyanotik doğuştan kalp hastalarında demir tedavisinin viskozite üzerindeki etkisini araştırmak amacı ile planlanmıştır.Gazi Üniversitesi Tıp Fakültesi Çocuk Kardiyoloji Bilim Dalında izlenen 20(%51) kız, 19(%49) erkek, ortalama yaşları 9,9 ± 6,2 yıl, ortalama ağırlıkları 33 ± 18,4 kg olan 39 siyanotik doğuştan kalp hastalıklı hasta değerlendirilmiştir. Hastalar ferritin düzeylerine göre demir eksikliği olan ve olmayan iki guruba ayrılmış; demir eksikliği saptanan guruba 4 mg/kg/gün dozda iki değerlikli demir tedavisi 3 ay süre ile uygulanmıştır. Demir eksikliği saptanmayan guruba ise herhangi bir tedavi verilmemiştir. Her iki gurubun da başlangıçta ve üç ay sonra klinik ve laboratuvar bulguları değerlendirilmiş, viskozite ölçümleri yapılmıştır.Otuzdokuz hastanın 21(%53,8)?inde demir eksikliği saptanmıştır. demir eksikliği ile MCH, MCV düşüklüğü ve RDW yüksekliği arasında korelasyon bulunmuştur. Üç ay süre ile demir tedavisi uygulandıktan sonra hastaların Hb ve Hct değerlerinde artış olmasına karşın viskozite değerlerinde artış görülmemiştir. Demir tedavisinden sonra hastaların hiperviskozite semptomlarında azalma, ortalama oksijen saturasyonlarında ise artış görülmüştür. Bu çalışma siyanotik doğuştan kalp hastalığı olan çocuklarda demir tedavisinin gerekli ve güvenli olduğunu göstermiştir.Demir eksikliği olmayan hastaların 3 ay sonra yapılan kontrol değerlendirmelerinde ise; demir eksikliği anemisi gelişmese bile Hb, Hct, serum demir, ferritin düzeylerinin anlamlı olarak düştüğü görülmüştür. Bu sonuç hastaların izleminde idame dozdan demir desteğini gündeme getirmelidir. Secondary erythrocytosis is seen in cyanotic congenital heart disease (CCHD) patients which leads Hb and Hct levels to be above the expected values. Iron consumption is increased as a natural result of secondary erythrocytosis. Iron deficiency is a common problem and supplementation is frequently recommended during follow-up. Levels of Hb and Hct also increases with iron replacement therapy and hyperviscosity, one of the most importatnt clinical problems of CCHD is directly related with elevated Hb and Hct levels. This study was designed to evaluate the effects of iren supplementation on viscosity in CCHD.Thirty nine CCHD patients 20 girls (51 %) and 19 boys (49%), followed up in Gazi University Department of Pediatric Cardiology with mean age 9,9 ± 6,2 years and mean wight 39 33 ± 18,4 kg were evaluated. Patients were divided into two groups according to their ferritine levels as iron deficient and non-iron deficient, and 4 mg/kg/day Fe +2 therapy was applied for 3 months. Non-iron deficient group were not given any therapy. Clinical and laboratory findings of both groups at the beginning of the study and after 3 months were evaluated and viscosity measurements were performed.Iron deficiency was detected in 21 out of 39 patients (53,8 %) and a correlation was found between iron deficiency, low MCH-MCV and high RDW values. Although Hb and Hct levels increased after 3 month therapy, no increase in viscosity was detected. Hyperviscosity symptoms decreased after iron supplementation and average oxygen values of patients have increased. This study showed iron replacement therapy is essential anf safe in pediatric CCHD.Follow up after 3 months of non-iron deficient group showed signifiicant decrease in Hb, Hct and ferritine levels although iron deficiency anemia did not occur. This situation supports the importance of iron supplementation in CCHD. 77

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