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Aim: This study aimed to evaluate the role of real-time three-dimensional (four-dimensional) and speckle tracking echocardiography for early detection of left ventricular systolic dysfunction and also for the relationship between myocardial deformation parameters and myocardial iron load which is measured by cardiac magnetic resonance relaxation time T2* values in asymptomatic children with beta-thalassemia major. Material and Methods: This multicenter cross-sectional study included 40 patients (mean age 15.4 ± 2.9, 42.1% male) and 40 healthy children whose age, gender, and body mass index-matched with patients. Each participant underwent conventional echocardiography and tissue Doppler imaging. Left ventricular ejection fraction; global longitudinal, circumferential, radial strains; twist; and torsion were measured by real-time three-dimensional and speckle tracking echocardiography. Cardiac magnetic resonance imaging T2* was measured in patients. Results: Left ventricular global longitudinal, circumferential, and radial strains were decreased despite preserved global ventricular function in patients compared to healthy children (p = p =.029, p = p

Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD.Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients’ demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively.A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up.This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.Anjiyofolliküler lenf nodu hiperplazisi olarak da bilinen Castleman hastalığı (CH), nadir bir hastalık olup başlıca hiyalin vasküler ve plazma hücreli olmak üzere 2 histolojik alt tipi vardır. Hastalığın anatomik yayılımı ve tutulan lenf nodu bölgelerinin sayısına göre unisentrik (UCH) ya da multisentrik (MCH) olarak sınıflandırılır. Bu çok merkezli çalışmanın amacı bugüne kadar Türkiye’de tanımlanan tüm CH olgularını tanımlamak, ulusal bir veri tabanı oluşturarak CH’de erken tanı, tedavi ve takip sürecine katkı sağlamaktır.Çalışmaya lenf nodu ya da tutulan alandan yapılmış biyopsi sonucu CH olarak rapor edilen hem erişkin (n=130) hem de pediyatrik (n=10) hastalar dahil edildi. Hastaların demografik bilgileri, klinik ve laboratuvar özellikleri, görüntüleme bulguları, aldıkları tedaviler ve tedavi sonuçları geriye dönük olarak değerlendirildi.Dahil edilen 140 hastanın 69’u kadın, 71’i erkekti. Yetmiş üç hasta UCH, 67 hasta MCH olarak sınıflandırılmıştı. Yaş ortalaması UCH’de 39, MCH’de 47 yıl idi. Kadın hastalar UCH’de daha fazlaydı. Hem UCH hem de MCH için en sık histolojik alt tip hiyalin vaskülerdi. Asemptomatik hastalar UCH’de daha fazlaydı. Anemi, akut faz reaktanı yüksekliği ve hipoalbuminemi MCH’de daha sıktı. UCH’de en sık uygulanan tedaviler sırasıyla cerrahi eksizyon, rituksimab ve radyoterapiydi. Median 19,5 aylık takipte tüm UCH’li hastalar hayattaydı. MCH’de 1. basamak tedaviler metil prednizolon, R-CHOP, R-CVP ve rituksimab idi. Median 34 aylık takipte 13 MCH’li hasta kaybedilmişti.Çalışmamız Türkiye’deki CH hastalarının özellikleri ve tedavi yaklaşımlarını yansıtması açısından önemli olup hastalıkla ilgili farkındalığın arttırılması potansiyeline sahiptir. Tedavi verileri özellikle ülkemizde olduğu gibi siltuksimaba ulaşımı zor olan ülkelerde tedavi seçimi konusunda fikir verebilir. Kesin sonuçlar çıkarmak için büyük ölçekli prospektif çalışmalara ihtiyaç vardır.

Plasminogen has an important role in intravascular and extravascular fibrinolysis (1-3). Severe hypoplasminogenemia is associated with ligneous conjuctivitis, but ligneous lesions can also occur in many different mucosal membranes like cervix and endometrium (4,5). Despite numerous clinical evaluations, biopsies, and laboratory tests, these kind of patients' diagnosis remained elusive for many years (6). By presenting this case, we want to increase the awareness of this peculiar disease, for its proper diagnosis. WOS:000730491500014 34555888 Q4

Background Genetic deficiencies of immune system, referred to as inborn errors of immunity (IEI), serve as a valuable model to study human immune responses. In a multicenter prospective cohort, we evaluated the outcome of SARS‐CoV‐2 infection among IEI subjects and analyzed genetic and immune characteristics that determine adverse COVID‐19 outcomes. Methods We studied 34 IEI patients (19M/15F, 12 [min: 0.6‐max: 43] years) from six centers. We diagnosed COVID‐19 infection by finding a positive SARS‐CoV‐2 PCR test (n = 25) and/or a lung tomography scoring (CORADS) ≥4 (n = 9). We recorded clinical and laboratory findings prospectively, fitted survival curves, and calculated fatality rates for the entire group and each IEI subclass. Results Nineteen patients had combined immune deficiency (CID), six with predominantly antibody deficiency (PAD), six immune dysregulation (ID), two innate immune defects, and one in the autoinflammatory class. Overall, 23.5% of cases died, with disproportionate fatality rates among different IEI categories. PAD group had a relatively favorable outcome at any age, but CIDs and IDs were particularly vulnerable. At admission, presence of dyspnea was an independent risk for COVID‐related death (OR: 2.630, 95% CI; 1.198–5.776, p, 34 IEI patients aged between 0.6 and 43 years, eight patients (23.5%) succumbed to COVID‐19, indicating a highly vulnerable condition to COVID‐19. Laboratory markers associated with mortality included elevated acute phase reactants, ferritin, troponin T, TLS, and reduced ALC levels, albumin, and baseline IgG. Coughing, dyspnea, CORADS category 4–6, and negative SARS‐CoV‐2 PCR at admission were among the predictors of lethal outcome.