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PMID: 35110115 Objective: Although many pediatric studies on children infected with coronavirus disease 2019 (COVID-19) have been published, the diagnosis, clinical symptoms, laboratory findings, and treatment of COVID-19 in children are still unclear. Materials and Methods: This study was conducted with an aim to examine the hematological findings of symptomatic pediatric patients diagnosed with COVID-19 in May 2020 at the Pandemic Hospital in Dicle University. Patient records were evaluated retrospectively. This study involved 59 symptomatic pediatric patients with a definite diagnosis of COVID-19 who had positive SARS-CoV-2 RT-PCR test results on nasopharyngeal swab between March 15, 2020 and May 31, 2020. Results: The records of a total of 10 (16.9%) patients under the age of 1; 21 (35.6%) patients aged 1-10 years, and, 28 (47.5%) patients aged 10-18 years, who had been diagnosed with COVID-19 were evaluated. Based on severity, 35 (59.3%) patients were in the mild group (group 1) and 24 (40.7%) patients were in the moderate-severe group (group 2). The blood parameters of WBC, neutrophil, lymphocyte, monocyte, and thrombocyte counts, the hemoglobin (Hgb) level, and NLR, PLR, MPV, fibrinogen, ferritin, and D-dimer levels were compared between groups, the difference was not statistically significant (P > .05). LDH was higher in group 2 (P = .014). Conclusion: Since children infected with COVID-19 show mild clinical symptoms or are asymptomatic, fewer pediatric patients may be detected than adults. Therefore, it should be known that the laboratory findings typical for adults may not accompany the disease in pediatric cases. More studies are needed to determine the most appropriate COVID-19 treatment approach for children, as hospitalization history and testing rates are less reported among children.

Maturity-Onset Diabetes of Young (MODY), otozomal dominant geçiş gösteren beta hücre defekti ve insülin salgısında bozuklukla giden bir hastalıktır ve tüm diyabetli olguların yaklaşık %1-2'sini oluşturmaktadır. MODY tip 2; klinik olarak osmotik semptomlar olmaksızın hafif açlık hiperglisemisi ile seyretmektedir. Tanı tesadüfen konur.

Aim: Due to risk of severe bleeding of the organs and inveteracy acute immune thrombocytopenic purpura (ITP) is a disease that still preserves importance. In our region, cases of ITP retrospectively reviewed in terms of clinical characteristics and responses to the treatment options. Matarial and Methods: Acute and chronic cases, patients who are presenting symptoms over the age of two years and under two years of age were compared in terms of symptoms, examination findings and responses to the treatment options. Results: A total of 151 patients (male n=78, female n=73) and mean age was 5.1±3.4 years were included. The ratios of acute and chronic patients' were 75% and 25%, retrospectively. Chronicity rate was higher in patients more than two years age (p=0.01). The most frequently presenting symptom was skin lesions and 64.6% of patients with silent symptoms developed chronic immune thrombocytopenic purpura. Seasonal factors and gender had no effect on acute and chronic immune thrombocytopenic purpura (p=0.119, p=0.061). The chronicity rate of patients who have partial remission at the end of the steroid treatment as a first-line treatment option was found (p=0.001). In acute ITP cases high rate of positive response was found to steroid therapy (p=0.04). Conclusions: The clinical characteristics of the patients in our region are similar to other reported cases in our country. Economic and effective treatment selection to preventing morbidity and mortality due to disease in patients was thought to make a positive contribution of the disease prognosis. Key words: ITP; Childhood; Treatment., Amaç: Akut immün trombositopenik purpura çocukluk çağında ciddi organ kanamaları ve kronikleşme riskinden dolayı hala önemini koruyan önemli bir hastalıktır. Bölgemizdeki immun trombositopenik purpura olgularını klinik özellikleri ve farklı tedavi metotlarına ve yaklaşımlarına verdikleri yanıtlar bakımından retrospektif olarak inceledik. Gereç ve Yöntem: Çalışmada akut ve kronik gidişli immün trombositopenik purpuralı olgular, iki yaş altı ve iki yaş üzerindeki olgular ilk başvuru semptomları, muayene bulguları ve almış oldukları tedavi seçeneklerine verdikleri yanıt bakımından karşılaştırıldı. Bulgular: Çalışmaya dahi edilen olguların 78 tanesi erkek, 73 tanesi kız, ortalama yaş 5,1±3,4/yıl ve %75 akut, %25 olgu kronik immun trombositopenik purpura tanısı ile izlenmişti. İki yaşından büyük olguların kronikleşmeye gidiş oranı, iki yaşından küçük gruba göre daha yüksek bulundu (p=0.01). Olgular en sık cilt bulguları ile hastaneye başvurmuş ve semptomları daha az belirgin olan olguların %64,6'sı kronikleşmişti. Mevsim ve cinsiyet faktörü akut-kronik immun trombositopenik purpura olmayı etkilemediği saptandı (p=0.119 ve p=0.061). İlk tedavide steroid alan ve tedavi sonunda parsiyel remisyon gerçekleşen hastalarının kronikleşme oranı (p=0.001) ve akut olguların steroide olumlu yanıt oranı yüksekti (p=0.04). Sonuç: Olgularımızın klinik özellikleri ülkemizdeki diğer bildirilmiş immun trombositopenik purpura olgularına paralellik göstermekte olup ekonomik ve etkili tedavi seçimlerinin hastalığa bağlı morbidite ve mortaliteyi önleyerek hasta prognozuna olumlu katkı sağlayacağı düşünülmektedir. Anahtar kelimeler: İTP; çocuk; tedavi.

Perinatal asfiksi, tanı ve tedavi yöntemlerindeki tüm gelişmelere rağmen gelişmiş ülkelerde bile yenidoğan mortalite ve morbiditesinin en başta gelen sebeplerindendir. Biz bu tez çalışmamızda hastanemiz yenidoğan servisinde 2007-2010 yılları arasında perinatal asfiksi tanısı ile yatırılan hastaların antenatal, natal ve postnatal risk faktörlerini belirlemeyi, hastaların Sarnat&Sarnat HİE evrelemesine göre sınıflandırılmasını, klinik bulgularını, mortalite oranını ve asfiksinin derecesi ile hastaların prognozları arasındaki ilişkiyi göstermeyi ve yıllar içerisinde olgularda birçok parametre açısından değişim olup olmadığını göstermeyi amaçladık.Çalışmada Dicle Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Kliniği Yenidoğan Servisine Ocak 2007-Aralık 2010 tarihleri arasındaki dört yıllık süre içerisinde perinatal asfiksi tanısı ile yatırılan 210 yenidoğanın dosya kayıtları retrospektif olarak değerlendirildi. Bu süre içinde perinatal asfiksi sıklığı %9.7 olarak bulundu. Perinatal asfiksi tanısı alan 210 olgunun 102'si (%48.6) erkek ve 108'i(%51.4) kız bebeklerden oluşmaktaydı. Sarnat&Sarnat evrelemesine göre olgularımızın 90'ı (%42.9) evre I, 63'ü (% 30) evre II ve 57'si (% 27.1) evre III olarak değerlendirildi. Perinatal asfiksili olguların 91'i (%43.3) yenidoğan döneminde eksitus oldu. Tüm olgular arasında 47 (%22.3) olgu konvulziyon geçirmişti, Konvülziyon geçiren hastalar evrelere göre karşılaştırıldığında; evre I olguların 9'u (%10), evre II olguların 22'si (%34.9), evre III olguların 16'sını (%28.1) oluşturmaktaydı. Evre II ve III te; evre I'e göre konvülziyon oranı daha yüksekti. Bu dağılım istatistiksel olarak çok ileri düzeyde anlamlıydı (p

Centipede bites have been reported rarely in humans. The mechanism of centipede toxin is not fully understood. Skin reactions develop more in centipede bites. In this text, we will present a case that developed thrombocytopenia after a centipede bite. An 18-month old female infant was bitten on the back of the right hand and the right side of the forehead. She applied to the emergency department with the complaint of redness and swelling that started in the morning hours after the bite. The vital signs of the patient were stable. The patient had no systemic complaints. The patient's laboratory tests, PLT was 28,000 K/UL were determined. During follow-up of the patient, bleeding symptoms developed associated with thrombocytopenia. The follow-up examination 10 days later, thrombocyte count was determined as 302,000 in the full blood count. Centipede bites are not greatly reported and the actual incidence is undoubtedly higher than assumed. Although the majority spontaneously recover with only simple local reactions that have formed, they can lead to serious complications such as acute myocardial infarcts, acute coronary ischemia, acute renal damage, anaphylaxis, or thrombocytopenia. Therefore, following a centipede bite, patients must be evaluated carefully and attention must be paid in respect of complications. İnsanlarda kırkayak ısırıkları nadir olarak bildirilmiştir. Salgıladığı toksinin mekanizması tam olarak bilinmemektedir. Deri reaksiyonları daha çok gelişir. Bu yazıda; kırkayak ısırması sonrası trombositopeni gelişen bir olguyu sunacağız. On sekiz aylık kız hasta gece saatlerinde sağ el sırtından ve başın sağ ön kısmından ısırılmış. Isırık sonrası sabah saatlerinde başlayan kızarıklık ve şişlik şikayetiyle acil servise başvurdu. Hastanın yaşamsal bulguları stabildi. Bakılan tetkiklerinde trombosit sayısı 28.000 K/UL saptandı. Hastada trombositopeniye ait kanama semptomları yoktu. Kontrol amaçlı 10 gün sonra bakılan tam kan sayımında trombosit sayısı 302.000 K/UL olarak bulundu. Kırkayak ısırmaları çok fazla bildirilmese de sıklığı sanıldığından fazladır. Çoğunluğu kendiliğinden düzelen sadece basit lokal reaksiyonlar oluşturmasına rağmen akut miyokard enfarktüsü, akut koroner iskemi, akut böbrek hasarı, anaflaksi, trombositopeni gibi ciddi komplikasyonlara yol açabilirler.

© 2021, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disorder with recurrent fever, abdominal pain, serositis, articular manifestations, erysipelas-like erythema, and renal complications as its main features. Caused by the mutations in the MEditerranean FeVer (MEFV) gene, it mainly affects people of Mediterranean descent with a higher incidence in the Turkish, Jewish, Arabic, and Armenian populations. As our understanding of FMF improves, it becomes clearer that we are facing with a more complex picture of FMF with respect to its pathogenesis, penetrance, variant type (gain-of-function vs. loss-of-function), and inheritance. In this study, MEFV gene analysis results and clinical findings of 27,504 patients from 35 universities and institutions in Turkey and Northern Cyprus are combined in an effort to provide a better insight into the genotype-phenotype correlation and how a specific variant contributes to certain clinical findings in FMF patients. Our results may help better understand this complex disease and how the genotype may sometimes contribute to phenotype. Unlike many studies in the literature, our study investigated a broader symptomatic spectrum and the relationship between the genotype and phenotype data. In this sense, we aimed to guide all clinicians and academicians who work in this field to better establish a comprehensive data set for the patients. One of the biggest messages of our study is that lack of uniformity in some clinical and demographic data of participants may become an obstacle in approaching FMF patients and understanding this complex disease.

Background. Based on the information obtained so far, COVID- 19 is relatively mild in children. We will present a 6-month-old male patient infected with COVID -19 in April 2020, while receiving HLH 2004 chemotherapy protocol with the diagnosis of familial (Genetic / Primary) Hemophagocytic Lymphohistiocytosis (HLH). Case. Herein we present a case accompanied by a defective perforin gene defect in the primary HLH pathogenesis, Covid-19 infection with the presence of fever and hyperferritinemia, which was evaluated in favor of reactivation and the patient was given both the HLH-2004 chemotherapy protocol treatment and COVID -19 therapy as recommended by the guidelines. Our patient improved clinically and in terms of laboratory test results at the end of the 15th day of hospitalization and was discharged. Conclusions. It should be remembered that COVID-19 can be seen with different clinical manifestations in the pediatric age group, and COVID-19 tests should be recommended, especially in children with immunosuppression and fever.

Introduction: Rare factor deficiencies are predominantly autosomal recessively inherited disorders with a frequency of approximately 1: 500000 to 1: 2000000 in the general population. They account for 3-5% of all inherited coagulation disorders. In this study, we aimed to evaluate the demographic features and clinical findings of 36 patients who were followed up and treated with the diagnosis of rare factor deficiency. Materials and Methods: A total of 36 patients aged between 0-16 years diagnosed with rare coagulation deficiencies were evaluated in terms of demographic, physical examination, clinical follow-up, and laboratory findings at the Dicle University Pediatric Hematology Unit. Ethics committee approval was obtained from Dicle University for the study on 16.03.2018 with decision no 115. Results: Rare factor deficiencies were diagnosed in 36 (35 %) of 103 patients who were followed up with coagulation disorders. Hemophilia a, hemophilia b, and von Willebrand disease constituted 67 of our patients. Familial consanguinity was present in 75, 6 %, and positive family history was found at 16.6% of the patients. 11 (32.4%) of our patients were diagnosed under the age of one year. Most of our patients diagnosed with factor X deficiency (38.8%). The most common symptoms were mucocutaneous bleeding (50%). Intracranial hemorrhage was detected in 7 (%19,5) patients. Conclusions: Early diagnosis and treatment are very important in the case of rare factor deficiency since severe bleeding complications such as intracranial hemorrhage may develop. Rare factor deficiencies are seen more frequently in places where consanguineous marriage is more common than the general population. Families should be informed about this issue, and family screening should be done early. Giriş: Nadir faktör eksiklikleri; çoğunlukla otozomal resesif geçiş gösteren,prevalansı 1/ 500.000 -1/2.000.000 arasında değişen pıhtılaşma faktör eksiklikleridir. Kalıtsal pıhtılaşma faktör eksikliklerinin %3-5’ini oluşturmaktadır. Bu çalışmada, nadir faktör eksikliği tanısı ile takip ve tedavi ettiğimiz hastaların demografik özellikleri ve klinik bulguları değerlendirilmiştir. Gereç ve Yöntem: Çalışmamızda, Dicle Üniversitesi Tıp Fakültesi Çocuk Hematolojisi ve Onkolojisi biriminde nadir faktör eksikliği tanısı ile takip ve tedavi edilen, yaşları 0-16 yıl arasında değişen toplam 36 hasta değerlendirilmiştir. Hastaların demografik özellikleri,klinik izlem sonuçları ve labaratuar verileri incelenmiştir.Çalışma için Dicle Üniversitesi’nden 16.03.2018 tarihinde 115 nolu karar ile etik kurul onayı alınmıştır. Bulgular: Kalıtsal faktör eksikliği nedeniyle takip ve tedavi ettiğimiz 103 hastanın 36’sında (% 35) nadir faktör eksikliği tanısı mevcuttu. Hastalarımızın 67’sini hemofili A, hemofili B ve von Willebrand hastalığı oluşturuyordu. Anne ve babaların akrabalık oranı % 75,6 olup,aile öyküsü %16.6 olarak bulunmuştur. Hastalarımızın 11’i (%32,4) bir yaşından önce tanı almıştır.Hasta grubumuzda %38,8 oranı (14 hasta) ile FX eksikliği en fazla görülmektedir.Tanı anında en sık başvuru semptomunu %50 ile cilt-mukoza kanamaları oluşturmaktaydı. Ciddi kanamalardan intrakranial kanama yedi (%19,5) hastada görülmüştür. Sonuç: İntrakranial kanama gibi ciddi kanama komplikasyonlarıyla seyretmesinden dolayı nadir faktör eksikliğinde erken tanı ve tedavi çok önemlidir. Nadir faktör eksiklikleri, akraba evliliğinin fazla görüldüğü yerlerde genel popülasyona oranla sık görülmektedir. Ailelerin bu konuda bilgilendirilmesi,aile taramalarının erken dönemde yapılması gerekliliktir

PMID: 34738369 Background. The aim of this study is to examine the thickness of choroidal, macular and peripapillary retinal nerve fiber layer by spectral-domain optical coherence tomography (SD-OCT) in pediatric patients with sickle cell anemia (SCA) without retinopathy. Methods. A total of 75 children (30 SCA patients (Group 1) and 45 healthy individuals (Group 2) were included in the study. Macular (central, superior, inferior, nasal, temporal), choroidal (subfoveal, at nasal distances from the central fovea of 1000 μm [N1], 2000 μm [N2], 3000 μm [N3], at temporal distances from the central fovea of 1000 μm [T1], 2000 μm [T2], 3000 μm [T3]) and RNFL (average, temporal, superotemporal, inferotemporal, nasal, inferonasal and superonasal) measurements were performed by SD-OCT. These parameters were compared with healthy children with similar demographic characteristics. Results. The mean age was 14.11±3.86 (11-18) in sickle cell anemia patients and 13.15± 2.69 (10-18) in the healthy control group. Of the patients, 56.6% (n=17) of Group 1 and 44.4% (n=20) of Group 2 were male. Choroidal measurements made in the subfoveal, N1, N2, N3, T1, T2 and T3 quadrants showed that the choroid was thinner in 6 quadrants in SCA patients compared to the healthy group (p = 0.003, p = 0.039, p = 0.035, p = 0.595, p = 0.006, p = 0.005, p = 0.047, respectively). In RNFL measurements, there was significant thinning in the temporal, inferotemporal, and nasal quadrants of SCA patients compared to the healthy group. Changes in other quadrants were not significant. Conclusions. SD-OCT is a useful imaging method in the diagnosis and screening in patients with SCA without retinopathy. Early diagnosis of retinopathy during subclinical disease will prevent visual loss in these patients.