Serum lipids in Turkish patients with β-thalassemia major and β-thalassemia minor

It is well-known that β-thalassemia is associated with changes in plasma lipids and lipoproteins [1,2,3]. To our knowledge, no data are available on lipid profiles in Turkish β-thalassemia major (TM) and β-thalassemia trait (TT) patients together. The aim of this study was to evaluate lipid profiles in two groups of patients with β-TM and β-TT and to compare them with healthy controls. The study included a total of 311 subjects. Group 1 included 131 β-TM patients (mean age: 16.3±7.58 years). Group 2 included 68 β-TT patients (mean age: 7.25±4.43 years). Group 3 consisted of 112 age- and sex-matched healthy controls (mean age: 9±4.7 years). Serum ferritin level was 2487±1103 (range: 661-5745) ng/mL in Group 1. In comparing the correlation between ferritin and lipid parameters, while a significantly negative relationship was detected between ferritin and highdensity lipoprotein cholesterol (HDL-C) (p=0.000, r=-0.602), a significantly positive relationship was detected between ferritin and triglyceride (TG) levels (p=0.02) in TM patients. Serum lipid profiles of the 3 groups are shown in Table 1

A hemophagocytic lymphohistiocytosis case with newly defined UNC13D (C.175G>C; p.Ala59Pro) mutation and a rare complication

Hemophagocytic lymphohistiocytosis (HLH) represents a severe hyperinflammatory condition with cardinal symptoms of prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages with impaired function of natural killer cells and cytotoxic T lymphocytes. A 2-month-old girl, who was admitted with fever, was diagnosed with HLH and her genetic examination revealed a newly defined mutation in the UNC13D (c.175G>C; p.Ala59Pro) gene. She was treated with dexamethasone, etoposide, and intrathecal methotrexate. During the second week of treatment, after three doses of etoposide, it was noticed that there was a necrotic plaque lesion on the soft palate. Pathologic examination of debrided material in PAS and Grocott staining revealed lots of septated hyphae, which was consistent with aspergillosis infection. Etoposide was stopped and amphotericin B treatment was given for six weeks. HLH 2004 protocol was completed to eight weeks with cyclosporine A orally. There was no patient with invasive aspergillosis infection as severe as causing palate and nasal septum perforation during HLH therapy. In immuncompromised patients, fungal infections may cause nasal septum perforation and treatment could be achieved by antifungal therapy and debridement of necrotic tissue. © 2015 Turkish Society of Hematology. All rights reserved

Hemophagocytic syndrome due to leishmania infection diagnosed with immunofluorescence antibody test

Leishmaniasis is a reticuloendothelial system disease that mostly observed before the age of 5. Visceral infection causes long-standing fever, weight loss, weakness, pancytopenia, and hepatosplenomegaly. Leishmania infantum is responsible for visceral leishmaniasis (VL) in Turkey. We present a case of hemophagocytic syndrome due to Leishmania infection diagnosed with an immunofluorescence antibody test (IFAT). Leishmania amastigotes were not observed on bone marrow aspiration. We consider that IFAT is very important for parasite detection in the diagnosis of VL in children, particularly when amastigotes are not obtained on bone marrow aspiration

Evaluation of visceral leishmaniasis diagnosis using indirect fluorescent antibody tests in 4 pediatric patients.

Visceral Leishmaniasis (VL) in infancy is mostly seen between the ages of 2-4,and visceral infection causes long standing fever, weakness, weight loss, hepatosplenomegaly and pancytopenia. Leishmania infantum is responsible for VL in Turkey. In this study, 4 pediatric cases of VL were analysed retrospectively. Bone marrow aspirate was obtained in two cases and Leishmania amastigotes were not obtained in these cases. Leishmania antibodies by the indirect immun fluorescent antibody test (IFAT) were positive in all cases. We consider that IFAT was a suitable alternative to parasite detection in the conclusive diagnosis of visceral leishmaniasis in pediatric patients when strong clinical suspicion is present

Abdominal ultrasonographic findings in patients with thalassemia

AMAÇ: Bu çalışmanın amacı, Denizli Devlet Hastanesi Talasemi Merkezinde izlenen talasemi major ve talasemi intermedia hastalarının rutin abdominal ultrasonografik incelemeleri seyrinde görülen hepatosplenomegali dışındaki abdominal solid organlar ile ilgili bulgularını değerlendirmektir. GEREÇ veYÖNTEMLER: Bu çalışmaya, 73'ü talasemi major, 5'i talasemi intermedia olmak üzere, yaşları 2 ile 30 yıl arasında, 40 kız, 38 erkek hasta alınmıştır. BULGULAR: Safra kesesinde taş ve çamur (%8.9) en sık görülen bulgu idi. Aksesuar dalak 6 hastada (%7.7), karaciğerde ekojenite artısı 2 hastada (%2.6), karaciğerde hemanjiom 1 hastada(%1.3), portal ven trombozu 1 hastada(%1.3) saptandı. Bir talasemi major hastasında, bilateral renal kist ve sag renal tas ve beraberinde safra kesesinde taş görüldü. SONUÇ: Talasemi hastalarında abdominal ultrasonografik inceleme, komplikasyonların önlenmesi ve etkili klinik izlem için düzenli aralıklar ile yapılmalıdır.OBJECTIVE: The aim of this study was to evaluate the type and prevalance of abdominal ultrasonographic abnormalities except hepatosplenomegaly in patients with thalassemia major and thalassemia intermedia. MATERIAL and METHODS: Seventy-eight patients; 73 thalassemia major, 5 thalassemia intermedia followed in Denizli State Hospital Thalassemia Center were included in the study. The patients' ages ranged from 2 to 30 years, with 40 females, 38 males. RESULTS: The most frequent ultrasonografic findings were cholelithiasis (8.9%), accesory spleen (7.7%), hepatic grade I-II echogenity (2,6%), hepatic hemangioma (1.3%) and portal vein thrombosis (1.3%). One case was diagnosed with polycystic kidney disease with coexisting nephrolitiasis and cholelithiasis. CONCLUSION: Abdominal ultrasonographic imaging of patients with thalassemia patients should be done regularly to prevent complications and for effective clinical observation

Mikofenolat mofetil ile remisyon sağlanan steroide dirençli otoimmün hemolitik anemi

Autoimmune hemolytic anemia (AIHA) is a disease that is seen in 1/10.000 people and characterized by forming antibodies against red blood cells and degradation of these red blood cells in reticuloendothelial system. Even autoimmune diseases can accompany, it is seen usually idiopathic. Paleness due to anemia, jaundice, tachycardia, darkening in urine color, hepatosplenomegaly are frequently seen clinical findings. Clinically normochrome and normocytic anemia, reticulocytosis, polychromasia in peripheral smear, spherocytes, indirect hyperbilirubinemia and increased lactate dehydrogenase are seen. Direct coombs test is the diagnostic test. Steroids are the first line drugs in treatment. The dosage and the treatment duration is formed according to patient's clinical situation. The treatment is checked by complete blood count, reticulocyte and Coombs test. Immunosuppressor treatments are given to patients that don't respond to treatment in 4-6 weeks or less patients who has recurrence at the time of decreasing the treatment dosage of corticosteroids. In few patient, that did not respond steroids, immunosuppressive treatments are used. Here, we present a patient, who is diagnosed with AIHA that we couldn't manage remission by steroid treatment, and no response to rituxumab as an immunosuppressor, but treated successfully with microphenolat mofetil

The seroprevalance and vaccination status of Hepatitis B in children 1-6 years of age in Denizli Province

AMAÇ: Bu çalışmanın amacı Denizli ilinde, 1-6 yas arası çocuklarda, HBsAg seroprevalansı, anti-HBs sıklıgı ve hepatit B aşılanma durumunu araştırmaktır. GEREÇ ve YÖNTEM: Çalışmaya alınan çocukların hiçbirinde kronik hastalık ve immun yetmezlik yoktu. Hepatit belirleyicileri (HBsAg, anti-HBs, anti-Hbc) 545 çocukta ELISA yöntemi ile tarandı. Ayrıca çocukların anne ve babasının Hepatit B infeksiyon durumu sorgulandı ve kayıt edildi. BULGULAR: Çalışmamızdaki 545 çocuğun 314 (%57.6)'sı kız, 231 (%42.4)'i erkek olup, ortalama yas 3.9±1.2 (2-6) yıl idi. 475 (%87.1) çocukta anti-HBs pozitifliği, 58 (%10.6) çocukta HBsAg ve anti HBs negatifliği, 12 (%2.3) çocukta HBsAg pozitifliği saptandı. SONUÇ: Tüm çocuklar içinde aşılanma oranı %84.9 olarak bulundu. Sonuçta, 1-6 yas arası çocukların aşılanmasının önemine dikkat çekilmiştir.OBJECTIVE: The aim of this study was to investigate seroprevalence of HBsAg and frequency of anti-HBs positivity and hepatitisBvaccination status in children, 1- 6 years of age, in Denizli province. MATERIALS and METHODS: None of the children had chronic diseases and immun deficiency. In 545 children, Hepatitis markers (HBsAg, anti-HBs, anti-Hbc) were screened by ELISA method. We questioned Hepatitis-B infection of parents. RESULTS: Of 545 patients, 314 (57.6%) were male, 231 (42.4%) were female, mean age was 3.9±1.2 (2-6) years. We found anti-HBs positivity in 475 (87.1%) children. 58 (10.6%) children had anti-HBs and HBsAg negativity. 12 (2.3%) children hadHBsAg positivity. CONCLUSION: The rate of vaccination in all children was found 84.9% (463 children). Importance of vaccination of 1-6 years of age was emphasized

Patients who developed osteonecrosis during the treatment of acute lymphoblastic leukemia

Osteonekroz; çocuklarda akut lenfoblastik lösemi tedavisi sırasında veya sonrasında nadir görülen fakat ciddi sorunlara yol açabilen bir komplikasyondur. Yapılan çalışmalarda ALL tedavisi alan çocuklarda semptomatik osteonekroz insidansı %1,6-9,3 olarak bulunmuştur. Yüksek doz steroid tedavisi en önemli risk faktörüdür. Osteonekrozis fizyopatolojisi tam olarak açıklanamamıştır. Kortikosteroidlerin, sinüzoidal venlerdeki tromboembolilere bağlı mekanik tıkanmaya neden oldukları bunun sonucunda kan akımının azalmasıyla birlikte hipoksiye bağlı olarak osteoblast aktivitesinin azalıp, osteoklast aktivitesinin artmasıyla oluştuğu düşünülmektedir. Osteonekroz tanısında manyetik rezonans görüntüleme erken dönemde en duyarlı tanı yöntemidir. Burada ALL tanısıyla takip edilirken idame tedavisi sırasında osteonekroz gelişen 15 ve 14 yaşlarında 2 kız hasta sunulmuştur.Osteonecrosis is a rare and serious complication that can be developed during or after treatment for acute lymphoblastic leukemia (ALL). The incidence of symptomatic osteonecrosis related to ALL treatment is 1.6-9.3%. High-dose steroid therapy is the major risk factor for osteonecrosis. The pathophysiology is unclear. It is thought that osteonecrosis occurs due to steroid use causing thromboembolism in sinusoidal veins leading to hypoxia resulting in decreased osteoblast activity and increased osteoclast activity. Magnetic resonance imaging is the most sensitive diagnostic method in early period. We present two girls, 15 and 14-year-old, in whom osteonecrosis developed during ALL maintenance therapy

Muğla Adli Tıp Şube Müdürlüğü’ne Başvuran Cinsel Saldırı Olgularının Değerlendirilmesi

Amaç: Bu çalışmada, son iki yıl içinde Muğla Adli Tıp Şube Müdürlüğü’nden cinsel saldırıya uğrama nedeniyle adli rapor istenen olguların, durum tespiti amacıyla değerlendirilmesi amaçlanmıştır. Gereç ve Yöntem: Bu amaçla 01 Haziran 2012 – 31 Mayıs 2014 tarihleri arasında cinsel saldırı nedeniyle Muğla Adli Tıp Şube Müdürlüğü’ne müracaat ettirilen 341 olgu için düzenlenen 385 rapor retrospektif olarak incelenmiştir. Olguların, yaş ve cinsiyet gibi demografik verileri yanı sıra saldırganın mağdura yakınlık derecesi, olay yeri,  hangi konularda rapor istendiği, olay tarihi ile ilk muayenesi ve Muğla Adli Tıp Şube Müdürlüğü’ndeki muayenesi arasında geçen süreler ile muayene sonuçları değerlendirilmiştir. Bulgular: Çalışma kapsamında 341 olgu için düzenlenmiş 385 rapor değerlendirilmiştir. Olguların %90.3’ü kadın, %9.7’si erkek, %90.6’sı TC vatandaşı, %9.4’ü yabancı uyrukludur. Olguların % 63.3’ü 18’yaş ve altında iken %36.7’si 19 yaş ve üzerindedir. Toplam olguların yarısı (% 50.1) 13-18 yaş arasındaki ergenlerdir. Olguların %23.8’inde şüpheli saldırganlar eski/yeni erkek arkadaş, sevgili, nişanlı, eş vb. kişiler iken %17.3’ünde kan bağı olan ve olmayan akrabalardır. Olguların %56.3’ünde olay ev ortamında gerçekleşmiştir. Olguların %17.6’sında cinsel saldırı/taciz olayının 2011 ve daha önceki yıllarda gerçekleşmiş/başlamış olduğu saptanmıştır. Olguların %50.1’i Mayıs-Ağustos ayları arasında meydana gelmiştir. Olguların %33.7’si olaydan sonraki ilk 72 saat içinde muayene edilebilmiş, bunların da % 52.2’si Adli Tıp Şube Müdürlüğü’nde muayene edilmiştir. Sonuç: Mağdurların, olay sonrası şikâyet sürelerinin gecikmesi dikkat çekicidir; bu sebeple mağdurların kolluk kuvvetleri ve/veya savcılıkça ifadeleri alınırken tekrarlı travmaya uğramadan değerlendirilebilecekleri, multidisipliner bir yaklaşımla delil toplanmasını mümkün kılacak “cinsel saldırı başvuru merkezlerine” ihtiyaç duyulmaktadır. Anahtar Kelimeler: Adli tıp, cinsel saldırı, genital muayen

Prevalence and Risk Factors of Anemia among Adolescents in Denizli, Turkey

Objective: The aim of this study was to evaluate the prevalence and risk factors of anemia among adolescents in Denizli where Mediterranean cuisine (fresh fruit and vegetables) is adopted. Methods: We accepted hemoglobin values below 12 g/dl for girls and 13 g/dl for boys as the criteria of anemia. We recorded a detailed history including nutritional habits, consumption of animal source foods especially red meat, consumption of fresh fruit and vegetables, presence of parasitic infestation, psychosocial status, school success and any materials consumed except ordinary food. Findings: We investigated the prevalence of anemia in 1120 children (672 girls and 448 boys), aged 12 to 16 years. We found that the overall prevalence of anemia was 5.6%. 8.3% of the girls and 1.6% of the boys were anemic. We diagnosed iron deficiency anemia in 37(59%) anemic patients and combined iron deficiency and vitamin B12 deficiency anemia in 26 (41%) anemic patients. None of the patients had folic acid deficiency. Conclusion: Our results suggest that the socioeconomic status of the family, traditional eating habits of the region, the fear of gaining weight and irregular eating habits are of great importance in the development of adolescent anemia in Denizli