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Hemolytic anemia is characterized by a decrease in the number of circulating erythrocytes due to an increase in their hemolysis. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common erythrocyte enzyme defects related to hemolysis. The G6PD enzyme abrogates the hemolysis of erythrocytes by protecting them against oxidative stress due to its involvement in the glutathione metabolism. G6PD enzyme deficiency-related hemolytic anemia may present as neonatal jaundice or become manifest due to exposure to infections, favism and medications later in life. Dapsone is a medication that is preferred by doctors in the treatment of many dermatological disorders such as pemphigus vulgaris, and leads to hemolysis in the presence of G6PD enzyme deficiency. In this type of non-immune hemolysis, haptoglobulin is low and Coombs' tests are negative. Hemolytic anemia, a serious complication that may appear subsequent to dapsone use, can be prevented by testing G6PD enzyme levels prior to dapsone therapy. In this case, we emphasized that the hemolytic anemia in the patient using dapsone may be due to G6PD enzyme deficiency.

Gastronomy tourism embraces a wide range of gastronomic opportunities for tourists while contributing to sustainability, rural development and the protection of culinary heritage. It diversifies and revitalizes tourism as an essential element and emerging segment of the tourism industry. However, there is a scarcity of research on the subject in the context of travel agencies. The purpose of this research is to determine whether travel agencies in İzmir use culinary elements and gastronomic sources as themes in their tour packages to highlight the gastronomy tourism potential of the city, along with how a better development can be achieved. Thus, the contribution of this study is threefold. First, travel agencies in downtown İzmir were surveyed in terms of their actual gastronomy tourism product offers and future plans to create one. The survey results revealed that gastronomy was not promoted as a principal theme due to a lack of awareness of potential local and regional culinary sources. Second, experts in the gastronomy and tourism industries were contacted using purposive sampling. Their opinions were collected to create a list of places, products and activities that could be included in a gastronomy tour package. Third, an example tour program was created based on expert opinions along with the author’s contribution.

AMAÇ: Tüberküloz (TB), Mycobacterium tuberculosis kompleks (MTBK) grubuna ait mikroorganizmaların neden olduğu kronik gidişatlı, bir enfeksiyon hastalığıdır. Anti tüberküloz ilaçlar ile tedavi edilmekte ve kontrol altına alınmakta olan bu hastalık; bazen ilaçlara yanıt vermeyebilir. Tüberkülozda ilaç direnç araştırması yapılması tüberküloz kontrol ve tedavi programlarının temelini oluşturmaktadır.GEREÇ VE YÖNTEM: Çalışmamızda, laboratuvarımızda izole edilen MTBK izolatlarının primer anti tüberküloz ilaçlara duyarlılıklarının araştırılması ve direnç paternlerinin belirlenmesi amaçlanmıştır. Kliniğimizde 2011 - 2019 yılları arasında tüberküloz şüphesi ile mikrobiyoloji laboratuvarına gönderilen 8804 klinik örnek değerlendirmeye alınmıştır.BULGULAR: Çalışmaya alınan örneklerden 126’sında tüberküloz kültürü pozitif gelmiştir. 126 örneğe (%1.4) anti-tüberküloz duyarlılık testi uygulanmıştır. 28 (%22.3) izolat ise test edilen anti tüberküloz ilaçlardan en az birine dirençli olarak tespit edilmiştir. Çok ilaca direnç ise %2,38 oranında bulunmuştur.SONUÇ: Tüberküloz tedavisinde direnç gelişimini önlemek ve tedavinin sağlıklı bir şekilde yapılabilmesi için her hastaya kültür ve antibiyotik duyarlılık testlerinin çalışılması, verilerin elde edilmesi gerekmektedir.

Thrombotic microangiopathy (TMA) associated with hematopoietic stem cell transplantation, remains a difficult complication due to its high mortality rate, lack of standardised diagnostic criteria, and limited treatment options. Although the etiology of the disease is not clear, medications, radiotherapy, graft-versus-host disease (GVHD), angioinvasive fungal, viral infections and complement activation have been implicated. Agents such as therapeutic plasma exchange (TPE), steroids, rituximab, defibrotide, eculizumab are used in its treatment. The use of treatments such as TPE, steroids, defibrotide, and rituximab did not yield satisfactory results in transplant-associated TMA (TA-TMA). Recent studies have reported successful results with eculizumab in the treatment of TA-TMA. As there is no standard dose for eculizumab in the treatment of TA-TMA, doses used in atypical hemolytic uremic syndrome (aHUS) were administered. In this paper, we present a case that was unresponsive to TPE and steroids, but which was successfully treated with eculizumab. Key Words: Acute myeloid leukemia, Stem cell transplantation, Thrombotic microangiopathy, Apheresis, Eculizumab.

We aimed to investigate whether there is a predisposition to COVID-19 with ABO and Rh blood group systems This study was a retrospective study that investigate the patients admitted to our hospital between March 16 -May 20 due to Covid-19 pandemic and conducted with data revealed from the hospital Information Management System A total of 392 patients were included in this study, including 227 PCR test positive patients with blood group information in the system and 165 possible patients with CT findings in favor of Covid-19 Data from a blood group study conducted with 127091 people in our province in 2019 were used as a control group In our study, a significant increase was observed in the blood group A in patients diagnosed with Covid-19, and a decrease was found in the blood groups B, AB and especially O However, statistical analysis showed no significant difference between Covid-19 patients and healthy individuals in terms of ABO blood group system When analyzed in terms of Rh blood group system, it was found that Rh positivity was statistically significantly higher in patients with Covid-19 (p= 0 000) Our study suggests that the Rh (-) blood group is protective and the Rh (+) blood group is predisposed to Covid 19 significantly We think that it is valuable because it is the first study to reveal the relationship between Covid-19 and blood type in our country and the only one to reveal the relationship between Covid-19 and Rh (+) in the world literature

Background/aim Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare clonal hematopoietic stem cell disease characterized by chronic hemolytic anemia and thrombosis. We report data from a study of the occurrence of PNH among patients with idiopathic portal vein thrombosis (PVT). Materials and methods Patients who were followed up with the diagnosis of idiopathic PVT were enrolled into this study. Those with laboratory and/or imaging evidence of any local or systemic factor that could lead to PVT were excluded. PNH clone was examined in all patients using established FLAER methodology. Results A total of 112 patients (42 males and 70 females), none of them had a markedly PNH clone, but 4 patients (3.6%) with confirmed tests two times had small PNH clones (size between 3.02% and 4.62%). The median ages of PNH clone (-) and PNH clone (+) patients were 42 (range; 24–59) vs 39 (range; 36–42) years, respectively. The median hemoglobin concentration, platelet count and leukocyte count were lower in the PNH clone (+) group than the PNH clone (-) group. Anemia, thrombocytopenia, and leukopenia were detected in all PNH clone (+) patients. In addition, the PNH clone positivity size in monocytes was higher than erythrocytes in all of 4 patients. Conclusions PNH should be considered during differential diagnosis among patients with idiopathic PVT. Small PNH clones can be detected in PVT patients that we cannot clearly determine its relationship with PVT. We need furthermore studies to explore the potential role of this finding.

Dear Editor,A typical hemolytic uremic syndrome (aHUS), which occurs with the uncontrolled activity of the complement system, can occur at any age. Triggers of aHUS include pregnancy, drug use, aut...

Karakus, Volkan/0000-0001-9178-2850; Demirkan, Fatih/0000-0002-1172-8668; Demircioglu, Sinan/0000-0003-1277-5105; Arat, Mutlu/0000-0003-2039-8557; Cagliyan, Gulsum Akgun/0000-0002-2073-1949; Ozturk, Hacer Berna Afacan/0000-0001-9386-7604; WOS: 000473249400014 PubMed: 31036516 Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

Objective: Patients with chronic lymphocytic leukemia (CLL) show defective cellular and humoral immune responses. Although such immune failure is known to be associated with an increase in the frequency of particularly gram–positive and –negative bacterial infections, data on the increase in the frequency of zoonoses such as brucellosis and viral infections such as the hepatitis B virus (HBV) are inconclusive. This study aims to investigate the frequency of brucellosis and HBV seropositivity in patients diagnosed with CLL. Methods: Patients followed–up for CLL between 2005 and 2019 were evaluated. Results of patients who were tested for HBsAg and anti–HBs serology using the ELISA assay and for brucellosis using the serum agglutination (Wright) test were recorded. Demographic data and laboratory results of all patients included in the study were evaluated. Results: This study included 188 patients diagnosed with CLL, of whom 56 (29.8%) were female and 132 (70.2%) were male. Median age was 62 (33–92) years. Complete blood count parameters at diagnosis were as follows: median leukocyte count, 54.4×109/L (5.1–312.3×109/L); median lymphocyte count, 42.3×109/L (2.8–296.8×109/L); median platelet count, 148×109/L (86.3–342.3×109/L); median hemoglobin level, 13.4 g/dL (8.5-16.9 g/dL). HBsAg and anti–HBs were tested in 142 patients. A total of 16 (11.27%) patients were HBsAg–positive; with 5 (3.52%) positive cases in females and 11 (7.75%) in males. A total of 105 (73.95%) patients were anti–HBs–positive; with 32 (22.54%) positive cases in females and 73 (51.41%) in males. Wright agglutination test was performed on 82 patients. A total of 4 (4.88%) patients reacted positively to the Wright test; with 3 (3.66%) positive cases in females and 1 (1.22%) in males. Conclusion: Compared with the epidemiological studies conducted in the same region; the rate of positive reactions to the Wright agglutination test was consistent with the literature data; however, a higher rate of HBsAg positivity was determined. This may be linked to the increase in the risk of HBV transmission due to the immune defect caused by CLL or the immunosuppressive picture induced by the medication used in the treatment, or viral reactivation.

Problem Durumu: Günümüz insanı, eski çağlardan daha hızlı bir şekilde değişen bir dünyada yaşamaktadır. Bilimler ise elde edilen bulgular neticesinde sürekli değişmekte, yenilenmekte ve gelişim göstermektedir. Toplumların 25 yıl önce kullandığı birçok araç bugün kullanılmamakta, ulaşım araçlarının hızı sürekli artmakta, bir önceki neslin kullandığı araçların hızı ise gülünç düzeyde yavaş görünmektedir. Kısacası, artan nüfus karşısında azalan dünya kaynakları toplumlar için yeni ve gitgide ağırlaşan problemler çıkarmaktadır. Bu problemler karşısında insanlar yeni çözüm yolları bulmak zorundadır, yeni çözüm yolları ise değişmeyi ve gelişmeyi gerekli kılmaktadır. Bu noktada, bireyi hem çevresindeki değişimlere uyum sağlamasına olanak sağlayacak hem de çevrede istenen değişimi ortaya çıkarabilecek yeterliğe eriştirmek eğitimin sorumluluğundadır. Bu hedeflere ulaşabilmek için milli eğitim sistemimizde çeşitli dönemlerde ani ve sık değişiklikler yapılmış ancak belirtilen hedeflere istendik düzeyde ulaşılamamıştır. Bu gereksinimden yola çıkarak, erken çocukluktan hayat boyu öğrenmeye kadar çağın gerektirdiği eğitim ihtiyaçlarını saptamak ve geleceği gören bir eğitim sistemine sahip olmak için Milli Eğitim Bakanlığımız tarafından 2023 Eğitim Vizyonu Belgesi düzenlenmiştir. Cumhurbaşkanlığı programına uygun olarak, MEB 2023 Eğitim Vizyonu belgesinin hedeflerinin genel amacı vatandaşları 21. yüzyıl için becerilerle donatmak ve en uygun insan sermayesi gelişimini teşvik eden eğitim sistemini oluşturacak küresel değişime uyum sağlamak olarak ifade edilmiştir. Belgede eğitim sistemi, çocuğu eleştirel düşünen, akıl yürüten ve üretken vatandaşlara dönüştüren faaliyetleri içeren bütünsel bir sistem olarak sunulmaktadır. Ayrıca öğrencinin kök değerlerine bağlı ama aynı zamanda küresel rekabete hazır nitelikte donatılmış bireyler olması hedefi eğitim sisteminin en önemli görevleri içinde sayılarak net bir şekilde tanımlanmıştır. Türkiye 2023 Eğitim Vizyonunun felsefeden politikaya, ölçme değerlendirmeden rehberlik hizmetlerine, yabancı dil eğitiminden özel öğretime kadar tüm eğitim sisteminin ihtiyaçlarına yönelik hedefleri kapsadığı görülmektedir. Sonuç itibariyle öğretim görevlilerinin bakış açılarından vizyon belgesinin çeşitli açılardan ele alınmasının alan yazınına faydalı olacağı değerlendirilmiştir.Araştırmanın Amacı: Bu çalışmada Milli Eğitim Bakanlığı tarafından hazırlanan 2023 Eğitim Vizyonu Belgesine ilişkin eğitim fakültelerinde görev yapan akademisyen görüşlerinin ortaya çıkarılması amaçlanmıştır. Öğretim üyelerinin, vizyon belgesinde ifade edilen yeni düzenlemelerden ortaöğretimde ders saatlerinin azaltılması ve pedagojik formasyonun yüksek lisans eğitimi olarak verilmesi hakkındaki görüşleri nelerdir? Eğitime ilişkin çeşitli ve önemli değişiklik ve hedefleri kapsayan bu belge hakkında eğitim konusunda uzman akademisyenlerin görüşlerinin alınmasının belgenin değerlendirilmesi açısından önem taşıdığı ve konu ile ilgili yapılacak uygulamalara yön vermesi açısından da önemli olduğu düşünülmektedir.Araştırmanın Yöntemi: Bu çalışmada nitel araştırma teknikleri kullanılmıştır. Çalışmamızın desenini betimsel nitel araştırma deseni oluşturmuştur. Bu desende araştırmaların amacı, bireyler veya birey grupları tarafından yaşanan belirli olayları kapsamlı bir özetlenmesidir. Araştırmacı katılımcıların bir durum veya olguyu nasıl anlamlandırdığını anlamakla ilgilenmektedir. Betimleyici analiz, bir nitel araştırma şeklidir. Bu nitel araştırmanın anahtarı anlatıların veri olarak kullanılmasıdır. Betimsel nitel araştırma deseni kategorik olmayan bir sorgulama yöntemi olarak görülmelidir. Çalışmamızda akademisyenlerin aynı soru hakkındaki farklı düşüncelerini ele alınmış ve görüşülenlerden elde edildiği şekliyle aktarılmıştır. Bu amaçla elde edilecek verileri sistematik olarak belirlemek üzere; konuşma metinlerinin yazıya dökülmesi, alınan cevapların sınıflandırılması gibi işlem başmakları takip edilmiştir. Betimsel analiz ile elde edilen veriler, daha önceden belirlenen başlıklar altında özetlenmiş ve yorumlanarak sonuca bağlanmıştır. Yukarıda belirtilen durum için en uygun olarak değerlendirilen görüşme yöntemiyle veri toplanmıştır. Formdaki soruların görüşme yapılacak olan öğretim üyeleri tarafından anlaşılabilirliğinin incelenmesi için Bülent Ecevit Üniversitesinde görev alan bir öğretim üyesi ile bir pilot görüşme yapılmıştır. Pilot görüşmenin ardından görüşmecilerin gözlemleri ve görüşme yaptıkları öğretim üyesinin sorulara ilişkin değerlendirmeleri çerçevesinde formda düzenlemeler yapılmıştır. Değişikliklere ilişkin son düzenlemeler gerçekleştirilerek görüşme formuna nihai şekli verilmiştir. Görüşme Formu “Akademisyen Görüşme Formu” olarak adlandırılmıştır., Purpose: This study was conducted to reveal the views of academicians, working in the Faculty of Education, on the 2023 Vision Document of the Ministry of National Education.Method: Qualitative research method was used in this study. The study group consisted of six faculty members. In the determination of participants, easily accessible situation sampling, which is one of the purposive sampling methods, was used. A descriptive qualitative research design was used in this study; interview technique and semi-structured interview form were used as data collection tools.Findings: The findings showed that the views of academicians, who participated in this study, about the education system were based on a philosophical foundation that continues from individuality to sociality, from material dimension to spiritual dimension, and emphasizes an original national and spiritual thought system extending from local to universal. Also, results indicated that, firstly, students should gain national and universal values and then should be equipped with 21st-century skills and competencies if the courses are satisfactory concerning quality, the reduction of the course hours can be positive; emphasis can be given on the necessity of giving the pedagogical formation education rather than giving it.Implications for Research and Practice: The findings obtained from this study suggest that the views of the academicians may contribute to the successful implementation of the document. Studies should be carried out to reveal the opinions of teachers and school administrators, which are important components of education programs, within the framework of the document.

WOS:000650535000001 PMID: 33988739 The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17-77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7-183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.

Bilgisayarli nicel metin incelemesi, okuma yoluyla elde edemeyecegimiz verilere ve sonuclara ulasmamizi saglar. Bilgisayar yardimiyla dilsel birimlerin sikligi ve dagilimi olculur, dahasi, cesitli karsilastirma ve istatistiksel incelemeler yapilir. Attilâ Ilhan’in Sokaktaki Adam adli romani 22 bolumden olusur ve her bir bolum donusumlu olarak 7 ayri anlatici tarafindan anlatilir. Farkli sosyal cevrelerden gelen bu anlaticilarin her biri icin ayri bir bireysel dilin gerekliligi ortaya cikar. Bu calismada, anlaticilarin bireysel diline ait ozellikleri ortaya cikarmak icin, anlaticilara ait sozcuk siklik listesi, sozcuk bulutlari ve sozcuk dagilim grafikleri cikarilmis; sozcuk uzunlugu, sozcuk yogunlugu ve tumce uzunlugu hesaplanmistir. Bu calismanin amaci bilgisayar destegi ile metin incelemesinin uslup calismalarina ne derece katkisi olabilecegini gostermektir.

The aim of this study was to identify gram-negative anaerobic bacilli isolated from various clinical specimens that were obtained from patients with suspected anaerobic infections and to determine the antibiotic resistance profiles by using the antibiotic concentration gradient method. The study was performed in Afyon Kocatepe University Ahmet Necdet Sezer Research and Practice Hospital, Medical Microbiology Laboratory between 1 November 2014 and 30 October 2015. Two hundred and seventyeight clinical specimens accepted for anaerobic culture were enrolled in the study. All the samples were cultivated anaerobically by using Schaedler agar with 5% defibrinated sheep blood and Schaedler broth. The isolated anaerobic gram-negative bacilli were identified by using both the conventional methods and automated identification system (VITEK 2, bioMerieux, France). Antibiotic susceptibility tests were performed with antibiotic concentration gradient method (E-test, bioMerieux, France); against penicillin G, clindamycin, cefoxitin, metronidazole, moxifloxacin, imipenem, meropenem, ertapenem and doripenem for each isolate. Of the 28 isolated anaerobic gram-negative bacilli; 14 were identified as Bacteroides fragilis group, 9 were Prevotella spp., and 5 were Fusobacterium spp. The highest resistance rate was found against penicillin (78.5%) and resistance rates against clindamycin and cefoxitin were found as 17.8% and 21.4%, respectively. No resistance was found against metronidazole, moxifloxacin, imipenem, meropenem, ertapenem and doripenem. As a result, isolation and identification of anaerobic bacteria are difficult, time-consuming and more expensive when compared with the cost of aerobic culture. The rate of anaerobic bacteria isolation may be increased by obtaining the appropriate clinical specimen and appropriate transportation of these specimens. We believe that the data obtained from the study in our center may offer benefits for the follow up and treatment of infections caused by anaerobic bacteria and may contribute to the current literature. Because of high resistance rate detected against penicillin, this antibiotic should not be used as a first choice in empirical treatment. Cefoxitin may be used in empirical antimicrobial treatment of anaerobic gram-negatives; but the rate of antibiotic resistance should be detected for more useful and proper treatment. The prior selection of the most effective antibiotic, may contribute to decrease the rate of high resistance. In our study, no resistance was observed against carbapenem group antibiotics and metronidazole; so these antibiotics should be reserved as treatment options in the future for infections caused by resistant gram-negative anaerobic bacteria.

WOS: 000425633300017, PubMed ID: 28707314, Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is a rare subtype of Hodgkin's lymphoma. In this study, we aimed to investigate the clinical features and therapeutic outcomes of patients with NLPHL who were diagnosed at different institutes in Turkey. We retrospectively reviewed the records of the patients diagnosed with NLPHL. Adult patients who were diagnosed after 2005 with histological confirmation were selected for the study. Forty-three patients were included in the study. Median age of patients was 37.5years (18-70) at the time of diagnosis. About 60.5% patients were diagnosed as stage I and II NLPHL, and remaining 39.5% had stage III and IV disease. Median follow-up was 46months. During follow-up, none of the patients died. Seven patients relapsed or progressed after initial therapy at a median of 12months. Five of 7 relapsed/refractory patients (71.4%) were salvaged with chemotherapy only (DHAP, ICE), and the remaining 2 (28.6%) were salvaged with chemoimmunotherapy. All of relapsed/refractory patients were able to achieve complete remission after salvage therapy. Lactate dehydrogenase levels were significantly higher in patients with progressive disease compared with nonprogressive disease. Our study showed an excellent outcome with all patients alive at last contact with a median follow up of 46months despite a wide range of different therapeutic approaches. All relapsed and refractory patients were successfully salvaged despite a low frequency of patients received immunotherapy in conjunction with chemotherapy. Our results suggest that immunotherapy may be reserved for further relapses.

Chronic lymphocytic leukemia (CLL) is a neoplastic condition of B cells that frequently affects the lymph nodes, liver, spleen and bone marrow. The extranodal involvement of CLL is most commonly observed in the skin, whereas gastrointestinal and genitourinary involvement is rare. Renal involvement may not always present with renal failure. In this study, we aimed to present a patient with CLL infiltration in the kidney without renal failure and proteinuria.

Introduction Haemophilia patients may exhibit lower levels of bone mineral density (BMD) than the general population for a variety of reasons. Aim We aimed to investigate decreased BMD in people with severe adult haemophilia A (PWH) living in eastern Turkey, and to evaluate the related potential risk factors. Methods The study included 41 PWH and 40 healthy volunteers. Dual-energy x-ray absorptiometry (DXA) was used to measure the BMD. Blood tests and body mass index (BMI) were recorded. The Functional Independence Score in Hemophilia (FISH) test was used to measure functional ability status. Results There was a significant difference between the PWH and control groups with respect to femoral neck and total hip BMD (in g/cm(2)), but the difference for lumbar spine was not significant (P = .017, P < .001, P = .071, respectively). In PWH, patients under 50 years of age, 19.4% were found to have "lower than expected" BMD levels for their age, while 27.8% showed "low normal" levels. In PWH, osteoporosis was found in 60% of the patients over 50 and osteopenia in 20%. Vitamin D insufficiency and deficiency were present in 63.4% of the PWH, significantly higher than the control group (37.5%; P < .001). Conclusion The results indicated that the decrease in BMD was significantly greater in patients with severe haemophilia A than in the normal healthy population. This reduction was correlated with BMI, vitamin D and low functional ability status. However, in multivariate analysis, none of these was a strong independent risk factor.