Secondary severe thrombocytosis in a patient who underwent splenectomy due to hereditary spherocytosis and its treatment using hydroxyurea

Thrombocytosis is a frequently seen condition during childhood. While it usually develops secondarily due to reasons such as infection or anemia, it may rarely develop due to clonal causes. Thrombocytosis becomes a life-threatening condition by causing severe complications such as hemorrhage and thrombosis development. Treatment is not recommended in patients who are asymptomatic and with a platelet count below 1,500,000/mm3, however, treatment is required in cases who are symptomatic and with a platelet count above 1,500,000/mm3 in conditions such as primary thrombocytosis. This article present the outcomes of a patient who was treated using low-dose hydroxyurea when he developed severe thrombocytosis after splenectomy

The effects of close kinetic chain exercises on proprioception and physical activity level in pediatric patients with hemophilia

Introduction: Knee joint proprioception is affected, and lower extremity functioning declines over time in patients with hemophilia A. Aim: To investigate the effects of a structured exercise programme consisting of the close kinetic chain (CKC) exercises on proprioception and physical activity level in pediatric patients with hemophilia. Methods: A total of 21 patients with hemophilia A who had at least one target knee joint were randomized into three groups: Study Group (SG, n = 7), Conventional Treatment Group (CTG, n = 7) and Control Group (CG, n = 7). The SG received a structured, lower limb-specific exercise protocol consisting of CKC exercises 2 days a week for 12 weeks, in addition to prophylactic treatment. The CTG received exercise training as described in the published literature. The CG continued to receive prophylactic treatment during the study period. Proprioception was measured using a digital goniometer before and after treatment in open and closed kinetic chain positions (CKCPs). The Five Times Sit to Stand (STS), Timed Up and Go (TUG) and Functional Independence Score in Hemophilia were used for the assessment of physical activity level. Results: A significant pre/post-treatment difference was found among the groups in proprioception (p =.001) and physical activity level (TUG p =.008, STS p =.001, FISH p =.006). Improvements in proprioception and physical activity level were greater in the SG compared to the other two groups (p <.05). Conclusion: Compared to conventional exercise, the structured exercise protocol consisting of CKC exercise training produced improvements in proprioception and physical activity in patients with hemophilia A. © 2022 John Wiley & Sons Ltd

Hemophilia patients treated with radioactive synovectomy

AMAÇ: Kolay, ucuz, invaziv olmayan, hastanede çok kısa süre kalmanın yeterli olduğu, gerektiğinde tekrarlanabilen bir girişim olan radyoaktif sinovektomi, yeterince faktör kullanılmayan ülkemiz gibi gelişmekte olan ülkeler için eklem sakatlıklarını önlemede değerli bir tedavi şekli olarak görünmektedir. Amaç, hedef eklemde kanama sıklığını azaltmak ve henüz kalıcı hasar oluşmadan kıkırdağı ve mevcut eklem fonksiyonlarını korumaktır. GEREÇ VE YÖNTEMLER: Çocuk Hematoloji Ünitemizde Hemofili A tanısıyla takipli ve radyoaktif sinovektomi yapılan yaşları 3-12 arasında değişen sekiz hastamızın verileri retrospektif olarak incelendi. BULGULAR: Radyoaktif sinovektomi; 8 Hemofili A hastasının 10 ayrı eklemine uygulandı. Uygulama günü preop faktör düzeyleri %50 olacak şekilde faktör uygulandı ve 3 gün sürdürüldü. Hastalarımızda herhangi bir komplikasyon gelişmediği gözlendi. SONUÇ: Hastalarımızda ortalama takip süresi 4 ay-2 yıl (9 ay) idi. Takipleri esnasında herhangi bir komplikasyon izlenmedi. Tüm hastalarda hedef eklemdeki kanama sayısı en az % 50 düzeyinde azaldı. Radyoaktif sinovektomi, ileri dönem hemofilik artropati bulguları gelişmeden hastalara uygulanabilirse daha iyi sonuçlar alınabilecektir. Hastaların ağrı derecelendirmesi ve klinik bulguları değerlendirildiğinde, radyosinovektominin hastanın yaşam kalitesinin iyileştirilmesi açısından önemli olduğu gözardı edilmemelidir.OBJECTIVE: Radioactive synovectomy is an easy, cheap,noninvasive, reproducible procedure when necessary and need short time to stay in hospital. It is a valuable treatment modality for prevention of joint disability in developing countries like our country in where there is not enough factor for using. The aim is to reduce frequency of bleeding in targeted joint and to preserve the available joint function and cartilage before permanent damage has occurred. MATERIALS AND METHODS: This is a retrospective analysis of our 8 patients with diagnosis of hemophilia A and treated with radioactive synovectomy in our pediatric hematology unit. RESULTS: Their ages varried between 3-12 years old. Radioactive synovectomy was applied to 10 different joints of these 8 hemophilia patient. The factor was given during the day of treatment and continued for 3 days so as to maintain the 50% factor level. No complication was seen in our patients. CONCLUSION: Radioactive synovectomy raised popularity recently in hemophilia management and it is valuable in prevention of possible joint disability. When comparing with surgical synovectomy, radiosynovectomy has many advantageous. This treatment modality is becoming more attractive in patients with recurrent hemartrozis of hemophilia patients because of it is a minimal invasive, easy usable procedure and does not need hospitalization, preserving joint movement ability, need of coagulation factor replacement is low and rehabilitation period is short. When considering pain scoring and clinical signs of patients, radiosynovectomy should not be ignored especially from aspect of improving life quality of patients

Evaluation of the Viscoelastic Properties of Lower-Extremity Muscles of Pediatric Hemophilia Patients Using Myotonometric Measurements

This study aimed to evaluate the viscoelastic properties of lower-extremity muscles in pediatric hemophilia (FVIII-IX) patients. The study included 20 severe- and moderate-type right-dominant hemophilia patients diagnosed with hemophilia A–B and 20 healthy children. Viscoelastic properties (tone, stiffness, elasticity) of the lower-extremity muscles were measured using a MyotonPRO device. The physical characteristics of the pediatric hemophilia patients (mean age: 11.9 ± 3.95 years) and the control group (mean age: 12.6 ± 3.41 years) were found to be similar. A difference was observed only in the elasticity of the right vastus lateralis (p p > 0.05). The dominant-side vastus lateralis muscle elasticity (the ability of the muscle to regain its original shape after contraction or removal of an external force) of hemophilia patients was found to be lower compared to healthy children. The fact that 45% of hemarthroses occur in the knee joint and that recurrent bleeding may affect the flexibility of the vastus lateralis, which is the main muscle within the quadriceps muscle group and responsible for the stabilization of the patella, can be associated with the study results

Is YouTube a Reliable Source of Medical Information About Hemophilia?

Objective: YouTube videos have an important role in public health. This study aimed to assess the content, quality, and reliability of hemophilia videos on the YouTube platform. Methods: The keywords, namely, hemophilia, hemophilia factor, hemophilia inhibitors, and hemophilia treatment were used to identify videos on YouTube. The Global Quality Scale and the modified DISCERN questionnaire were used to assess the quality and reliability of videos, respectively. The videos were compared based on their quality and uploaders (physician, healthcare organization, company, patient, and independent users). Results: A total of 140 videos were reviewed for the study. There were 65 high, 51 moderate, and 24 low-quality videos. The physicians uploaded more videos and high-quality videos than others, with a few videos uploaded by patients and healthcare organizations. The like ratio was different for high-quality videos (P .05). The median Global Quality Scale and DISCERN scores of all videos were the same (3 points) and DISCERN scores increased with improved video quality (P < .05). Various significant differences were found in video characteristics between uploader groups (P < .05). The Global Quality Scale score of pysicians was significantly different when compared to other groups (P < .05). The DISCERN scores of physician videos were different compared to the patient and independent users-sourced videos (P=.006 and P=.001) and DISCERN scores of patient videos were also different from those of healthcare organization videos (P=.004). Conclusion: YouTube can be considered as a good source of information on hemophilia for people with hemophilia and may improve their self-management skills

The Assessment of Quality of Life, Depression and Anxiety in Siblings of Children with Cancer: A Case-Control Study

OBJECTIV

Eosinophilic leukemoid reaction in a male adolescent with Löeffler syndrome

The Löeffler syndrome is characterized by pulmonary infiltrates on a chest x-ray accompanied with peripheral eosinophilia. In this article, we have highlighted the Löeffler syndrome complicated with a eosinophilic leukemoid reaction in a previously healthy boy. The patient was treated with albendazole for five days, with a successful result. In countries where parasitic diseases are endemic, the Löeffler syndrome must always be considered in patients who present with a eosinophilic leukemoid reaction