Children with Behçet Disease-associated Thrombosis: A Single-Center Experience

Behçet disease (BD) is a systemic vasculitis that can be complicated with thrombosis, which is an important cause of mortality and morbidity. The course of BD is more severe, and the diagnosis is usually delayed. In children, thrombosis associated with BD is very rare. In this study, we aimed to evaluate the characteristics of children with BD complicated with thrombosis. Forty-six patients with BD who were followed-up at a pediatric rheumatology department between January 2012 and September 2019 were evaluated retrospectively. Thrombosis was detected in 10 patients (21.7%), and it was the first sign of BD in 7 patients. Four patients had cerebral sinus venous thrombosis, 4 patients had deep-vein thrombosis, 1 patient had renal vein thrombosis, 1 had pulmonary artery thrombosis, and 1 had intracardiac thrombosis. None of the patients had arterial thrombosis. All patients had received anticoagulant therapy with immunosuppressive treatment. Any complication due to anticoagulant therapy was not detected. One patient had recurrent thrombosis, and none of the patients died during follow-up. Vasculitic diseases such as BD may cause a predisposition to thrombosis, and thrombosis might be the first sign of BD. Therefore, in children presenting with unprovoked thrombosis, BD should also be investigated. © 2021 Lippincott Williams and Wilkins. All rights reserved

Evaluation of Leukemia and Solid Tumors in Refugee Children in Turkey: A Tertiary Center Experience

Cancer care is progressively became as a significant worldwide challenge. Wars can cause destructions and delays in cancer diagnosis and treatment of displaced people. Cancer cure rates need to be improved in indefensible populations such as refugees. In this study, we purposed to highlight the clinical peculiarities and outcomes of refugee children with cancer in our hospital. Our purpose was to present our findings and contribute to improve the health care for these children. Seventy one refugee pediatric patients admitted to the oncology and hematology units of our hospital between April 2011 and January 2019 were included in this study. The demographic characteristics of the patients at the initial diagnosis, their countries of origin, living conditions, histopathological diagnoses, treatments, relapse, and mortality data were analyzed retrospectively from the patient files. The median age of patients was 6.5±4.5 years, and the male-to-female ratio was 39/32. While 44 patients (61.9%) presented with complaints and had primary diagnoses in our hospital, the remaining 27 patients (38.1%) were diagnosed in their country and applied to our hospital for treatment. Our mean follow-up period was 18.2±18.8 months (1-90 months). As a result, 44 patients (62%) were alive and 22 (31%) were dead. The survival rate without relapse in the second year was 83.6%. Two and fiveyear survival rates were 77.5% vs. 58.1% respectively. Compared to Turkish children, lower survival rates were found in refugee children. In addition to cancer-specific factors such as tumor type and stage, some problems such as shelter, communication, adherence to treatment, and difficulties supplying medicine may be responsible for lower survival rates in refugee children. Further studies are needed to improve the survival rates of patients

Low-Energy Multilevel Vertebral Fracture in a Pediatric Patient during Follow-up for Idiopathic Hypercalciuria: A Case Report

Çocuklarda hiperkalsiürinin kemik mineral yoğunluğu üzerindeki etkilerini araştıran çalışmalar erişkinlerle yapılan çalışmalara göre kısıtlıdır. Bu olguda idiyopatik hiperkalsiüri (İH) tanısı ile takip edilen bir hastada gelişen düşük enerjili çok seviyeli vertebra fraktürü anlatılmış ve çocuklarda da İH'ye bağlı kırık gelişebileceğine dikkat çekilmesi amaçlanmıştır. On yaşındaki erkek çocuk, yaklaşık 50 cm yükseklikten zıplama sonrası sırtta oluşan ağrı şikayeti ile polikliniğimize başvurdu. Hastanın özgeçmişinde İH tanısıyla 8 yıldır dış merkezde takip edildiği soy geçmişinde, babasında böbrek taşı ve hiperkalsiüri hikayesi bulunmaktaydı. Muayenesinde, alt torakal ve lomber vertebralar üzerinde hassasiyet ve hareket kısıtlılığı dışında anormallik saptanmadı. Tam kan sayımı ve biyokimyasal parametreleri alkalen fosfataz yüksekliği dışında normaldi. Çekilen dorsal ve lomber lateral direk grafilerinde T4, T6, T8 ve L3 de kompresyon fraktürü olduğu tespit edilmesi üzerine lomber ve dorsal vertebra manyetik rezonans görüntülemesi (MRG), kemik mineral dansitometresi (KMD) ve vitamin D düzeyi ölçümü istendi. MRG'de L3 de akut kompresyon fraktürü ve medüller ödem, T4, T6 ve T8 de kronik osteoporotik kırık saptandı. KMD'de ise lomber total Z skoru -2,9 ve vitamin D düzeyi de 13,7 ng/ml (10-24 ng/ml arası orta düzeyde eksiklik) idi. Kontrol renal ultrasonografide böbrek taşı veya kalsifikasyon izlenmedi. Hastaya polietilenmold torakolomber korse reçete edilerek, hiperkalsiüri ve nefrokalsinozisi arttırmayacak şekilde D vitamini desteği başlandı. Takiplerde hastanın ağrısının azaldığı gözlendi. Toplam kemik kitlesinin önemli bir kısmının çocukluk çağında kazanıldığı göz önünde bulundurularak bu hastalığa neden olan faktörlerin erken tanımlanması ve önlem alınmasının ileride gelişebilecek problemlerin önüne geçilmesinde sağlayacaktır.Compared to adult studies, there are a limited number of pediatric studies exploring the effects of hypercalciuria on bone mineral density. The aim of this paper was to report a case of low-energy multilevel vertebral fracture in a pediatric patient during follow-up for idiopathic hypercalciuria (IH); it was also attempted to remind clinicians that IH-induced fractures may also occur in children. A 10-year-old male child presented to our outpatient clinic with back pain after jumping off from a height of approximately 50 cm. History of the patient showed that the patient had been followed-up for idiopathic hypercalciuria for 8 years and his father had renal stones and hypercalciuria. There was no abnormality on physical examination, with an exception for tenderness and limitation of movement in lower thoracic and lumbar vertebrae. Complete blood count and biochemical parameters were normal except for an elevated alkaline phosphatase level. Dorsal and lumbar lateral plain graphs showed compression fractures of T4, T6, T8, and L3 vertebrae, therefore, lumbar and dorsal vertebral magnetic resonance imaging (MRI), bone mineral densitometry (BMD), and vitamin D level measurement were ordered. MRI revealed acute compression fracture and medullary edema in L3; there were also chronic osteoporotic fractures in T4, T6, and T8. BMD showed a lumbar total Z score of -2.9 and the Vitamin D level was 13.7 ng/mL (10-24 ng/mL indicates moderate deficiency). A control renal ultrasonography revealed no kidney stones or calcification. The patient was prescribed polyethylene mold thoracolumbar corset and vitamin D support at a dose not to enhance hypercalciuria and nephrocalcinosis. His pain was alleviated at follow-up. Considering that most of the total bone mass is acquired at childhood, identification of causative factors and taking necessary measures at an early stage may prevent future complications of I

Central nervous system thrombosis in pediatric acute lymphoblastic leukemia in Turkey: A multicenter study

BackgroundIn patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increases due to hemostatic changes secondary to the primary disease and due to treatment-related factors. In this multicenter study, we aimed to research the frequency of central nervous system (CNS) thrombosis occurring during treatment, hereditary and acquired risk factors, clinical and laboratory features of patients with thrombosis, treatment approaches, and thrombosis-related mortality and morbidity rates in pediatric ALL patients. ProcedurePediatric patients who developed CNS thrombosis during ALL treatment from 2010 to 2021 were analyzed retrospectively in 25 different Pediatric Hematology Oncology centers in Turkiye. The demographic characteristics of the patients, symptoms associated with thrombosis, the stage of the leukemia treatment during thrombosis, the anticoagulant therapy applied for thrombosis, and the final status of the patients recorded through electronic medical records were determined. ResultsData from 70 patients with CNS thrombosis during treatment, out of 3968 pediatric patients with ALL, were reviewed. The incidence of CNS thrombosis was 1.8% (venous: 1.5 %; arterial: 0.03%). Among patients with CNS thrombosis, 47 had the event in the first 2 months. Low molecular weight heparin (LMWH) was the most commonly used treatment with a median of 6 months (min-max: 3-28 months). No treatment-related complications occurred. Chronic thrombosis findings occurred in four patients (6%). In five (7%) patients who developed cerebral vein thrombosis, neurological sequelae (epilepsy and neurological deficit) remained. One patient died related to thrombosis, and the mortality rate was 1.4%. ConclusionCerebral venous thrombosis and, less frequently, cerebral arterial thrombosis may develop in patients with ALL. The incidence of CNS thrombosis is higher during induction therapy than during other courses of treatment. Therefore, patients receiving induction therapy should be monitored carefully for clinical findings suggestive of CNS thrombosis