Abstract:
:Factor X (FX) is the component of both extrinsic and intrinsic coagulation cascade and is the first enzyme of the common pathway which results in thrombus. Congenital FX deficiency (FXD) is an extremely rare coagulation defect. In this study, we aimed to investigate the clinical and laboratory data of the patients diagnosed with FXD. The files of the 15 patients (7 female, 8 male) diagnosed and followed up for FXD within the last 4 years were evaluated retrospectively. The mean age of the patients was 29 months (min-max:1-144 months). The most presenting complaints were easy bruisability (n = 8; 53%) and epistaxis (n = 8; 53%). FX levels were <1% in six patients, 1-5% in four patients, and >5% in five patients. Heparin added-Protrombin Complex was used for prophlaxy (n = 11; 73%). Any treatment-related complication was not observed. Heparin-added PCC can be used safely for effective prophlaxy. We suggest that family history is important when considering prophlaxy and in patients with life-threatening bleeding or with FXD sibling the prophlaxy should be introduced in the early course.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Karabel M,Söker M,Yıldırım AT,Oymak Y,Kelekçi S,Karabel Ddoi
10.3109/08880018.2013.782380subject
Has Abstractpub_date
2013-11-01 00:00:00pages
717-22issue
8eissn
0888-0018issn
1521-0669journal_volume
30pub_type
杂志文章abstract::Brucellosis produces a variety of nonspecific hematologic abnormalities. Hematologic complications of mild anemia and leukopenia have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. In this study, records of children with brucellosis aged under or equal to 16 years, admitte...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.536298
更新日期:2011-04-01 00:00:00
abstract::The aim of this study was to evaluate the efficacy and safety of meropenem plus amikacin compared with piperacillin-tazobactam plus netilmicin for initial empirical antibiotic treatment of high-risk febrile neutropenia in children with cancer. Patients with hematologic malignancy (leukemia or stage III/IV non-Hodgkin ...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880010490277321
更新日期:2004-03-01 00:00:00
abstract::Infantile hemangioendothelioma is the most common hepatic vascular tumor in infants less than 6 months of age, with a prevalence of 1%. Serum alpha-fetoprotein levels have been used as an important tumor marker for hepatoblastoma, hepatocellular carcinoma, and germ cell tumors. It is rarely elevated in hepatic hemangi...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.1080/08880010600954397
更新日期:2006-12-01 00:00:00
abstract:OBJECTIVE:Physiologic uptake of 18F-fluorodeoxyglucose (FDG) in brown adipose tissue (adipose tissue) of cancer patients may confound interpretation of positron emission tomography (PET) scans. Uptake in adipose tissue occurs in up to half of pediatric oncology patients undergoing PET scans, and is especially common in...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880018.2017.1338806
更新日期:2017-04-01 00:00:00
abstract::An 11-year-old girl was referred to the authors' hospital with a complaint of growth retardation. Physical examination revealed splenomegaly. Laboratory examination revealed increased sedimentation rate. Her imaging studies showed a splenic mass. Splenectomy was performed and histopathological examination revealed scl...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/07357900903114010
更新日期:2009-10-01 00:00:00
abstract::Neutropenic enterocolitis is an acute, life-threatening inflammation of the small and large bowel, often seen in children with malignancies during periods of prolonged or severe neutropenia. During the period 1990-1995, 180 children were treated at the authors' center for acute lymphoblastic leukemia using a standard ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276712
更新日期:2000-01-01 00:00:00
abstract::Chronic granulomatous disease (CGD) is a rare genetically determined immunodeficiency. Neutrophils from CGD patients show a defective killing of phagocytosed fungi and bacteria, due not only to an impairment in oxidative burst, but also to absence of normal pH value within phagocytic vacuole following phagocytosis. Be...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010590907267
更新日期:2005-03-01 00:00:00
abstract::A major problem of hemophilia A (HA) treatment is the development of factor VIII (FVIII) inhibitor, which usually occurs shortly after initiating replacement therapy. Several studies showed the correlation between inhibitor development and polymorphisms in inflammatory and immune response genes of HA patients; however...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880018.2019.1585503
更新日期:2019-02-01 00:00:00
abstract::Low iron stores in children, absolute iron deficiency (AID), can lead to impaired neurodevelopment and requires iron therapy. In the presence of infection/inflammation, like in cystic fibrosis (CF), serum ferritin (SF) is not a reliable biomarker for AID. Red blood cell distribution width (RDW) is a promising alternat...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880018.2016.1268226
更新日期:2017-02-01 00:00:00
abstract::This study investigated the influence of levamisole therapy on immunocompetent cells and lymphocyte reactivity to mitogens in 25 children with brain tumor. Eleven (11/25) patients were receiving chemotherapy and immunomodulating drug levamisole 3 months after neurosurgery, during maintenance chemotherapy, 2.5 mg/kg of...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800199277164
更新日期:1999-07-01 00:00:00
abstract::A firm, painless tumor of the temporal region was excised in a 3-year-old girl. The diagnosis of nonossifying fibromyxoid tumor was established on pathologic examination, which showed that most of the cells formed cords and tubulelike structures in a myxoid background. Bone and osteoid were lacking. The child is free ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019409140547
更新日期:1994-07-01 00:00:00
abstract::Sixty-one long-term survivors, treated for brain tumors in childhood, were evaluated in term of neurological impairments, disability, and handicap. Thirty-eight patients (pts) (62%) had at least one impairment. Visual impairment was detected in 14 pts (24%), associated with recurrence (p = .012). Thirty-four patients ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/0880010390158595
更新日期:2003-03-01 00:00:00
abstract::Ondansetron, a highly selective 5-HT3 receptor antagonist, is available in an intravenous (IV) formulation and tables, but syrup would be particularly useful in children. As chemotherapy can affect taste perceptions, this study was undertaken to determine the preference between two flavors of ondansetron syrup in chil...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.3109/08880019609030816
更新日期:1996-03-01 00:00:00
abstract::This is a pilot study performed to determine the maximum tolerated number of courses of high-dose thiotepa and carboplatin with autologous peripheral blood progenitor cell (PBPC) transplantation in poor-risk pediatric central nervous system (CNS) tumor patients. Twelve patients were enrolled and a total of 24 PBPC tra...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010490501060
更新日期:2004-10-01 00:00:00
abstract:UNLABELLED:Intravenous (IV) ferric iron (Fe)-carbohydrate complexes are used for treating Fe deficiency in children with iron-refractory iron-deficiency anemia (IRIDA). An optimal treatment has yet to be determined. There are relatively little publications on the responsiveness to IV iron therapy in children with IRIDA...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.829540
更新日期:2014-02-01 00:00:00
abstract::Between 1983 and 2008, prenatal diagnostic procedures for identifying hemoglobinopathies were performed in 947 at-risk fetuses. Seventy-six percent of the fetuses were at risk for β-thalassemia major and 16% for sickle cell anemia; only a small percentage (7%) were at risk for compound heterozygosity of β-thalassemia ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.507690
更新日期:2011-02-01 00:00:00
abstract::Hepatoblastoma, the commonest primary malignant liver tumor in infants and children, is usually associated with elevated serum alpha-fetoprotein (AFP) levels. The authors sought to determine if AFP levels can be used to modify treatment, thereby avoiding the wait for formal imaging studies and prolonged suboptimal tre...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800101753328475
更新日期:2001-12-01 00:00:00
abstract::Patients with thalassemia intermedia (TI) experience many complications, of which the incidence varies greatly among cases. Considering the high prevalence of thalassemia in Iran, the study was carried out to determine the frequency of TI complications in Iranian patients and to find possible risk factors for each of ...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2011.572144
更新日期:2011-09-01 00:00:00
abstract:INTRODUCTION:Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruc...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880018.2017.1313919
更新日期:2017-03-01 00:00:00
abstract::Neuroblastoma (NB) is the most common extracranial malignant solid tumors of childhood, and the majority of these high-risk tumors is resistant to nearly all the treatments and has a significantly worse outcome. The mammalian target of rapamycin (mTOR) plays a critical role in oncogenesis and cancer progression of man...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018.2013.798058
更新日期:2013-10-01 00:00:00
abstract::This is a report of a case of acute respiratory failure following the administration of intrathecal methotrexate (MTX) for prophylaxis of central nervous system leukemia (CNS) in a 3-year-old girl with acute lymphoblastic leukemia. This could be attributed to acute metabolic or direct toxic effects of methotrexate to ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019009033390
更新日期:1990-01-01 00:00:00
abstract::A 2-year-old girl with acute lymphoblastic leukemia (ALL) showing a t(4;11)(q21;q23) karyotype underwent allogeneic bone marrow transplantation (BMT) with the conditioning regimen of L-PAM (70 mg/m2/d for 3 days), busulfan (140 mg/m2/d for 2 days), and total body irradiation (12 Gy). On day 57, the patient developed p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019809009512
更新日期:1998-01-01 00:00:00
abstract::Ewing's sarcoma is one of the most common malignant tumors of the skeletal system in children and young adults. It most frequently stems from the long bones of the extremities. However, though uncommon, extraosseous localization can be seen. Epidural extraosseous presentations are extremely rare. In this case, the aut...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010701534163
更新日期:2007-10-01 00:00:00
abstract::It has been suggested that breastfeeding may prevent inhibitor formation in patients with hemophilia. In a single-center retrospective cohort study, the authors studied the association between breastfeeding and inhibitor development in 90 severe hemophilia A patients born 1975-2003. Mean follow-up was 13.2 years (stan...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010500198764
更新日期:2005-10-01 00:00:00
abstract::In this study, we aimed to determine serum adrenomedullin levels and compare them with levels of C-reactive protein (CRP) and procalcitonin (PCT). Cancer patients aged 0-18 years who experienced febrile neutropenia attacks were included in the study. Adrenomedullin, CRP, and PCT were analyzed at admission, day 3, and ...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2015.1057310
更新日期:2015-01-01 00:00:00
abstract::Fanconi's anemia is an autosomal recessive disorder characterized by progressive pancytopenia and congenital malformation of the skeleton. This study investigated the oral health status of 15 children with Fanconi's anemia, including oral lesions, gingival and periodontal status, and dental abnormalities. All children...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010591002413
更新日期:2005-09-01 00:00:00
abstract::The aim of the study is to evaluate the etiologic and clinical characteristics, treatment regimens, and outcome of the patients with intracranial tumors presenting with central diabetes insipidus (DI). Sixty-nine patients with intracranial tumors presenting with central DI between 1972 and 2012 were retrospectively ev...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.816984
更新日期:2013-10-01 00:00:00
abstract::Primary erythrocytosis diagnosed in a 10-month-old female and followed for 12 years is described. The erythrocytosis was associated with an abnormally elevated set point of erythropoietin production in which the sensitivity fluctuated independently, but corresponded to the alterations in the oxygen-carrying capacity o...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019109033455
更新日期:1991-07-01 00:00:00
abstract::Few studies have been performed during adolescents' cancer treatment to evaluate its interference on health-related quality of life (HRQL). The purpose of this prospective cohort study was to evaluate adolescents' HRQL during cancer treatment. The Health Utilities Index (HUI) was used for scoring. Forty-five individua...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.775617
更新日期:2013-05-01 00:00:00
abstract::A retrospective analysis was performed on febrile neutropenic episodes in patients with acute lymphoblastic leukemia (ALL) from 1992 to 2002. There were 222 febrile neutropenic episodes in 266 ALL patients with documented ANC < 500/mm(3). Of the 222 episodes, 98 (44%) had documented focus of infection; the rest were f...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802106564
更新日期:2008-06-01 00:00:00