Abstract:
:An 11-year-old girl with hereditary factor-VII deficiency and her family have been studied for factor VII coagulation activity (VII:C) and factor VII antigen level (VII:Ag). The proband had 11% VII:C, whereas her VII:Ag was at a level that corresponds to about 50% coagulation activity. Forty-two members of the proband's family were tested. This study disclosed that the proband's factor VII deficiency had been inherited through both her mother and her father. Although her father was dead, testing of her father's family showed a factor VII defect of the type VII+ variant, that is, normal production of VII:Ag but reduced VII:C. The maternal side of her family was found to have reduced but identical levels of VII:C and VII:Ag (variant VIIR). The proband's factor VII deficiency is therefore apparently inherited through two different variants, and that could explain this rare genetic type of factor VII deficiency. To our knowledge no such variant has ever been described.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Svendsen J,Osterud Bdoi
10.3109/08880018709141261subject
Has Abstractpub_date
1987-01-01 00:00:00pages
145-51issue
2eissn
0888-0018issn
1521-0669journal_volume
4pub_type
杂志文章abstract::This study evaluated the effects of urokinase in the prevention of central venous catheter (CVC)-related complications in children with malignancy. Fifteen patients with 16 CVCs (study group A) received an intraluminal application of urokinase (10,000 IU in each catheter lumen for 4 h) once a week. They were monitored...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/088800102753541323
更新日期:2002-04-01 00:00:00
abstract::A 7-year-old boy with prolonged and marked leukopenia diagnosed at 6 months of age is described. The polymorphonuclear cells presented no hypersegmented nuclei or concentrated nuclear chromatin, although vacuolated myeloid cells appeared in bone marrow smears. Neutrophils reversed in response to administration of G-CS...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276532
更新日期:2000-03-01 00:00:00
abstract::A 3-year-old boy presented with recurrent strokes and pallor. Hematological investigations revealed severe iron deficiency anemia without thrombocytosis. The magnetic resonance angiogragraphy findings were suggestive of moyamoya syndrome. The association of moyamoya syndrome with severe iron deficiency anemia has not ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2011.650838
更新日期:2012-05-01 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::Three children with malignant solid tumors developed hyponatremia with renal sodium wasting associated with other signs of tubular dysfunction, such as hypokalemia or hypomagnesemia, a few days after cis-diammine dichloroplatinum (CDDP) administration. The normalization of serum electrolyte disturbances was obtained w...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018709141287
更新日期:1987-01-01 00:00:00
abstract::Euthyroid sick syndrome is related to profound changes in thyroid metabolism induced by nonthyroidal diseases. To determine whether children with newly diagnosed Hodgkin disease might present thyroid abnormalities and to establish their predictive value, the authors performed regular thyroid function testing. Seven ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010151114886
更新日期:2001-04-01 00:00:00
abstract::A 20-year-old female developed a relapse of B-precursor acute lymphoblastic leukemia (ALL) as a mass in her left breast after 6 years of maintained continuous complete remission. No leukemic lesions were identified in other sites such as the bone marrow or cerebrospinal fluid. The relapsed leukemic cells in the breast...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802258399
更新日期:2008-09-01 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::Advancements in surgery, radiotherapy, and chemotherapy have greatly improved the cures rates for children with Wilms tumor. However, a number of therapy-related late effects have been observed in long-term survivors. Generally, late complications are a consequence of treatment type and intensity; the use of radiother...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880010903019344
更新日期:2009-09-01 00:00:00
abstract::The authors report the results of 58 children with ALL in 2CR after related (n = 31) or unrelated (n = 27) AHSCT. Characteristics at diagnosis and initial and after relapse antileukemic treatment were similar in the related donor (RD) and the unrelated donor (UD) groups. Conditioning consisted of TBI/CY +/- VP-16 for ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802016557
更新日期:2008-06-01 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::Acute basophilic leukemia is a distinct entity of Acute Myeloid Leukemia (AML) with primary differentiation to basophils. Increased basophil count has been described in AML cases with translocation t(6;9)(p23;q34) or other chromosomal abnormalities. We describe a 15-year old female teenager with AML and excess periphe...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2014.883655
更新日期:2014-03-01 00:00:00
abstract::Indoor radiation, especially radon exposure, has been in focus in the public domain during the past several years. The growing concern among parents of children with cancer possibly having high radiation levels in their homes led us to study the levels of gamma- and alpha-radiation levels in the homes of a group of ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018709141249
更新日期:1987-01-01 00:00:00
abstract::A prospective, population-based registration of children with immune thrombocytopenic purpura (ITP) was performed in Norway in 1996 and 1997. Ninety-two cases were identified, indicating an incidence of 5.3 per 100,000 children under 15 years. The sex ratio (female/male) was 1.2/1. Fifty-six percent presented with cut...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010050122816
更新日期:2000-10-01 00:00:00
abstract::Hyperleukocytosis in patients with leukemia is associated with early mortality, especially due to the pulmonary and neurological complications of leukostasis. The prompt use of leukapheresis may improve patients' survival in the initial treatment period. The medical records of all previously untreated acute leukemia p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.818747
更新日期:2014-05-01 00:00:00
abstract::Signaling between leukemia cells and nonhematopoietic cells in the bone marrow microenvironment contributes to leukemia cell growth and survival. This complicated extrinsic mechanism of chemotherapy resistance relies on a number of pathways and factors, some of which have yet to be determined. Research on cell-cell cr...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.1080/08880018.2017.1395938
更新日期:2017-01-01 00:00:00
abstract::The difference between the effects of administration of recombinant human granulocyte colony-stimulating factor (rhG-CSF) and recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) was studied in 39 children with neutropenia secondary to chemotherapy (absolute neutrophil count (ANC) less than 1,...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880019509030769
更新日期:1995-11-01 00:00:00
abstract::Neuroblastoma is the most common solid tumor in childhood and is the most frequent neural crest tumor (NCT). More than 90% of the patients excrete high levels of vanilmandelic acid (VMA) and homovanillic acid (HVA) in the urine. Original biochemical methods for measuring these two metabolites of catecholamines employe...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019709009495
更新日期:1997-05-01 00:00:00
abstract:INTRODUCTION:Children with low von Willebrand factor (VWF) activity or type 1 von Willebrand disease (VWD) have increased risk of bleeding after adenotonsillar procedures and the optimal perioperative management to minimize bleeding is unknown. AIM:To report the effectiveness and safety of an institutional protocol in...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2020.1806970
更新日期:2020-08-17 00:00:00
abstract::Three cases of vitamin B12 deficiency that occurred during infancy are presented. These cases appeared to be the result of pre-existing maternal deficiency. All three infants demonstrated evidence of neurodevelopmental delay at presentation, and one had sustained loss of milestones and developed involuntary motor move...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018909034282
更新日期:1989-01-01 00:00:00
abstract::Infections remain a serious complication in pediatric oncology patients. To determine if daily bathing with Chlorhexidine gluconate can decrease the rate of nosocomial infection in pediatric oncology patients, we reviewed rates of infections in pediatric oncology patients over a 14-month span. Intervention group recei...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2015.1013588
更新日期:2015-01-01 00:00:00
abstract::The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2011.642941
更新日期:2012-02-01 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::Patterns of relapse were determined for 20 high-risk neuroblastoma patients treated with chemotherapy, surgery, primary and metastatic site radiation (21 Gray), myeloablative chemotherapy, peripheral blood stem cell rescue, and 13-cis-retinoic acid. The median follow-up duration after transplant is 21 months (range, 8...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:
更新日期:2003-01-01 00:00:00
abstract::Homozygous beta-thalassemia is usually characterized by severe anemia requiring regular blood transfusion for survival. For homozygous patients with milder clinical manifestations and no dependence on transfusion therapy, the term thalassemia intermedia is usually applied. Genetic mechanisms that may ameliorate the cl...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029526
更新日期:1995-01-01 00:00:00
abstract::This study evaluates the efficacy of heparinization in prolonging patency of arterial and central venous catheters in children. A randomized double-blind trial in a tertiary 10-bed pediatric intensive care unit was used to evaluate 300 children (age older than 4 weeks, younger than 18 years). Trial medication consiste...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1080/08880010290097404
更新日期:2002-12-01 00:00:00
abstract::Histone deacetylase inhibitors (HDACis) are epigenetic agents that display antitumor activities in experimental medulloblastoma (MB). Fingolimod (FTY720), an immunosuppressant agent currently used in the treatment of multiple sclerosis, also has anticancer actions and can act as an HDACi. Here we examined whether fing...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2019.1699213
更新日期:2020-03-01 00:00:00
abstract::Malignant rhabdoid tumors (MRT) of soft tissues are aggressive tumors, which can be detected in almost any part of the body. MRT are rare, and very few cases have been reported in the literature. Prognosis of these tumors is extremely poor despite intensive therapy. Some risk factors such as young age or disseminated ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2014.914112
更新日期:2015-04-01 00:00:00
abstract::Growth was studied longitudinally in 19 children who were long-term survivors after acute lymphoblastic leukemia (ALL). Of the children, 13 were girls; 6 were boys. They had all undergone a 3-year cytostatic treatment period which included vincristine, adriamycin, asparaginase, methotrexate, purinethol, and prednisone...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019209016589
更新日期:1992-07-01 00:00:00
abstract::The aim of this study was to establish reference values and factors associated with serum AFP elevation in infants. Five hundred twenty-four samples collected from infants up to the age of 2 years at the University Hospital Düsseldorf (Germany) were analyzed. At birth mean serum AFP levels were 41,687 ng/ml in 256 ter...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019809167228
更新日期:1998-03-01 00:00:00