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 of the blood, when the hematocrit was lowered by phlebotomies. Extensive work for secondary erythrocytoses failed to demonstrate a recognizable cause for this abnormal erythropoietin production. Erythroid cell cultures from peripheral blood mononuclear cells showed the existence of at least two populations: one consistent with dramatic expansion of the erythron in keeping with enhanced sensitivity to endogenous erythropoietin, and the other consistent with the features of typical colonies derived from burst-forming units-erythroid (BFU-Es), seen in normal peripheral blood on days 12 to 14 of culture. The expanded population was characterized by the appearance of single colonies on days 4 to 6 and enormous response to the increasing amounts of erythropoietin, which enhanced their number, size, and maturation. The combination of clinical and in vitro data as well as the absence of any abnormality in the erythropoiesis of the parents and sibling suggest that the erythrocytosis in this child represents a new form with a benign course.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Kalmantis T,Kalmanti Mdoi
10.3109/08880019109033455subject
Has Abstractpub_date
1991-07-01 00:00:00pages
221-30issue
3eissn
0888-0018issn
1521-0669journal_volume
8pub_type
杂志文章abstract::Primary bone marrow lymphoma (PBML) is hard to diagnose in children, due to the difficult identification of malignant cells in bone marrow. The first case, a 5-year-old boy, showed knee swelling with an intermittent fever. The second case, a 12-year-old girl, showed fever of unknown origin without lymphadenopathy or h...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2018.1459984
更新日期:2018-03-01 00:00:00
abstract::Thalassemic children are at a high risk of graft rejection in cord blood transplantation. To investigate this possible mechanism, the authors evaluated the effect of panel reactive antibody on the growth of CD34(+) cells in vitro. On semisolid medium, CD34(+) cells derived from cord blood were incubated with thalassem...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010902976098
更新日期:2009-07-01 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::A 12-year-old female is reported who presented with right hip pain for 6 months. With massive splenohepatomegaly and leukocytosis, CML was suspected and confirmed on bone-marrow examination and cytogenetics. Further investigations confirmed avascular necrosis (AVN) of the right femoral head. CML was treated by hydroxy...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.831961
更新日期:2014-09-01 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::Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF 1). A substantial number of plexiform neurofibroma causes morbidity. Various treatment modalities are considered to decrease pain. In this paper a case with plexiform neurofibroma causing severe pain...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802315983
更新日期:2008-09-01 00:00:00
abstract::Li-Fraumeni syndrome (LFS) is one of the familial cancers characterized by different tumors and hereditary TP53 mutations. The adrenocortical carcinoma (ACC) association with acute leukemia is unusual in childhood, even in LFS. The authors here present a family with pR337P mutation in TP53 gene who had a child with ac...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880011003663374
更新日期:2010-05-01 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::The aim of this study was to investigate the impact of therapy on long-term gonadal function of young people cured of childhood lymphomas and to assess whether a prepubertal state during the treatment protects the gonads from chemotherapy and/or radiotherapy late effects. Clinical evaluation, semen analysis, and endoc...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276415
更新日期:2000-04-01 00:00:00
abstract::The objective of this study was to ascertain the reactions and experiences of parents whose children were defined as false-positive cases in a research program of screening 6-month-old babies for neuroblastoma. Parents of seven of the eight infants falling into this category participated in the study. Parents of five ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019409141651
更新日期:1994-03-01 00:00:00
abstract::According to the Fifth National Wilms Tumor Study (NWTS-5), tumor-specific loss of heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of patients with Wilms tumor (WT) who despite having favorable histology (FH) have a significantly increased risk of relapse and death. We aimed to find out 1p and 16q ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2015.1071902
更新日期:2015-01-01 00:00:00
abstract::The authors report their experience with 70 pediatric patients with spinal cord compression (SCC) due to malignancies identified among 898 patients with solid tumors. An extradural tumor was the most frequent cause of SCC (71%); 54% of these were soft tissue sarcomas and neuroblastoma. Most intradural tumors (70%) wer...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:
更新日期:2003-09-01 00:00:00
abstract::Beta-thalassemia major is a severe, transfusion-dependent anemia that also causes infertility due to iron deposition to endocrine organs after overtransfusion. Very few pregnancies have been reported among such patients after modern therapies. In this study, 32 women with thalassemia who were admitted to Ali Asghar Ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.1080/08880010500313306
更新日期:2006-01-01 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::Focal nodular hyperplasia (FNH) of the liver is rare in children, and it is usually diagnosed through a biopsy of the liver or hepatectomy. The authors report a case of a 10-year-old girl with multiple focal nodular hyperplasia lesions of the liver after the completion of tumor therapy for advanced neuroblastoma, and ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880010903464206
更新日期:2010-04-01 00:00:00
abstract::A discussion of a 5-year-old child with congenital hemolytic anemia and severe hypotonia caused by triosephosphate (TPI) deficiency is presented. The complexities in the diagnosis and management of this condition is discussed and the relevant literature is reviewed. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019809018318
更新日期:1998-11-01 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::A prospective, open-label, randomized, comparative study in pediatric cancer patients was conducted to evaluate the efficacy and safety of cefepime and meropenem in the empiric therapy of febrile neutropenic patients. Febrile episodes were classified as microbiologically documented infection, clinical documented infec...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,随机对照试验
doi:10.1080/08880010500506867
更新日期:2006-04-01 00:00:00
abstract::Two children are described who presented with fever and generalized seizures, days 50 and 200, respectively, after matched unrelated donor-bone marrow transplantation. Upon antiepileptic treatment the seizures vanished but somnolence and fever remained. Magnetic resonance imaging (MRI) of the brain was performed and r...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010050034337
更新日期:2000-07-01 00:00:00
abstract::The majority of the anemias during childhood are hypochromic and microcytic. The aim of the present study was to determine the status of α-thalassemia mutations and its association with other etiologies, such as iron deficiency anemia (IDA) and β-thalassemia trait, that are frequently seen hypochromic microcytic anemi...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2012.661831
更新日期:2012-04-01 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:AIM:To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS:Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH)...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880010903447375
更新日期:2010-04-01 00:00:00
abstract::The purpose of this study was to evaluate the feasibility and tumor response of 3 cycles of sequential high-dose chemotherapy (HDCT) in advanced pediatric solid tumor patients. Medical records of 11 children who underwent 2 consequent courses of reduced conditioning HDCT followed by final HDCT with autologous HSC infu...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/14992020903352226
更新日期:2010-02-01 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 study examined both the endogenous and exogenous (therapy-related) pharmacokinetics of the cytokine granulocyte-macrophage colony-stimulating factor (GM-CSF) in neutropenic children with solid and systemic malignancies. The daily endogenous GM-CSF serum concentration before application was 29 pg/mL. During the 10...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800199276796
更新日期:1999-11-01 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::Ten children receiving 5 to 6 week courses of radiotherapy after brain tumor surgery were given ondansetron treatment for persistent nausea and emesis. All patients continued the ondansetron treatment until the end of their radiotherapy course. Nausea, emesis, appetite, and adverse events were scored throughout the on...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880019609030823
更新日期:1996-05-01 00:00:00
abstract::The percentage of chemotherapy-induced necrosis in primary tumors corresponds with outcome in several childhood malignancies, including high-risk metastatic diseases. In this retrospective pilot study, the authors assessed the importance of postchemotherapy necrosis in high-risk neuroblastoma with a histological and c...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.526684
更新日期:2011-03-01 00:00:00
abstract::We report a patient who developed chronic myelogenous leukemia (CML) at 12 months of age. She was treated aggressively with stem cell transplant (SCT), interferon, donor lymphocytes and imatinib, with subsequent molecular progression. She received dasatinib, achieving a complete molecular response. Dasatinib was disco...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2020.1751755
更新日期:2020-08-01 00:00:00
abstract::Thrombo-embolism in childhood is a multifactorial disorder. The present study is a case-control study that investigated the role of genetic and acquired risk factors in 60 children with thrombosis and compared the results with the controls. Acquired and inherited risk factors precipitating thrombosis were present in 7...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010600646324
更新日期:2006-07-01 00:00:00