Abstract:
:Primary immunodeficiency disorders (PID) are under-reported from the developing world. We present data regarding diagnosis and outcome from a hospital-based registry in India. Forty-seven patients fulfilled diagnostic criteria. Majority were males. Subgroups were disorders of immune dysregulation-29%, B&T-cell abnormalities-28%, predominant antibody deficiencies-23%, other well-defined immunodeficiencies-15%, and phagocyte disorders-4%. Molecular diagnosis was attempted in 12 and was positive in seven. Overall 24 children died. Only three out of 28 children needing stem cell transplant (SCT) underwent the same. Registry data highlights that molecular diagnosis and SCT are a rarity for children with PIDs in the developing world and mortality is high.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Chinnabhandar V,Yadav SP,Kaul D,Verma IC,Sachdeva Adoi
10.3109/08880018.2013.805346subject
Has Abstractpub_date
2014-04-01 00:00:00pages
207-11issue
3eissn
0888-0018issn
1521-0669journal_volume
31pub_type
杂志文章abstract::Adrenocortical carcinoma (ACC) is a rare, aggressive endocrine neoplasm. Complete surgical resection is the single most important treatment. Most available information has been learned from experience with its more frequent adult counterpart. In this study, we assessed the features and survival outcome of patients wit...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2019.1710309
更新日期:2020-04-01 00:00:00
abstract::The malignant cells of childhood acute lymphoblastic leukemia (ALL) do not form a homogenous entity but a collection of differently maturated blasts. The most immature leukemia cells may be more resistant to therapy than the bulk of more differentiated blasts. We studied 42 patients with childhood ALL treated accordin...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2013.859189
更新日期:2014-05-01 00:00:00
abstract::Ondansetron (Zofron, Glaxo) and tropisetron (Navoban, Sandoz) are selective serotonin (5HT3) antagonists that have proven very effective in the prevention of vomiting and nausea in adults and children receiving cancer chemotherapy. This study compared the efficacy of the two agents in the prevention of vomiting and na...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1080/088800199277425
更新日期:1999-03-01 00:00:00
abstract::Vascular endothelial growth factor (VEGF) seems to play a central role in angiogenesis-lymphangiogenesis in hematological malignancies. There are limited data related to childhood hematologic malignancies. The aim of the study was to evaluate soluble VEGF (sVEGF) levels in children with acute leukemia and malignant ly...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.493574
更新日期:2010-10-01 00:00:00
abstract::Anaplastic lymphoma kinase (ALK) inhibitors such as crizotinib and alectinib have been shown to have significant activity in ALK-rearranged non-small cell lung cancers (NSCLC). There are no data for alectinib's safety or efficacy in younger patients, though it is superior to crizotinib in adult trials. We present a 14...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2018.1541492
更新日期:2018-01-01 00:00:00
abstract:OBJECTIVE:Venous thrombotic events (VTE) are a well-recognized complication in pediatric cancer patients. Population-based data on the incidence and characteristics of VTE in all pediatric cancer patients are limited. This information is crucial to identify patients at high risk and design targeted interventions accord...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2017.1319450
更新日期:2017-03-01 00:00:00
abstract::The development of myeloid leukemias and myelodysplastic syndrome (MDS) is common in children with trisomy 8 mosaicism. However, the mechanisms by which the presence of an additional chromosome 8 translates to an increased risk of leukemias and MDS is currently unknown. The authors describe the analysis of stromal cel...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010490276917
更新日期:2004-04-01 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
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.3109/08880018.2015.1102998
更新日期:2016-02-01 00:00:00
abstract:BACKGROUND:Alterations in the tumor suppressor gene TP53 have been associated with poor outcome in adult hematological malignancies. We have earlier reported an increased expression of the TP53 encoded protein p53, in bone marrow samples from pediatric patients with aggressive leukemia. Our aim was now to evaluate p53 ...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2014.898723
更新日期:2014-05-01 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::Twelve cases of neuroblastoma (NB) (7 boys and 5 girls) and 4 cases of primitive peripheral neuroectodermal tumor (PNET) (3 boys and 1 girl) were investigated for the presence of apoptosis and retinoic acid receptor (RAR) by immunhistochemical method. The apoptotic index in NB was zero or 1% in 8 children and relative...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276307
更新日期:2000-06-01 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::During the last 5 years massive chemotherapy and autologous bone marrow transplantation have been increasingly explored in the treatment of pediatric solid tumors, mainly for neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, Wilms' tumor, germ cell tumors, osteosarcoma, and retinoblastoma. Although the disease course ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019009034318
更新日期:1990-01-01 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::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::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::Disaccharidases activity in the intestinal mucosa samples of rats was examined after intragastric and intramuscular methotrexate therapy. Methotrexate was given on a twice a week schedule (1 mg/kg body weight). The animals were sacrificed after 2 weeks, 1 month, and 2 months of this therapy. A statistically significan...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019009033397
更新日期:1990-01-01 00:00:00
abstract::All pediatric osteosarcomas treated in our hospital between 1985 and 1995 were reviewed. There were 26 patients, 15 males and 11 females, aged 20 or less at diagnosis. All had limb primaries. Nineteen patients had localized disease and seven presented with metastases. Intensive multiagent chemotherapy was given both p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019809028789
更新日期:1998-05-01 00:00:00
abstract::The aim of this study was to evaluate children with lymphadenopathy and clinical approach to the suspicion of malignancy. The authors evaluated 457 patients with peripheral lymphadenopathy, less than 19 years of age, and referred to the Pediatric Oncology Department of Gazi University Medical School during the periods...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010600856907
更新日期:2006-10-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 信件
doi:10.1080/08880010490457295
更新日期:2004-07-01 00:00:00
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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::A case of a child with paroxysmal nocturnal hemoglobinuria (PNH) is characterized by an increased sensitivity of the erythrocyte to hemolytic action of complement. The widely used Ham test may not always be reliable. Recently, a panel of monoclonal antibodies has become available to detect various glycosylphosphatidyl...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019309029514
更新日期:1993-10-01 00:00:00
abstract::Immune thrombocytopenia (ITP) is a frequently encountered disease in childhood. Recent reports pointed to the benefit of high-dose steroid in ITP treatment since it resulted in a better outcome in a shorter time than IV immunoglobulin therapy. In the authors' clinic, mainly after 1984, megadose methyl prednisolone (MD...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880010590964291
更新日期:2005-07-01 00:00:00
abstract::Outcomes in pediatric B-Non-Hodgkin Lymphoma (B NHL) have improved with intensive chemotherapy protocols, with long-term survival now over 80%. However, long-term adverse effects of therapy and poor outcomes for patients who relapse remain challenges. In this study, we aimed to evaluate the potential risks and benefit...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.834400
更新日期:2014-04-01 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
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2020.1779885
更新日期:2020-11-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:
更新日期:2003-06-01 00:00:00
abstract::Ghosal-type hemato-diaphyseal dysplasia is a rare autosomal recessive disorder with distinctive diaphyseal and metaphyseal dysplasia of long bones and steroid-dependant anemia. The authors describe a 20-month-old girl who had had a severe transfusion-dependent anemia since late infancy and marked locomotion difficulti...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:
更新日期:2003-07-01 00:00:00
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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