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 minimizing postoperative bleeding in children with type 1 VWD or low VWF activity. METHODS:We conducted a retrospective chart review in children with type 1 VWD or low VWF activity treated via an institutional protocol that utilizes repeated doses of Desmopressin acetate (DDAVP, 1-deamino 8-D arginine- vasopressin) or VWF concentrate, brief hospitalization for observation and extended use of oral epsilon aminocaproic acid (EACA). RESULTS:From 2010 to 2017, 13 children underwent an adenotonsillar procedure and were treated with this protocol. Although 7.6% had minor immediate bleeding and 23% had minor delayed bleeding, no patients experienced major bleeding or required transfusion, additional surgery or other measures not specified by the protocol. Mild hyponatremia was observed in 80% of patients who received DDAVP. CONCLUSION:Our institutional protocol specifying repeated dosing of DDAVP or VWF concentrate to sustain elevated VWF levels during periods of highest bleeding risk and extended use of EACA is effective at preventing major bleeding episodes after adenotonsillar procedures. However, this analysis raised safety concerns that prompted changes in the institutional protocol and highlight the need for further prospective studies to determine the optimal strategy for safely reducing bleeding complications in these patients.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Diaz R,Musso M,Mahoney Ddoi
10.1080/08880018.2020.1806970subject
Has Abstractpub_date
2020-08-17 00:00:00pages
1-11eissn
0888-0018issn
1521-0669pub_type
杂志文章abstract:PURPOSE:To describe clinical and CT features of L-asparaginase-associated pancreatitis (L-AP) and to correlate CT grades with clinical parameters. METHODS:A total of 16 children (M:F = 9:7; mean age, 8.1 years) who developed L-AP after L-asparaginase (L-asp) treatment and underwent abdominal CT scan were included. We ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2014.918681
更新日期:2014-10-01 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::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...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018709141261
更新日期:1987-01-01 00:00:00
abstract:BACKGROUND:Clinical data and data on outcome of extra-osseous Ewing tumors are scarce. PROCEDURE:After a search for Ewing tumors in the database of a single institution over a period of 20 years, 16 out of 192 cases were found to have extra-osseous primary tumors. RESULTS:Ages at initial diagnosis ranged from 2.5 to ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010902855581
更新日期:2009-06-01 00:00:00
abstract::To investigate the possible clinical application of the hypothesis that insufficient induction of 3,4-dihydroxyphenylalanine decarboxylase (DDC) causes accumulation and secretion of 3,4-dihydroxyphenylalanine (DOPA) in unfavorable neuroblastomas, we measured plasma DOPA in 28 neuroblastoma patients. Abnormally high le...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019609033369
更新日期:1996-01-01 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::Growth hormone deficiency (GHD) is a recognized late effect of successful treatment of tumors requiring cranial irradiation. Growth after treatment was assessed in 16 patients with sarcomas of the orbital and parameningeal regions. Median age at diagnosis was 6.35 years and median follow-up was 7.2 years. Treatment co...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800199277560
更新日期:1999-01-01 00:00:00
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::Four children with beta-thalassemia intermedia ages 7 to 11 years developed a clinical picture characterized by headache, hypertension, convulsion, and cerebral hemorrhage after blood transfusion. Successive transfusions did not result in a similar picture. Factors responsible for this syndrome are discussed. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019409141694
更新日期:1994-09-01 00:00:00
abstract::Most of the extragonadal teratomas are located in the sacrococygeal region. Teratoma with malignant sarcomatous differentiation is a rare form of germ cell tumor. The authors describe a 5-year-old-girl with sacrococygeal teratoma in which sarcomatous elements were observed. The patient was treated with complete surgic...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802016342
更新日期:2008-06-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/088800199277272
更新日期:1999-05-01 00:00:00
abstract::Since the mid-1960s intrathecal chemotherapy (methotrexate [MTX], cytarabine [Ara-C], or both, plus hydrocortisone) has constituted the standard approach to prophylaxis and treatment of central nervous system (CNS) leukemia and lymphoma. Intrathecal chemotherapy-related neurotoxicity has been described in a variable p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029588
更新日期:1995-07-01 00:00:00
abstract::Tolerance of full-dose methotrexate/6-mercaptopurine (MTX/6MP) maintenance therapy for childhood acute lymphoblastic leukemia (ALL) without side effects could reflect insufficient systemic drug exposure, and drug withdrawals due to toxicity might reduce the chance of cure. The present study included 122 children with ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019109028803
更新日期:1991-10-01 00:00:00
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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::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::Renal and metabolic complications of tumor lysis syndrome (TLS) were recognized frequently in the 1960s and 1970s. Strategies were designed to prevent TLS. We conducted a retrospective chart review study to identify the current TLS risk in children with acute leukemia. Children were considered to have "laboratory tumo...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029545
更新日期:1995-03-01 00:00:00
abstract::In five patients under the age of 15 with hemophilia and inhibitors, due to frequent joint bleedings, 13 joints were treated with synoviorthesis (intra-articular injection of radioactive gold). The patients received high doses of factor VIII/IX for 2-4 days or were treated simultaneously with tolerance induction accor...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019209018332
更新日期:1992-04-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802434818
更新日期:2009-01-01 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
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
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更新日期:2001-12-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010390232763
更新日期:2003-10-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
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更新日期:2002-06-01 00:00:00
abstract::At present, urinary vanillylmandelic acid (VMA) and/or homovanillic acid (HVA), metabolites of catecholamines, are the most sensitive diagnostic markers of neuroblastoma. They can be measured by the high-performance liquid chromatographic method, which has been used to screen for neuroblastoma in infants in Japan. Fro...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019709030914
更新日期:1997-11-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
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更新日期:2017-01-01 00:00:00
abstract::Two children with inflammatory pseudotumor (IPT) of the lung are reported. Symptomless "cystic" lesions were present on routine chest x ray. Morphological study of these peculiar lesions included light microscopic, immunohistochemical, and ultrastructural analysis. Histologic appearance of the lesion varied from the f...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019109033439
更新日期:1991-04-01 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
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更新日期:2008-06-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
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abstract::We present the case of a female newborn with life-threatening bleeding of a ruptured infantile fibrosarcoma (IFS) and consecutive multiorgan dysfunction syndrome shortly after birth. After stabilization, the tumor could be treated without amputation due to surgery, laser therapy, and chemotherapy. The patient is free ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
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更新日期:2012-09-01 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
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更新日期:2013-10-01 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
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更新日期:1990-01-01 00:00:00