Abstract:
OBJECTIVES:Despite about 3 decades of clinical experience with the therapy of inherited thrombocytopathies (HTP) with desmopressin (DDAVP) the mechanisms of haemostatic effects of DDAVP in these diseases remain unclear. Therefore platelet function diagnostics was carried out in whole blood (WB) from children with aspirin-like defect as one of the clinically mild forms of HTP after DDAVP administration. DESIGN AND METHODS:11 children (age range: 3-16 years) were treated with DDAVP i.v. (0.3 μg/kg as short infusion). Before, after 120, and 240 min of DDAVP administration the following parameters were measured: platelet aggregation (PA) and ATP release induced by ADP, collagen, ristocetin and thrombin; PFA-100 closure times (CT), factor VIII activity (FVIII:C), Von Willebrand factor antigen (VWF:Ag), collagen binding activity (VWF:CB) and blood count. RESULTS:PA, ATP release and blood count were not influenced by DDAVP administration. PFA-100 CTs were markedly reduced at 120 and 240 min after DDAVP, respectively. FVIII:C, VWF:Ag and VWF:CB were increased after 120 min. CONCLUSION:The DDAVP-induced improvement of primary haemostasis in patients with aspirin-like defect is mainly due to the marked increase of the VWF. For the evaluation of the clinical effect of DDAVP administration in patients with aspirin-like defect the investigation of a larger group of patients is needed.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Tauer JT,Gneuss A,Lohse JE,Jürgens T,Knöfler Rdoi
10.1055/s-0031-1275347subject
Has Abstractpub_date
2011-05-01 00:00:00pages
169-72issue
3eissn
0300-8630issn
1439-3824journal_volume
223pub_type
杂志文章abstract:BACKGROUND:It has been shown that Interleukin 10 (IL-10) is able to inhibit alloreactivity in a mixed lymphocyte culture. Therefore, here IL-10 production in patients after allogeneic BMT was investigated and correlated with the incidence of acute GvHD. PATIENTS:14 patients after allogeneic BMT have been investigated....
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046464
更新日期:1996-07-01 00:00:00
abstract::The Thorax-Tube according to Matthys and its successful application in 18 premature and mature newborns and older children is described. The advantages of this new device are discussed. ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-03-01 00:00:00
abstract::A case of a severe heroin withdrawal syndrom in a newborn infant is reported. Diagnostic and therapeutic aspects of the disease are reviewed. ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1976-11-01 00:00:00
abstract::A case of arteriovenous aneurysm of the great vein of Galen with congestive heart failure in a newborn infant is presented. Efficiancy of medical management and stop of growth of the head circumference point towards a good prognosis. Patients with this malformation have been divided into four clinical groups presentin...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-09-01 00:00:00
abstract::Report of accidental ingestion and aspiration of lamp oil of a boy aged 16 months. He develops in a few hours a severe pneumonitis with tachycardia. Echocardiography shows diminished contractability. After losing his symptoms, the chest x-rays on the 13th day show bilateral pneumatoceles. The course remains asymptomat...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1043916
更新日期:1998-11-01 00:00:00
abstract:BACKGROUND:Intrauterine growth restriction seems to be a risk factor for an aggravated course of secondary renal diseases in children. Catch-up growth after birth may play a critical role. We tested if there is an association between an aggravated course of nephritis in Henoch-Schönlein Purpura (PSHN) and low birth wei...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0030-1263135
更新日期:2010-12-01 00:00:00
abstract:BACKGROUND:Spinal compression can be a complication of neuroblastoma (NBL). Delayed or insufficient treatment of this condition may lead to permanent neurological sequelae. Therefore, appropriate treatment should be introduced promptly. Therapeutic options include neurosurgery, chemotherapy, and radiation therapy. CAS...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1065340
更新日期:2008-05-01 00:00:00
abstract::Corticosteroids for treatment or prevention of pulmonary disease are milestones in the management of preterm infants nowadays. Prenatal maternal administration as well as postnatal treatment of the mechanically ventilated preterm infant with bronchopulmonary dysplasia (BPD) are considered to be established concepts. H...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1043969
更新日期:1997-09-01 00:00:00
abstract::The progression of alimentary fatty liver to liver cirrhosis is a very rare observation. During the year after surgical extirpation of a suprasellar craniopharyngioma in a seven years old boy developed severe obesity and again six years later at autopsy a complete liver cirrhosis with fatty liver was established. Inju...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046584
更新日期:1994-01-01 00:00:00
abstract::The selective uptake and accumulation of 131J-Metaiodobenzylguanidine in neuroblastoma cells in vivo may be utilized for targeted irradiation. The experience with 32 neuroblastoma patients refractory to conventional high dose chemotherapy is reported. At diagnosis 8 patients had Evans stage III and 22 stage IV. 11/32 ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033713
更新日期:1988-05-01 00:00:00
abstract::In a randomised study the efficacy of a cytomegalic hyperimmune globulin preparation (CMV-HIGP) which had been treated with beta-propiolactone was analysed. The study included 85 patients with acute lymphoblastic leukemia (ALL) and Non-B-Non-Hodgkin-lymphoma (NHL) who were treated initially or underwent a relapse ther...
journal_title:Klinische Padiatrie
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1055/s-2008-1026722
更新日期:1989-07-01 00:00:00
abstract::The observation of constitutional chromosome abnormalities in a one and half year old boy with Wiedemann-Beckwith syndrome (WBS) and hepatoblastoma prompted us to review the currently available literature dealing with this subject. In approximately 500 published cases with complete and incomplete forms of WBS, 42 mali...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026804
更新日期:1987-07-01 00:00:00
abstract::Infectious heart diseases in childhood are--with less than 1% of hospital admissions--rare, but serious diseases. Among several causes of myocarditis in our region virus myocarditis plays the most important role. Besides of the acute course an autoimmune mediated chronic myocarditis and the transition to dilated cardi...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2007-1025390
更新日期:1991-01-01 00:00:00
abstract::The following paper is the manuscript of an oral presentation during the annual GPOH meeting on May 22nd, 2000 in Berlin. Following her experiences as a member of the psychosocial care team of a children's cancer unit the author discusses the emotional impact of stemcell or bone marrow transplantation in children. Psy...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2001-16857
更新日期:2001-07-01 00:00:00
abstract::Guillain Barré syndrome (GBS; Synonyma: Polyneuritis, Polyradikulitis) is an acute, inflammatory, demyelinating disease of the peripheral nerve system. Clinical hallmarks are symmetric muscle paralysis, areflexia and pronounced autonomic disturbances. Respiratory failure and cardiovascular instability are the main rea...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-971050
更新日期:2008-03-01 00:00:00
abstract:BACKGROUND:Current resuscitation guidelines recommend the use of simulation-based medical education (SBME) as an instructional methodology to improve patient safety and health. We sought to investigate the evidence-base for the effectiveness of SBME for neonatal and pediatric resuscitation training. METHOD:Therefore, ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0034-1372621
更新日期:2014-09-01 00:00:00
abstract::An unbalanced structural chromosome aberration was found in a 6-year-old girl. Mother and maternal grandmother were shown to be carriers of a complex balanced translocation involving chromosomes 1, 5 and 15 and are of normal phenotype. The unbalanced status in the proband was defined as a partial trisomy 1p21-1p31. Pa...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026754
更新日期:1987-01-01 00:00:00
abstract:BACKGROUND:Burning pain of red and warm hands and/or feet are the classical symptoms of erythromelalgia. CASE REPORT:We describe the symptoms of acute idiopathic erythromelalgia and arterial hypertension in a five-year-old boy. Five days after a gastroenteritis the patient developed burning hands and feet in combinati...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1043837
更新日期:1999-11-01 00:00:00
abstract::Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disease. The clinical manifestations are diverse. Some of the skeletal changes are most relevant to the patient. We report on 9 patients with NF1 who presented with typical pseudarthrosis. In 8 of these children the lower extremity was involved. In 2 cas...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2000-9647
更新日期:2000-01-01 00:00:00
abstract::The German Society of Pediatric Oncology (GPO) designed a cooperative study to improve the outlook of patients with malignant (testicular) germ cell tumors. According to stage and histology of the tumor different surgical approaches to retroperitoneal lymphadenectomy are suggested. Local radiotherapy is not recommende...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034068
更新日期:1983-05-01 00:00:00
abstract:BACKGROUND:A fast and comprehensive diagnostic by means of whole-body CT has been shown to reduce mortality in the adult trauma population. Therefore whole-body CT seems to be the standard in adult trauma-patients. Due to the higher radiation exposure of whole-body CT the use of this diagnostic toll in pediatric trauma...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,多中心研究
doi:10.1055/s-0035-1547311
更新日期:2015-07-01 00:00:00
abstract::Glutaracidemia/glutaraciduria type I is an acute or subacute neuropathic disorder of infancy or early childhood. The following symptoms characterize the clinical course: macrocephalus present at birth, cerebral atrophy revealed by CT or MRI scans, most striking in the frontal and temporal lobes, choreoathetosis and dy...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025211
更新日期:1993-03-01 00:00:00
abstract::In the present study the possibility of immunophenotyping of routinely prepared (air dried) peripheral blood and bone marrow smears is described after storage. The immunoenzymatical alkaline-phosphatase-anti-alkaline-phosphatase (APAAP)-method was carried out in differently stored blood smears (+4 degrees C and -80 de...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025311
更新日期:1989-07-01 00:00:00
abstract::The case of a newborn with duplications of rectum, presenting as a tumor of the labia majora, and duplication of bladder and urethra is reported. In addition malformations from the VACTERL-association (sacral malformation, anal atresia with vestibulum fistula, renal dysplasia, only eleven pairs of ribs with hypoplasia...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026776
更新日期:1987-03-01 00:00:00
abstract::The oscillatory resistance to respiration (Ros) was measured in 218 children and 260 adults using the Siregnost FD5 (Siemens, Erlangen), and compared with whole body plethysmography (Raw) and with spirometry. There was a markedly better correlation between Ros and Raw (r=0.82;n=88) in children than in adults (r=0.53;n...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034434
更新日期:1981-03-01 00:00:00
abstract::630 neonates with risk factors of perinatal hearing impairment were screened of hearing loss by means of registration of transient otoacoustic emissions before discharge from the newborn nursery. Neonates were screened additionally by means of brainstem evoked response audiometry, if they had bilateral negative emissi...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1043853
更新日期:1998-03-01 00:00:00
abstract:BACKGROUND:Due to rising survival rates in cancer and autoimmune diseases fertility preservation before gonadotoxic therapies has become increasingly important. Although fertility can be significantly affected by gonadotoxic therapies, the possibility of fertility preservation during childhood has not been sufficiently...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0044-100396
更新日期:2018-02-08 00:00:00
abstract:BACKGROUND:The aim of the present study was to evaluate paediatric reference values for platelet function using a point-of-care whole blood impedance aggregometry. METHODS, RESULTS & CONCLUSION: In 265 healthy infants and children aged journal_title:Klinische Padiatrie pub_type: 杂志文章 doi:10.1055/s-0030-1249081 更新日期:2010-05-01 00:00:00
abstract:BACKGROUND:Neurotrophins and their cognate receptors TrkA, TrkB and TrkC regulate proliferation, differentiation and death of neuronal progenitor cells and may be implicated in the progression of medulloblastoma and other primitive neuroectodermal brain tumors (PNET). These common childhood brain tumors are composed of...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2000-10044
更新日期:2000-07-01 00:00:00
abstract::Since 1962, desferrioxamine (deferoxamine, DFO) has been utilized for the treatment of secondary hemosiderosis. For about 30 years, DFO therapy has been performed as nightly continuous subcutaneous infusion. About 20 years ago, the first oral iron chelator (deferiprone, DFP) was presented. Concerns about potential sid...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2007-973845
更新日期:2007-05-01 00:00:00