Abstract:
:Polycythemia vera - the most frequent form of a primary erythrocytosis in adult patients - represents an extremely rare disease in pediatric and juvenile patients as do congenital primary and secondary erythrocytoses. Frequently, in patients with these diseases clinical problems do not occur before adulthood. Systematic data on clinical and laboratory evaluations as well as on treatment regimens are sparse. In addition, in the majority of cases with congenital erythrocytosis, the etiology is unknown. For those reasons, a protocol (PV-ERY-KA 03) for the systematic collection of clinical, hematological, biochemical, biological as well as treatment data of children and adolescents with polycythemia vera or congenital erythrocytosis including the hemoglobinopathies with high affinity hemoglobin, familial 2,3-BPG deficiencies, and those of unknown origin, has been developed. These data are combined with molecular analyses which focus initially on EPO-receptor and vHL-gene examination, but will later be extended into presently unexplored pathophysiologic regulatory circuits. In addition, pathophysiologic changes due to the erythrocytosis will be studied. The co-ordinated medical care for patients with those rare diseases within a collaborative trial accompanied by scientific projects is aimed at the improvement of the treatment of these patients as well as to a better understanding of the underlying biological processes.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Cario H,Schwarz K,Debatin KM,Kohne Edoi
10.1055/s-2004-822628keywords:
subject
Has Abstractpub_date
2004-05-01 00:00:00pages
157-62issue
3eissn
0300-8630issn
1439-3824journal_volume
216pub_type
杂志文章abstract::The normal values of the LH and FSH excretion in 12-h-overnight-urine-samples were measured by the commercial technique for the estimation of LH (Luteonosticon) and FSH (FSH-nosticon). These methods were used to determine the hormone excretion of these hormones in healthy individuals, 94 boys (0-18 years) and 48 girls...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-07-01 00:00:00
abstract::In Germany, about 1,800 new cases of childhood cancer are diagnosed every year. The chances of survival have increased significantly over the last 40 years due to the continuous improvement of treatment strategies. The number of childhood cancer survivors in Germany thus ranges around 30,000 nowadays. But their treatm...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0035-1548840
更新日期:2015-05-01 00:00:00
abstract::Problems of terminology, differential diagnosis, etiology and therapy of language development disorders are demonstrated by a casuistic example. Besides the importance of clinical date special attention was laid on neuropsychological diagnostic procedures and their therapeutic implications. Therapeutic interventions c...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-09-01 00:00:00
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 aspir...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0031-1275347
更新日期:2011-05-01 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
abstract:BACKGROUND:Epidemiology and resistance patterns of bacterial pathogens in pediatric UTI show large interregional variability and rates of bacterial resistances are changing due to different antibiotic treatment. We intended to evaluate data from northern Germany. PATIENTS AND METHODS:In 100 children (53 female, 47 mal...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2004-823143
更新日期:2004-03-01 00:00:00
abstract::15 parameters of coagulation and fibrinolysis were investigated in 38 children with type I diabetes mellitus without clinical signs of diabetic angiopathy. Compared to an age matched non diabetic control group spontaneous platelet aggregation was enhanced, plasma levels for factor VIII C, von Willebrand factor, antith...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025468
更新日期:1991-11-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2002-30142
更新日期:2002-05-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034034
更新日期:1983-01-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1055/s-2008-1046460
更新日期:1996-05-01 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::A boy with dysplastic features had to be treated for recurrent diarrhoea and bronchitis since his tenth month of life. Defects in humoral and cellular immunity were found. The ecto-mesodermal abnormalities are so characteristic that in connection with the immunological changes it seems to be justified to assume a yet ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-11-01 00:00:00
abstract:INTRODUCTION:Gender dysphoria (GD) in childhood and adolescence is characterized by an incongruence between sex at birth and gender identity, which usually increases during puberty. Gender dysphoric children and adolescents often suffer from psychological comorbidities such as depression. The purpose of our study is to...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/a-1066-4625
更新日期:2020-01-01 00:00:00
abstract::Prolactin levels were measured immediately after the seizure in some, and 15 to 20 minutes later in all of 67 children aged between 6 months and 17 years. Values were determined after grand mal, complex partial and petit mal seizures and psychogen seizures. A more than 2 to 3 fold prolactin increase over the baseline ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025341
更新日期:1992-05-01 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::The presented paper includes the medical records of five children with familial adenomatous polyposis coli on the one hand and the detailed description of the pathologic findings of the disease on the other hand. Particular emphasis is given to the morphologic criteria of the disorder predisposing the affected individ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033975
更新日期:1985-05-01 00:00:00
abstract::Prognostic factors to estimate the risk of relapse are crucial for risk-adapted therapy in acute lymphoblastic leukemia (ALL). In a cooperative multicenter treatment study for childhood ALL (COALL-03-85) the prognostic relevance of the bone marrow (BM) blast count at day 28 was evaluated. Treatment was adjusted to the...
journal_title:Klinische Padiatrie
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1055/s-2007-1025434
更新日期:1991-07-01 00:00:00
abstract::During angiogenesis new blood vessels sprout from an existing vascular bed. This is a prerequisite for tumor growth beyond a certain size and for metastasis formation. Tumors produce a number of cytokines. The development of in vivo bio-assays for angiogenesis and in vitro analysis of endothelial cells permits charact...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1043960
更新日期:1997-07-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/a-0684-9794
更新日期:2018-10-01 00:00:00
abstract::This report describes the case of a 10 days old newborn of a diabetic mother with gestosis, who developed erythema and swelling of the right cheek. A diagnosis of acute suppurative parotitis due to S. aureus was made. Following this case report the most important facts of epidemiology, pathogenesis, clinical manifesta...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2004-822675
更新日期:2005-03-01 00:00:00
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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
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1975-11-01 00:00:00
abstract:BACKGROUND:Most patients with terminal renal failure show arterial hypertension. In addition to casual blood pressure measurements in the clinic, home blood pressure measurement is recommended for these patients to control arterial blood pressure. PATIENTS:The study was performed in children with hemodialysis (HD; n =...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2001-11268
更新日期:2001-01-01 00:00:00
abstract::We report the first case of non-immune hydrops fetalis (NIHF) with trisomy 15q11/17q22.5 resulting from a familial translocation 15/17. Furthermore the cytogenetic findings of 50 cases with hydrops fetalis are presented. Of the 30 cytogenetic analyzable cases 7 showed chromosomal abnormalities (4x45,X; 2x47,XY,+21; 1x...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026810
更新日期:1987-07-01 00:00:00
abstract::The control of severe infectious complications by preventive strategies, early diagnosis of infections and empiric broad-spectrum antibiotic therapy contributed to a marked improvement of survival in children with cancer over the last 20 years. This article overviews of the importance of prevention of infectious compl...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2001-17498
更新日期:2001-09-01 00:00:00
abstract::The case is described of a 6 months old infant who developed purulent pericarditis during the course of pneumonia. Pericarditis probably developed in this condition by contiguous spread from the pleura. Diagnosis of impending cardiac tamponade was masked by the occurrence of massive unilateral empyema. The management ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033831
更新日期:1982-09-01 00:00:00
abstract::The biologic behaviour of teratomas depends on various interdependent clinical and epidemiologic variables such as the age at diagnosis, sex, tumor site, histology which all correlate to different cytogenetic and molecular biologic aberrations. Thus, testicular teratomas of infancy are generally benign. Accordingly, p...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2006-942271
更新日期:2006-11-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1979-07-01 00:00:00
abstract::The phagocytic and NBT reduction indices of 94 preterm and underweight babies, divided into 7 groups and two weight classes, were observed over a period of six weeks while they were observed over a period of six weeks while they were given four commercial baby foods (Humana 0, Meb, Multival, Pomil). In all groups the ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-03-01 00:00:00
abstract::A multi-center retrospective survey was conducted to evaluate the incidence and types of hemostatic complications occurring in children with acute lymphoblastic leukemia (ALL) during treatment according to the ALL-BFM-90 treatment protocol. All of the BFM-treatment centers (n = 77) were approached and a 95% response r...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,多中心研究
doi:10.1055/s-2008-1043788
更新日期:1999-07-01 00:00:00