Abstract:
:Review of a rare multisystem disorder with the main symptoms achalasia of the esophagus, alacrimia and glucocorticoid deficiency as well as multiple other symptoms especially of the nervous system. Report of one case diagnosed in the age of 11.5 years with a case history of about 7 years. Description of the danger to live of the affected patients primarily by the underlying glucocorticoid deficiency in various stages with bearing the peril of hypoglycemic shock and even death after heavy stress conditions.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Hübschmann Kdoi
10.1055/s-2008-1046527subject
Has Abstractpub_date
1995-05-01 00:00:00pages
126-9issue
3eissn
0300-8630issn
1439-3824journal_volume
207pub_type
杂志文章,评审abstract:BACKGROUND:Children suffering from life limiting diseases are frequently cared for by adult palliative care teams due to missing paediatric structures in that field. However it is questionable whether palliative care curricula for physicians comprehensively cover issues of paediatric palliative care (PaedPC). AIM:To i...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0035-1569266
更新日期:2016-01-01 00:00:00
abstract:BACKGROUND:Late effects become progressively more important for the evaluation of therapeutic success in paediatric oncology. Thus, in 1998, the Late Effects Surveillance System (LESS) started to register and assess multicentrally, prospectively and longitudinally late effects of treatment for the group of Ewing's, sof...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2005-836503
更新日期:2005-05-01 00:00:00
abstract::Carcinomas of the plexus choroideus (PCC) represent the rare malignant variety of plexus choroideus papillomas and take a particularly unfavourable course. Tumors of the plexus choroideus account for about 2-4% of all primary brain tumors in children and 0.5% of those in adults. The PCC is more frequent in children th...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1046511
更新日期:1995-03-01 00:00:00
abstract::While leadpoisoning has been with mankind since the dawn of civilization, only in recent years this disease was recognized as a serious public health problem. It has been estimated that more than half a million children are suffering from lead intoxication in the U.S.A. In most instances this disease is caused by eati...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1975-09-01 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
abstract:BACKGROUND:The association between Hepatitis B virus infection and membranous nephropathy has been confirmed by sources in several countries. Most commonly, the illness is seen as a nephrotic syndrome. Optimal treatment remains undefined. Antiviral therapies observed with recombinant human interferon alpha may be the b...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2000-9689
更新日期:2000-09-01 00:00:00
abstract:BACKGROUND:The aim of most of the trials of the Competence Network Malignant Lymphoma is to optimize the standard treatment of lymphoma using only registered drugs in the case of medicinal therapies (quality assurance protocols). In contrast to regulatory trials, special regulations for quality assurance protocols are ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2003-45493
更新日期:2003-11-01 00:00:00
abstract:BACKGROUND:One of the strongest predictive factors for therapy outcome in childhood acute lymphoblastic leukaemia (ALL), treated according to ALL-BFM protocols, is the response to initial prednisone treatment. Prednisone response is characterized by the peripheral leukaemic blast count. The threshold value for the char...
journal_title:Klinische Padiatrie
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1055/s-2001-16848
更新日期:2001-07-01 00:00:00
abstract::A congenital diverticulum of the ventricle is a very rare cardiac lesion which is almost always associated with additional cardiovascular anomalies. In the majority of cases a diverticulum of the heart has been diagnosed during cardiac catheterization for evaluation of additional cardiovascular anomalies. We report no...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025221
更新日期:1993-05-01 00:00:00
abstract::A two month old boy with Fournier's Gangrene of the scrotum as a result of unnecessary needle puncture of the hydrocele is being reported. The importance of appropriate diagnosis operation and drug-therapy is also described. ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034104
更新日期:1984-11-01 00:00:00
abstract::In 37 children with extrahepatic biliary atresia between 12 days in 9 years of age we performed 72 duplexsonographic examinations of the portal system and the hepatic artery. In the control group we investigated 61 healthy children. All investigations were performed with computer sonography (Acuson 128) with a 3.5 MHz...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025481
更新日期:1990-01-01 00:00:00
abstract::340 consecutive patients with neuroblastoma stage IV were analyzed for the possible impact of chemotherapy on general condition, remission status, event free survival and survival. The children entered the trials NB 79, NB 82 and NB 85 of the German Pediatric Oncology Society (GPO). The patients did benefit from chemo...
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-2007-1025531
更新日期:1990-07-01 00:00:00
abstract::Wegener's granulomatosis is together with Churg-Strauss syndrome and microscopic polyangiitis a very rare cause of hemoptysis in childhood. We report on a 14 years old girl who developed arthralgia, purpura, glomerulonephritis and a diffuse alveolar haemorrhage with hemoptysis after a short time period of unspecific s...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2005-836847
更新日期:2006-01-01 00:00:00
abstract::From 1968--1978 a longitudinal study was performed concerning development of thorax, upper arm, thigh and calf circumferences in 709 boys and 711 girls 2--15 years old. In boys the mean increase in thoracic circumference amounted to 32.9 cm, in upper are circumference to 8.3 cm, in thigh circumference to 20.6 cm and i...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033856
更新日期:1980-01-01 00:00:00
abstract::In 20 children with mumps and mumps meningitis thrombocyte functions, the thrombopoiesis and the platelet count were examinated over a four week period. The spontaneous, standardized platelet adhesion and aggregation on silikonized glass surface showed a statistically significant increase from the 5th to the 15th day ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-09-01 00:00:00
abstract::Since 1980, a growing number of pediatric patients with mutism following posterior fossa surgery have been recognized. This syndrome typically affects children and in rare cases young adults who become mute one or two days after tumor operation but do not show disturbances of consciousness or language comprehension. T...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1043886
更新日期:1998-07-01 00:00:00
abstract::A HPLC-method is described to determine the Pseudouridine/creatinine ratio in spontaneous urine samples in infancy and childhood. The urines of 74 healthy children between 1 and 18 years of age and of 231 children with different diseases were examined for this ratio, making 1097 measurements. 157 children suffered fro...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025295
更新日期:1989-05-01 00:00:00
abstract:BACKGROUND:Drug handling in paediatric intensive care units (PICU) is prone to medication errors. We aimed to identify type and prevalence of those errors and to assess preventative interventions. METHODS:Prospective intervention study investigating a 3-step intervention for preventing errors in drug handling in a 10-...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0033-1364030
更新日期:2014-04-01 00:00:00
abstract::In our experience the prognosis of hematological relapse in patients with acute lymphatic leukemia treated according to the Pinkel programme is very bad. It may be a little better for patients in whom relapse occurred after the end of therapy and who were submitted to intensified cytostatic treatment. The chances for ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::Positive anti-DNA-antibodies and lowered C3 and C4 levels were found in serum of a 13 year old girl presenting with edema, microscopic hematuria and proteinuria. Renal biopsy revealed diffuse endo- and extracapillary glomerulonephritis with mesangial deposits of IgA, IgG, IgM, C1q, C3 and fibrinogen. In spite of treat...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034411
更新日期:1983-11-01 00:00:00
abstract::16 term and preterm neonates received intravenous nutriton for at least three days. The dosage was 1--2 g amino acids, 1 g of fat, and 8--10 g glucose per kg body weight and day. Compatibility was investigated by daily determinations of the amino acid pattern in the serum and measurement of free fatty acids and trigly...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1979-07-01 00:00:00
abstract::48 patients between one and seventeen years of age were stimulated with GRF (1-29) NH2. Seventeen of them showed a constitutional delay in growth and puberty, twenty-three suffered from growth-hormone deficiency (GHD) and eight had a suspected GHD as a result of pharmacological tests. Although the results demonstrate ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025510
更新日期:1990-05-01 00:00:00
abstract:BACKGROUND:Intracellular antibodies (intrabodies) have been used for the generation of phenotypic knockouts in vivo by surface depletion of extracellular or transmembrane proteins. Intrabodies present an alternative to methods of gene inactivation that target genomic DNA or m-RNA, such as RNA interference. Several stud...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,收录出版
doi:10.1055/s-2006-933415
更新日期:2006-05-01 00:00:00
abstract:BACKGROUND:There is a subgroup of pediatric patients with an immature immunophenotype of proB-ALL that still poses a therapeutic challenge, even if the overall prognosis in B cell precursor acute lymphoblasic leukemia (BCP-ALL) is very good. Due to impaired treatment response these patients are prone to suffer relapse ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0028-1086028
更新日期:2008-11-01 00:00:00
abstract:BACKGROUND:For quick detection of neonatal early-onset bacterial infection (EOBI) pro-inflammatory cytokines like Interleukin-6 (IL-6) and Interleukin-8 (IL-8) in combiantion with C-reactive Protein (CRP) have been used. Automated determination of immature myeloid information (IMI) seems to be an additional useful tool...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0034-1395552
更新日期:2015-03-01 00:00:00
abstract::The aims of trial NHL-BFM 90 for patients (pts) suffering from B-cell neoplasms are to prove whether: 1. Treatment for pts with complete resection can be reduced from 3 to 2 therapy courses; 2. Results for pts of stage III with large abdominal tumors can be improved by high dose (HD) chemotherapy; 3. Survival for pts ...
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-2008-1046610
更新日期:1994-07-01 00:00:00
abstract::Immune neutropenia in infancy comprises alloimmune neutropenia and autoimmune neutropenia. Both diseases are caused by granulocyte-specific antibodies. Patients are newborns and children, often being younger than three years. Neutropenia usually leads to recurrent mild bacterial infections. Beside the blood count and ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1026742
更新日期:1989-11-01 00:00:00
abstract:BACKGROUND:Gender differences in overall neonatal survival and in short term pulmonary outcome have been reported. Furthermore gender differences in childhood chronic lung disorders have been described all in favor of females. METHODS:A typical survey on published data regarding gender differences in lung development ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1333758
更新日期:2013-03-01 00:00:00
abstract::Purpose To determine the population prevalence and risk factors of myopia in children and adolescents in Germany. Methods The prevalence of myopia in Germany was computed using data from the 2003-2006 German Health Interview and Examination Survey for Children and Adolescents (N=17 640, 49% girls). Status of myopia wa...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0043-102938
更新日期:2017-07-01 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