Abstract:
BACKGROUND:The prognosis of children with multisystem Langerhans cell histiocytosis (LCH) has improved with the application of chemotherapy. However, treatment strategies used varied from conservative approach with treatment only during disease exacerbation to intensive chemotherapy starting immediately after diagnosis. No single drug or regimen has been proven to be superior to the others. Thus, optimal treatment of multisystem LCH remains still an unsolved problem. PATIENTS:Three hundred and twenty-four patients enrolled in the DAL-HX 83 and DAL-HX 90 studies were retrospectively re-evaluated by using the current definition for disease extent. Sixty-three patients fulfilling the criteria for multisystem LCH (involvement of > or = 2 organ systems) were object of the present study. These were 33 males and 30 females, median age at diagnosis 11.5 months (range, birth-13 years 2 months). The median observation time was 7 years 6 months (4 years-11 years 8 months). METHODS:All patients had morphologically confirmed diagnosis, which was additionally verified through demonstration of CD1a antigen, presence of Birbeck granules or central pathologic review. Uniform evaluation including a complete medical history and physical examination, laboratory tests (complete blood count, liver function tests, coagulation profile) and radiographic survey (skeletal survey and/or radionuclide bone scan) was performed in all patients. Additional investigations (bone marrow tap, CT, MRI etc.) were performed upon specific indications. The 63 patients with multisystem LCH were evaluated with respect to response to therapy, clinical course, outcome and development of permanent disabilities. The results of the DAL-HX studies were compared with the results of the first randomized international clinical trial on multisystem LCH (LCH-I). RESULTS:Response to 6 weeks of initial therapy showed a clear discrimination between responders and non-responders, with only 6% of the patients having intermediate response. When correlated to survival response to initial therapy appears to be a powerful prognosticator in multisystem LCH. There were some typical patterns of clinical course. Complete disease resolution at some point of the clinical course was documented in 50 (79%) patients. Thirty-five of them remained disease free, while 15 experienced one or more episodes of disease reactivation. Chronic reactivating course without complete disease resolution was observed in one patient. Deteriorating disease with fatal outcome was shown in 12 (19%) patients. The overall survival after 5 years of observation was 81%. One or more disease-related permanent disabilities were documented in 24 patients, in 4 of them these were shown at diagnosis and in 20 patients these developed after therapy had been commenced. Despite more intensive chemotherapy, the overall survival in DAL-HX 83/90 cohort was comparable with that in LCH-I studies. However, LCH-I compares unfavorably to DAL-HX 83/90 in some very important aspects. With respect to reactivation rate, reactivation free interval and development of permanent disabilities better results were achieved with the more intensive initial and prolonged continuation therapy concept of the DAL-HX studies. Even after extended analysis it remains unclear whether the superiority of the DAL-HX studies has to be attributed to the administration of continuous steroids, to the combination of vinblastine and etoposide, or to the prolonged continuation therapy including mercaptopurine. Answers to these questions are expected from the ongoing international clinical trial LCH-II.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Minkov M,Grois N,Heitger A,Pötschger U,Westermeier T,Gadner Hdoi
10.1055/s-2000-9667keywords:
subject
Has Abstractpub_date
2000-07-01 00:00:00pages
139-44issue
4eissn
0300-8630issn
1439-3824journal_volume
212pub_type
杂志文章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::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: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::Human growth hormone was given to 6 boys with constitutional delay of growth and development. Three patients had normal growth hormone responses following exercise or insulin-induced hypoglycemia. Subnormal growth rate (less than 2 SD below normal for bone age) was found in 4 patients. The mean growth rate of the pati...
journal_title:Klinische Padiatrie
pub_type: 临床试验,杂志文章
doi:10.1055/s-2007-1025580
更新日期:1984-03-01 00:00:00
abstract::Infants exposed to opiates antenatally display withdrawal symptoms after birth referred to as neonatal abstinence syndrome (NAS).A total of 366 newborns (166 females, 10 twins) from 361 mothers were diagnosed with NAS from 2000 to 2011 at a single large metropolitan referral center.Retrospective chart review of all ne...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0042-115300
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:Children with Epidermolysis bullosa (EB) suffer from an intractable, burdensome skin disease that may result in cognitive as well as social and emotional problems. PATIENTS:To assess cognitive problems in patients with EB, we investigated 20 affected children and adolescents, 6-17 years of age (mean: 10.8 y...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0031-1295437
更新日期:2012-01-01 00:00:00
abstract::The psychosocial and intellectual development of 4 boys with nephropathic cystinosis and brain atrophy documented by cranial computerized tomography was investigated by use of biographical data and psychological tests (HAWIK, Deutscher Rechtschreibtest). Inspite of the brain atrophy the patients showed low-normal inte...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1979-09-01 00:00:00
abstract:BACKGROUND:In the German guidelines for prophylaxis of group B streptococcal (GBS) early onset sepsis in neonates (EOS), GBS screening of all pregnant women has been recommended, but is not yet included in the Maternity Directives. Aim of the study was to identify temporal trends in incidence of EOS and their associati...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/a-1202-1758
更新日期:2021-01-01 00:00:00
abstract::In view of present-day knowledge and findings relating to the development of young children, the stimulating interaction between the child and its social and material surroundings must be considered of supreme importance. Any disturbance or radical restriction of the structure of social relationships can thus seriousl...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034076
更新日期:1984-09-01 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
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abstract::Neurological symptoms developed in a girl aged three-and-a-half years during treatment with nalidixic acid. The symptoms progressed even after the preparation had been discontinued, and culminated in the development of a transient intracranial pressure increase. ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1979-07-01 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
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046621
更新日期:1994-07-01 00:00:00
abstract:UNLABELLED:The congenital syphilis is an infectious fetopathy which is able to affect the whole organism. In most cases symptoms are not obvious before week fourth to twelve of life. The infection of the mother is the precondition for the child's disease. The placental transfer takes place after the fifth to sixth mont...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2000-9612
更新日期:2000-11-01 00:00:00
abstract:BACKGROUND:Most recent studies analyzing candidate biological prognostic factors in childhood medulloblastoma (MB) are limited by small patient numbers due to dependence on fresh-frozen tumor material. By contrast, large archives of formalin-fixed, paraffin-embedded MB samples exist from homogeneously treated patients....
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-985843
更新日期:2007-11-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0034-1372587
更新日期:2014-11-01 00:00:00
abstract:AIM:Reports describing coronavirus disease 2019 (Covid-19) in children are fewer than adult studies due to milder clinical picture. We aimed to share our experience at a single center with an emphasis on collective decision making. MATERIALS AND METHODS:A suspected case was defined as the presence of symptoms suggesti...
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doi:10.1055/a-1263-1222
更新日期:2021-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2007-03-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章,多中心研究
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更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Pachyonychia congenita is considered to be a genodermatosis of autosomal inheritance. It is characterized by nail hypertrophy, shortly present after birth. Later on follicular keratosis of the extremities and hyperkeratosis of palms and soles can be found. HISTORY AND CLINICAL FINDINGS:We report a child wit...
journal_title:Klinische Padiatrie
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pub_type: 杂志文章
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journal_title:Klinische Padiatrie
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更新日期:2004-07-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2002-34015
更新日期:2002-09-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034484
更新日期:1981-07-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 传,历史文章,杂志文章
doi:10.1055/s-0033-1337966
更新日期:2013-05-01 00:00:00
abstract::Autoimmune hemolytic anaemia (AIHA) in childhood is associated with antibodies produced by the patient himself, which coat his red cells causing their hemolysis. Although in some cases no underlying disease could be found, in the majority of children a virus etiology is apparent. There are very few reports regarding t...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-05-01 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
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更新日期:1991-01-01 00:00:00
abstract::CML is a rare malignancy in childhood not yet being treated by an established protocol of the Society of Pediatric Oncology and Hematology (GPOH). Up to now only allogeneic bone marrow transplantation (BMT) offers a curative treatment option. After being enrolled into the study "CML-päd" patients with a matched siblin...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,多中心研究
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更新日期:1996-07-01 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
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更新日期:1989-07-01 00:00:00
abstract::Iron concentration was determined by the aid of the ESA Ferrochem 3050 analyzer and the bathophenanthroline method with deproteinization. Both methods showed a good precision and accuracy. The investigation was carried out in 505 sera with an iron concentration between 9 and 333 micrograms/dl. The comparison of the tw...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
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