Abstract:
:Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of infancy and young childhood. The clinical presentation includes recurrent unexplained fever with hepatosplenomegaly. Cytopenia, hypofibrinogenemia and/or hypertriglyceridemia and hemophagocytosis in bone marrow, spleen and lymphnode confirm the diagnosis. Hemophagocytosis may not be present at the beginning. In these cases, diagnosis is facilitated by a positive family history, a relapsing course of the disease, the frequent involvement of the central nervous system and positive findings on immunological work-up. Treatment by chemotherapy and immunosuppressants can achieve sustained remissions in most patients and reinduction of remission after relapse is possible. Most children however, eventually die from progressive disease. At present, allogeneic bone marrow transplantation is the only curative therapeutic option. Between August 1992 and May 1997 eleven consecutive patients with HLH received bone marrow from unrelated (n = 7) or matched sibling donors (n = 4). The conditioning regimen consisted of busulfan, VP-16 and cyclophosphamide. Patients engrafted after a median time of 16 days (13-43). Only one patient developed grade III acute GVHD, another patient, grade II acute GVHD. Although regimen-related toxicity was extensive, all patients have survived without signs of HLH after a median follow up of 20 months (8-63). One patient suffers from chronic GVHD, three patients reveal psychomotoric retardation and one patient has severe impairment with spastic tetraparesis, amaurosis and seizures. Our experience shows that HLH can be successfully treated by allogeneic BMT from unrelated donors.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Dürken M,Schneider EM,Blütters-Sawatzki R,Stollmann-Gibbels B,Nessler G,Bretz R,Körholz D,Probst EN,Holsten-Griffin H,Harps E,Zander AR,Janka GEdoi
10.1055/s-2008-1043876subject
Has Abstractpub_date
1998-07-01 00:00:00pages
180-4issue
4eissn
0300-8630issn
1439-3824journal_volume
210pub_type
杂志文章abstract::Since 1987, the authors have examined 186 patients (76 girls, 110 boys) with a variety of congenital and acquired heart diseases by means of magnetic resonance imaging (MRI). The patients' ages ranged from 2 days to 20 years (mean age 3.97 years). During the study new techniques were developed, which extend the applic...
journal_title:Klinische Padiatrie
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abstract::Cytological examination of cerebrospinal fluid needs special techniques of cell collecting and staining. On condition of realizing the methodical requirements cytology of the cerebrospinal fluid brings more advancement in diagnostics than other laboratory examinations. The advantages of this procedure are: 1. Meningit...
journal_title:Klinische Padiatrie
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pub_type: 杂志文章,评审
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journal_title:Klinische Padiatrie
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abstract:BACKGROUND:Congenital Central Hypoventilation Syndrome (CCHS, Ondine's curse) is a rare syndrome of dysfunction of the autonomic nervous system characterized by a decreased response to hypercarbia requiring mechanical ventilation in most cases. CCHS is an autosomal-dominant disease associated with tumors of neural cres...
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-985872
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0043-119416
更新日期:2018-03-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-07-01 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:OBJECTIVE:To investigate the change of psychological symptoms and quality of life in children and adolescents with congenital heart disease, cancer, or cystic fibrosis after a family-oriented inpatient rehabilitation program. METHODS:302 participants in the intervention (aged 4-17 years, mean=8.7) were assessed using ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0030-1262831
更新日期:2011-03-01 00:00:00
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journal_title:Klinische Padiatrie
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doi:10.1055/s-2008-1065340
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journal_title:Klinische Padiatrie
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更新日期:2000-07-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0031-1280766
更新日期:2011-12-01 00:00:00
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journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1043932
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journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1035616
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046648
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-09-01 00:00:00
abstract::Nasopharyngeal carcinoma (NPC) is a rare malignant tumor arising from epithelial cells of the nasopharynx. Its incidence is highest in Southeast Asia. Age distribution of NPC is bimodal, with one peak in young adolescents and another in patients 55-59 years of age. EBV appears to be the primary etiologic agent in the ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0041-111180
更新日期:2016-04-01 00:00:00
abstract:BACKGROUND:Lupus vulgaris (LV) is the most common form of cutaneous tuberculosis (TB) in Europe, nevertheless the overall incidence is low. It constitutes about 1.5% of all extra-pulmonary cases worldwide. A slight raise in TB incidence rates among children was recently registered in Germany, which can be explained by ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1343412
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2005-836937
更新日期:2007-01-01 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::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