Abstract:
:The therapy of complicated Kaposiform hemangioendothelioma (KHE) is still difficult. We present the first case of laryngomalacia with simultaneous mammalian target of Rapamycin (mTOR)-positive KHE of the neck and thoracic inlet and concurrent Kasabach-Meritt Phenomenon (KMP) in an 11-month-old boy suffering life-threatening progress despite intravenous vincristine, corticosteroids, propranolol and local interstitial laser-application. The laryngomalacia restored after laser-supraglottoplasty. Successfully treatment of the prior fatal course of the KHE with KMP was initiated not till adding the mTOR inhibitor sirolimus to therapy. After 16 months single therapy of KHE with oral sirolimus the boy presented free of symptoms with minimal residual disease and excellent functional long-term results. Thus we stopped sirolimus therapy. The results are stable for 9 months without therapy. The special features including full report of histopathologic findings of this utmost complicated case are demonstrated in detail underlining the effectiveness of sirolimus for KHE.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Schroeder U,Lauten M,Stichtenoth G,Gebhard MP,Buchholz M,Kaiser MMdoi
10.1055/s-0034-1372587subject
Has Abstractpub_date
2014-11-01 00:00:00pages
362-8issue
6-7eissn
0300-8630issn
1439-3824journal_volume
226pub_type
杂志文章abstract::A boy aged 15 years is described in whom a combined autoimmune neutro- and thrombocytopenia developed since the age of 11. Cell-membrane bound IgG antibodies were detected on neutrophils and platelets. A therapy with prednisone and/or immunoglobulins showed only a transient normalization of the peripheral blood values...
journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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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
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
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journal_title:Klinische Padiatrie
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journal_title:Klinische Padiatrie
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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,评审
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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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