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Effective initial treatment of diffuse pulmonary lymphangiomatosis with sirolimus and propranolol: a case report

Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available,...

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Main Authors: Dimiene, Ieva (Author) , Bieksiene, Kristina (Author) , Zaveckiene, Jurgita (Author) , Andrulis, Mindaugas (Author) , Optažaite, Daiva-Elžbieta (Author) , Vaguliene, Neringa (Author) , Zemaitis, Marius (Author) , Miliauskas, Skaidrius (Author)
Format: Article (Journal)
Language:English
Published: 29 November 2021
In: Medicina
Year: 2021, Volume: 57, Issue: 12, Pages: 1-7
ISSN:1648-9144
DOI:10.3390/medicina57121308
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.3390/medicina57121308
Verlag, kostenfrei, Volltext: https://www.mdpi.com/1648-9144/57/12/1308
Verlag, kostenfrei, Volltext: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8706407
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Author Notes:Ieva Dimiene, Kristina Bieksiene, Jurgita Zaveckiene, Mindaugas Andrulis, Daiva-Elzbieta Optazaite, Neringa Vaguliene, Marius Zemaitis, Skaidrius Miliauskas
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Summary:Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available, which makes working with patients with DPL challenging for clinicians. We present here a case of a young man with DPL in whom treatment with sirolimus and propranolol rapidly achieved positive radiological and clinical effects.
Item Description:Gesehen am 11.04.2022
Physical Description:Online Resource
ISSN:1648-9144
DOI:10.3390/medicina57121308

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