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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/10/22 19:35:26」(JST)

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A box of Pulmozyme

Dornase alfa (proprietary name Pulmozyme from Genentech) is a highly purified solution of recombinant human deoxyribonuclease I (rhDNase), an enzyme which selectively cleaves DNA. Dornase alfa hydrolyzes the DNA present in sputum/mucus of cystic fibrosis patients and reduces viscosity in the lungs, promoting improved clearance of secretions. This protein therapeutic agent is produced in Chinese hamster ovary cells.

Cystic fibrosis

Dornase alfa is the most recent therapeutic agent developed with this basic mechanism of action. Prior to the cloning of the human enzyme, bovine DNase I was on the market for many years, though its utility was limited by the inherent antigenic response to a cow protein in the lungs of patients. Other DNases, such as DNase II, have therapeutic potential as well, but no further DNases have been brought to market yet for cystic fibrosis.

Dornase alfa is an orphan drug.^[1]

Contraindications

Hypersensitivity to dornase alfa
Hypersensitivity to Chinese hamster ovary cell products

Economy

Dornase alpha 1000 units (1mg/ml)2.5ml (2500units) = £18.52 (GBP)

Off label use

Dornase alfa has recently been shown to improve lung function in non-cystic fibrosis pre-term infants suffering from atelectasis where other therapies have failed.^[2]^[3]

References

^ Collier, Joe (1 September 1995). "Dornase-alfa and orphan drugs". The Lancet 346 (8975): 633. doi:10.1016/S0140-6736(95)91460-9.
^ Erdeve O, Uras N, Atasay B, Arsan S (2007). "Efficacy and safety of nebulized recombinant human DNase as rescue treatment for persistent atelectasis in newborns: case-series.". Croat Med J 48 (2): 234–9. PMC 2080511. PMID 17436388.
^ Hendriks T, de Hoog M, Lequin MH, Devos AS, Merkus PJ (2005). "DNase and atelectasis in non-cystic fibrosis pediatric patients.". Crit Care 9 (4): R351–6. doi:10.1186/cc3544. PMC 1269442. PMID 16137347.

UpToDate Contents

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1. 嚢胞性線維症：肺疾患治療の概要 cystic fibrosis overview of the treatment of lung disease
2. 最新の診療アップデート practice changing updates
3. 慢性閉塞性肺疾患（COPD）治療における粘膜作用剤の役割 role of mucoactive agents in the treatment of copd
4. 小児における無気肺 atelectasis in children
5. 成人における肺炎随伴性胸水および膿胸 parapneumonic effusion and empyema in adults

English Journal

Adherence to therapies in cystic fibrosis: a targeted literature review.

Narayanan S1, Mainz JG2, Gala S1, Tabori H2, Grossoehme D3.
Expert review of respiratory medicine.Expert Rev Respir Med.2017 Feb;11(2):129-145. doi: 10.1080/17476348.2017.1280399. Epub 2017 Jan 20.
INTRODUCTION: Cystic fibrosis (CF) is a life-shortening condition with no cure. Available therapies relieving the symptoms of CF are complex and time-consuming. A comprehensive review assessing adherence to different CF therapies, association of adherence with outcomes, and factors influencing adher
PMID 28107795

Pilot study of dornase alfa (Pulmozyme) therapy for acquired ventilator-associated infection in preterm infants.

Scala M1, Hoy D2, Bautista M3, Palafoutas JJ2, Abubakar K2.
Pediatric pulmonology.Pediatr Pulmonol.2017 Jan 3. doi: 10.1002/ppul.23656. [Epub ahead of print]
OBJECTIVE: Evaluate the feasibility, safety, and efficacy of adjunctive treatment with dornase alfa in preterm patients with ventilator-associated pulmonary infection (VAPI) compared to standard care.WORKING HYPOTHESIS: We hypothesize that therapy with dornase alfa will be safe and well tolerated in
PMID 28052587

Dornase alfa for cystic fibrosis.

Yang CL1, Chilvers M2, Montgomery M3, Nolan SJ4.
Paediatric respiratory reviews.Paediatr Respir Rev.2017 Jan;21:65-67. doi: 10.1016/j.prrv.2016.09.001. Epub 2016 Sep 15.
PMID 27769785

Japanese Journal

症例報告ドルナーゼアルファとトブラマイシンの吸入療法が著効した嚢胞性線維症の幼児例

日本小児呼吸器学会雑誌 = Japanese journal of pediatric pulmonology 26(1), 28-34, 2015
NAID 40020527015

嚢胞性線維症の肺機能改善薬ドルナーゼアルファ (プルモザイム^【○!R】) の薬理学的特徴および臨床試験成績

日本薬理學雜誌 = Folia pharmacologica Japonica 141(4), 213-219, 2013-04-01
NAID 10031165478

嚢胞性線維症の肺機能改善薬ドルナーゼアルファ（プルモザイム<sup>®</sup>）の薬理学的特徴および臨床試験成績

日本薬理学雑誌 141(4), 213-219, 2013
NAID 130003362553

「ドルナーゼアルファ」

Dornase alfa
Clinical data
Trade names	Pulmozyme
AHFS/Drugs.com	monograph
MedlinePlus	a694002
Pregnancy cat.	US: B;
Legal status	US: ℞-only;
Routes	Inhalation
Identifiers
CAS number	9003-98-9
ATC code	R05CB13
DrugBank	DB00003
UNII	953A26OA1Y
Chemical data
Formula	C1321H1999N339O396S9
Mol. mass	29253.9 g/mol
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