Aerosolised heparin in the treatment of Fontan-related plastic bronchitis

Eason, Daniel E.; Cox, Kristin; Moskowitz, William B.

doi:10.1017/s1047951112002089

Cited by 16 publications

(8 citation statements)

References 13 publications

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“…It has been reported that inhalation of TPA and heparin can improve patients with BP, but there are many adverse effects limiting their use [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Idiopathic Plastic Bronchitis

Raoufi

Achachi

Mrabet

et al. 2017

Case Reports in Pulmonology

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Plastic bronchitis is a rare disorder characterized by formation of large, branching bronchial casts, which are often expectorated. We present an interesting case of a 35-year-old woman who presented for evaluation of a chronic cough productive of voluminous secretions. Clinical and radiological examination confirmed a total left lung atelectasis without any pathological mediastinal node. Flexible bronchoscopy demonstrated tenacious, thick, and sticky whitish secretions blocking the left stem bronchus. This material was extracted, and inspection demonstrated a bronchial cast, whose pathological analysis revealed necrotic epithelial cells, some eosinophils, and Charcot-Leyden crystals. Two days after bronchoscopy, the patient rejected more bronchial casts, and dyspnea improved. Control of chest x-ray revealed complete left lung aeration and the diagnosis of idiopathic plastic bronchitis was obtained. This article shows the interest in clinical practice to evoke the diagnosis of plastic bronchitis in front of a productive chronic cough. Our case illustrates a rare clinical presentation represented by an atelectasis of an entire lung.

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“…It has been reported that inhalation of TPA and heparin can improve patients with BP, but there are many adverse effects limiting their use [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Idiopathic Plastic Bronchitis

Raoufi

Achachi

Mrabet

et al. 2017

Case Reports in Pulmonology

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“…25 There is evidence that inhibiting tissue factor with inhaled heparin may decrease the formation of these casts by decreasing fibrinogenesis. 26 …”

Section: Fibrinolytics and Tissue Factor Inhibitionmentioning

confidence: 99%

Aerosol Medications for Treatment of Mucus Clearance Disorders

Rubin

2015

Respir Care

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Airway mucus hypersecretion and secretion retention can result from inflammation, irritation, stimulation, or mucus-producing tumors. Secretion clearance can be furthered hampered by ciliary dysfunction and by weakness or restrictive lung disease, leading to an ineffective cough. There are a number of different mucoactive medications that have been used to reduce hypersecretion, make secretions easier to transport, or increase the efficiency of cough or mucus clearance. In this paper, I review the pathophysiology of secretory hyper-responsiveness and mucus hypersecretion and discuss the different aerosol medications that can be used to augment secretion clearance.

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“…Inhaled UFH use in PB has been documented in two case reports, each of which reported success with regimens of 5000 units every 8 hours and 5000 units every 4 hours during acute exacerbations . One of the patients was maintained on an inhaled regimen of 5000 units every 8 hours for an additional 12 months until she was tapered off of this therapy . Intravenous or subcutaneous administration of UFH in patients with PB has been documented in multiple case reports with variable success .…”

Section: Plastic Bronchitis Pharmacotherapymentioning

confidence: 99%

“…67 One of the patients was maintained on an inhaled regimen of 5000 units every 8 hours for an additional 12 months until she was tapered off of this therapy. 67 Intravenous or subcutaneous administration of UFH in patients with PB has been documented in multiple case reports with variable success. 14,20,68 However, one report claimed nearly complete resolution of fibrin cast formation using a regimen of UFH 5000 units/day administered subcutaneously in a patient with PB and CHD.…”

Section: Unfractionated Heparinmentioning

confidence: 99%

Pharmacotherapy Challenges of Fontan-Associated Plastic Bronchitis: A Rare Pediatric Disease

et al. 2013

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Pediatric pharmacotherapy is often challenging due to the paucity of available clinical data on the safety and efficacy of medications that are commonly used in children. This quandary is even more prevalent in children with rare diseases. Although extrapolations for dosing and administration are often made from available adult data with similar disease states, this translation becomes even more problematic in rare pediatric diseases. Understanding of disease pathophysiology is typically poor, and few, if any, effective therapies have been studied and identified. One condition that illustrates these issues is plastic bronchitis, a rare, most often pediatric disease that is characterized by the production of obstructive bronchial airway casts. This illness primarily occurs in children with congenital heart disease, often after palliative surgery. Plastic bronchitis is a highly clinically relevant and therapeutically challenging problem with a high mortality rate, and, to date, a generally accepted effective pharmacotherapy regimen has not been identified. Furthermore, the disease is ill defined, which makes timely identification and treatment of children with plastic bronchitis difficult. The pharmacotherapies currently used to manage this disease are largely anecdotal and vary between the use of macrolide antibiotics, mucolytics, bronchodilators, and inhaled fibrinolytics in a myriad of combinations. The purpose of this review is twofold: first, to highlight the dilemma of treating plastic bronchitis, and second, to bring attention to the continuing need for studies of drug therapies used in children so that safe and effective drug regimens can be established, particularly for rare diseases, which often have no treatment options.

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Aerosolised heparin in the treatment of Fontan-related plastic bronchitis

Cited by 16 publications

References 13 publications

A Rare Case of Idiopathic Plastic Bronchitis

A Rare Case of Idiopathic Plastic Bronchitis

Aerosol Medications for Treatment of Mucus Clearance Disorders

Pharmacotherapy Challenges of Fontan-Associated Plastic Bronchitis: A Rare Pediatric Disease

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