Extrapleural Bronchial Artery Ligation for Life-Threatening Hemoptysis in Cystic Fibrosis

Angiology ◽  
2000 ◽  
Vol 51 (9) ◽  
pp. 787-792 ◽  
Author(s):  
H.K. Kaukuntla ◽  
K.M.A. Amer ◽  
D. Honeybourne ◽  
D.E. Stableforth ◽  
J.F. Khalil-Marzouk
Keyword(s):  
Cystic Fibrosis ◽  
Bronchial Artery ◽  
Artery Ligation ◽  
Life Threatening

Management of an unusual cause of life-threatening haemoptysis: a multidisciplinary approach

2021 ◽  
Vol 14 (5) ◽  
pp. e240448
Author(s):  
Kim Pramanik ◽  
Philip Webb ◽  
RanaShoaib Hamid
Keyword(s):  
Cystic Fibrosis ◽  
Interventional Radiology ◽  
Tranexamic Acid ◽  
Medical Management ◽  
Multidisciplinary Approach ◽  
Bronchial Artery ◽  
Successful Resuscitation ◽  
Emergency Medical ◽  
Life Threatening

We present to you a case of life-threatening haemoptysis secondary to non-cystic fibrosis bronchiectasis complicated by bronchial artery pseudoaneurysms. We discuss this patient’s emergency medical management using intravenous tranexamic acid, which resulted in successful resuscitation and eventual survival, and evaluate the need for urgent anaesthetic and interventional radiology input in such a case.

PRO–CF–MED, Clinical Proof of concept for a RNA–targeting Oligonucleotide for a Cystic fibrosis–F508del MEDication, Horizon2020

Impact ◽  
2018 ◽  
Vol 2018 (3) ◽  
pp. 52-54
Author(s):  
Nicolas Lamontagne
Keyword(s):  
Cystic Fibrosis ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Rna Targeting ◽  
Threatening Condition ◽  
Life Threatening ◽  
Cftr Protein ◽  
Disease Modifying ◽  
Cystic Fibrosis Transmembrane ◽  
Specific Challenge

Cystic fibrosis (CF) is a progressive life–shortening disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene leading to a dysfunctional CFTR protein. The disease affects over 70,000 patients worldwide and while many mutations are known, the F508del mutation affects 90% of all patients. The absence of CFTR in the plasma membrane leads to a dramatic decrease in chloride efflux, resulting in viscous mucus that causes severe symptoms in vital organs like the lungs and intestines. For CF patients that suffer from the life threatening F508del mutation only palliative treatment exist. PRO–CF–MED addresses the specific challenge of this call by introducing the first disease modifying medication for the treatment of the CF patients with F508del mutation. The PRO–CF–MED project has been designed to assess the potential clinical efficacy of QR–010, an innovative disease modifying oligonucleotide–based treatment for F508del patients. Partners within PRO–CF–MED have generated very promising preclinical evidence for QR–010 which allows for further clinical assessment of QR–010 in clinical trials. PRO–CF–MED will enable the fast translation of QR–010 towards clinical practice and market authorisation. PRO–CF–MED has the potential to transform this life–threatening condition into a manageable one.

Outcomes Following Bronchial Artery Embolisation for Haemoptysis in Cystic Fibrosis

2017 ◽  
Vol 40 (8) ◽  
pp. 1164-1168 ◽  
Author(s):  
W. G. Flight ◽  
P. J. Barry ◽  
R. J. Bright-Thomas ◽  
S. Butterfield ◽  
R. Ashleigh ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Bronchial Artery ◽  
Bronchial Artery Embolisation

Control of Tubercular Haemoptysis by Bronchial Artery Embolization

1997 ◽  
Vol 27 (3) ◽  
pp. 149-150 ◽  
Author(s):  
Sanjeev Mani ◽  
Rajesh Mayekar ◽  
Ravi Rananavare ◽  
Deepti Maniar ◽  
J Mathews Joseph ◽  
...  
Keyword(s):  
Bronchial Artery ◽  
Bronchial Artery Embolization ◽  
Artery Embolization ◽  
Selective Embolization ◽  
Bronchial Arteries ◽  
Bleeding Vessel ◽  
Life Threatening ◽  
Abnormal Bleeding ◽  
Recurrent Haemoptysis

Thirty-seven patients presenting with massive or recurrent haemoptysis secondary to tuberculous aetiology were subjected to bronchial artery angiography. Of these, failure to catheterize the bleeding vessel occurred in two patients while embolization was withheld in two patients due to the presence of anterior spinal artery arising from a common intercosto-bronchial trunk. Immediate arrest of bleeding was performed in the remaining 33 patients by selective embolization of the abnormal bronchial arteries with a resorbable material (Gelfoam). Regular follow up for a duration of 6 months after the procedure revealed relapse of haemoptysis in four patients; three were treated by re-embolization of the abnormal bleeding vessels while one patient died due to aspiration immediately on admission. No recurrence of bleeding was seen in the remaining 29 patients. It is concluded that bronchial artery embolization is an effective treatment for immediate control of life-threatening haemoptysis.

scholarly journals Can newborn screening be cost benefit procedure if preventing serious complications of cystic fibrosis?

2021 ◽  
pp. 020-023
Author(s):  
Šubat-Dežulović Mirna ◽  
Pelčić Gordana ◽  
Valković Ana ◽  
Flajšman-Raspor Sanja ◽  
Pelčić Goran ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Newborn Screening ◽  
Metabolic Alkalosis ◽  
Cost Benefit ◽  
Inherited Disease ◽  
Eu Countries ◽  
Life Threatening ◽  
Long Term Prognosis ◽  
The Cost

Cystic Fibrosis (CF) is a common inherited disease with reported mean prevalence of 0.737/10,000 in 27 EU countries (Farrell J Cyst Fibros. 2008). Still, many EU countries have not implemented CF in the Newborn Screening (NBS) programme, including our country. We report the case of a 7-month-old boy whose presenting signs of CF were life-threatening neurological symptoms caused by severe metabolic alkalosis and hypoelectrolytemia. By presenting this case, we argue hoping to persuade the authorities in any country that the available newborn screening for CF is the cost benefit procedure in preventing life treating consequences with the obvious impact on the long-term prognosis of this chronic disease.

Two Bronchial Artery Aneurysms in Cystic Fibrosis

2021 ◽  
Author(s):  
Elif S. Duran ◽  
◽  
Mina F. Hanna ◽  
Keyword(s):  
Cystic Fibrosis ◽  
Bronchial Artery

scholarly journals Sphenopalatine Artery Ligation for Life-Threatening Epistaxis in a 4-Year-Old Child With Glanzmann Thrombasthenia

2019 ◽  
Vol 98 (7) ◽  
pp. 405-406
Author(s):  
Nayellin Reyes-Chicuellar ◽  
Neela Mouli Doddi ◽  
Akash Kalro ◽  
Hemi Patel
Keyword(s):  
Artery Ligation ◽  
Glanzmann Thrombasthenia ◽  
Sphenopalatine Artery ◽  
Life Threatening

Life-Threatening Hemoptysis

2020 ◽  
Author(s):  
Himanshu Deshwal ◽  
Ankur Sinha ◽  
Atul C. Mehta
Keyword(s):  
Bronchial Artery ◽  
Vascular Anatomy ◽  
Massive Hemoptysis ◽  
Evidence Based ◽  
Airway Protection ◽  
Artery Embolization ◽  
Life Saving ◽  
Advanced Therapies ◽  
Life Threatening ◽  
And Control

AbstractLife-threatening hemoptysis (LTH) is any amount of hemoptysis that causes significant hemodynamic decompensation or respiratory distress which may lead to death if left untreated. While the amount of hemoptysis that qualifies as massive hemoptysis has continued to be debated, any amount between 100 to 1,000 mL/day is considered significant. Up to 15% cases of hemoptysis are LTH and need urgent life-saving intervention. Understanding of pulmonary vascular anatomy is of paramount importance to manage LTH. The goal of treatment lies in airway protection, appropriate oxygenation, and prevention of exsanguination. Once the airway is stabilized, a quick diagnosis and control of bleeding site is targeted. This chapter highlights current practices and approach to LTH including medical management, bronchoscopic approach, and advanced therapies such as bronchial artery embolization and surgical resection. We review situations, such as bronchiectasis, vascular malformation, diffuse alveolar hemorrhage, and tracheostomy bleed and specific approach to management of these conditions in a systematic and evidence-based manner.

scholarly journals The daily physiotherapy regimen in cystic fibrosis?

1986 ◽  
Vol 42 (3) ◽  
pp. 73-76
Author(s):  
L. M. Davids
Keyword(s):  
Cystic Fibrosis ◽  
Personal Experience ◽  
Recent Literature ◽  
Psychological Impact ◽  
Fibrocystic Disease ◽  
Breathing Exercises ◽  
Postural Drainage ◽  
The Family ◽  
Acute Exacerbations ◽  
Life Threatening

The aim of this paper is to question the accepted physiotherapy regimen for children with Fibrocystic Disease. Is it the most effective way of keeping the lungs clear of secretions? How much are we imposing added stress on an already stressed family? Is it not possible to design a programme which reduces stress and is socially acceptable?The traditional physiotherapy regimen of breathing exercises, postural drainage and percussion is described. Forced expiratory technique is described as well as various forms of nebulisation.The psychological impact on the family of a chronic, life-threatening disease, is discussed. Compliance with set physiotherapy regimens and the family's (especially the mother's) reaction to them is discussed.The most recent literature on the effectiveness of traditional physiotherapy techniques is reviewed.From this review as well as from personal experience, it is concluded that a rigid physiotherapy regimen is stressful and compliance is frequently poor. Suggestions are made on how to overcome this. It is stressed that this paper deals with the home programme, not with the hospital programme for acute exacerbations of the disease.

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