The pathogenetic mechanisms of cough in idiopathic pulmonary fibrosis

AbstractIdiopathic pulmonary fibrosis is a peripheral subpleural interstitial lung disorder limited to the lung not involving the airways. It has a poor prognosis (survival less than 5  years) and commonly an interstitial pneumonia radiological pattern. Patients complain of a chronic dry cough in 80% of cases. A cough is often the first symptom of this rare disease, preceding dyspnea by years, and is associated with a poor prognosis, high dyspnea scores and low FVC percentages. T he pathogenetic mechanisms leading to coughing in IPF are unclear. This review focuses on recent evidence of cough pathophysiology in this disease. Gastroesophageal reflux may promote coughing in IPF patients; bile salts and pepsin may be abundant in BAL of these patients, inducing overproduction of TGF-β by airway epithelial cells and mesenchymal transition with fibroblast recruitment/activation and extracellular matrix deposition. Patients have an enhanced cough reflex to capsaicin and substance P with respect to control subjects. Moreover, patients with the MUC5B polymorphism show more sev ere coughing as MUC5B encodes for the dominant mucin in the honeycomb cysts of IPF patients. Comorbidities, including asthma, gastroesophageal reflux, hypersensitivity pneumonitis, bronchiectasis, chronic obstructive pulmonary disease and emphysema, can induce coughing in IPF patients. There is no c lear explanation of the causes of coughing in IPF. Further research into the pathophysiology of IPF and ...
Source: Internal and Emergency Medicine - Category: Emergency Medicine Source Type: research

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We describe this case of a young gentleman presenting with acute dyspnoea on a background history of known, long-standing asthma. His dramatic presentation, notable for profound hypoxia and cyanosis, led to an unexpected additional diagnosis of type one congenital methaemoglobinaemia.Case presentationA 26-year-old Irish gentleman was transferred urgently to the emergency department resuscitation room with marked cyanosis and tachypnoea. His oxygen saturation was 70% on 100% high flow oxygen. His arterial blood gas (On Fi02 90%) demonstrated a PaO2 = 76.8 kPa, SpO2 = 99%, pCO2 = 3 kPa and pH = 7.51. A satura...
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research
Conclusion: Although left-sided gallbladder is a very rare disease, it is possible to diagnose it preoperatively and perform laparoscopic cholecystectomy safely by adjusting port position. The common important features of left-sided gallbladder include distribution of the left portal vein crossing over to the right side of the liver and increased size of the left portal vein. These variations may have important clinical implications in the management of hepatic resection including donor hepatectomy. PMID: 31824885 [PubMed]
Source: Annals of Surgical Treatment and Research - Category: Surgery Tags: Ann Surg Treat Res Source Type: research
Source: Journal of Asthma and Allergy - Category: Allergy & Immunology Tags: Journal of Asthma and Allergy Source Type: research
The FDA unexpectedly reversed course Thursday, approving the second treatment developed by Sarepta Therapeutics for the rare disease Duchenne muscular dystrophy just a few months after rejecting the drug.   Sarepta (Nasdaq: SRPT) announced Thursday evening that the agency had approved its second drug, called Vyondys 53. After the drug was originally rejected by the FDA in August due to concerns over the risk of infection at the infusion site and renal toxicity, the company filed an appeal. The comp any…
Source: Health Care:Pharmaceuticals headlines - Category: Pharmaceuticals Authors: Source Type: news
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Source: Reuters: Health - Category: Consumer Health News Tags: healthNews Source Type: news
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Source: Frontiers in Neurology - Category: Neurology Source Type: research
Quantitative mapping of the magnetic susceptibility and the effective transverse relaxation rate (R2*) are suitable to assess the iron content in distinct brain regions. In this prospective, explorative study the iron accumulation in deep gray matter nuclei (DGM) in myotonic dystrophy type 1 (DM1) and 2 (DM2) and its clinical and neuro-cognitive relevance using susceptibility and R2* mapping was examined. Twelve classical DM1, four childhood-onset DM1 (DM1c.o.), twelve DM2 patients and twenty-nine matched healthy controls underwent MRI at 3 Tesla, neurological and neuro-cognitive tests. Susceptibility, R2* and volumes were...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
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Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
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Source: Medscape Neurology and Neurosurgery Headlines - Category: Neurology Tags: Neurology & Neurosurgery News Alert Source Type: news
Today, the @US_FDA authorized the marketing of the first test to aid in newborn screening for Duchenne Muscular Dystrophy (DMD), a rare genetic disorder that causes progressive muscle deterioration and weakness. Find out more about the new approval
Source: Food and Drug Adminstration (FDA): CDRHNew - Category: Medical Devices Authors: Source Type: news
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