Symptoma

Bronchiectasis with or without Elevated Sweat Chloride 3

Idiopathic Bronchiectasis

A rationalisation of the 1350 boxes used throughout the book gives a simpler and clearer presentation of the various categories. [books.google.com]

A 66-year-old man with bronchiectasis presented with shortness of breath to the emergency department of our institution, a district hospital in the UK. His medications included long-term supplemental oxygen therapy and prophylactic azithromycin. [ncbi.nlm.nih.gov]

Acronym BESC3 Synonyms Cystic fibrosis-like syndrome Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. [uniprot.org]

  • Chronic Infection

    Subsequently, chronic infection with Pseudomonas aeruginosa becomes established in most patients, although the prevalence of chronic infection is being reduced by attention to prevention of cross-infection in hospitals, and aggressive eradication regimes [erswhitebook.org]

    His sputum is chronically infected with pseudomonas aeruginosa, Alcaligenes xylosoxidans (highly resistant with variable antibiotic susceptibilities), and Methicillin-resistant Staphylococcus aureus and his pulmonary function test [fig 3] has remarkably [article.sapub.org]

    This interaction between chronic infection and excessive inflammation, which is predominantly neutrophilic, ensures ongoing damage to the airways and the development and maintenance of the features seen in bronchiectasis. [clinicalgate.com]

    CF exacerbations in children 6 years old Dornase alfa – clears respiratory secretions; may reduce CF exacerbations Ibuprofen – reduces inflammatory airway response; may slow progression of disease Antibiotics (tobramycin, azithromycin) – treatment of chronic [pedclerk.bsd.uchicago.edu]

    Pulmozyme (Dornase alfa) a mucus thinning drug shown to reduce the number of lung infections and improve lung function 5 Azithromycin, an antibiotic recently proven to be effective in CF whose lungs are chronically infected with pseudomonas aeruginosa [quizlet.com]

  • Physician

    New ‘In Adolescence’ boxes recognise the fact that many chronic disorders begin in childhood and become the responsibility of physicians practising adult medicine. [books.google.com]

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    Atypical Cystic Fibrosis and the Primary Care Physician The increasing number of atypical CF, which represents approximately 2% of affected patients [2], will make clinical diagnosis more challenging. [article.sapub.org]

  • Sputum

    Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. [uniprot.org]

    A breathing treatment with salt water may help to cough up a sputum sample. This is called a sputum induction. Sometimes a video scope may be used to look into the airways and collect a sputum sample. [bronchiectasisandntminitiative.org]

    They had chronic purulent sputum production, in contrast to patients with chronic bronchitis in whom the sputum is usually not purulent except during exacerbations ( 15 ). [jtd.amegroups.com]

    Daily expectoration of large volumes of very purulent sputum. Haemoptysis. Sputum colonisation with P. aeruginosa. Unexplained haemoptysis or nonproductive cough. [patient.info]

  • Cough

    The disease is characterized by hypersecretion of mucus accompanied by a chronic (more than 3 months in 2 consecutive years) productive cough. Infectious agents are a major cause of chronic bronchitis. [ncbi.nlm.nih.gov]

    Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. [uniprot.org]

    He was treated with a course of doxycycline with near compete resolution of his cough. [jtd.amegroups.com]

  • Pneumonia

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    It may be caused by pneumonia, bronchitis, tuberculosis, or other severe lung infection, smoking ... ... More on Bronchiectasis » Chronic Bronchitis : Chronic Bronchitis is a chronic, severe lung disease that is part of COPD. [familydiagnosis.com]

    During the preceding year, he had five pneumonias, each with acute symptoms and radiographic evidence of pneumonia. [jtd.amegroups.com]

    The majority of cases of bronchiectasis follow severe pneumonia or other lower respiratory infection (10). [hawaii.edu]

  • Productive Cough

    The disease is characterized by hypersecretion of mucus accompanied by a chronic (more than 3 months in 2 consecutive years) productive cough. Infectious agents are a major cause of chronic bronchitis. [ncbi.nlm.nih.gov]

    Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. [uniprot.org]

    Seven years previously, he was seen by a Pulmonologist at Detroit medical center, Michigan for recurrent productive cough, which was associated with weight loss and shortness of breath. [article.sapub.org]

    This should then be used on a daily basis for those with chronic productive cough, and on an intermittent basis for those with productive cough during exacerbations. [patient.info]

  • Sputum Production

    Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. [uniprot.org]

    His recurrent pneumonias were prevented and his productive cough decreased, going from 6 to 7 teaspoons of daily sputum production to just 2 teaspoons. [jtd.amegroups.com]

    production, malaise, anorexia, weight loss, clubbing Colonization of airways with pathogenic bacteria Staphylococcus aureus Haemophilus influenzae Pseudomonas aeruginosa Sinus disease Opacification of paranasal sinuses on radiographs Nasal polyposis, [pedclerk.bsd.uchicago.edu]

    Children Respiratory symptoms, especially cough and sputum production, should be assessed and recorded in all children with possible bronchiectasis. [patient.info]

  • Failure to Thrive

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    […] to thrive Fat soluble vitamin deficiency(ADEK) 22. [slideshare.net]

    […] sinuses on radiographs Nasal polyposis, chronic rhinosinusitis Pancreatic disease Exocrine pancreatic insufficiency – usually present from birth Insufficient lipase causing steatorrhea and protein malabsorption Infants & children commonly present with failure [pedclerk.bsd.uchicago.edu]

    He had history of pneumonia 2 years back with failure to thrive since 15 years. The patient remained on his regular medications which included albuterol, multivitamins and pancreatic enzymes. [tmg.org.rs]

  • Sweating

    MalaCards based summary : Bronchiectasis with or Without Elevated Sweat Chloride 3, also known as cystic fibrosis-like syndrome, is related to bronchiectasis with or without elevated sweat chloride 1 and bronchiectasis with or without elevated sweat chloride [malacards.org]

    More on Chronic Bronchitis » Elevated sweat chloride : Summary An increased concentration of chloride in the sweat. 1, 2 ... ... [familydiagnosis.com]

    Test Cost : 3000.00 AED Reporting Time: 4 Week Sample Type: EDTA blood or DNA Bronchiectasis with or without elevated sweat chloride type 2 (SCNN1A) Test Description : Bronchiectasis with or without elevated sweat chloride type 2 (SCNN1A) genetic dna [dnalabsuae.com]

    Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. [uniprot.org]

    Sweat chloride concentrations of 30-60 mmol/l are seen in about 4% of sweat tests and 23% of these will subsequently be found to have two CF causing mutations. [cfmedicine.com]

  • Suggestibility

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    Also, some evidence suggests the CFTR mutations are associated with non-CF bronchiectasis and rheumatoid arthritis. [clinicalgate.com]

    Our case series suggests that this phenotype is also seen in adults, although it is less well characterized. [jtd.amegroups.com]

    Recent evidence suggests that a proportion of patients with “classical CF” have chloride concentrations of 30-60 mmol/l. [cfmedicine.com]

  • Stroke

    A new chapter specifically on Stroke Disease recognises the emergence of Stroke Medicine as a distinct clinical and academic discipline. [books.google.com]

An extensive workup was negative including liver function tests, viral hepatitis panel, Alpha 1 antitrypsin (stool, blood), abdominal ultrasound and upper GI endoscopy. [tmg.org.rs]

Authors examine how to evaluate the success of therapy for bronchiectasis (what endpoints to use) and then discuss a variety of therapy options: inhaled and systemic antibiotic treatment, chest physiotherapy, pharmacologic agents for mucous clearance, [books.google.com]

It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice . [uniprot.org]

Despite prior optimal treatment according to recommended practice guidelines [7], her lung function declined significantly in the two months prior to treatment. [elynsgroup.com]

Newer treatments are likely to be even more expensive. The emerging issue of the costs of genotype-specific treatments will be discussed in detail later. [erswhitebook.org]

Complications The complications of bronchiectasis include: Recurrent infection Life-threatening haemoptysis Lung abscess Pneumothorax Poor growth and development Prognosis The long-term prognosis is entirely dependent on the underlying cause. [teachmepaediatrics.com]

Oral supplements, enteral feeds w/ gastrostomy to increase caloric intake Routine vaccinations, including annual influenza vaccine Regular monitoring: pulmonary function tests, CXRs, bone density, glucose, and respiratory cultures for P. aeruginosa Prognosis [pedclerk.bsd.uchicago.edu]

Other important respiratory complications include allergic bronchopulmonary aspergillosis, pneumothorax (which carries a bad prognosis because of associated severe lung disease), massive haemoptysis, and lung or lobar collapse. [erswhitebook.org]

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This case report also shows that despite the longstanding nature of the disease the sequelae of CF can be ameliorated, including the improvement in FEV1 and weight which will improve prognosis in CF [8,9]. [elynsgroup.com]

Pathogenesis, etiology and treatment of bronchiectasis. [thoracicmedicine.org]

A ciliary defect is thought to be the etiology but has not been consistently demonstrated (15). [hawaii.edu]

Am J Respir Cell Mol Biol. 2009; 41(5): 620. 4 Bronchiectasis in a diverse US population: effects of ethnicity on etiology and sputum culture. McShane PJ, Naureckas ET, and Strek ME. [floridamd.com]

Although BE is commonly due to cystic fibrosis (CF) in developed countries, non-CF etiologies are also common in developing countries [ 1, 2, 3 ]. [karger.com]

However, the etiology for the cystic fibrosis was uncertain. He was well until age 16, when adynamia, chronic cough, and dyspnea during exercise ensued. His pulmonary symptoms worsened, and the cough became productive. [tmg.org.rs]

Epidemiology, airway defense mechanisms, pathogenesis, imaging and genetic causes of bronchiectasis are discussed. Allergic bronchopulmonary aspergillosis is addressed, as well as non-tuberculous mycobacteria as a cause of bronchiectasis. [books.google.com]

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Epidemiology In the UK, the estimated prevalence of non-CF bronchiectasis is 172/million children aged Pathophysiology There are several aetiologies of non-CF bronchiectasis, which are categorised below. [teachmepaediatrics.com]

Konstan MW, Morgan WJ, Butler SM, Pasta DJ, Craib ML, Silva SJ, Stokes DC, Wohl ME, Wagener JS, Regelmann WE, Johnson CA; Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. [tmg.org.rs]

Epidemiologic study of cystic fibrosis: design and implementation of a prospective, multicentre, observational study of patients with cystic fibrosis in the U.S. and Canada. Pediatr Pulmonol 1999; 28: 231-241. [cfmedicine.com]

In the absence of CFTR function, this downregulation is relieved, and sodium reabsorption increases, contributing to reduction in fluid volume and probably to the airway pathophysiology. [pedsinreview.aappublications.org]

In this article, we will discuss the pathophysiology, clinical features, management and complications of bronchietasis. [teachmepaediatrics.com]

Pathophysiology and management of pulmonary infections in cystic fibrosis. [article.sapub.org]

Bronchiectasis is a chronic lung disease whose pathophysiology is poorly understood. It has traditionally been considered "permanent and irreversible" (1). [hawaii.edu]

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Goals of CF include: a) preventing and controlling lung infections b) loosening and removing thick, sticky mucus c) preventing or treating blockages in the intestines d) providing enough nutrition e) preventing dehydration Treatment of CF -depends on [quizlet.com]

Antibiotics are used for the treatment of acute exacerbations and prevention of exacerbations. Sputum cultures should be obtained prior to antibiotic therapy, and will help with antibiotic choices. [floridamd.com]

His recurrent pneumonias were prevented and his productive cough decreased, going from 6 to 7 teaspoons of daily sputum production to just 2 teaspoons. [jtd.amegroups.com]