Cystic Fibrosis: Adult Symptoms and Management

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Questions and Answers

What is a common symptom of cystic fibrosis in adults?

Liver disease (correct)

Hyperthyroidism

Increased appetite

Nasopharyngitis

What symptom is associated with lung disease in cystic fibrosis?

Severe headaches

Rapid weight loss

Shortness of breath on exertion (correct)

Persistent fever

Which complication is related to the gastrointestinal system in cystic fibrosis?

Osteoporosis

Chronic gastritis

Diverticulitis

Pancreatic obstruction (correct)

Which assessment is important when evaluating lung symptoms in a cystic fibrosis patient?

Volume and purulence of sputum Signup and view all the answers

Which of the following is NOT a symptom of CF-related lung disease?

Severe abdominal pain Signup and view all the answers

What condition may result from distal intestinal obstruction syndrome in cystic fibrosis?

Dehydration Signup and view all the answers

Which of the following findings in a cystic fibrosis patient indicates a complication related to respiratory disease?

Mucus plugging Signup and view all the answers

Which question should be included in a patient's history to evaluate for cystic fibrosis symptoms?

Do you have a chronic cough? Signup and view all the answers

What is the respiratory rate at the time of ICU admission?

48 breaths/minute Signup and view all the answers

Which of the following values indicates an elevated level of C-reactive protein?

372 mg/l Signup and view all the answers

What does the PaO2:FIO2 ratio of 96 mmHg suggest about the patient's respiratory status?

Severe hypoxemia Signup and view all the answers

What is the hemoglobin level recorded at ICU admission?

9.8 g/dl Signup and view all the answers

How much is the lactate level of the patient at the time of admission?

1.2 mmol/l Signup and view all the answers

Which laboratory result is significantly elevated in this case?

D-Dimer at 3.2 Signup and view all the answers

What is the arterial blood gas pH level at the time of admission?

7.45 Signup and view all the answers

What condition does the elevated ferritin level (1646 ng/ml) indicate?

Acute inflammatory response Signup and view all the answers

Which of the following is a nebulized therapy specifically used for allergic bronchopulmonary aspergillosis?

Dornase Alpha Signup and view all the answers

What is the primary use of pancreatic enzyme tablets like 'Creon' in cystic fibrosis management?

To promote digestive health Signup and view all the answers

Which of these is a psychosocial benefit of a multidisciplinary team approach in cystic fibrosis treatment?

Increased patient education Signup and view all the answers

Which of the following is a common respiratory pathogen identified in cystic fibrosis patients?

Stenotrophomonas Maltophilia Signup and view all the answers

What is the role of oral prednisolone in the treatment of allergic bronchopulmonary aspergillosis?

It reduces inflammation Signup and view all the answers

What does FEV1 measure in cystic fibrosis monitoring?

Airway obstruction Signup and view all the answers

Which supplementation is important for cystic fibrosis patients to prevent malabsorption of fat-soluble vitamins?

Aquadek Signup and view all the answers

What is a primary goal of stringent infection control policy in cystic fibrosis care?

To minimize lung infections Signup and view all the answers

What FEV1 level is generally considered abnormal in cystic fibrosis patients?

40-60 mEq/L Signup and view all the answers

Which of the following is NOT a common respiratory microbiological finding in cystic fibrosis?

Escherichia coli Signup and view all the answers

What is the primary purpose of gene testing in cystic fibrosis?

To know specific mutations Signup and view all the answers

Which of the following symptoms is associated with the diagnosis of acute exacerbations in cystic fibrosis?

Increased dyspnea Signup and view all the answers

Which treatment is NOT included in the maintenance treatment for cystic fibrosis?

Intravenous antibiotics Signup and view all the answers

Which pulmonary condition is characterized by decreased mucociliary clearance in cystic fibrosis?

Obstructive airways disease Signup and view all the answers

What is the recommended duration for intravenous antibiotics during acute treatment of cystic fibrosis?

2 weeks Signup and view all the answers

What mechanism is primarily affected in cystic fibrosis resulting in recurrent pulmonary infections?

Decreased mucociliary clearance Signup and view all the answers

What is the highest recorded activity level for PR3?

0.3407 nM Signup and view all the answers

Which component has the lowest activity level reported?

PR3 Signup and view all the answers

What is the activity level of neutrophils measured at 80 (106/ml)?

0.6154 nM Signup and view all the answers

At a neutrophil count of 6000 (106/ml), what is the reported activity level for Cat G?

0.9771 nM Signup and view all the answers

Which measured activity level corresponds to 0.0180 nM?

PR3 Signup and view all the answers

Which activity level is not associated with the category 'Neutrophils' despite its proximity in readings?

0.8445 nM Signup and view all the answers

If the Cat G activity level at a neutrophil count of 4000 (106/ml) is reported, what is its value?

0.0086 nM Signup and view all the answers

What is the activity level for neutrophils at a count of 20 (106/ml)?

0 nM Signup and view all the answers

At a measurement of 10000, what is the Cat G activity level?

0.0202 nM Signup and view all the answers

What is the activity level for post Trika1a cytokines recorded in the data?

0.0141 nM Signup and view all the answers

Study Notes

Cystic Fibrosis Overview

Cystic fibrosis (CF) is a genetic disorder affecting multiple organ systems, primarily the lungs and digestive system.
Key symptoms in adults include respiratory issues, pancreatic insufficiency, and liver disease.

Childhood Presentation

Birth: Symptoms like failure to thrive and meconium ileus may indicate CF.
Newborn screening has been in place since 2012, allowing for early detection.

Adult Clinical Manifestations

Respiratory complications include pan-bronchiectasis and obstructive airway disease.
Gastrointestinal symptoms comprise distal intestinal obstruction, pancreatic obstruction, and malabsorption issues.
Associated conditions include CF-related diabetes and osteoporosis.

Respiratory Disease Characteristics

Decreased mucociliary clearance leads to recurrent pulmonary infections and respiratory failure.
Symptoms include chronic cough with increased sputum volume and purulence.

Lung Disease Symptoms Assessment

Key questions assess the nature of cough, sputum production, and breathlessness during activity.
Examination may reveal signs of respiratory distress, like wheezing or chest pain.

Treatment Options

Nebulized antibiotics like Tobramycin and Colistin are used to manage infections.
Inhaled therapies include bronchodilators and dornase alfa to improve mucus clearance.
Oral treatments consist of anti-inflammatories like azithromycin and pancreatic enzyme supplements.

Management for Malabsorption and Diabetes

Pancreatic enzyme replacement therapy and vitamin supplements (A, D, E, K) are vital.
Insulin therapy is required for managing CF-related diabetes.
Liver disease may be treated with Ursofalk.

Outpatient Monitoring

Regular assessments include lung function tests (FEV1), body mass index (BMI), and sputum cultures.
Annual evaluations cover OGTT (oral glucose tolerance test), liver ultrasound, and nutritional status.

Respiratory Microbiology

Frequent pathogens include Pseudomonas aeruginosa, Staphylococcus aureus, and Burkholderia cepacia.
Management should focus on targeted antimicrobial therapy based on susceptibility profiles.

Improving Patient Outcomes

A multidisciplinary team approach enhances care coordination and adherence to treatment plans.
Emphasis on strict infection control measures and ongoing education for patients and families.

Prognostic Insights

FEV1 levels are correlated with morbidity and mortality, but should be interpreted in conjunction with clinical presentation.
Genetic testing helps determine specific mutations, guiding tailored therapies.

Clinical Features in Advanced Cases

Signs such as cachexia, clubbing, and respiratory failure indicate disease progression.
Examination findings like abnormal lung sounds and elevated liver function tests can direct treatment.

Acute Exacerbation Diagnosis and Treatment

Increased cough, sputum production, dyspnea, fatigue, and appetite changes signal exacerbations.
Treatment includes intravenous antibiotics, intensive physiotherapy, and nutritional support.

Recombinant DNase Therapy

Recombinant dornase alfa is used to reduce mucus viscosity, improving lung function and overall health in CF patients.

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Description

This quiz focuses on recognizing the symptoms of cystic fibrosis in adults and the evaluation of therapeutic and surgical options for those with advanced conditions. It also covers early presentations of cystic fibrosis during childhood and necessary screenings. Test your knowledge on this critical health topic.