A) Incorrect. CF is an autosomal recessive genetic disorder, meaning that both copies of the CFTR gene must carry a mutation for the condition to manifest.

B) Correct. CF is caused by the inheritance of two recessive genetic mutations, one from each parent, resulting in defective CFTR protein function.

C) Incorrect. CF is primarily a genetic disorder present from birth, not one caused by spontaneous mutations during a person's lifetime.

D) Incorrect. CF is primarily a genetic disorder with well-established genetic mutations associated with its development.

A) Incorrect. Frequent nosebleeds are not a hallmark symptom of CF.

B) Correct. A chronic cough with thick, sticky mucus production is a hallmark feature of CF due to the accumulation of mucus in the respiratory tract.

C) Incorrect. Rapid weight gain is not a typical symptom of CF; in fact, individuals with CF often struggle with poor weight gain and malnutrition.

D) Incorrect. High blood pressure is not a characteristic symptom of CF.

A) Incorrect. CF primarily affects the lower respiratory tract, particularly the bronchi and bronchioles, rather than the upper respiratory tract.

B) Correct. CF leads to thickened mucus in the airways, which can cause inflammation, recurrent infections, and progressive lung damage.

C) Incorrect. Enlarged tonsils and adenoids are not a typical manifestation of CF-related respiratory complications.

D) Incorrect. CF affects both the bronchial tubes and the entire respiratory system, often leading to chronic lung infections.

A) Incorrect. CF primarily affects the pancreas and small intestine, not the stomach.

B) Incorrect. While liver complications can occur in some individuals with CF, such as liver disease or cirrhosis, it is not a primary manifestation of the disease.

C) Correct. CF often results in thickened mucus obstructing the pancreatic ducts, causing pancreatic insufficiency and malabsorption of essential nutrients.

D) Incorrect. CF primarily affects the pancreas and small intestine rather than the large intestine, and chronic constipation is not a typical symptom of CF.

A) Incorrect. Both partners having a family history of CF increases the likelihood of carrying CF gene mutations, making it possible for their child to inherit the condition.

B) Incorrect. Having a single CF gene mutation (carrier status) does not guarantee that a child will develop CF; it requires inheriting two CF gene mutations, one from each parent.

C) Correct. CF is an autosomal recessive genetic disorder, which means that a child will have CF only if they inherit two copies of the CF gene mutation, one from each parent.

D) Incorrect. The risk of a child having CF is determined by both parents' genetic history, not solely the mother's.

A) Incorrect. CF is an autosomal recessive genetic disorder, meaning that both copies of the CFTR gene must carry a mutation for the condition to manifest.

B) Incorrect. CF is not caused by dominant genetic mutations.

C) Correct. CF results from inheriting two recessive genetic mutations, one from each parent, leading to defective CFTR protein function.

D) Incorrect. CF is primarily a genetic disorder with well-established genetic mutations associated with its development.

A) Incorrect. The CFTR protein primarily functions in the respiratory and digestive systems, not the kidneys.

B) Incorrect. While CFTR plays a role in chloride secretion, its primary role is related to mucus production and chloride ion movement in the respiratory and digestive systems.

C) Incorrect. The CFTR protein is not involved in the production of red blood cells.

D) Correct. The CFTR protein is responsible for regulating mucus consistency and chloride ion transport in the respiratory and digestive systems.

A) Incorrect. CF is not caused by an extra chromosome; it is a genetic disorder resulting from mutations in specific genes.

B) Incorrect. CF is not characterized by a duplication of the CFTR gene but rather by various mutations in this gene.

C) Correct. CF is primarily caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, leading to the production of dysfunctional chloride channels.

D) Incorrect. CF is not characterized by the absence of the CFTR gene; instead, it involves various mutations in this gene.

A) Incorrect. The CFTR protein does not regulate blood sugar levels.

B) Incorrect. CFTR does not play a role in oxygen transport in the bloodstream.

C) Correct. The primary function of the CFTR protein is to regulate chloride ion and water movement across cell membranes, which is disrupted in individuals with CF.

D) Incorrect. The production of digestive enzymes in the pancreas is not a direct function of the CFTR protein.

A) Incorrect. While inflammation can be a consequence of CF, it is not the primary cause of airway constriction.

B) Correct. In CF, thickened mucus accumulates in the airways, leading to chronic inflammation, recurrent infections, and progressive lung damage.

C) Incorrect. CF does not result in the overproduction of surfactant, which is a substance that helps reduce surface tension in the lungs.

D) Incorrect. The CFTR protein does not produce oxygen but rather regulates chloride ion and water transport in the lungs

A) Correct. CF is primarily caused by mutations in a single gene, the CFTR gene, and it can be inherited from one parent, resulting in a carrier state, or from both parents for the disease to manifest.

B) Incorrect. While genetic factors are the primary cause of CF, environmental factors can exacerbate symptoms but are not the root cause.

C) Incorrect. CF is not an X-linked genetic disorder; it follows an autosomal recessive pattern of inheritance.

D) Incorrect. CF is not caused by exposure to respiratory pathogens during infancy; it is primarily a genetic disorder.

A) Incorrect. CF occurs at a similar frequency in both males and females.

B) Incorrect. CF is not significantly associated with any specific ethnicity, and the risk is not influenced by being of African descent.

C) Correct. A family history of CF increases the risk because CF is an autosomal recessive genetic disorder, and having affected family members increases the likelihood of carrying CF gene mutations.

D) Incorrect. While CF can affect individuals from any ethnicity, it is primarily a genetic disorder, and family history plays a crucial role in the risk of inheritance.

A) Incorrect. If both parents are carriers, their child's risk of having CF is 25%, not 50%.

B) Incorrect. While the risk is increased, having two carrier parents does not guarantee that a child will have CF.

C) Incorrect. If both parents are carriers, their child can inherit two normal genes (no CF), one normal and one CF gene (carrier status), or two CF genes (CF).

D) Correct. When both parents are carriers, there is a 25% chance that their child will inherit two CF genes and have CF, a 50% chance of being a carrier, and a 25% chance of inheriting two normal genes (no CF).

A) Incorrect. Genetic testing for CF is important for individuals of all ages, not limited to infants.

B) Correct. Genetic testing is a crucial diagnostic tool for confirming the presence of CF gene mutations, regardless of the individual's age.

C) Incorrect. Genetic testing is primarily used for diagnosis, not for determining the severity of CF symptoms.

D) Incorrect. Genetic testing is not reserved solely for family members of individuals already diagnosed with CF; it is used for diagnostic purposes in suspected cases of CF.

A) Incorrect. Wheezing and chest pain can occur in CF, but a chronic cough with thick mucus production is often one of the earliest respiratory symptoms.

B) Correct. A chronic cough with thick, sticky mucus is a hallmark symptom of CF due to the accumulation of mucus in the respiratory tract.

C) Incorrect. Blood in the urine is not a common early sign of CF; it may indicate other issues.

D) Incorrect. Digestive discomfort and diarrhea are more related to CF's impact on the gastrointestinal system, rather than being early respiratory symptoms.

A) Incorrect. Frequent nosebleeds are not common gastrointestinal symptoms in CF.

B) Correct. Abdominal pain and bloating are common gastrointestinal symptoms in CF, often due to the impact of thickened mucus on the pancreas and intestines.

C) Incorrect. Rapid weight gain is not a typical gastrointestinal symptom in CF; individuals with CF often struggle with poor weight gain and malnutrition.

D) Incorrect. Muscle weakness is not a common gastrointestinal symptom in CF but may be associated with other complications.

A) Incorrect. Clubbed fingers in CF can be more than a cosmetic concern and may indicate underlying medical issues.

B) Correct. Clubbing of the fingers can be associated with advanced lung disease or reduced oxygen levels in CF.

C) Incorrect. Clubbing is not related to excessive sodium intake in CF patients.

D) Incorrect. While CF can affect the digestive system, clubbing of the fingers is primarily associated with respiratory issues.

A) Incorrect. In advanced CF-related lung disease, a chronic, productive cough with thick mucus production is more common than a dry, nonproductive cough.

B) Correct. Decreased breath sounds may occur in clients with advanced CF-related lung disease due to airway obstruction and lung damage.

C) Incorrect. Hematuria is not a typical respiratory symptom of CF.

D) Incorrect. Rapid weight gain is not associated with advanced CF-related lung disease; individuals with advanced CF often experience weight loss and malnutrition.

A) Incorrect. Meconium ileus is a complication typically seen in newborns with CF, not in adults.

B) Incorrect. Failure to thrive is a concern in infants and young children with CF but not typically in adults.

C) Correct. Osteoporosis is a common complication in adults with CF due to malabsorption of calcium and vitamin D, chronic inflammation, and reduced physical activity.

D) Incorrect. Rapid growth and development are typically observed in adolescents, not as a complication but as a part of normal growth.

A) Correct. The sweat chloride test is commonly used for newborn screening for CF. Elevated sweat chloride levels are indicative of CF.

B) Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF but are not a primary diagnostic test, especially in newborns.

C) Incorrect. Pulmonary function tests are used to evaluate lung function but are not typically performed as part of newborn screening for CF.

D) Incorrect. A complete blood count (CBC) is a general blood test and is not specific for CF diagnosis.

A) Incorrect. Genetic testing can confirm CF but is not typically the initial diagnostic test used in infants with symptoms suggestive of CF.

B) Correct. The sweat chloride test is often used as the initial diagnostic test to assess for CF in infants who exhibit symptoms.

C) Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF but are not typically the initial diagnostic test in infants.

D) Incorrect. Pulmonary function tests are used to evaluate lung function but are not typically the initial diagnostic test for CF in infants.

A) Incorrect. Genetic testing is used to confirm the presence of CF gene mutations but is not a measure of lung function.

B) Incorrect. The sweat chloride test is used for CF diagnosis and may not be performed regularly to monitor lung function.

C) Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF, but they do not directly measure lung function.

D) Correct. Pulmonary function tests are commonly performed to assess lung function, monitor disease progression, and guide treatment in individuals with CF.

A) Incorrect. The sweat chloride test is used for CF diagnosis, but it does not analyze genetic mutations.

B) Incorrect. Chest X-rays are used to assess lung changes but do not directly confirm CF by analyzing genetic mutations.

C) Incorrect. Pulmonary function tests assess lung function but do not confirm CF by analyzing genetic mutations.

D) Correct. Genetic testing is a diagnostic procedure commonly used to confirm CF by analyzing the specific CF gene mutations present in an individual.

A) Incorrect. Genetic testing is used to confirm CF but does not directly measure chloride ion concentration in sweat.

B) Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF but are not used to measure chloride ion concentration in sweat.

C) Incorrect. Pulmonary function tests assess lung function but do not directly measure chloride ion concentration in sweat.

D) Correct. The sweat chloride test measures the concentration of chloride ions in sweat and is commonly used to confirm CF. Elevated sweat chloride levels are indicative of CF.

A) Incorrect. Postural drainage involves positioning the body to help drain mucus but does not use a mechanical device.

B) Incorrect. Autogenic drainage is a technique that involves controlled breathing and airflow, not a mechanical device.

C) Incorrect. PEP therapy uses a device to provide positive pressure during exhalation but is different from HFCWO.

D) Correct. High-frequency chest wall oscillation (HFCWO) involves using a mechanical device to deliver oscillations to the chest wall, helping to mobilize and clear mucus from the airways.

A) Incorrect. While monitoring carbohydrate intake is important in CF, limiting carbohydrates is not the primary focus of nutrition management.

B) Incorrect. Increasing sodium intake is not a general recommendation for individuals with CF; it depends on the individual's needs.

C) Correct. Individuals with CF often require a high-calorie diet rich in fats and proteins to address malabsorption and maintain adequate nutrition.

D) Incorrect. Restricting fluid intake is not recommended for individuals with CF, as staying hydrated is important for preventing thickening of mucus.

A) Incorrect. Long-term oxygen therapy is typically reserved for individuals with chronic hypoxemia and is not the primary treatment for a pulmonary exacerbation.

B) Correct. Bronchodilators and corticosteroids are commonly used to manage pulmonary exacerbations in CF by reducing airway inflammation and improving airflow.

C) Incorrect. Surgical lung transplantation is considered in severe cases of CF with irreversible lung damage but is not typically the first-line treatment for a pulmonary exacerbation.

D) Incorrect. Bed rest and minimal physical activity are not recommended as the primary treatment for pulmonary exacerbations; maintaining mobility and airway clearance techniques are important.

A) Incorrect. While airway clearance techniques can help maintain lung function, their primary goal is not to improve lung capacity.

B) Incorrect. Preventing respiratory infections is an important aspect of CF care, but it is not the primary goal of airway clearance techniques.

C) Incorrect. Maintaining electrolyte balance is important in CF, but it is not the primary goal of airway clearance techniques.

D) Correct. The primary goal of airway clearance techniques in CF is to mobilize and clear mucus and secretions from the airways, improving breathing and reducing the risk of infection.

A) Incorrect. PERT is not primarily used to control blood sugar levels; it is used to improve nutrient absorption.

B) Incorrect. While CF management aims to improve lung function, PERT is specifically used to address pancreatic insufficiency and digestive issues.

C) Correct. The primary purpose of PERT in CF management is to replace the missing digestive enzymes and aid in the digestion of fats and nutrients.

D) Incorrect. PERT does not increase mucus production in the airways; it is focused on the digestive system.

A) Incorrect. Protein intake is important for growth and repair but should not be limited in CF. High-calorie intake is more critical.

B) Correct. CF can lead to malabsorption of fat-soluble vitamins and calories, so a high-fat diet is recommended to support weight gain and nutrient absorption.

C) Incorrect. Dairy products are a good source of calcium and calories, and they are generally well-tolerated by CF patients. Avoiding them is not necessary.

D) Incorrect. Increasing sugary snacks is not advisable for CF patients. They need a balanced diet to meet their nutritional needs.

A) Correct. Postural drainage and chest percussion help mobilize and remove thick, sticky mucus from the airways, which is crucial in managing CF.

B) Incorrect. While bronchodilators may be used, they are not the primary therapy for managing CF. Airway clearance techniques like postural drainage are more important.

C) Incorrect. Oxygen therapy should be administered as needed, but it is not required around the clock for all CF patients.

D) Incorrect. Deep breathing exercises should be encouraged regularly, not just during illness, to maintain airway clearance and lung function.

A) Incorrect. Hypertension is not typically associated with CF.

B) Incorrect. Hypoglycemia is not a common manifestation of CF.

C) Incorrect. Chronic diarrhea is not a common symptom of CF.

D) Correct. CF is characterized by thick, sticky mucus that can lead to chronic respiratory infections, making this a key clinical manifestation to monitor.

A) Incorrect. While coughing is important, huff coughing is a more effective and less strenuous airway clearance technique for CF patients.

B) Incorrect. Controlled breathing exercises may help with relaxation but are not the primary airway clearance technique.

C) Correct. Huff coughing is a technique where the client takes a deep breath and forcefully exhales in short bursts, helping to move mucus from the airways.

D) Incorrect. Vigorous exercise is beneficial for CF patients but should not replace specific airway clearance techniques.

A) Correct. Staying well-hydrated is important for CF patients to help keep mucus thin and easier to clear.

B) Correct. It's important to administer all prescribed medications as directed to manage the condition effectively.

C) Incorrect. While infection control is important, it is not necessary for the child to avoid close contact with other children. Common-sense hygiene measures should be followed.

D) Correct. Regular follow-up appointments with the CF care team are crucial to monitor the child's health and adjust the treatment plan as needed.