Regarding the genetics of CF:

Correct Answer: B

Rationale: the affected protein in cystic fibrosis (CF) is the cystic fibrosis transmembrane conductance regulator (CFTR), which is a chloride channel, not a sodium channel. The other options are incorrect: (a) The commonest mutation in Caucasians is ΔF508, not ~F-580. (b) Parents of an affected child are typically carriers and do not show symptoms. (c) Prenatal diagnosis is not possible in all cases due to the vast number of mutations. (d) There are over 2000 known mutations causing CF.

Correct Answer: C

Rationale: In the management of thalassaemia, the correct option is C) Bone marrow transplant. Thalassaemia is a genetic blood disorder characterized by abnormal hemoglobin production. A bone marrow transplant offers a potential cure by replacing the faulty bone marrow with healthy stem cells that can produce normal hemoglobin. A) Whole blood transfusions are used to provide symptomatic relief by replenishing red blood cells, but they do not cure thalassaemia. B) Pneumovax is a vaccine used to prevent pneumococcal infections, which are not directly related to the management of thalassaemia. D) Penicillin V prophylaxis may be prescribed to prevent infections in individuals with thalassaemia, but it is not a curative treatment. Educationally, understanding the management of thalassaemia is crucial for pediatric nurses caring for children with this condition. Knowing the appropriate interventions, such as bone marrow transplant, can help nurses provide comprehensive and effective care to improve outcomes for their patients. It is essential for nurses to be knowledgeable about the different treatment options available to provide safe and evidence-based care for children with thalassaemia.

Correct Answer: B

Rationale: In the context of pediatric nursing and cardiovascular disorders, the correct answer, option B, states that antenatal steroids are usually given as two oral doses of dexamethasone, is false. The rationale behind this is that antenatal steroids are typically administered as intramuscular injections, specifically betamethasone or dexamethasone, to enhance fetal lung maturation and reduce the risk of respiratory distress syndrome. Option A, stating that Curosurf is a natural surfactant, is a true statement. Curosurf is a natural surfactant derived from porcine lung extract and is used to treat respiratory distress syndrome in neonates by reducing surface tension in the alveoli. Option C, suggesting that increasing the rate on the ventilator raises the mean airway pressure, is a true statement. When the ventilator rate is increased, the amount of air delivered per unit time increases, subsequently raising the mean airway pressure. Option D, indicating that CPAP is a common weaning tool in ventilated preterms, is a true statement. Continuous positive airway pressure (CPAP) is often used as a means of weaning preterm infants off mechanical ventilation by providing continuous pressure to keep the airways open and improve oxygenation. Educationally, understanding the correct administration of antenatal steroids, the role of surfactants like Curosurf, the impact of ventilator settings on mean airway pressure, and the use of CPAP in weaning preterm infants off ventilation are crucial aspects of pediatric nursing care in cardiovascular disorders. It is essential for nurses to have a solid foundation in pharmacology to ensure safe and effective care for pediatric patients with complex health needs.

Correct Answer: A

Rationale: In the context of pediatric nursing and cardiovascular disorders, understanding neurodegenerative diseases is crucial as they can impact a child's overall health and well-being. In this question, the correct answer is A) Niemann-Pick disease, a neurodegenerative disorder characterized by the abnormal accumulation of lipids in cells, leading to progressive neurological deterioration. Option B) Metachromatic leukodystrophy is a metabolic disorder that affects the nervous system primarily, causing demyelination. While it is a neurodegenerative disease, it is not specifically related to cardiovascular disorders, making it an incorrect choice for this question. Option C) Leigh's encephalopathy is a rare, inherited neurometabolic disorder that affects the central nervous system. While it is a neurodegenerative disease, it does not fall under the category of cardiovascular disorders, making it an incorrect choice in this context. Option D) Canavan disease is a rare, inherited disorder that damages the ability of nerve cells to send and receive messages. Similar to the other incorrect options, Canavan disease is a neurodegenerative disorder but is not directly associated with cardiovascular disorders, making it an incorrect choice for this question. Educationally, knowing the specific neurodegenerative diseases associated with pediatric patients with cardiovascular disorders helps nurses provide comprehensive care and anticipate potential complications. Understanding the distinctions between these diseases enhances clinical reasoning skills and improves patient outcomes through early recognition and intervention.

Correct Answer: D

Rationale: In this scenario, a VII nerve injury at the exit from the stylomastoid foramen results in facial nerve paralysis. The correct answer, D) Inability to open the ipsilateral eye, is explained by the fact that the facial nerve innervates the muscles responsible for eyelid closure. This paralysis causes the inability to close the eye, not open it. Option A) Loss of sensation over the vallecula is incorrect because sensation over the vallecula is innervated by the glossopharyngeal nerve (IX), not the facial nerve (VII). Option B) Loss of taste over the anterior third of the tongue is incorrect as taste sensation over the anterior two-thirds of the tongue is carried by the chorda tympani nerve, a branch of the facial nerve. Option C) Increased responsiveness to loud noises is unrelated to a VII nerve injury and is not a typical consequence of facial nerve dysfunction. Educationally, understanding the specific functions of cranial nerves and their areas of innervation is crucial in clinical practice, especially in pediatric nursing where accurate assessment and interpretation of symptoms are vital for providing appropriate care. This knowledge helps in differentiating between various cranial nerve lesions and determining the appropriate interventions for the patient's condition.