Regarding cystic fibrosis:

Correct Answer: A

Rationale: The correct answer is A) It is inherited as autosomal recessive. Cystic fibrosis (CF) is a genetic disorder that follows an autosomal recessive pattern of inheritance, meaning that an individual must inherit two copies of the defective gene (one from each parent) to develop the condition. This explains why CF tends to run in families and why siblings of affected individuals have a 25% chance of also having the disease. Option B) There is increased risk of bronchial carcinoma in late adulthood is incorrect because CF primarily affects the lungs and digestive system, leading to complications such as chronic respiratory infections and pancreatic insufficiency. While individuals with CF do have an increased risk of respiratory issues, including bronchiectasis, the risk of bronchial carcinoma is not a common feature of the disease. Option C) Biliary cirrhosis is a recognised feature is incorrect because CF primarily affects the respiratory and digestive systems, and biliary cirrhosis is not a typical manifestation of the disease. The main complications related to CF involve the lungs, pancreas, and intestines. Option D) In neonates, intestinal obstruction may be the first presentation is incorrect because while intestinal obstruction can occur in individuals with CF due to meconium ileus (a blockage in the intestines present at birth), it is not the most common initial presentation of the disease in neonates. Respiratory symptoms, such as meconium ileus equivalent, are more commonly observed in newborns with CF. Educationally, understanding the genetic basis of CF is crucial for healthcare providers working with pediatric patients. Recognizing the inheritance pattern of autosomal recessive conditions like CF can aid in genetic counseling, family education, and early detection of the disease in at-risk populations. It is essential for nurses and other healthcare professionals caring for pediatric patients to have a solid foundation in pediatric pharmacology to provide safe and effective care.

Correct Answer: C

Rationale: In a 6-week-old infant, the features mentioned in option C (biopsy specimen showing proliferation of bile ductules, periportal fibrosis, and bile lakes) are more suggestive of biliary atresia rather than neonatal hepatitis. Biliary atresia is a serious condition where there is a blockage or absence of bile ducts, leading to bile accumulation and liver damage. The biopsy findings in option C are characteristic of the changes seen in biliary atresia. Option A (hepatomegaly) and option B (prolonged INR) can be seen in both biliary atresia and neonatal hepatitis, making them less specific for differentiating between the two conditions. Option D (HIDA scan revealing delayed uptake and excretion of contrast material) is also more indicative of biliary atresia due to the impaired bile flow characteristic of this condition. Understanding the distinguishing features between biliary atresia and neonatal hepatitis is crucial for pediatric nurses to provide prompt and appropriate care. Recognizing the diagnostic criteria and clinical presentation of these conditions can lead to timely interventions such as surgical correction in biliary atresia cases, improving outcomes for these vulnerable patients.

Correct Answer: A

Rationale: Peak expiratory flow rate (PEFR) is a valuable parameter in assessing improvement in acute bronchial asthma because it measures the maximum rate at which a person can exhale air. Option A is correct because changes in PEFR can indicate the effectiveness of therapy in managing acute asthma exacerbations. By regularly monitoring PEFR, healthcare providers can assess the severity of airflow limitation and response to treatment. Option B is incorrect because PEFR primarily reflects large airway resistance rather than small airway resistance. Small airway resistance is better assessed by other pulmonary function tests such as forced expiratory volume in 1 second (FEV1). Option C is incorrect because PEFR is actually more related to age and gender rather than height. Age and gender can influence lung development and function, impacting PEFR values. Option D is incorrect because using PEFR to guide aminophylline therapy in acute asthma is not evidence-based practice. A PEFR less than 50% of normal may indicate severe airflow limitation, but treatment decisions should be based on clinical guidelines and individual patient factors. In an educational context, understanding the significance of PEFR in pediatric cardiovascular nursing is crucial for providing comprehensive care to children with respiratory conditions like asthma. By grasping the role of PEFR in monitoring asthma severity and treatment response, nurses can effectively collaborate with the healthcare team to optimize patient outcomes.

Correct Answer: B

Rationale: In pediatric patients with renal osteodystrophy, the correct contributing feature is hyperparathyroidism (option B). Hyperparathyroidism occurs as a result of chronic kidney disease, leading to secondary hyperparathyroidism, which contributes to the development of renal osteodystrophy. Option A, hypophosphatemia, is not typically associated with renal osteodystrophy. Instead, renal osteodystrophy is characterized by abnormalities in calcium, phosphate, and parathyroid hormone levels. Option C, increased loss of calcium in the kidney, may seem plausible, but the primary issue in renal osteodystrophy is not increased loss but rather disturbances in the balance of calcium, phosphate, and parathyroid hormone metabolism. Option D, hypoalbuminemia, is not directly linked to renal osteodystrophy. While hypoalbuminemia can occur in renal disease, it is not a primary contributing factor to the development of renal osteodystrophy. Educationally, understanding the pathophysiology of renal osteodystrophy in pediatric patients is crucial for nurses caring for these individuals. By grasping the specific factors involved, nurses can better assess, manage, and educate patients and families about the condition and its implications. This knowledge aids in providing holistic care and promoting positive outcomes for pediatric patients with renal osteodystrophy.

Correct Answer: B

Rationale: In this question, the correct answer is B) Giardiasis. Malabsorption refers to the impaired absorption of nutrients in the intestine. Giardiasis is an intestinal infection caused by the Giardia parasite, which can lead to malabsorption due to damage to the intestinal lining and interference with nutrient absorption processes. Option A) Ischaemia of the gut is incorrect because although gut ischemia can lead to various gastrointestinal issues, malabsorption is not a typical manifestation of this condition. Option C) Lymphoma of the ileum is incorrect because while lymphoma can affect the intestine and cause obstruction or other complications, it is not directly associated with malabsorption. Option D) Chronic lead poisoning is incorrect because although lead poisoning can cause various systemic effects, malabsorption is not a primary characteristic of this condition. Educationally, understanding the link between specific diseases or conditions and their effects on the gastrointestinal system is crucial in pediatric nursing. Recognizing the signs and symptoms of malabsorption can help nurses provide appropriate care and interventions for pediatric patients with these conditions. It is vital to differentiate between various causes of malabsorption to ensure accurate diagnosis and effective management for optimal patient outcomes.