All of the following are true about congenital central hypoventilation syndrome (Ondine curse) EXCEPT

Correct Answer: C

Rationale: Congenital central hypoventilation syndrome (CCHS), also known as Ondine curse, is a rare disorder affecting the autonomic control of breathing. The correct answer is C) most patients present as adolescents. This is because CCHS typically presents in the neonatal period or early infancy, not during adolescence. Option A, there is an association with Hirschsprung disease, is true. CCHS is often associated with Hirschsprung disease, a condition where nerves are missing in parts of the intestine. Option B, some cases are familial, is also true as CCHS can have a genetic component with familial cases being reported. Educationally, understanding CCHS is crucial for pediatric respiratory nurses as it is a life-threatening condition that requires specialized care. Recognizing the typical presentation and associated conditions can aid in early diagnosis and management, improving patient outcomes. By knowing the key features of CCHS, healthcare providers can provide appropriate support and interventions for affected individuals and their families.

Correct Answer: C

Rationale: In the case of a 7-month-old with a low-grade fever, nasal congestion, and a mild cough, the correct nursing care management should include option C: Instilling saline nose drops and bulb suctioning. This is the most appropriate choice because nasal congestion can be distressing for infants and can lead to difficulty breathing and feeding. By using saline nose drops, you can help to loosen the mucus, making it easier to suction out with a bulb syringe, thus improving the infant's breathing and overall comfort. Option A, maintaining strict bedrest, is not recommended for infants with mild respiratory symptoms as it can lead to decreased muscle tone and potential complications such as pneumonia. Infants should still have supervised tummy time and opportunities for movement. Option B, avoiding contact with family members, is unnecessary in this scenario unless a family member is sick to prevent the spread of infection. It is important for infants to have close contact with caregivers for comfort and emotional well-being. Option D, keeping the head of the bed flat, is not ideal for infants with respiratory symptoms as elevating the head of the bed slightly can help improve breathing by reducing nasal congestion and postnasal drip. Educationally, it is crucial for nurses to understand the appropriate management of common respiratory symptoms in infants to provide safe and effective care. Teaching parents about the use of saline nose drops and bulb suctioning can empower them to alleviate their child's discomfort at home and know when to seek further medical assistance. This knowledge equips nurses to support families in managing common pediatric respiratory issues.

Correct Answer: D

Rationale: In the context of a post-tonsillectomy assessment in a child, the most concerning observation is option D) Swallowing excessively. This is because excessive swallowing could indicate bleeding, which is a critical post-operative complication that requires immediate medical attention. Bleeding after a tonsillectomy is a serious concern due to the rich blood supply to the tonsil area. Option A) Elevated heart rate and blood pressure could be expected in the immediate post-operative period due to the stress of surgery, anesthesia, and pain; however, it is not the most concerning observation in this context. Option B) Complaining of a sore throat is a common and expected symptom after a tonsillectomy due to the surgical removal of the tonsils, so it is not the most concerning observation. Option C) Refusing to eat solid foods is also a common occurrence after a tonsillectomy due to throat discomfort, so it is not as concerning as the potential sign of bleeding indicated by excessive swallowing. Educationally, this question highlights the importance of recognizing and prioritizing critical assessment findings in pediatric post-operative care. It underscores the need for nurses to be vigilant in monitoring for signs of complications, especially in procedures with potential risks like tonsillectomies. Nurses must be prepared to intervene promptly and effectively to ensure the safety and well-being of pediatric patients in the post-operative period.

Correct Answer: C

Rationale: The correct answer is C) Change the child to a higher-calorie formula. In pediatric cystic fibrosis (CF), there is a higher metabolic demand due to increased work of breathing and poor nutrient absorption. Therefore, meeting the nutritional needs of infants with CF is crucial for growth and development. Choosing a higher-calorie formula helps ensure that the infant receives adequate calories to support growth despite potential malabsorption issues common in CF. Option A) Increasing the number of fresh fruits and vegetables is not sufficient to meet the high caloric needs of infants with CF. While fruits and vegetables are nutritious, they may not provide the necessary calorie density required. Option B) Advancing the child's diet to whole cow's milk is not recommended for infants with CF because whole cow's milk is difficult to digest and may exacerbate gastrointestinal symptoms common in CF, such as malabsorption and diarrhea. Option D) Increasing the child's carbohydrate intake alone may not address the overall increased caloric needs of infants with CF. While carbohydrates are an essential energy source, focusing solely on carbohydrates may not provide the necessary nutrients and calories required for optimal growth in CF. Educationally, it is important to understand the specific nutritional needs of children with chronic conditions like CF to provide appropriate care and support. By choosing a higher-calorie formula, healthcare providers can better ensure that infants with CF receive the necessary nutrients to thrive despite the challenges posed by the disease.

Correct Answer: A

Rationale: The correct answer is option A) A 2-month-old born at 32 weeks' gestation is at the highest risk for requiring hospitalization to treat respiratory syncytial virus (RSV). Rationale: 1. Premature infants, especially those born before 35 weeks' gestation, are at higher risk for severe RSV infections due to their immature immune systems and underdeveloped lungs. This places them at increased risk for respiratory distress and complications requiring hospitalization. 2. Option B) A 16-month-old with a tracheostomy is at risk for respiratory complications; however, the highest risk for severe RSV infection leading to hospitalization lies with premature infants due to their vulnerability. 3. Option C) A 3-year-old with a congenital heart defect is at increased risk for respiratory infections, but the highest risk for severe RSV infection requiring hospitalization is in premature infants. 4. Option D) A 4-year-old born at 30 weeks' gestation is also at risk for RSV infection, but the highest risk remains with the 2-month-old born at 32 weeks' gestation due to their younger age and corresponding vulnerability. Educational Context: Understanding the risk factors for severe RSV infection leading to hospitalization is crucial for pediatric nurses. Knowledge of high-risk populations allows nurses to provide targeted education to parents, implement preventative measures, and recognize early signs of respiratory distress in vulnerable infants. This knowledge is essential for delivering optimal care and improving outcomes for pediatric patients with RSV infections.