All the following are features of Stickler syndrome EXCEPT

Correct Answer: B

Rationale: Stickler syndrome is a genetic disorder characterized by a group of features that can affect multiple parts of the body. The correct answer, B) autosomal recessive inheritance, is the exception among the given options. Stickler syndrome is actually inherited in an autosomal dominant pattern, meaning that only one copy of the mutated gene is needed for the individual to develop the syndrome. Option A) prominent joints is a common feature of Stickler syndrome, where individuals may have joint hypermobility or joint issues. Option C) hypotonia, or low muscle tone, can also be present in individuals with Stickler syndrome due to the connective tissue abnormalities associated with the condition. Option D) retinal detachment is another feature of Stickler syndrome, as individuals with this condition are at risk for eye problems, including retinal issues. In an educational context, understanding the genetic inheritance pattern of Stickler syndrome is crucial for healthcare professionals working with pediatric patients. Recognizing the features of the syndrome can aid in early diagnosis and appropriate management of the condition. By knowing the correct inheritance pattern and typical clinical manifestations, nurses can provide comprehensive care and support for children and families affected by Stickler syndrome.

Correct Answer: D

Rationale: Rationale: The correct answer is option D) usually diagnosed before school age. Achalasia is a rare motility disorder of the esophagus characterized by the inability of the lower esophageal sphincter to relax, resulting in difficulty swallowing (dysphagia), regurgitation of undigested food, chest pain, and weight loss. While achalasia can occur at any age, it is more commonly diagnosed in adults rather than in children. Option A) dysphagia for solids and liquids is a feature of achalasia as the impaired esophageal motility affects the passage of both solids and liquids through the esophagus. Option B) may be accompanied by undernutrition is true as untreated achalasia can lead to weight loss and malnutrition due to difficulty in swallowing and inadequate intake of nutrients. Option C) may be misdiagnosed as asthma is also a common occurrence as the symptoms of achalasia such as chest pain and difficulty breathing can mimic asthma, leading to a misdiagnosis. In an educational context, understanding the clinical features of achalasia is crucial for pediatric nurses to provide early identification, intervention, and support for children with this condition. By differentiating achalasia from other gastrointestinal and respiratory disorders, nurses can collaborate with healthcare providers to ensure timely diagnosis and appropriate management to improve the quality of life for pediatric patients with achalasia.

Correct Answer: A

Rationale: Hypertrophic pyloric stenosis (HPS) is a condition characterized by thickening of the pylorus muscle, leading to obstruction at the gastric outlet in infants. The association of HPS with conditions like eosinophilic gastroenteritis, Apert syndrome, Zellweger syndrome, and trisomy 21 is crucial for pediatric gastrointestinal nurses to understand. A) Eosinophilic gastroenteritis is NOT associated with hypertrophic pyloric stenosis. Eosinophilic gastroenteritis is an inflammatory disorder involving eosinophils in the gastrointestinal tract, while HPS is a mechanical obstruction at the pylorus. B) Apert syndrome is a genetic disorder characterized by craniosynostosis and syndactyly but is not directly linked to HPS. C) Zellweger syndrome is a rare genetic disorder affecting multiple organ systems, including the liver, but does not have a direct association with HPS. D) Trisomy 21 (Down syndrome) has not been linked to HPS. Down syndrome is characterized by an extra chromosome 21 and is associated with various medical issues, but not specifically HPS. Understanding these associations helps nurses in pediatric gastrointestinal care differentiate between various conditions, provide appropriate care, and educate families effectively. It also highlights the importance of thorough assessment and accurate diagnosis in pediatric patients presenting with gastrointestinal symptoms.

Correct Answer: B

Rationale: In healthy full-term infants, passing meconium within the first 48 hours of birth is expected. The correct answer is B) 90%. This timing is indicative of normal functioning of the gastrointestinal system in newborns. Meconium is the first stool passed by a newborn and consists of amniotic fluid, intestinal epithelial cells, lanugo, mucus, and bile. Option A) 99% is incorrect as not all infants will pass meconium within the first 48 hours. While it is a majority, it is not the case for every single infant. Option C) 80% and option D) 70% are also incorrect as they underestimate the frequency of passing meconium within the specified time frame. Understanding the expected timeline for passing meconium is crucial for nurses caring for newborns. It serves as an important indicator of gastrointestinal health and functioning. Nurses should be aware of this normal variation to identify any potential issues promptly. Educating nursing students on these normal milestones helps them provide quality care and intervene appropriately if deviations from the norm occur.

Correct Answer: C

Rationale: In pediatric gastrointestinal nursing, understanding the typical presenting symptoms of peptic ulcer disease in infants and younger children is crucial for accurate diagnosis and effective interventions. In this case, the correct answer is C) epigastric pain. Epigastric pain is a common symptom in older children and adults with peptic ulcer disease, but it is not a usual presenting symptom in infants and younger children. Young children may not be able to articulate or localize their pain in the epigastric region as effectively as older individuals. Feeding difficulty (Option A) is often seen in infants with peptic ulcer disease due to discomfort while eating. Vomiting (Option B) can occur as a result of irritation in the gastrointestinal tract. Hematemesis (Option D), which is vomiting blood, can be a severe symptom of peptic ulcer disease in children. Educationally, it is important for pediatric nurses to be aware of the differences in how peptic ulcer disease may present in infants and younger children compared to older patients. This knowledge enables nurses to conduct thorough assessments, provide appropriate interventions, and collaborate effectively with healthcare providers to ensure optimal care for pediatric patients with gastrointestinal issues.