Features of Tangier disease include the following EXCEPT

Correct Answer: D

Rationale: Rationale: In Tangier disease, there is a deficiency in the ATP-binding cassette transporter A1 (ABCA1) protein, leading to impaired cellular cholesterol efflux. As a result, individuals with Tangier disease exhibit extremely low levels of plasma cholesterol, not increased levels as seen in the general population. This is why option D, "increased plasma cholesterol," is the correct answer. Option A, "orange tonsils," is a feature of Tangier disease due to cholesterol accumulation in the tonsils, leading to their characteristic orange appearance. Option B, "hepatosplenomegaly," is also seen in Tangier disease due to cholesterol deposition in the liver and spleen. Option C, "relapsing neuropathy," is associated with Tangier disease as a result of lipid deposition in nerve tissues. Understanding the features of Tangier disease is crucial for nurses and healthcare professionals, especially those preparing for the NCLEX exam. Recognizing these distinct clinical manifestations can aid in early diagnosis and appropriate management of patients with rare genetic disorders like Tangier disease. It also highlights the importance of lipid metabolism in maintaining overall health and the consequences of its dysregulation in various diseases.

Correct Answer: C

Rationale: In this question, the correct answer is C) Yersinia. Yersinia enterocolitica is a bacterium that can cause gastroenteritis. Immunoglobulin A (IgA) nephropathy is a condition where IgA immune complexes deposit in the kidneys, leading to inflammation and damage. Yersinia infection can trigger an immune response that results in IgA nephropathy as an extraintestinal manifestation. A) Salmonella is a common cause of food poisoning but is not typically associated with IgA nephropathy. B) Shigella is another bacterium that causes intestinal infection but is not linked to IgA nephropathy. D) Campylobacter is a common cause of bacterial diarrhea but is not known to be associated with IgA nephropathy. Educationally, understanding the extraintestinal manifestations of enteric infections is crucial for healthcare professionals, especially when considering systemic complications like IgA nephropathy. This knowledge helps in early recognition, appropriate management, and prevention of potential long-term consequences for patients. It reinforces the importance of thorough assessment and understanding of the interconnectedness of different body systems in pediatric patients.

Correct Answer: C

Rationale: Ciprofloxacin is the drug of choice for the treatment of Campylobacter jejuni infections because it is effective against this particular bacterium. Campylobacter jejuni is a common cause of bacterial gastroenteritis in children and adults, and ciprofloxacin has shown good efficacy in treating these infections. Option A) Salmonella: Ciprofloxacin is not typically the first-line treatment for Salmonella infections. In fact, the use of ciprofloxacin in Salmonella infections is discouraged due to concerns of increasing antibiotic resistance. Option B) Shigella: While ciprofloxacin may be used in some cases of Shigella infections, resistance to this drug is becoming more common. Other antibiotics like azithromycin or ceftriaxone are often preferred for treating Shigella infections. Option D) Clostridium difficile: Ciprofloxacin is not the drug of choice for Clostridium difficile infections. The primary treatment for C. difficile infections involves stopping the causative antibiotic, supportive care, and in some cases, the use of specific antibiotics like metronidazole or vancomycin. Educational context: Understanding the appropriate use of antibiotics in treating pediatric gastrointestinal infections is crucial to prevent antibiotic resistance and ensure effective treatment. By knowing the specific pathogens targeted by each antibiotic, healthcare providers can make informed decisions to provide the best care for their patients.

Correct Answer: A

Rationale: In pediatric patients, advanced appendicitis is a serious condition that requires prompt diagnosis and treatment. In ultrasound findings, asymmetric wall thickening is not typically associated with advanced appendicitis. Option A is the correct answer because asymmetric wall thickening is more commonly seen in early stages of appendicitis, while advanced cases may present with a uniformly thickened appendix. This distinction is important for accurate diagnosis and appropriate management. Option B, increased local tenderness to compression, is a common clinical finding in advanced appendicitis due to inflammation and irritation of the peritoneum surrounding the appendix. This symptom supports the diagnosis rather than suggesting a different condition. Option C, abscess formation, is a complication of untreated or advanced appendicitis. The presence of an abscess on ultrasound would indicate a more severe and progressed stage of the disease, not ruling out advanced appendicitis. Option D, associated free intraperitoneal fluid, is another concerning finding in advanced appendicitis. The presence of fluid in the peritoneal cavity suggests perforation or rupture of the appendix, which is a serious complication requiring immediate intervention. Educationally, understanding the nuances of imaging findings in pediatric appendicitis is crucial for healthcare professionals, especially those preparing for exams like the NCLEX. Recognizing the specific ultrasound characteristics of advanced appendicitis can aid in timely diagnosis and appropriate management to prevent complications and improve patient outcomes.

Correct Answer: B

Rationale: The correct answer is B) serum lipase. Serum lipase is the preferred test for diagnosing acute pancreatitis because it is more specific and remains elevated for a longer period compared to serum amylase. Lipase is released from the pancreas into the blood when the pancreas is inflamed, making it a more reliable marker for pancreatic injury. Elevated serum lipase levels are a key indicator of acute pancreatitis. Option A) serum amylase is a common test for pancreatitis, but it is less specific and can be elevated in conditions other than pancreatitis, such as salivary gland disorders. Option C) hyperglycemia is a symptom of uncontrolled diabetes rather than a specific test for acute pancreatitis. Option D) elevated y-glutamyl transpeptidase is associated with liver and biliary tract disorders, not acute pancreatitis. Educationally, understanding the appropriate diagnostic tests for acute pancreatitis is crucial for nurses and healthcare professionals caring for pediatric patients. This knowledge ensures timely and accurate diagnosis, leading to appropriate treatment interventions and improved patient outcomes. Regular review of these concepts will enhance clinical decision-making skills and promote quality care delivery in pediatric gastrointestinal health.