Atypical hemolytic uremic syndrome is associated with

Correct Answer: B

Rationale: Atypical hemolytic uremic syndrome (aHUS) is associated with Factor H deficiency. Factor H is a regulatory protein that helps control the alternative complement pathway, which is involved in the pathophysiology of aHUS. In aHUS, there is uncontrolled activation of the complement system leading to endothelial damage, platelet activation, and red blood cell destruction. Option A, Factor VII deficiency, is incorrect because Factor VII is involved in the coagulation cascade, not the complement system related to aHUS. Option C, Interleukin 10 deficiency, is incorrect because Interleukin 10 is a cytokine involved in regulating immune responses, not directly linked to aHUS pathogenesis. Option D, Properdin deficiency, is incorrect because Properdin is a positive regulator of the complement system, and deficiencies in Properdin are associated with an increased risk of infections, not aHUS. Understanding the association between Factor H deficiency and aHUS is crucial for nurses caring for pediatric patients. Recognizing the genetic basis of aHUS can aid in early diagnosis and appropriate management strategies to prevent complications. Nurses need to be knowledgeable about the genetic factors contributing to pediatric diseases to provide comprehensive care and education to patients and families.

Correct Answer: B

Rationale: Blau syndrome is a rare autosomal dominant disorder characterized by the triad of granulomatous arthritis, uveitis, and dermatitis. It is caused by mutations in the NOD2 gene. The correct answer is B) Sarcoidosis because Blau syndrome is considered a familial, early-onset form of sarcoidosis. Sarcoidosis is a systemic disease characterized by the formation of non-caseating granulomas in multiple organs, commonly affecting the lungs and lymph nodes. Option A) Amyloidosis is incorrect because it is a group of diseases characterized by the abnormal deposition of amyloid protein in tissues and organs, leading to organ dysfunction. There is no direct association between Blau syndrome and amyloidosis. Option C) Hemochromatosis is incorrect as it is an inherited disorder characterized by excessive absorption of dietary iron, leading to iron overload in various organs. There is no known association between Blau syndrome and hemochromatosis. Option D) Myeloma is incorrect as it is a type of cancer that affects plasma cells in the bone marrow. There is no established link between Blau syndrome and myeloma. Educationally, understanding the associations of Blau syndrome with sarcoidosis is crucial for pediatric nurses caring for children with this condition. Recognizing these connections can aid in early identification, appropriate management, and providing comprehensive care to pediatric patients with Blau syndrome. This knowledge is essential for nurses preparing for the NCLEX exam to ensure they can accurately differentiate between various pediatric cardiovascular conditions.

Correct Answer: D

Rationale: The correct answer is D) Monogenic inheritance. In the context of congenital malformations, monogenic inheritance plays a significant role in the etiology of certain conditions. This means that the condition is caused by a mutation in a single gene and can be passed down from parents to their offspring. Maternal infections (Option A) can also contribute to congenital malformations, but they are not the most common etiology. Maternal illness (Option B) can have an impact on fetal development, but it is not as directly related to congenital malformations as monogenic inheritance. Environmental agents (Option C) such as exposure to toxins or chemicals can also cause congenital malformations, but again, they are not as prevalent as monogenic inheritance in this context. Understanding the role of genetics in congenital malformations is crucial for pediatric nurses as it can help in assessing risk factors, providing genetic counseling to families, and delivering appropriate care to children with inherited conditions. By recognizing the importance of monogenic inheritance, nurses can better support families and provide comprehensive care to pediatric patients with congenital malformations.

Correct Answer: C

Rationale: In pediatric nursing, understanding cardiovascular conditions and scoring systems is crucial for providing safe and effective care to young patients. The correct answer to the question, "Which among the following is an anatomical scoring system?" is option C) SYNTAX Score. The SYNTAX Score is an anatomical scoring system used in adults to assess the complexity of coronary artery disease based on the anatomical features of the lesions. It helps guide treatment decisions, such as whether a patient would benefit more from percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG). Option A) STS Score and option B) Euroscore II are both scoring systems used in cardiac surgery, but they are not specifically anatomical scoring systems like the SYNTAX Score. The STS Score is used to predict the risk of mortality and complications after cardiac surgery, while the Euroscore II assesses the risk of mortality in patients undergoing cardiac surgery. Option D) GRACE Score is a scoring system used in patients with acute coronary syndrome to predict the risk of death and complications. It is not an anatomical scoring system like the SYNTAX Score, which focuses on the complexity of coronary artery lesions. Understanding different scoring systems in pediatric cardiovascular care is essential for nurses to collaborate effectively with the healthcare team and deliver comprehensive care to pediatric patients with cardiac conditions. Nurses must be knowledgeable about these scoring systems to contribute to informed clinical decision-making and ensure optimal patient outcomes.

Correct Answer: C

Rationale: The correct answer is C) 9 for the Duke Treadmill score in this scenario. The Duke Treadmill score is used to assess the severity of coronary artery disease based on exercise tolerance, ST segment deviation, and angina. In this case, the patient exercised for 10 minutes (equivalent to 12 METS), had 1 mm ST depression at peak exercise without angina. These factors contribute to a Duke Treadmill score of 9, indicating moderate risk. Option A) 5 would be incorrect because the patient did not experience angina during exercise, which would lower the Duke Treadmill score. Option B) 7 would be incorrect as well because the presence of 1 mm ST depression without angina at peak exercise would place the patient at a higher risk level than indicated by a score of 7. Option D) 10 would also be incorrect as a score of 10 is typically associated with a lower risk level on the Duke Treadmill score, which is not the case in this scenario. Understanding the Duke Treadmill score is crucial for nurses and healthcare providers working in pediatric cardiovascular care. It helps in risk stratification, treatment planning, and monitoring of patients with suspected or known coronary artery disease. By interpreting the Duke Treadmill score correctly, healthcare professionals can make informed decisions regarding the management and care of pediatric patients with cardiovascular conditions.