A 5-year-old boy has systemic juvenile idiopathic arthritis (sJIA) with systemic manifestations including fever, hepatosplenomegaly, and pericarditis. Of the following, the BEST initial treatment for this boy is

Correct Answer: B

Rationale: In the case of a 5-year-old boy with systemic juvenile idiopathic arthritis (sJIA) presenting with systemic manifestations such as fever, hepatosplenomegaly, and pericarditis, the BEST initial treatment is systemic glucocorticoids (Option B). Systemic glucocorticoids are the first-line treatment for sJIA due to their potent anti-inflammatory effects. In this acute systemic inflammatory condition, glucocorticoids can rapidly reduce inflammation and control symptoms like fever, pericarditis, and organomegaly. NSAIDs (Option A) are commonly used in arthritis, but in sJIA with systemic features, systemic glucocorticoids are preferred for their broader anti-inflammatory action. TNF-a inhibitors (Option C) and IL-1 antagonists (Option D) are biologic agents used in refractory cases or when glucocorticoids are not effective. They are not typically considered as initial treatments due to their higher cost, route of administration, and potential side effects in pediatric patients. Educationally, understanding the rationale behind selecting the appropriate initial treatment in sJIA is crucial for pediatric nurses. It highlights the importance of recognizing the specific manifestations of the disease and tailoring treatment to address the underlying inflammatory process effectively while considering the safety and efficacy profiles of different medications in pediatric populations.

Correct Answer: D

Rationale: Juvenile dermatomyositis (JDM) is a rare autoimmune disease that primarily affects the skin and muscles in children. The correct answer to this question is D) facial erythema sparing the nasolabial folds is not a common cutaneous manifestation of JDM. Facial erythema sparing the nasolabial folds is typically seen in systemic lupus erythematosus (SLE) rather than JDM. This is an important differentiating factor between the two conditions. A) heliotrope rash of the eyelids, B) photosensitivity to ultraviolet light, and C) Gottron papules are all common cutaneous manifestations of JDM. The heliotrope rash is a classic finding in JDM, appearing as a purplish discoloration of the eyelids. Photosensitivity and Gottron papules (raised, scaly patches over the knuckles and finger joints) are also frequently observed in JDM cases. Understanding the specific cutaneous manifestations of pediatric diseases like JDM is crucial for nurses caring for pediatric patients. Recognizing these signs early can lead to prompt diagnosis and appropriate management, ultimately improving patient outcomes. It is essential for pediatric nurses to be knowledgeable about common pediatric conditions and their clinical presentations to provide optimal care to their young patients.

Correct Answer: A

Rationale: In patients with familial Mediterranean fever (FMF), secondary amyloidosis can develop due to chronic inflammation. The organ most seriously affected by secondary amyloidosis in FMF is the kidney. This is because amyloid deposits in the kidney can lead to renal failure, which is a serious complication of the disease. Option A, kidney, is the correct answer because renal involvement is a hallmark feature of secondary amyloidosis in FMF. The deposition of amyloid in the kidney can impair its function and ultimately lead to renal failure if left untreated. Options B, lung, C, nerve, and D, heart, are incorrect because while secondary amyloidosis can affect multiple organs, the kidney is most commonly and severely affected in FMF. Lung involvement is rare in FMF-related amyloidosis, nerve involvement typically manifests as peripheral neuropathy rather than systemic amyloidosis, and heart involvement is less common compared to renal involvement in FMF-related secondary amyloidosis. In an educational context, understanding the specific organ involvement in secondary amyloidosis associated with FMF is crucial for nurses caring for pediatric patients with this condition. Recognizing the signs and symptoms of renal impairment can help in early detection and intervention to prevent complications such as renal failure. This knowledge is essential for providing comprehensive care and improving outcomes for pediatric patients with FMF.

Correct Answer: D

Rationale: The correct answer is D) Takayasu arteritis, as it predominantly affects large blood vessels. Takayasu arteritis is a rare autoimmune disease that causes inflammation in the walls of the aorta and its main branches. This results in narrowing or blockage of these arteries, leading to reduced blood flow to various organs. Option A) Henoch-Schonlein purpura primarily involves small blood vessels and is characterized by a rash, joint pain, gastrointestinal symptoms, and kidney inflammation. Option B) Polyarteritis nodosa affects medium-sized arteries, leading to organ damage due to poor blood supply. It typically does not involve large blood vessels as in the case of Takayasu arteritis. Option C) Kawasaki disease mainly affects medium-sized arteries, particularly the coronary arteries in children. It is characterized by fever, rash, swollen lymph nodes, red eyes, lips, and tongue, and can lead to coronary artery aneurysms if not treated promptly. Educationally, understanding the differences in the types of vasculitis based on the size of blood vessels affected is crucial for pediatric nurses. Recognizing the specific characteristics and target organs of each disease aids in accurate diagnosis, appropriate treatment, and prevention of complications. Nurses play a key role in early identification, monitoring, and management of vasculitis in children to promote optimal outcomes and quality of life.

Correct Answer: D

Rationale: In cases of retinal hemorrhage caused by abusive head trauma (AHT), one of the key distinguishing features is the presence of traumatic retinoschisis. This condition is not typically associated with AHT-related retinal hemorrhages, making option D the correct answer. Multiple retinal hemorrhages are a common finding in AHT cases, which aligns with option A. These hemorrhages often involve multiple layers of the retina (option B) and are frequently located centrally (option C). However, traumatic retinoschisis, which involves a splitting of the retinal layers, is not a typical feature of AHT-related retinal hemorrhages. Educationally, understanding the specific characteristics of retinal hemorrhages in cases of AHT is crucial for pediatric nurses as they play a vital role in identifying and reporting suspected cases of child abuse. Recognizing these distinctive features can aid in early detection and intervention, ultimately safeguarding the well-being of vulnerable pediatric patients.