Maltese crosses' are identified in the urinary sediments in

Correct Answer: D

Rationale: In this question, the correct answer is D) Fabry disease. Maltese crosses are lipid-laden tubular epithelial cell casts seen in the urine sediment of patients with Fabry disease. This finding is due to the accumulation of globotriaosylceramide in the lysosomes of renal tubular cells. Option A) Niemann Pick disease is incorrect because it presents with foam cells in the bone marrow, liver, and spleen, not Maltese crosses in the urine. Option B) Gaucher disease is incorrect as it presents with Gaucher cells (lipid-laden macrophages), not Maltese crosses in the urine sediment. Option C) Krabbe's disease is incorrect as it is a lysosomal storage disorder that results in the accumulation of galactocerebroside, not leading to the presence of Maltese crosses in the urine. Educationally, understanding the specific urine sediment findings in different diseases is crucial for healthcare professionals, especially in diagnosing and managing rare conditions like Fabry disease. Connecting these findings to the underlying pathophysiology helps in clinical decision-making and providing appropriate care to patients.

Correct Answer: C

Rationale: In the scenario presented, the most appropriate initial treatment for the 18-month-old infant experiencing acute anaphylaxis with bronchospasm towards antibiotic injection is option C) Inj. Epinephrine 0.01 mg IV/IM stat. This is the correct choice because epinephrine is the first-line treatment for anaphylaxis, especially when there is respiratory compromise such as bronchospasm. In this pediatric case, the dose of epinephrine is weight-based (0.01 mg/kg), making it the most suitable option for this 10 kg infant. Option A) Inj. Hydrocortisone 100 mg IV stat is not the most appropriate initial treatment in this acute situation. While corticosteroids like hydrocortisone play a role in the management of anaphylaxis, they are not the first-line treatment for acute bronchospasm and should be administered after epinephrine. Option B) Inj. Epinephrine 0.1 mg IV/IM stat is a higher dose than recommended for this infant's weight and age. Using this higher dose could lead to adverse effects, especially in pediatric patients. Option D) Nebulization Salbutamol + oxygen is not the most appropriate initial treatment for acute anaphylaxis with bronchospasm. While bronchodilators like salbutamol can help with bronchospasm, epinephrine remains the first-line treatment due to its broader effects in anaphylactic reactions. Educationally, it is crucial for healthcare providers to understand the principles of managing anaphylaxis, especially in pediatric patients. Knowing the correct dosages and medications for different age groups is essential to provide safe and effective care in such critical situations. Weight-based dosing is particularly crucial in pediatrics to avoid medication errors and ensure optimal outcomes for the patient.

Correct Answer: C

Rationale: In the context of recurrent Neisserial meningitis in children, the correct answer is C) C8. This is because a deficiency in C8 leads to impaired formation of the membrane attack complex (MAC), which plays a crucial role in the host defense against Neisseria species. The MAC is responsible for creating pores in the bacterial cell membrane, ultimately leading to bacterial lysis and destruction. Option A) C3 and option B) C4 are incorrect because deficiencies in these components would not directly impact the formation of the MAC, which is critical in combating Neisseria infections. Option D) All is also incorrect as the specific deficiency linked to recurrent Neisserial meningitis is that of C8, not all complement components. Educationally, understanding the role of the complement system in immune defense is crucial for healthcare professionals, especially in the context of infectious diseases like Neisserial meningitis. This knowledge aids in proper diagnosis, treatment, and prevention strategies for patients, particularly in pediatric populations where such conditions can have severe consequences.

Correct Answer: D

Rationale: In the context of newborns, understanding common dermatological conditions is essential for healthcare providers. In this question, the correct answer is D) Infantile acropustulosis. Infantile acropustulosis is not a normal variant in newborns; it is a skin condition characterized by itchy, small, red bumps on the hands and feet. This condition is not considered a typical occurrence in healthy newborns. A) Milia is a common and benign condition in newborns, presenting as small white bumps on the face due to blocked oil glands. B) Neonatal pustular melanosis is a transient condition characterized by pustules on a pigmented background, commonly seen in newborns of color. C) Dermal melanocytosis, such as Mongolian spots, are common in newborns with darker skin tones and are considered normal variants. Educationally, understanding these differences is crucial for healthcare providers working with newborns. Recognizing normal variants versus abnormal conditions helps in providing appropriate care and reassurance to parents. It also aids in early identification and management of any potential health issues that may arise in newborns. It highlights the importance of thorough assessment and knowledge of pediatric dermatology in clinical practice.

Correct Answer: A

Rationale: The presence of "blueberry muffin" lesions in infants with rubella is due to dermal erythropoiesis. This occurs when the bone marrow, in response to an infection like rubella, releases immature erythroid cells into the bloodstream, which then accumulate in the skin, causing the characteristic purplish skin lesions. Option B, thrombocytopenia, is incorrect because it refers to a low platelet count, which would not directly cause the blueberry muffin lesions seen in rubella. Option C, ecchymosis, is incorrect as ecchymosis presents as bruising due to blood leaking from damaged vessels, not the same mechanism as seen in blueberry muffin lesions. Option D, vasculitis, is also incorrect as it refers to inflammation of blood vessels, which is not the primary cause of these lesions in rubella. In an educational context, understanding the pathophysiology behind skin manifestations in pediatric conditions like rubella is crucial for nurses to provide effective care. Recognizing these distinctive skin findings can aid in early diagnosis and appropriate management of the underlying condition. Pharmacological interventions may also be necessary in some cases, highlighting the importance of pharmacology knowledge in pediatric nursing practice.