Which of the following IS TRUE regarding Wilson's disease

Correct Answer: C

Rationale: Wilson's disease is an autosomal recessive disorder characterized by impaired hepatic copper transport, leading to copper accumulation in various organs. The correct answer is C) Urinary copper is high. This is because in Wilson's disease, there is impaired excretion of copper into bile, resulting in high levels of copper in the body, including the urine. Option A) Serum ceruloplasmin is high is incorrect because in Wilson's disease, there is decreased ceruloplasmin levels due to impaired synthesis by the liver. Option B) Autosomal dominant disorder is incorrect as Wilson's disease follows an autosomal recessive pattern of inheritance, requiring two copies of the defective gene for the disease to manifest. Option D) Hepatic dysfunction is usually mild is incorrect because Wilson's disease can lead to severe hepatic dysfunction, including hepatitis, cirrhosis, and liver failure if left untreated. From an educational perspective, understanding the pathophysiology of Wilson's disease is crucial for pediatric nurses to recognize and manage this condition in pediatric patients. By knowing the key clinical manifestations and diagnostic findings associated with Wilson's disease, nurses can provide comprehensive care, educate patients and families about the importance of compliance with treatment, and facilitate timely interventions to prevent complications.

Correct Answer: A

Rationale: In Tetralogy of Fallot (TOF), the correct statement is A) Squatting is a common clinical presentation. This is because squatting helps decrease the systemic vascular resistance, which can improve blood flow to the lungs in TOF patients, relieving cyanosis. This physiological response is known as the squatting maneuver and is a characteristic finding in TOF. Option B) Hypercyanotic spells in TOF are managed by increasing systemic vascular resistance, typically with methods like knee-chest position or administration of oxygen, not IV digoxin. IV digoxin is not recommended in this scenario. Option C) Brain abscess is not a common neurological complication in TOF. Complications like hypoxic brain injury or developmental delays due to chronic hypoxemia are more likely. Option D) The typical murmur heard in TOF is a systolic ejection murmur, not a pansystolic murmur. Understanding the characteristic findings in TOF is crucial for nurses caring for pediatric patients with this congenital heart defect. Recognizing these clinical presentations and appropriate interventions are essential for providing safe and effective care to these children.

Correct Answer: C

Rationale: Wilson's disease is an autosomal recessive disorder characterized by the accumulation of copper in the liver, brain, and other tissues. The correct answer is C) Low ceruloplasmin in serum. Ceruloplasmin is a protein responsible for transporting copper in the blood, and in Wilson's disease, there is a decrease in ceruloplasmin levels due to impaired copper excretion, leading to copper buildup. Option A) Brushfield spots are not associated with Wilson's disease but are seen in individuals with Down syndrome. Option B) Autosomal dominant disorder is incorrect as Wilson's disease follows an autosomal recessive pattern of inheritance. Option D) High ceruloplasmin in serum is also incorrect as the hallmark of Wilson's disease is low ceruloplasmin levels. In an educational context, understanding the key clinical manifestations and diagnostic markers of Wilson's disease is crucial for pediatric nurses to provide safe and effective care to pediatric patients. By knowing that low ceruloplasmin levels are indicative of Wilson's disease, nurses can collaborate with healthcare providers to ensure timely diagnosis and management of this potentially life-threatening condition.

Correct Answer: C

Rationale: Encopresis is a condition where a child voluntarily passes stool in inappropriate places, such as in their underwear. This behavior is usually not due to a medical issue but rather psychological factors like stress or resistance to toilet training. Therefore, option C is correct as it directly describes the characteristic feature of encopresis. Option A, rabbit pellets form of stool, describes a possible appearance of stool but does not define encopresis. Option B, diarrhea, is a completely different bowel issue characterized by loose, watery stools. Option D, hard infrequent painful defecation, describes constipation, which is also distinct from encopresis. In an educational context, understanding the correct definition of encopresis is crucial for pediatric nurses to provide appropriate care and support to children and their families. By recognizing the behavior patterns associated with encopresis, nurses can tailor interventions to address the underlying causes and help the child overcome this condition effectively.

Correct Answer: D

Rationale: In pediatric nursing, understanding acute hepatitis A is crucial. The correct answer is D) Extrahepatic manifestations like cutaneous vasculitis and arthritis. This is because acute hepatitis A is primarily a hepatic illness but can also present with extrahepatic manifestations such as cutaneous vasculitis and arthritis. These manifestations can help healthcare providers differentiate hepatitis A from other types of hepatitis. Option A) More than one serotype recognized is incorrect because hepatitis A is caused by a single serotype of the hepatitis A virus, which is an RNA virus. Option B) Maternal transmission is common is incorrect as hepatitis A is primarily transmitted through the fecal-oral route due to poor sanitation and hygiene practices, rather than being commonly transmitted from mother to child. Option C) DNA virus is incorrect because hepatitis A is an RNA virus, not a DNA virus. Educationally, understanding the clinical manifestations of hepatitis A is essential for pediatric nurses to provide accurate assessment, diagnosis, and care for pediatric patients. Recognizing extrahejsonatic manifestations can aid in prompt diagnosis and appropriate management of acute hepatitis A cases in children. Nurses play a vital role in educating families about prevention strategies, such as vaccination and hygiene practices, to reduce the spread of hepatitis A in pediatric populations.