Pediatrics NUR 335 Exam Questions

Correct Answer: B. Drowsiness, depressed fontanelle, and decreased skin turgor.

Severe dehydration in infants is characterized by:

Drowsiness/Lethargy: Due to reduced blood flow and oxygen to the brain.

Depressed (sunken) fontanelle: A sign of fluid loss causing reduced intracranial pressure.

Decreased skin turgor: Poor skin elasticity due to reduced fluid in tissues.

Why the Other Options Are Incorrect:

1. Anxiety and increased skin turgor: Anxiety may occur in mild dehydration, but increased skin turgor is not a sign of dehydration. Decreased turgor is.

3. Excessive crying and excessive thirst: These are early signs of mild to moderate dehydration, not severe.

4. Drowsiness, bulging fontanelle, and decreased skin turgor: A bulging fontanelle indicates increased intracranial pressure, often seen in infections like meningitis, not dehydration.

Summary:

Severe dehydration in infants is marked by drowsiness, sunken fontanelle, and decreased skin turgor. Prompt rehydration with oral rehydration solutions (ORS) or IV fluids is crucial to prevent complications.

Correct Answer: B) Glucuronyl transferase conjugates bilirubin with UDPGA to form conjugated bilirubin.

In the liver, bilirubin is conjugated by the enzyme glucuronyl transferase. This enzyme conjugates bilirubin with uridine diphosphoglucuronic acid (UDPGA) to form bilirubin diglucuronide, also known as conjugated bilirubin. This conjugation process makes the bilirubin water-soluble, enabling it to be excreted in bile.

Why Other Options are Wrong:

Option A: While hemoglobin is broken down into bilirubin, this process primarily occurs outside the liver in reticuloendothelial cells (mainly macrophages), not in the liver.

Option C: Although conjugated bilirubin is eventually excreted in bile, bilirubin is not immediately excreted in the urine unless there is a dysfunction in bile secretion or conjugation, such as in conditions like biliary obstruction or liver disease.

Option D: Red blood cells are indeed removed from circulation by reticuloendothelial cells (like macrophages), but this process occurs before bilirubin is processed in the liver. This is part of the destruction of aged or damaged red blood cells, which releases hemoglobin that is then converted to bilirubin.

Summary:

The liver plays a key role in the conjugation of bilirubin, a byproduct of hemoglobin breakdown. The enzyme glucuronyl transferase conjugates bilirubin with UDPGA to form conjugated bilirubin, making it water-soluble and enabling its excretion via bile. Understanding this metabolic pathway is essential for recognizing conditions that affect bilirubin metabolism, such as jaundice or liver dysfunction.

Correct Answer: C. Place the child on droplet precautions

Explanation:

The child is presenting with signs consistent with bacterial meningitis—fever, altered level of consciousness, nuchal rigidity, petechiae, and CSF findings (elevated WBCs, low glucose). Bacterial meningitis is highly contagious, especially in the early stages, and requires droplet precautions to prevent transmission to others. Implementing these precautions is the priority action to protect other patients and healthcare workers from infection.

Why Other Options are Wrong:

A. Administer antipyretic therapy

While the child does have a high fever (104°F), and antipyretics can provide comfort and reduce metabolic demand, infection control is the higher priority. Fever management can follow once isolation measures are in place.

B. Place the child in a lateral Sims position

Although this position might be used post-lumbar puncture or for comfort in some neurological conditions, it does not address the most urgent risk, which is the potential for disease transmission. This makes it a lower priority in the initial moments of care.

D. Reduce all environmental stimuli

Reducing stimuli may help prevent seizure activity or further increase in intracranial pressure, but it is not the immediate priority. Droplet precautions should come first to contain the possible spread of infection.

Correct Answer: C. Decompression with a nasogastric and rectal tube.

The patient is presenting with signs of toxic megacolon, a severe complication of Hirschsprung disease or inflammatory bowel disease (like ulcerative colitis).

Toxic megacolon is a life-threatening condition in which the colon becomes severely dilated, and the patient can experience abdominal distension, fever, and bowel obstruction.

The initial treatment of toxic megacolon typically involves:

1. Supportive care (fluid resuscitation and broad-spectrum antibiotics).

2. Decompression of the colon to reduce the distention and prevent further bowel perforation. This is achieved using a nasogastric tube to relieve gastric distension and a rectal tube to relieve colon distention.

Decompression is critical in managing toxic megacolon to avoid perforation, sepsis, or further complications.

Why the Other Options are Incorrect:

A. Colostomy proximal to the aganglionic segment:

Colostomy is used as part of surgical management for Hirschsprung disease or when surgical intervention is needed to bypass the aganglionic portion of the colon. However, this is not the initial management step for suspected toxic megacolon, as the patient needs to be stabilized and decompressed first.

B. Complete resection of the aganglionic segment of the colon:

Resection of the aganglionic segment is the definitive treatment for Hirschsprung disease but is usually not performed during the acute phase of toxic megacolon. Initial management focuses on decompression and stabilization rather than immediate surgery.

D. Saline enema:

Saline enemas may be used to help relieve constipation, but they are not appropriate in the management of toxic megacolon, which requires more urgent decompression and stabilization due to the risk of perforation and sepsis.

Summary:

In the case of a toxic megacolon, the priority is to decompress the colon and provide supportive care. This is best achieved by using a nasogastric tube and a rectal tube to relieve the distension. Surgical interventions like colostomy or resection are considered after initial stabilization.

Correct Answer: A. Amniotic bands

Amniotic band syndrome (ABS) occurs when strands of the amniotic sac separate and wrap around parts of the fetus's body, including limbs.

This constriction can disrupt the development of the limbs, leading to partial or complete amputations. ABS is considered one of the most common causes of congenital limb deficiencies, and its severity can vary based on when the bands constrict the developing tissues.

Why the Other Options are Incorrect:

B. Cytomegalovirus (CMV) CMV is a viral infection that can cause a range of birth defects when a mother contracts it during pregnancy. While CMV infection can lead to various congenital abnormalities, including hearing loss and developmental delays, it is not a common cause of limb amputations. The main effects of CMV are typically neurological, not limb-related.

C. Maternal diabetes Maternal diabetes, particularly if poorly controlled during pregnancy, can lead to several complications, including macrosomia (a large baby), congenital heart defects, and neural tube defects. While it can cause limb abnormalities, it is not the most common cause of congenital limb deficiencies. The primary impact of diabetes on limb development is often related to other abnormalities, such as clubfoot or polydactyly (extra fingers or toes), rather than complete limb amputations.

D. Vitamin A toxicity Excessive intake of Vitamin A during pregnancy can cause birth defects, including facial and cranial malformations, but it is not a major cause of congenital limb deficiencies. Vitamin A toxicity can interfere with normal cell differentiation during pregnancy, leading to defects in organ development, but limb malformations are not the primary concern in these cases.

Summary:

The most common cause of congenital limb amputations or deficiencies is amniotic band syndrome (A), where the amniotic bands constrict developing limbs in the womb. While other factors like CMV, maternal diabetes, and Vitamin A toxicity can cause birth defects, they are less commonly associated with congenital limb amputations.

Correct Answer: B. Oligoarticular.

Oligoarticular juvenile idiopathic arthritis (JIA) is the most common form of JIA, accounting for about 50% of cases. It is characterized by involvement of four or fewer joints during the first 6 months of the disease.

It is more common in young children and is often associated with a better prognosis, especially in girls.

Why the Other Options are Incorrect:

A. Psoriatic:

Psoriatic JIA is a form of JIA that can occur with or without psoriasis. It is less common than oligoarticular JIA and typically involves a higher number of joints than oligoarticular JIA, with skin involvement (psoriasis) being a hallmark.

C. Polyarticular:

Polyarticular JIA involves five or more joints and represents about 30% of cases of JIA. This form tends to involve large and small joints, including those in the hands, wrists, and knees, and can be more severe than oligoarticular JIA.

D. Enthesitis-related:

Enthesitis-related JIA typically involves inflammation at the site where tendons and ligaments attach to bone (entheses). This type is more common in boys and is associated with HLA-B27 positivity. It is less common than the other types of JIA.

Summary:

The most common type of juvenile idiopathic arthritis (JIA) is oligoarticular JIA, which involves four or fewer joints and is most frequently seen in young children.

Correct Answer: c) Place the infant on their back to sleep.

Place the infant on their back to sleep is the most important recommendation for reducing the risk of sudden infant death syndrome (SIDS). Research has consistently shown that placing infants on their back to sleep significantly reduces the risk of SIDS compared to placing them on their stomach or side. The supine (back) position is recommended for every sleep period, including naps and overnight sleep.

Why Other Options are Wrong:

a) Place the infant on their stomach to sleep:

This position is associated with an increased risk of SIDS. Infants placed on their stomach to sleep are more likely to re-breathe exhaled carbon dioxide, which can contribute to suffocation.

b) Use a soft blanket to keep the infant warm:

Soft bedding, including blankets, pillows, and stuffed animals, can pose a suffocation risk. It is recommended to dress the infant appropriately for sleep and avoid placing soft bedding in the crib.

d) Allow the infant to sleep with a stuffed animal for comfort:

Stuffed animals or any soft items in the crib increase the risk of suffocation. The sleep environment should be kept free of these items to promote safe sleep.

Summary:

To reduce the risk of SIDS, parents should be instructed to place the infant on their back for all sleep periods. This practice, along with a safe sleep environment that includes a firm mattress and no soft bedding, is the most effective way to protect infants from SIDS.

The correct answer is b: Hemarthrosis

Hemarthrosis refers to bleeding into the joints, which is a common complication of hemophilia. Hemophilia is a bleeding disorder where the blood does not clot properly, leading to spontaneous or excessive bleeding. Hemarthrosis is particularly common in weight-bearing joints, such as the knees, elbows, and ankles, and can result in joint damage over time.

Why Other Options are Wrong:

Hematuria: This refers to the presence of blood in the urine, which can be caused by various conditions but is not a typical complication of hemophilia.

Hemoptysis: This is the coughing up of blood, often associated with respiratory conditions, not specifically a complication of hemophilia.

Hematemesis: This refers to vomiting blood, which can occur in conditions such as gastrointestinal bleeding, but is not a common complication of hemophilia.

Thus, hemarthrosis is the most relevant complication related to hemophilia.

Correct Answer: C) Macrocytic RBCs with elevated mean corpuscular volume (MCV)

Diamond-Blackfan anemia is characterized by macrocytic red blood cells (RBCs) and elevated mean corpuscular volume (MCV). The condition is due to a failure in erythropoiesis (production of RBCs) in the bone marrow, leading to a lack of RBC precursors. As a result, the RBCs that are produced are often larger than normal, and the MCV is elevated. The peripheral blood also shows a lack of reticulocytes, which further supports the diagnosis of anemia due to impaired RBC production.

Why Other Options are Wrong:

A) Presence of reticulocytes in peripheral blood:

In Diamond-Blackfan anemia, there is a lack of reticulocytes in the peripheral blood, not an increase. This distinguishes it from other types of anemia, where reticulocyte production might be elevated.

B) Involvement of multiple blood cell lineages:

Diamond-Blackfan anemia primarily affects the RBC lineage and does not typically involve other blood cell lineages (e.g., white blood cells or platelets). The lack of other cell line involvement is a hallmark of this condition.

D) Low RBC mean corpuscular volume (MCV):

In Diamond-Blackfan anemia, the MCV is elevated, not low, due to the macrocytic nature of the RBCs produced in the absence of adequate RBC precursors.

Summary:

Diamond-Blackfan anemia is characterized by macrocytic red blood cells, an elevated mean corpuscular volume (MCV), lack of reticulocytes in peripheral blood, and no involvement of other blood cell lineages. It results from impaired erythropoiesis in the bone marrow, leading to a distinct presentation of anemia in affected neonates.