Uworld PEDDIATRY

A A A The nurse planning teaching for the parents of a child newly diagnosed with hemophilia will include information about which long-term complication? 1. Heart valve injury [33%] 2. Intellectual disability [3%] 3. Joint destruction [54%] 4. Recurrent pneumonia [8%] Explanation: Hemophilia is a bleeding disorder caused by a deficiency in coagulation proteins. Clients with classic hemophilia, or hemophilia A, lack factor VIII. Clients with hemophilia B (Christmas disease) lack factor IX. When injured, clients with hemophilia should be monitored closely for external as well as internal bleeding. The most frequent sites of bleeding are the joints (80%), especially the knee. Hemarthrosis can occur with minimal or no trauma, with episodes beginning during toddlerhood when the child is active and ambulatory. Over time, chronic swelling and deformity can occur. (Option 1) Heart valve injury is common with rheumatic heart disease not hemophilia. (Option 2) Intellectual disability in children is commonly seen with fetal alcohol syndrome, Down syndrome, hypothyroidism, and lead poisoning. In rare cases, hemophilia can cause life-threatening intracranial bleeding. However, isolated intellectual disability is not seen. (Option 4) Recurrent pneumonia is commonly seen with cystic fibrosis not hemophilia. Educational objective: Clients with hemophilia are at risk for permanent joint destruction due to frequent bleeds into the joint spaces. Assisting clients with decreasing the incidence of bleeding episodes and prompt treatment when bleeding occurs can help minimize joint destruction. A A A The clinic nurse supervises a graduate nurse who is teaching the parents of a 2-year-old with acute diarrhea about home management. The nurse would need to intervene when the graduate nurse provides which instruction? 1. "Do not administer antidiarrheal medications to your child." [26%] 2. "Follow the bananas, rice, applesauce, and toast diet for the next few days." [32%] 3. "Record the number of wet diapers and return to the clinic if you notice a decrease." [28%] 4. "Use a skin barrier cream such as zinc oxide in the diaper area until diarrhea subsides." [12%] Explanation: During bouts of acute diarrhea and dehydration, treatment focuses on maintaining adequate fluid and electrolyte balance. The first-line treatment is oral rehydration therapy, using oral rehydration solutions (ORSs) to increase reabsorption of water and sodium. Even if the diarrhea is accompanied by vomiting, ORS should still be offered in small amounts at frequent intervals. Continuing the child's normal diet (solid foods) is encouraged as it shortens the duration and severity of the diarrhea. The BRAT (bananas, rice, applesauce, and toast) diet is not recommended as it does not provide sufficient protein or energy. (Option 1) Use of antidiarrheal medications is discouraged as these have little effect in controlling diarrhea and may actually be harmful by prolonging some bacterial infections and causing fatal paralytic ileus in children. (Option 3) Parents should be taught to monitor their child for signs of dehydration by checking the amount of fluid intake, number of wet diapers, presence of sunken eyes, and the condition of the mucous membranes. (Option 4) Protecting the perineal skin from breakdown during bouts of diarrhea can be accomplished by using skin barrier creams (eg, petrolatum or zinc oxide). Educational objective: When a child is experiencing acute diarrhea, the priority is to monitor for dehydration. Treatment is accomplished with oral rehydration solutions and early reintroduction of the child's normal diet (usual foods). A A A The mother of a 6-year-old child with cystic fibrosis (CF) has received instruction on the use of pancreatic enzymes. Which statement made by the mother indicates a need for further teaching? 1. "I need to monitor the total amount of this medication that I give to my child every day." [6%] 2. "I should give this medication with or just before my child has a meal or snack." [10%] 3. "It is okay for my child to chew this medication." [61%] 4. "It is okay to open the capsule and sprinkle the medicine on a tablespoon of applesauce." [21%] Explanation: In CF, unusually thick mucus obstructs the pancreatic ducts, preventing pancreatic enzymes (amylase, trypsin, and lipase) from reaching the small intestine. The result is malabsorption of carbohydrates, fats, and proteins; the inability to absorb fatsoluble vitamins (A, D, E, and K) is of particular concern. Gastrointestinal signs and symptoms of CF include flatulence, abdominal cramping, ongoing diarrhea, and/or steatorrhea. Nutritional therapy includes the administration pancreatic enzyme supplements with or just before every meal or snack (Option 2). These enzymes are enteric-coated beads designed to dissolve only in an alkaline environment similar to that of the small intestine. They must not be mixed with a substance that would cause them to dissolve prior to reaching the jejunum. Capsule contents may be sprinkled on applesauce, yogurt, or acidic, soft, room-temperature foods with pH <4.5. Capsules should be swallowed whole and not crushed or chewed; chewing the capsules could cause irritation of the oral mucosa. Excessive intake of pancreatic enzymes can result in fibrosing colonopathy (Option 1). (Option 4) This is a true statement; some children have difficulty taking a whole capsule. Capsule contents can be sprinkled in acidic substances such as applesauce. Capsules should not be taken with milk as they can cause it to curdle. Educational objective: Pancreatic enzyme supplements are used to aid the absorption of carbohydrates, fats, and proteins in a child with CF. They are taken with or just before every meal (not as needed); should be swallowed whole or sprinkled on an acidic food; and should not be crushed or chewed. They should not be taken with milk. Excessive intake could result in fibrosing colonopathy