A 4-year-old boy is brought to the physician by his parents because of fever and mild abdominal pain for 7 days. His parents report that he developed a rash 2 days ago. He has had no diarrhea or vomiting. Four weeks ago, he returned from a camping trip to Colorado with his family. His immunization records are unavailable. His temperature is 39.4°C (102.9°F), pulse is 111/min, respirations are 27/min, and blood pressure is 96/65 mm Hg. Examination shows bilateral conjunctival injections and fissures on his lower lips. The pharynx is erythematous. There is tender cervical lymphadenopathy. The hands and feet appear edematous. A macular morbilliform rash is present over the trunk. Bilateral knee joints are swollen and tender; range of motion is limited by pain. Which of the following is the most appropriate treatment for this patient's condition?
Q2
A 4-year-old boy is brought to a pediatrician by his parents with a history of fever for the last 5 days and irritability, decreased appetite, vomiting, and swelling of the hands and feet for the last 3 days. The patient’s mother mentions that he has been taking antibiotics and antipyretics prescribed by another physician for the last 3 days, but there has been no improvement His temperature is 39.4°C (103.0°F), pulse is 128/min, respiratory rate is 24/min, and blood pressure is 96/64 mm Hg. On physical examination, there is significant edema of the hands and feet bilaterally. There is a 2.5 cm diameter freely moveable, nontender cervical lymph node is palpable on the right side. A strawberry tongue and perianal erythema are noted. Conjunctival injection is present bilaterally. Laboratory findings reveal mild anemia and a leukocytosis with a left-shift. Erythrocyte sedimentation rate (ESR) and serum C-reactive protein (CRP) are increased. If not treated appropriately, this patient is at increased risk of developing which of the following complications?
Q3
A previously healthy 4-year-old boy is brought to the physician by his parents because he has had a fever, diffuse joint pain, and a rash on his abdomen for the past week. Acetaminophen did not improve his symptoms. He emigrated from China with his family 2 years ago. He attends daycare. His immunization records are not available. His temperature is 38.5°C (101.3°F), pulse is 125/min, and blood pressure is 100/60 mm Hg. Examination shows polymorphous truncal rash. The eyes are pink with no exudate. The tongue is shiny and red, and the lips are cracked. The hands and feet are red and swollen. There is right-sided anterior cervical lymphadenopathy. Which of the following is the most appropriate next step in management?
Q4
A 4-year-old boy is brought to the emergency department by his mother with a rash on his trunk, malaise, and fever with spikes up to 38.5°C (101.3°F) for the past 2 weeks. The patient's mother says she tried giving him Tylenol with little improvement. Past medical history includes a spontaneous vaginal delivery at full term. The patient's vaccines are up-to-date and he has met all developmental milestones. On physical examination, his lips are cracking, and he has painful cervical lymphadenopathy. The rash is morbilliform and involves his trunk, palms, and the soles of his feet. There is fine desquamation of the skin of the perianal region. Which of the following anatomical structures is most important to screen for possible complications in this patient?
Q5
A previously healthy 3-year-old girl is brought to the physician by her parents for the evaluation of recurrent episodes of pain in her left wrist and right knee for 4 months. She has not had any trauma to the knee or any previous problems with her joints. She attends daycare. Her mother has rheumatoid arthritis. Her vital signs are within normal limits. Examination of the right knee and left wrist shows mild swelling, tenderness, warmth, and erythema; range of motion is slightly decreased. No other joints are affected. The remainder of the examination shows no abnormalities. Laboratory studies show:
Hemoglobin 12.4 g/dL
Leukocyte count 9,000/mm3
Platelet count 200,000/mm3
Erythrocyte sedimentation rate 50 mm/h
Serum
Antinuclear antibodies 1:320
Rheumatoid factor negative
This patient is at increased risk for which of the following complications?
Q6
A 4-year-old boy is brought to the emergency department by his parents after 10 days of fever, varying from 38.0–40.0°C (100.4–104.0°F). On physical examination, the child is ill-looking with an extensive rash over his trunk with patchy desquamation. His hands are swollen, and he also shows signs of a bilateral conjunctivitis. The laboratory test results are as follows:
Hemoglobin 12.9 g/dL
Hematocrit 37.7%
Mean corpuscular volume 82.2 μm3
Leukocyte count 10,500/mm3
Neutrophils 65%
Lymphocytes 30%
Monocytes 5%
Platelet count 290,000/mm3
Erythrocyte sedimentation rate (ESR) 35 mm/h
What is the next best step in the management of this patient’s condition?
Q7
A 9-year-old boy is brought to the emergency department because of progressively worsening shortness of breath for 3 days. He has had fever and malaise for the past 5 days. He had a sore throat 3 weeks ago that resolved without treatment. He appears ill. His temperature is 38.6°C (101.5°F), pulse is 98/min and blood pressure is 84/62 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 93%. Examination shows jugular venous distension and bilateral ankle edema. There are erythematous, ring-shaped macules and patches over his trunk that are well-demarcated. Auscultation of the chest shows crackles at the lung bases bilaterally. An S3 is heard on cardiac auscultation. His hemoglobin concentration is 12.2 g/dL, leukocyte count is 13,600/mm3, and platelet count is 280,000/mm3. A urinalysis is normal. An x-ray of the chest shows cardiac silhouette enlargement with prominent vascular markings in both the lung fields. Which of the following is the most likely etiology of this patient's symptoms?
Q8
A 5-year-old girl is brought to the emergency department by her father due to a 6-day history of fevers and irritability. His father reports that the fevers have ranged from 101-104°F (38.3-40°C). He tried to give her ibuprofen, but the fevers have been unresponsive. Additionally, she developed a rash 3 days ago and has refused to wear shoes because they feel "tight." Her father reports that other than 2 ear infections she had when she was younger, the patient has been healthy. She is up-to-date on her vaccinations except for the vaccine boosters scheduled for ages 4-6. The patient's temperature is 103.5°F (39.7°C), blood pressure is 110/67 mmHg, pulse is 115/min, and respirations are 19/min with an oxygen saturation of 98% O2 on room air. Physical examination shows bilateral conjunctivitis, palpable cervical lymph nodes, a diffuse morbilliform rash, and desquamation of the palms and soles with swollen hands and feet. Which of the following is the next step in management?
Q9
A 4-year-old male is brought to the pediatrician for a low-grade fever. His mother states that he has had a waxing and waning fever for the past 6 days with temperatures ranging from 99.8°F (37.7°C) to 101.0°F (38.3°C). She reports that he had a similar episode three months ago. She also reports symmetric joint swelling in the child’s knees and wrists that has become increasingly noticeable over the past 8 weeks. He has not had a cough, difficulty breathing, or change in his bowel movements. The child was born at 40 weeks gestation. His height and weight are in the 45th and 40th percentiles, respectively. He takes no medications. His temperature is 100.1°F (37.8°C), blood pressure is 100/65 mmHg, pulse is 105/min, and respirations are 18/min. On examination, there is a non-pruritic, macular, salmon-colored truncal rash. Serological examination reveals the following:
Serum:
Rheumatoid factor: Negative
Anti-nuclear antibody: Negative
Anti-double stranded DNA: Negative
Anti-SSA: Negative
Anti-SSB: Negative
Human leukocyte antigen B27: Positive
Erythrocyte sedimentation rate: 30 mm/h
This patient is most likely at increased risk of developing which of the following?
Q10
A 4-year-old presents with fever, rash, and red, cracked lips. Physical exam reveals cervical lymphadenopathy. Which of the following disease processes is most likely?
Kawasaki disease US Medical PG Practice Questions and MCQs
Question 1: A 4-year-old boy is brought to the physician by his parents because of fever and mild abdominal pain for 7 days. His parents report that he developed a rash 2 days ago. He has had no diarrhea or vomiting. Four weeks ago, he returned from a camping trip to Colorado with his family. His immunization records are unavailable. His temperature is 39.4°C (102.9°F), pulse is 111/min, respirations are 27/min, and blood pressure is 96/65 mm Hg. Examination shows bilateral conjunctival injections and fissures on his lower lips. The pharynx is erythematous. There is tender cervical lymphadenopathy. The hands and feet appear edematous. A macular morbilliform rash is present over the trunk. Bilateral knee joints are swollen and tender; range of motion is limited by pain. Which of the following is the most appropriate treatment for this patient's condition?
A. Supportive treatment only
B. Intravenous immunoglobulin (Correct Answer)
C. Oral ibuprofen
D. Oral penicillin
E. Oral doxycycline
Explanation: ***Intravenous immunoglobulin***
- This patient presents with symptoms highly suggestive of **Kawasaki disease**, including a persistent fever for over 5 days, **bilateral conjunctival injection**, **lip fissures**, **erythematous pharynx**, **cervical lymphadenopathy**, and **edema of hands and feet** followed by a rash.
- **IV immunoglobulin (IVIG)** is the cornerstone of treatment for Kawasaki disease, significantly reducing the risk of **coronary artery aneurysms** if given early in the disease course.
*Supportive treatment only*
- While supportive care (fever reduction, hydration) is important, relying solely on it for **Kawasaki disease** would increase the risk of severe complications, particularly **coronary artery involvement**.
- **Kawasaki disease** is a systemic vasculitis requiring specific immunomodulatory therapy to prevent long-term cardiac sequelae.
*Oral ibuprofen*
- **Ibuprofen**, a non-steroidal anti-inflammatory drug (**NSAID**), can help manage fever and joint pain, but it does not address the underlying **vasculitis** or prevent the cardiac complications of **Kawasaki disease**.
- **Aspirin** (high-dose initially, then low-dose) is part of Kawasaki disease treatment, but ibuprofen alone is insufficient as primary therapy.
*Oral penicillin*
- **Penicillin** is an antibiotic used to treat bacterial infections, such as **streptococcal pharyngitis**.
- This patient's symptoms are inconsistent with a typical bacterial infection requiring penicillin and are more indicative of a **systemic inflammatory condition** like Kawasaki disease, which is not bacterial.
*Oral doxycycline*
- **Doxycycline** is an antibiotic often used for **rickettsial infections** (e.g., Rocky Mountain spotted fever) or Lyme disease, which might be considered given the camping trip.
- However, the classic constellation of symptoms (conjunctival injection, lip changes, edema of extremities, diffuse rash) points strongly away from these and towards **Kawasaki disease**, for which doxycycline is ineffective.
Question 2: A 4-year-old boy is brought to a pediatrician by his parents with a history of fever for the last 5 days and irritability, decreased appetite, vomiting, and swelling of the hands and feet for the last 3 days. The patient’s mother mentions that he has been taking antibiotics and antipyretics prescribed by another physician for the last 3 days, but there has been no improvement His temperature is 39.4°C (103.0°F), pulse is 128/min, respiratory rate is 24/min, and blood pressure is 96/64 mm Hg. On physical examination, there is significant edema of the hands and feet bilaterally. There is a 2.5 cm diameter freely moveable, nontender cervical lymph node is palpable on the right side. A strawberry tongue and perianal erythema are noted. Conjunctival injection is present bilaterally. Laboratory findings reveal mild anemia and a leukocytosis with a left-shift. Erythrocyte sedimentation rate (ESR) and serum C-reactive protein (CRP) are increased. If not treated appropriately, this patient is at increased risk of developing which of the following complications?
A. Lower gastrointestinal hemorrhage
B. Coronary artery ectasia (Correct Answer)
C. Acute renal failure
D. Pulmonary embolism
E. Acute disseminated encephalomyelitis (ADEM)
Explanation: ***Coronary artery ectasia***
- The patient's symptoms, including **prolonged fever**, **conjunctival injection**, **strawberry tongue**, **cervical lymphadenopathy**, **edema of hands and feet**, and **perianal erythema**, are characteristic of **Kawasaki disease**.
- If left untreated, **Kawasaki disease** can lead to significant cardiovascular complications, most notably **coronary artery aneurysms** or ectasia, due to vasculitis of medium-sized arteries.
*Lower gastrointestinal hemorrhage*
- While **gastrointestinal symptoms** such as vomiting and diarrhea can occur in **Kawasaki disease**, severe complications like **lower gastrointestinal hemorrhage** are rare and not the most common or critical untreated complication.
- Other conditions, like **Meckel's diverticulum** or **inflammatory bowel disease**, are more typically associated with lower GI hemorrhage in children.
*Acute renal failure*
- **Renal involvement** in **Kawasaki disease** is uncommon and usually presents as **mild proteinuria** or **sterile pyuria**; **acute renal failure** is an extremely rare complication.
- Significant renal dysfunction is more commonly seen in conditions like **hemolytic-uremic syndrome** or severe dehydration.
*Pulmonary embolism*
- **Pulmonary embolism** is rare in young children and not a typical complication of untreated **Kawasaki disease**.
- It is more commonly associated with conditions causing **hypercoagulability** or prolonged immobility.
*Acute disseminated encephalomyelitis (ADEM)*
- **ADEM** is an **immune-mediated demyelinating disorder** of the central nervous system that typically follows an infection or vaccination.
- It is not a recognized complication of untreated **Kawasaki disease**, whose primary pathology involves systemic vasculitis.
Question 3: A previously healthy 4-year-old boy is brought to the physician by his parents because he has had a fever, diffuse joint pain, and a rash on his abdomen for the past week. Acetaminophen did not improve his symptoms. He emigrated from China with his family 2 years ago. He attends daycare. His immunization records are not available. His temperature is 38.5°C (101.3°F), pulse is 125/min, and blood pressure is 100/60 mm Hg. Examination shows polymorphous truncal rash. The eyes are pink with no exudate. The tongue is shiny and red, and the lips are cracked. The hands and feet are red and swollen. There is right-sided anterior cervical lymphadenopathy. Which of the following is the most appropriate next step in management?
A. HHV-6 immunoglobulin M (IgM) detection
B. ANA measurement
C. Echocardiography (Correct Answer)
D. Monospot test
E. Antistreptolysin O titer measurement
Explanation: ***Echocardiography***
- This patient presents with classic symptoms of **Kawasaki disease**, including protracted fever (>5 days), polymorphous rash, conjunctival injection, cracked lips and red tongue (**strawberry tongue**), swollen hands and feet, and cervical lymphadenopathy.
- The diagnosis can be made clinically when fever ≥5 days plus ≥4 of 5 principal criteria are present, which this patient meets.
- **Echocardiography** is essential to establish a **baseline cardiac assessment** and screen for **coronary artery aneurysms**, which occur in 15-25% of untreated patients.
- Among the diagnostic options listed, echocardiography is the most appropriate next step, though in clinical practice, **treatment with IVIG and high-dose aspirin should be initiated promptly** (ideally within 10 days of fever onset) and should not be delayed pending echocardiography results.
*HHV-6 immunoglobulin M (IgM) detection*
- **Human Herpesvirus 6 (HHV-6)** causes **roseola infantum** (exanthem subitum), which presents with high fever for 3-5 days followed by a rash that appears **as the fever resolves**.
- The mucocutaneous findings in this case (strawberry tongue, cracked lips, conjunctival injection, extremity swelling) are not consistent with roseola.
*ANA measurement*
- **Antinuclear antibody (ANA)** testing screens for **autoimmune diseases** such as systemic lupus erythematosus or juvenile idiopathic arthritis.
- While this patient has fever and joint pain, the specific mucocutaneous changes (strawberry tongue, cracked lips, conjunctival injection, polymorphous rash, extremity edema) and cervical lymphadenopathy are pathognomonic for Kawasaki disease, not typical autoimmune conditions.
*Monospot test*
- The **Monospot test** diagnoses **infectious mononucleosis** caused by **Epstein-Barr virus (EBV)**.
- While EBV can cause fever, rash, and lymphadenopathy (typically posterior cervical), the characteristic mucocutaneous findings of Kawasaki disease (strawberry tongue, cracked lips, conjunctival injection without exudate, extremity changes) are not seen in mononucleosis.
- Additionally, mononucleosis is uncommon in preschool-aged children.
*Antistreptolysin O titer measurement*
- **Antistreptolysin O (ASO) titer** detects recent **Group A Streptococcus** infection and can support a diagnosis of **acute rheumatic fever (ARF)**.
- ARF presents with migratory polyarthritis, carditis, and **erythema marginatum** (not a polymorphous rash), occurring 2-4 weeks after streptococcal pharyngitis.
- The mucocutaneous features in this case are specific to Kawasaki disease and not consistent with ARF.
Question 4: A 4-year-old boy is brought to the emergency department by his mother with a rash on his trunk, malaise, and fever with spikes up to 38.5°C (101.3°F) for the past 2 weeks. The patient's mother says she tried giving him Tylenol with little improvement. Past medical history includes a spontaneous vaginal delivery at full term. The patient's vaccines are up-to-date and he has met all developmental milestones. On physical examination, his lips are cracking, and he has painful cervical lymphadenopathy. The rash is morbilliform and involves his trunk, palms, and the soles of his feet. There is fine desquamation of the skin of the perianal region. Which of the following anatomical structures is most important to screen for possible complications in this patient?
A. Mitral valve
B. Kidneys
C. Gallbladder
D. Coronary artery (Correct Answer)
E. Pylorus
Explanation: ***Coronary artery***
- The constellation of symptoms, including prolonged fever, rash on trunk, palms, and soles, cracked lips, cervical lymphadenopathy, and perianal desquamation, is highly indicative of **Kawasaki disease**.
- **Coronary artery aneurysms** are the most serious complication of Kawasaki disease, occurring in 15-25% of untreated children, necessitating close monitoring and screening.
*Mitral valve*
- While other forms of vasculitis or rheumatic fever can affect heart valves, **mitral valve** involvement is not a primary or characteristic complication of Kawasaki disease.
- The main cardiac concern in Kawasaki disease is direct arterial inflammation, not valvular dysfunction.
*Kidneys*
- **Renal involvement**, such as acute kidney injury, is not a typical or prominent feature of Kawasaki disease.
- Kawasaki disease primarily targets medium-sized muscular arteries throughout the body, with a predilection for the coronary arteries.
*Gallbladder*
- **Hydrops of the gallbladder** can occur in Kawasaki disease, leading to acute cholecystitis-like symptoms, but it is generally a self-limiting complication.
- While it's a potential finding, it is not as life-threatening or essential to screen for as coronary artery complications.
*Pylorus*
- There is no direct association between Kawasaki disease and primary involvement or complications of the **pylorus**.
- Gastrointestinal symptoms can occur, but these are typically non-specific and do not involve anatomical changes to the pylorus.
Question 5: A previously healthy 3-year-old girl is brought to the physician by her parents for the evaluation of recurrent episodes of pain in her left wrist and right knee for 4 months. She has not had any trauma to the knee or any previous problems with her joints. She attends daycare. Her mother has rheumatoid arthritis. Her vital signs are within normal limits. Examination of the right knee and left wrist shows mild swelling, tenderness, warmth, and erythema; range of motion is slightly decreased. No other joints are affected. The remainder of the examination shows no abnormalities. Laboratory studies show:
Hemoglobin 12.4 g/dL
Leukocyte count 9,000/mm3
Platelet count 200,000/mm3
Erythrocyte sedimentation rate 50 mm/h
Serum
Antinuclear antibodies 1:320
Rheumatoid factor negative
This patient is at increased risk for which of the following complications?
A. Airway obstruction
B. Inflammation of sacroiliac joints
C. Coronary artery aneurysm
D. Blindness (Correct Answer)
E. Valvular lesion
Explanation: ***Blindness***
- This patient presents with symptoms highly suggestive of **oligoarticular juvenile idiopathic arthritis (JIA)**, characterized by arthritis in fewer than five joints during the first six months of the disease and a **positive ANA**.
- Children with oligoarticular JIA, especially those with positive ANAs, are at high risk for developing **chronic anterior uveitis**, which can lead to **vision loss** or **blindness** if not recognized and treated early.
*Airway obstruction*
- Airway obstruction, such as due to **laryngeal involvement**, can occur in systemic JIA, but is less characteristic of **oligoarticular JIA**.
- While JIA can affect various organs, this particular complication is not typically associated with the features presented here.
*Inflammation of sacroiliac joints*
- Inflammation of the **sacroiliac joints** (sacroiliitis) is a hallmark of **spondyloarthritis** (formerly called enthesitis-related JIA), which typically presents in older boys and is associated with **HLA-B27 positivity**.
- The patient's age, gender, and presentation (oligoarticular, ANA positive) do not align with enthesitis-related JIA.
*Coronary artery aneurysm*
- **Coronary artery aneurysms** are a serious complication primarily associated with **Kawasaki disease**, a systemic vasculitis.
- The clinical picture of joint pain and swelling, chronic inflammation, and positive ANA does not fit the diagnostic criteria for Kawasaki disease.
*Valvular lesion*
- **Valvular lesions** (e.g., aortic regurgitation) can be seen in some forms of JIA, particularly in **systemic JIA** or enthesitis-related JIA.
- However, it is not the most common or highest-risk complication for a young girl with oligoarticular, ANA-positive JIA; **uveitis** is significantly more prevalent.
Question 6: A 4-year-old boy is brought to the emergency department by his parents after 10 days of fever, varying from 38.0–40.0°C (100.4–104.0°F). On physical examination, the child is ill-looking with an extensive rash over his trunk with patchy desquamation. His hands are swollen, and he also shows signs of a bilateral conjunctivitis. The laboratory test results are as follows:
Hemoglobin 12.9 g/dL
Hematocrit 37.7%
Mean corpuscular volume 82.2 μm3
Leukocyte count 10,500/mm3
Neutrophils 65%
Lymphocytes 30%
Monocytes 5%
Platelet count 290,000/mm3
Erythrocyte sedimentation rate (ESR) 35 mm/h
What is the next best step in the management of this patient’s condition?
A. High-dose aspirin (Correct Answer)
B. Influenza vaccine
C. Echocardiography
D. Low-dose aspirin
E. Corticosteroids
Explanation: ***High-dose aspirin***
- This patient presents with classic **Kawasaki disease**: prolonged fever (10 days), bilateral conjunctivitis, rash with desquamation, and extremity changes (swollen hands).
- Standard treatment for Kawasaki disease includes **IVIG (2 g/kg) plus high-dose aspirin (80-100 mg/kg/day)**. While both should be given together, **high-dose aspirin** is the best *therapeutic* option among those listed.
- High-dose aspirin provides anti-inflammatory effects during the acute phase and helps reduce fever and systemic inflammation.
- Treatment should be initiated promptly (ideally within 10 days of fever onset) to reduce the risk of **coronary artery aneurysms**.
*Influenza vaccine*
- The influenza vaccine is not a treatment for acute illness and has no role in managing Kawasaki disease.
- Vaccination would not address the ongoing systemic inflammation or prevent cardiac complications.
*Echocardiography*
- **Echocardiography should be performed** in all cases of Kawasaki disease to assess for coronary artery abnormalities, both at diagnosis and during follow-up.
- However, it is a **diagnostic/monitoring tool**, not a therapeutic intervention. Medical treatment to reduce inflammation takes priority over imaging.
- The question asks for the "next best step in management," which implies therapeutic action rather than diagnostic testing.
*Low-dose aspirin*
- Low-dose aspirin (3-5 mg/kg/day) is used during the **convalescent phase** for its antiplatelet effects, typically after fever resolution.
- It is transitioned to after the acute inflammatory phase is controlled with high-dose aspirin.
- Not appropriate for initial acute management where anti-inflammatory dosing is needed.
*Corticosteroids*
- Corticosteroids are reserved for **IVIG-refractory cases** or patients with severe coronary artery involvement.
- They are not part of initial first-line therapy and should not be used before IVIG administration.
- Their use is indicated only when standard therapy fails.
Question 7: A 9-year-old boy is brought to the emergency department because of progressively worsening shortness of breath for 3 days. He has had fever and malaise for the past 5 days. He had a sore throat 3 weeks ago that resolved without treatment. He appears ill. His temperature is 38.6°C (101.5°F), pulse is 98/min and blood pressure is 84/62 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 93%. Examination shows jugular venous distension and bilateral ankle edema. There are erythematous, ring-shaped macules and patches over his trunk that are well-demarcated. Auscultation of the chest shows crackles at the lung bases bilaterally. An S3 is heard on cardiac auscultation. His hemoglobin concentration is 12.2 g/dL, leukocyte count is 13,600/mm3, and platelet count is 280,000/mm3. A urinalysis is normal. An x-ray of the chest shows cardiac silhouette enlargement with prominent vascular markings in both the lung fields. Which of the following is the most likely etiology of this patient's symptoms?
A. Systemic lupus erythematosus
B. Acute rheumatic fever (Correct Answer)
C. Kawasaki disease
D. Viral myocarditis
E. Infection with Borrelia burgdorferi
Explanation: ***Acute rheumatic fever***
- This patient presents with signs of cardiac involvement (**shortness of breath**, **S3 gallop**, **cardiomegaly**, **pulmonary edema**), a history of a recent **strep throat infection**, and **erythema marginatum** (the ring-shaped rash), all consistent with **acute rheumatic fever**.
- **Acute rheumatic fever** is a delayed, non-suppurative complication of Group A Streptococcus pharyngitis, leading to systemic inflammation, particularly affecting the heart, joints, brain, and skin.
*Systemic lupus erythematosus*
- SLE can cause cardiac complications like pericarditis or myocarditis, but the characteristic rash of SLE (malar or discoid) differs from the described **erythema marginatum**.
- While SLE can cause systemic symptoms, the clear history of a preceding **sore throat** and the specific rash point away from SLE.
*Kawasaki disease*
- Kawasaki disease primarily affects **children under 5 years old** and is characterized by fever, conjunctivitis, oral changes, rash, lymphadenopathy, and extreme irritability.
- The patient's age (9 years) and the specific rash described (erythema marginatum) are not typical for Kawasaki disease.
*Viral myocarditis*
- Viral myocarditis can cause **heart failure** symptoms (shortness of breath, S3, cardiomegaly), but it typically lacks the preceding **sore throat** and the specific skin findings of **erythema marginatum** seen in this patient.
- While a viral infection could cause myocarditis, the constellation of symptoms points strongly to a specific post-streptococcal etiology.
*Infection with Borrelia burgdorferi*
- *Borrelia burgdorferi* (Lyme disease) can cause cardiac manifestations like **carditis** (often AV block) and a characteristic rash called **erythema migrans**.
- However, **erythema migrans** is typically a single, expanding bull's-eye rash, which is different from the described **erythematous, ring-shaped macules and patches** of erythema marginatum.
Question 8: A 5-year-old girl is brought to the emergency department by her father due to a 6-day history of fevers and irritability. His father reports that the fevers have ranged from 101-104°F (38.3-40°C). He tried to give her ibuprofen, but the fevers have been unresponsive. Additionally, she developed a rash 3 days ago and has refused to wear shoes because they feel "tight." Her father reports that other than 2 ear infections she had when she was younger, the patient has been healthy. She is up-to-date on her vaccinations except for the vaccine boosters scheduled for ages 4-6. The patient's temperature is 103.5°F (39.7°C), blood pressure is 110/67 mmHg, pulse is 115/min, and respirations are 19/min with an oxygen saturation of 98% O2 on room air. Physical examination shows bilateral conjunctivitis, palpable cervical lymph nodes, a diffuse morbilliform rash, and desquamation of the palms and soles with swollen hands and feet. Which of the following is the next step in management?
A. Acetaminophen
B. High dose aspirin
C. IVIG and high-dose aspirin (Correct Answer)
D. Nafcillin
E. Penicillin V
Explanation: ***IVIG and high-dose aspirin***
- The patient's symptoms (prolonged fever >5 days, bilateral conjunctivitis, diffuse rash, extremity changes with edema and desquamation, cervical lymphadenopathy) meet diagnostic criteria for **Kawasaki disease**.
- **IVIG (Intravenous Immunoglobulin)** is the PRIMARY treatment and must be given within 10 days of fever onset to reduce the risk of **coronary artery aneurysms** from 25% to <5%.
- **High-dose aspirin** is given as ADJUNCTIVE therapy for its anti-inflammatory effects, then transitioned to low-dose aspirin for antiplatelet effects once fever resolves.
- This combination is the **standard of care** and should be initiated as soon as the diagnosis is suspected.
*High dose aspirin*
- While aspirin is an essential component of Kawasaki disease treatment, it should **not be given as monotherapy**.
- Aspirin alone without **IVIG** is insufficient and fails to adequately reduce the risk of coronary complications.
- The question asks for the "next step in management," which requires **both IVIG and aspirin** together.
*Acetaminophen*
- Acetaminophen is an antipyretic but lacks the anti-inflammatory effects needed for **Kawasaki disease**.
- The patient's fever has already been unresponsive to **ibuprofen**, indicating that simple antipyretics are inadequate.
- It has no role in preventing the vascular complications of Kawasaki disease.
*Nafcillin*
- **Nafcillin** is an antibiotic for bacterial infections, particularly *Staphylococcus aureus*.
- **Kawasaki disease** is a vasculitis of unknown etiology (possibly post-infectious immune response) and is **not treated with antibiotics**.
- The clinical presentation does not suggest an acute bacterial infection requiring antibiotic therapy.
*Penicillin V*
- **Penicillin V** is used for streptococcal infections such as strep pharyngitis.
- The constellation of findings (persistent fever, conjunctivitis, rash, extremity changes, lymphadenopathy) is classic for **Kawasaki disease**, not a bacterial infection.
- Antibiotics have no role in the management of Kawasaki disease.
Question 9: A 4-year-old male is brought to the pediatrician for a low-grade fever. His mother states that he has had a waxing and waning fever for the past 6 days with temperatures ranging from 99.8°F (37.7°C) to 101.0°F (38.3°C). She reports that he had a similar episode three months ago. She also reports symmetric joint swelling in the child’s knees and wrists that has become increasingly noticeable over the past 8 weeks. He has not had a cough, difficulty breathing, or change in his bowel movements. The child was born at 40 weeks gestation. His height and weight are in the 45th and 40th percentiles, respectively. He takes no medications. His temperature is 100.1°F (37.8°C), blood pressure is 100/65 mmHg, pulse is 105/min, and respirations are 18/min. On examination, there is a non-pruritic, macular, salmon-colored truncal rash. Serological examination reveals the following:
Serum:
Rheumatoid factor: Negative
Anti-nuclear antibody: Negative
Anti-double stranded DNA: Negative
Anti-SSA: Negative
Anti-SSB: Negative
Human leukocyte antigen B27: Positive
Erythrocyte sedimentation rate: 30 mm/h
This patient is most likely at increased risk of developing which of the following?
A. Dactylitis
B. Aortitis
C. Sacroiliitis (Correct Answer)
D. Scoliosis
E. Iridocyclitis
Explanation: ***Sacroiliitis***
- The patient presents with classic symptoms of **systemic juvenile idiopathic arthritis (sJIA)**, including **waxing and waning fever**, symmetric polyarthritis, and a **salmon-colored truncal rash**. The positive **HLA-B27** in a child with these findings significantly increases the risk of developing **spondyloarthropathy-related complications**, such as sacroiliitis.
- While sJIA itself doesn't directly cause sacroiliitis, a subset of patients, especially those with **HLA-B27 positivity**, may develop features of **spondyloarthritis** over time, with sacroiliitis being a hallmark.
*Dactylitis*
- **Dactylitis** (sausage digits) is a characteristic feature of **psoriatic arthritis** and some **spondyloarthropathies**, but it is not a direct complication of sJIA.
- Psoriatic arthritis typically presents with skin lesions and often affects older children or adults.
*Aortitis*
- **Aortitis** is a rare but serious complication, primarily associated with **Takayasu arteritis** or other large vessel vasculitides, not typically seen in sJIA.
- Systemic JIA can cause **pericarditis** or **myocarditis**, but isolated aortitis is not a common long-term risk.
*Scoliosis*
- **Scoliosis** is an abnormal curvature of the spine and can be idiopathic or secondary to neuromuscular conditions or vertebral anomalies.
- While chronic arthritis and inflammation can rarely alter spinal growth, it is not a direct or highly specific complication of sJIA, particularly in comparison to the increased risk of sacroiliitis in an HLA-B27 positive individual.
*Iridocyclitis*
- **Chronic anterior uveitis (iridocyclitis)** is a significant complication of **oligoarticular JIA** and, less commonly, polyarticular JIA; however, it is relatively rare in active **sJIA** itself.
- The symptoms in this case point to sJIA, and while eye involvement can occur, sacroiliitis is a more specific and expected long-term complication given the HLA-B27 positivity and systemic inflammatory picture.
Question 10: A 4-year-old presents with fever, rash, and red, cracked lips. Physical exam reveals cervical lymphadenopathy. Which of the following disease processes is most likely?
A. Kawasaki disease (Correct Answer)
B. Polyarteritis nodosa
C. Buerger's disease
D. Infectious vasculitis
E. Temporal arteritis
Explanation: ***Correct Option: Kawasaki disease***
- This presentation is classic for **Kawasaki disease**, characterized by fever, rash, **red cracked lips** (**strawberry tongue**), and **cervical lymphadenopathy** in a young child.
- It is an acute systemic vasculitis affecting medium-sized arteries, and the primary concern is the potential for **coronary artery aneurysms**.
*Incorrect Option: Polyarteritis nodosa*
- This is a systemic necrotizing vasculitis that typically affects **adults**, with rare incidence in childhood.
- While it can present with fever and rash, the specific mucocutaneous and lymph node findings seen here are not typical for polyarteritis nodosa.
*Incorrect Option: Buerger's disease*
- This is a segmental, inflammatory, thrombosing vasculitis that primarily affects **small and medium-sized arteries and veins** in the limbs.
- It is strongly associated with **tobacco use** and causes symptoms like intermittent claudication, Raynaud's phenomenon, and digital ischemia, which are not present in this child.
*Incorrect Option: Infectious vasculitis*
- While infections can cause vasculitis, the specific constellation of symptoms (**red cracked lips**, **strawberry tongue**, **cervical lymphadenopathy**, and rash) points more specifically to Kawasaki disease than a general infectious vasculitis.
- This is a broad category, and Kawasaki disease is a more specific and likely diagnosis given the findings in a child.
*Incorrect Option: Temporal arteritis*
- This is a vasculitis primarily affecting the **large arteries of the head and neck**, including the temporal artery.
- It almost exclusively affects **older adults** (typically over 50 years old) and presents with symptoms like headache, jaw claudication, and vision changes, which are not present in this 4-year-old.
Question 11: A 3-year-old boy is taken to the ER by his parents due to his elevated temperature. He has had a fever (>101.1 deg F) for a little over a week, and over that time, his parents noticed his eyes had gotten a little pink, and his palms and soles were red and swollen. His lips and tongue are also peeling. His parents note he has not taken any new medications, and they did not notice any runny nose, sore throat, cough, or changes in his bowel or bladder habits. In the ER, his vitals are as follows: temperature is 101.3 deg F (38.5 deg C), blood pressure is 90/60 mmHg, pulse is 125/min, and respirations are 20/min. His exam is notable for bilateral injected conjunctivae, right-sided cervical lymphadenopathy, erythematous and edematous palms and soles, and erythema multiforme-like rash over his trunk. Appropriate lab tests and imaging were performed. Which of the following is the most worrisome complication of this boy's disease process?
A. Endocardial valve damage
B. Toxic epidermal necrolysis
C. Digital gangrene
D. Coronary artery aneurysms (Correct Answer)
E. Glomerulonephritis
Explanation: ***Coronary artery aneurysms***
- The constellation of symptoms (prolonged fever, bilateral injected conjunctivae, swollen and red palms/soles, peeling lips/tongue, cervical lymphadenopathy, rash) in a 3-year-old boy is highly suggestive of **Kawasaki disease (KD)**.
- **Coronary artery aneurysms** are the most serious and common cardiovascular complication of KD, leading to potential myocardial infarction, sudden death, or long-term cardiac issues if not properly treated.
*Endocardial valve damage*
- While KD can affect the heart, **endocardial valve damage** leading to significant valvulopathy is a less frequent and less specific complication compared to coronary artery involvement.
- Valvular issues, if present, are often transient and mild, unlike the potentially devastating and long-term consequences of coronary aneurysms.
*Toxic endodermal necrolysis*
- **Toxic epidermal necrolysis (TEN)** is a severe mucocutaneous reaction, usually triggered by medications, characterized by widespread epidermal detachment.
- The rash described in the patient (erythema multiforme-like) is distinct from the widespread blistering and skin detachment seen in TEN, and KD is not associated with TEN.
*Digital gangrene*
- While extreme peripheral ischemia can lead to **digital gangrene**, this is not a typical or common complication of Kawasaki disease.
- KD primarily affects medium-sized arteries, and although peripheral vascular changes can occur, gangrene is rare.
*Glomerulonephritis*
- **Glomerulonephritis** is an inflammatory kidney condition that involves damage to the glomeruli.
- While some renal manifestations like sterile pyuria can occur in KD, clinically significant widespread glomerulonephritis leading to renal failure is not a characteristic or major complication of Kawasaki disease.
