IVIG resistance US Medical PG Practice Questions and MCQs
Practice US Medical PG questions for IVIG resistance. These multiple choice questions (MCQs) cover important concepts and help you prepare for your exams.
IVIG resistance US Medical PG Question 1: A 24-year-old man presents with a complaint of breathlessness while jogging. He says that he recently started marathon training. He does not have any family history of asthma nor has any allergies. He currently takes no medication. The blood pressure is 120/80 mm Hg, and the heart rate is 67/min. With each heartbeat, he experiences pounding in his chest, and his head bobs. On physical examination, he has long fingers, funnel chest, and disproportionate body proportions with a decreased upper-to-lower segment ratio. On auscultation over the 2nd right intercostal space, an early diastolic murmur is heard, and 3rd and 4th heart sounds are heard. Echocardiography shows aortic root dilatation. The patient is scheduled for surgery. Which of the following is associated with this patient's condition?
- A. Klinefelter syndrome
- B. Intravenous drug abuse
- C. Marfan's Syndrome (Correct Answer)
- D. Kawasaki syndrome
- E. Gonorrhea
IVIG resistance Explanation: ***Marfan's Syndrome***
- The patient presents with **tall stature**, **long fingers (arachnodactyly)**, **funnel chest (pectus excavatum)**, and **aortic root dilation** with **aortic regurgitation** (early diastolic murmur, head bobbing, pounding in the chest), all classic features of Marfan syndrome.
- This is a **connective tissue disorder** caused by a mutation in the **FBN1 gene**, leading to defective **fibrillin-1**, which is crucial for structural integrity in the heart, blood vessels, eyes, and skeleton.
*Klinefelter syndrome*
- Characterized by a **47, XXY karyotype** and typically presents with infertility, small testes, gynecomastia, and tall stature, but not the specific cardiovascular or skeletal features described.
- While it can cause tall stature, it does not explain the **arachnodactyly**, **pectus excavatum**, or the severe **aortic root dilation** and regurgitation.
*Intravenous drug abuse*
- Primarily associated with **infective endocarditis**, particularly affecting the **tricuspid valve**, leading to heart murmurs related to infection, not the skeletal and aortic root abnormalities seen here.
- This history would lead to a different clinical presentation, potentially involving fever, chills, and vegetations on valve leaflets, none of which are mentioned.
*Kawasaki syndrome*
- An **acute inflammatory vasculitis** primarily affecting young children, characterized by fever, rash, conjunctivitis, lymphadenopathy, and oral mucosal changes.
- While it can cause **coronary artery aneurysms**, it does not explain the skeletal abnormalities or the specific presentation of aortic root dilation with regurgitation in an adult.
*Gonorrhea*
- A **sexually transmitted infection** that can lead to disseminated gonococcal infection, causing arthritis, tenosynovitis, and dermatitis.
- It does not cause the specific skeletal abnormalities or the primary cardiac pathology of aortic root dilation and regurgitation described in this patient.
IVIG resistance US Medical PG Question 2: 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
IVIG resistance 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.
IVIG resistance US Medical PG Question 3: A 53-year-old woman comes to the physician in February because of a 1-day history of fever, chills, headache, and dry cough. She also reports malaise and generalized muscle aches. She works as a teacher at a local high school, where there was recently an outbreak of influenza. She has a history of intermittent asthma, for which she takes albuterol as needed. She declined the influenza vaccine offered in the fall because her sister told her that a friend developed a flulike illness after receiving the vaccine. She is worried about possibly becoming ill and cannot afford to miss work. Her temperature is 37.9°C (100.3°F), heart rate is 58/min, and her respirations are 12/min. Physical examination is unremarkable. Her hemoglobin concentration is 14.5 g/dL, leukocyte count is 9,400/mm3, and platelet count is 280,000/mm3. In addition to analgesia, which of the following is the most appropriate next step in management?
- A. Inactivated influenza vaccine
- B. Amantadine
- C. Live attenuated influenza vaccine
- D. Oseltamivir (Correct Answer)
- E. Supportive therapy only
IVIG resistance Explanation: ***Oseltamivir***
- This patient presents with classic symptoms of **influenza** (fever, chills, headache, dry cough, malaise, myalgias) during an outbreak, making **antiviral therapy** like oseltamivir appropriate.
- She is at risk for complications due to her history of **asthma**, and early treatment (within 48 hours of symptom onset) can reduce illness severity and duration.
*Inactivated influenza vaccine*
- An **inactivated influenza vaccine** is a **preventive measure** and is not effective as a treatment once symptoms have already begun.
- Vaccination in the past fall would have been appropriate, but it will not help resolve her current acute illness.
*Amantadine*
- **Amantadine** is an older antiviral agent active only against **influenza A**, and its use is limited due to widespread **resistance**.
- It is generally not recommended for routine influenza treatment due to its narrow spectrum and resistance profile.
*Live attenuated influenza vaccine*
- The **live attenuated influenza vaccine (LAIV)** is a **preventive measure** indicated for healthy individuals aged 2-49 years and is contraindicated in individuals with **asthma**.
- Like the inactivated vaccine, it is not used for treating active influenza infection.
*Supportive therapy only*
- While supportive care (analgesia, hydration) is important, relying solely on it is not the most appropriate step given the patient's **risk factors** (asthma) and the availability of effective antiviral treatment.
- Early antiviral therapy can reduce serious complications in at-risk individuals.
IVIG resistance US Medical PG Question 4: 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
IVIG resistance 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.
IVIG resistance US Medical PG Question 5: 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
IVIG resistance 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.
IVIG resistance US Medical PG Question 6: 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)
IVIG resistance 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.
IVIG resistance US Medical PG Question 7: 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
IVIG resistance 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.
IVIG resistance US Medical PG Question 8: 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
IVIG resistance 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.
IVIG resistance US Medical PG Question 9: 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
IVIG resistance 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.
More IVIG resistance US Medical PG questions available in the OnCourse app. Practice MCQs, flashcards, and get detailed explanations.
