A 43-year-old man presents to the emergency department following a work-related accident in which both arms were amputated. The patient lost a substantial amount of blood prior to arrival, and his bleeding is difficult to control due to arterial damage and wound contamination with debris. His complete blood count (CBC) is significant for a hemoglobin (Hgb) level of 5.3 g/dL. The trauma surgery resident initiates the massive transfusion protocol and orders whole blood, O negative, which she explains is the universal donor. The patient receives 6 units of O negative blood prior to admission. He subsequently develops fever, chills, hematuria, and pulmonary edema. Several hours later, the patient goes into hemodynamic shock requiring the emergent administration of vasopressors. Of the following options, which hypersensitivity reaction occurred?
Q102
A 37-year-old man presents to an urgent care clinic with complaints of speech problems and yellowing of his eyes for a week. He admits to using illicit intravenous drugs. His vital signs include: blood pressure 110/60 mm Hg, pulse rate 78/min, and respiratory rate 22/min. On examination, the patient appears jaundiced, and his speech is slurred. His liver enzymes and viral markers are as follows:
Aspartate aminotransferase 6,700 IU/L
Alanine aminotransferase 5,000 IU/L
HBsAg Negative
Anti-HBs Negative
Anti-HCV Ab Positive
HCV RNA Positive
If this patient develops chronic hepatitis C infection, he is at risk of developing a secondary dermatological condition. A biopsy of this skin condition would most likely show which of the following findings?
Q103
A 36-year-old man comes to the physician for a 4-week history of swollen legs. He has difficulty putting on socks because of the swelling. Two years ago, he was diagnosed with sleep apnea. He takes no medications. He emigrated from Guatemala with his family when he was a child. He is 171 cm (5 ft 6 in) tall and weighs 115 kg (253 lb); BMI is 39 kg/m2. His pulse is 91/min and blood pressure is 135/82 mm Hg. Examination shows periorbital and bilateral lower extremity edema.
Serum
Albumin 3.1 g/dL
Total cholesterol 312 mg/dL
Urine
Blood negative
Protein +4
RBC 1-2/hpf
RBC cast negative
Fatty casts numerous
A renal biopsy is obtained. Which of the following is most likely to be seen under light microscopy of the patient's renal biopsy specimen?
Q104
A 15-year-old African American boy presents to a pediatrician with complaints of yellow discoloration of the sclerae for the last 3 days. His mother informs the pediatrician that the boy developed prolonged jaundice during the neonatal period. On physical examination, vital signs are stable and general examination shows mild icterus and pallor. Examination of the abdomen suggests mild splenomegaly. Laboratory results are as follows:
Hemoglobin 9.9 g/dL
Total leukocyte count 7,500/mm3
Platelet count 320,000/mm3
Reticulocyte count 5%
Mean corpuscular hemoglobin 27.7 pg/cell
Mean corpuscular hemoglobin concentration 32% g/dL
Mean corpuscular volume 84 μm3
Serum total bilirubin 4.2 mg/dL
Serum direct bilirubin 0.3 mg/dL
Coombs test Negative
Peripheral smear shows polychromasia, blister cells, and Heinz bodies. An abdominal ultrasonogram shows the presence of gallstones. Which of the following tests is most likely to be useful in diagnosing this patient?
Q105
A 38-year-old woman presents to a physician’s office for progressive weakness and pallor during the last few weeks. She also complains of shortness of breath during her yoga class. She denies fevers, cough, rhinorrhea, or changes in appetite or bowel and bladder habits. She is generally healthy except for an occasional migraine, which is relieved by acetaminophen. For the last month, she has been having more frequent migraine attacks and was started on prophylactic aspirin. The vital signs include: pulse 102/min, respirations 18/min, and blood pressure 130/84 mm Hg. Her blood pressure on previous visits has been 110/76 mm Hg, 120/78 mm Hg, and 114/80 mm Hg. The physical examination is otherwise unremarkable. Stool for occult blood is positive. In addition to a low hemoglobin concentration, which other laboratory finding is expected in this patient?
Q106
A 72-year-old nursing home resident is complaining of pruritis. She is noted to have multiple, tense blisters on her trunk as well as the flexor surfaces of her extremities. The blisters have an erythematous base. You are unable to extend the blisters when you apply lateral traction. You suspect an autoimmune bullous dermatosis. Which of the following is the cause of the likely condition?
Q107
A 53-year-old woman presents to the emergency room with severe chest pain radiating to the back. She was diagnosed with acute aortic dissection. A few hours into the resuscitation, she was having oliguria. Laboratory findings show a serum creatinine level of 5.3 mg/dL. Which of the following casts are most likely to be seen on urinalysis?
Q108
A 9-year-old boy is brought to the emergency department by his parents with a 2-day history of weakness and joint pain. He was adopted 3 weeks ago from an international adoption agency and this is his first week in the United States. He says that he has been healthy and that he had an episode of sore throat shortly before his adoption. Physical exam reveals an ill-appearing boy with a fever, widespread flat red rash, and multiple subcutaneous nodules. The type of hypersensitivity seen in this patient's disease is also characteristic of which of the following diseases?
Q109
A 73-year-old man presents to his primary care doctor with his son who reports that his father has been acting strangely. He has started staring into space throughout the day and has a limited attention span. He has been found talking to people who are not present and has gotten lost while driving twice. He has occasional urinary incontinence. His past medical history is notable for a stroke 5 years ago with residual right arm weakness, diabetes, hypertension, and hyperlipidemia. He takes aspirin, glyburide, metformin, lisinopril, hydrochlorothiazide, and atorvastatin. On examination, he is oriented to person and place but thinks the year is 1989. He is inattentive throughout the exam. He takes short steps while walking. His movements are grossly slowed. A brain biopsy in this patient would most likely reveal which of the following?
Q110
A 4-year-old girl is brought to the physician because of increasing swelling around her eyes and over both her feet for the past 4 days. During this period, she has had frothy light yellow urine. Her vital signs are within normal limits. Physical examination shows periorbital edema and 2+ pitting edema of the lower legs and ankles. A urinalysis of this patient is most likely to show which of the following findings?
Systemic Pathology US Medical PG Practice Questions and MCQs
Question 101: A 43-year-old man presents to the emergency department following a work-related accident in which both arms were amputated. The patient lost a substantial amount of blood prior to arrival, and his bleeding is difficult to control due to arterial damage and wound contamination with debris. His complete blood count (CBC) is significant for a hemoglobin (Hgb) level of 5.3 g/dL. The trauma surgery resident initiates the massive transfusion protocol and orders whole blood, O negative, which she explains is the universal donor. The patient receives 6 units of O negative blood prior to admission. He subsequently develops fever, chills, hematuria, and pulmonary edema. Several hours later, the patient goes into hemodynamic shock requiring the emergent administration of vasopressors. Of the following options, which hypersensitivity reaction occurred?
A. Type 1 hypersensitivity reaction
B. Combined type 1 and type 4 hypersensitivity reaction
C. Type 3 hypersensitivity reaction
D. Type 2 hypersensitivity reaction (Correct Answer)
E. Type 4 hypersensitivity reaction
Explanation: ***Type 2 hypersensitivity reaction***
- This scenario describes an **acute hemolytic transfusion reaction (AHTR)**, a classic example of a **Type II hypersensitivity reaction**. The recipient's antibodies (IgM) recognize and bind to antigens on the transfused red blood cells, leading to their destruction (hemolysis) via complement activation and cellular mechanisms.
- Symptoms like **fever, chills, hematuria (due to hemoglobinuria)**, and subsequent **shock** are characteristic of AHTR, even with O negative blood if other minor blood group antigens (e.g., Kell, Duffy) are incompatible or if the patient developed antibodies against these from previous transfusions or pregnancies.
*Type 1 hypersensitivity reaction*
- This type involves **IgE-mediated mast cell degranulation** and is associated with allergic reactions such as anaphylaxis, asthma, and hives.
- While anaphylaxis can cause shock, the systemic symptoms of **hemolysis and hematuria** are not characteristic of a Type 1 reaction.
*Combined type 1 and type 4 hypersensitivity reaction*
- This combination is uncommon in an acute transfusion setting and does not align with the presented symptoms.
- Type 1 is immediate allergic, and Type 4 is delayed cell-mediated, neither fully explaining the hemolytic features observed.
*Type 3 hypersensitivity reaction*
- This reaction involves the formation of **immune complexes** (antigen-antibody complexes) that deposit in tissues, leading to inflammation and damage (e.g., serum sickness, lupus nephritis).
- While immune complexes can cause systemic symptoms, the prominent hemolytic features and immediate presentation of a transfusion reaction are more indicative of Type 2.
*Type 4 hypersensitivity reaction*
- This is a **delayed type hypersensitivity** reaction mediated by **T cells**, taking 24-72 hours or longer to develop (e.g., contact dermatitis, tuberculin skin test).
- The acute onset of symptoms following transfusion makes a Type 4 reaction highly unlikely.
Question 102: A 37-year-old man presents to an urgent care clinic with complaints of speech problems and yellowing of his eyes for a week. He admits to using illicit intravenous drugs. His vital signs include: blood pressure 110/60 mm Hg, pulse rate 78/min, and respiratory rate 22/min. On examination, the patient appears jaundiced, and his speech is slurred. His liver enzymes and viral markers are as follows:
Aspartate aminotransferase 6,700 IU/L
Alanine aminotransferase 5,000 IU/L
HBsAg Negative
Anti-HBs Negative
Anti-HCV Ab Positive
HCV RNA Positive
If this patient develops chronic hepatitis C infection, he is at risk of developing a secondary dermatological condition. A biopsy of this skin condition would most likely show which of the following findings?
A. Intraepithelial cleavage with acantholysis
B. Noncaseating granulomas
C. Microabscesses with fibrin and neutrophils
D. Crypt abscesses containing neutrophils
E. Lymphocytic infiltrate at the dermal-epidermal junction (Correct Answer)
Explanation: ***Lymphocytic infiltrate at the dermal-epidermal junction***
- Chronic hepatitis C infection is strongly associated with **lichen planus**, a dermatological condition characterized histologically by a **lymphocytic infiltrate along the dermal-epidermal junction**, often described as a "sawtooth" pattern.
- The history of IV drug use, jaundiced eyes, elevated liver enzymes, and positive HCV RNA confirm a diagnosis of **acute hepatitis C**, which can progress to chronic infection and lead to extrahepatic manifestations like lichen planus.
*Intraepithelial cleavage with acantholysis*
- This finding is characteristic of **pemphigus vulgaris**, an autoimmune blistering disorder where antibodies target desmoglein 3, leading to loss of cohesion between keratinocytes.
- Pemphigus vulgaris is not typically associated with chronic hepatitis C infection.
*Noncaseating granulomas*
- **Noncaseating granulomas** are the hallmark histological feature of **sarcoidosis** and Crohn's disease.
- While sarcoidosis can have cutaneous manifestations, it is not directly linked to chronic hepatitis C in the way lichen planus is.
*Microabscesses with fibrin and neutrophils*
- This description aligns with findings in conditions such as **dermatitis herpetiformis** (neutrophilic infiltrates in dermal papillae) or acute neutrophilic dermatoses, but it is not the characteristic feature of skin conditions directly associated with chronic hepatitis C.
- Skin conditions like vasculitis can also show neutrophilic infiltrates, but the primary association with HCV and the provided options points away from this.
*Crypt abscesses containing neutrophils*
- **Crypt abscesses** are histological features typically found in the intestinal lining in conditions like **ulcerative colitis**, an inflammatory bowel disease.
- This finding is not relevant to dermatological conditions associated with chronic hepatitis C.
Question 103: A 36-year-old man comes to the physician for a 4-week history of swollen legs. He has difficulty putting on socks because of the swelling. Two years ago, he was diagnosed with sleep apnea. He takes no medications. He emigrated from Guatemala with his family when he was a child. He is 171 cm (5 ft 6 in) tall and weighs 115 kg (253 lb); BMI is 39 kg/m2. His pulse is 91/min and blood pressure is 135/82 mm Hg. Examination shows periorbital and bilateral lower extremity edema.
Serum
Albumin 3.1 g/dL
Total cholesterol 312 mg/dL
Urine
Blood negative
Protein +4
RBC 1-2/hpf
RBC cast negative
Fatty casts numerous
A renal biopsy is obtained. Which of the following is most likely to be seen under light microscopy of the patient's renal biopsy specimen?
A. Diffuse thickening of glomerular capillaries
B. Amyloid deposition in the mesangium
C. Eosinophilic nodules within the glomeruli
D. Fibrin crescents within the glomerular space
E. Segmental sclerosis of the glomeruli (Correct Answer)
Explanation: ***Segmental sclerosis of the glomeruli***
- The patient presents with **nephrotic syndrome** (edema, proteinuria, hypoalbuminemia, hypercholesterolemia) and **obesity** (BMI 39 kg/m2), which are strong risk factors for **focal segmental glomerulosclerosis (FSGS)**.
- **FSGS** is characterized by **segmental sclerosis** within some glomeruli, often affecting juxtamedullary glomeruli initially.
*Diffuse thickening of glomerular capillaries*
- **Diffuse thickening of glomerular capillaries** (due to subepithelial immune complex deposition causing SPIKES on silver stain) is characteristic of **membranous nephropathy**, which typically presents with nephrotic syndrome but is not directly linked to obesity in the way FSGS is.
- While membranous nephropathy could cause nephrotic syndrome, the association with **morbid obesity** makes FSGS a stronger consideration.
*Amyloid deposition in the mesangium*
- **Amyloid deposition** typically presents with nephrotic syndrome and is associated with chronic inflammatory conditions or plasma cell dyscrasias, not primarily with obesity or the specific presentation described.
- On light microscopy, amyloid appears as **acellular, eosinophilic congophilic deposits** in the mesangium and capillary walls, showing apple-green birefringence under polarized light, which is not suggested by the clinical picture.
*Eosinophilic nodules within the glomeruli*
- **Eosinophilic nodules within the glomeruli** (Kimmelstiel-Wilson lesions) are characteristic of **diabetic nephropathy**, which commonly causes nephrotic syndrome.
- While the patient is obese, there is no information about diabetes or hyperglycemia to suggest diabetic nephropathy as the primary cause.
*Fibrin crescents within the glomerular space*
- **Fibrin crescents within the glomerular space** are indicative of **rapidly progressive glomerulonephritis (RPGN)**, which typically presents as nephritic syndrome (hematuria, hypertension, azotemia) rather than pure nephrotic syndrome.
- The patient's urine microscopic findings show only 1-2 RBC/hpf and no RBC casts, making RPGN unlikely.
Question 104: A 15-year-old African American boy presents to a pediatrician with complaints of yellow discoloration of the sclerae for the last 3 days. His mother informs the pediatrician that the boy developed prolonged jaundice during the neonatal period. On physical examination, vital signs are stable and general examination shows mild icterus and pallor. Examination of the abdomen suggests mild splenomegaly. Laboratory results are as follows:
Hemoglobin 9.9 g/dL
Total leukocyte count 7,500/mm3
Platelet count 320,000/mm3
Reticulocyte count 5%
Mean corpuscular hemoglobin 27.7 pg/cell
Mean corpuscular hemoglobin concentration 32% g/dL
Mean corpuscular volume 84 μm3
Serum total bilirubin 4.2 mg/dL
Serum direct bilirubin 0.3 mg/dL
Coombs test Negative
Peripheral smear shows polychromasia, blister cells, and Heinz bodies. An abdominal ultrasonogram shows the presence of gallstones. Which of the following tests is most likely to be useful in diagnosing this patient?
A. Hepatoiminodiacetic acid scanning
B. Serum thyroxine, triiodothyronine, and thyroid-stimulating hormone
C. Methemoglobin reduction test
D. Serum lipoprotein-X level
E. G6PD enzyme assay (Correct Answer)
Explanation: ***Correct: G6PD enzyme assay***
- The clinical presentation (jaundice, pallor, splenomegaly, gallstones, neonatal jaundice history) along with laboratory findings (anemia, elevated reticulocyte count, indirect hyperbilirubinemia, negative Coombs test, **polychromasia**, **blister cells**, and **Heinz bodies** on peripheral smear) strongly suggests **glucose-6-phosphate dehydrogenase (G6PD) deficiency**.
- **Heinz bodies** (denatured hemoglobin precipitates) and **blister cells** (bite cells from splenic removal of Heinz bodies) are **pathognomonic** for oxidative hemolysis due to G6PD deficiency.
- The **G6PD enzyme assay** is the **gold standard diagnostic test** that directly measures G6PD enzyme activity in red blood cells, confirming the diagnosis.
- Note: Testing should ideally be done after the acute hemolytic episode resolves, as reticulocytes (which have higher G6PD levels) may give falsely normal results during active hemolysis.
*Incorrect: Methemoglobin reduction test*
- This test assesses the ability of red blood cells to reduce methemoglobin and is used to diagnose **methemoglobinemia** or defects in the methemoglobin reductase system.
- While G6PD deficiency can theoretically affect methemoglobin reduction (both require NADPH), this is **not** the standard or recommended diagnostic test for G6PD deficiency.
*Incorrect: Hepatoiminodiacetic acid scanning*
- This scan (HIDA scan) evaluates **gallbladder function** and patency of the **biliary tree**, primarily for diagnosing acute cholecystitis or biliary obstruction.
- While the patient has gallstones (a complication of chronic hemolysis due to bilirubin pigment stones), the primary diagnostic need is to identify the underlying cause of hemolytic anemia.
*Incorrect: Serum thyroxine, triiodothyronine, and thyroid-stimulating hormone*
- These tests assess **thyroid function** and diagnose thyroid disorders.
- The patient's symptoms and laboratory results (hemolytic anemia with characteristic peripheral smear findings) clearly point to a hematologic disorder, not a thyroid condition.
*Incorrect: Serum lipoprotein-X level*
- **Lipoprotein-X** is an abnormal lipoprotein elevated in **cholestatic** conditions and is associated with **target cells** on peripheral smear.
- This patient has **indirect (unconjugated) hyperbilirubinemia** (total 4.2 mg/dL, direct 0.3 mg/dL), indicating hemolysis rather than cholestasis, and the peripheral smear shows blister cells and Heinz bodies, not target cells.
Question 105: A 38-year-old woman presents to a physician’s office for progressive weakness and pallor during the last few weeks. She also complains of shortness of breath during her yoga class. She denies fevers, cough, rhinorrhea, or changes in appetite or bowel and bladder habits. She is generally healthy except for an occasional migraine, which is relieved by acetaminophen. For the last month, she has been having more frequent migraine attacks and was started on prophylactic aspirin. The vital signs include: pulse 102/min, respirations 18/min, and blood pressure 130/84 mm Hg. Her blood pressure on previous visits has been 110/76 mm Hg, 120/78 mm Hg, and 114/80 mm Hg. The physical examination is otherwise unremarkable. Stool for occult blood is positive. In addition to a low hemoglobin concentration, which other laboratory finding is expected in this patient?
A. Prolonged prothrombin time (PT)
B. Decreased platelet count
C. Prolonged activated partial thromboplastin time (aPTT)
D. Elevated D-dimer
E. Prolonged bleeding time (Correct Answer)
Explanation: ***Prolonged bleeding time***
- The patient's symptoms (weakness, pallor, shortness of breath, positive stool occult blood) suggest **gastrointestinal bleeding** leading to **iron deficiency anemia**.
- **Aspirin** use can impair platelet function by irreversibly inhibiting **cyclooxygenase-1 (COX-1)**, leading to a prolonged bleeding time.
*Prolonged prothrombin time (PT)*
- **PT** measures the extrinsic and common pathways of coagulation and is prolonged in deficiencies of factors VII, X, V, II, or fibrinogen, or with **warfarin use**.
- There is no clinical information suggesting a vitamin K deficiency or liver disease that would prolong PT.
*Decreased platelet count*
- The patient's presentation does not suggest **thrombocytopenia**, which would be indicated by spontaneous bleeding, petechiae, or purpura.
- While aspirin affects platelet function, it does not typically decrease the **platelet count**.
*Prolonged activated partial thromboplastin time (aPTT)*
- **aPTT** measures the intrinsic and common pathways of coagulation and is prolonged in deficiencies of factors XII, XI, IX, VIII, X, V, II, fibrinogen, or with **heparin use**.
- There are no indications of these conditions or **heparin** administration in the patient's history.
*Elevated D-dimer*
- An **elevated D-dimer** indicates recent or ongoing fibrinolysis, often seen in conditions like deep vein thrombosis, pulmonary embolism, or **disseminated intravascular coagulation (DIC)**.
- The patient's symptoms are characteristic of chronic blood loss and anemia, not a thrombotic or hypercoagulable state.
Question 106: A 72-year-old nursing home resident is complaining of pruritis. She is noted to have multiple, tense blisters on her trunk as well as the flexor surfaces of her extremities. The blisters have an erythematous base. You are unable to extend the blisters when you apply lateral traction. You suspect an autoimmune bullous dermatosis. Which of the following is the cause of the likely condition?
A. Antibodies to epidermal transglutaminase
B. Epidermal necrolysis
C. Antibodies to hemidesmosomes (Correct Answer)
D. Antibodies to desmoglein
E. Antibodies to desmosomes
Explanation: **Antibodies to hemidesmosomes**
- The patient's presentation with **tense blisters** that do not extend with lateral traction (negative Nikolsky sign) is classic for **bullous pemphigoid**.
- **Bullous pemphigoid** is an autoimmune disease caused by antibodies targeting components of the **hemidesmosomes** (specifically BP180 and BP230) at the dermal-epidermal junction.
*Antibodies to epidermal transglutaminase*
- Antibodies against **epidermal transglutaminase** are characteristic of **dermatitis herpetiformis**, which presents with intensely pruritic, grouped vesicles and papules, often on extensor surfaces.
- This condition is also associated with **celiac disease**, and its classic lesions are small and vesicular, not the large, tense bullae described.
*Epidermal necrolysis*
- **Epidermal necrolysis**, including Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), is characterized by widespread epidermal detachment and necrosis, often triggered by medications.
- It presents with diffuse painful erythema and sheet-like skin loss, which is distinctly different from the described tense blisters of bullous pemphigoid.
*Antibodies to desmoglein*
- Antibodies to **desmoglein 1 and/or 3** (components of desmosomes) are the hallmark of **pemphigus vulgaris** and **pemphigus foliaceus**.
- These conditions cause **flaccid blisters** that are easily ruptured and often exhibit a positive Nikolsky sign, contrary to the tense blisters in this case.
*Antibodies to desmosomes*
- Antibodies to **desmosomes** (specifically desmogleins) lead to **pemphigus**, which is characterized by intraepidermal blistering and a positive Nikolsky sign.
- The described **tense blisters** and negative Nikolsky sign rule out pemphigus, where the defect is in keratinocyte adhesion within the epidermis.
Question 107: A 53-year-old woman presents to the emergency room with severe chest pain radiating to the back. She was diagnosed with acute aortic dissection. A few hours into the resuscitation, she was having oliguria. Laboratory findings show a serum creatinine level of 5.3 mg/dL. Which of the following casts are most likely to be seen on urinalysis?
A. RBC casts
B. Fatty casts
C. Muddy brown casts (Correct Answer)
D. Waxy casts
E. Hyaline casts
Explanation: ***Muddy brown casts***
- **Acute tubular necrosis (ATN)**, likely caused by **renal hypoperfusion** in the context of an aortic dissection, is characterized by the presence of **muddy brown granular casts** in urinalysis. The significantly elevated **creatinine (5.3 mg/dL)** and **oliguria** support a diagnosis of acute kidney injury with ATN.
- These casts are pathognomonic for ATN and are formed from shed **epithelial cells** and debris accumulating in the renal tubules.
*RBC casts*
- **Red blood cell (RBC) casts** are indicative of **glomerulonephritis** or other causes of **glomerular injury**, which are not directly suggested by the presentation of aortic dissection and subsequent oliguria.
- While hematuria can occur in various renal conditions, the presence of **RBC casts** points to bleeding originating from the glomerulus, which is a different pathology than ATN.
*Fatty casts*
- **Fatty casts** are typically associated with **nephrotic syndrome**, a condition characterized by significant proteinuria, hypoalbuminemia, and edema.
- There is no clinical information to suggest nephrotic syndrome in this patient, whose acute renal failure is likely due to hypoperfusion.
*Waxy casts*
- **Waxy casts** are generally indicative of **chronic kidney disease** and highly advanced, severe tubular damage, representing a later stage of kidney injury.
- While the patient has acute kidney injury, the timeline and acute nature of the insult make muddy brown casts more likely than waxy casts.
*Hyaline casts*
- **Hyaline casts** are composed primarily of Tamm-Horsfall mucoprotein, a normal protein secreted by renal tubule cells.
- These casts can be seen in normal urine, especially after exercise or dehydration, and are not specific for any particular kidney pathology or acute kidney injury.
Question 108: A 9-year-old boy is brought to the emergency department by his parents with a 2-day history of weakness and joint pain. He was adopted 3 weeks ago from an international adoption agency and this is his first week in the United States. He says that he has been healthy and that he had an episode of sore throat shortly before his adoption. Physical exam reveals an ill-appearing boy with a fever, widespread flat red rash, and multiple subcutaneous nodules. The type of hypersensitivity seen in this patient's disease is also characteristic of which of the following diseases?
A. Contact dermatitis
B. Serum sickness
C. Asthma
D. Goodpasture syndrome (Correct Answer)
E. Osteogenesis imperfecta
Explanation: ***Goodpasture syndrome***
- The patient's presentation with a recent **sore throat**, **fever**, **widespread rash**, **joint pain**, and **subcutaneous nodules** is highly suggestive of **rheumatic fever**.
- **Rheumatic fever** is a **Type II hypersensitivity reaction** where antibodies against streptococcal antigens cross-react with host tissues, a mechanism also seen in **Goodpasture syndrome** where antibodies target basement membranes in the kidneys and lungs.
*Contact dermatitis*
- This is a **Type IV hypersensitivity reaction**, mediated by T-cells upon re-exposure to an allergen.
- It presents as a localized, eczematous rash and is not characterized by systemic symptoms like fever, joint pain, or subcutaneous nodules.
*Serum sickness*
- This is a **Type III hypersensitivity reaction** involving immune complex deposition, often occurring 7-14 days after exposure to certain drugs or foreign proteins.
- While it can manifest with fever, rash, and joint pain, the formation of **subcutaneous nodules** and the typical progression following a **strep throat** episode are more indicative of rheumatic fever.
*Asthma*
- Asthma is primarily a **Type I hypersensitivity reaction**, characterized by IgE-mediated mast cell degranulation and bronchoconstriction.
- It presents with respiratory symptoms like wheezing and shortness of breath, not directly related to joint pain, rash, or subcutaneous nodules seen here.
*Osteogenesis imperfecta*
- This is a **genetic disorder** affecting collagen production, leading to brittle bones and other connective tissue abnormalities.
- It is not a hypersensitivity reaction and does not present with acute symptoms like fever, rash, or joint pain; rather, it is a congenital condition.
Question 109: A 73-year-old man presents to his primary care doctor with his son who reports that his father has been acting strangely. He has started staring into space throughout the day and has a limited attention span. He has been found talking to people who are not present and has gotten lost while driving twice. He has occasional urinary incontinence. His past medical history is notable for a stroke 5 years ago with residual right arm weakness, diabetes, hypertension, and hyperlipidemia. He takes aspirin, glyburide, metformin, lisinopril, hydrochlorothiazide, and atorvastatin. On examination, he is oriented to person and place but thinks the year is 1989. He is inattentive throughout the exam. He takes short steps while walking. His movements are grossly slowed. A brain biopsy in this patient would most likely reveal which of the following?
A. Large intracellular vacuoles within a spongiform cortex
B. Intracellular round aggregates of hyperphosphorylated microtubule-associated protein
C. Marked diffuse cortical atherosclerosis
D. Eosinophilic intracytoplasmic inclusions (Correct Answer)
E. Extracellular amyloid plaques
Explanation: **Eosinophilic intracytoplasmic inclusions**
* The constellation of **fluctuating cognitive impairment**, **visual hallucinations** (talking to people not present), and **parkinsonian features** (slowed movements, short steps) are highly characteristic of **dementia with Lewy bodies (DLB)**.
* Brain biopsies in DLB reveal **Lewy bodies**, which are **eosinophilic intracytoplasmic aggregates of alpha-synuclein**.
*Large intracellular vacuoles within a spongiform cortex*
* This description is characteristic of **spongiform encephalopathy**, such as **Creutzfeldt-Jakob disease**.
* CJD presents with rapidly progressive dementia, myoclonus, and ataxia, which are not the primary features in this patient.
*Intracellular round aggregates of hyperphosphorylated microtubule-associated protein*
* This describes **neurofibrillary tangles**, primarily composed of **hyperphosphorylated tau protein**, which are a hallmark of **Alzheimer's disease**.
* While Alzheimer's involves memory loss and cognitive decline, the prominent visual hallucinations and parkinsonism are more suggestive of DLB.
*Marked diffuse cortical atherosclerosis*
* While the patient has a history of stroke and vascular risk factors, **diffuse cortical atherosclerosis** is a pathological finding associated with **vascular dementia**.
* Vascular dementia typically presents with a stepwise decline in cognitive function and focal neurological deficits, but the prominent hallucinations and parkinsonism point away from a primary diagnosis of vascular dementia alone.
*Extracellular amyloid plaques*
* These are **beta-amyloid plaques**, another key pathological feature of **Alzheimer's disease**.
* While patients with DLB can sometimes have co-existing Alzheimer's pathology, the primary and most defining characteristic of DLB are the Lewy bodies.
Question 110: A 4-year-old girl is brought to the physician because of increasing swelling around her eyes and over both her feet for the past 4 days. During this period, she has had frothy light yellow urine. Her vital signs are within normal limits. Physical examination shows periorbital edema and 2+ pitting edema of the lower legs and ankles. A urinalysis of this patient is most likely to show which of the following findings?
A. Fatty casts (Correct Answer)
B. Epithelial casts
C. WBC casts
D. Waxy casts
E. Muddy brown casts
Explanation: ***Fatty casts***
- The presence of **periorbital edema**, **pitting edema** of the lower legs and ankles, and **frothy urine** in a child is highly suggestive of **nephrotic syndrome**.
- **Fatty casts** are formed when lipid-laden renal tubular epithelial cells (oval fat bodies) aggregate and are expelled into the urine, and are characteristically associated with **nephrotic syndrome** due to massive proteinuria and lipiduria.
- Among the cast types listed, fatty casts are the most specific finding for nephrotic syndrome.
*Epithelial casts*
- **Epithelial cell casts** indicate **acute tubular necrosis (ATN)** or other conditions involving damage to the renal tubular epithelium.
- While ATN can cause edema, the clinical presentation here (frothy urine, periorbital edema, absence of oliguria or acute kidney injury) is more classic for nephrotic syndrome.
*WBC casts*
- **White blood cell (WBC) casts** are indicative of **renal parenchymal inflammation** or **infection**, such as pyelonephritis or interstitial nephritis.
- The patient's symptoms do not suggest infection, and there is no mention of fever, dysuria, or flank pain.
*Waxy casts*
- **Waxy casts** typically indicate **chronic kidney disease** and advanced renal failure, representing severe urinary stasis and dilated, damaged tubules.
- The acute onset of symptoms (4 days) in a 4-year-old child makes chronic kidney disease less likely as the primary diagnosis.
*Muddy brown casts*
- **Muddy brown casts** are pathognomonic for **acute tubular necrosis (ATN)**, often seen in cases of ischemia or nephrotoxic injury.
- While ATN is a severe renal injury, the patient's presentation with frothy urine and prominent edema without signs of acute kidney injury points more specifically to nephrotic syndrome.