SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Pregnancy and Kidney Disease
A 36-year-old woman is evaluated for microscopic hematuria. She is now in the early first trimester. Her BP, physical examination, kidney function, and urine protein excretion are normal. A urinalysis shows trace blood, negative protein, and 3 to 5 dysmorphic erythrocytes per high power field. A urine culture is negative. A kidney ultrasound is normal. She is concerned about the potential impact on her pregnancy..
Question: In the absence of a change in her clinical findings during pregnancy, which of the following would you advise regarding the impact of the hematuria on her pregnancy?
A. It increases her risk for gestational hypertension
B. It increases the risk of preeclampsia three-fold
C. It places her at increased risk for preterm labor
D. It does not increase the risk for a small gestation baby
Answer: D
Subject: Pregnancy and Kidney Disease
A 32-year-old woman with ESRD on intensive hemodialysis is admitted at 37-weeks gestation with increased BP (160/98 mmHg) and headache. Fetal growth has been poor, and umbilical artery Doppler studies show absent end diastolic flow. She is admitted for urgent delivery for severe preeclampsia. The obstetrician wishes to commence intravenous magnesium..
Question: Which ONE of the following is the MOST appropriate dosing regimen for intravenous magnesium for this patient?
A. A reduction in the loading dose only
B. A reduction in the maintenance dose only
C. A reduction in the loading dose and the maintenance dose
D. No change in the loading or maintenance dose
Answer: C
Subject: Pregnancy and Kidney Disease
A 29-year-old woman, gravida 3, para 2, is referred for evaluation of new-onset proteinuria and hematuria. She is currently at 32-weeks gestation. Her first two pregnancies were term spontaneous vaginal deliveries. She has no prior medical history. Thus far, her pregnancy has been uneventful. She is on no medications except prenatal vitamins. She complains of bilateral stiffness in her hands but otherwise, feels well. On physical examination, the BP is 125/64 mmHg, and the cardiovascular examination is normal. There is no rash or synovitis. There is 1+ bilateral lower extremity edema. The urinalysis shows 2+ protein and 3+ blood. The urine sediment reveals 10–20 dysmorphic erythrocytes per high-power field with no casts. A 24-hour urine collection shows 2.4 g protein excretion. Serum electrolytes are normal. The serum creatinine is 1.1 mg/dl (increased from 0.6 mg/dl at her first prenatal visit), the hemoglobin is 10.4 g/dl, and the platelet count is 146,000/µl. Serologic testing reveals a positive antinuclear antibody with a titer of 1:320, positive anti–dsDNA antibodies, and low serum C3 and C4. Serologic studies for HIV, hepatitis B virus, and hepatitis C virus are negative. A kidney ultrasound reveals bilateral hydronephrosis..
Question: Which ONE of the following is the MOST appropriate initial management strategy?
A. Referral to urology for bilateral ureteral stent placement
B. Immediate admission, betamethasone for fetal maturation, and induction of labor
C. Empiric therapy with prednisone and azathioprine, followed by adjusted treatment after delivery based on kidne biopsy performed postpartum
D. Supportive management and induction of delivery at term followed by kidney biopsy and treatment after delivery
Answer: C
Subject: Pregnancy and Kidney Disease
A 32-year-old woman with biopsy-proven FSGS is seen for preconception counseling. Her BP is well controlled (128/72 mmHg) on candesartan and amlodipine. She has 2.8 g urine protein on 24-hour urine collection along with significant renal insufficiency, with a serum creatinine level of 2.6 mg/dl. She asks about her risks of pregnancy related complications..
Question: Which ONE of the following statements is CORRECT regarding her risk of CKD progression and preterm delivery?
A. Her risk of progression to ESRD during or shortly postpartum is about 20%
B. Her risk of progression to ESRD during or shortly postpartum is about 50%
C. Her risk of preterm delivery (< 37-weeks gestation) is about 20%
D. Her risk of extreme preterm delivery (< 34-weeks gestation) is about 20%
Answer: A
Subject: Primary & Secondary Glomerular Diseases
A 24-year-old Asian man who is a graduate student is discovered to have asymptomatic microscopic hematuria during a routine examination for a sports-related injury. The BP is 118/72 mmHg. His body mass index (BMI) is 33 kg/m2. The remainder of the history and physical examination is unremarkable. The serum creatinine level is 0.78 mg/dl. The urine sediment reveals 10–15 erythrocytes per high power field, of which 50% are dysmorphic. A spot urine albumin-to-creatinine ratio is 39 mg/g. The fasting blood glucose is 110 mg/dl..
Question: Which ONE of the following lesions is MOST likely to be found on renal biopsy?
A. FSGS
B. Thin basement membrane nephropathy
C. Minimal change disease
D. IgA nephropathy
E. Early diabetic glomerulosclerosis
Answer: D
Subject: Primary & Secondary Glomerular Diseases
An 82-year-old man is referred for evaluation of increased albuminuria detected on a recent spot urine albumin-to-creatinine ratio (33 mg/g) on routine follow-up for diet–controlled type 2 diabetes. He does not smoke. He takes no medication other than an occasional nonsteroidal anti–inflammatory drug for osteoarthritis. His BP is 130/65 mmHg. The BMI is 20 kg/m2. His physical examination is unremarkable. The serum creatinine is 0.95 mg/dl (eGFR 74 ml/min per 1.73 m2). A complete blood count is normal. The hemoglobin A1c is 5.7%. The urine dipstick shows no protein or blood. The addition of sulfosalicylic acid (eight drops of 20% sulfosalicylic acid to 10 ml urine) yields no turbidity..
Question: Which ONE of the following is the next MOST appropriate step in this patient’s management?
A. Start low-dose ramipril
B. Check the urine protein-to-creatinine ratio
C. Measure or estimate 24-hour creatinine excretion rate
D. Reduce protein intake to 0.8 g/kg
E. Start metformin therapy
Answer: C
Subject: Primary & Secondary Glomerular Diseases
A 12-year-old boy has experienced multiple relapses of steroid–sensitive nephrotic syndrome since age 6 years. At age 10 years, he underwent a kidney biopsy during a relapse that revealed minimal-change disease. Despite attempts to reduce the frequency of relapses with a short course of oral cyclophosphamide (8 weeks) and subsequently, a 6-month course of levamisole, he continues to relapse two to three times per year. Each relapse is initially responsive to corticosteroids, but the proteinuria worsens when the steroid dose is tapered to ,0.4 mg/kg per day. His therapy has been complicated by mood swings, abdominal striae, growth retardation, and osteoporosis. He recently had a florid relapse manifest with .15 g/d of protein excretion. His serum albumin level is 1.2 g/dl, and the serum creatinine level is 1.23 mg/dl (eGFR 40 ml/min per 1.73 m2)..
Question: Which ONE of the following is the next MOST appropriate step in this patient’s management?
A. Repeat kidney biopsy
B. Mycophenolate mofetil plus steroids
C. Tacrolimus plus steroids
D. Retreat with oral cyclophosphamide and steroids
E. Rituximab
Answer: E
Subject: Primary & Secondary Glomerular Diseases
A 77-year-old woman is referred to you for evaluation of steroid–resistant minimal-change disease and persisting nephrotic syndrome. She had gradual onset of worsening edema about 8 months ago and was found to have a 24-hour urine protein excretion of 18 g/d and serum albumin of 2.1 g/dl. The serum creatinine is 1.21 mg/dl (eGFR 43 ml/min per 1.73 m2). A kidney biopsy showed minimal changes by light microscopy (15 glomeruli, four of which were globally sclerotic). Immunofluorescence microscopy showed no staining for IgG and C3. Electron microscopy was not performed (no glomeruli were available for analysis). After 6 weeks of oral corticosteroids, she continues to show evidence of severe nephrotic syndrome..
Question: Which ONE of the following would you do NEXT?
A. Repeat kidney biopsy with electron microscopy
B. Perform laser dissection/mass spectrometry on prior kidney biopsy
C. Stain prior kidney biopsy for B7.1 deposits
D. Stain prior kidney biopsy with Congo Red
E. Stain prior kidney biopsy for phospholipase A2 receptor (PLA2R) antigen
Answer: D
Subject: Primary & Secondary Glomerular Diseases
A 48-year-old black woman with nephrotic syndrome (serum albumin 3.1 g/dl and urine protein excretion 5.2 g/d) is evaluated in consultation. The serum creatinine is 1.91 mg/dl (eGFR  47 ml/min per 1.73 m2). There is no family history of kidney disease. A kidney biopsy shows a lesion of FSGS not otherwise specified involving eight of 22 glomeruli in the specimen. Immunofluorescence microscopy shows segmental IgM and C3 deposition, and electron microscopy shows >  80% foot process effacement. No microcytic changes or tubuloreticular structures are present. An HIV test is negative..
Question: Before starting therapy, which ONE of the following is the MOST appropriate next step in this patient’s diagnostic evaluation at this time?
A. Serum soluble urokinase–type plasminogen activator receptor level
B. Genetic testing for apolipoprotein A1 (APOL1) high–risk alleles
C. Genetic testing for an NPHS2 gene mutation
D. Genetic testing for polymorphisms of the angiotensin–converting enzyme gene
E. No additional diagnostic studies are required
Answer: E
Subject: Primary & Secondary Glomerular Diseases
A 15-year-old Hispanic boy is discovered to have proteinuria of 3.6 g/d and a serum creatinine of 1.45 mg/dl (eGFR 70 ml/min per 1.73 m2). The serum albumin is 4.1 g/dl. The urine sediment is unremarkable. His BMI is 31 kg/m2. His BP is 139/88 mmHg. A kidney biopsy reveals 19 glomeruli, three of which are globally sclerotic. No glomerular hypertrophy is observed. Electron microscopy reveals only minor podocyte foot process effacement. Immunofluorescence studies are negative..
Question: Which ONE of the following is the MOST appropriate treatment for this patient?
A. Oral angiotensin receptor blockers (ARBs)
B. Oral high–dose steroids
C. Mycophenolatemofetil plus low-dose steroids
D. Corticotropin injection gel
E. Cyclosporine plus low-dose steroids
Answer: A
Subject: Primary & Secondary Glomerular Diseases
Question: Question 2529:
In patients with IgA nephropathy with eGFRs.60 ml/min per 1.73 m2 and persistent proteinuria (0.75 g/d) after a 3- to 6-month trial of angiotensin blockade, which ONE of the following has been consistently shown to reduce protein excretion over the short term (e.g., 2 years) in randomized clinical trials?
A. Oral glucocorticoids
B. Mycophenolate mofetil
C. Omega-3 fatty acids
D. Cyclophosphamide
E. Azathioprine
Answer: A
Subject: Primary & Secondary Glomerular Diseases
A 30-year-old woman with renal biopsy–proven lesion of presumed primary FSGS not otherwise specified is seen in follow-up. At the time of diagnosis, the serum albumin was 2.1 g/dl, and the urine protein excretion was 18 g/d. She was initially treated with a 6-month course of high–dose oral corticosteroids (prednisone at 1 mg/kg per day) and renin-angiotensin system inhibition. The urine protein excretion is now 12 g/d, and the serum albumin is 2.9 g/dl. The serum creatinine is 1.21 mg/dl (eGFR 61 ml/min per 1.73 m2). She has gained 12 kg in weight..
Question: Which ONE of the following is the MOST appropriate management?
A. Continue steroids at reduced dosage (0.5 mg/kg per day) for another 3 months
B. Oral cyclosporine and low-dose steroids for  greater than or equal to 4 months
C. Rituximab and tapering doses of steroids
D. Corticotropin injection gel and tapering of oral steroids
E. Mycophenolate mofetil and tapering dosage of steroids
Answer: B
Subject: Primary & Secondary Glomerular Diseases
A 56-year-old man is evaluated after recently undergoing kidney biopsy to evaluate the nephrotic syndrome. Light microscopy shows a segmental glomerulosclerotic lesion in at least one glomerulus. He previously was in excellent health and has taken no medications or herbal preparations apart from rare use of acetaminophen. He does not have evidence of an underlying neoplasm or underlying viral infection..
Question: Which ONE of the following BEST describes the criteria for establishing a presumptive diagnosis of primary FSGS in a patient without evidence of an adverse drug reaction, cancer, or chronic viral infection?
A. A segmental glomerulosclerotic lesion in at least one glomerulus in a biopsy specimen examined by light microscopy
B. As in A plus focal and segmental IgM de-position by immunoﬂuorescence microscopy
C. As in A plus widespread effacement of the podocyte foot processes by electron microscopy
D. As in A plus elevated serum levels of soluble urokinase–type plasminogen activator receptor by immunoassay
E. As in A plus an elevated plasma level of a permeability-inducing factor by bioassay
Answer: C
Subject: Primary & Secondary Glomerular Diseases
A 68-year-old man develops nephrotic syndrome and is found to have a lesion of membranous nephropathy on kidney biopsy..
Question: Which ONE of the following ﬁndings would indicate that a primary form of the disorder is MOST likely present?
A. Extensive deposition of C3 in the capillary loops by immunoﬂuorescence microscopy
B. Coalescent electron–dense deposits in the subepithelial space by electron microscopy
C. Hyperexpression of PLA2R antigen by immunohistochemical studies of pronase digested, parafﬁn–preserved specimens
D. IgG subclass 2 dominance of the IgG deposits by immunoﬂuorescence microscopy with negative staining for PLA2R
E. Increased inﬁltration of capillary loops by polymorphonuclear leukocytes by light microscopy
Answer: C
Subject: Primary & Secondary Glomerular Diseases
A 46-year-old woman develops nephrotic syn-drome and is found to have a stage 1 membranous nephropathy lesion on kidney biopsy. The BP is 142/72 mmHg. Moderate pedal edema is present. The serum creatinine is 1.1 mg/dl (eGFR 79 ml/min per 1.73 m2), and the serum cholesterol is 320 mg/dl. The serum albumin is 3.2 g/dl, and the urine protein excretion is 4.8 g/d. The anti PLA2R antibody is positive at low titer by ELISA..
Question: In addition to treatment with an angiotensin–converting enzyme inhibitor (ACEI) and ARB, a statin agent, and a low-sodium diet, which ONE of the following regimens would you prescribe NEXT?
A. Intravenous rituximab at 1000 mg for two doses 2 weeks apart
B. Cyclical oral cyclophosphamide and steroids for 6 months
C. Oral mycophenolate mofetil monotherapy for 6 months
D. Oral tacrolimus monotherapy for 6 months
E. Continued supportive care and observation
Answer: E
Subject: Primary & Secondary Glomerular Diseases
A 30-year-old woman is referred for assessment and management of proteinuria (3 g/d) discovered during an evaluation for edema. She is otherwise asymptomatic. Review of prior laboratory studies shows a normal serum creatinine, normal serum complement levels, and negative serologies for antinuclear antibodies, HIV, hepatitis B virus, and hepatitis C virus. A kidney biopsy is performed and shows a lesion of mild membranous nephropathy by light microscopy and special stains. By electron microscopy, stage 1 subepithelial electron–dense deposits are seen. A few mesangial electron–dense deposits are also observed. Immunoﬂuorescence microscopy shows very strong and intense granular C3 deposits along the capillary walls. Staining for Igs and kappa- and lambda -light chains is negative. Serum anti–PLA2R antibodies are negative..
Question: Which ONE of the following should be done NEXT to identify the cause of the lesion?
A. Pronase digestion of parafﬁn-embedded material and stain for PLA2R antigen
B. Pronase digestion of parafﬁn-embedded material and staining for monoclonal IgG (light and heavy chains)
C. Staining of biopsy specimen for C1q and C4d
D. Staining of the biopsy with Congo Red or thioﬂavine T
Answer: B
Subject: Primary & Secondary Glomerular Diseases
A 48-year-old man with nephrotic syndrome is found to have a lesion of membranous nephropathy on kidney biopsy. He is a nonsmoker. Because of a chronic cough and mild dyspnea, a computed tomography scan of the chest is per-formed that reveals bilateral hilar adenopathy and scattered interstitial lesions at the base of both lungs. No pleural effusion or thickening is observed. Laboratory studies show serum creatinine of 1.3 mg/dl, serum albumin of 2.8 g/dl, and a urine protein excretion of 10.5 g/d. The serum PLA2R antibody is positive (by ELISA), and a special stain of the kidney biopsy specimen reveals hyperexpression of PLA2R antigen in the capillary loops..
Question: Which ONE of the following is the MOST likely diagnosis of his renal disease?
A. Sarcoidosis causing secondary membranous nephropathy
B. Lung cancer causing secondary membranous nephropathy
C. Hodgkin disease causing secondary membranous nephropathy
D. SLE causing secondary membranous nephropathy
E. Primary membranous nephropathy
Answer: A
Subject: Primary & Secondary Glomerular Diseases
Question: Question 2536:
Which ONE of the following is preferred initial therapy for a 30-year-old man with IgA nephropathy, 1.2 g proteinuria per day, eGFR 80 ml/min per 1.73 m2 and BPof 128/75 mmHg?
A. Dietary salt and protein restriction only
B. Oral ACEIs or ARBs
C. Dihydropyridine calcium channel blockers
D. Oral steroids plus ACEI or ARB
E. Oral calcitriol
Answer: B
Subject: Primary & Secondary Glomerular Diseases
A 59-year-old man is found to have nephrotic syndrome (serum albumin of 1.9 g/dl and urine protein excretion of 8.2 g/d). His serum creatinine is 1.48 mg/dl (eGFR 51 ml/min per 1.73 m2). A serum protein electrophoresis reveals a small M spike of around 0.2 g/dl, but a free light–chain assay is normal. A renal biopsy reveals amyloidosis by light (Congo Red stain positive) and electron microscopy. Immunoﬂuorescence microscopy shows no staining for antisera against monoclonal light or heavy chains. Stains for serum amyloid A are negative..
Question: Which ONE of the following is the MOST appropriate next step in this patient’s diagnostic evaluation?
A. Stain the specimen for serum amyloid P component
B. Perform serum immunoﬁxation assay
C. Perform a urinary electrophoresis
D. Perform a laser dissection/mass spectrometry analysis of the biopsy specimen
E. Perform Ig isotype and subclass stains after pronase digestion of the parafﬁn-embedded specimen
Answer: D
Subject: Primary & Secondary Glomerular Diseases
Question: Question 2539:
In a patient presenting with membranoproliferative GN who has no serologic evidence of hepatitis B or C infection and whose biopsy shows predominantly IgM on immunoﬂuorescence, which ONE of the following diagnoses should be seriously considered?
A. Occult hepatitis B
B. An underlying autoimmune disorder
C. Solid organ cancer
D. Cocaine abuse
Answer: B
Subject: Primary & Secondary Glomerular Diseases
A 40-year-old Asian man is found to have 4 g/d proteinuria and mild glomerular hematuria during an evaluation for new–onset leg edema. The serum creatinine concentration was 1.2 mg/dl. Complement levels were normal, and antinuclear antibodies were negative. The patient was hepatitis B surface antibody positive and hepatitis B surface antigen negative. A kidney biopsy was performed. The pathology was interpreted as membranous nephropathy with atypical features, with staining for both IgG and IgA on immuno-ﬂuorescence microscopy. Electron microscopy showed subepithelial and mesangial deposits..
Question: Which ONE of the following tests would be of the MOST help to conﬁrm this patient’s diagnosis?
A. Antibody levels to double-stranded DNA
B. Staining the kidney biopsy for hepatitis B surface antigen and core antigen
C. Measurement of serum anti–PLA2R antibodies
D. Stool for occult blood
Answer: B
Subject: Primary & Secondary Glomerular Diseases
A 46-year-old man with known hepatitis C virus infection was found to have proteinuria, hematuria, and lower extremity necrotic skin lesions. Circulating cryoglobulins were present. Kidney biopsy showed cryoglobulinemic membranoproliferative GN. The patient’s serum creatinine concentration was 1.5 mg/dl, and a 24-hour urine collection showed 4 g proteinuria. Viral genotyping suggested a genotype that may be less susceptible to IFN..
Question: Which ONE of the following is the BEST course of treatment for this patient?
A. Rituximab plus plasma exchange
B. Rituximab plus plasma exchange plus cyclo-phosphamide
C. Sofosbuvir plus simeprevir
D. Sofosbuvir plus simeprevir plus rituximab
Answer: D
Subject: Primary & Secondary Glomerular Diseases
A 29-year-old black patient with long-standing HIV secondary to past intravenous drug use injured his leg and was admitted to the hospital with an active cellulitis in the area of the injury. Staphylococcus was cultured from the leg. He had been on combined antiretroviral therapy for years, and his HIV viral load has been undetectable. He had never had kidney problems but developed renal insufﬁciency and nephrotic-range proteinuria with acanthocytes and red blood cell casts in his urine sediment..
Question: Which ONE of the following is the MOST likely diagnosis?
A. Collapsing FSGS
B. Postinfectious GN
C. IgA–dominant, infection–related GN
D. Hepatitis C–associated membranous nephropathy
Answer: C
Subject: Primary & Secondary Glomerular Diseases
A 24-year-old man with microscopic hematuria is found to have thin basement membrane disease on kidney biopsy. No other glomerular basement membrane abnormalities were noted on electron microscopy. His eGFR and BP are normal. A 24-hour urine collection showed 185 mg protein. His general health has been excellent, and he does not have evidence of hearing impairment. He recalls that, as a child, he was told that he could not play on the school football team, because he had some blood in his urine, but no additional evaluation was pursued at that time. He had no family history of kidney disease, but his father thought a doctor once told him that he had blood in his urine..
Question: Which ONE of the following would be the BEST advice for this patient?
A. Kidney prognosis is excellent, and there is no need for concern or additional follow-up
B. Begin an ACEI
C. Kidney prognosis is likely to be good long term, but uncertainty exists; therefore, yearly follow-up is advisable
D. Undergo genetic testing to achieve a molecular diagnosis
Answer: C
Subject: Primary & Secondary Glomerular Diseases
A 29-year-old man is referred for ankle swelling. During the initial evaluation, the patient is found to have proteinuria of 5 g/d. The serum creatinine concentration was 0.82 mg/dl, the serum albumin was slightly low at 3.4 g/dl, and total cholesterol was elevated at 336 mg/dl. A kidney biopsy showed FSGS with a well preserved tubulointerstitial compartment but extensive (> 90%) foot process effacement by electron microscopy. The patient said his father was on dialysis, and proteinuria was found in his sister during pregnancy, but it never resolved. A genetic cause for the patient’s FSGS was suspected. The patient had genotyping done and was found to have a pathogenic mutation in transient receptor potential channel 6. An ACEI was started, but proteinuria only decreased to 3.5 g/d after 3 months..
Question: Which ONE of the following treatments would you choose now?
A. A calcineurin inhibitor
B. Prednisone at 1 mg/kg per day for 16 weeks
C. Cyclophosphamide
D. Mycophenolate mofetil
Answer: A