SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Disorders of Divalent Ions, Renal Bone Disease and Nephrolithiasis

A 45-year-old woman who has been in generally good health is found to have an asymptomatic increase in her serum calcium of 10.3 mg/dl. Subsequent evaluation showed persistent mild hypercalcemia and a PTH level of 78 pg/ml (reference range 12–72 pg/ml). A 24-hour urine determination reveals a daily calcium excretion of 246 mg. The results of all other laboratory studies and routine radiology studies are normal..
Question: Recent studies would suggest which ONE of the following as the BEST clinical approach to her care?
1. Parathyroidectomy
2. Observation on a twice-a-year schedule
3. Cinacalcet
4. Estrogen therapy
5. Denosumab
Answer: B
Subject: Secondary Glomerular Disease
A 20-year-old white man is referred for asymptomatic hematuria discovered during a routine health examination for a new job. You see him with a nephrology fellow. His medical history is unremarkable, and he has been healthy all his life. He has never seen any blood in his urine, even with a cold or sore throat. His father has normal kidney function but was told he had blood in his urine. His mother is healthy with no history of any kidney problems. The patient’s physical examination is normal. His urine dipstick shows a speciﬁc gravity of 1.020, no protein, and large blood. Microscopic examination of the urine sediment demonstrates > 10 erythrocytes per high power ﬁeld; about 30%of the erythrocytes are acanthocytes. The serum creatinine level is 0.8 mg/dl. Under your super-vision the fellow talks with the patient about his kidney disease but makes one mistake..
Question: Which ONE of the following statements made by the fellow is WRONG?
A. The fellow tells the patient that his sister may also have hematuria
B. The fellow advises the patient that his kidney disease will most likely not result in ESRD, but progressive kidney disease can occur in a small number of patients
C. The fellow does not advocate a kidney biopsy at this time but notes that a biopsy would most likely show alterations in the thickness of the glomerular basement membrane (GBM)
D. The fellow tells the patient that his kidney disease is caused by a mutation in the gene that encodes the alpha-5 chain of type IV collagen
Answer: D
Subject: Secondary Glomerular Disease
A 30-year-old white woman is seen in consultation for a second opinion concerning steroid-resistant focal and segmental glomerulosclerosis (FSGS). Her initial evaluation, done elsewhere, showed that she had 4 g per day of proteinuria, a serum albumin of 3.2 g/dl, lipiduria, and a serum creatinine concentration of 1.3 mg/dl. Analysis of a kidney biopsy specimen showed an FSGS pattern of glomerular injury with nearly complete podocyte foot process effacement. She was treated with high-dose prednisone for 2 months without response. Her course was complicated by the development of Cushingoid features and weight gain. Thereafter, her management consisted of a low-sodium diet, a maximally tolerated dose of an angiotensin-converting enzyme inhibitor, and a diuretic. The patient’s father is on dialysis and had heavy proteinuria preceding the onset of ESRD. Currently the patient has about 2.5 g per day of proteinuria and poorly controlled hypertension. Her estimated GFR has fallen from 45ml/min per 1.73 m2 1year ago to 23ml/min per 1.73m2. She seeks your advice because of progressive CKD. A physical examination reveals a BP of 160/92 mmHg, a body mass index of 32 kg/m2, and trace pedal edema..
Question: Which ONE of the following would be the MOST helpful in her management?
A. Genetic analysis for hereditary forms of FSGS
B. Repeat kidney biopsy
C. Bariatric surgery because the FSGS is likely secondary to her obesity
D. Forgo kidney transplantation because the disease is likely to recur in the allograft
Answer: A
Subject: Secondary Glomerular Disease
A 48-year-old man is referred by your nephrology colleague for consultation regarding the evaluation and management of crescentic glomerulonephritis (GN). He presented with the nephritic syndrome and was found to have a serum creatinine level of 2 mg/dl. A physical examination reveals bilateral ankle edema, clear lungs, a normal cardiac examination, and no skin lesions. His urine dipstick is positive for protein and blood, and his urine sediment shows erythrocyte casts and several acanthocytes. A kidney biopsy performed 1 week ago shows a membranoproliferative pattern on light microscopy with < 20% cellular and ﬁbrocellular crescents, one area of glomerular capillary ﬁbrinoid necrosis, and strong linear GBM IgG staining by immunoﬂuorescence; however, no circulating anti-GBM antibodies were found. The IgG was lambda-light chain restricted..
Question: Which ONE of the following should you tell your colleague about this man’s glomerular disease?
A. The IgG in the kidney biopsy may be monoclonal
B. The patient will most likely have a positive assay for an anti-neutrophil cytoplasmic antibody (ANCA)
C. The patient will most likely require dialysis within 1 year
D. Treatment with cyclophosphamide, corticosteroids, and plasmapheresis should be started immediately
Answer: A
Subject: Secondary Glomerular Disease
You are asked to consult on a 56-year-old man who has been receiving sunitinib, a growth factor inhibitor, after unilateral nephrectomy for renal cell carcinoma. He has a history of diabetes mellitus and has had long-standing proteinuria of about 1.5 g/d and a serum creatinine concentration that has been about 2 mg/dl for the past 8 years. The patient’s oncologist found that the serum creatinine concentration recently increased to 3.2 mg/dl. When the test was repeated a month later, the serum creatinine level was 3.3 mg/dl. In general, the patient has been doing well and has been tolerating sunitinib. The BP has averaged about 140/ 85 mmHg on a modest dose of an angiotensin receptor blocker. His diabetes has been well controlled, with a hemoglobin A1c of 7.2%. The patient has had no recent infections, treatment with anti-biotics, new medications, or radiologic studies with intravenous contrast, and he was not taking any over-the-counter medications including nonsteroidal anti-inﬂammatory drugs. A urinalysis showed 3+ protein and 1+ blood, with about 6 erythrocytes per high power ﬁeld, 0–2 leukocytes per high power ﬁeld, and no bacteria. A 24-hour urine showed that proteinuria was increased to 3 g/d. The results of liver function studies were normal except for a mildly increased serum lactate dehydrogenase of 326 U/L (reference range, 60–100 U/L). The patient was anemic, with a hemoglobin of 11 g/dl, a leukocyte count of 5600/µL, and a platelet count of 160,000/µL. The international normalized ratio was normal..
Question: Which ONE of the following recommendations do you provide to the oncologist?
A. The worsening kidney function is most likely due to progression of diabetic nephropathy, and the patient needs better control of his BP
B. The worsening kidney function may be due thrombotic microangiopathy from the sunitinib, and you would like to do a kidney biopsy for diagnosis before recommending any changes to the patient’s cancer therapy
C. The worsening kidney function may be due to thrombotic microangiopathy from the sunitinib, which can be easily diagnosed by examining a peripheral blood smear for schistocytes and assessing the patient’s haptoglobin and complement levels
D. The worsening kidney function may be due to thrombotic microangiopathy from the sunitinib, and you recommend stopping the drug immediately
Answer: B
Subject: Secondary Glomerular Disease
You are asked to see a 62-year-old woman with peripheral vascular disease hospitalized for treat-ment of foot cellulitis for AKI. Her serum creatinine concentration was 1.4 mg/dl on admission but was known to be 1.0 mg/dl about 6 months ago. Her systolic BP on admission was 100 mmHg, and she initially received intrave-nous saline with stabilization of the systolic BP at 115 mmHg. However, over the next few days her serum creatinine slowly increased to 2.3 mg/dl, and you are called to see her for AKI. Her urine output over the preceding 24 hours was about 800 ml. Blood cultures obtained on admission were positive for methicillin-resistant Staphylococcus aureus, and she has been receiving intravenous vancomycin. On physical examination she does not appear toxic. The BP is 120/74 mmHg. The right foot cellulitis has a receding margin of erythema. Ultrasonography of the kidneys is un-remarkable. The urinalysis shows a speciﬁc gravity of 1.020, 3+ protein, 3+ blood, no bacteria, 4 leukocytes per high power ﬁeld, and 5–10 erythrocytes per high power ﬁeld, several of which are acanthocytes. There are occasional granular casts. Blood cultures have been negative for the past 24 hours. A vancomycin trough is 24 µg/ml (reference range, 10–20 µg/ml) on the day of the consultation. The serum C3 level is low at 62 mg/dl (reference range 88–171 mg/dl) and myeloperoxidase (MPO)-ANCA is positive at 49 U/ml (reference range < 21 U/ml)..
Question: Which ONE of the following should you recommend?
A. Supportive care because the patient most likely has postinfectious GN, and this will resolve with treatment of the infection
B. Discontinue vancomycin because this patient most likely has AKI due to vancomycin toxicity
C. Supportive care because the patient most likely has acute tubular necrosis due to hemodynamic compromise before being admitted to the hospital and given ﬂuids
D. A kidney biopsy to verify your suspicion that this patient has staphylococcal-associated IgA GN
E. High-dose intravenous corticosteroids plus cyclophosphamide for ANCA-associated nephritis
Answer: D
Subject: Secondary Glomerular Disease
Question: Question 2738:
Which ONE of the following statements about hepatitis C virus cryoglobulinemia and GN is CORRECT?
A. Interferon-based antiviral therapies do not decrease the chances of developing cryoglobulinemia and GN in patients infected with hepatitis C, genotype 1
B. GN cannot develop after successful treatment of hepatitis C with direct-acting anti-viral agents in patients who have achieved a sustained remission
C. Late treatment with interferon-based anti-viral therapies is as effective as early treatment in preventing the kidney manifestations of hepatitis C
D. Cryoglobulinemia can occur with hepatitis C and hepatitis B
Answer: D
Subject: Secondary Glomerular Disease
A patient with light-chain amyloidosis and known kidney involvement is being treated by a hematologist with a proteasome inhibitor–based regimen. The patient comes to your clinic and wants to know your opinion about his chances of requiring permanent renal replacement therapy. All the patient’s records are being sent to you but have not yet arrived. You call the hematologist, who is aboard a plane that is about to leave, and you have time to ask one question before the cell phone is switched off..
Question: Which ONE of the following questions will be MOST helpful to you in providing the patient with an immediate answer?
A. How much interstitial ﬁbrosis and tubular atrophy were present on the kidney biopsy specimen taken before treatment was started
B. How much proteinuria did the patient have when treatment was started
C. What was the patient’s eGFR when treatment was started
D. What was the magnitude and time course of the patient’s hematologic response to therapy
Answer: D
Subject: Secondary Glomerular Disease
A patient was referred to your clinic with impaired kidney function and proteinuria. Ex-amination of a kidney biopsy specimen revealed a membranoproliferative pattern of glomerular injury on light microscopy. Immunoﬂuorescence showed IgG deposits with k-light chains. IgG subclass staining showed only IgG3. Your nephropathologist thinks that this is proliferative GN with monoclonal immuno-globulin deposits..
Question: Which ONE of the following is the BEST next test(s) to help you decide on therapy?
A. Urine protein electrophoresis
B. Serum immunoﬁxation plus serum free light chains
C. Serum complement levels
D. Laser dissection/mass spectrometry analysis of the kidney biopsy
Answer: B
Subject: Secondary Glomerular Disease
A patient with non–small cell lung cancer is to be treated with the checkpoint inhibitor pembrolizumab. The patient’s oncologist refers the patient to you to determine if the patient is at risk for kidney injury while taking this medication and to offer advice on how to prevent kidney problems. The patient has no history of kidney disease and has a normal serum creatinine level and urinalysis. The patient is not taking any other prescribed medications but has been using a proton pump inhibitor for 3 years because of dyspepsia from nonsteroidal anti-inﬂammatory drugs used for chronic back pain..
Question: Which ONE of the following should you recommend?
A. Serologic testing for lupus
B. Hemoglobin A1c and fasting glucose level
C. Avoid nonsteroidal anti-inﬂammatory drugs and proton pump inhibitors
D. Begin a low-dose angiotensin converting enzyme inhibitor
Answer: C
Subject: Secondary Glomerular Disease
You are asked to consult on a 57-year-old woman who presented to the hospital with hemoptysis and kidney failure. The patient had been coughing up blood for about 2 weeks and was initially treated with antibiotics for bronchitis. She is found to have a serum creatinine concentration of 4 mg/dl. Her baseline kidney function is unknown, but she has no prior history of any medical problems. She does not take any medications routinely and has no family history of kidney disease. A urinalysis shows 10 erythrocytes per high power ﬁeld, of which about 25% are acanthocytes. Additionally, you ﬁnd two erythrocyte casts. Serologic studies for lupus, ANCA vasculitis, and anti-GBM disease are ordered and are pending. You perform an urgent kidney biopsy on the initial day of consultation because of concern for a rapidly progressive pulmonary renal syndrome. Examination of the biopsy specimen shows necrotizing crescentic GN with linear IgG immunoﬂuorescence along the GBMs. You start plasmapheresis, high-dose corticosteroids, and cyclophosphamide. Eventually the serologic data report comes back. The patient does not have antinuclear antibodies but does have anti-GBM antibodies and proteinase 3 (PR3)-ANCA..
Question: Which ONE of the following is the most appropriate management?
A. You stop cyclophosphamide and begin treatment with rituximab
B. You continue treatment for anti-GBM disease with plasmapheresis, steroids, and cyclophosphamide. and after the patient goes into remission you taper off all immunosuppression
C. You continue treatment for anti-GBM disease with plasmapheresis, steroids, and cyclophosphamide. and after the patient goes into remission you provide the patient with maintenance immunosuppression
D. You stop immunosuppression because short-term and long-term kidney survival in patients with double-positive anti-GBM disease and ANCA vasculitis is very poor, and the patient is likely to require long-term dialysis
Answer: C
Subject: Secondary Glomerular Disease
A 24-year-old Chinese woman is referred to you because of hematuria and proteinuria. She also describes joint pain and a facial rash, and lately she has noticed some unusual mouth ulcers. The urine sediment shows several acanthocytes and two erythrocyte casts. A 24-hour urine collection has 3 grams of protein. Her serum creati-nine is 1.4 mg/dl. A kidney biopsy specimen shows class IV lupus nephritis. You recommend starting high-dose corticosteroids in combination with cyclophosphamide. The patient refuses cyclophosphamide because of what she has learned on the internet about premature ovarian failure..
Question: Which ONE of the following is the BEST choice for induction therapy for this patient?
A. A combination of low-dose tacrolimus and low-dose mycophenolate mofetil (MMF) with prednisone
B. Azathioprine plus prednisone
C. Rituximab alone
D. Belimumab
Answer: A
Subject: Secondary Glomerular Disease
A 30-year-old black woman you treated for class IV lupus nephritis continues to have 1.5 g per day of proteinuria. She received a diagnosis of SLE and lupus nephritis about 3 years ago. She completed induction therapy with “Euro-lupus” cyclophosphamide and subsequently received maintenance therapy with mycophenolate mofetil (MMF) 1 g twice daily until now. She was also treated with prednisone, which was tapered off about 9 months ago. The patient initially had a good response to treatment, with a reduction in proteinuria from 4 g per day to 1.5 g per day during the ﬁrst year of treatment. The urine sediment became bland, and the hematuria re-solved. Her serum creatinine fell from 1.8 mg/dl at the time of diagnosis to 1.1 mg/dl after 6 months and has remained stable since that time. Serum C3 and C4 levels have normalized. She continues to be positive for antinuclear antibodies, but antibodies to double-stranded DNA are now negative. The patient wants to discontinue MMF in preparation for pregnancy..
Question: Which ONE of the following is the BEST course of action for this patient?
A. Stop MMF, begin azathioprine, and tell patient it is OK to conceive immediately
B. Stop MMF, begin tacrolimus, and tell the patient it is OK to conceive immediately
C. Talk to the patient about repeating a kidney biopsy to determine if her lupus nephritis is still active or if the proteinuria represents chronic disease and immunosuppression can be safely tapered
D. Re-treat the patient with high-dose cyclo-phosphamide to cause the proteinuria to remit
Answer: C
Subject: Secondary Glomerular Disease
A patient with class IV lupus nephritis is being treated with prednisone and MMF after initial induction therapy with “Euro-lupus” cyclophosphamide. She wants to avoid dialysis in the future and seeks your opinion as to what else she should be doing to prevent her disease from progressing to ESRD..
Question: Which ONE of the following do you suggest?
A. Add a calcineurin inhibitor to the regimen
B. Add an antimalarial agent to the regimen
C. Add rituximab to the regimen
D. Use azathioprine for maintenance instead of MMF
Answer: B
Subject: Secondary Glomerular Disease
A 23-year-old man with ESRD secondary to lupus nephritis is evaluated for a possible lupus ﬂare. He has been doing well with maintenance hemodialysis for the past 6 years. He has been maintained on immunosuppressive agents since starting dialysis and has had no extrarenal ﬂares of lupus. He has a history of antiphospholipid antibodies but has not had antiphospholipid syndrome. Over the past 2 weeks he has experienced a cough, pleuritic chest pain, joint aches, and a mouth ulcer. It is cold and ﬂu season, and several patients in the dialysis unit have upper respiratory infections. On physical examination, there is a shallow ulcer in the left buccal mucosa. There is no synovitis. Laboratory studies show a normal complete blood count and an increased sedimentation rate of 84 mm/h (reference range < 15 mm/h). Both C3 and C4 have been persistently low over the past several months..
Question: Which ONE of the following factors makes it LESS likely that he is experiencing a lupus ﬂare?
A. The fact that he is male
B. The history of IgM antibodies to cardiolipin and β2-glycoprotein
C. Dialysis vintage > 5 years
D. Low complement factor C4
E. His age
Answer: C
Subject: Secondary Glomerular Disease
A 32-year-old black woman with pure class V lupus nephritis was successfully treated with MMF and corticosteroids and achieved complete remission 4 years ago. Since the time of re-mission, her kidney function has been normal, and she has not had proteinuria. She has not used any immunosuppressive agents for over a year. She returns to your ofﬁce because of a 6-week history of ankle edema. On physical examination, her BP is 140/90 mmHg. She has a mild facial rash and moderate ankle edema. The urinalysis shows 1+  blood and 1+ protein by dipstick. The urinary sediment shows many acanthocytes. Additional laboratory studies show a C3 level of 74 mg/dl, which is just below the lower limit of normal for your laboratory (reference range, 88–171 mg/dl). Anti-dsDNA antibodies are negative. The serum albumin is 3.4 g/dl, and the serum creatinine level is 1.1 mg/dl. The patient’s baseline serum creatinine is generally 0.7 to 0.8 mg/dl. A 24-hour urine collection contains 5 grams of protein. You recommend a repeat kidney biopsy, but the patient declines. A urine pregnancy test is negative..
Question: In addition to dietary sodium restriction, treatment with renin-angiotensin-aldosterone system (RAAS) blockade, plus diuretic therapy, which ONE of the following is the MOST appropriate next step in management?
A. Treat the patient with rituximab monotherapy for recurrent class V lupus nephritis
B. Treat the patient with a calcineurin inhibitor for recurrent class V lupus nephritis
C. Recommend close follow-up without immunosuppression
D. Restart MMF and corticosteroids to treat a presumptive diagnosis of class V plus class III or IV lupus nephritis
Answer: D
Subject: Secondary Glomerular Disease
You are asked to see a 50-year-old man in consultation for PR3-ANCA–associated small vessel vasculitis. He has experienced upper re-spiratory symptoms, epistaxis, and malaise for several weeks. His initial evaluation revealed a nasal ulcer and a serum creatinine level of 1.4 mg/dl. A urinalysis showed 2+ blood, 2+ protein, and many erythrocyte casts. A kidney biopsy revealed pauci-immune necrotizing GN with focal crescents. His chest radiograph was clear, and sinus radiographs showed opacities in the maxillary sinuses. Serologic studies showed a negative antinuclear antibody panel, negative anti-GBM antibody, and a positive ANCA sub-typed as PR3-ANCA. He was given two 500-mg intravenous boluses of methylprednisolone over 2 days and prednisone at a dose of 1 mg/kg per day..
Question: Which ONE of the following is the MOST appropriate treatment recommendation?
A. No additional immunosuppressive therapy
B. Intravenous cyclophosphamide plus plasma-pheresis
C. Rituximab
D. Mycophenolate mofetil
Answer: C
Subject: Secondary Glomerular Disease
A 45-year-old man is seen in consultation for MPO-ANCA–associated vasculitis with kidney involvement. His induction regimen was intravenous cyclophosphamide, corticosteroids, and ﬁve sessions of plasmapheresis. The patient did well and went into remission with a Birmingham Vasculitis Activity Score of zero, stabilization of serum creatinine at 1.5 mg/dl, and a reduction in proteinuria from 2.8 g to 1.0 g per day. He had been prescribed azathioprine for maintenance therapy. He is seeking a second opinion on the likelihood of disease relapse. You review all his clinical and laboratory data and conclude that he has a low risk of relapse..
Question: In addition to his MPO-ANCA status (as compared to PR3-ANCA), which ONE of the following BEST supports your conclusion?
A. ANCA titers decreased but were still positive when maintenance therapy was started
B. The patient was treated with intravenous cyclophosphamide
C. ANCA became undetectable after induction therapy
D. The patient was treated with plasma exchange
Answer: C
Subject: Secondary Glomerular Disease
A 26-year old patient you have been treating for pauci-immune GN is gradually progressing to ESRD. Extrarenal signs and symptoms of vasculitis have resolved, but the patient has remained MPO-ANCA positive. The patient has no additional medical problems. You initiate a discussion regarding renal replacement therapy..
Question: Which ONE of the following should you tell her regarding management options for ESRD?
A. Patients with ESRD due to ANCA-associated vasculitis have inferior survival while using hemodialysis compared with patients with ESRD from other causes
B. Patients with ESRD due to ANCA-associated vasculitis who receive a kidney transplant consistently have inferior allograft survival compared with patients receiving transplants for other reasons
C. Because the patient is still ANCA positive, transplantation should be delayed until she is ANCA negative for at least 6 months because of a higher risk of disease recurrence
D. Because the patient did not have lung or upper respiratory vasculitis, her chances of disease relapse while she is using dialysis are lower
Answer: D