SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Primary Glomerular Disease
An 18-year-old Hispanic man experiences nephrotic syndrome (serum albumin 2.9 g/dl and urine protein excretion of 6 g per day). A kidney biopsy reveals a lesion of FSGS, not otherwise speciﬁed, by light microscopy with diffuse foot process effacement on electron microscopy. Immunoﬂuorescence microscopy shows mesangial deposits of IgM and C3. There is no family history of kidney disease. His physical examination shows a body mass index of 28 kg/m2, a blood pressure of 146/90 mmHg, and moderate peripheral edema. His serum creatinine level is 1.4 mg/dl. After 8 weeks of prednisone at a dosage of 1 mg/kg per day and angiotensin inhibition with losartan, he remains nephrotic with a stable serum creatinine level. HIV serology is negative..
Question: Which ONE of the following is the MOST appropriate next step in this patient’s evaluation?
A. Perform genetic testing for a podocytopathy
B. Determine the APOL1 risk allele status
C. Search for an underlying lymphoma
D. Request B7-1 (CD80) stain of the kidney biopsy specimen
Answer: A
Subject: Primary Glomerular Disease
A 38-year-old woman is found to have a lesion of membranous nephropathy on kidney biopsy per-formed to evaluate nephrotic syndrome (serum albumin 3.0 g/dl and urine protein excretion 9 g per day). The biopsy specimen showed characteristic diffuse thickening of the glomerular basement membrane with no associated endocapillary hypercellularity. Electron microscopy showed numerous subepithelial immune complex–type deposits. No enhanced anti–phospholipase A2 receptor (PLA2R) antigen expression was present in the glomeruli. No mesangial deposits were seen by electron microscopy. IgG4 was the dominant IgG subclass in the immune deposits. Kappa and lambda light chains were of equal intensity. The result of ELISA for serum anti–PLA2R antibody was negative. Serologic test results for hepatitis B and C were negative..
Question: Which ONE of the following tests would you order next?
A. Serum C3 and C4
B. Anti–nuclear antibody and anti–double stranded DNA antibody
C. Anti–thrombospondin 7A staining of the renal biopsy specimen
D. Serum free light chains
Answer: C
Subject: Primary Glomerular Disease
A 38-year-old man is found to have a lesion of membranous nephropathy when a kidney biopsy is performed to evaluate nephrotic syndrome (serum albumin 3.0 g/dl and urine protein excretion 6 g per day). The results of his physical examination are normal except for minimal peripheral edema. An anti–PLA2R antibody test of serum is positive in low titer (30 RU/ml). A PLA2R antigen is hyperexpressed in glomeruli. The serum creatinine is 1.0 mg/dl. A complete blood count is normal. Results of serologic studies for hepatitis B and C are negative..
Question: Which ONE of the following would you do next?
A. Evaluate for an underlying malignancy
B. Initiate treatment with cyclosporine plus steroids
C. Initiate treatment with cyclophosphamide plus steroids
D. Initiate treatment with rituximab
E. Observe with renin-angiotensin inhibition therapy only
Answer: E
Subject: Primary Glomerular Disease
Question: Question 2799:
Which ONE of the following statements is CORRECT concerning the use of rituximab for treatment of primary membranous nephropathy?
A. The response to rituximab is conditioned by the prior response to immunosuppressive therapeutic agents
B. Combinations of rituximab with low-dose cyclophosphamide can achieve very high remission rates
C. Four doses of rituximab at a dosage of 375 mg/m2 per week are superior to a regimen of rituximab dosing based on serial measurement of circulating CD19-positive B cell counts in achieving a reduction in proteinuria
D. The concomitant use of corticosteroids is required for rituximab to be effective
Answer: B
Subject: Primary Glomerular Disease
A 32-year-old Hispanic man with hematuria, impaired kidney function, and proteinuria (serum creatinine 1.9 mg/dl and urine protein excretion of 2.1 g per day) is found to have IgA nephropathy with an Oxford-MEST-C score of M1, E1, S0, T1, C2 on kidney biopsy. Four days later his serum creatinine is 2.8 mg/dl..
Question: Which ONE of the following treatment regimens would be MOST likely to reduce the rate of progression to ESRD?
A. Aggressive use of renin-angiotensin inhibition
B. A 6-month course of low-dose corticosteroids
C. A 6-month course of combined mycophenolate mofetil (MMF) plus low-dose steroids
D. Treatment with high-dose steroids, cyclo-phosphamide, and plasmapheresis
E. Treatment with high-dose steroids only
Answer: D
Subject: Primary Glomerular Disease
Question: Question 2801:
Which ONE of the following ﬁndings would NOT indicate a worse prognosis for a patient with recently diagnosed IgA nephropathy, with a MEST-C Score of M1, E1, S0, T1, C0 and persistent proteinuria of about 1.0 gram per day?
A. Extensive C4d deposition in the mesangium
B. Concomitant persistent microscopic hematuria
C. Low birth weight
D. Low glomerular density (number of glomeruli per cubic millimeter)
E. Increased serum C3 and normal C4 levels
Answer: E
Subject: Primary Glomerular Disease
Question: Question 2802:
For patients ESRD due to IgA Nephropathy, which ONE of the following is MOST likely to unfavorably affect the risk of recurrence of the disease in a renal allograft?
A. A posttransplantation regimen consisting of tacrolimus, MMF, and low-dose corticosteroids
B. A posttransplantation regimen consisting of tacrolimus, MMF, and early corticosteroid withdrawal
C. An Oxford-MEST-C score in the native kidney at the time of diagnosis of M1, E1, S1, T2, C0
D. Elevated IgA serum levels at the time of transplantation
Answer: B
Subject: Primary Glomerular Disease
A 68-year-old woman has a diagnosis of C3 glomerulonephritis with a membranoproliferative pattern of injury by light microscopy on kidney biopsy performed because of recent hematuria, proteinuria, and impaired renal function (serum creatinine of 1.6 mg/dl). The serum C3 level is low, and C4 level is normal..
Question: Which ONE of the following studies should be performed?
A. Genetic analysis for pathogenic mutations of complement factor H, I, and membrane cofactor protein
B. Serum levels of soluble C5b–C9
C. Serum free light chains and immunoﬁxation
D. Serum level of C3 nephritic factor
E. Serum anti–nuclear antibody and anti–double stranded DNA antibody
Answer: C
Subject: Primary Glomerular Disease
A 63-year-old woman is found to have hematuria, proteinuria, and impaired kidney function (serum creatinine of 1.8 mg/dl). A kidney biopsy shows a membranoproliferative pattern of injury with 10% glomerular involvement with crescents on light microscopy. Immunoﬂuorescence microscopy reveals strong IgG and C3 deposition of about equal intensity. Deposits of kappa and lambda light chains are equal in intensity. No specimen is available for electron microscopy. The serum C3 and C4 levels are normal. Test results for hepatitis C virus infection are negative. The anti–nuclear antibody is 1:40. Serum free light chain levels are normal..
Question: Which ONE of the following studies would be MOST useful to clarify the nature of the disease present in this patient?
A. Stain biopsy specimen for immunoglobulin sub-class deposition
B. Stain biopsy specimen for PLA2R antigen
C. Stain biopsy specimen for C4d deposition
D. Stain biopsy specimen for DNAJB9 deposition
E. Stain biopsy specimen with Congo red
Answer: D
Subject: Primary Glomerular Disease
Question: Question 2805:
Which ONE of the following is MORE commonly found in the collapsing variant of FSGS compared with the other variants of this lesion?
A. Presence of two APOL1 high-risk alleles
B. Hyperexpression of CD80 in glomeruli
C. IgM mesangial deposits
D. Absence of thrombotic microangiopathy
Answer: A
Subject: Primary Glomerular Disease
A patient with a lesion of FSGS of uncertain cause and an initial proteinuria level of 3.1 g per day and a serum creatinine level of 1.2 mg/dl undergoes treatment with immunosuppression and angiotensin blockade. The proteinuria declines to 0.2 g per day after eight months of therapy, and the serum creatinine level remains stable..
Question: Which ONE of the following values corresponds to an approximate risk of kidney failure (per 100 patient years) for this patient?
A. 3.2 per 100 patient years
B. 6.6 per 100 patient years
C. 9.6 per 100 patient years
D. 15.4 per 100 patient years
E. 20.0 per 100 patient years
Answer: B
Subject: Primary Glomerular Disease
A 16-year-old young man of Scottish descent with nephrotic syndrome (serum albumin 3.0 g/dl and urine protein excretion 5.4 g per day) has a lesion of FSGS, not otherwise speciﬁed. He fails to respond to a 12-week course of initially high-dose and then tapering doses of oral prednisone. His serum creatinine level is 1.6 mg/dl..
Question: Which ONE of the following is MOST likely to provide an indication for a possible monogenic or digenic cause of his disease?
A. The histologic variant of FSGS
B. The level of renal function
C. The level of proteinuria
D. His ancestry
E. His age
Answer: E
Subject: Primary Glomerular Disease
An African-American man is found to have a “collapsing” variant of an FSGS lesion on a kidney biopsy performed for the evaluation of nephrotic syndrome. His serum albumin is 1.9 g/dl, and his urine protein excretion is 12 g per day. The serum creatinine concentration is 1.9 mg/dl. HIV serology is negative. Genetic studies show that he carries two high-risk alleles at the APOL1 locus..
Question: Which ONE of the following statements is MOST correct concerning this patient?
A. The genetic studies strongly indicate that he will be unresponsive to treatment
B. Information on his birth weight will be of value in determining his prognosis
C. The prognosis for renal survival is significantly worse in comparison with an FSGS lesion not otherwise speciﬁed
D. He is at low risk for the development of a thrombotic microangiopathy
Answer: B
Subject: Primary Glomerular Disease
Question: Question 2809:
Which ONE of the following serologic and immunohistologic ﬁndings BEST describes a patient with recently diagnosed primary membranous nephropathy with normal kidney function who is in the process of expe-riencing a spontaneous remission of the nephrotic syndrome?
A. Serum anti–PLA2R antibody negative and PLA2R1 antigen positive in glomeruli
B. Serum anti–PLA2R antibody negative and PLA2R1 antigen negative in glomeruli
C. Serum anti–PLA2R antibody positive and PLA2R1 antigen positive in glomeruli
D. Serum anti–PLA2R antibody positive and PLA2R1 antigen negative in glomeruli
Answer: A
Subject: Primary Glomerular Disease
A 36-year-old woman with multiple episodes of relapsing, steroid-sensitive nephrotic syndrome due to minimal change disease is treated with MMF and low doses of oral corticosteroids because of her fears of the gonadal effects of cyclophosphamide and the cosmetic effects of cyclosporine and high-dose steroids..
Question: Which ONE of the following statements is MOST correct concerning this patient?
A. A relapse is not likely when the MMF is discontinued
B. Tacrolimus is a good alternative treatment because of a low risk of long-term nephrotoxicity
C. Rituximab therapy would likely offer better prospects for long-term control of her disease
D. She is at high risk for the development of advanced chronic kidney disease and ESRD
Answer: C
Subject: Primary Glomerular Disease
Question: Question 2811:
Which ONE of the following is of LEAST value for discriminating pure diabetic nephropathy from a nondiabetic glomerular lesion in a patient with type 2 diabetes mellitus and overt proteinuria?
A. Duration of diagnosed diabetes mellitus
B. Presence of proliferative retinopathy
C. Magnitude of proteinuria
D. Presence of glomerular hematuria
Answer: C
Subject: Primary Glomerular Disease
A 26-year-old white man is undergoing an evaluation to serve as a potential kidney donor to his cousin. He is found to have microscopic hematuria (8–10 dysmorphic red blood cells per high power ﬁeld). The results of physical examination, blood pressure, serum creatinine, C3 and C4, renal imaging, and urinary albumin excretion are all normal. There is no family history of kidney disease except in his cousin, who has ESRD of unknown cause. A kidney biopsy is performed..
Question: Which ONE of the following is the MOST likely pathologic feature to be found in the kidney biopsy specimen?
A. C3 glomerulopathy
B. IgA nephropathy
C. Thin basement membrane nephropathy
D. Normal with no abnormality seen
Answer: D
Subject: Primary Glomerular Disease
A 41-year-old man has a diagnosis of nephrotic syndrome due to minimal change disease by clinical examination and by kidney biopsy, including light, immunoﬂuorescence, and electron microscopy..
Question: Which ONE of the following ﬁndings in the kidney biopsy specimen would suggest a high risk for relapse following a remission after an adequate course of steroid therapy?
A. Lower mesangial proliferation grade
B. Absence of glomerular enlargement
C. Presence of acute tubular necrosis
D. Extent of focal global glomerulosclerosis
Answer: A
Subject: Primary Glomerular Disease
A 28-year-old man has a diagnosis of nephrotic syndrome due to primary FSGS, not otherwise speciﬁed. His serum creatinine level is 1.5 mg/dl, his serum albumin is 2.1 g/dl, and his urine protein excretion is 8.6 g per day..
Question: Which ONE of the following ﬁndings on immunoﬂuorescence microscopy would MOST likely indicate a poor response to cortico-steroids and a poor long-term prognosis?
A. Absence of IgM or C3 deposition
B. IgM deposition only
C. C3 deposition only
D. IgM and C3 deposition
Answer: D
Subject: Primary Glomerular Disease
A 38-year-old woman undergoes a kidney biopsy for nephrotic syndrome and hematuria. Anti–nuclear antibodies are positive at a titer of 1:80, but serum C3 and C4 levels are normal. An anti–DNA antibody test result is negative. Hepatitis B serologies are negative. The light and electron microscopic studies are most compatible with a lesion of membranous nephropathy. Immuno-ﬂuorescence microscopy shows only scanty IgG deposits and prominent C3 deposition in a granular distribution along the capillary wall..
Question: Which ONE of the following studies should be done next?
A. Serum anti–PLA2R antibody
B. Serum complement alternative pathway activation
C. Repeated immunoﬂuorescence study of a pronase digested parafﬁn block section
D. Laser dissection–mass spectrometry analysis of biopsy specimen
Answer: C
Subject: Primary Glomerular Disease
A 34-year-old woman undergoes evaluation for severe nephrotic syndrome (serum albumin 1.4 g/dl, serum creatinine 1.7 mg/dl, urine protein excretion 14 g per day, and anasarca). Both serum C3 and C4 are normal, hepatitis B serologies are negative, and anti–nuclear antibodies are positive at a titer of 1:40. The result of an ELISA anti–PLA2R antibody titer is 450 RU/ml (normal < 14 RU/ml). The blood pressure is 140/90 mmHg. She is recently married and wishes to become pregnant in the near future..
Question: Which ONE of the following should be done next?
A. Initiate treatment with cyclophosphamide
B. Initiate treatment with rituximab
C. Perform a kidney biopsy
D. Initiate treatment with prednisone 1 mg/kg per day for at least 6 weeks
Answer: B
Subject: Primary Glomerular Disease
Question: Question 2817:
Which ONE of the following pathologic features is generally regarded as indicating a poor prognosis in IgA nephropathy, in addition to ﬁndings that are incorporated into the OX-FORD-MEST-C scores?
A. Increased C4d mesangial deposition
B. Increased glomerular density
C. Deposition of IgA exclusively in the mesangium
D. Increased IgG codeposition
Answer: A
Subject: Primary Glomerular Disease
A 72-year-old man is found to have membranoproliferative glomerulonephritis by light microscopy on kidney biopsy performed to evaluate proteinuria, hematuria, and impaired kidney function (serum creatinine 2.1 mg/dl). Immunoﬂuorescence microscopy showed extensive C3 deposition in the mesangium and in subendothe-lial areas, with only a trace of IgG/IgM deposition. Electron microscopy showed extensive mesangial and subendothelial electron dense deposits, without any substructure..
Question: Which ONE of the following tests would you do next?
A. Serum anti–nuclear antibody and anti–dsDNA antibody
B. Serum C3 nephritic factor
C. Serum free light chain assay
D. Serum cryoglobulins
Answer: C
Subject: Primary Glomerular Disease
A 62-year-old man experiences rapidly progressive glomerulonephritis. A kidney biopsy shows extensive crescents by light microscopy and “pseudo-linear” deposits of IgG by immunoﬂuorescence. No electron microscopy is performed because of an insufﬁcient specimen. The result of ELISA for serum anti–glomerular basement mem-brane antibody is negative..
Question: Which ONE of the following tests should you do next?
A. Serum anti–DNA antibody
B. Serum free light chain assay
C. Staining of renal biopsy specimen for DNAJB9
D. Repeat kidney biopsy for electron microscopy
Answer: C
Subject: Primary Glomerular Disease
Question: Question 2820:
Which ONE of the following indicates a high probability of recurrence of a primary form of an FSGS lesion in an adult patient receiving a living donor transplant?
A. Urine protein < 6 g per day at the time of diagnosis prior to transplant
B. Serum albumin < 2.5 g/dl at the time of diagnosis prior to transplant
C. Lower eGFR at the time of diagnosis prior to transplant
D. Family history of nephrotic syndrome
Answer: B
Subject: Primary Glomerular Disease
A 12-year-old boy has frequently relapsing, steroid-dependent nephrotic syndrome due to biopsy-proven minimal change disease. Prior therapy with a cyclosporine and oral cyclophosphamide has not reduced the frequency of relapses. He has severe Cushing syndrome from repeated treatment courses of oral corticosteroids. His serum creatinine level is 1.0 mg/dl..
Question: Which ONE of the following treatment regimens would be BEST for this patient?
A. Oral tacrolimus 0.1 mg/kg per day for 1 year
B. Oral MMF 2 g per day for 1 year
C. Rituximab 375 mg/m2 weekly for 4 doses
D. Azathioprine 2 mg/kg per day for 1 year
Answer: C
Subject: Primary Glomerular Disease
A 40-year-old woman of Korean descent is referred for evaluation of persistent asymptomatic microscopic hematuria that was ﬁrst discovered during an assessment for life insurance. She has had 5–25 erythrocytes per high power ﬁeld on urinalysis on three occasions over the past 6 months. The urine albumin-to-creatinine ratio was found to be elevated at 306 mg/g on one occasion. She does not exercise regularly and has not had any associated febrile illnesses. She does not have a family history of hematuria or kidney disease. The physical examination is normal. A urinalysis shows trace proteinuria and con-ﬁrms 5–10 erythrocytes per high power ﬁeld, of which 10% are dysmorphic. No crystals or bacteria are seen. No acanthocytes or erythrocyte casts are seen. Her serum creatinine level is 0.6 mg/dL. The urine albumin-to-creatinine ratio is 326 mg/g. Which ONE of the following is the MOST likely cause of her hematuria?.
Question: A. IgA nephropathy
B. Nutcracker syndrome
C. Hypercalciuria
D. Urethral tear
E. Thin basement membrane nephropathy
likely to receive inpatient palliative care services based on data from the United States National Inpatient Sample Data?
A. IgA nephropathy
B. Nutcracker syndrome
C. Hypercalciuria
D. Urethral tear
E. Thin basement membrane nephropathy
A. Acute respiratory distress syndrome
B. Cardiogenic shock
C. End-stage liver disease with cirrhosis
D. AKI requiring dialysis
Answer: A
Subject: Acute Kidney Injury & Critical Care Nephrology
Question: Question 2824:
The transfusion of longer-term stored packed red blood cells compared with fresher blood has been associated with which ONE of the following?
1. Increased risk of transfusion-related reactions
B. No difference in mortality at 90 days
C. Decreased number of RRT-free days within 28 days of transfusion
D. Increased risk of AKI requiring RRT
E. Decreased duration of mechanical ventilation
Answer: B
Subject: Acute Kidney Injury & Critical Care Nephrology
A 79-year-old man with stage G4 CKD and recently diagnosed colon cancer is seen in consultation after admission to the intensive care unit for septic shock. The patient has a mean arterial pressure of 62 mmHg on norepinephrine and dobutamine infusions. On physical exami-nation, he is cachectic, intubated, and sedated. The lung ﬁelds are clear to auscultation. There is 1+ leg edema. The kidney function is at base-line, and serum electrolytes are normal. Urine output is 1.3 ml/kg per hour after administration of 60 mg of intravenous furosemide. The intensivist is interested in initiating enteral feeds as soon as possible. You begin a discussion of the relative merits of this intervention in light of the recent ﬁndings from the NUTRIREA-2 study..
Question: Based on data from the NUTRIREA-2 study, enteral feeding is MORE likely to be associ-ated with which ONE of the following?
A. Decreased incidence of systemic bacteremia
B. Increased incidence of gastrointestinal complications
C. Decreased incidence of hypoglycemia
D. Increased incidence of 28-day mortality
E. Decreased incidence of 28-day mortality
Answer: B
Subject: Acute Kidney Injury & Critical Care Nephrology
A 67-year-old woman with a history of stage G2 CKD secondary to diabetic nephropathy is evaluated for AKI complicating septic shock. She recently underwent autologous stem cell trans-plantation for acute myelogenous leukemia. Her serum creatinine level has risen from 1.0 mg/dl to 4.9 mg/dl. She now has fever, hypotension, and suspected septic shock. Over the past 10 hours, the urine output has decreased to 0.2 mg/kg per h. A norepinephrine infusion increases the mean arterial pressure to 65 mmHg. You initiate continuous renal replacement therapy (RRT). An angiotensin II infusion is initiated, and the mean arterial pressure increases to 75 mmHg..
Question: Which ONE of the following is CORRECT about the impact of adding the angiotensin II infusion?
1. She is at decreased risk for inpatient mortality
B. Her duration of renal replacement therapy (RRT) will be shorter
C. Her Sequential Organ Failure Assessment (SOFA) score will increase
D. Her duration of mechanical ventilation will be shorter
Answer: B
Subject: Acute Kidney Injury & Critical Care Nephrology
You are caring for a 71-year-old man with stage G3a CKD disease who has recently undergone a right hemicolectomy for colon cancer. Post-operatively, he arrives in the intensive care unit receiving mechanical ventilatory support. He does not require vasopressor support. On physical examination, the blood pressure is 130/82 mmHg. He is alert and follows commands. The heart and lung examination results are unremarkable, and there is trace peripheral edema. His surgical and anesthesia teams initially plan a “restrictive” intravenous ﬂuid regimen designed to provide a net zero ﬂuid balance..
Question: Which ONE of the following is associated with this restrictive ﬂuid strategy compared with a liberal ﬂuid strategy?
A. A decreased risk of surgical site infections
B. An increased risk of AKI requiring RRT
C. An increased length of stay in the intensive care unit
D. An increased risk of mortality at 1 year
Answer: B