SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Secondary Glomerular Disease
You have successfully induced remission in a patient with ANCA-associated nephritis and have begun giving him azathioprine maintenance therapy. The patient has had 18 months of total therapy and 12 months of complete remission and wants to stop immunosuppression. You agree that immunosuppression can be tapered..
Question: In which ONE of the following situations is withdrawal of therapy BEST supported?
A. The patient had mild kidney disease and minimal extrarenal signs or symptoms of vasculitis, so he is unlikely to experience relapse
B. The patient has been PR3-ANCA positive throughout his treatment course
C. The patient was PR3-ANCA positive, be-came negative with rituximab treatment, but became PR3-ANCA positive again at month 16
D. The patient had moderately severe MPO-ANCA–associated nephritis, received induction therapy with oral cyclophosphamide, and became ANCA negative when maintenance therapy was initiated
Answer: D
Subject: Secondary Glomerular Disease
You are asked to see a 75-year-oldman in consultation for rapidly progressive GN. He does not have extrarenal manifestation of vasculitis. Ex-amination of a kidney biopsy specimen reveals ﬁndings consistent with anti-GBM GN with 40%crescents. His serum creatinine level at presentation was 6.4 mg/dl. He asks about his prognosis..
Question: Which ONE of the following should you tell him about his prognosis?
A. His serum creatinine level at presentation is an independent predictor of dialysis de-pendency at 1 year
B. His age is associated with a reduced hazard of dialysis dependency at 1 year
C. His percentage of crescents shown by ex-amination of a kidney biopsy specimen is predictive of dialysis dependency at 1 year
D. Recent studies indicate that renal survival in anti-GBM nephritis is now > 50% in survivors
Answer: A
Subject: Secondary Glomerular Disease
A 28-year-old woman with a recent diagnosis of Fabry disease based on clinical features and genetic testing is referred for an opinion regarding enzyme replacement therapy with agalsidase-b. Her initial evaluation revealed 900 mg of daily urinary protein excretion and a serum creatinine level of 0.7 mg/dl. She has acroparesthesias and intermittent mild abdominal pain and diarrhea. On physical examination, her BP is 132/80 mmHg. A corneal examination reveals verticillata. Several angiokeratomas are present in the upper thighs. The urinalysis shows 1+ protein, negative blood, and rare ﬁne granular casts. A serologic evaluation investigating other potential causes of glomerular disease was unremarkable. An echocardiogram shows mild left ventricular hypertrophy..
Question: In addition to RAAS blockade, which ONE of the following is the BEST treatment recommendation for this woman?
A. Begin enzyme replacement therapy only if the daily protein excretion remains above 500 mg per day after 6 months of RAAS inhibition
B. Begin enzyme replacement therapy now
C. Delay enzyme replacement therapy until the serum creatinine level exceeds 1.5 mg/dl
D. Do not recommend enzyme replacement therapy because she has a favorable renal prognosis without treatment
Answer: B
Subject: Secondary Glomerular Disease
A 42-year-old Greek man is evaluated for progressive CKD. His sister has known microscopic hematuria. His BP is 126/80 mmHg. The result of physical examination is otherwise normal. Laboratory studies show a serum creatinine level of 1.8 mg/dl and a urine protein-to-creatinine ratio of 1456 mg/g. A urinalysis shows 1+ blood, 1+ protein, and15to20dysmorphic erythrocytes per high power ﬁeld. A kidney biopsy specimen reveals FSGS on light microscopy. Electron microscopy shows thinning of the GBM with focal regions of podocyte foot process effacement..
Question: Genetic testing will MOST likely reveal a mutation in which ONE of the following genes?
A. NPHS1 (nephrin)
B. ACTN4 (alpha-actinin 4)
C. COL4 (type IV chain of collagen)
D. NPHS2 (podocin)
Answer: C
Subject: Secondary Glomerular Disease
A 62-year-old man with hepatitis C virus infection is seen in consultation for rapidly progressive GN in association with mixed cryoglobulinemia. He sought medical attention after 2 weeks of worsening digital pain and ulcers, rash, weakness, and arthralgias. His serum creatinine level was found to have risen from 1.2 mg/dl 1 month ago to 7 mg/dl. He requires initiation of hemodialysis for progressive oliguria. A physical examination reveals palpable purpura with ulceration over the trunk and extremities, digital necrosis of several ﬁngers, and synovitis involving the hands, wrists, knees, and ankles. Laboratory studies show serum C3 low at 73 mg/dl (reference range 88–171 mg/dl), C4 low at 4mg/dl (reference range 15–48 mg/dl), and serum cryoglobulins with a polyclonal increase in IgG and a monoclonal anti-IgG IgM rheumatoid factor consistent with type II cryoglobulinemia. Testing for hepatitis C is positive with a viral load of 2,180,385 IU/ml and genotype 1b. The results of serologic studies for HIV and hepatitis B are negative. A kidney biopsy shows a pattern of membranoproliferative GN with cryoprecipitates in capillary loops on light microscopy. Immunoﬂuorescence microscopy reveals diffuse IgM deposition in the capillary loops..
Question: In addition to intravenous “pulse” cortico-steroids, which ONE of the following is the MOST appropriate management?
A. Antiviral therapy without additional immunosuppression
B. Rituximab or cyclophosphamide plus plasmapheresis without antiviral therapy
C. Antiviral therapy plus rituximab
D. Antiviral therapy, rituximab or cyclophosphamide, plus plasmapheresis
Answer: D
Subject: Secondary Glomerular Disease
A 62-year-old man with hepatitis B–related cirrhosis complicated by portal hypertension is seen in follow-up after a recent diagnosis of membranous glomerulopathy. On physical examination, the BP is 136/80 mmHg. He has minimal ascites and 1+ leg edema. Laboratory studies show a daily urinary protein excretion of 4 g per day, serum creatinine level of 1.6 mg/dl (stable compared with his most recent creatinine value 1 month ago), positive hepatitis B surface and e antigens, negative serologic results for hepatitis C and D, serum alanine transaminase of 89 U/L, and hepatitis B virus DNA of 2500 U/ml. The kidney biopsy specimen shows membranous nephropathy with positive staining for hepatitis B antigen..
Question: Which ONE of the following is the MOST appropriate management?
A. Pegylated interferón
B. Entecavir
C. Rituximab
D. No antiviral or immunosuppressive therapy
Answer: B
Subject: Secondary Glomerular Disease
A 32-year-old woman with mitral valve endocarditis due to methicillin-sensitive Streptococcus mutans is seen in consultation for AKI. A small (3 mm) mitral vegetation and mild mitral re-gurgitation were found on an initial transesophageal echocardiogram. She has been receiving intravenous penicillin G for 6 weeks, and she has not had fever for the past month, repeated blood cultures have been negative, and a repeated trans-esophageal echocardiogram did not show persistent vegetations or other abnormalities. The serum creatinine level increased from 0.8 mg/dl on admission to 4.5 mg/dl. Urinalysis shows erythrocyte casts and 1+ proteinuria. Her complete blood count shows white blood cell count of 6800/mL, hemoglobin of 9.2 g/dl, and platelets of 146,000/mL. Serum C3 is slightly low, C4 is normal, and test results for ANA, ANCA, anti–double-stranded DNA, hepatitis B and C, and HIV are negative. An electrocardiogram is normal. You do a kidney biopsy and ﬁnd 35%cellular crescents without ﬁbrinoid necrosis, and diffuse granular mesangial and segmental granular capillary wall staining for IgG (3+), IgM (2+), C3 (3+), C1q (3+), kappa-light chains (2–3+), and lambda-light chains (1–2+), but no staining for IgA or phospholipase A2 receptor antigen. Electron microscopy shows scattered subepithelial and mesangial deposits..
Question: Which ONE of the following is the MOST appropriate management?
A. Supportive care without further antibiotics or immunosuppression
B. Refer for mitral valve replacement
C. Eculizumab
D. Cyclophosphamide plus corticosteroids
Answer: D
Subject: Secondary Glomerular Disease
A 55-year-old man is referred for further evaluation and management of GN. He presented with increased edema and was found to have microscopic hematuria and proteinuria. He did not have rash, abdominal pain, diarrhea, or arthralgias. A kidney biopsy specimen showed membranoproliferative GN with mesangial and subendothelial deposits. Immunoﬂuorescence microscopy revealed intense mesangial and subendothelial deposits of monotypic IgA, C3, and kappa-light chains, with negative staining for lambda-light chains. Electron microscopy showed no organized deposits. His BP has been well controlled by losartan over the past 2 months. His laboratory studies show a serum creatinine level of 1.4 mg/dl, albumin of 3.4 g/dl, hemoglobin of 9.6 g/dl, and a urine protein-to-creatinine ratio of 2600 mg/g..
Question: Which ONE of following is the next BEST step in this patient’s management?
A. Begin prednisone 1 mg/kg per day for 6 to 8 weeks
B. Rituximab 1 g every other week for 2 doses
C. Conduct further hematologic investigation to search for a B cell clone
D. Check the antitissue transglutaminase antibody level
Answer: C
Subject: Secondary Glomerular Disease
A 51-year-old woman with longstanding type 1 diabetes mellitus is seen in consultation for nephrotic syndrome that developed over the past 3 months. Laboratory studies show a serum creatinine level of 1.14 mg/dl, an albumin of 2.7 g/dl, and serum protein electrophoresis and immunoﬁxation demonstrating trace monoclonal free l-light chains that are too low to accurately quantify. The serum free kappa-light chain level is 3.16 mg/dl, the lambda-light chain level is 2.83 mg/dl, and the free light chain ratio is 1.12. A urinalysis demonstrates 3+ protein and oval fat bodies. The urine protein-to-creatinine ratio is 7267 mg/g, and the albumin-to-creatinine ratio is 4987 mg/g. The white blood cell count is 9000/µL, hemoglobin is 11.8 g/dl, and the platelet count is 339,000/µL. A kidney biopsy shows ﬁndings consistent with IgG3 lambda-light heavy chain deposition disease. A bone marrow biopsy is consistent with small lymphocytic lymphoma, and ﬂow cytometry reveals a clone of a CD5/CD23 dual-positive mature B cell population..
Question: Which ONE of the following is the MOST appropriate initial therapy?
A. Cyclophosphamide, bortezomib plus dexamethasone
B. Referral for autologous hematopoietic cell transplantation now
C. Anti-B cell therapy with rituximab plus bendamustine
D. Plasmapheresis
Answer: C
Subject: Secondary Glomerular Disease
A 56-year-old man is referred for evaluation and management of AKI. Three months ago, he noted the onset of fatigue, blurred vision, and diplopia. A physical examination revealed bilateral upper eyelid edema. Hard masses were palpable under the upper lids. Images of 2-[18F]-ﬂuoro-2-deoxy- D-        glucose positron emission tomography/CT displayed enlargement and increased uptake in the bilateral lacrimal and submandibular glands, aorta, and kidneys. Laboratory studies showed a serum creatinine of 3.2 mg/dl, C3 of 46 mg/dl (reference range 88–171 mg/dl), C4 of 38 mg/dl (reference range 15–48 mg/dl), positive antinuclear antibodies at 1:40, negative anti–double-stranded DNA, and positive PR3-ANCA at 132 U/ml (reference range < 20 U/ml). A kidney biopsy specimen showed pauci-immune crescentic necrotizing GN. A biopsy specimen from an enlarged lacrimal gland showed whirling ﬁbrotic tissue encasing lymphoplasmacytic inﬁltrates..
Question: In this case, ANCA-associated vasculitis is MOST likely overlapping with which ONE of the following diagnoses?
A. IgG4-related disease
B. Sarcoidosis
C. Kikuchi disease
D. Cogan’s syndrome
Answer: A
Subject: Secondary Glomerular Disease
A 54-year-old woman is referred for further evaluation and management of recently diagnosed ﬁbrillary GN. She presented with a serum creatinine of 3.4 mg/dl and proteinuria of 5.4 g per day. She has many questions about her prognosis and management..
Question: Which ONE of following should you tell her about ﬁbrillary GN?
A. The majority of persons with this lesion have occult solid tumors
B. > 50% of patients treated with rituximab can expect improvement or stabilization of kidney function
C. Further evaluation should include testing for hepatitis C virus infection
D. Future kidney transplantation is contraindicated because recurrent ﬁbrillary GN always occurs
Answer: C
Subject: End Stage Renal Disease & Dialysis
You are caring for a 64-year-old man who has been maintained on three-times-per-week hemodialysis (HD) for the past 4 years. He indicates that he is tolerating his HD treatments well. The monthly predialysis laboratory studies show single-pool Kt/V of 1.25, hemoglobin of 10.8 g/dl, sodium of 138 mEq/L, potassium of 4.2 mEq/L, chloride of 103 mEq/L, total CO2 of 23 mmol/L, calcium of 9.0 mg/dl, phosphorus of 4.8 mg/dl, and al-bumin of 3.8 g/dl. He recently learned that sudden cardiac death (SCD) is the leading cause of death in ESRD, and he asks about your assessment of his risk..
Question: Which ONE of the following should you tell him has been associated with an INCREASED risk of sudden cardiac arrest in patients with ESRD?
A. An abnormal exercise stress test result
B. Elevated serum lipid levels
C. A QRS-T angle of greater than or equal to 75 degrees on signal-averaged electrocardiogram
D. A serum potassium level > 5 mEq/L on nondialysis days
E. Ventricular premature beats on 48-hour ambulatory electrocardiogram monitoring
Answer: C
Subject: End Stage Renal Disease & Dialysis
You are treating a 57-year-old man who has ESRD due to diabetic nephropathy. He has been on HD for 2 years. Over the past year his body mass index (BMI) has increased from 44 kg/m2 to 49 kg/m2. The dialysis center’s dietitian is concerned about the impact of the increasing weight on his outcomes. His recent laboratory studies show an equilibrated Kt/V of 1.3 and a C-reactive protein of 75 mg/L (reference range < 10 mg/L)..
Question: Which ONE of the following should you tell the dietician about elevated BMI in patients who are treated with HD?
A. Elevated BMI will predispose him to cardiac complications and increase his mortality risk
B. Elevated BMI will worsen metabolic control of his diabetes and increase his mortality risk
C. As long as he can maintain his vascular access (and avoid catheter-based vascular access) his mortality risk will be similar to that of other ESRD patients on dialysis
D. Elevated BMI is associated with improved survival in patients who have evidence of systemic inﬂammation
E. Elevated BMI is a surrogate indicator of nutritional status and thus is associated with improved survival on dialysis
Answer: D
Subject: End Stage Renal Disease & Dialysis
A 57-year-old woman with ESRD is evaluated during routine dialysis rounds. She complains of several weeks of increased pruritus aggravated by the recent cold weather. During a discussion of her recent laboratory studies, you explain that her serum parathyroid hormone (PTH) concentration is currently elevated above the target range. This has occurred despite optimization of the serum calcium and phosphorus laboratory values. Her medications include calcium acetate 1334 mg three times daily with meals and paricalcitol 4 µg with HD. She was unable to tolerate oral cinacalcet because of nausea. Her most recent laboratory studies show corrected calcium of 9.7 mg/dl, serum phosphorus of 4.5 mg/dl, and PTH of 1130 pg/ml. In addition to continuing her diet, vitamin D sterol, and phosphate binder therapy, you recommend initiation of intravenous (IV) etelcalcetide therapy with her dialysis treatments..
Question: Which ONE of the following should you tell her about the beneﬁts of etelcalcetide therapy?
A. It will be less likely to cause hypocalcemia than cinacalcet
B. It will have fewer gastrointestinal side effects than cinacalcet
C. It will decrease the need to adjust calcium concentration in her dialysate compared with cinacalcet
D. It is not inferior to cinacalcet in lowering PTH concentrations
E. It will reduce her risk for cardiovascular events, including SCD (sudden cardiac death)
Answer: D
Subject: End Stage Renal Disease & Dialysis
A 72-year-old man with stage 5 CKD due to autosomal dominant polycystic kidney disease is evaluated during a scheduled ofﬁce visit. He now has dysgeusia, pruritus, and intermittent nausea. He has received predialysis modality education. He has read about complications of different treatments, and he expresses concern about the high reported rate of SCD (sudden cardiac death) in patients with ESRD maintained on HD. He asks whether peritoneal dialysis (PD) offers any advantage over HD with regard to the risk of SCD..
Question: Which ONE of the following should you tell him about the risk of SCD?
A. The risk of SCD is the same in HD and PD patients
B. The risk of SCD is higher in HD than in PD patients
C. The risk of SCD is higher in HD than in PD patients, but only during the ﬁrst 6 months of treatment
D. The risk of SCD is higher in HD than in PD patients, but only in patients with known coronary artery disease
E. PD patients have lower risks of SCD compared with HD patients
Answer: A
Subject: End Stage Renal Disease & Dialysis
You are evaluating a 57-year-old man with ESRD maintained on in-center HD for recurrent intra-dialytic hypotension (IH). The patient’s prescribed dry weight is 90 kg, and he typically requires 2.5 to 3 L of ultraﬁltration during his dialysis sessions. He frequently cannot achieve his dry weight because his systolic BP de-creases from typical predialysis measurements of approximately 140 to 150 mmHg to intra-dialytic systolic BPs as low as 85 mmHg. He is not symptomatic with these events. He receives 4 hours of HD three times weekly. A newly hired advanced practice provider accompanies you on rounds and asks about the signiﬁcance of the IH..
Question: Which ONE of the following should you tell them about the signiﬁcance of IH?
A. Asymptomatic IH does not increase mortality risk
B. IH does not have an impact on dialysis recovery time
C. A drop in systolic pressure to < 90 mmHg is associated with increased mortality risk
D. The frequency of IH is reduced by blood volume monitoring–guided ultraﬁltration biofeedback
E. IH is not associated with increased risk of hospital readmission
Answer: C
Subject: End Stage Renal Disease & Dialysis
A retired 62-year-old man with ESRD is on maintenance HD and is under your care. He is planning a trip to Europe, and he is working with the clinic social worker to arrange for dialysis treatments while he is away. When the social worker presents clinic options for him, the patient learns that some of the clinics offer hemodiaﬁltration (HDF) as well as conventional HD. He asks you for information regarding the differences between the two modalities..
Question: Compared with conventional HD, which ONE of the following should you tell him about HDF?
A. HDF improves patient-reported posttreatment recovery times
B. HDF provides superior β-2 microglobulin clearance
C. HDF is consistently associated with im-proved survival
D. HDF reduces the frequency of myocardial stunning
E. HDF is associated with an increased risk of bacteremia
Answer: B
Subject: End Stage Renal Disease & Dialysis
A recent quality improvement analysis at your dialysis center revealed a high rate of hospitalizations for ﬂuid overload in patients maintained on HD. Your senior partners ask you to review the current status of lung ultrasonography and on-line optical technology in managing volume in ESRD patients before they will consider implementing these interventions..
Question: Which ONE of the following should you report about the use of lung ultrasonography and on-line optical technology?
A. Lung ultrasonography is equivalent to the clinical assessment of interstitial lung edema assessed by the absence of crackles and peripheral edema
B. Moderate interstitial lung edema by lung ultrasonography is associated with in-creased risk of death
C. The assessment of volume status with on-line optical technology is likely to be equivalent to the clinical assessment of volume status
D. Lung ultrasonography is a reliable tool to detect underhydration
Answer: B
Subject: End Stage Renal Disease & Dialysis
A 70-year-old man with ESRD and obesity is admitted to the medicine service for management of acute myocardial infarction. He requires placement of a right coronary artery stent and is discharged 3 days later. His medications are tailored in such a manner that he is discharged on four medications, decreased from ten at the time of admission..
Question: Which ONE of the following factors is associated with an INCREASED risk of hospital readmission within 30 days of discharge?
A. His age
B. His sex
C. The decrease in the number of his pre-scribed medications
D. His diagnosis of acute myocardial infarction
E. His obesity
Answer: D
Subject: End Stage Renal Disease & Dialysis
You are treating a 75-year-old woman with ESRD on maintenance HD. You recently noted on rounds that her heart rate has been increased with her treatments in the preceding week. On physical examination, the heart rate is 118 beats/min and it is irregularly irregular. She denies any chest pain, dyspnea, palpitations, or change in her exercise tolerance. An electrocardiogram conﬁrms the diagnosis of atrial ﬁbrillation (AF). You begin a discussion about the implications of this diagnosis and the beneﬁts and risks of anticoagulation..
Question: Which ONE of the following should you tell her about the risks associated with AF and treatment with warfarin?
A. AF is associated with an increased risk of ischemic stroke
B. AF is associated with increased mortality only in the ﬁrst 90 days after diagnosis
C. Asymptomatic supraventricular rhythms are not associated with increased mortality risk
D. Warfarin use in HD patients does not increase the risk of bleeding
Answer: A
Subject: End Stage Renal Disease & Dialysis
An 82-year-old woman with ESRD on HD is diagnosed with atrial ﬁbrillation. She is discharged from the emergency department on apixaban 5 mg twice daily. She weighs 55 kg. She declines warfarin therapy because of the need for repeated blood monitoring..
Question: Which ONE of the following is the MOST appropriate management?
A. Reduce apixiban to 2.5 mg twice daily
B. Change apixiban to rivaroxaban
C. No change in management
D. Recommend adding a supplemental 2.5-mg dose after each HD session
Answer: A
Subject: End Stage Renal Disease & Dialysis
You are treating a 51-year-old man with ESRD on HD. Five years ago he required aortic valve re-placement with a St. Jude prosthetic valve, and he has been maintained on warfarin since that time. His medications include calcium acetate, cinacalcet, ergo-calciferol, and intravenous (IV) epoetin. On physical examination, he is thin, with a BMI of 20 kg/m2. He complains of pain and tenderness over a 5-cm ulcerative lesion on the lateral aspect of his left calf concerning for a lesion resulting from calciﬁc uremic arteriolopathy. His laboratory studies show a hemoglobin of 10.5 g/dl, calcium of 8.1 mg/dl, phosphorus of 7.8 mg/dl, and PTH of 650 pg/ml..
Question: In addition to warfarin, which ONE of the following factors in this patient has been as-sociated with an INCREASED risk of calciﬁc uremic arteriopathy (calciphylaxis)?
A. His low BMI
B. His low serum calcium level
C. Use of IV epoetin
D. Ergocalciferol
Answer: D