SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Primary & Secondary Glomerular Diseases
An 84-year-old man is found to have proteinuria, hematuria, and renal insufﬁciency with a serum creatinine of 3.8 mg/dl. A kidney biopsy is performed, and it shows a crescentic necrotizing GN with no immune deposits. Serologic testing reveals the patient to be anti–myeloperoxidase-ANCA positive. He does not seem to have any extrarenal signs or symptoms of vasculitis..
Question: Which ONE of the following choices for treatment is MOST correct for this patient?
A. Treat only with corticosteroids
B. Initiate therapy with intravenous pulse methylprednisolone at 1 g/d for 3 days followed by oral prednisone at 1 mg/kg per day and intravenous cyclophosphamide at 1 g/m2 six times or oral cyclophosphamide at 2 mg/kg per day
C. Treat with cyclophosphamide and cortico-steroids as outlined in choice B but reduce the doses by greater than or equal to 20%
D. Treat only with mycophenolate mofetil
Answer: C
Subject: Primary & Secondary Glomerular Diseases
A 52-year-old woman is referred for management of pauciimmune necrotizing GN. She initially was found to have hematuria, proteinuria, a serum creatinine of 4.5 mg/dl, and an eGFR of 16 ml/min per 1.73 m2 during an evaluation for epistaxis, migratory arthralgias, mild hearing loss, and marked fatigue. She did not have hemoptysis. Serologic testing showed negative antinuclear antibodies, normal serum complement levels, negative serologies for hepatitis B and C viruses, and a positive anti–proteinase-3-ANCA. Kidney biopsy showed a pauciimmune, necrotizing GN. A chest radiograph was normal..
Question: In addition to intravenous methylprednisolone (500–1000 mg/d three times) followed by oral prednisone beginning at 1 mg/kg per day, which ONE of the following approaches is the MOST appropriate induction therapy for this patient?
A. Methotrexate at 20–25 mg/wk
B. Either intravenous cyclophosphamide or intravenous rituximab
C. Mycophenolate mofetil at 1 g twice daily
D. Plasmapheresis
Answer: B
Subject: Primary & Secondary Glomerular Diseases
63-year-old man with proteinase-3-ANCA–associated vasculitis was treated with cyclophosphamide and corticosteroids and attained remis-sion after 3 months of therapy. The choice of maintenance therapy is between rituximab, azathioprine, mycophenolate, and 6 more months of cyclophosphamide (extended cyclophosphamide) before switching to azathioprine. These immunosuppressive agents have been ranked below in the order of preferred use..
Question: Which ONE of the following choices lists of maintenance drugs is in the PREFERRED order of use?
A.            Rituximab > azathioprine > mycophenolate > extended cyclophosphamide
B.            Extended cyclophosphamide > mycophenolate > azathioprine
C.            Azathioprine = mycophenolate > extended cyclophosphamide
D.            Azathioprine > mycophenolate > extended cyclophosphamide
Answer: A
Subject: Primary & Secondary Glomerular Diseases
A 29-year-old woman is seen in consultation in the intensive care unit after developing hemoptysis and increased respiratory distress. She has multiple sclerosis that is in remission after recent treatment with alemtuzumab. On physical examination, she is tachypneic with increased work of breathing. The temperature is 36.0C. The oxygen saturation on ambient air is 85%. She has crackles in both lower lung ﬁelds and trace leg edema. Laboratory studies show a serum creatinine concentration of 2 mg/dl. The serum lactate dehydrogenase and haptoglobin levels are normal. The leukocyte count is 11,000/ml, the hemoglobin is 9 g/dl, and the platelet count is 326,000/µl. The urinalysis shows 1+ protein, 2+ blood, and numerous acanthocytes, and erythrocyte casts. A chest radiograph shows a diffuse alveolar ﬁlling process. A bronchoscopy conﬁrms the presence of diffuse pulmonary hemorrhage..
Question: Which ONE of the following is the MOST appropriate treatment for this patient?
A. Plasmapheresis, high-dose corticosteroids, and cyclophosphamide
B. Cyclosporine A
C. Rituximab
D. Intravenous Ig
Answer: A
Subject: Primary & Secondary Glomerular Diseases
An 80-year-old man is admitted for additional evaluation of a 2-week history of progressive fatigue and darkening of the urine. He does not have pulmonary symptoms or fever. On physical examination, he is afebrile. He has 1+ leg edema. The remainder of the examination is unremarkable. Laboratory studies show an initial serum creatinine concentration of 6.4 mg/dl that increased from 0.9 mg/dl 3 months ago. He is oliguric, with a 24- hour urine volume of only 200 ml. A kidney biopsy shows necrotizing crescentic GN involving 100% of 22 glomeruli in the specimen, with linear deposition of IgG along glomerular basement membranes. Serologic studies show dual positivity for antiglomerular basement mem-brane antibodies and myeloperoxidase-ANCAs. A chest radiograph was normal. He requires initiation of dialysis on the third hospital day for progressive pulmonary congestion and hyperkalemia..
Question: Which ONE of the following is the MOST appropriate management for this patient?
A. Provide supportive care and management for ESRD
B. High-dose corticosteroids, plasmapheresis, and cyclophosphamide
C. Intravenous rituximab at 1000 mg two times 2 weeks apart
D. High-dose corticosteroids plus rituximab
Answer: A
Subject: Primary & Secondary Glomerular Diseases
A 30-year-old white woman was treated for proliferative lupus nephritis (LN) with the Euro lupus protocol of corticosteroids, low-dose cyclophosphamide for 3 months, and maintenance with azathioprine. When treatment was started, her serum creatinine concentration was 1.2 mg/dl, and she had 2 g/d proteinuria. She had 15 red blood cells per high power ﬁeld on urinalysis, and several of these were acanthocytes. Her complement levels were very low, and she had high–titer antidouble–stranded DNA antibodies. After a year of treatment, the proteinuria has fallen to 600 mg/d, the serum creatinine concentration is 0.7 mg/dl, and the urine sediment is unremarkable. She does not have any extrarenal SLE symptoms. Serum complement levels are now normal, and anti-DNA antibodies are not detected..
Question: Which ONE of the following is the MOST likely prognosis for this patient?
A. The long-term prognosis is favorable, be-cause the proteinuria declined to < 0.7 g/d after 1 year
B. The long-term prognosis is guarded, be-cause her baseline eGFR was < 60 ml/min per m2
C. The long-term prognosis is guarded, be-cause baseline complement C3 and C4 levels were very low and antidouble–stranded DNA antibodies greatly elevated at the beginning of therapy
D. The long-term prognosis is favorable, because the urine sediment became inactive with treatment
Answer: A
Subject: Primary & Secondary Glomerular Diseases
A 40-year-old Hispanic woman who had been treated successfully for proliferative LN in the past with intravenous cyclophosphamide and corticosteroids was seen for a relapse of active LN. After 6 months of induction therapy, the patient received another year of maintenance cyclophosphamide given as quarterly intravenous pulses. The patient achieved complete renal remission 9 months after treatment began and remained in remission for 2 years after maintenance cyclophosphamide had been discontinued. In addition to treatment for LN, the patient had received a course of oral cyclophosphamide for central nervous system lupus several years before. As a result, the patient’s cumulative cyclophosphamide dose was 20 g. She now has low serum complement levels, an increase in serum creatinine from 1 to 1.3 mg/dl, and an increase in proteinuria from 350 mg/d to 2.5 g/d. You decide to treat the patient with oral prednisone and mycophenolate mofetil at 3 g/d. Mycophenolate mofetil is started at 500 mg twice a day, but she is unable to tolerate mycophenolate mofetil or mycophenolate sodium because of gastrointestinal symptoms..
Question: Which ONE of the following would be the BEST way to treat this relapse?
A. Add intravenous-pulse cyclophosphamide at a dose of 1 g/m2 monthly for 6 months to corticosteroids
B. Add low–dose Euro lupus cyclophosphamide for 3 months to corticosteroids
C. Add tacrolimus to corticosteroids
D. Add abatacept to corticosteroids
Answer: B
Subject: Primary & Secondary Glomerular Diseases
30-year-old black woman is evaluated 3 years after being diagnosed with severe LN (class 4 [G]). She received induction therapy with 6 months of intravenous cyclophosphamide (1 g/m2) and prednisone. She then was placed on maintenance mycophenolate mofetil at 2 g/d, low-dose prednisone (5 mg/d), and hydroxychloroquine. The patient achieved a complete renal remission after 1 year of overall therapy. You are considering tapering the intensity of her immunosuppression, because she has not had relapses and has normal kidney function and urinalyses. She has been enrolled in a clinical trial that assessed APOL1 risk allele status in addition to a number of novel biomarkers..
Question: Which ONE of the following criteria would MOST favor tapering of this patient’s immunosuppression?
A. The absence of high–risk APOL1 risk alleles
B. A sustained remission for at least 24 months
C. A high number of CD81 T cells in her urine
D. The absence of C3b autoantibodies
Answer: B
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
46-year-old man on hemodialysis three times per week is transferred to your unit. He feels relatively well. A review of his most recent laboratory testing shows calcium of 9.2 mg/dl, phosphorus of 6.1 mg/dl, alkaline phosphatase of 146 U/ml (reference range 36–92 U/ml), hemoglobin of 10.1 g/dL, and intact parathyroid hormone (PTH) of 324 pg/ml (reference range17–70 pg/ml). His current regimen includes sevelamer carbonate at 2400 mg three times daily with meals and paricalcitolat 2µg intravenously three times weekly with each dialysis..
Question: Which ONE of the following is the MOST appropriate next step in management of this man's hyperphosphatemia?
A. Increase sevelamer to 3200 mg three times daily
B. Add calcium carbonate at 1.25 g at night
C. Increase the dialysis treatment time by 30 minutes
D. Increase paricalcitol to 3 µg intravenously three times weekly
E. Conduct a detailed evaluation of his diet
Answer: E
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
Question: Question 2554:
Which ONE of the following is NOT associated with elevated serum alkaline phosphatase values?
A. Hip fracture
B. Mortality
C. Coronary calciﬁcation
D. Osteitis ﬁbrosa
E. Adynamic bone
Answer: E
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
Question: Question 2555:
Which ONE of the following about parathyroidectomy for patients on dialysis is CORRECT?
A. Outpatient minimally invasive parathyroidectomy can achieve excellent control of secondary hyperparathyroidism
B. All surgical approaches, such as partial, subtotal, or total parathyroidectomy with or without autotransplantation, are effective
C. Hospital admission is uncommon after parathyroidectomy
D. Patients with a history of parathyroidectomy have an increased risk for all-cause and cardiovascular mortality compared with patients with similar degrees of hyperparathyroidism
Answer: B
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 65-year-old man with ESRD has been on hemodialysis for 2 months and transfers to your dialysis unit. Laboratory values are calcium of 10.2 mg/dl, phosphorus of 6.5 mg/dl, intact PTH of 645 pg/ml (reference range 17–70 pg/ml), and alkaline phosphatase of 212 U/ml (reference range 36–92 U/ml). He has been taking calcium acetate at 2001 mg three times daily with each meal..
Question: Which ONE of the following is the MOST appropriate treatment?
A. No additional intervention
B. Begin cinacalcet therapy
C. Increase calcium acetate to 2668 mg three times daily with meals
D. Refer for parathyroidectomy
E. Add calcitriol at 0.25 µg intravenously three times weekly
Answer: B
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 74-year-old woman maintained on hemodialysis for the past 4 years is evaluated during dialysis rounds for complaints of frequent pain in the hips, legs, shoulders, and lower back. She has a history of severe peripheral vascular disease, coronary artery disease, renal artery stenosis, and chronic atrial ﬁbrillation. Her regimen includes paricalcitol at 2 µg three times weekly with each dialysis and calcium acetate at 667 mg three times daily with meals. A review of her laboratory studies shows that the serum calcium has ranged from 9.2 to 9.5 mg/dl, that the serum phosphorus has ranged from 4.7 to 5.4 mg/dl, that the serum alkaline phosphatase has ranged from 310 to 349 IU/ml (reference range 36–92 U/ml), and that the intact PTH has ranged from 303 to 434 pg/ml (reference range 17–70 pg/ml) over that past 12 months. The γ-glutamyl transferase is normal, and the 25-hydroxy vitamin D level is 32 (reference range 30–50 ng/ml). She had a dual–energy x-ray absorptiometry (DEXA) scan showing a T score of 22.9 at the hip and 22.5 at the lumbar spine..
Question: Which ONE of the following is the MOST appropriate management for this patient?
A. Denosumab
B. Increase calcium acetate
C. Zoledronic acid
D. Cinacalcet
E. Increase paracalcitol
Answer: D
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 68-year-old woman with ESRD caused by diabetes mellitus maintained on peritoneal dialysis for 4 years is evaluated in the peritoneal dialysis clinic. Two weeks ago, she sustained a stress fracture of the right femur. A right hip radiograph shows a linear cortical defect crossing the right femoral neck typical for a stress fracture and diffuse osteopenia. Her primary physician started her on alendronate and ordered a DEXA scan. This showed a T score of -22.1 for the spine and -22.5 for the hip. Her dialysate calcium is 2.0 mEq/L. Her laboratory data show PTH of 17 pg/ml (reference range 17–70 pg/ml), calcium of 10.2 mg/dl, phosphorus of 5.2 mg/dl, and alkaline phosphatase of 83 U/ml (reference range 36–92 U/ml)..
Question: In addition to discontinuing alendronate, which ONE of the following is the MOST appropriate management of this patient's CKD-mineral bone disorder?
A. Add calcitriol
B. Add denosumab
C. Increase the dialysate calcium to 2.5 mEq/L
D. Add teriparatide
Answer: D
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
Question: Question 2559:
Which ONE of the following statements is CORRECT?
A. The effect of phosphate binders does not increase linearly with dose
B. Noncalcium–containing phosphate binders have not been associated with decreased mortality
C. Randomized trials have shown that phos-phate binders lead to decreased mortality
D. Nutritional databases provide accurate phos-phate content of foods
E. Ferric citrate as a phosphate binder does not correct iron deﬁciency
Answer: A
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 52-year-old woman with bipolar disorder is evaluated for hypercalcemia. She complains of fatigue, and her family has noted that she has become more withdrawn over the past 2 months. Review of her parents' and brother's laboratory studies does not disclose hypercalcemia. Her medications are lithium and quetiapine. Laboratory studies show serum sodium of 138 mEq/L, potassium of 4.3 mEq/L, chloride of 100 mEq/L, total carbon dioxide (CO2) of 25 mmol/L, BUN of 22 mg/dl, creatinine of 1.3 mg/dl, calcium of 11.3 mg/dl, albumin of 4.3 g/dl, magnesium of 2.6 mg/dl, and phosphorus of 2.4 mg/dl. The PTH level is 136 pg/ml (reference range 17–70 pg/ml). The 25-hydroxy vitamin D level is 32 ng/ml (reference range 30–50 ng/ml), and the 1,25-dihydroxy vitamin D is 35 pg/ml (reference range 25–65 pg/ml). A urinalysis shows speciﬁc gravity of 1.012, trace protein, and no cells or casts. The 24-hour urine shows a volume of 3.1 L, creatinine of 1056 mg, and calcium of 99 mg..
Question: Which ONE of the following is the MOST likely diagnosis?
A. Sarcoidosis
B. Familial hypocalciuric hypercalcemia
C. Lithium–related primary hyperparathyroidism
D. Familial isolated hyperparathyroidism
Answer: C
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 70-year-old man with recently diagnosed mono-clonal Ig deposition disease is evaluated for hyperphosphatemia. He began therapy with bortezomib, lenalidomide, and dexamethasone 2 days ago. His urine output has been normal, and he complains of fatigue. Laboratory studies show sodium of 136 mEq/L, potassium of 4.2 mEq/L, chloride of 100 mEq/L, total CO2 of 20 mmol/L, BUN of 25 mg/dl, creatinine of 1.3 mg/dl (eGFR 55 ml/min per 1.73 m2), calcium of 9.1 mg/dl, albumin of 3.8 g/dl, phosphorus of 6.6 mg/dl, lactate dehydrogenase of 80 U/L (reference range 60–100 U/L), uric acid of 4.2 mg/dl, leukocyte count of 4700/µl, hemoglobin 9.6 of g/dl, and platelets of 131,000/µl. The serum IgG is 14,400 mg/dl (reference range 600–1700 mg/dl), IgM is 25 mg/dl (reference range 35–290 mg/dl), and IgA is 29 mg/dl (reference range 40–400 mg/dl). Serum free kappa-light chains are 1288 mg/dl, and serum free lambda light chains are 2.16 mg/dl..
Question: Which ONE of the following is the MOST likely cause of this man's hyperphosphatemia?
A. Tumor lysis syndrome
B. Decreased GFR
C. Elevated IgG kappa monoclonal protein
D. Bortezomib
E. Lenalidomide
Answer: C
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 65-year-old woman with ESRD on maintenance hemodialysis is admitted for additional evaluation management of an exquisitely painful abdominal nodule. She has been maintained on hemodialysis for 6 years. She has coronary artery disease and atrial ﬁbrillation. Medications are sevelamer, warfarin, carvedilol, and doxercalciferol. The dialysate calcium is 2.5 mEq/L. On physical examination, she is in moderate distress because of pain localized to the abdominal nodule. She is afebrile. Her body mass index is 38 kg/m2.A cardiac examination shows an irregular rhythm. Examination of the abdomen shows a 4x5-cm tender nodule in the right lower quadrant. Labo-ratory studies show intact PTH of 184 pg/ml (reference range 17–70 pg/ml), calcium of 9.6 mg/dl, and phosphorus of 5.8 mg/dl. A punch biopsy of the nodule shows scattered calciﬁcations in the subcutaneous tissue in a concentric pattern. There are calciﬁcations in the walls of subcutaneous small blood vessels and the adipose tissue around the vessels consistent with calciﬁc uremic arteriolopathy..
Question: In addition to discontinuing warfarin and doxercalciferol, which ONE of the following is the MOST appropriate management strategy for this woman?
A. Add cinacalcet
B. Parathyroidectomy
C. Wide excision of the abdominal lesion
D. Add sodium thiosulfate
Answer: D
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
You are asked to see a previously healthy 46-year-old man with a 5-month history of T5–T6-level paraplegia after a motor vehicle accident for hypercalcemia and hyperphosphatemia. He was initially admitted to the Neurologic Intensive Care Unit with pneumonia. After extensive antibiotic therapy and mechanical ventilation, he was extubated. It was then noted that the patient had a persistently elevated serum phosphate of 6.5–6.7 mg/dl. His serum phosphorous level had been 3.1 mg/dl during a previous hospitalization at the time of his initial accident. The physical examination is remarkable for T5–T6-level paraplegia and left elbow calciﬁcation. Laboratory studies show serum calcium of 10.4 mg/dl, phosphorus of 6.7 mg/dl, creatinine of 1.2 mg/dl, PTH of 10 pg/ml (reference range 17–70 pg/ml), hemoglobin of 13 g/L, and albumin of 2.8 g/dl. The 24-hour calcium excretion is 310 mg..
Question: Which ONE of the following is the next BEST step in management?
A. Start bisphosphonate therapy
B. Start acute PTH infusions
C. Perform Sestamibi scan of the parathyroids
D. Start denosumab
Answer: A
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 64-year-old man with advanced alcohol–related cirrhosis is admitted for management of intractable ascites. You are asked to see him for persistent hyperphosphatemia. The patient had no history of phosphate and calcium abnormalities in the past. His only medications are furosemide and lactulose. On physical examination, he has diffuse muscle wasting. He has marked ascites and 3+ leg edema. He has no asterixis. Laboratory stud-ies show serum calcium of 8.1 mg/dl, phosphorus of 6.4 mg/dl, creatinine of 0.8 mg/dl, PTH of 30 pg/ml (reference range 17–70 pg/ml), hemoglobin of 7.1 g/dL, total bilirubin of 21 mg/dl, and albumin of 1.3 g/dl..
Question: Which ONE of the following is the MOST appropriate next step in the evaluation of this man’s hyperphosphatemia?
A. Measure 24-hour urine phosphate
B. Measure ionized calcium
C. Identify the method of serum phosphate measurement
D. Measure tubular reabsorption of phosphate
Answer: C
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 45-year-old man is seen in consultation for longstanding hypercalcemia. At age 18 years old, he had kidney stones followed by a recurrent right renal calculus at age 26 years old. Further evaluation at that time showed a serum calcium level that was elevated between 10.7 and 11.5 mg/dl, a phosphorus level of 4.0 mg/d, a serum creatinine of 1.1 mg/dl, and 24-hour urine calcium of 369 mg. Despite the fact that all PTH levels were at or below the lower limit of the assay, the concern for hyperparathyroidism led to a neck exploration; 3 years later, a mediastinal exploration was unsuccessful in recovering abnormal parathyroid tissue. Histologic ﬁndings of these two operations included only two normal parathyroid glands. The patient's hypercalcemia and hypercalciuria persisted. Further workup revealed a normal angiotensin–converting enzyme level. The serum 1,25-dihydroxy vitamin D was 75 pg/ml (reference range 25–65 pg/ml). Treatment with oral prednisone at 30 mg/d for 1 week did not reduce the serum calcium. The patient takes no over the counter medications or vitamins. A family history revealed a cousin with a history of some sort of “calcium problem,” but no other familial diseases were described. A DEXA scan showed normal bone density. A chest radiograph was normal..
Question: Which ONE of the following laboratory tests is MOST likely to explain this patient's condition?
A. Intact PTH level
B. PTH–related protein level
C. 25-dihydroxy vitamin D level
D. 24,25-dihydroxy vitamin D level
E. Fibroblast growth factor 23 level
Answer: D
Subject: Disorders of Divalent Ions Renal Bone Disease & Nephrolithiasis
A 54-year-old man with stage G3b CKD is evaluated for persistent hypercalcemia after undergoing autologous hematopoietic stem cell transplantation for multiple myeloma. Despite treatment with a bisphosphonate 2 weeks ago, the serum calcium level remains elevated. A physical examination shows a cachectic appearing man in no acute distress. He is confused but alert. The BP is 146/90 mmHg, and the pulse rate is 90/min. The rest of the examination is unremarkable. Laboratory studies show serum calcium of 12.8 mg/dl, phosphorus of 4.6 mg/dl, creatinine of 2.2 mg/dl, hemoglobin of 7.1 g/dL, albumin of 3.3 g/dl, and total protein of 7.7 g/dl..
Question: In addition to saline infusions, which ONE of the following is the MOST appropriate treatment of this man's hypercalcemia?
A. Standard dose denosumab
B. Weight-based dose of denosumab
C. Weight-based dose of zoledronic acid
D. Standard dose calcitonin
Answer: B