Snapshot A 6-year-old boy is brought to the emergency department by his mother due to swelling around his eyes and legs. The mother reports that the patient recently recovered from an upper respiratory tract infection. Physical exam is significant for periorbital and lower extremity edema. Laboratory testing is significant for hypoalbuminemia and normal complement levels. Urinalysis demonstrates 4+ protein. A presumptive diagnosis of minimal change disease is made and the patient is started on steroid therapy. Introduction Clinical definition a type of kidney disease that results in proteinuria, peripheral edema, hyperlipidemia, and hypoalbuminemia Epidemiology incidence annually there are 3 cases per 100,000 adults Etiology primary glomerular disease focal segmental glomerulosclerosis membranous nephropathy minimal change disease secondary causes diabetic nephropathy systemic lupus erythematosus amyloidosis Pathogenesis the glomerulus becomes permeable to large molecules (e.g., albumin) this loss of albumin (proteinuria) results in hypoalbuminemia and edema associated with a hypercoagulable state pathophysiology unclear but may be due to loss of antithrombin and plasminogen proteins increased lipid synthesis secondary to proteinuria this in turn results in hypercholesterolemia and hyperlipidemia Associated conditions chronic kidney disease Prognosis depends on the underlying cause e.g., patients with minimal change disease typically respond well to steroid therapy Presentation Symptoms edema periorbital, lower extremity, and genital edema frothy urine ascites weight gain fatigue shortness of breath Physical exam hypertension edema leukonychia suggestive of a low albumin state and presents as white streaking on the fingernails Studies Labs hypoalbuminemia (serum albumin of < 2.5 g/dL) hyperlipidemia Urine studies proteinuria > 3-3.5 g/day or > 300-350 mg/mmol on spot urine protein to creatinine ratio fatty casts with "maltese cross" sign Nephrotic Syndrome Type Pathophysiology Renal Biopsy Treatment and Notes Focal segmental glomerulosclerosis Podocyte injury or decreased glomerular filtration barrier integrity Light microscopy segmental scarring Treat underlying etiology in secondary causes Steroid therapy Can be secondary to HIV sickle cell disease heroin abuse interferon treatment Minimal change disease Unclear but may be due to an immune-related mechanism Light microscopy normal appearing Electron microscopy effacement of the foot processes Steroid therapy Most common in children May follow recent infection, immunizations, or may be idiopathic Membranous nephropathy Antibody-immune complex deposition IgG antibodies target podocyte antigens or antigens in close proximity to the podocytes Complement-mediated podocyte injury Light microscopy glomerular basement membrane thickening Immunofluoresence immune complex deposition leading to granular appearance Electron microscopy "spike and dome" subepithelial deposits Immunosuppressive therapy in primary cases steroids and cyclophosphamide Most common cause of primary nephrotic syndrome in Caucasian adults Primary causes antibodies targeting phospholipase A2 receptors Secondary causes medications systemic lupus erythematosus nonsteroidal anti-inflammatory drugs gold penicillamine hepatitis B and C infection Amyloidosis Amyloid deposits in the mesangium Electron microscopy apple-green birefringence on Congo red stain under polarized light Treatment involves addressing the plasma cell dyscrasia Diabetic glomerulonephropathy Glomerular hyperperfusion and hyperfiltration result in albumin leaking under these conditions the glomerulus responds via glomerular basement membrane thickening due to non-enzymetic glycosylation hypertrophy sclerosing podocyte injury Light microscopy expansion of the mesangium Kimmelstiel-Wilson lesions Angiotensin converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) Adequately controlling diabetes
QUESTIONS 1 of 16 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 Previous Next Lab Values Blood Hematologic Cerebrospinal Sweat, Urine, and BMI Blood, Plasma, Serum Reference Range ALT 8-20 U/L Amylase, serum 25-125 U/L AST 8-20 U/L Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL Calcium, serum (Ca2+) 8.4-10.2 mg/dL Cholesterol, serum Rec: < 200 mg/dL Cortisol, serum 0800 h: 5-23 μg/dL //1600 h: 3-15 μg/dL 2000 h: ≤ 50% of 0800 h Creatine kinase, serum Male: 25-90 U/LFemale: 10-70 U/L Creatinine, serum 0.6-1.2 mg/dL Electrolytes, serum Sodium (Na+) 136-145 mEq/L Chloride (Cl-) 95-105 mEq/L Potassium (K+) 3.5-5.0 mEq/L Bicarbonate (HCO3-) 22-28 mEq/L Magnesium (Mg2+) 1.5-2.0 mEq/L Estriol, total, serum (in pregnancy) 24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL 28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL Ferritin, serum Male: 15-200 ng/mLFemale: 12-150 ng/mL Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL Female: premenopause: 4-30 mIU/mL midcycle peak: 10-90 mIU/mL postmenopause: 40-250 pH 7.35-7.45 PCO2 33-45 mmHg PO2 75-105 mmHg Glucose, serum Fasting: 70-110 mg/dL2-h postprandial:<120 mg/dL Growth hormone - arginine stimulation Fasting: <5 ng/mLProvocative stimuli: > 7ng/mL Immunoglobulins, serum IgA 76-390 mg/dL IgE 0-380 IU/mL IgG 650-1500 mg/dL IgM 40-345 mg/dL Iron 50-170 μg/dL Lactate dehydrogenase, serum 45-90 U/L Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL Female: follicular phase: 5-30 mIU/mL midcycle: 75-150 mIU/mL postmenopause 30-200 mIU/mL Osmolality, serum 275-295 mOsmol/kd H2O Parathyroid hormone, serume, N-terminal 230-630 pg/mL Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L Phosphorus (inorganic), serum 3.0-4.5 mg/dL Prolactin, serum (hPRL) < 20 ng/mL Proteins, serum Total (recumbent) 6.0-7.8 g/dL Albumin 3.5-5.5 g/dL Globulin 2.3-3.5 g/dL Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h Thyroxine (T4), serum 5-12 μg/dL Triglycerides, serum 35-160 mg/dL Triiodothyronine (T3), serum (RIA) 115-190 ng/dL Triiodothyronine (T3) resin uptake 25%-35% Urea nitrogen, serum 7-18 mg/dL Uric acid, serum 3.0-8.2 mg/dL Hematologic Reference Range Bleeding time 2-7 minutes Erythrocyte count Male: 4.3-5.9 million/mm3Female: 3.5-5.5 million mm3 Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/hFemale: 0-20 mm/h Hematocrit Male: 41%-53%Female: 36%-46% Hemoglobin A1c ≤ 6 % Hemoglobin, blood Male: 13.5-17.5 g/dLFemale: 12.0-16.0 g/dL Hemoglobin, plasma 1-4 mg/dL Leukocyte count and differential Leukocyte count 4,500-11,000/mm3 Segmented neutrophils 54%-62% Bands 3%-5% Eosinophils 1%-3% Basophils 0%-0.75% Lymphocytes 25%-33% Monocytes 3%-7% Mean corpuscular hemoglobin 25.4-34.6 pg/cell Mean corpuscular hemoglobin concentration 31%-36% Hb/cell Mean corpuscular volume 80-100 μm3 Partial thromboplastin time (activated) 25-40 seconds Platelet count 150,000-400,000/mm3 Prothrombin time 11-15 seconds Reticulocyte count 0.5%-1.5% of red cells Thrombin time < 2 seconds deviation from control Volume Plasma Male: 25-43 mL/kgFemale: 28-45 mL/kg Red cell Male: 20-36 mL/kgFemale: 19-31 mL/kg Cerebrospinal Fluid Reference Range Cell count 0-5/mm3 Chloride 118-132 mEq/L Gamma globulin 3%-12% total proteins Glucose 40-70 mg/dL Pressure 70-180 mm H2O Proteins, total < 40 mg/dL Sweat Reference Range Chloride 0-35 mmol/L Urine Calcium 100-300 mg/24 h Chloride Varies with intake Creatinine clearance Male: 97-137 mL/minFemale: 88-128 mL/min Estriol, total (in pregnancy) 30 wks 6-18 mg/24 h 35 wks 9-28 mg/24 h 40 wks 13-42 mg/24 h 17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 hFemale: 2.0-8.0 mg/24 h 17-Ketosteroids, total Male: 8-20 mg/24 hFemale: 6-15 mg/24 h Osmolality 50-1400 mOsmol/kg H2O Oxalate 8-40 μg/mL Potassium Varies with diet Proteins, total < 150 mg/24 h Sodium Varies with diet Uric acid Varies with diet Body Mass Index (BMI) Adult: 19-25 kg/m2 Calculator ( ) xy AC 7 8 9 ÷ 4 5 6 × 1 2 3 - 0 . = + Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.RL.88) A 57-year-old male with diabetes mellitus type II presents for a routine check-up. His blood glucose levels have been inconsistently controlled with medications and diet since his diagnosis 3 years ago. At this current visit, urinalysis demonstrates albumin levels of 250 mg/day. All prior urinalyses have shown albumin levels below 20 mg/day. At this point in the progression of the patient’s disease, which of the following is the most likely finding seen on kidney biopsy? Review Topic QID: 101042 1 Normal kidney biopsy, no pathological finding is evident at this time 0% (0/19) 2 Glomerular hypertrophy with slight glomerular basement membrane thickening 26% (5/19) 3 Glomerular basement membrane thickening and mesangial expansion 37% (7/19) 4 Kimmelstiel-Wilson nodules and tubulointerstitial fibrosis 26% (5/19) 5 Significant global glomerulosclerosis 11% (2/19) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (M1.RL.84) A 6-year-old girl presents to your clinic two weeks after receiving a routine immunization in preparation for a trip overseas. Periorbital edema is present on exam, and 24 hour urine collection shows excretion of 4.3 grams of protein/day. Which pathological change would likely be seen on microscopy? Review Topic QID: 101038 1 Linear IgG deposition on light microscopy 5% (2/38) 2 IgA-immune complexes in the mesangium on electron microscopy 13% (5/38) 3 “Tram-track” appearance on light microscopy 5% (2/38) 4 Subepithelial deposits with “spike and dome” appearance on electron microscopy 11% (4/38) 5 Podocyte effacement on electron microscopy 61% (23/38) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 5 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.RL.70) A 7-year-old boy suffers from generalized edema. Urine protein excretion is 5.2 g over 24 hours, and serum analysis reveals hyperlipidemia. The patient responds to treatment with prednisone, and, 8 weeks later, his urine does not contain measurable protein. If a kidney biopsy had been performed while the patient’s condition was pathologic, which of the following would you expect to find upon glomerular electron microscopy? Review Topic QID: 101024 1 Effacement of podocyte foot processes 83% (101/122) 2 Subepithelial ‘spike and dome’ deposits 6% (7/122) 3 Subepithelial humps 3% (4/122) 4 Thin glomerular basement membrane 6% (7/122) 5 Subendothelial thickening 1% (1/122) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 1 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.RL.59) A 52-year-old African American male with a history of HIV and obesity presents to his primary care physician because of recent changes to his health. He states that while he has been compliant on his HIV medication, he has noticed recent fatigue and increased swelling in his face and legs. A routine urinalysis demonstrates proteinuria and fatty casts. Based on these findings, the physician decided to proceed with a kidney biopsy. Which of the following figures most likely represents the findings of this patient's kidney biopsy? Review Topic QID: 104506 FIGURES: A B C D E 1 Figure A 8% (6/79) 2 Figure B 59% (47/79) 3 Figure C 14% (11/79) 4 Figure D 10% (8/79) 5 Figure E 6% (5/79) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 2 (M1.RL.74) A 57-year-old female visits her primary care physician with 2+ pitting edema in her legs. She takes no medications and does not use alcohol, tobacco, or illicit drugs. 4.5 grams of protein are collected during 24-hour urine excretion. A kidney biopsy is obtained. Examination with light microscopy shows diffuse thickening of the glomerular basement membrane. Electron microscopy shows subepithelial spike and dome deposits. Which of the following is the most likely diagnosis: Review Topic QID: 101028 1 Minimal change disease 0% (0/22) 2 Postinfectious glomerulonephritis 0% (0/22) 3 Focal segmental glomerulosclerosis 5% (1/22) 4 Rapidly progressive glomerulonephritis 5% (1/22) 5 Membranous glomerulopathy 91% (20/22) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (M1.RL.24) An 11-year-old boy presents to your office with pitting edema and proteinuria exceeding 3.5g in 24 hours. You suspect that this patient has experienced a loss of polyanions in his glomerular basement membranes. Which of the following findings would confirm your diagnosis? Review Topic QID: 100978 1 WBC casts in the urine 6% (4/70) 2 RBC casts in the urine 6% (4/70) 3 Selective albuminuria 80% (56/70) 4 Negatively birefringent crystals in the urine 0% (0/70) 5 Bence-Jones proteinuria 7% (5/70) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (M1.RL.19) A 55-year-old African American male presents to his primary care physician with complaints of persistent back pain and fatigue over 12 months. Physical examination reveals a blood pressure of 190/150 mm Hg, and laboratory tests reveal hyperlipidemia and a serum creatinine level of 3.0 mg/dL. 4.5 g of protein are excreted in the urine over 24 hours. Renal biopsy shows eosinophilic, acellular material in the glomerular tuft and capillary walls that display apple green-colored birefringence in polarized light upon Congo red tissue staining. The patient most likely suffers from which of the following: Review Topic QID: 100973 1 Membranous nephropathy 12% (2/17) 2 Focal segmental glomerular sclerosis 6% (1/17) 3 Drug-induced acute tubular necrosis 0% (0/17) 4 Multiple myeloma 82% (14/17) 5 Malignant hypertension 0% (0/17) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (M1.RL.8) A 5-year-old female presents to your office with periorbital edema and proteinuria. Her mother reports that she "just got over a cold" last week. On examination, you note a pulse rate of 70/minute and a blood pressure of 95/53. Which protein(s) would likely be found in large amounts in this patient's urine? Review Topic QID: 100962 1 IgG 7% (11/163) 2 IgA 10% (16/163) 3 Albumin 53% (87/163) 4 Albumin and IgG 16% (26/163) 5 Albumin, IgG, and IgA 12% (20/163) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (M1.RL.81) A 67-year-old man presents to your office with a chief complaint of constipation and many other perturbing minor medical concerns. He reports tiring easily, which he attributes to old age and years of persistent pain in his back and ribs. A complete blood count shows low hemoglobin and elevated serum creatinine. A peripheral blood smear shows stacks of red blood cells among other findings, and serum electropheresis reveals an abnormal concentration of protein resulting in a spike. Which of the following additional findings would you expect to see in this patient? Review Topic QID: 101035 1 Early satiety and splenomegaly 17% (4/24) 2 Smudge cells on peripheral smear 8% (2/24) 3 An elevated PSA and a nodular prostate 0% (0/24) 4 Bence-Jones proteins in the urine 75% (18/24) 5 No additional findings - normal aging explains symptoms 0% (0/24) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 4 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
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