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 SyndromeTypePathophysiologyRenal BiopsyTreatment and NotesFocal segmental glomerulosclerosisPodocyte injury or decreased glomerular filtration barrier integrityLight microscopysegmental scarringTreat underlying etiology in secondary causesSteroid therapyCan be secondary toHIVsickle cell diseaseheroin abuseinterferon treatmentMinimal change diseaseUnclear but may be due to an immune-related mechanismLight microscopynormal appearingElectron microscopyeffacement of the foot processesSteroid therapyMost common in childrenMay follow recent infection, immunizations, or may be idiopathicMembranous nephropathyAntibody-immune complex depositionIgG antibodies target podocyte antigens or antigens in close proximity to the podocytesComplement-mediated podocyte injuryLight microscopyglomerular basement membrane thickeningImmunofluoresenceimmune complex deposition leading to granular appearanceElectron microscopy"spike and dome"subepithelial depositsImmunosuppressive therapy in primary casessteroids and cyclophosphamideMost common cause of primary nephrotic syndrome in Caucasian adultsPrimary causesantibodies targeting phospholipase A2 receptorsSecondary causesmedicationssystemic lupus erythematosusnonsteroidal anti-inflammatory drugsgoldpenicillaminehepatitis B and C infectionAmyloidosisAmyloid deposits in the mesangiumElectronmicroscopyapple-green birefringence on Congo red stain under polarized lightTreatment involves addressing the plasma cell dyscrasiaDiabetic glomerulonephropathyGlomerular hyperperfusion and hyperfiltration result in albumin leakingunder these conditions the glomerulus responds viaglomerular basement membrane thickeningdue to non-enzymetic glycosylationhypertrophysclerosingpodocyte injuryLight microscopyexpansion of the mesangiumKimmelstiel-Wilson lesionsAngiotensin 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 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 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.15.59) 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? QID: 104506 FIGURES: A B C D E Type & Select Correct Answer 1 Figure A 8% (12/154) 2 Figure B 55% (85/154) 3 Figure C 15% (23/154) 4 Figure D 9% (14/154) 5 Figure E 10% (15/154) M 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (M1.RL.15.84) 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? QID: 101038 Type & Select Correct Answer 1 Linear IgG deposition on light microscopy 3% (4/127) 2 IgA-immune complexes in the mesangium on electron microscopy 10% (13/127) 3 “Tram-track” appearance on light microscopy 6% (8/127) 4 Subepithelial deposits with “spike and dome” appearance on electron microscopy 6% (8/127) 5 Podocyte effacement on electron microscopy 71% (90/127) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.RL.15.81) 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? QID: 101035 Type & Select Correct Answer 1 Early satiety and splenomegaly 10% (11/111) 2 Smudge cells on peripheral smear 11% (12/111) 3 An elevated PSA and a nodular prostate 9% (10/111) 4 Bence-Jones proteins in the urine 67% (74/111) 5 No additional findings - normal aging explains symptoms 2% (2/111) M 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.RL.13.74) 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: QID: 101028 Type & Select Correct Answer 1 Minimal change disease 0% (0/84) 2 Postinfectious glomerulonephritis 6% (5/84) 3 Focal segmental glomerulosclerosis 6% (5/84) 4 Rapidly progressive glomerulonephritis 4% (3/84) 5 Membranous nephropathy 83% (70/84) M 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (M1.RL.13.88) 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? QID: 101042 Type & Select Correct Answer 1 Normal kidney biopsy, no pathological finding is evident at this time 3% (2/64) 2 Glomerular hypertrophy with slight glomerular basement membrane thickening 11% (7/64) 3 Glomerular basement membrane thickening and mesangial expansion 31% (20/64) 4 Kimmelstiel-Wilson nodules and tubulointerstitial fibrosis 47% (30/64) 5 Significant global glomerulosclerosis 5% (3/64) M 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (M1.RL.13.70) 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? QID: 101024 Type & Select Correct Answer 1 Effacement of podocyte foot processes 86% (173/202) 2 Subepithelial ‘spike and dome’ deposits 4% (8/202) 3 Subepithelial humps 2% (5/202) 4 Thin glomerular basement membrane 4% (8/202) 5 Subendothelial thickening 1% (3/202) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (M1.RL.13.24) 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? QID: 100978 Type & Select Correct Answer 1 WBC casts in the urine 5% (7/150) 2 RBC casts in the urine 7% (10/150) 3 Selective albuminuria 78% (117/150) 4 Negatively birefringent crystals in the urine 4% (6/150) 5 Bence-Jones proteinuria 5% (7/150) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (M1.RL.13.19) 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: QID: 100973 Type & Select Correct Answer 1 Membranous nephropathy 15% (11/74) 2 Focal segmental glomerular sclerosis 15% (11/74) 3 Drug-induced acute tubular necrosis 1% (1/74) 4 Multiple myeloma 64% (47/74) 5 Malignant hypertension 4% (3/74) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic
All Videos (1) Login to View Community Videos Login to View Community Videos Membranoproliferative Glomerulonephritis Thomas Heineman Renal - Nephrotic Syndrome D 11/4/2012 159 views 5.0 (6) Renal | Nephrotic Syndrome Renal - Nephrotic Syndrome Listen Now 18:5 min 6/29/2021 134 plays 0.0 (0)