Snapshot A 14-year-old boy presents with hematuria, problems with vision, and deafness. Approximately 2 weeks prior to symptom development, he recovered from a viral upper respiratory tract infection. Family history is significant for deafness and early initiation of lisinopril in his father. His temperature is 99°F (37.2°C), blood pressure is 140/90 mmHg, pulse is 75/min, and respirations are 18/min. Physical examination is signifcant for anterior lenticonus and sensorineural hearing loss. Urinalysis demonstrates dysmorphic red blood cells and red blood cell casts. (Alport syndrome) Introduction Clinical definition renal disease secondary to an inflammatory process injuring the glomerulus this results in damage involving the basement membrane capillary endothelium mesangium Presentation symptoms hypertension hematuria oliguria headache physical exam edema can be peripheral and/or periorbital Diagnosis studies complete blood cell count anemia may be noted azotemia complement levels C3, C4, and CH50 should be obtained urinalysis dysmorphic red blood cells (RBCs) suggests hematuria is of glomerular origin RBC casts subnephrotic range proteinuria (< 3.5 g/day) if the nephritic syndrome is severe enough it can lead to nephrotic range proteinuria (> 3.5 g/day) renal biopsy may be necessary to arrive to a definitive diagnosis and to determine prognosis Nephritic SyndromeTypePathophysiologyRenal BiopsyDiagnostic Studies and TreatmentAcute poststreptococcal glomerulonephritisGlomerulonephritis secondary to nephritogenic strains of streptococcustype III hypersensitivity reactionLight microscopyglomerular hypercellularitye.g.,polymorphonuclear leukocytesImmunofluorescencediffuse granular pattern on glomerular capillary walls and mesangiumdeposition of IgG, IgM, and C3Electron microscopyelectron-dense, glomerular subepithelial immune-complex deposits ("humps")Streptococcus titers and serologies are positive↓ serum C3 levelsTypically self-resolvesRapidly progressive glomerulonephritisGoodpasture syndrometype II hypersensitivityanti-GBM antibodies against α3-chain of collagen type IVantibodies to the alveolar basement membrane result in hemoptysis and lung diseasePauci-immune processesgranulomatosis with polyangiitismicroscopic polyangiitisLight microscopy and immunofluorescencecrescent-shaped deposition offibrin, C3b, glomerular parietal cells, monocytes, andmacrophagesGranulomatosis with polyangiitisPR3-ANCA/c-ANCA positiveMicroscopic polyangiitisMPO-ANCA/p-ANCACorticosteroids and cyclophosphamideIgA nephropathy (Berger's disease)IgA immune-complex deposition in glomerular mesangial cells that results in its proliferationpatients present with hematuria and upper respiratory tract or gastrointestinal infectionLight microscopymesangial proliferationImmunofluorescenceIgA immune-complex deposition in the mesangiumACE inhibitor or ARB for proteinuria and hypertensionAlport syndromeCollagen type IV mutation that results in an abnormal basement membranemore commonly an X-linked dominant disordercharacterized byrenal involvementocular involvementsensorineural hearing lossElectron microscopyglomerular basement membrane lamellation"basket weave" appearance No curative treatmentMembranoproliferative glomerulonephritis (MPGN)Immune-complex and/or complement protein deposition in the mesangium and subendothelium of the capillariesthis results in proliferation of the mesangium and remodeling of the capillary wallmay be secondary tohepatitis C virushepatitis B virusC3 nephritic factorMPGN type IILight microscopymesangial proliferation and thickening of the capillary wallImmunofluorescence"tram-track" appearance↓ serum C3 and C4 levelsTreat underlying disease in secondary causese.g., control of hepatitis C virus infectionDiffuse proliferative glomerulonephritis (DPGN)> 50% of glomeruli (thus diffuse) demonstrates an inflammatory process on histologyCommon in patients with systemic inflammatory diseases (e.g., systemic lupus erythematosus)can be secondary tomembranoproliferative glomerulonephritisLight microscopycapillary"wire-looping"Electron microscopyimmune complex depositionaffecting thesubendothelial regionmaybe intramembranousImmunofluorescencegranularappearanceCan lead to death in patients with systemic lupus erythematosusAggressively treat with steroids
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 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.15.4671) A 62-year-old man presents to his primary care doctor complaining of recent-onset hemoptysis. He has not had any fevers, night sweats, or weight loss. He recently traveled to Italy with his wife. He has a 5 pack-year smoking history. On review of systems, he reports that his urine has been “red-tinged” for several months. Urinalysis reveals the findings shown in Figure A. He is referred to a nephrologist and undergoes a renal biopsy. Immunofluorescence staining of the biopsy is shown in Figure B. Which of the following is the most likely underlying pathogenesis of his disease? QID: 107162 FIGURES: A B Type & Select Correct Answer 1 Type III hypersensitivity reaction 10% (18/176) 2 Antibodies to collagen type IV 66% (116/176) 3 Presence of C-ANCA 15% (27/176) 4 Mycobacterial infection 1% (2/176) 5 Metastatic malignancy 4% (7/176) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (M1.RL.13.67) A 21-year-old male presents to your office with hematuria 3 days after the onset of a productive cough and fever. Following renal biopsy, immunofluorescence shows granular IgA deposits in the glomerular mesangium. Which of the following do you suspect in this patient? QID: 101021 Type & Select Correct Answer 1 Lipoid nephrosis 2% (2/95) 2 Berger’s disease 77% (73/95) 3 Poststreptococcal glomerulonephritis 16% (15/95) 4 Systemic lupus erythematosus 3% (3/95) 5 HIV infection 1% (1/95) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (M1.RL.13.32) A 9-year-old Caucasian girl presents to your office with hematuria. An electron micrograph of her renal biopsy is shown below in Figure A. Which of the following is the most likely composition of the structures marked by the white arrows? QID: 100986 FIGURES: A Type & Select Correct Answer 1 Albumin 2% (4/173) 2 Non-enzymatic glycosylation 1% (2/173) 3 IgA 13% (23/173) 4 IgG 11% (19/173) 5 IgG, IgM, C3 71% (122/173) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (M1.RL.13.73) A 25-year-old male visits his primary care physician with complaints of hemoptysis and dysuria. Serum blood urea nitrogen and creatinine are elevated, blood pressure is 160/100 mm Hg, and urinalysis shows hematuria and RBC casts. A 24-hour urine excretion yields 1 gm/day protein. A kidney biopsy is obtained, and immunofluorescence shows linear IgG staining in the glomeruli. Which of the following antibodies is likely pathogenic for this patient’s disease? QID: 101027 Type & Select Correct Answer 1 Anti-DNA antibody 1% (1/111) 2 Anti-neutrophil cytoplasmic antibody (C-ANCA) 10% (11/111) 3 Anti-neutrophil perinuclear antibody (P-ANCA) 4% (4/111) 4 Anti-glomerular basement membrane antibody (Anti-GBM) 82% (91/111) 5 Anti-phospholipid antibody 2% (2/111) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (M1.RL.13.35) A 6-year-old boy presents to your office with hematuria. Two weeks ago the patient had symptoms of a sore throat and fever. Although physical exam is unremarkable, laboratory results show a decreased serum C3 level and an elevated anti-DNAse B titer. Which of the following would you most expect to see on renal biopsy? QID: 100989 Type & Select Correct Answer 1 Large, hypercellular glomeruli on light microscopy 58% (65/112) 2 Polyclonal IgA deposition on immunofluorescence 9% (10/112) 3 Immune complex deposits with a "spike and dome" appearance on electron microscopy 22% (25/112) 4 Wirelooping and hyaline thrombi on light microscopy 3% (3/112) 5 Antibodies to GBM resulting in a linear immunofluorescence pattern 4% (5/112) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (M1.RL.13.85) Three weeks after recovering from pharyngitis, a nine-year-old girl presents with periorbital edema and dark brown urine. An image of the kidney biopsy is shown in Figure A. What is responsible for these changes? QID: 101039 FIGURES: A Type & Select Correct Answer 1 Glomerular basement membrane lamellation 2% (2/81) 2 IgA deposition and subsequent mesangial proliferation 9% (7/81) 3 Neutrophil infiltration in response to immune complex deposition along the glomerulus basement membrane and mesangium 74% (60/81) 4 Loss of negative charge on the glomerular basement membrane 4% (3/81) 5 Widespread intermembraneous deposits due to an autoantibody against C3 convertase 9% (7/81) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (M1.RL.13.11) Multiple patients present to your office with hematuria following an outbreak of Group A Streptococcus. Biopsy reveals that all of the patients have the same disease, characterized by large, hypercellular glomeruli with neutrophil infiltration. Which patient has the best prognosis? QID: 100965 Type & Select Correct Answer 1 65-year-old nulliparous woman 1% (2/180) 2 50-year-old man with a history of strep infection 7% (13/180) 3 8-year-old boy who undergoes no treatment 69% (125/180) 4 38-year-old man with sickle cell trait 3% (6/180) 5 18-year-old man treated with corticosteroids 16% (28/180) M 4 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic
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