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 Syndrome Type Pathophysiology Renal Biopsy Diagnostic Studies and Treatment Acute poststreptococcal glomerulonephritis Glomerulonephritis secondary to nephritogenic strains of streptococcus type III hypersensitivity reaction Light microscopy glomerular hypercellularity e.g., polymorphonuclear leukocytes Immunofluorescence diffuse granular pattern on glomerular capillary walls and mesangium deposition of IgG, IgM, and C3 Electron microscopy electron-dense, glomerular subepithelial immune-complex deposits ("humps") Streptococcus titers and serologies are positive ↓ serum C3 levels Typically self-resolves Rapidly progressive glomerulonephritis Goodpasture syndrome type II hypersensitivity anti-GBM antibodies against α3-chain of collagen type IV antibodies to the alveolar basement membrane result in hemoptysis and lung disease Pauci-immune processes granulomatosis with polyangiitis microscopic polyangiitis Light microscopy and immunofluorescence crescent-shaped deposition of fibrin, C3b, glomerular parietal cells, monocytes, and macrophages Granulomatosis with polyangiitis PR3-ANCA/c-ANCA positive Microscopic polyangiitis MPO-ANCA/p-ANCA Corticosteroids and cyclophosphamide IgA nephropathy (Berger's disease) IgA immune-complex deposition in glomerular mesangial cells that results in its proliferation patients present with hematuria and upper respiratory tract or gastrointestinal infection Light microscopy mesangial proliferation Immunofluorescence IgA immune-complex deposition in the mesangium ACE inhibitor or ARB for proteinuria and hypertension Alport syndrome Collagen type IV mutation that results in an abnormal basement membrane more commonly an X-linked dominant disorder characterized by renal involvement ocular involvement sensorineural hearing loss Electron microscopy glomerular basement membrane lamellation "basket weave" appearance No curative treatment Membranoproliferative glomerulonephritis (MPGN) Immune-complex and/or complement protein deposition in the mesangium and subendothelium of the capillaries this results in proliferation of the mesangium and remodeling of the capillary wall may be secondary to hepatitis C virus hepatitis B virus C3 nephritic factor MPGN type II Light microscopy mesangial proliferation and thickening of the capillary wall Immunofluorescence "tram-track" appearance ↓ serum C3 and C4 levels Treat underlying disease in secondary causes e.g., control of hepatitis C virus infection Diffuse proliferative glomerulonephritis (DPGN) > 50% of glomeruli (thus diffuse) demonstrates an inflammatory process on histology Common in patients with systemic inflammatory diseases (e.g., systemic lupus erythematosus) can be secondary to membranoproliferative glomerulonephritis Light microscopy capillary "wire-looping" Electron microscopy immune complex deposition affecting the subendothelial region maybe intramembranous Immunofluorescence granular appearance Can lead to death in patients with systemic lupus erythematosus Aggressively 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? Tested Concept QID: 107162 FIGURES: A B Type & Select Correct Answer 1 Type III hypersensitivity reaction 12% (16/135) 2 Antibodies to collagen type IV 67% (91/135) 3 Presence of C-ANCA 13% (18/135) 4 Mycobacterial infection 1% (2/135) 5 Metastatic malignancy 2% (3/135) M 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review tested concept (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? Tested Concept QID: 101021 Type & Select Correct Answer 1 Lipoid nephrosis 2% (1/44) 2 Berger’s disease 75% (33/44) 3 Poststreptococcal glomerulonephritis 18% (8/44) 4 Systemic lupus erythematosus 2% (1/44) 5 HIV infection 2% (1/44) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review tested concept (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? Tested Concept QID: 100986 FIGURES: A Type & Select Correct Answer 1 Albumin 3% (4/126) 2 Non-enzymatic glycosylation 1% (1/126) 3 IgA 15% (19/126) 4 IgG 11% (14/126) 5 IgG, IgM, C3 67% (85/126) M 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review tested concept (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? Tested Concept QID: 100965 Type & Select Correct Answer 1 65-year-old nulliparous woman 1% (1/143) 2 50-year-old man with a history of strep infection 8% (12/143) 3 8-year-old boy who undergoes no treatment 68% (97/143) 4 38-year-old man with sickle cell trait 3% (4/143) 5 18-year-old man treated with corticosteroids 16% (23/143) M 4 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept (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? Tested Concept QID: 100989 Type & Select Correct Answer 1 Large, hypercellular glomeruli on light microscopy 45% (26/58) 2 Polyclonal IgA deposition on immunofluorescence 17% (10/58) 3 Immune complex deposits with a "spike and dome" appearance on electron microscopy 26% (15/58) 4 Wirelooping and hyaline thrombi on light microscopy 2% (1/58) 5 Antibodies to GBM resulting in a linear immunofluorescence pattern 5% (3/58) M 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review tested concept (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? Tested Concept QID: 101027 Type & Select Correct Answer 1 Anti-DNA antibody 0% (0/63) 2 Anti-neutrophil cytoplasmic antibody (C-ANCA) 10% (6/63) 3 Anti-neutrophil perinuclear antibody (P-ANCA) 2% (1/63) 4 Anti-glomerular basement membrane antibody (Anti-GBM) 84% (53/63) 5 Anti-phospholipid antibody 2% (1/63) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review tested concept (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? Tested Concept QID: 101039 FIGURES: A Type & Select Correct Answer 1 Glomerular basement membrane lamellation 6% (2/33) 2 IgA deposition and subsequent mesangial proliferation 12% (4/33) 3 Neutrophil infiltration in response to immune complex deposition along the glomerulus basement membrane and mesangium 73% (24/33) 4 Loss of negative charge on the glomerular basement membrane 3% (1/33) 5 Widespread intermembraneous deposits due to an autoantibody against C3 convertase 3% (1/33) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept
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