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ABIM Nep GN & Renal

ABIM Nep GNs & Renal Diseases

QuestionAnswer
Alpha-1 antitrypsin is assoc with which ANCA? anti-PR3 but not anti-MPO
Describe IgG4-related disease. Marked plasma cell infiltrate (tubulointerstitial nephritis) + autoimmune pancreatitis, but all organs affected. IgG4 deposited in the tubules. ++activate complement. May see eosinophilia and low+ANA , MGN, obstruction . pulm and renal masses.
What disease presents with picture of low complements, AIN, renal/lung masses, low grade fever? IgG4-related systemic disease.
What disease presents with picture of pulmonary, eye, skin, and renal involvement, along with renal biopsy findings of a cellular infiltrate and granuloma? sarcoidosis
What disease presents with picture of dry eye, sicca syndrome, and interstitial nephritis (without granuloma) +/- systemic inflammation? autoimmune dz of Sjögren’s syndrome. Usu no glomerular dz.
What disease presents with picture of uveitis + interstitial nephritis (sometimes granulomas), fanconi syn? TINU. idiopathic IAN + ant uveitis usu in adolescent girls.
What disease presents with picture of
von Hippel-Lindau syndrome abnormalities include? cysts in the kidneys, pancreas, and genital tract. increased risk of developing clear cell renal cell carcinoma and pancreatic neuroendocrine tumor & pheochromocytoma
What disease presents with skin papule, cystic kidney disease, possible mental retardation? Tuberous Sclerosis
When do you start IS for IgAN? IgA nephropathy "with persistent proteinuria > 1 g daily, despite 3-6 months of med rx(ACE-I or ARBs & BP control), and GFR > 50 mL/min/1.73 m2, receive a 6-month course of corticosteroid therapy.”
Tumors assoc w tuberous sclerosis? angiomyolipomas. AD inheritance Major bleeding rx’d w/ nephron-sparing surgery
How can mTORs help in tuberous sclerosis? mTOR pathway, may be effective in markedly reducing the volume of target angiomyolipomas.
Thrombotic thrombocytopenic purpura is diagnosed with what lab test? ADAMTS13 activity LESS THAN 5%
Albumin < ___ is the most significant independent predictor of venous thrombotic risk in NS? 2.8 g/dL. Clin J Am Soc Nephrol. 2012;7:43-51
Rx IgAN? 6-mo prednisone for persistent proteinuria >1 g/24 hr after a 3-6 therapeutic trial of RAAS ing & adequate BP control. Nephrol Dial Transplant. 2009; 24:3694-3701
Poor prognostic indicators for IgAN? HTN, impaired GFR at presentation, and proteinuria. J Am Soc Nephrol. 2012;23:1753-1760
Abnormalities of alternative pathway cause what renal issues? C3GN and DDD. Caused by factor H defect/deficiency or antibodies against alt complement pathway (aka C3 nephritic factor). assoc w low C3 and nl C4.
Rx C3GN? Eculizumab. Two recent reports utilizing Ecu for DDD or C3 GN were assoc w stabilization of GFR and improved renal path or less proteinuria in some but not all patients. J Am Soc Nephrol. 2012;23:1229-1237
Nephrotic syndrome in inflammatory bowel disease caused by? Secondary (AA) amyloidosis. Often assoc w/ orthostasis suggesting autonomic neuropathy, hematuria (fragile bladder mucosa),
Renal conditions w Crohn’s? Mesalamine-induced AIN, calcium oxalate stones.
Most common histologic entity associated with adult-onset nephrotic syndrome in Caucasians? Membranous nephropathy
Biopsy findings of membranous GN? LM thickened capillary walls; PAS shows spikes; IF + IgG + complement. EM subepithelial deposits give “spike” appearance.
target for MGN rx? partial remission (<3.5 g/d & 50% reduction from peak proteinuria). Partial remission is assoc w both significant slowing CKD progression & doubling of kidney survival at 10 years
Risk of progression from membranous GN complete remissions in proteinuria occur in 20% to 30%. Low risk for progression if nl GFR & prot <4g. High-risk if proteinuria ≥8 g/d over 3 to 6 mo and/or declining CrCl during this observation period. medium risk if in between.
Rx for primary MGN steroids + cytotoxic (cyclophosphamide, chlorambucil, MMF) for med risk. BUT high risk pt start w/ cyclosporine. Resistant cases-ACTHAR, rituximab,
Rx FSGS prednisone 1 mg/kg max 60 g/d x 3-6 mos; CsA 3-5 mg/kg/d divided BID x 6-12 mos; tacrolimus 0.2-0.3 mg/kg/d divided BID x 6-12 mos; MMF 25-35 mg/kg/d divided BID (max 2g/d) WITH dex. [tac or CsA slow taper]
Distinguish thin BM dz vs Alport. Thin BM dz has GBM that is thin throughout. Alports has some thick/thin areas.
Thin Basement membrane clinical features min proteinuria, hematuria, nl GFR, benign course. May be carrier state of Alport.
Alport clinical features persistent hematuria, proteinuria 1-2g/d, renal failure progressive, deafness, lenticonus, Leiomyomitosis, cognitive impairment, retinopathy. X-linked most common. Carriers look like thin BM.
Sub epithelial deposits on EM are classic of _____ GN. membranous nephropathy, + PLA2R. Decline of anti-PLA2R titer precedes clinical response.
_______ is the first podocytic antigen responsible for human membranous nephropathy (MN) in adults. Thrombospondin type-1 domain-containing 7A (THSD7A). Neutral endopeptidase (NEP) in neonates & cationic bovine serum albumin (cBSA in children.
cANCA pattern associated with ____ PR3 (think c3po). Has higher rate of relapse
pANCA pattern associated with ____ MPO (think c3po)
Microscopic Polyangiitis (MPA) Pauciimune necrotizing vasculitis of small/medium arteries. Associated with pulmonary disease
Hemolytic uremic syndrome without diarrhea is known as _______ atypical HUS. Could be from infections or complement deficiency (acquired/inherited).
T/F alternate pathway is constantly activated at low levels . TRUE. It is usually turned off, but failure to down regulate (by Factor H 1, etc) results in unchecked complement activation.
C3GN previously called MPGN. no IgG deposition or staining but ++C3 stain. Caused by alternative pathway dysregulation from abnormal proteins or antibodies.
C3nephritic factor present + DDD treatment is ______ plasma exchange or infusion, Rituximab
CFH mutation present in DDD treatment is ______ plasma infusion
T/F FSGS tip lesion has worst prognosis. False since has best prognosis
T/F FSGS collapsing lesion has worst prognosis TRUE. Maybe idiopathic or secondary to viral infections, medications
T/F FSGS NOS is rare FALSE. It’s most common.
T/F FSGS cellular variant is rare TRUE; it is the least common
Viruses associated w FSGS HIV, parvo B19, HCV, CMV, EBV
Drugs assoc w FSGS pamidronate, heroin, interferon, lithium, calcineurin inh
APOL1 associated with? collapsing FSGS, HIVAN, sickle cell, SLE, focal global sclerosis (HTN). 2 risk variants assoc w 20% chance of developing renal dz.
Genetic testing for FSGS usu not done, but NPHS2pR229Q is a permissive mutation that requires 2cnd mutation (compound heterozygotes) to cause FSGS. esp common in Western Europe.
Nail patella syndrome AD loss of fcn mutation of LMX1B, assos w symmetric nail, skeletal, ocular and kidney disease. Biopsy + “whorling” pattern.
When is genetic testing for FSGS undertaken? children w congenital NS, children w SRNS, familial FSGS, (sopradic FSGS consider R229Q NPHS2 variant)
AL-Type Amyloidosis histology Renal, pul, GI & cardiac involvement most common. definitive dx congo red stain apple birefringence; amorphous eosinophilic nodular infiltrate in glom. IF + smudgy lambda>kappa. EM random oriented fibrils.
Immunotactoid acute tubulopathy looks like? glomerular deposits, Large organized fibril in GBM. IF + either kappa or lamb not both; EM Tubular deposits > 30 nm
LCDD acute tubulopathy looks like? fanconi + AKI + LC crystals (light blue on H&E). EM: LC crystals needle shaped, rectangular, rod-like and electron dense.
T/F Free light chains maybe elevated with low GFR. true
The presence of free light chains indicates a biopsy when ____. kappa/lambda ratio is skewed (ie normal is 0.26 - 1.6). Especially with tubulopathy and AKI.
Rituximab in ANCA-Associated Vasculitis (RAVE) trial showed ritux + steroids had similar results to cyclophosphamide+steroids for induction of remission and may be superior in relapsing disease. Alveolar hemorrhage pts had same outcomes but relapsers fared better in ritux arm. Similar SAE noted.
LCCD histology nodular glomerulsclerosis; IF makes dx staining for kappa>lambda; EM finely granular amorphous deposits
HIV assoc kidney disease? HIVAN, MCD, postinfectious GN, amyloidosis, and IgAN. Coinfection with hepatitis B and C common—> MGN + MPGN
myeloma kidney presents with _______ prox RTA (tubulopathy), acidic urine, usu kappa chains. UA trace pro, + glu, bland sediment. 24 hr urine ++ protein. Labs show nl glucose, low phos
AKI + back pain make you think of? multiple myeloma
ESRD + back pain make you think of? paraspinal abscess from bacteremia
What agent is contraindicated to treat/prevent TLS in G6PD deficient pts? Rasburicase.
T/F Rasburicase.and allopurinol are equally effective to treat TLS. TRUE
Role of allopurinol in TLS? safe drug assuming good kidney function. Lowers further uric acid production but does NOT correct pre-existing hyperuricemia.
T/F alkalinizing urine is recommended for TLS. NO. IV saline is best. watch if patient has AKI or vol overload. Increasing the urine pH decreases UA precipitation that enhances Ca-Ph precipitation.
Histologic features of FSGS? LM shows segmental sclerosis, focal. IF: NO immune complex deposition… but may rarely see IgM in scarred areas. EM: extensive foot process effacement (>80%)
Amyloid path diagnosis? AKI, CKD, + lambda light chains, +SPEP/EPEP, proteinuria, hyperglycemia, NODULES, linear staining on IF for mesangial collagen. congo red +apple-green birefringence
Renal microaneurysms are found in? Polyarteritis nodosa
Polyarteritis nodosa affects which vessels? small and medium sized arteries, especially Reno and visceral, nerves and coronaries… Causes aneurysms that look like tiny bead
Classic EM finding of Alports? is variable thickening, thinning, basket weaving, and lamellation of GBM
Classic EM finding of thin basement membrane disease? GBM Thickness measuring < 200nm
Dense deposit disease is best treated with _____. eculizumab.
The best treatment for C3GN is —-. Rituximab + plasma exchange 
(eculizumab) has been shown to be useful in Rx of DDD, aHUS).
RITUXIMAB has been shown to be useful in RX of___. ANCA vasculitis (induction or relapse per RAVE TRIAL), GPA(Wegener’s), HCV cryoglobulinemia or C3GN, TTP. [Rituximab has not been useful in DDD which is rx'd w ecu or plasma.]

Renal effects of antibodies against epidermal growth factor (cetuximab, panitumumab, matuzumab) renal magnesium wasting in the distal tubule. Hypomagnesemia was associated with hypokalemia and hypocalcemia.
Syndromes that cause hypoMg Barrters, Gitelmans
Created by: ka1usg