Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Renal II - Midterm > Glomerulonphritides > Flashcards

Glomerulonphritides Flashcards

1
Q

Neuroblastoma characteristics

A

ALK gene - anapestic lymphoma kinase

adrenal medulla, sympathetic chain (paravertebral of abdomen, posterior mediastinum, pelvis, neck, brain

invade kidney, venal v., vena cava, envelop aorta

Homer-Wright pseudo rosettes
peripheral halo

Mets: lymph spread; blood to liver, lungs, bone marrow, bones; periorbital (proptosis, ecchymosis)

less than 2, large abdominal mass, fever, weight loss, older kids: mets S/S: bone pain, respiratory symptoms, GI complaints

Bladder/bowel dysfunction

neonates - blueberry muffin baby

Elevated serum catecholamines, elevated urine VMA and HVA

Unfavorable: MYCN amplification, Chr 1q and 11q loss, TRKB expression, mutations of neuritogenesis genes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Staging of NB

A

1: local, complete excision, LN negative
2A: local, incomplete excision, LN negative
2B: local, w/ or w/o complete excision, ipsilateral non adherent LN positive, enlarged contralateral LN
3: unresectable, cross midline, w/ or w/o LN OR local unilateral tumor w/ contralateral LN positive
4: distant LN involvement, bone, bone marrow, liver, skin involvement
4S: local tumor, dissemination limited to skin, liver and/or bone marrow - less than 1 yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Wilms tumor genetics

A

WAGR: 11p13 deletion - WNT1, PAX6

DDS: dominant negative missense - zinc finger of WT1

BWS: 11q15.5 WT2 imprinting - loss of IGF2 imprinting = over expression); CDKN1C (p57 or KIP2) low risk

Beta-catenin - WNT - sporadic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Wilms tumor characteristics

A

Nephrogenic rests

Blastemal: small blue cells
Epithelial: abortive tubules or glomeruli
Stromal: fibrocytic or myxoid, sk.m. differentiation

Anaplasia - T53 mutation - resistance to chemo

Lower pole large expansile tumor

large abdominal mass, unilateral crossing midline, down to pelvis

hematuria, abd pain after trauma, intestinal obstruction, HTN

Pulmonary mets

Poor prognosis: anaplasia, Chr 11q, 16p loss; gain Chr 1q

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

WAGR syndrome

A

Wilms tumor, Aniridia, Genital anomalies, mental Retardation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Denys-Drash Syndrome

A

gonadal dysgenesis
early onset nephropathy leading to renal failure
diffuse mesangial sclerosis
Risk of WT and gonadoblastomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Beckwith-Widemann Syndrome

A 
Organomegaly
macroglossia
hemihypertrophy
omphalocele
adrenal cytomegaly

Risk: WT, hepatoblastoma, pancreatoblastoma, adrenocortical tumors, rhabdomyosarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Post infectious Glomerulonephritis

A

Streptococcal pyogenic exotoxin B (speB)
Types 12, 4, 1 M protein

Elevated Antistreptococcal antibody titers
Decline in C3 concentration
elevated BUN

Granular IgG (IgA in staph) and C3 in GBM and mesagium

Early sub endothelial deposits, complex dissociates, crosses GBM, reforms as sub epithelial humps

nephritic syndrome

abrupt onset 1-2 weeks after sore throat

Adults: sudden HTN, edema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Good pasture syndrome (RPGNI)

A

Anti-GBM COL4-A3 antigen
HLA-DRB1

Diffuse linear IgG and C3
Fibrin crescents

Recurrent hemoptysis (lung hemorrhage)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

RPGNII - immune complex

A

Granular immune complex deposition

influx of leukocytes in glomerular tuft

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

RPGNIII - pauci immune

A

ANCAs
no anti GBM antibodies or immune complexes

c-ANCA - Wegner’s - sinusitis
p-ANCA - asthma and polyangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Chronic Glomerulonephritis

A

90% cresentric GN

Hyalinized glomeruli
Contracted kidneys, thinned cortex, peripelvic fat
atrophy of tubules, irregular interstitial fibrosis

loss of appetite, anemia, V, weakness, edema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Membranous nephropathy

A 
PLA2R antigen
HLA-DQA1
Rising serum creatinine
C5b-C9 MAC- capillary walls leaky
Granular IgG4 and C3 diffuse
Sub epithelial Ig deposits
Effacement of podocyte foot processes
Spikes and dones
Segmental sclerosis
Protein droplets in PT
Nephrotic syndrome

Women- spontaneous remission, benign

SLE: self nuclear proteins and autoAb
Tumors: lung, colon, melanoma
Drugs: NSAIDs, penicillamine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Minimal change disease

A

Angiopoietin-like 4
Loss of foot processes
Nephrotic syndrome - albumine proteinuria
2-6
Follows respiratory infection or immunization
respond to corticosteroids or immunosuppressive tx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Focal Segmental glomerulosclerosis

A

Slit diaphragm protein mutations:
NPHS1: 19q13 - nephron
NPHS2 - 1q25-q31 - podocin - steroid resistance in kids
a-actinin -fast progression to renal insufficiency
TRPC6 - increase Ca2+ influx in podocytes

Focal IgM and C3
Loss of foot processes, focal detachment, epithelial denudation
Lipid droplets and foam cells

HIV, heroin, sickle cell, massive obesity

Risk: APOL1 Ch 22 varient, black

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

HIV associated nephropathy

A

collapsing glomerulopathy: retraction/collapsed glomerular tuft, micro cysts
tubuloreticular inclusions w/in endothelial cells (ER mods by INF-a)

17
Q

Membranoproliferazive glomerulonephritis type I

A

splitting tram track
IgG and C3 granular pattern; C1q and C4
Sub endothelial deposits
MIXED nephrotic and nephritic syndromes

Secondary: chronic antigenemia, HBV, HCV w/ cryoglobinemia, HIV, schistosomiasis, SLE, endocarditis, infected ventriculoatrial shunts, chronic visceral abscesses, CLL, lymphomas, melanoma, a1-antitrypsin

18
Q

Dense deposit disease (MPGNII)

A

Alternative complement pathway
C3NeF: properdin

Decreased serum C3, normal C1 and C4; decreased Factor B and properdin

Factor H mutations

GBM thickening, splitting
C3 granular or linear in GBM - C3 glomerulopathy
Dense deposits permeate GBM - ribbon like
C3 in mesangium

Nephritic syndrome - young
poor prognosis

19
Q

IgA nephropathy

A

Defect O-link glycans to hinge of IgA1
activate alt complement pathway
IgA (IgG, IgM and C3/properidin) in mesangium- NO C1q and C4

coincident URI, GE, abscess

Berger Disease - no systemic

Henoch-Schonlein purpura: - skin (extensor arms, buttock, legs), and pain, V, GI bleed, arthralgia

Gluten enteropathy, liver disease

20
Q

Alport Syndrome

A

AR, AD exist; X-linked

Chr 2 or 13, X: COL4A5 mutations - early ESRD

Moth-eaten, frayed, basket weave GBM and tubular BM, lamination of lamina dense focal thinning

X linked: Ab to A3, A4, A5 don’t stain; A5 in skin missing

Nerve deafness, eye: lens dislocation, posterior cataracts, corneal dystrophy

21
Q

Thin Basement Membrane Lesion (Benign familial hematuria)

A

A3 or A4 COL4 mutations

diffusely thinned GBM -asymptomatic hematuria, mild-mod proteinuria

22
Q

Diabetic nephropathy

A

advanced glycosylation end products

thick GBM and tubular BM
Diffuse mesangial sclerosis
Hemodynamic changes: glomerular hypertrophy leads to nodular glomerulosclerosis

Advanced renal hyaline arteriosclerosis: thick, tortuous AA

End stage: diffuse, granular, pitted surface, thin cortex, irregular cortical depression - pyelo
30% of all ESRD

23
Q

Diffuse Proliferative Lupus Nephritis

A

Sub endothelial “wire loops” and mesangial dense deposits

large cellular glomerulus “stuffed” into Bowman’s capsule

Anti-IgG Ab to mesangial and capillary wall - sub endothelial

Recurrent hematuria
Nephritic/nephrotic

24
Q

Fibrillary Glomerulonephritis

A

amyloid-like fibrils - mesangium, glomerular capillary walls
IgG4, C3 Igk, Iglambda light chain deposits

Nephrotic, hematuria, progressive renal insufficiency

Renal II - Midterm (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions