Week 2 Flashcards
underlying etiology of glomerulonephropathies?
damage to GBM
findings in nephritic syndrome?
PHAROH proteinuria (mild, <3.5 g/d) hematuria azotemia (abn high levels of nitrogen containing compounds such as BUN, Cr) red blood cell casts oliguria HTN ssxs: edema, possible rash and/or heart murmur
causes of nephritic syndrome?
post-infectious: GABHS –> PSGN
autoimmune: goodpasture’s, SLE, Henoch-schonlein purpura, Guillain-Barre, amyloidosis, Wegener’s
primary KD dz: IgA nephropathy, Berger’s dz
ddx of nephritis syndrome?
cirrhosis/liver failure, severe HTN, AIN, RCHF, DM, hemolytic-uremic syndrome
how to differentiate glomerular from urologic bleeding? urine color, RBC morphology, renal fxn
glomerular urine color will be dark red, brown, cola-colored, RBCs will be dysmorphic, renal fxn is reduced
urologic urine color will be bright red, RBCs will be isomorphic, renal fxn will be normal
general w/u for nephritis?
retinal exam for HTN changes, hearing test (Alport’s), skin rash (SLE, Fabry dz, vasculitis, Henoch-Schonlein), jt changes (collagen vascular dz)
labs: 24 hr urine protein, serum Cr, serum complement, anti-DNA, ANCA, c-ANCA, p-ANCA, anti-GBM, HBV, HCV, CXR, echo, renal U/S
post infectious GN is what type of hypersensitivity reaction?
type III
ag-ab complexes lodged in GBM podocytes leads to complement acitvation
prior ssxs of GABHS infxn followed by rash, fever, confusion, HTN, periorbital edema, hematuria, HA, N/V, malaise
dx of post infectious GN?
UA: cola-colored urine, RBCs, WBCs, RBC casts (PATHOGNOMONIC!) present, proteinuria <3.5
CMP: increase in BUN & Cr, mild decrease in GFR
streptozyme test fro anti-streptolysin, anti-hyaluronidase, anti-streptokinase, anti-nicotinamide-adenine dinucleotidase, anti-DNAse B and complement
light microscopy shows thickened GBM
tx for post infectious GN?
tx infxn if present treat edema or HTN limit protein and sodium bed rest anti-inflammatories: curcumin, boswellia, quercetin, bromelain antimicrobials: echinacea antioxidants: vit C, Vit E, taraxacum constitutional hydro or wet sheet wrap
complication with untreated post infectious GN?
MAY LEAD TO NEPHROTIC SYNDROME, AKI, HTN ENCEPHALOPATHY
when do you need to refer a post infectious GN pt?
fluid overload present or unresponsive to therapy, refractory HTN, worsening renal fxn via Cr levels
main DDX of post infectious GN?
rapidly progressing GN - cresenteric GN, most commonly caused by drugs
auto immune causes of GN?
ANCA associated
anti-GBM GN and Goodpasture’s syndrome
subtypes of ANCA associated GN
ANCA = necrotizing GN with GU sxs (hematuria and proteinuria) as well as either
Wegener’s granulomatosis - bleeding respiratory tract nodules, hemoptysis, crackles, skin, eye, sinuses affected
OR
Churg-Strauss syndrome - asthma w/eosinophilia which can lead to RPGN
asthma with eosinophilia
Can lead to RPGN. Considered “pauci-immune” since vasculitis shows little sign of hypersensitivity on immunofluorescence. Work-up includes: CBC (eosinophilia) ANCA titer, IgE levels—inc, Chest CT, PFTs, bronchoaveolar lavage
is what condition?
autoimmune GN specifically Churg-Strauss syndrome
bleeding respiratory tract nodules—hemoptysis, crackles– skin, eye, sinuses. Work-up includes: ANCA titers, Chest CT, bronchoaveolar lavage is what condition?
Wegener’s granulomatosis
anti-GBM GN and Goodpasture’s syndrome is associated with what? presents w/what? need to run what titers to differentiate from Wegener’s?
influenza A infxn, hydrocarbon exposure or HLA-DR2 ag
presents w/pulmonary hemorrhage, dyspnea, hemoptysis, crackles, edema, HTN
have to run ANCA to differentiate from Wegener’s
assoc with influenza A infection, hydrocarbon solvent exposure or HLA-DR2 antigen. Concomitant pulmonary hemorrhage—dyspnea, hemoptysis, crackles—and renal symptoms (edema, HTN). Type II hypersensitivity reaction. Often idiopathic. Possible death by aspiration of blood.
Work-up includes: Anti-GBM titers, Renal biopsy
May have Wegener’s as well, thus ANCA titers run
TX with steroids, chemo, plasma exchange.
is what condition?
anti-GBM GN and Goodpasture’s syndrome
what is the MC nephritic syndrome worldwide?
primary KD dz - IgA nephropathy (Berger’s dz)
what is IgA nephropathy (Berger’s dz)?
IgA deposition in the glomerular mesangium w/mesangial proliferation
unk etiology, can be assoc w/ celiac, HBV, alcoholic cirrhosis, sarcoidosis, HIV, SLE, RA, Sjogren’s
ssxs of IgA nephropathy?
1 or recurrent episodes of gross hematuria < 5 d after viral or bacterial URI or gastroenteritis, flank pn, mild fever, HTN
how to dx IgA nephropathy?
UA: proteinuria, RBC and casts, WBCs
serum Cr mb elevated
serum IgA increased (only in 50% of cases)
KD bxs: if persistent proteinuria >1g/d - see IgA deposits on mesangium
progressive IgA nephropathy seen when?
increased serum Cr
HTN > 140/90
persistent proteinuria >1 g/d
Tx for IgA nephropathy?
if mild, monitor GF diet artemesia fish oil saccharomyces boulardii cordyceps sinesis perilla frutescens rheum palmatum
ages and sex: All ages, 2:1 male nephrotic syndrome in 10-20% HTN in 70% ARF in 50% (transient) latent period 1-3 wk labs: ASO titers immunogenetics: HLA-B12 light microscopy: Diffuse proliferation prognosis: 95% resolve spont tx: Supportive
PSGN
ages and sex: 15-35 yo, 2:1 male nephrotic syndrome Rare HTN 30-50% ARF is rare follows viral syndromes labs: Serum IgA (50% cases) immunogenetics: HLA-Bw light microscopy: Focal proliferation prognosis: Slow progression in 25-50% tx: ACEi, ARB, steroids if resistant
IgA nephropathy
age and sex: 15-30 yo, 6:1 male nephrotic syndrome: Rare HTN: Rare ARF: 50% pulmonary hemorrhage labs: Positive anti GBM ab immunogenetics: HLA-DR2 light microscopy: focal diffuse c crescents prognosis: 75% stabilize or improve with tx early tx: Plasma exchange, steroids
Goodpasture’s
age and sex: Mean 58 yr, 2:1 male nephrotic syndrome: 10-20% HTN: 25% ARF: 60% fast progression, drug hx? labs: Positive ANCA immunogenetics: None light microscopy: Cresentic GN progression: 75% stabilize or improve with tx early tx: steroids
RPGN
general 10 tx ideas for nephritic syndrome?
- avoid high sodium, high potassium foods; low protein in diet, low antigen diet
- incorporate immune amphoterics (ganoderma, grifola, tinospora, artemesia)
- diuretics with edema
- fish oil
- treat HTN
- probiotics
- remove allergens
- quit smoking, limit/no EtOH
- maintain healthy wt
- conventional approach (drugs)
5 tx options specifically for IgA nephropathy?
- fish oil
- saccharomyces boulardii
- cordyceps
- perilla
- rheum palmatum
worst prognosis with nephritis syndrome?
many forms of acute GN can progress to chronic glomerulonephritis which is IRREVERSIBLE which can lead to CKD and ESRD
nephrotic syndrome is result of what?
end result of variety of diseases that damage the glomerular filtration barrier leading to protein wasting, increased permeability of glomerular capillaries and lipids can pass through as well
key findings of nephrotic syndrome?
severe proteinuria >3.5 g/d on 24 hr urine collection which leads to decreased oncotic P and ultimately edema HTN oliguria edema foamy urine cough, exertional dyspnea
RFs and secondary causes of nephrotic syndrome?
poorly controlled DM, IgA nephropathy, nephritic conditions in general, SLE, amyloidosis, HIV, pre-eclampsia, drugs, snake bite, CA, FHx of congenital KD dz
ddx nephrotic syndrome?
CHF, liver failure, ATN, PN, malignant HTN, multiple myeloma
pneumonic for ssxs associated with multiple myeloma?
CRAB calcium (elevated) renal failure anemia bone lesions
5 nephrotic syndromes?
minimal change dz (lipoid nephrosis)
focal semental glomerulosclerosis
membranous nephropathy
membranoproliferative glomerulonephritis
MC nephrotic syndrome present in kids <10 yo?
minimal change dz
MC nephrotic syndrome in young adults, African Americans?
focal segmental glomerulosclerosis
MC nephrotic syndrome in white adult pts?
membranous nephropathy
not specifically MC for any subpopulation
membranoproliferative glomerulonephritis
etiology: Unclear in most cases. Can occur after URI or immunization
In adults may be assoc with drugs (NSAID, antibiotics, lithium) Hodgkin’s dz, Syphilis, TB, Allergy
symptoms: No to mild HTN, edema
signs/history: Hematuria,
proteinuria
Light microscopy: mild mesangial prolif and few deposits
Electron microscopy:
epithelial foot process flattened
minimal change dz
etiology: 1°: Idiopathic :can be rapid onset
2°: Heroin, lithium, NSAIDs HIV, Parvovirus B19, obesity, vesicoureteral reflux, sickle cell symptoms: HTN, edema
signs/history: Hematuria,
proteinuria
Light microscopy: segments of mesangial collapse sclerosis, hyaline deposits
prognosis: Poor outcome
focal segmental glomerulosclerosis
etiology: Idiopathic
Hodgkin’s, SLE, malignancy, HBV, HCV, syphilis, malaria, penicillamine, Captopril, NSAIDs mercury and gold symptoms: HTN, edema,
Complications:
Renal vein thrombosis
signs/history: Elec microscopy: immune complex deposits (IgG or IgM) on thickened capillary loops
Spike and Dome appearance
membranous nephropathy
etiology: SLE, RA, HCV, HBV
Variant: C3 GN—C3 deposits: hematuria after URI symptoms: Insidious, mild HTN, may progress to RPGN
signs/history: Hematuria, hypocomplementemia
Diffuse thickening (hypercellularity) of glomerular capillary wall with Ig deposits
membranoproliferative glomerulonephritis
secondary diseases that can cause nephrotic dz?
lupus diabetic nephropathy amyloidosis infective endocarditis associated GN HBV, HCV HIV-associated nephropathy multiple myeloma related KD dz
is a rare, x-linked deficiency of alpha-galactosidase A (a-Gal A) leading to accumulation of glycolipids in blood vessels. In kidney, causes proteinuria, renal insufficiency, and renal failure. SSx also in joints, skin, eyes, heart, and GI. IV enzyme replacement given.
Fabry dz - DDX of nephrotic syndrome
is an inherited collagen (Type IV) synthesis defect leading to GBM thinness and dysfunction, as well as sensorineural hearing loss and ocular lesions (eg cataracts). Presents with hematuria possibly after a URI, then can progress with worsening proteinuria/edema/HTN/oliguria to ESKD
Rare, more severe and persistent in males
Alport syndrome - is a DDX of nephrotic syndrome
is an immune-mediated (IgA deposits) systemic vasculitis common in children 3-8 yrs. Purpura, arthralgias, abdominal pain and renal disease (hematuria, proteinuria, inc creatinine). Skin biopsy reveals vasculitis. Resolvable
Henoch-Schonlein purpura - is a DDX of nephrotic syndrome
treatment considerations for nephrotic syndrome?
immunosuppressive for primary dz: steroids, abx, immunologics anti-HTN anti-hyperlipidemic anti-coagulants limit dietary protein and sodium remove allergens fish oil anti-inflammatory, anti-oxidant, immune amphoteric, renal protective herbs constitutional hydro control DM
specific tx options for FSGS?
CoQ10 and ganoderma
specific tx options for MPGN?
ALA, Vit E, rosmarinic acid
specific tx for membranous nephropathy?
astragalus
Inflammation of the renal interstitium, from cell-mediated immune response binding to interstitial proteins, leading to a decrease in renal function. Interstitial compartment infiltrated by T-cells, monocytes, and plasma cells
acute interstitial nephritis
categories of AIN?
drug hypersensitivity infections tubulointerstitial nephritis-uveitis syndrome related to systemic AI dz idiopathic
general presentation of AIN?
acute onset of dec in renal fxn days to 2 wks post-administration of infection or drugs
symptoms: rash, fever, hematuria, oliguria, N/V, malaise, flank pain, arthralgai (uveitis in TINU)
signs: decreased urine concentration, decreased GFR
dx of AIN?
UA: hematuria, mild to mod proteinuria, high WBCs, WBC casts, eosinophiluria may be present CBC: eosinophilia inc serum BUN and Cr FENa >1% indicating tubular damage bx if persistent sxs
AIN management and tx considerations?
normal renal fxn usu returns w/discont of suspected causative agent
low protein, low K, low Na diet
anti-inflammatories, antioxidants
Renafood
alternative natural tx for conditions treated w/offending agentes
pharmacologic (short dose of prednisone)
some pts may require dialysis
Patchy focal necrosis of tubule. Lumens fill with casts, cellular debris (Tamm-Horsfall mucoprotein)
acute tubular necrosis - can be due to toxins (drugs - nephrotoxins) or ischemia
3 forms of ATN?
contrast induced nephropathy
cisplatin induced nephropathy
lithium nephropathy
possible presentation of ATN?
uremic pruritus, pericardial friction rub, asterixis, HTN, edema, oliguria
ATN tx considerations?
correct ischemic cause or remove drugs, manage ARF
chelation tx may be needed once sxs managed if dt heavy metals
NAC to prevent radiation nephropathy
general protectives: silybum, gingko, cordyceps, urtica, CoQ10, selenium, vit C
green tea to protect
specific tx options for cisplatin induced ATN?
lipoic acid, NAC, selenium, quercetin
Symptoms and signs: nocturia, uremia sx, small kidneys, hyperkalemia, reduced SG,
Hyperchloremic metabolic acidosis from:
1. Reduced ammonia production
2. Inability to acidify the distal tubules
3. Proximal tubule bicarbonate wasting
chronic tubulointerstitial disease
5 forms of acute tubulointerstitial disease?
prolonged obstructive uropathy reflux neuropathy analgesic nephropathy lead nephropathy cadmium nephropathy
Causes: renal stone, prostate dz, carcinoma of cervix, colon, bladder
SSX: in partial obstruction urine output alternates from polyuria (vasopressin insensitivity) and oliguria (dec GFR)
Azotemia and HTN
Hematuria or pyuria, but often benign UA
obstructive uropathy - chronic tubulointerstitial dz
“Vesicoureteral reflux” VUR
congenital incompetent vesicoutereral sphincter allows retrograde flow during voiding leads to inflam
SSX: HTN, polyuria, nocturia, renal insufficiency, hx of recurrent UTI
Renal scarring can lead to proteinuria, Na wasting, metabolic acidosis and CKD.
Pelvic ultrasound
Voiding cystourethrography VCUG can visualize ureteral dilatation
Prophylactic Ab and surgery
reflux neuropathy - chronic tubulointerstitial dz
Acetaminophen—reactive metabolites bind to interstitial cells causing necrosis
NSAIDs decrease medullary blood flow and decrease glutathione
Aspirin—unknown mechanism
SSX: many are asx, found incidentally
polyuria, HTN, flank pain, malaise, dyspepsia
Lab: hematuria, non-nephrotic proteinuria, anemia, inc creatinine, low SG
Sloughed papillae can be found in the urine late in the disease
CT: small kidneys, papillary calcifications
analgesic neuropathy - chronic tubulointerstitial dz
Pb: filtered by glomerulus and transported across the proximal convoluted tubules and accumulated. Progressive tubular atrophy and interstitial fibrosis
See hyperuricemia, HTN
Multisystem involvement—GI, Neuro, hemo
X-ray fluorescence shows bone accumulation
Lead nephropathy - chronic tubulointerstitial dz
Deposits cause tubular dysfunction leading to proteinuria, glucosuria, increase in urinary cadmium
cadmium nephropathy - chronic tubulointerstitial dz
management and tx of chronic tubulointerstitial dz?
prevent renal scarring if early stages - treat the cause
tubular dysfxn may require K and Ph restriction, Na, Ca and bicarb supplementation
chelation therapy for heavy metals
natural analgesics, HP and physical medicines for chronic pain syndromes
anti-inflammatories (turmeric, boswellia, bromelain)
renal protectives: nettle seed, salvia miltiorrhiza
renal anti-oxidants: ginseng, coptis, vaccininum, quercetin, Vit C, ALA
fish oil
Damaged proximal tubule leads to wasting of those products normally reabsorbed there—thus phosphaturia, glucosuria, albuminuria, uricosuria, bicarbonate wasting.
The condition may be familial or acquired (eg. assoc w multiple myeloma, lead or cadmium intoxication, antiretroviral drugs, tetracycline).
SSx: Presents as osteomalacia in adults, hypophosphatemic rickets in kids, polyuria, polydipsia.
Tx: Phosphate supplementation, vit D
Faconi syndrome - type of RTA
results in hyperchloremic metabolic acidosis
Causes include heredity, autoimmune disease (Sjogren’s, Lupus, RA), drugs/toxins (toluene, lithium, ibuprofen, amphotericin B, lead), heavy metals, chronic obstruction.
SSX: urinary stone formation (calcium oxalate, uric acid), bone demineralization, hypokalemia, poor growth in kids
Tx: sodium bicarb or sodium citrate, potassium citrate, thiazide diuretic may be needed
renal tubular acidosis
Granulomatous deposits caseate and can rupture into the tubular lumen, can lead to calcifications and progressive medullary injury. Scarring, abscess, fibrosis occurs
Complications: urinary obstruction, hydronephrosis, recurrent UTI
Early signs are pyuria without positive culture on routine media, hematuria, mild proteinuria
X-ray show calcifications
CT show caseous masses
renal tuberculosis
Inherited (autosomal recessive) defect in thick ascending loop:
Low SG, hypercalciuria, hypokalemia, metabolic alkalosis, normal BP
In kids, presents as polyuria, polydipsia, dehydration, growth and mental retardation.
Treated with dietary Na, K, ACE inhibitors, Anti-inflammatories
bartter syndrome
Inherited (autosomal recessive) defect in the distal convoluted tubule leading to excess excretion of sodium, magnesium, chloride and potassium.
Presents with fatigue, ms cramps, polyuria, HTN
Labs: hypochloremic metabolic acidosis, hypokalemia, hypocalciuria, hypomagnesmia
Gitelman syndrome
Very rare. Autosomal dominant defect in tubules. Presents as severe and/or refractory HTN, metabolic alkalosis from hypokalemia, hypoaldosteronism
Liddle syndrome - pseudoaldosteronism
Nephropathy induced by consuming aristocholic acid containing herbs. Nephrotoxin produces interstitial fibrosis with atrophy and loss of tubules in renal cortex, and renal insufficiency. Most cases in Eastern Europe and China
AA nephropathy
Congenital (x-linked) or Acquired (eg chronic lithium use, amphotercin-B, PN, PKD) defect to renal tubular responsiveness to ADH.
SSx—polyuria, polydipsia, failure to thrive, hypernatremia, dehydration, urine concentration <200 Osm/kg, SG<1.005.
TX with hydration, low sodium, low protein diet, chlorothiazide
nephrogenic diabetes insipidus
