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32 Cards in this Set
- Front
- Back
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Tubulointerstitial disease |
commonly there is involvement of the tubules and intersitium in renal disease can be primary or secondary may acct for up to 25% of kidney disease |
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anatomic relationships |
intersitium is the connective tissue, few cells, capillaries which derive from efferent arterioles, capillaires supply tuubules from more than more thanhpron loss of one glom can result in injury to multiple surrounding nephrons |
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Urinalysis |
acute inflammation (acute intersitial nephrtis or pyelonephritis) WBCs, WBC casts, bacteriuria with infection acute tubular necrosis = epithelial cells, granular casts chronic tubulointersitial disease = normal or mildly abnormal often little proteinuria in general |
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General clinical manifestations |
defect in proximal tubule absorption defect in distal nephron absorption leading to Na wasting, hyperkalemia, metabolic acidosis |
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mechanisms of injury |
bacterial infection; ascending infection from bladder, vesicoureteral reflux can cause spread of bladder infection to kidney spread through bloodstream which is far less common reflux nephropathy; infants may have repeated episodes of infection which are not diagnosed due to the reflux, can lead to loss of functioning renal parenchyma, termed chronic pyelonephritis |
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Chronic pyelonephritis |
chronic damage due to recurrent infection of the kidnye occurs in patients who have major anatomic abnormalities, vesicoureteral reflux most common, obstruction, renal calculi clinical presentation = usually presents with urinary tract infection |
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Dx |
ultrasound or CT which shows, decreased renal size, blunting of calyces, segmental cortical scanning |
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Acute interstitial nephritis |
immunologically mediated disease characterized by inflammatory infiltrates in the intersitum pathogenesis; usually hypersensitivty reaction to drug or infectious agent, shown to involve both cell mediated immunity or antibody mediated immunity |
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Chronic intersitial nephritis |
progressive scarring of tubulointersitium; tubular atrophy, macrophage and lymphocyte infiltration, interstitial fibrosis importance of tubulointersitial fibrosis: correlates best with renal function preduct outcome in all renal diseases |
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Analgesic abuse of nephropathy |
incidence variable according to difference in analgesic consumption; US 2% of cases of ESRD, australia up to 20% early diagnosis impt becasue progressive renal failure may be stopped by discontinuing drugs association with transitional cell cancer of UT |
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Pathogenesis of analgesic abuse |
usually association with ingestion of phenactin and aspirin- not aspirin alone can be seen with other NSAIDs in general requires ingestion of 3 kg drug takes years to develop metabolites accumulate in the medullary intersitium generate free radical which cause cell injury leads to medullary and papillary necoris |
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Myeloma kidney |
path; malignant plasma cells produce monoclonal intact globulins and free light chains only light chains are nephrotoxic light chains cause renal failure by tubular obstruction and direct tubular toxicity |
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clinical kidney myeloma |
weakness, bone pain, anemia, lytic bone lesions, hypercalcemia, renal dysfunction dx demonstration of paraprotein in serum or urine, confirmation of plasma cell dyscrasia on bone marrow clinical course; dependent on response of malignant plasma cells to chemo, slowly progressive renal failure if poor response |
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Polycystic kidney disease |
systemic inherited disaes equally affecting males and females which is characterized by bilateral renal cysts hereditary type has two major types ADPKF and ARPKD prevalence with worldiwde distribution, occurs in 1 in 400 to 1 in 1000 birhts |
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ADPKD genetics |
autosomal dominant disorder that can be casued by mutations in at least 2 different genes short arm chromosome 16 is type 1 chromosome 4 is type 2 |
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PKD1 |
85% of dominant cases protein product is polycystin 1 460 kD with several membrane spanning regions, extracellular domains analgous to those found in cell to cell or cell to matrix adhesion proteins |
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PKD2 |
15% of dominant protein product polycystin 2 topology and stucture suggest it is a cation channel, type 1 and 2 interact directly |
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Hypothesis of PKD |
PKD1 proteins are receptors to regulate the channels of PKD2 genes 2 proteins are part of a conserved signaling mechanism in whihc translocation of ions serves as teh second message plat a role in the funciton of the normal ciliuum which maintains the differentiated phenotype of tubular cells |
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second hit hypothesis |
ADPKD; every kidney cell has mutation yet cysts occur in less than 5% of nephrons due to maybe? haploinsufficiency dominant negatie mutation second hit needded |
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Birth of a cyst |
tubular epithelium proliferates abrnomally to generate the wall around the cyst end up with expanding sac of fluid this is associated with changes in the surrounding extracullular matrix and renal intersitium |
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Characterisitics of ADPKD |
disease begins in utero both kidneys are affected kidney size varies widely irigin of the cysts, is an outpouching of the kidney tubule and occurs in less than 5% of tubules |
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Dx of ADPKD |
primary criteria; many fluid filled cysts scattered through both kidneys; age less than 30 expect 2 ore more cysts in each kidney, age greater than 30 but less than 60 2 or more cysts in each kidney, more than 60 expect 4 or more ycsts definite history of PKD infailty negative ultrasound cant rule anything out until after the age of 30 family history positive 80% of the time, may not be obvious because not all of those affected are dx can have genetic linkage analysis to screen if cysts arent apparent |
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Secondary criteria for ADPKD dx |
polycycstic liver 60% cerebral artery aneurysms 5-10% pancreatic cysts 10% renal insufficiency |
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physical exma |
palpable kidneys increased abdominal girth hepatomegally hypertrension in more than 50% |
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clinical manifestation: renal |
renal cysts 100% hematuria 40% proteinuria 33% renal pain 60% infections are common, increased renal production impaired conc. in 100% |
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clincal: extrarenal |
hypertension75% hepatic cysts 60% colonic diverticuli 80% intracranial aneurysms without FH 5% with FH 20% heart valve abrnomalities 25% pancreatic cysts 10% splenic cysts 5% |
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MGMT of PKD |
pain is the most common symptom, due to cyst enlargement, bleeding, or stones hematuria, due to cyst rupture, stones, infection, malginancy GN conservative mgmt usually effetive infection; UTIs are more common in women and elderly, UA, cultrue and determine if due to obstruction use antibiotics nephrolithiasis; due to mechanical and metabolic fators, uric acid is the most common then Ca oxalate, treatment ESWL |
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Mgmt of extrarenal mainfestaiton |
hypertension; 50-75% of patients with normal renal function, more than 80% of those with renal failure, RAS important contributer, treat with salt restriction ACE i diuretics hepatic cysts, cytsitc dilatations of bile ducts, liver function is the same cerbral aneurysms; most common in those with severe HTN under 50, headache nausea and vomiting presenting symptoms, routine sceeening and surgery for lesions |
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Counseling |
need to have consent before diagnosits eval due to socioeconomic consequences, no curative treatment dont screen until about 18 adults without symtpoms should be advised |
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Treatmnet |
nothing primary trials of vasopressin receptor antagonist reduced intracellular conc. of cAMP by targeting the V2 receptor aggressive treatment of HTN treat infections |
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Outcomes |
ESRD PKD1 20% by 50 50% by 60 60-75 by 75 PKD2 50% at age 70 survival on dialysis better than with other causes of ESRD, transplant is highly successful |
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Medullary sponge kidney |
relatively common disorder characterized by dilated medullary papillary collecting ducts that give the medulla a spongy appearance defect in pyramids, less than 5% is familial, patients usually develop with calcium stones clinical manifestation = nephrolithiasis, hematuria, infection, dx with intravenous pyelogram |
