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32 Cards in this Set

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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

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

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



General clinical manifestations

defect in proximal tubule absorption




defect in distal nephron absorption leading to Na wasting, hyperkalemia, metabolic acidosis

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

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

Dx

ultrasound or CT which shows, decreased renal size, blunting of calyces, segmental cortical scanning

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

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

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

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

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

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

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

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

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

PKD2

15% of dominant




protein product polycystin 2




topology and stucture suggest it is a cation channel, type 1 and 2 interact directly

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

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

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

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

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

Secondary criteria for ADPKD dx

polycycstic liver 60%


cerebral artery aneurysms 5-10%


pancreatic cysts 10%


renal insufficiency

physical exma

palpable kidneys




increased abdominal girth




hepatomegally




hypertrension in more than 50%

clinical manifestation: renal

renal cysts 100%


hematuria 40%


proteinuria 33%


renal pain 60%


infections are common, increased renal production impaired conc. in 100%

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%

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

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

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

Treatmnet

nothing primary




trials of vasopressin receptor antagonist reduced intracellular conc. of cAMP by targeting the V2 receptor




aggressive treatment of HTN




treat infections

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

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