Renal and Metabolic Disease

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1

glomerular, tubular, interstitial, and vascular

the four types of renal disease

2

glomerular

caused by immune mediated

3

tubular and interstitial

caused by toxic or infectious substance

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vascular

caused by reduction in renal perfusion

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secondary glomerular diseases

systemic diseases that initially and principally involve other organs but also affect kidneys

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primary glomerular diseases

specifically affect kidneys, often only organ involved; several different types

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cellular proliferation

increased numbers of capillary endothelial, mesangial, and epithelial cells in glomerular tuft

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leukocyte infiltration

neutrophils and macrophages attracted by a local chemotactic response

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glomerular basement thickening

any process that results in enlargement of basement membrane immune complexes and diabetes

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hyalinization with sclerosis

accumulation of homogenous eosinophilic extracellular material

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hematuria, proteinuria, oliguria, azotemia, edema, and hypertension

clinical features of glomerular diseases

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heavy proteinuria, hypoproteinuria, hyperlipidemia, lipiduria, edema, and urine microscopic

nephrotic syndrome clinical features

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acute glomerulonephritis, rapid glomerulonephritis, membranous glomerulonephritis, minimal change disease, focal segmental glomerulonephritis, IgA nephropathy, and chronic glomerulonephritis

types of glomerulonephritis

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acute (post streptococcal) glomerulonephritis

commonly caused by streptococcal infection occurring within 1-2 weeks of infection of throat or skin; caused by certain strains of group A beta hemolytic strep that contain M protein

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IgA nephropathy

caused by deposition of IgA in glomerular mesangium; 1-2 days after mucosal infection of respiratory, GI or urinary tract from bacteria and/or viruses

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systemic lupus erythematosus

autoimmune disorder with immune complex deposits and complement activation

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diabetes mellitus

carbohydrate metabolism disorder that leads to glomerular syndrome, hypertension, susceptibility to pyelonephritis

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amyloidosis

systemic disease involving many organs; characterized by deposits of a pathologic protein substance; leads to proteinuria and nephrotic syndrome

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acute tubular necrosis

includes ischemic and toxic

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ischemic ATN

seen in sepsis shock and trauma

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toxic ATN

from exogenous or endogenous nephrotoxins

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tubular dysfunction

Fanconi's syndrome, cystinosis and cystinuria, renal glycosuria, renal phosphoturia, and renal tubular acidosis

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Fanconi's syndrome

any condition that presents with a generalized loss of proximal tubular function

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cystinosis and cystinuria

inherited disorders that cause renal tubular dysfunction

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renal glucosuria

excessive glucose excretion despite normal blood glucose levels

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renal phosphaturia

inability of distal tubules to reabsorb inorganic phosphorus

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renal tubular acidosis

inability of tubules to secrete adequate hydrogen ions despite normal GFR

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upper urinary tract infections

casts commonly seen

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lower urinary tract infections

casts not common, usually leukocyturia and bacteruria

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acute pyyelonephritis

bacterial infection that involves renal tubules, interstitial and the renal pelvis; caused from ascending UTI infection from gram negative organism

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chronic pyelonephritis

persistent inflammation of renal tissue causes permanent scarring of renal calyces and renal pelvis

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acute interstitial nephritis

any immune response in the interstitial of the kidney commonly caused by kidney rejection

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yeast infections

most commonly caused by Candida species, normal flora in GI tract and vagina

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acute renal failure

sudden decrease in glomerular filtration rate, azotemia, and oliguria; high mortality rate; prerenal or post renal; functional abnormality but no apparent cellular changes

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chronic renal failure

progressive loss of renal function; due to hypertrophy of remaining healthy nephrons; not clinically recognizable until 80-85% of renal function is lost; azotemia, acid base imbalance, abnormal calcium and phosphate metabolism; fixed specific gravity of 1.010, proteinuria, hematuria, and presence of casts (waxy and broad)

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calculi "stones"

aggregates of mineral salts in a matrix of proteins and lipids; 75% contain calcium

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supersaturation of chemical salts in urine, optimal urinary pH, urinary stasis, and nucleation or original crystal formation

four factors influencing calculi formation

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urea

must be eliminated from the body and is done through the kidneys

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aminoacidurias

an imbalance of urea in the body

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primary diseases

inborn errors of indicators of metabolism

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secondary aminoacidurias

induced by severe liver disease or from renal tubular dysfunction

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cystinosis

inherited disease; cystine is deposited throughout the body and accumulates causing damage and disrupting cellular functions

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cystinuria

inherited disorder; nephrons are unable to reabsorb amino acids and excrete large amounts of cystine and other amino acids

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maple syrup urine disease (MSUD)

rare inherited disease in which branched chain amino acids (leucine, isoleucine, and valine) accumulate in the blood, CSF, and urine; has distinctive caramelized sugar odor; can lead to mental impairment or spasticity in infants if not treated

45

phenylketonuria (PKU)

inherited disease characterized by increased urinary excretion of a ketone acid; can lead to severe mental impairment; mousy or musty odor of urine, sweat and breath; also have decreased skin pigmentation; all infants screened

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alkaptonuria, tyrosinuria, and melanuria

amino acid metabolism disorders

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diabetes mellitus

problems with glucose metabolism; chronic hyperglycemia and glycosuria; glomerular damage and chronic renal failure

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galactosemia

inherited disorder in which an enzyme in the galactose metabolic pathway is deficient or defective; type 1 GALT most common form; screened for in newborns and tested for with reducing substances tablet test

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diabetes insipidus

decreased ADH hormone or nephrons are resistant to ADH; results in polyuria; unable to produce concentrated urine even when fluids are restricted

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porphyrias

hereditary defects of the heme synthesis pathway; increased porphyrins and porphyrin precursors in blood and urine; turns urine reddish color