Lecture 19 - Renal Disease 1 Flashcards
what are glomeruli?
network of capillaries between afferent arteriole (bringing blood to the capillary bed) and efferent arteriole (drains blood away from capillary bed)
what does the capillary wall consist of?
endothelium, basement membrane, and epithelium (lining the urinary space)
what part of the glomeruli connects adjacent processes (called the slit diaphragm) and is important in preventing proteinuria?
membrane
the capillary wall is permeable to water and small molecules and impermeable to _____ and _____ proteins
impermeable to ALBUMIN and LARGER proteins
the capillaries are supported by connective tissue called what?
mesangium
what part of the kidney resorbs some substances and secretes other substances, eventually forming urine
tubular epithelium
what part of the kidney is formed by collagen and blood vessels between the tubules and glomeruli
interstitium
the ______ arterioles supply the capillary bed around some of the tubules (vasa recta)
efferent arterioles
*Absence of blood flow through the glomeruli reduces oxygen delivery to the tubules
what is azotemia?
elevation of the blood urea nitrogen (BUN) and creatine levels, due to decreased filtration of blood through the glomeruli (decreased glomerular filtration rate)
what is uremia?
association of azotemia with clinical signs and symptoms, including gastroenteritis, peripheral neuropathy, pericarditis, dermatitis, hyperkalemia, and metabolic acidosis
what is acute nephritic syndrome?
results from glomerular injury and is characterized by acute onset hematuria, mild to moderate proteinuria, azotemia, and hypertension
what is nephrotic syndrome?
glomerular syndrome characterized by heavy proteinuria (>3.5 grams per day), hypoalbuminemia, severe edema, hyperlipidemia, and lipiduria
what is acute renal failure?
acute onset of azotemia with oliguria (or anuria)
what is the prevalence of autosomal dominant (adult) polycystic kidney disease?
1 out of every 500-1000 people
what are the two examples given of congenital cystic renal disease?
- autosomal dominant (adult) polycystic kidney disease
2. Autosomal recessive (childhood) polycystic kidney disease
what is the clinical presentation of autosomal dominant (adult) polycystic kidney disease?
characterized by multiple expanding cysts in both kidneys
-Gradual onset of renal failure in adult, urinary tract hemorrhage (hematuria), pain, hypertension, UTI
what is the etiology of autosomal dominant (adult) polycystic kidney disease?
defective gene is PKD1 (in 90% of families) located on chromosome 16
*The gene encodes of polycystin-1
in autosomal dominant (adult) polycystic kidney disease, what ratio of patient have cysts in the liver and aneurysms may develop where?
1/3 of patients have cysts in liver
aneurysms may develop in the CIRCLE OF WILLIS (intracranial)
in regard to the pathology of autosomal dominant (adult) polycystic kidney disease, up to how large can the kidneys get and what arises in every part of the tubular system?
-very large (up to 4 kg) kidneys with numerous cysts that arise in every part of the tubular system
what is the prevalence of autosomal recessive (childhood) polycystic kidney disease?
1 in 20,000 live births
what is the clinical presentation in autosomal recessive (childhood) polycystic kidney disease?
renal failure develops from infancy to several years of age - it is rare
autosomal recessive (childhood) polycystic kidney disease is due to mutations in what gene?
PKHD1 gene
what is the extrarenal pathology of autosomal recessive (childhood) polycystic kidney disease?
almost all have liver cysts and progressive liver fibrosis
what is the pathology in autosomal dominant (adult) polycystic kidney disease?
numerous small uniform-size cysts from collecting tubules in cortex and medulla
what are the mechansisms of glomerular injruy in glomerular diseases?
- Immune complex deposits in glomerular basement membrane (GBM) of mesangium. These may result from circulating immune complexes that deposit in the glomerulus or circulating antibodies directed against glomerular components or non-glomerular antigens “planted” in the glomerulus
- Epithelial and endothelial cell injury
what are the 3 pathologic evaluations of kidney biopsies?
- light microscopy
- immunogluorescence
- electron microscopy
what is the most common cause of nephrotic syndrome in children?
minimal change disease
*Good response to treatment (esp. in children)
what is the pathology in minimal change disease
normal-appearing glomeruli by light microscopy
- no immune complexes
- electron microscopy demonstrates effacement of epithelial cell foot processes
what is the pathology of focal and segmental glomerulosclerosis?
- Partial (segmental) sclerosis of some (focal) glomeruli characterized by increased mesangial matrix collagen with obliteration of capillary loops
- The idiopathic form ha no immune complexes
- It is one of the most common causes of nephrotic syndrome in adults
- may be primary (idiopathic) or secondary to other glomerular diseases, loss or scarring of other glomeruli, or genetic
focal and segmental glomerulosclerosis has poor response to ______ treatment - renal failure in 50% of people after 10 years
corticosteroid
membranous nephropathy (glomerulonephritis) is most common in what age group?
most common in adults age 30-50