module 12 intra-renal disorders Flashcards
categories of intra-renal disorders
congenital neoplastic infectious obstructive glomerular
intra-renal disorders occur
primarily within the kidney and have the potential to result in renal insufficiency or failure
common s/s of intra renal disorders
pain: nephralgia
- felt at costovertebral angle (CVA)
- d/t distention/inflammation of renal capsule
Damage to kidney without pain is common d/t dec pain receptors in kidney
Abnormal urinalysis
- foundation for Dx
- dark, strong-smelling urine: dec. renal function
- cloudy pungent urine: infectious process
agenesis
congenital abnormality
- kidneys do not develop in fetus
- bilateral; not compatible with life
- unilateral: compensatory hypertrophy of functional kidney
hypoplasia
congenital abnormality
some fetal kidney development, but kidney is smaller than normal
- can lead to pediatric ESRF
single kidney can maintain normal renal function
cystic kidney disease (polycystic)
congenital abnormality genetically transmitted renal disorder resulting in fluid-filled cysts - can expand and disrupt blood flow - localized or both kidneys - can lead to renal failure, requiring dialysis or transplant 2 types - autosomal recessive form - autosomal dominant more common in men inc. in prevalence with aging
polycystic diseases and genes
both types, genes code for proteins associated with primary cilium on surface of tubule cells
- cilium senses urine flow and linked to regulatory responses, including Ca channels
- mutation -> growth dysregualtion and abnormal remodeling
cystic kidney disease autosomal dominant
chromosome 16, PKD1: 85%
chromosome 4, PKD2: 15%
- reduction of intracellular Ca and excessive concentrations of intracellular cAMP
-> normal functioning tissue reduced
-> many round cysts of varying size
-> other organs can develop cysts: liver
Dec. GFR and ability to concentrate urine
manifests in 40-59 y/o
cysts multiply and expand -> kidney hypertrophy -> dec. GFR., compacts and distorts vascular system -> ischemia -> RAAS activation
cystic kidney disease s/s
early: dec. ability to concentrate urine HTN pain proteinuria hematuria concomitant cystic liver involvement Dx: genetic hx, ultrasound Tx: supportive, control BP and managing associated pathologies
renal cell carcinoma
neoplasm
VHL gene
metastatic disease (clear cell)
originate in renal cortex from cells of proximal convoluted tubule
May have familial pattern, risk inc. with 1st degree relative
staging system I-IV
metastases resistance to radiation, immunotherapy, and chemo.
risk factors for renal cell carcinoma
smoking
obesity
HTN
first degree relative
s/s of renal cell carcinoma
asymptomatic till advanced
CVA tenderness
hematuria
palpable mass
papillary carcinoma
MET gene
Stage I neoplasm
tumor within capsule
Stage II neoplasm
tumor invades perirenal fat
Stage III neoplasm
tumor extends into renal vein or regional lymphatics
Stage IV neoplasm
matastases to common areas
- lungs
- heart
- liver
- other kidney
- bone
nephroblastoma (wilm’s tumor)
5th most common pediatric malignancy
- 65% < 5 y/o
- usually unilateral
- develop from embryonic pluipotent kidney precusor cells (nephrogenic rests)
- genes: WT1, chromosome 11; WT2
Large encapsulated tumors with rapid growth
-> hemorrage, cystic changes, necrosis
-> renal pelvis compression, infiltration into blood
nephroblastoma (wilms tumor) s/s
palpable mass abd. pain HTN hematuria tumor thrombus in inferior vena cave -> dec. venous return and lower extremity edema
normal protective mechanisms against infection
Acidic pH: hostile environment for bacteria
Presence of urea: hostile environment
Men: bacteriostatic prostatic secretions
Women: glands in distal urethra secrete mucous
Micturition: wash out pathogens: reflex to expel urine
Unidirectional urine flow: prevents reflux
Epithelial cells: physical barrier
pyelonephritis
infection of kidney - renal tubules, pelvis, calculi most common cause - ascending infection from the lower urinary tract -- vesicoureteral reflux -- intrarenal reflux other cause: hematogenous infection
pyelonephritis causative agents
E. coli enterobacter enterococcus proteus mirabilis klebsiella staph strep
risk factors for UTI
inc. age vesicoureteral reflux congenital anatomoic anomalies female pregnancy diaphragms with spermicidal agents for contraception neurogenic bladder instrumentation of urinary tract urinary obstuction glucocorticoids, radiation, chemo immunodeficiency DM obesity sickle cell trait
acute pyelonephritis
infection of renal pelvis/parenchyma usually from ascending UTI
Unlikely to occur without obstruction or ureteral reflux
Dx: presence of WBC casts indicative of upper UTI
Tx: abx
