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Define agenesis
- Complete failure of an organ to develop
- Unilateral renal 1 in 1000 boys and left more common
- Usually asymtomatic
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Define hypoplasia
- Organ does not develop to normal size
- Unilateral more common
- Secondary common true rare
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Define dysgenesis
Failure of an organ to develop properly
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Cite the effect of urinary obstruction in the fetus
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Describe the inheritance, pathology, and manifestation of the different types of polycystic kidney disease
- Dom (adult) most common
- Type I dom pkd1 gene 85%
- Type II dom pkd2 gene 15%
- Dominant pckd in every segment of the nephron
- RECESSIVE - 75% fatal 1st yr
- Cystic dilation of CT, PKHD1 gene
- Recessive pckd in collecting ducts
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How often do abnormality of the kidneys ureters occur and what are the most common?
- 3-4% of newborns
- Anomolies of shape and position are most common
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What is the progression of the fetal kidneys?
- Develop in fifth week
- Function 8th week, urine 9-12w
- Urine main part of amniotic fluid
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What is oligohydramnios?
Small amount of amniotic fluid due to nonfunctioning or obstructed fetal kidneys. Compresses fetus and can cause impaired lungs
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What is potters syndrome?
- Low widely seperated ears nose broad and flat chin receding limb defects
- Related to oligohyramnios
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What is the mostcommon cause of an abdominal mass in newborns?
Unilateral multicystic renal dysplasia
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What is nephronophthisis-medullary cystic disease complex?
- Cysts at cortical medullary junction in dct
- 10-25% of renal fail in peds
- Impaired urine concentration
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What are simple and aquired renal cysts?
- Simple common usually asymptomatic
- Aquired in end stage renal failure on dialysis
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List four common causes of urinary tract obstruction
Most common is urinary calculi
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Define the term hydronephrosis and relate it to the destructive effects of urinary tract obstruction
- Urine filled dilation of renal pelvis and calices
- Caused by urinary obstruction
- Acute cause pain chonic doesnt but both destroy kidney
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Describe the role of urine supersaturation, nucleation, and inhibitors of stone formation in the development of kidney stones
- Supersaturated urine has high concentrations of stone components
- A nucleation or nidus of crystal calcium oxalate begin formation
- Inhibitors such as magnesium, citrate, and tamm-horsfall mucoprotein may bind calcium
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Describe methods used in the diagnosis and treatment of kidney stones
- Symptoms of pain, radiology, urinalysis, iv pyelography, ultrasound
- treat for pain, <5mm should pass, strain urine to test,
- Uteroscopic, percutaneous, or extracorpreal lithtripsy less invasive
- Large may need open surgery
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What is the difference between upper/lower urinary tract obstruction?
Above or below uterovesical junction
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Cite organisms responsible for UTIs and why catheters, obstructions, and reflux predispose to infections
- E. Coli - most uncomplicated lower UTI but could be S. Saprophyticus
- complicated UTIs - gram neg rods (Proteus Mirabilis, Klebsiealla, Enterobacter, Psuedomonas A.) and gram pos cocci (S. Aureus)
- uti usually comes up from urethra
- reflux in interupted voiding, coughing, squatting,
- catheter most common gram neg septicemia
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List three physiologic mechanisms that protect against UTIs
- washout phenomenon, perstalic ureters
- mucin lining of bladder
- IgA and phagocytic blood cells
- normal flora of periurethral in women
- prostate secrections in men
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Describe the signs and symptoms of UTIs
- acute cystitis - frequent urination, lower ab or back discomfort, burning and pain on urination, ocassional urine foul smelling and cloudy
- symptoms usually cease within 48 hrs of tx
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Describe the factors that predispose UTIs to children, sexually active women, pregnant women, and older adults
- CHILDREN - female short urethra, fecal proximity
- WOMEN - sex pushs bacteria up, spermicide can kill flora
- PREGNANT - dilated upper urinary, decreased ureter peristalsis, displaced bladder
- OLDER - decreased mobility, decreased bactericidal, obstructions, bladder ischemia
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Compare the manifestations of UTIs in different age groups including infants toddlers teens adults and older
- INFANTS - commonly upper UTI (pyelonephritis), bacteremia, septicemia, fever, hypothermia, apnea, abdominal distention, diarrhea, vomiting, lethargy, irritability
- TODDLERS - abdominal pain, vomiting, diarrhea, abnormal voiding patterns, foul smelling urine, fever, and poor growth
- TEENS - enuresis, freq, dysuria, suprapubic discomfort, unexplained fever
- ADULTS - same but usually no fever
- OLDER - typical sx may be absent, may have freq urgent incontent w/o UTI
- vauge sx - anorexia, fatigue, weakness, changed in mental status
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Cite measures used in the diagnosis and treatment of UTIs
- UTI diagnosed by symptoms and positive urine culture. gram stain to differentiate. nitrites and leukocyte esterace less sensitive,
- usually TMP-SMX for acute lower but e.coli becoming resistant.
- find and treat cause of chronic (obstruction, reflux)
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Describe the two types of immune mechanisms involved in glomerular disorders
- 1-injury from antibody reaction to fixed glmerular antigens or antigens planted within the glomerulus
- 2-injury from circulating antigen-antibody complex trapped in glomerular membrane
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User the terms proliferation, sclerosis, membranous, diffuse, focal, segmental, and mesangial to explain changes in glomerular structure that occur with glomerulonephritits
- proliferative - increase in glomerular or inflammatory cell number
- membranous - basement membrane thickening
- sclerosis - increase in mesangial extracellular matrix
- fibrosis - deposition of collegan fibers
- diffuse - all glomeruli and all parts
- focal - only some glomeruli
- segmental - certain segment of glomeruli
- mesangial - only mesangial cells
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relate the proteinuria, hematuria, pyuria, oliguria, edema, hypertension, and azotemia that occur with glomerulonephritis to changes in glomerular structure
- proteinuria,hemtauria from damage to glomerulus filter
- pyuria from inflammatory immune response
- oliguria from permeability
- edema, azotemia, and hypertension from renal insufficiency
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what is the difference among the nephritic syndromes, rapidly progressive glomerulonephritis, nephrotic syndrome, asymptomatic glomerular disorders, and chronic glomerulonephritis
- Nephritic syndrome- produce proliferative inflammatory response
- Rapid - immunologic SLE and Goodpasture
- nephrotic syndrome - produces increased permeability
- asymptomatic - significant 16% of population have small amounts of proteinuria,hematuria, lowGFR
- chronic - progression of asymptomatic or acute
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Cite a definition of tubulointersitial kidney disease
- disoders which affect renal tubular structures and the intersitial tissue
- acute - sudden interstitial edema by pyelonephritis or drug hypersensitivity
- chronic - intersitial fibrosis,atrophy, by imbalances caused by changes in tubular function
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differentiate between the defects in tubular function that occur in proximal and distal tubular acidosis
- PROXIMAL RTA - defect in PCT where 85% of bicarb is reabsorbed, pt has decreased plasma bicarb, seldom severe acidosis
- DISTAL RTA - DCT and CT, hypokalemia, hyperchloremic metabolic acidosis, inability to acidify the urine, nephrocalcinosis, nephrolithiasis
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explain the pathogenesis of kidney damage in acute and chronic pyelonephritis
- ACUTE - gram neg (e.coli etc) thru UTI, S.aureus thru blood, abrupt chills fever loin pain,
- CHRONIC - scarring and deformation, reccurent caused by reflux, may be insidious, severe hypertension contributes
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explain the vulnerability of the kidneys to injury caused by drugs and toxins
drugs can decrease renal blood flow, obstruct urine, directly damage structures, or produce hypersensitivity reactions
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Characterize Wilms tumor in terms of age of onset, oncogenic origin, manifestations, and treatment
- 3-5 yo, most common malignant abdominal tumor
- congenital, large asymptomatic abdominal mass and hypertension,
- TX - Surgery, chemo, radiation, 60% all, 88-98 I-III
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cite the risk factors for renal cell carcinoma, describe its manifestation, and explain why the 5 year survival rate has been so low
- heavy smoking, obesity in women, petroleum heavy metals asbestos, aquired cystic KD
- most commonly upper pole, clear cell PCT epithelial
- usually asymptomatic until advanced except small hematuria,
- 90-95% of kidney cancers
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