exam3 guide Flashcards
Childhood Polycystic Kidney Disease Etiology
Autosomal recessive
Childhood Polycystic Kidney Disease Characteristics
Enlarged bilateral kidneys that resemble sponges, non-fx at birth
Childhood Polycystic Kidney Disease Clinical Presentation
Quickly leads to renal failure shortly after birth
Childhood Polycystic Kidney Disease histo
- Numerous small cysts in cortex and medulla all the way to calecis
- Cyst appears to arise from epithelium of collecting duct
Adult Polycystic kidney Disease Etiology
Autosomal dominant affects 1 in 600 people
Manifests in 3rd or 4th decade with s/s of renal failure.
Adult Polycystic kidney Disease Characteristics
Multiple, expanding serous or purulent-filled cysts of both kidneys that destroy intervening parenchyma. Pressure from expansion can => hemorrhage, infx, HTN.
Kidneys can get up to size of liver, 4000 gms
Cysts derived from obstructed tubules but reason for obstruction is unknown
Adult Polycystic kidney Disease Clinical Presentation
Early sign: dull, aching pain in abd. or back (d/t pressure from expanding cysts). ESRD by age 50, tx with kidney transplant
- Peritonitis, hemorrhage, infection
- HTN – 75% of patients
- Hematuria – d/t ruptured intracystic hemorrhage
Adult Polycystic kidney Disease histo
- Enlarged kidneys composed solely of mass of cysts of varying sizes, with little intervening parenchyma
- No demarcation of pelvis, calices
-Cyst appear to arise from any part of nephron and have atrophic lining
Adult Polycystic kidney Disease a/w
- Saccular berry aneurysms – 10-30% of patients who have increased risk of rupture, esp. combined with HTN.
- Also assoc w/ liver cysts
• Horseshoe kidney
Occurs 1 in 600 persons. fusion of kidney at midline. Usually no problem unless defect favors obstruction to renal flow. More susceptible to UTI due to flow of ureters.
• Renal cystic dysplasia –
- MC of renal develop disorders. Abnormal differention of renal structures during embryonic period + cystic formation.
- Usually unilaterally and histologically tube-like structures enclosed by mesenchyme, foci of cartilage, immature glomeruli and tubules. No association with malignancy. Kidney doesn’t function.
- Can also cause Potter’s syndrome
Acute UTI Cause
- Gram negative rods. E Coli.
- Antibx can be used, but infx usually subsides if patient is immunocompetent
Acute UTI Description
Acute pyelonephritis = foci of pus and focal abscesses. In severe cases, may permeate entire kidney and fill renal pelvis.
Acute UTI Clinical Presentation
Acute pyelonephritis: sudden, sharp pain in costovertebral angle along with chills, fever, malaise. Urinalysis: pyuria and bacteriuria, along with leukocytosis
Acute UTI Grossly (pyelo)
Acute pyelonephritis:
Round, yellow, raised abscesses present on renal surface surrounded by areas of congestion.
Lots of neutrophils, white pus pockets
Acute UTI grossly (cystitis)
Acute cystitis: hemorrhagic wall, lots of neutrophils
Acute UTI comp
Renal Papillary necrosis
Chronic UTI Cause
Chronic pyelonephritis: can evolve from acute pyelonephritis, esp. if recurrent. Also radiation, prior surgery, autoimmune
Chronic UTI Description
Chronic pyelonephritis: Recurrent infections superimposed on obstructive lesions => recurrent bouts of interstitial inflammation and scarring. Kidneys become small and irregularly scarred.
Chronic UTI Grossly
Chronic pyelonephritis: Hallmark = scarring involving pelvis or calyces, or both, => papillary blunting and calyceal deformities
- May involve one or both kidneys.
- Kidneys are small and irregularly scarred.
Chronic UTI Histology
Microscopically: interstitial fibrosis of mononuclear cell infiltration
-Dilation and contration of tubular lining epitelium with atrophy of cells. Dilated tubules contain pink, glassy coloid casts that look like thyroid tissue (thyroidization). Proliferative arteriosclerosis
Chronic UTI comp
Can be important cause of CRF, esp. if obstruction is underlying cause
General features of stones
● Most stones found in renal pelvis or urinary bladder
● Stones may resemble crystals, or be small, round, or irregular masses
• Typical symptoms of stones
● Hematuria, urinary colic (spasmotic pain caused by contraction of obstructed ureter). Painful, lower back pain radiating towards groin
Tx stones
-prompt treatment important, obstruction increases risk of infection, stone formation, and permanent renal atrophy
● Smaller stones may be voided. Large stones require surgery or mechanical extraction after stones have been broken down by lithotripsy
mc Stone
Composition: calcium oxalate (US) or phosphate (England)
Accounts for 75% of all stones
Calcium (MC)
Associated with hyperexcretion of calcium in patients who have abnormal calcium metabolism (hyperparathyroidism, diffuse bone disease, other hypercalcemic states)
Struvite Aka triple ammonia or ammonia stones or staghorn calculi
- Typically complication of UTI, which lead to formation of ammonia from urea in the urine.
- Infections with urea-splitting bacteria (e.g. proteus) converts urea to ammonia.
- Form some of the largest stones, can fill entire pelvis
assoc. with infx
Uric Acid
50% of patients have gout. Others have diseases involving rapid cell turnover, like leukemia
Stones are usually radiolucent (not easily detected)
RENAL CELL CARINCOMA Epidemiology
- Median age: 55 years;
- Men>women
- Risk factors unknown, link to cigarette smoking
RENAL CELL CARINCOMA Grossly
Nodules or masses sharply demarcated from renal parenchyma
Arise from either upper or lower poles
Cross section: yellow, bosselated, encapsulated tumors that can extend through renal capsules into perirenal fat, adrenal, renal vein
RENAL CELL CARINCOMA HIstology
Cuboidal cells reminiscent of renal tubules. Clear or granular cytoplasm filled with glycogen and lipids (adenocarcinoma)
RENAL CELL CARINCOMA Clinical
MC presentation is hematuria. Classic triad: dull flank pain, palpable abd. mass, hematuria. Some may have non-specific symptoms like weight loss, long-standing fever, HTN.
May have distant mets to lungs and bones
RENAL CELL CARINCOMA dx and tx
-50% diagnosed accidentally on CT scan
- Surgically
5 year survival 35%
TRANSITIONAL CELL CARCINOMA OF RENAL PELVIS Epidemiology
Papillary neoplasms of renal pelvis, resemble carcinomas of urinary bladder
Low to high grade lesions
TRANSITIONAL CELL CARCINOMA OF RENAL PELVIS Clinical
Hematuria or urinary obstruction early in course
TRANSITIONAL CELL CARCINOMA OF RENAL PELVIS tx:
Surgical removal = good results for grade 1 or 2 lesions. 5 year survival 70%
WILMS TUMOR Epidemiology
1 pediatric malignancy (1 in 10,000)
Present at time of birth, manifests between 2nd and 4th years of life
Highly malignant
WILMS TUMOR Grossly
Solitary or multinodular abd. mass that replaces kidney – usu palpable
WILMS TUMOR histo
composed of blastic or immature cells
WILMS TUMOR tx
Good prognosis with surgery and chemo – 85% cures
urinary bladder carcinoma Epidemiology
MC urinary tract neoplasms
52,000 new cases each year, with 10,000 deaths. 2x as common as renal cell carcinoma but same number of deaths
Peak incidence in men 60-80 years old
urinary bladder carcinoma Etiology
● Transitional cell carcinomas (90%)
-other types as well but not common
urinary bladder carcinoma Risk Factors
Unknown. Cigarette smoking – most important factor (proportioned to total number smoked)
- Also linked to industrial carcinogens: Azo dyes and chemicals used in rubber industry, textile printing
- In Egypt, Schistosoma haemotabium infx can lead to cancer of urinary bladder
urinary bladder carcinoma Grossly
Tumors are either papillary or flat; invasive or non-invasive by histologic examination
Progression leads to extension of tumor into muscle layers of bladder and adjacent pelvic organs
Mets in pelvic lymph nodes, later stages tumor may met to distant sites
urinary bladder carcinoma Clinical
Hematuria, dysuria, lower abd. pain
urinary bladder carcinoma dx
Cystoscopy and histologic biopsy
urinary bladder carcinoma tx
Surgical resection and chemo
Prognosis based on histological grade and type, clinical staging. Stage A Grade I tumor = 98% 5-year survival. Stage D (distant mets) have 15% 5-year survival
Nephrotic S/SxS
● Heavy proteinuria (3-3.5 g/pro/day) ● Hypoalbuminemia ● Edema ● Hyperlipidemia ● Lipiduria
Nephrotic Pathology
Permeability defect in glomerular capillaries allows protein to be lost from plasma into urine => hypoproteinemia => edema => decreased plasma volume =. RAS activation => worsening edema
Decreased plasma proteins => increased production of proteins by liver (inc. liproproteins)
Nephrotic Etiology
● Minimal Change Disease (nils Disease) responsible for 70% of Nephrotic Syndrome in children, only 20% of adults
● Systemic diseases that involve kidney (DM, amyloidosis, SLE) responsible for 20-30% in adults, rarely in children. #1 cause in adults membranous glomerulonephritis
● Also associated with infx, malignancies, drugs (heroin)
Nephrotic and nephritic dx
Renal biopsy is only definitive diagnosis
Nephritic S/SxS
Hematuria variable degrees of proteinuria, decreased GFR Elevated BUN/SCr Oliguria Fluid retention HTN Mild edema RBC casts in urine
Nephritic Pathology
Mild proteinuria and hematuria d/t injury of glomerular capillaries
Manifestations can develop rapidly, progress rapidly, and persist for years (continuous or intermittent) and progress slowly to CRF
Nephritic Etiology
Diseases that cause this syndrome characterized by inflammatory changes in glomeruli with leukocytes and necrosis.
● #1 cause: Acute post-infectious glomerulonephritis
CL = capillary lumen
- DM = tufts in glomerulus (glomerulosclerosis or Kimmelstiel-Wilson Disease)
- Nephrotic syndrome = permeability defect
- Nephritic syndrome = injury of glomerular capillaries
- Chronic glomerulonephritis = thick walled arteries with narrowed lumina
FP = food processes
Minimal Change Disease = fusion of epithelial foot processes
acute post strept GMN Epidemiology
Typically affects children 6-10, but can affect adults (can be pretty bad in adults)
Uncommon d/t antibiotics, but in undeveloped areas
acute post strept GMN Pathology
- Immune-mediated inflammatory glomerulopathy that occurs 1-2 weeks after strep throat OR strep skin infection with GABHS.
- Inflammatory mediators attract neutrophils and monocytes, stimulate proliferation of mesangial and endothelial cells => marked hypercellularity of glomerulus (big honkin’ glomeruli)
- Deposition of antigen and antibody (immune complex) in glomerular basement membrane. Immune complex trapped in GBM, activate complement and inflammatory cells (esp. neutrophils) into glomeruli.
acute post strept GMN S/S
90% of people will have low complement d/t consumption in glomeruli
acute post strept GMN HIstology On EM
On EM subepithelial dense deposits shaped like humps on epithelial side of basement membrane
acute post strept GMN On IF
On IF microscopy, granular deposits (lumpy bumpy) seen are both IgG and complement along basement membrane
acute post strept GMN tx
95% of children recover, only 1-2% progress to rapidly progressive GMN
membranous GMN Etiology
Immune-mediated glomerulopathy
Cause unknown in 85% of cases
membranous GMN Clinical
No inflammatory cells in glomeruli and urine devoid of RBCs (like in APS-GMN)
Causes nephrotic syndrome (unlike APS-GMN)
membranous GMN Histologically
Light microscopy: glomeruli have thickened BM but are normocellular
EM: thickening of BM attributed to deposition of dense immune complexes
IF: granularity of deposits of IgG
membranous GMN Prognosis
Prognosis better in children After 20 years: 25% have spontaneous remission 50% have persistent proteinuria with stable renal fx 25% have ESRD
membranous GMN Tx
Corticosteroids for those with progressive renal failure
Goodpasture’s Syndrome
- Autoimmune with formation of antibodies to body’s basement membrane components, collagen type 4
- Injury to lungs causes intraalveolar hemorrhage. Antibodies cause rupture of BM, causing macrophages to exit through holes of BM to accumulate in urinary space, forming crescents that compress capillary loops. Can lead to RPGN
- Involves lungs and kidneys
- Decreased blood flow through compressed capillary loops => glomerular filtration ceases => anuria
- Most patients don’t recover, need dialysis or transplantation to survive
Lumpy bumpy IF
Acute Post-Streptococcal GMN
Linear IF
Goodpasture’s Syndrome
Prolactinoma (MC) General
(typically) Microadenomas composed of prolactin-secreting cells
Prolactinoma (MC) S/S
- Women of reproductive age: amenorrhea, galactorrhea, infertility (Inhibits pulsatile secretion of LH needed for normal ovulation)
- Males: vague, may include. impotence or decreased libido
Prolactinoma (MC) Tx
Function can be inhibited with bromocriptine (inhibits prolactin secretion)
Transnasal surgery to sella tursica for larger tumors
Somatotrophic Adenoma general
Macroadenoma composed of growth hormone (75% visible with naked eye or CT scan).
Somatotrophic Adenoma s/s
Prepubertal patients: stimulate longitudinal skeletal growth => gigantism
Postpubertal: acromegaly => enlargement of acral parts of extremities, tongue, jaw (prognathism), nose. Internal organs also enlarged (heart, spleen, liver). Metabolic disturbance:, hyperglycemia hypercalcemia.
Somatotrophic Adenoma Tx
Surgical removal
Corticotrophic Adenoma general
Typically microadenoma. Composed of ACTH-secreting cells.
Corticotrophic Adenoma s/s
Typical s/s of Cushings disease, fatigue, weakness, mental instability
Corticotrophic Adenoma tx
Removal of tumor
Craniopharyngiomas
- Benign tumor that arises from pituitary
- Easily removed
- Does not produce hormones, but can get big enough to cause hypofunction of pituitary
Graves Disease
Hyperthyroidism Etiology
Autoimmune disease
Occurs 10x more often in women than men.
Graves Disease
Hyperthyroidism
Patho
Antibodies bind to surface of thyroid follicular cells, cause stimulus similar to TSH.
Causes hypersecretion of T3 and T4
Graves Disease
Hyperthyroidism Charc
Enlarged thyroid, composed of hyperplastic follicles lined with hyperactive, tall, cuboidal cells
Thyroid contains lymphoid follicles (signs of autoimmune disease)
Graves Disease
Hyperthyroidism dx
Microscopic material
High T3 and T4 / Low TSH
Graves Disease
Hyperthyroidism
tx
Antithyroid drugs; if ineffective, subtotal thyroidectomy
Hashimoto’s Thyroiditis
Hypothyroidism Etiology
Autoimmune – IgG antibodies cuase destruction of gland
More common in women, typically 45-65
Hashimoto’s Thyroiditis
Hypothyroidism Patho
Graduaal failure d/t immune destruction of gland
Increased risk for B-cell lymphomas
Hashimoto’s Thyroiditis
Hypothyroidism Charc
Painless, diffuse enlargement of thyroid
extensive infiltration of follicles and stroma with mononuclear infiltration (lymphocytes and plasma cells), which destroy thyroid follicles and germinal centers. This may lead to healing and fibrosis and reduce size of thyroid
Hashimoto’s Thyroiditis
Hypothyroidism dx
Low T3 and T4 / High TSH
Hashimoto’s Thyroiditis
Hypothyroidism tx
Synthetic thyroid hormones. Good results. Must take for rest of lives
Know the four types of thyroid tumors in general
- Benign tumors are more common
- Only 3-4 cases of thyroid cancer are diagnosed per 100,000 people, less than 1000 people die each year
- No risk factors are known, more common in females
Thyroid Adenoma Etiology
MC benign tumor
Composed of thyroid follicles
Thyroid Adenoma Presentation
Small, well-encapsulated with fibrous tissue.
Thyroid Adenoma Dx/Tx
Biopsy
Not premalignant, no tx if small nodules
Know general features of diabetes insipidis
- Compression of pituitary stalk by tumor
- Hypo release of ADH
- Excess release of low osmolarity urine
- Major symptoms: polyuria, noctura, polydipsia
