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8 in '08 Histo Final
Histological Diseases
24
Science
Professional
12/03/2008

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Term
Stage II membranous glomerulopathy
Definition
accumulation of immune complexes in subepithelial zone of glomerular capillaries. Glomerular basement membrane (GBM) material projects around subepithelial deposits. Glomeruli are normocellular. “Spikes/tram tracks” present here w/foot effacement (loss of foot processes). Meek typed, “what happens in stages 1,3,4?” Stage 1: has scattered subepithelial deposits. Stage 2: listed above. Stage 3: newly formed BM has surrounded the deposits. Stage 4: immune complex deposits lose their electron density, resulting in irregularly thickened BM w/irregular electron-lucent areas.
Term
Diabetic glomerulosclerosis
Definition
GBM thickening & expansion of mesangial matrix, mild mesangial hypercellularity. Sometimes focal, segmental, nodular lesions appear, i.e. in Kimmelstiel-Wilson nodules. Capillaries & mesangial cells are pushed to periphery in K-W. Focal foot effacement present. DG is leading cause of end-stage renal disease in US.
Term
Crescentic glomerulonephritis
Definition
Proliferation of the parietal cells of Bowman's capsule; cellular buildup resembles a cresent pushing on glomerulus. This proliferation can cover 50% of entire urinary space. Fibrin and macrophages w/i cresent. Proliferation of parietal epithelial cells, focal disruption of BM, focal foot effacement, ↑ # mesangial cells. This condition is not a disease but is a morphological component of an active disease.
Term
Mesangial glomerulonephritis, aka Type 1 membranoproliferative glomerulonephritis
Definition
mesangial and endothelial cell hypercellularity and subendothelial deposits along inner border of BM, glomeruli are inlarged.
Term
Nephrogenic diabetes insipidus
Definition
kidney does not respond to ADH b/c of defective AQP-2 and ADH receptor proteins synthesized by collecting tubule and collecting duct. Copious dilute urine is produced.
Background info: ADH regulates water permeability of epithelium of CD by 2 effects:
Short-term: translocation of AQP-2-containing intracytoplasmic vesicles into apical surface. This ↑es [AQP-2] available for ↑ing water permeability.
Long -term: synthesis of AQP-2s and their insertion into apical cell membrane
Term
Acute Tubular Necrosis
Definition
failure of tubular fxn due to poor oxygenation following a drop in BP. Cells swell as result of failure of Na+/K+ ATPase pumps  inadequate excretion of H2O into interstitium. In PCT cells die and block lumen. Results in: oliguria/anuria (scant/no urine), hyperkalemia, and acidosis
Term
Autosomal Dominant Polycyctic Kidney Disease (ADPKD)
Definition
results f/mutations in either of two genes: PKD1 and PKD2, encoding polycystin1 and polycystin2 proteins. These proteins are found predominantly in the cilium of principal cells of the CD. ADPKD Polycystin 1 and 2 are mechanosensors on non-motile cilia of principal cell. P1 also plays role in adhesive interaction on lateral cell border in the intercellular space. Complete loss of P1 or P2  extensive cyst formation. Cysts derived f/dilation of CD. Hypertension & renal failure = clinical presentations.
Term
Polycyctic ovarian disease
Definition
bilaterally enlarged ovaries due to fluid-filled follicular cysts, atrophic 2° follicles, and abnormally large tunica albuginea. AKA Stein-Levental syndrome when associated w/oligomenorrhea (scanty menstruation). Infertile b/c no ovulation. Pathogenesis unclear but related to defect in regulation of androgen synthesis  excess estrogen production. Abnormal selection process in follicle maturation; progesterone not produced  failure of mature follicle to transform into progesterone-producing corpus luteum (c.l.). Tx: hormones to stabilize & reconstruct E:P ratio; surgery via cortical ovarian incisions allow oocyte to exit after hormonal Tx. Affects 5-10% of child-bearing aged women.
Term
Ectopic (tubal) pregnancy
Definition
implantation of embryo in abnormal site. Oocyte fails to enter uterine tube; can implant on surface of ovary, intestine or inside rectouterine pouch, or uterine tube wall. Most ectopic implantations do not develop past early fetal stages. May be fatal to mother & need to be removed surgically.
Term
Endometriosis
Definition
presence of uterine endometrial tissue in pelvic peritoneal cavity. Associated w/hormone-induced changes in ectopic endometrium during menstrual cycle. As endometrium is shed, bleeding occurs in peritoneal cavity, causing severe pain, formation of cysts and adhesions. May  sterility; ovaries and uterine tube become deformed & embedded in scar tissue. No cure. Tx : ↓ing pain symptoms w/ medication; shrinking and/or slowing endometrial growths by blocking ovulation w/ hormones; restoring fertility by removal of growths by conservative surgery and hormonal tx; prevention of recurrence by complete removal of growths and ovaries by hysterectomy.
Term
Uterine fibroid
Definition
non-cancerous growths appearing in childbearing yrs. Uterine tissue  mass/polyp protruding into uterine cavity; sensitive to estrogen so ↑estrogen during cycle  growth of polyp. Present in ~10% of women; ID most in 40- 50 year olds. If blocks cervix or uterine tube  fertility issues; if in-vetro fertilization then ↑ risk of miscarriage. Sx include: irregular bleeding; bleeding b/t menstrual cycles; excessively heavy bleeding during menstrual cycle; bleed post-menopause. At ↑ risk if… obese; taking tamoxifen; have high BP; cervical polyps. Tx: surgical removal but can recur.
Term
Cervical carcinoma
Definition
80-90% originate from stratified squamous wet cells. 10 – 20% originate from cervical glands (adenocarcinomas). HPV infection main cause although majority of infections do NOT  cancer. Carcinoma in-situ: contained w/i epithelium, does not invade underlying stroma. Invasive carcinoma: penetrates basal lamina & metastasizes to other regions of body. In 2006 – 10,000 women in US diagnosed (half a million world-wide) & 4,000 women (~250,000 world-wide) die.
Term
Breast cancer
Definition
originates f/epithelium lining the ducts or terminal ductules. Ductal carcinoma: originates from epithelium lining ducts. Lobular carcinoma: originates from epithelium lining terminal ductules. If not treated early, tumor cells metastasize via lymph to axillary nodes near affected breast via blood to lungs, bone and brain. Deficiency/mutation in BRCA1 shown to ↓stability or ↑incidence of mutation rate of tumor suppressor genes (e.g. p53). p53 checkpoints are at G1  S and S  G2. Mutations in BRCA1 incapacitate checkpoint at G2-M of cell cycle and concurrently, # of centrosomes are ↑ed. 5-10% of women w/ breast cancer  very high lifetime risk of breast/ovarian cancer. Mutated cells, thus, have capability to proliferate. Presence of Est. or Prog. receptors = good prognosis; tamoxifen: estrogen receptor blocker & tx of choice. Presence of c-erb B2 oncoprotein on cell surface – poor prognosis. 180,000 new cases; 43,000 women die/yr.
Term
Teratomas
Definition
germ cell tumors. Tumors comprising multiple types of cells; derived from multiple germ layers therefore commonly seen in gonads (most in ovary; fewer in testes). Benign teratomas - well-differentiated (mature) cystic lesions. Malignant teratomas - immature & non-cystic (i.e., solid). Cells differentiate along various germ lines; recapitulate any tissue of the body (e.g., hair, teeth, fat, skin, muscle, & endocrine tissue).
3 classifications: Mature –benign (occasionally malignant); usually in young women; frequently contain hair,
sebaceous glands, bone, tooth & cartilage. Monodermal–rare; strumaovarii–ovarian tumor composed of well-developed thyroid follicles (hyperthyroidism); ovarian carcinoid–usually produces serotonin. Immature–fast growing malignant tumors; resemble fetal not mature tissues; usually adolescent & very young women
Term
Cryptorchidism
Definition
Refers to the absence of one or both testis from the scrotum due to improper descent. Most common genital problem encountered in pediatrics. 30% in premature boys, 3% of full term. Causes: (1)Hypothalamus-pituitary-gonadal axis, (2) Epididymis, (3) Gubernaculum (secretes material that moves testis through inguinal canal) and genitofemoral nerve. Testosterone synthesis is not affected. Increased storage temperature inhibits spermatogenesis.
Term
Seminoma
Definition
Testis germ cell tumor. Most common testicular cancer in men, peak incidence at 30-35. Not found before puberty. Tumor cells have fried egg appearance and lymphocytes infiltrate around the STubules.
Term
5alpha-reductase deficiency (5-ARD)
Definition
Autosomal recessive sex-limited condition  inability to convert testosterone to dihydroxytestosterone (DHT). DHT is required for masculinization of the external genitalia in utero. Genetic males are born with ambiguous gentialia (male pseudohermaphroditism). Uterus and fallopian tube are absent due to mullerian-inhibiting factor. Testes remain intact as wolffian structures (normal).
Term
Varicocele
Definition
Dilation of veins of the pampiniform plexus, gives “bag of worms” appearance. Typically seen in left testicle due to blockage of the left renal vein and thus increased venous pressure.
Term
Leydig cell tumor
Definition
Rare tumor that is less common than seminoma. Functionally active tumor secreting androgens, estrogens, or both.
Term
Benign prostatic hypertrophy (BPH)
Definition
Occurs almost exclusively in the transitional and perurethral zones,  partial or total obstruction of the urethra. Enlargement of mucosal and submucosal glands. Believed that DHT causes stromal and glandular epithelial cells to proliferate causing hypertrophy. Increased secretion of prostate specific antigen (PSA) and prostate acid phosphatase (PAP). Believed to occur in all men by age 80. Dx: digital rectal exam (DRE). Tx: Transurethral resection of the prostate (TURP).
Term
Prostate Carcinoma
Definition
One of most common cancers in male, affecting approximately 1:20. Incidence increases with age. Tumors usually develop in peripheral zone of the gland. Enlarged main gland. Increased PSA and PAP. Increased osteoblastic activity related to increase in bone formation. Dx: DREs and detection of PSA. Tx: Surgery, radiotherapy, hormonal therapy (advanced cancer with metastases).
Term
Transitional Cell Carcinoma (TCC)
Definition
Most common bladder cancer, male dominant, replacement of normal epithelial cells with one that are enlarged, crowded with enlarged hyperchromatic nuclei and prominent mitotic figures; epithelium is not thickened. Epithelium changes are likely to progress to invasive.
Term
Turner Syndrome
Definition
All or partial loss of X chromosome. 98% of affected fetus result in miscarriages; accounts for 10% of all spontaneous abortions in US; affects 1:2500 live female births. Appearance: short stature, lymphoedema, broad chest, low hairline, low-set ears, webbed neck, gonadal dysfunction including amenorrhea and infertility (ovarian failure). No cognitive effects but problem with visual-spatial relationships--math issues. Additional medical issues: ~1/3 thyroid disorders (hypothyroidism-autoimmune); ~1/3 kidney malformation; 5-10% constriction of aorta. IDed by Henry Turner (Oklahoman endocrinologist) in 1938. No cure--treat with GH, estrogen replacement, thyroid replacement, etc.
Term
Kleinfelter Syndrome
Definition
Observed in males with an extra X chromosome (47,XXY). They are 1.) phenotypically males—presence of the Y chromosome; 2.) have small testes and few spermatogenic cells are present; 3.) have high FSH levels because Sertoli cell function is abnormal (failure to produce inhibin); 4.) have low testosterone levels, but high estradiol levels.
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