Endo/Repro Pathology Flashcards

1
Q

Conn Syndrome - presentation(5)

A

Primary Hyperaldosertonism due to hyperplasia or tumor

  • hypertension
  • hyperkalemia
  • metabolic alkalosis
  • LOW plasma renin
  • transient weakness
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2
Q

Unilateral lesion of the lower vulvular vestibule

A

Bartholin cyst

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3
Q

High risk HPV (4)

A

16 ,18, 31, 33

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4
Q

Low risk HPV (2)

Distinguished by ?

A

6 , 11

DNA testing

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5
Q

Condyloma acuminatum

A

Genital wart

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6
Q

Lichen Sclerosis histology and timeline

Risk of cancer?

A

thinning of the epidermis and fibrosis(sclerosis) of the dermis -

postmenopause

“parchment like vulgar skin” - leukoplakia

Slight risk of squamous cell

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7
Q

Lichen Simplex Chronicus histo

Risk of Cancer

A

hyperplasia of vulval squamous epithelium
thick leathery vulva due to chronic itching and scratching

NO risk

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8
Q

2 paths of vulvar cancer

Presentation and age

A

leukoplakia

HPV - 40-50s, dyspalstic trasnfomeation

long standing Lichen Sclerosis - >70 yrs old

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9
Q

Koiliocyte

A

Histology of HPV

raisin like nucleus

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10
Q

Erythematous pruritic ulcerated vulvar skin

A

Extramammary paget disease

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11
Q

Histology of extramammary paget disease

Lab tests?

A

malignant epithelial cells in the epidermis

Distinguished from melanoma being
keratin +
S100 -
PAS +

NO underling carcinoma - not so w/ pagets of the breast

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12
Q

Adenosis
what is it?
higher risk for?

A

focal persistance columnar epithelium in the upper vagina (mullerin duct derived)

-normally taken over by nonketitinizing squamous epithelium of lower (1/3)
(urogenital duct derived)

Clear Cell Carcinoma risk

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13
Q

Diethylstilbestrol associated risks (2)

A

given in the 60s to help moms with pregnant

leads to in daughters

  • adenosis and clear cell carcinoma risk
  • Smooth muscle abnormalities (ectopics etc.)
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14
Q

Bleeding grapelike mass

Age of presentation?

A

Embryonal Rhabdomyosarcoma
- protruding from vagina or penis

<5 y.o

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15
Q

Cytoplasmic cross striations cell

2 stains associated

A

Rhabdomyoblast
- characteristic of embryonal rhabdomyosarcoma

desmin- intermediate filament of muscle cell
myogenin- nuclear TF

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16
Q

transformation zone

A

Junction in the cervix where nonkeritinizing squamous epithelium meets the single layer of columnar

Sharp change - all path occurs here

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17
Q

High risk HPV characterized by what 2 gene products?

A

E6 - destroys p53
- loss of G1-> S regulation, no DNA check
E7 - destroys Rb
- free floating E2F results

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18
Q

High grade dysplasia in CIN characterized by(2) - histo

A

hyperchomatic nuclei

high nuclear to cytoplasmic ratio

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19
Q

Confirmatory test w/ abnormal PAP

A

Colposcopy and biopsy

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20
Q

CIN I II and III compared to carcinoma in situ

A

carcinoma in situ does not regress and is full thickness

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21
Q

Cervical Carcinoma risk factors (3)

A

High risk HPV
smoking (odd alone w. prostrate)
immunodeficiency - AIDS defining

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22
Q

Asherman Syndrome

-Path and Cause?

A

secondary amenorrhea due to loss of basalis and scarring

Due to overaggressive D and C

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23
Q

Stem Cell of Endometrium

A

Basalis

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24
Q

Anovulatory cycle complications

Seen around

A

Cycle w/out ovulation

  • > estrogen driven proliferation building up
  • > no shedding with progesterone drop phase

Abnormal bleeding around menarche and menopause

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25
Q

Cause of acute endometritis

A

retained products of conception

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26
Q

Presentation of acute endometritis (3

A

fever
ab uterine bleeding
pelvic pain

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27
Q

Characteristic cell looked for in chronic over acute endometritis

A

Plasma cell

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28
Q

Chronic endometritis caused by

One additional risk of chronic over acute

A

retained products of conception, IUD, PID and TB

Infertility

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29
Q

Endometriosis Presentation and location
1 most common
5 others

A

Ovary most common - dysmenorrhea and pelvic pain

uterine ligaments- pelvic pain
pouch of douglas - pain w/ dedication
bladder wall - pain w/ urination
bowel serosa - ab pain and adhesions
fallopian tube mucosa - scarring and ectopic risk
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30
Q

gun powder nodules

A

yellow brown endometrial tissue in soft organs

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31
Q

chocolate cyst

A

endometriosis in the ovary

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32
Q

Adnomyosis

A

endometriosis in the myometrium

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33
Q

Hyperplasia of endometrial glands relative to stroma called______

Due to

A

endometrial hyperplasia

unopposed estrogen
- no progesterone phase

->postmenopausal bleeding

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34
Q

Most important predictor for hyperplasia progression to carcinoma is

A

presence of cellular atypic

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35
Q

2 pathways of endometrial carcinoma

A

Hyperplasia- 75%

Sporadic -25%

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36
Q

Histology of hyperplasia derived endometrial carcinoma

Age

A

endometrioid

50-60s

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37
Q

Histology of sporadic pathway derived endometrial carcinoma

A

serous papillary structure

  • necrotic and concentric calcification
    Psammoma body formation

Note: p53 associated

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38
Q

Benign neoplastic proliferation of smooth muscle arising from myometrium called what?

Increased risk?

A

leiomyoma

Estrogen exposure
premenopausal women

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39
Q

Multiple well defined white whirled mass of uterus

A

Leiomyoma

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40
Q

Most common tumor in females

A

leiomyoma

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41
Q

Leiomyosarcoma only arises?

A

de novo

  • not leiomyoma derived
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42
Q

Leiomyosarcoma differs from leiomyoma due to (4)

A

Malignant -
postmenopausal
single lesion
necrosis and hemorrhage (vs white whirled look)

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43
Q

functional unit of ovary

A

follicle

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44
Q

LH acts on what cell in the ovary

to make what?

A

thecal

androgen

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45
Q

FSH acts on what cell in the ovary

to make what?

A

granulosa

aromatase -> estrodiol

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46
Q

Polycystic Kidney Disease classic presentation

A

young
obese woman

w/ infertility
oligomenorrhea
infertility

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47
Q

polycystic kidney disease associated w/ what endocrine disorder?

Also at risk for?

A

Diabetes type II 10-15 yrs later on
- due to some insulin resistance seen

endometrial carcinoma
-high estrone levels

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48
Q

High LH and low FSH seen in ?

LH:FSH> 2

A

Polycystic kidney disease

Systemic androgen release from LF is converted in peripheral fat to estrone instead of glomerulosa cell -> disorder

leading to multiple ovarian follicular cysts

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49
Q

Coelomic epithelium

A

surface epithelial lining that can become cancerous

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50
Q

2 most common subtypes of surface epithelial tumors

A

serous
mucinous

most common ovarian cancer

Rare: endometroid, Brenner

of which they can either be benign, malignant or borderline

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51
Q

Cystadenoma

  • pathology
  • seen in
A

benign surface epithelial tumor
-either mucinous or serous

Single cyst w/ simple flat lining

premenopausal

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52
Q

Cystadenocarcinomas

  • pathology
  • seen in
A

malignant surface epithelial tissue
-either mucinous or serous

Complex cysts w/ thick shaggy liines

postmenopausal

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53
Q

BRACA1 mutation more commonly associated w/ this surface epithelial tumor

A

Serous carcinoma of ovary and breast

in addition to breast cancer risk

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54
Q

Endometroid tumor

-pathogenesis

A

less common surface epithelial tumor

endometrial-like glands that are malignant, may arise from endometriosis

-may be found w/ endometrial carcinoma as well

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55
Q

Brenner Tumor

  • pathogenesis
A

less common surface epithelial tumor

bladder like epithelium (urothelium) that are benign

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56
Q

Prognosis of surface epithelial tumors

spreads?

A

poor due to late presentations

Spreads locally -> peritoneum

-vague ab complaint and signs of compression

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57
Q

CA-125

A

Surface epithelial tumor marker

-Rx response and recurrence screening

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58
Q

Germ cell Tumors usually occur in what population

A

reproductive age (15-30)

second to surface epithelial tumors in prevalence at 15%

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59
Q

Cystic Teratoma

weird presentation?

A

benign tumor derived drom 2 or 3 embryogenic layers - most common germ cell tumor

10% bilateral

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60
Q

2 instances when cystic teratoma is malignant

A

Immature tissue present - usually neuro

somatic malignancy - squamous cell carcinoma of skin (cancer has cancer)

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61
Q

Struma Ovarii presentation

A

Teratoma w/ mostly thyroid tissue

  • hyperthyoirdism
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62
Q

Dysgerminoma

male counterpart?

A

large cell w/ clear cytoplasm and central nuclei

  • looks like oocyte
  • most common malignant germ cell tumor

seminoma

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63
Q

LDH is elevated in this germ cell tumor

A

Dysgerminoma

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64
Q

Endodermal Sinus tumor

produces?
Commonly seen in ?

A

mimics the yolk sac
kids
AFP - alpha fetoprotein made

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65
Q

glomerous like structures seen in biopsy is what germ cell tumor?

A

endodermal sinus tumor

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66
Q

tumor w/ cytotrophoblasts and syncytiotrophoblasts but villi are absent

A

Choriocarcinoma

  • due to normally finding blood vessels, small hemorragic tumor spreads easily
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67
Q

Choriocacinoma produces

A

High beta hCG -> thecal cysts

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68
Q

Embryonal Carcinoma

A

Malignant tumor of large primitive cells
- germ cell

aggressive

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69
Q

Tumor producing estrogen

Presentation?(3)

A

Granulosa-theca cell tumor
-> a sex cord stromal tumor

Depends on age

  • precocious puberty
  • menoohagia/metrorrhagia
  • postmenopause bleeding (most common setting for this tumor)
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70
Q

menorrhagia def

A

heavy prolonged bleeding

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71
Q

metrorrhagia def

A

outside of normal cycle bleeding

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72
Q

Reinke Crystals seen in?

What presentation associated w/ finding?

A

Seen in Sertoli Leydig cell tumors
-> a sex cord-stromal tumor

Produces androgen

(hirsutism and virilization in females)

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73
Q

A solid Benign tumor of fibroblasts

A

fibroma

-> a sex cord stromal tumor

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74
Q

Pleural effusion and ascots associated w/ this sex cord stromal tumor

A

Fibroma

Meigs syndrome

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75
Q

Psudeomyxoma peritonei presents as?

  • METS from where?
A

massive amounts of mucus in the peritoneum

from the appendix

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76
Q

bilateral ovarian tumor called?

Often due to?

A

Krukenberg tumor

METS from diffuse metastatic gastric carcinoma

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77
Q

Key risk factor for ectopic pregnancy

A

Scarring

-PID Hx or endometriosis

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78
Q

Classic presentation of ectopic

A

Lower quadrant abdominal pain a few weeks post missed period

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79
Q

Spontaneous abortion is?

Often due to ?

A

miscarriage of fetus before 20 weeks

Often due to chromosomal anomalies (trisomy 16)

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80
Q

Autoimmune disorder associated w/ recurrent spontaneous abortions

A

SLE - due to hypercoaguable state

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81
Q

Teratogen effects w/in 1st 2 weeks

A

spontaneous abortion

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82
Q

time of highest incidence of organ malformation with teratogen exposure during

A

weeks 3-8

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83
Q

Placenta Previa?

presentation-

A

preview of placenta
-implants lower in the uterus, over the os?

presents as 3rd trimester bleeding

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84
Q

Placental abruption

Complication?

A

Abrupt separation

Separation of placenta from decidua prior to delivery

  • common for still births
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85
Q

Placenta accreta

Complication?

A

implantation of placenta into myometrium
- difficult delivery of the placenta

post partum bleeding worry -> hysterectomy?

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86
Q

Thalidomide teratogen causes?

A

Teratogen

Limb defects

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87
Q

Isotertinoin teratogen causes

A

Spontataneous abortion, hearing and visual impairment

Acne

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88
Q

Tetracycline teratogen

A

Causes discolored teeth

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89
Q

Warfarin teratogen

A

fetal bleeding

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90
Q

Phenytoin teratogen

A

digit hypoplasia and cleft lip

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91
Q

Preeclampsia defined by(3)

Seen when?

A

hypertension
proterinuria
edema

3rd trimester

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92
Q

What separates preeclampsia from eclampsia

A

siezures

-> immediate delivery

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93
Q

HELLP Syndrome

A

Hemolysis
Elevated Liver enzymes
Low Platelets

Preeclampsia w/ thrombotic microangiopathic liver dysfunction
Warning signs -> immediate delivery

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94
Q

Complete mole risk of choricarcinoma

A

2-3%

Vs minimal in partial mole

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95
Q

46 chromosomes in a an empty ovum

A

Complete mole

vs Partial mole
-69 chromosomes (2 sperm and regular egg)

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96
Q

Fetal tissue is present in which abnormal conception and proliferation of trophoblasts

A

Partial mole

NONE in complete

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97
Q

most Villous edema and diffuse Trophoblast proliferation

A

Complete mole

Partial is focal proliferation and some villus edema

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98
Q

Suspect mole if (2)

A

uterus is larger than dates

beta hCG is higher

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99
Q

Passage of grape like masses in the vaginal canal w/ snowstorm appearance on US

A

mole pregnancy

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100
Q

how does choriocarcinoma arising from complete mole conceptions respond to chemo?

A

Respond well

Vs. germ cell pathway do not

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101
Q

dysmenorrhea

A

painful menstration

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102
Q

Hypospadius pathologenisis?

  • see?
A

failure of urethral folds to close

- opening of urethrea in inferior surface

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103
Q

Epispadius primary cause

see?

A

abnormal positioning of the genital tubercle (glans)

urethra on superior surface of penis

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104
Q

bladder extrophy associated w/ what in males?

A

epispadias

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105
Q

necrotizing granulomatous inflame of the inguinal lymphatics and lymph nodes is called?

-Associated w?

A

lymphogranuloma venereum

Chlamydia trachomatis serotype L1-L3

  • rectal stricture may be long term comp
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106
Q

Precursor in situ lesions of squamous cell carcinoma of penis (2)

A

Bowen disease - leukoplakia on shaft

Erythroplasia of Queyrat - erythroplakia on glans

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107
Q

Bowenoid papulosis is

A

an In situ lesions of the penis

multiple reddish papules (younger 40s’s and NO invasive carcinoma risk)

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108
Q

Penile squamous cell carcinoma risk (2)

A

High risk HPV (2/3)

Lack of circumcision

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109
Q

Cryptorchidism

what is it and how common is it?

A

failure of testicle decent

1% of population (most common congenital male issue)

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110
Q

Orchioplexy

A

taching down testicles to scrotum

  • done at 2yrs of age w/ crytorchidism
  • to prevent testicular atrophy and infertility
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111
Q

Causes of orchitis by age group (6)

A
young men
- Chlamydia (D-K) and nessiera gonorrhoeae
old men
-E coli and Pseudomonas
teen males
-mumps
Any
-Autoimmune - > granulomas involving seminiferous tubules
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112
Q

Sudden onset of testicular pain and absent cremaster reflex

A

testicular torsion

- twisting and compression of vein -> congestion and hemorrhagic infarction

113
Q

Which side does a varicocele affect and what is a common cause?

A

dilation of spermatic vein due to impaired drainage

Left side - spermatic vein drains into renal vein vs direct
Often due to L sided renal cell carcinoma

114
Q

Fluid collection w/in the tunica vaginallis that transilluminates

infants due to?
Adults due to?

A

Hydrocele

persistent processus vaginalis in infants
lymphatic blockage in adults

115
Q

Seeding of scrotum and and malignancy complications is the reason why we don’t do this?

A

Biopsy testicular tumors

95% germ cell anyway vs. sex cord

116
Q

Risk factors for germ cell tumors in males? (2)

A

cryptochidism (seminoma)

Klinefelter syndrome

117
Q

Seminoma ~ ? female tumor

-histo

A

dysgerminoma

- large cells w/ clear cytoplasm and central nuclei

118
Q

Seminomas response to therapy vs nonseminomas

A

highly responsive and metastasize late

  • NOTE: rarely make b hCG (think chorio normally)
119
Q

Germ cell tumors in males vs females

A

All 5 malignant

-even teratoma which is benign in female

120
Q

Homogenous mass w/ NO hemorrhage and NO necrosis found in the testicle

A

seminoma

  • ex:embryonal carcinoma is hemorrhagic and necrotic
121
Q

Chemotherapy leads to differentiation of tumor in a testicular cancer

A

Embryonal carcinoma

See increased AFP or b hCG

122
Q

Most common testicular tumor in kids

  • see what on histology
A

yolk sac tumor

  • a germ cell tumor

Schiller duval bodies (glomerular looking structures)

AFP is HIGH

123
Q

beta hCG is traditionally seen elevated in this germ cell tumor and has the following complication (2)

A

Choriocarcinoma

Hyperthyroidism and gynectomasia due to similar alpha subunit

124
Q

Most germ cell tumors are of this type in males

A

mixed germ cell

125
Q

Age >60 year old male and has bilateral testicular cancer

A

lymphoma

15-40 think germ cell tumor (5 of them)

126
Q

Sex cord stromal tumors in males (2)

problems seen?

A

Benign

Leydig cell - precocious puberty and gynectomastia w/ androgen production
– Reinke crystals

Sertoli cell - clinically silent

127
Q

tender boggy prostate in digital exam

caused by

A

Acure prostatitis

chlamydia, N gonnorrheae
E Coli, Psudomonas

128
Q

Differences between acute and chronic prostatitis (2)

A

Chronic has negative culture

pelvic and low back pain

129
Q

Central periurethal change w/ hyperplasia of the prostatic gland and stroma

due to _____ made by____

A

BPH

dihydrotestosterone

5 alpha reductase

130
Q

What is a worrisome level in prostate cancer

A

> 10ng/mL PSA

-especially if african american, older and high saturated fat diet

131
Q

Why is prostate cancer clinically silent till late

A

located in the peripheral, posterior region and no urinary issue till late

noticed on DRE

132
Q

Biopsy showing small invasive glands w/ prominent nucleoli in the nucleus

A

prostate cancer

133
Q

Gleason scale measures what and is based on

A

Based on architecture- not nuclear atypic

2 biopsies ranked 1-5. higher being bad

134
Q

Common METS of prostate cancer and associated serum results

A

Lumbar spine or pelvis

increased alkaline phosphatase w/ sclerotic change (build up)

increased prostatic acid phosphatase as well

135
Q

Lobules and ducts of breast tissue are lined by…

A

2 layers of epithelium

  • luminal layer
  • myoepithelial layer
136
Q

area where most great tissue by density

A

upper outer quadrent

137
Q

Common causes of galactorrhea(3)

A

NOT cancer

  • nipple stim
  • prolactinoma
  • drugs
138
Q

erythematous breast w/ purulent nipple discharge

common cause?
Rx?

A

mastitis

Staph aureus
dicloxacillin

139
Q

Periductal mastitis more common in?

Why?

A

Smokers

Vitamin A deficiency can’t maintained specialized epithelium of the duct

140
Q

Subareular mass and nipple retraction due to squamous metaplasia of lactiferous duct

A

periductal mastitis

pain w/ duct blockage and inflammation
- nipples contract w/ myofibroblasts and fibrosis w/ healing

141
Q

green-brown nipple discharge

A

mammary duct ectasia

142
Q

Biopsy of mammary duct ectasia see?(2)

A

Dialation

chronic inflammatiion
plasma cells

143
Q

What else can present as a mass other than breast cancer in a post menopausal female? (5)

A

mammary duct ectasia

Phyllodes tumor

Fibrocystic change

papillary carcinoma

fat necrosis

144
Q

Fat necrosis in a breast is often due to

A

trauma

  • mass on physical exam
  • abnormal calcification on mammography
145
Q

Most common change in premenopausal breast is?

Due to ?

A

fibrocystic change

horomone mediated stretching

146
Q

Fibrocystic change has the associated risk with breast cancer

A

NONE

-see blue dome appearance on gross exam

147
Q

Sclerosing adenosis

A

to many glands w/ fibrosis in-between
2x the risk of invasive breast cancer (both)

  • Calcified
148
Q

ductal hyperplasia

A

2 layers of duct abnormally proliferating w/ fibrocystic change
-2x risk of invasive breast cancer (both)

149
Q

apocrine metaplasia

A

w/ fibrosis and cysts

  • seen in fibrocystic change
  • NO increased risk
150
Q

Atypical ductal or lobulal hyperplasia seen has what CA risk

A

5x bilateral invasive CA

151
Q

Bloody nipple discharge in a PREmenopausal women

Malignant?

A

intraductal Papilloma

-NO malignant potential

152
Q

Bloody discharge in a POSTmenopausal woman

Malignant?

A

Papillary carcinoma

-malignant potential

153
Q

Fibrovascular projections lined by luminal cells in a breast

A

papillary carcinoma

-missing the epithelial cell seen normally in intraductal papilloma

154
Q

well circumscribed mobile marble mass in breast

associated symptoms (2)

A

Fibroadenoma - benign

grows w/ pregnancy
painful w/ menstration

155
Q

leaf like projections seen on biopsy

commonly seen in?

A

phyllodes tumor
-fibroadenoma w/ fibrous overgrowth

postmenopausal

156
Q

Calcified mass on mammography (3)

A

Fat necrosis (salponification)

sclerosing adenosis

Ductal carcinoma in-situ (dystrophic calcification)

157
Q

DCIS usually presents as

A

calcification, no mass

158
Q

Comedo type DCIS is characterized by (3)

A

high grade cells w/
necrosis
dystrophic calcification

159
Q

Paget’s Disease is what

differs from extramammary Paget’s disease how?

A

DCIS that extends up the ducts to involve skin and nipple

  • Differs in that there is cancer underneath (none in extrammamry Pagets)
160
Q

Most common invasive carcinoma

Presenting as?

A

Duct type

Presenting as a mass w. dimpling of skin or retraction of nipple late

161
Q

Duct like structures in a desoplastic stroma on biopsy indicates

A

invasive ductal carcinoma

4 types (3 good prog, 1 bad - inflammatory)

162
Q

Abundent extracellular mucin w. carcinoma

Seen in which population

A

mucinous carcinoma
- type of invasive ductal

old ladies (>70)

163
Q

well differentiates tubules w/out myoepithela cells

A

tubular carcinoma

-type of invasive ductal

164
Q

an inflammed swollen breast that does not resolve w/ Abx

A

Inflammatory Carcinoma

-type of invasive ductal

165
Q

Inflammmatory carcinoma needs 2 things

A
  • tumor cells in lymphatic ducts (swelling)
  • inflammatory response

poor prognosis

166
Q

Type of tumor associated w/ BRCA1

A

Medullary carcinoma

-type of invasive ductal

167
Q

Breast cancer found by accident called?

Why?

A

Lobular carcinoma in Situ

mulitfocal and bilateral w. no mass or calcifications

168
Q

Dyscohesive cells lacking in Lubular carcinoma due to lack of?

A

E Cadherin

169
Q

Single file pattern w/ no duct formation found on biopsy

A

invasive lobular carcinoma

-lack E cadherin and line up

170
Q

BRCA1 associated w/ 2 CA

A

Breast (medullary CA) and Ovarian

171
Q

BRCA2 is associated w/

A

Male breast CA - (invasive ductal CA)

  • Sub areolar
  • also seen in Klinefelter
172
Q

pulling sensation in the groin associated w. as acities and hydrothorax in a female

A

Meigs syndrome(3)

ovarian fibroma
ascities
hydrothorax - often R pleural effusion

173
Q

Nonfunctional pituitary adenomas present as (3)

A

Mass effect

  • bitemporal hemianppsia
  • hypopituitarism
  • HA

Can also be functional

174
Q

Glactorrhea and Amenorrhea associated neoplasia

in males see?

A

Prolactinoma - most common functional

low labido and HA (Prolactin -> decrease in GnRH and associated LH and FSH)

175
Q

Growth hormone cell presentation in kids (1)

compared to adults -3

A

Gigantism - epiphyses not fused yet

Acromegaly in adults

  • enlarged bones of hands, face, jaw
  • enlarged visceral organs -> heart
  • enlarged tounge
176
Q

Associated endocrine problem w/ GH tumor

A

Secondary DM

due to decreased glucose uptake of glucose into cells w/ GH around

inceased gluconeogenesis

177
Q

Insulin growth factor 1

A

liver responds to GH to make

178
Q

Hypopituitarism in Kids vs adults

A

Kids - craniopharyngioma - stella tucuica compression

Adults - pituitary adenoma - compression
- als pituitary apoplexy - derranged bleeding in adenoma and compression

179
Q

PRegnancy and hypopituiary

-Cause

A

Sheehan syndrome

Gland doubles in size w/ pregnancy and blood supply can’t keep up, susceptible to infarct - parturition

180
Q

Poor lactation loss of pubic hair and fatigue post giving birth

A

Sheehan Syndrome

181
Q

Empty Stella Syndrome(2)

A

Congenetal defect leading to herniation of arachnid and CSF

or can have trauma

182
Q

Poly uria and polydipsia w/ response to demopressin

A

Central Diabetes insipities

- ADH deficiency

183
Q

ADH deficiency and associated Na serum levels

Test for it?

A

Hypernatremia and high serum osmolarity due to lack of ability to keep in water

Water deprivation test fails to increase urine osmolarity

184
Q

Nephrogenic Diabetes insipid us looks like central except

A

It does not respond to desmopressin

185
Q

SIADH is?

Due to(4)?

A

Excessive ADH secretion

extopic production - small cell carcinoma of lung
CNS truama
pulmonary infection
Drugs - cyclophophamide

186
Q

Hyponatremia and low serum osmolarity and associated mental changes? seen w/ what could be a paraneoplastic caused condition

A

SIADH
-ADH from small cell carcinoma

Mental changes due to neuronal swelling and cerebral edema

187
Q

Hyperthyroidism characterized by what 2 pathologic processes

A

increased basal metabolic rate
- increased synthesis of Na/K ATPase

Increased sympathetic nervous system
- increased beta 1 adrenergic receptors

188
Q

Hyperthyroidsm clinical features especially

____cholesterolemia
_______glycemia

A
weight loss
heat intolerance
tachy
Arrythmia
Staring gaze w/ lid lag
diarrhea
Oligomenorrhea
decreased muscle mass

HYPOcholesterolemia
HYPERglycemia - gluconeogenesis and glycogenolysis

189
Q

Autoantibody IgG seen in ?

A

Graves disease

Acts on TSH receptor of the thyroid -> increased synth and release

190
Q

Scalloped colloid of the follicules

A

Graves disease

191
Q

Exophalthalos and pretrial myxedema due to

A

TSH receptors behind the orbits and under the shin

-> glycosaminoglycan buildup, fibrosis and edema
NOT hyperthyroidism

192
Q

Lab values is Graves (4)

T4?
TSH?
Sugars?
Cholesterol?

A

Increased T4
Decreased endogenous TSH - > less due to response of free T4
hyperglycemia
Hypocholesterol

193
Q

Feared complication of hyperthyroidism

See (2) due to ?

Presents as (4)

A

Thyroid Storm

elevated catecholamines and massive hormone excess

Due to Stress

arrythmia
hyperthermia
vomiting
hypovolemic shock

194
Q

Mulinodular goider caused by

A

relative iodide immune deficiency

Usually nontoxic

195
Q

When is a multi nodular goider worrisome

A

when some regions become TSH independent leading to T4 release w/out control

196
Q

Mental retardation, short stature and coarse facial features

Maye enlarged tounge and umbilical hernia

A

Cretinism

Hypothyroidism in neonates

197
Q

Causes of Cretinism(4)

A

Maternal hypothyroidism
thyroid agenesis
dyshormonogenetic goiter (peroxidase lacking)
iodine deficiency

198
Q

Myxedema

Associated clinical features (9)

A

Hypothyroidism in older children or adults

myexedema
weight gain
slowing of mental activity
cold intolerance
Bradycardia
oligomennorhea (both)
HYPERcholesterol
Constipation
199
Q

Common causes of hypothyroidsm(4)

A

iodine deficiency
Hashimoto thyroiditis
drugs - Li
surgical removal/radioablation

200
Q

HLA-DR5

A

Autoimmune destruction -> Hashimoto thryoditis

initially hyperthyroid

201
Q

Labs in hashimoto

T4?
TSH?

A

decreased T4

increased TSH

202
Q

Antithroglobulin Ab

A

hashimoto marker, not pathologic cause though

203
Q

antithyroid peroxidase Ab

A

hashimoto marker,

not pathological cause though

204
Q

Chronic inflammation w/ geminal centers and Hurthle cells

A

Hashimoto histology

Hurthle cells - eosinophilic metaplasia of cells that line the follicle (pink)

205
Q

Hashimoto s/ enlarging thyroid gland be concerned w/

A

B cell marginal zone lymphoma

206
Q

Painful thyroid w/ transient hyperthyroidism

A

Subacute granulomatous de quervain thyroiditis

207
Q

Dequervian progression?

A

Self limited and may progress to hypothyroidism

Granulomatous thyrodiditis

208
Q

Chronic inflammation w/ extensive fibrosis of thyroid

Presenting concern

A

Reidel fibrosing thyroiditis

Concern w/ mass effect and local structure - airway involvement

Mimics anaplastic carcinoma except patients are younger

209
Q

Iodine 131

A

Useful for imaging thyroid

Usually cold for adenoma and carcinoma

210
Q

Biopsy of a thyroid done w/

Useless in?

A

Fine needle aspiration

Differentiating between follicular adenoma and follicular carcinoma

211
Q

Defining feature of Follicular adenoma

A

Benign proliferation of follicle W/IN a fibrous capsule

212
Q

Orphan annie eyes and nuclear groves seen in?

also may see?

A

Papillary carcinoma

Most common carcinoma (80%)
- aslo pasmmona bodies

213
Q

Invasion of the fibrous capsule w/ proliferating follicles

A

Follicular carcinoma

METS hematogenously, worse prognosis

214
Q

Medullary Carcinoma proliferation

Secrete?

A

malignant proliferation of parafollicular C Cells

Calcitonin -> hypocalcemia

Calcitonin deposits as amyloid

215
Q

Medullary carcinoma associated w/

A

MEN 2A

  • p53 mutation
  • pheochromocytoma and parathyroid adenomas

MEN 2B

  • RET mutation
  • ganglioneuronomas and pheochromocytoma
216
Q

Worse tumor of thyroid

A

Anaplastic carcinoma seen in the elderly

local invasion -> dysphagia and respiratory compromise

217
Q

Most common cause of primary parahypothyroidism

other 2?

A

parathyroid adenoma (benign)

sporadic parathyroid hyperplasia
parathyroid carcinoma

218
Q

peptic ulcer disease
acute pancreatitis
Ostei fibrosa cystica

associated w?

A

some complications of hyperparathyroidism

Pancreatitis think high enzyme function

Others-
Nephrolithiasis
Nephrocalcinosis - calcification of renal tubules
CNS disturbances - 
Constipation
219
Q

Nephrolithiasis
Nephrocalcinosis - calcification of renal tubules
CNS disturbances -
Constipation

A

some complications of hyperparathyroidism

peptic ulcer disease
acute pancreatitis
Ostei fibrosa cystica

220
Q

Labs in hyperparathyroidism

PTH
serum Ca
serum phosphate
urinary cAMP
Serum alkaline phosphatase
A
PTH - increased
serum Ca - Increased
serum phosphate - decreased
urinary cAMP - increased (Gs signal)
Serum alkaline phosphatase - increased (osteoblasts turned on 1st to activate osteoclasts)
221
Q

Principles of too much parathyridism (3)

A

Want increased free serum Ca

increased osteoclast activity (via osteoblast activation)
increased small bowel absorption of Ca and phosphate (Vit D activation)
Increased renal Ca reabsorption and decreased phosphate reabsorbtion(dumping)

222
Q

Most common cause of secondary hyperparathyroidism?

Mech?

A

Chronic renal failure

renal insufficeincy -> decreased Phosphate excretion

all 4 gland stimulated to increase PTH release

223
Q

Labs? in secondary hyperparathyroidism

PTH
Serum Ca
Serum Phosphate
alkaline phosphate

A

PTH increased
Cerum Ca is decreased (due to increased Phos)
Serum Phosphate increased
alakine phosphatase increased (PTH activates osteoblasts -> alkaline env; osteoclasts later)

224
Q

Muscle spasms of the facial nerve or with blood pressure cuff indicative of what

A

Low serum Ca

Chvostek and Trosseau respectfully

225
Q

Periorbital tingling, numbness may indicate?

A

Low serum Ca

226
Q

Causes of hypoparathyrodism (3)

A

Autoimmune damage
accidental surgical excision
Digeourge syndrome and faille of 3rd and 4th pharyngeal arch

227
Q

Low PTH and low Ca in labs indicates

A

Hypoparathyrodism

228
Q

Pseudohypoparathyroidism is due to

PTH labs?

A

end organ resistance to PTH

Gs is not working like it should w/ decreased adenylate cyclase activity and ultimately less cAMP

Increased PTH

229
Q

Autosomal dominant disease w/ short stature and chart 4th and 5th digits associated w/

A

pseudohypoparathyroidism

230
Q

Insulin is secreted by ?

A

Beta cells in the center of the islets of langerhans

GLUT 4

231
Q

Glucagon is secreted by

A

alpha cells of pancreas

-> gycogenolysis and lipolysis

232
Q

HLA DR3 and HLA DR4

A

Associated w/ DM 1

233
Q

Chronic inflammation of islets due to

A

autoimmune destruction of beta cells by T lymphocytes

Type 4 hypersensitivity

234
Q

Presentation of DM1 (3)

A

childhood seeing

  • high serum glucose
  • weight loss, low muscle mass, polyphagia -unopposed glucagon (no stores w/ gycogenolysis and lipolysis)
  • polyuria -> polydipsia and glycosuria
235
Q

Eliciting factor in diabetic ketoacidosis and pathophys

A

DM1

Stress -> epinephrine -> glucagon secretion -> lypolysis -> increased FFA -> liver converts to ketone bodies

236
Q

ketone bodies(2)

A

beta hydroxybutyric acid

acetoacetic acid

237
Q

Hyperkalemia seen in diabetic ketoacidosis due to?

A

lack of insulin driving K into cells
K is exchanged for H in attempt to put protons into cells

NET LOSS of K though in urine - correct w/ Rx

238
Q

Kussmaul respirations

A

seen in ketoacidosis due to blowing off the acid

239
Q

Presentation of Ketoacidosis (6)

A
Kussmaul respirations
dehydration
nausea
vomiting
mental status change
fruity smelling breath -acetone
240
Q

Rx considerations w/ DKA (3)

A

fluids
insulin
electrolytes (esp K)

241
Q

Obesity leads to DMII due to

A

end organ resistance w/ decreased # of insulin receptors

Strong genetic component

242
Q

Histology of DMII

A

amyloid deposition in the islets

243
Q

DMII presentation (3)

A

often silent but

polyuria, polydipsia, hyperglycemia

244
Q

Lab values for DMII

Random Glucose
Fasting Glucose
Glucose tolerance

A

RG > 200 mg/dL
FG >126 mg/dL
serum > 200 2 hrs after loading

245
Q

Rx progession in DMII(3)

A
  1. Weight loss
  2. meds (metformin and sulfoylureas)
  3. insulin
246
Q

life threatening diuresis w/ hypotension and coma

A

DMII complication called hyperosmolar non-ketotic coma

no ketones dut to limited insulin

247
Q

Atherosclerosis seen in DMII due to?

Causes?

A

nonezymativ glycosylation of vascular BM of large and medium vessels

CV disease - leading cause of death
Peripheral vascular disaes - non trauma amputations

248
Q

NEG of small vessels leads to ?

Which organ? and complications?

A

nyaline ateriolosclerosis

Kidney renal arterioles

  1. efferent -> hyperfiltration injury, micoalbuminuria -> nephrotic syndrome (kimmelstiel-Wilson dodules)
  2. Afferent -> diffuse low blood flow and sclerosis -> chronic renal failure
249
Q

A1C

A

NEG of hemoglobin -> 120 day measure of sugar average

250
Q

Peripheral neuropathy in DMII due to

Same mech seen in?(2)

A

Schwann cells have glucose freely enter and covered to sorbitol by Aldose reductase -> osmotic damage

See also in pericytes of retinal blood vessels(aneurism and blindness) and lens (cataracts)

251
Q

Tumors of islet cells - associated w.

A

5% of pancreatic tumors

MEN 1 - parathyroid hyperplaia and pituitary adenomas

252
Q

Insulinomas present as

A

episodic hypoglycemia w/ mental status change

C peptide increased

253
Q

Zollinger Ellison Syndrome

A

Rx resistant peptic ulcer disease due to gastrinoma

jejunum can be affected

254
Q

Presentation of achlohydia, cholelithias and streatorrhea

A

Somatostainomas -> somatostatin increase -> decreased gastrin and CCK

255
Q

Watery diarrhea w/ hypokalemia and achlohydria

A

VIPoma - excessive vasoactive intestinal peptide

256
Q

3 layers of adrenal cortex and products

A

Glomerulosa - mineralcorticoids
fasiculata - glucocorticoids
reticularis - sex steroids

257
Q

Moon facies, buffalo hump ad truncal obesity characteristic of what and why?

A

cushing syndrome

gluconeogenesis -> increase insulin -> storage in these areas

258
Q

3 Immune mechanisms of cushing syndrome

A

high cortisol ->
inhibits phopholipase A2
Inhibits IL2
inhibits histamine release from mast cell -> vasodilatation

259
Q

hypertension seen in cushing syndrome?

A

cortisol -> alpha 1 receptors on vessels - increased tone and response to NE effect

260
Q

Clinicla features of Cushing (6)

A
muscle weakness- cortisol breaks down for glconeogen
moon facies and buffalo hump
abdominal striae
HYN
Osteoperosus
Immune suppression
261
Q

Causes of Cushing (4) and resulting adrenal gross look

A
exogenous corticosteriods
-bilateral atrophic
primary adrenal adenoma
-unlateral atrophy
ACTH secreting pituitary adenoma
-bilateral hypertrophy
Paraneoplastic ACTH secretion
-pilateral hypertrophy
262
Q

Dexamethasone use in Cushings?

A

Use to suppress ACTH from pituitary adenoma but fails to suppress ACTH paraneoplastic - like a small cell lung carcinoma

Measure endogenous cortisol response

263
Q

Aldosterone acts where

A

late distal tubule collecting duct

264
Q

High aldosesterone levels effect on

pH
Na
K

A

metabolic alkalosis - (H excreted from alpha intercalated cells)
hypernatremia - chief cell (exchanged) -> HTN
hypokalemia - chief cell (exchanged)

265
Q

Primary hyperaldoseronism causes
vs secondary

Renin?

A

Primary - sporadic adrenal hyperplasia, adrenal adenoma, adrenal carcinoma
high aldosterone, low renin (negative feedback)

Secondary - activation of renin angiotensin system - renal vascular hypertension, CHF, fibromuscula dysplasi etc…
high aldosterone, high renin (cause)

266
Q

bilateral adrenal hyperplasia w/ salt wasting hyperkalemia and hyponatremia; hypotension w/ lack of cortisol and clitoral enlargement /precocious puberty think?

A

21 hydroxylase deficiency

267
Q

bilateral adrenal hyperplasia, NO salt wasting, life threatening hypotension w/ lack of cortisol and clitoral enlargement/precosious puberty

A

11 hydroxylase deficiency

Get some weak adosterone metabolites due to the presence of 21 hydroxylase but do not get mature aldosterone

Still no cortisol and shift to sex steroids

268
Q

bilateral adrenal hyperplasia w/ NO sex steriods and no cortisol and a lot of mineral corticoid production

A

17 hydroxylase deficiency

269
Q

hemorragic necrosis of adrenals in child due to DIC

Name of disorder?
Causal agent

A

acute adrenal insufficiency/Waterhouse friderichsen

N meningitidis infection prior

270
Q

Cause of death w/ acute adrenal insufficiency

A

Lack of cortisol -> life threatening hypotension

271
Q

Chronic history of hypotension, hyponatremia and hypovolemia, hyperkalemia, and weakness

Giveaway clue?

A

Addisons - chronic adrenal insufficiency

ACTH overproduction -> hyperpigmentation

272
Q

Causes of addisons (3)

A
autoimmune destruction
TB
metastatic carinoma (LUNG)
273
Q

Adrenal medulla derived frome

A

neural crest cells - > chromaffin

274
Q

Adrenal medulla produces

A

catecholamines

epinephrine and NE

275
Q

Episodic HTN, HA, palpitations, Tachy and sweating think?

A

Pheochromocytoma

276
Q

Diagnose pheo by what?

A

increased serum netanephrines, increased urine metanephrins and vanillymandelic acids

277
Q

Prior to surgical excision of pheo give?

Why?

A

phenoxybenzamine

give to prevent hypertensive crisis due to leakage of catecholamines squeezed out

278
Q

Rule of 10s in pheo (4)

A
10% bilateral
10% familial
10% malignant
10% located outside of adrenal medulla 
-bladder wall or inferior mesenteric artery root
279
Q

Pheochromocytoma are a part of 4 syndromes

A

MEN2a
MEN2b
von hippel Lindau
neurofibromatosis type 1