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83 Cards in this Set
- Front
- Back
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How is male sex differentiated in development?
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Y chromosome --> SRY gene --> testes development -->
1) MIH release --> inhib fem int. genitalia 2) testosterone --> male int genitalia test --> DHT --> male ext genitalia |
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What is the first sign of puberty in males and females?
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Males: testicular enlargement
Females: breast development |
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21-hydroxylase
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converts:
progesterone --> DOC - - ->Aldo 17-OH Progesterone --> 11-deoxycortisol - - -> Cortisol *major defect in most CAD --> androgen excess |
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What are the signs of pre-pubertal testicular failure?
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small testes, more feminine/eunuchoidal skeletal proportions, high-pitched voice, dec muscle mass, delayed bone age, dec body hair
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What are the signs of post-pubertal testicular failure?
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loss of libido, soft testes, dec muscle mass/strength, dec pubic hair and gynecomastia
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Hypergonadotropic hypogonadism
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high LH/FSH w/ low test (= 1o testicular failure)
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Klinefelter's Syndrome
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47,XXY --> hypogonadism (extra X --> damage to seminferous tubules and Leydig cells --> dec testosterone)
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What are some infections that can cause hypogonadism?
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mumps orchitis, Cocksackie B virus
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Hypogonadotropic hypogonadism
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Low LH/FSH w/ dec testosterone (= 2o)
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Kallman Syndrome
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defective KAL gene that codes for adhesion molecules to allow for GnRH neuron migration from olfactory bulb
--> hypogonadism + hyposnia |
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What are the 2 classic presentations of CAH?
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Salt-wasting: severe cortisol and aldo def
Nonsalt wasting/simple virilizing - sufficient cortiol and aldo to avoid crisis but still has virilization features |
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Hydrocele
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accumulation of fluid in the tunica vaginalis that surrounds the testis
*most common cause of scrotal enlargement |
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What causes a congenital hydrocele?
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failure of processus vaginalis to close during first year of life...also asc w/ inguinal hernias
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What causes an acquired hydrocele?
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pluuged inguinal lymphatic system due to trauma, neoplasm and infections (Filaria nematode - parasite in tropical regions)
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Lymphatic filariasis
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lymph drainage obstruction caused from parasite (Filariasis Nematode) seen in tropical areas
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Spermatocele
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painless, retention cysts of a tubule of rete testis/head of epididymis
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Varicocele
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abnormal enlargement of pampiniform plexus veins in scrotum draining testicle
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How do varicoceles cause infertility?
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dilated veins and inc blood flow to area raises temp above limit for spermatogenesis
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What side do varicoceles usually present on and why?
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L side. Left spermatic vein enters L renal vein at 90o angle...less continuous flow compared to R (enters IVC at more obtuse angle)
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Orchitis
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painful, unilateral inflammation of the testes that generally occurs due to infection (mumps, bacterial in immunocomp pts, cocksackie, EPV, varicella and syphillis)
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What is the most commonly cause of acute epididymitis?
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retrograde spread of infection from urethra (GC, Chlamydia, E. coli)
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Cypotorchidism
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absense of 1 or both testes from scrotum usually due to failure of descent from abdomen
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What are some of the consequences/asc of undescended testes?
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reduced fertility (inc temp --> atrophy of seminiferous tubules), inc risk of testicular germ cell tumor and inc risk fo testicular torsion
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Orchiopexy
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surgical procedure where undescended testes are brought into scrotum
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What role to AMH and testosterone play in testicular descent?
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AMH - moves testes across abdomen to entrance of inguinal canal
test - induces gubernaculum to contract --> moves testes through inguinal canal into scrotum |
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Testicular torsion
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spermatic cord gets twisted and cuts off blood supply to testicle
*surgical emergency |
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Who does testicular torsion typically present in?
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pubertal adolescents
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How can testicular torsion and orchitis be differentiated?
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Cremasteric reflex - tough upper thigh --> contraction of the cremaster (testicle lifter)
present in orchitis absent in tt |
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What is the most common prediposition for testicular torsion?
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bilateral anomalous testicular suspension (bell clapper deformity)
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Bell clapper deformity
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testicles normally attached posteriorly to inner lining of mesorchium, if early termination of it, testis lie horizontally in tunica vaginalis
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Most common testicular cancer?
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primary germ cell tumors
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What is the prognosis of primary germ cell tumors?
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Very good - 95% cure rate
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What are the 2 types of germ cell tumors?
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nonseminoma and seminoma
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What are the 4 histologies seen in nonseminomatous GCT?
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embryonal carcinoma, teratoma, choriocarcinoma, endodermal sinus (yolk sac)
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Where do nonseminomatous GCT tend to met to?
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retroperitoneal lymph and lung parenchyma
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What is a cause of chronic epididymitis?
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TB
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What age group is most affected by testicular tumors?
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15-35
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Which germ cell tumors are radiosensitive?
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Seminomatous
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What are a-fetoproteins and hCG important marker of?
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nonseminomatous germ cell tumors
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What is penile cancer most commonly caused by?
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poor genital hygiene in uncircumcised men and w/ PVH 16 and 18
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What are the "3 P's" affected by MEN syndrome?
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parathyroid, pancreas and pituitary
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What is are the qualifications of MEN 1 syndrome?
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At least 2 or 3 main tumor types (parathyroid, pancrea and pituitary) AND first-degree relative w/ one of the tumors
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What other tumors are pts w/ MEN 1 at higher risk of developing?
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duodenal (gastrinoma), carcinoid (seratonin), adrenal adenoma, lipomas and cutaneous
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What is the mutation in MEN1?
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Menin
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What is the most common type of tumor in MEN 1?
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parathyroid (95%)
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How is hyperparathyroidism from MEN1 different from sporadic 1o?
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MEN1 - starts earlier, has multiple galnd involvment and has high risk of recurrence
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What is the most common type of pancreatic tumor in MEN1 and what syndrome does it cause?
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Gastrinoma --> Zollinger-Ellison syndrome (esophagitis and duodenal ulcers)
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What tumors are ac w/ MEN 2?
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medullary thyroid carcinoma and pheochromocytoma and 1o parathyroid hyperplasia
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What is the mutation in MEN2?
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RET
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What has a worse prognosis MEN 1 or 2?
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MEN2
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What is the difference between MEN2A and 2B?
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2A had medullary thyroid ca, pheochromocytome and 1o parathyroid hyperplasia
2B - MTC and pheo only |
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Which MEN 2 (a or B) has a worse prognosis?
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2B
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What is secreted in pheochromocytomas of MEN2A and MEN2B?
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2A - epi
2B - NE |
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What is the tx for medullary thyroid cancer?
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total thyroidectomy
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What is the inheritance pattern of MEN1?
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recessive gene but behaves in dominant fashion (needs 2nd hit and very high odds of acquiring it)
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What STD is asc w/ frothy, green discharge and strawberry cervix?
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Trichomoniasis
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How do you tx trichomoniasis?
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Flagyl
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What dz is asc w/ uterine tenderness and cervix motion tenderness upon bimanual exam?
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Pelvic Inflammatory Dz
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What infections are asx with PID?
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GC, Chlamydia...polymicrobial
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What is the classic triad of PID?
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pain, fever and leukocytosis
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How should be tx empirically for PID?
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Women w/ pelvic/abdominal pain and uterine/adnexal/cervical motion tenderness
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What hormone causes thelarche?
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estradiol
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What hormone cuases pubarche?
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adrenal androgens
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2-cell theory
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Theca cells - make estrogen precursor (late cycle LH receptors)
Granulosa cells - make estradiol (FSH receptors) |
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What occurs in the follicular phase of the menstural cycle?
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1. Follicular development and inc FSH
2. THeca produce androgens for release and conversion 3. FSH --> inc aromatase expression --> inc estradiol and inhibin 4. Inhibition of FSH, helps determine dominant follicle 5. At threshold, estradiol becomes stimulatory 6. LH surge 7. LH --> ovulation and re-entry of oocyte into meiosis |
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What occurs in the proliferative phase of the menstrual cycle?
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Estradiol --> endometrial proliferation/thickening
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What occurs in the luteal phase of the menstrual cycle?
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Corpus leuteum secretes progesterone and inhibin --> inhibits FSH secreion
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What occurs in the secretory phase of the menstrual cycle?
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Progesterone stimulates secretion and maintenance of the endometrium (prepares for implantation)
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What occurs in the menstrual cycle if fertilization occurs?
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hCG rescues corpus leutem from atresia by binding to the LH receptor and maintaining progesterone production
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Transverse vaginal septum
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vaginal plate doesn't canalize
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Cervical atresia
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cervix abnormally closed
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Muellerian agenesis (Rokitanksy)
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failure of int genitalia to form
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What are the risk factors for polycystic ovary syndrome?
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premature adrenarche, inc response to ACTH, DM, obesity
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What is the broad pathophysiology of PCOS?
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High LH, Low FSH --> ovary can't select dominant follicle
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How are SHBG (sex hormone binding globulins) asc w/ PCOS?
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there is inc peripheral conversion of androgen --> suppression of SHBG --> inc free testosterone
*cause of hirsutism |
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what does an ultrasound in PCOS look like?
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pearl necklace from multiple immature follicles
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what drugs are used to control hirsutism in PCOS?
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spironolactone (competes for androgen receptor)
Finasteride (sp 5a-reductase inhibitor) Flutamine (androgen receptor block) Eflornithine (inhibits I-ornithine decarboxylase) |
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What are some causes of early menopause?
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smoking, Fragile X, radiation/chem, hypothyroid, Lupron
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Why are cycles shorter just before menopause?
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fewer eggs --> less inhibin --> inc FSH --> drive follicular maturation faster --> early ovulation
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What is the concern for estrogen secretion w/o progesterone?
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Endometrial hyperplasia
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What are the levels of FSH and Estradiol in menopause?
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FSH >100 (not establsihed cut-off)
Estradiol <20 |
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What is hormone therapy good at treating?
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Hot flashes, osteoporosis
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What is an alternate tx to hormone therapy for hot flashes?
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SSRI
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