Enfermedades de Las Gonodas
-
Upload
arthur-henrique-caixeta -
Category
Documents
-
view
214 -
download
0
Transcript of Enfermedades de Las Gonodas
![Page 1: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/1.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 1/25
DISEASES OFTHE GONADS
Dr. Peter Igaz PhD
2nd Department of Medicine
Semmelweis University
![Page 2: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/2.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 2/25
Symptoms of androgen deficiency 1.
Organ before puberty after puberty
Bones eunuchoid appearance osteoporosis
Hair growth lack of beard growth, diminishing
forehead hair line, sexual hair
pubic hair line growthLarynx no voice change -
Skin decreased activity of atrophy
sebaceous glands, no acnes wrinkles
Bone marrow anemia anemia
Muscles underdeveloped atrophyPenis infantile no change in size
![Page 3: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/3.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 3/25
Organ before puberty after puberty
Prostate underdeveloped atrophy
Spermatogenesis not initiated halted
Libido, Potency no loss
Symptoms of androgen deficiency 2.
![Page 4: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/4.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 4/25
For ms of hypogonadism
Central, secondary, hypogonadotropLH, FSH low
Gonadal, primary,
hypergonadotrop
LH, FSH high
![Page 5: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/5.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 5/25
Causes of central hypogonadism
Kallmann-SyndromGnRH deficiency + Anosmia/Hyposmia
Prader-Willi-Labhart syndrom
GnRH deficiency, obesity, low body height,
newborn hypotony, diabetes melli tus type 1,strabismus, scol iosis, mental retadation.
Pituitary insufficiency
Hyperprolactinemia
![Page 6: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/6.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 6/25
Therapy of central hypogonadism
Androgens
Testosteron enanthat, cypionat i.m.,
transdermal, per os (hepatotoxic)
LH, FSH, hCGi.v. for ferti lity
GnRH, GnRH-agonists
pulsatile, pump for fertil ity
![Page 7: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/7.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 7/25
Causes of male gonadal hypogonadism
1. Klinefelter syndrome (small testes, no
spermatogenesis, gynecomastia, breast
cancer).
2. del Casti llo syndrome, Sertoli cell onlysyndrome, no spermatogenis
3. Noonan Syndrom (male Turner sy.)
4. Chemical and biological testis damage
![Page 8: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/8.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 8/25
Primary amenorrhoea
puberty
Secondary amenorrhoea
pregnancymenopause
![Page 9: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/9.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 9/25
Causes of amenorrhoea
HypothalamusKallmann Syndrom
Anorexia nervosa
Hypophyse
Sheehan Syndrom
(ischemic necrosis of the pituitary)
Hyperprolactinemia
Ovaries
Dysgenesis of the ovaries (Turnersyndrome)
Stein-Leventhal syndrom, PCO
![Page 10: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/10.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 10/25
Turner-syndrome
gonadal dysgenesis
Primary amenorrheaStreak gonads
Pterygium colli
Small stature (133-153 cm)
Cardiovascular Anomalies
Estrogen for secondary sexual
characteristics
GH for growth promotion
![Page 11: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/11.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 11/25
Pseudohermaphroditism
Pseudohermaphroditism: chromosomaland gonadal sex is different fromphenotpye
Female pseudohermaphrodit ism
Chromosomal and gonadal sex: womanPhenotype: male
Male pseudohermaphroditism
Chromosomal und gonadal sex: male
Phenotype: female
![Page 12: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/12.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 12/25
HYPERANDROGENISM
![Page 13: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/13.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 13/25
Hirsutismandrogen-dependent regions
e.g.: face, breast, belly
Hypertrichosis
Non-androgen-dependent regionse.g. exremities
Increased hair growth
![Page 14: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/14.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 14/25
Virilisation
In utero viri lisationIntersexuality
female pseudohermaphroditism
Viril isation in adultsclitor is hypertrophy
hirsutismmale type baldness
deeper voice
secondary amennorhoea
![Page 15: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/15.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 15/25
Causes of virilisation
1. endocrine diseases Adrenal causes
androgen-secreting adenoma
androgen-secreting adrenocort ical cancer
Cushing syndrome
congenital adrenogenital syndrome
Ovarian causes
Akromegaly
2. Idiopathic
3. Drugs
4. Other e.g. anorexia nervosa
![Page 16: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/16.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 16/25
Pathogenesis of adrenogenital syndromes
Other steroids
![Page 17: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/17.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 17/25
Adrenogenital syndrome (AGS)
Deficiency of enzyme products(cortisol, mineralocorticoids)
AND
Increased concentration of steroid precursors
VirilisationFemale pseudohermaphroditism
![Page 18: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/18.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 18/25
21-Hydroxylase deficiency
Most frequent cause of AGSMajor forms:
Classic 21-Hydroxylase deficiency (1:14.000)salt-losing form
simple virilising form
Non-classic 21-Hydroxylase deficiency(1:50-1:1000)
late-onset Form (postnatal viri lisation, hirsut ism,infertil ity, menstrual cycle abnormalities)
asymptomatic forms: only hormonal examinationscan detect them
![Page 19: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/19.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 19/25
Pathogenesis of 21-hydroxylase deficiency
Testosteron
![Page 20: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/20.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 20/25
21-hydroxylase deficiency
Salt-losing form:deficiency of cortisol and aldosterone:
hyperkalemia, hyponatremia, fluid
loss, shock.
Increased production of DHEA,androstendion and testosterone,
therefore virilisation.
![Page 21: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/21.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 21/25
Hormonal Diagnosis:
increased serum concentraton of steroid
precursors: 17-OH-Progesteron (>80ng/ml) and urinary pregnantriol.
Synacthen test (tetracosactid, 250 g i.v.)
Therapy of 21-hydroxylase deficiencyGlukokortikoids
Hydrocortison:
Adults 20-30 mg/Tag
Dexamethason
Adults: 0.25-0.5 mg am Abend
![Page 22: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/22.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 22/25
Polycystic Ovarian Syndrome (PCO),
Stein-Leventhal Syndrom
![Page 23: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/23.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 23/25
PCO diagnostic criteria
Hyperandrogenism with or without skinalterations
Abnormalities of menstrual cycle
raromenorrhoea or secondary
amenorrhoeaOther causes of hyperandrogenism
excluded (e.g.: adrenogenital syndrome,
Cushing syndrome)
![Page 24: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/24.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 24/25
Hormone results in PCO
Increased serum testosterone and
DHEAS
High insulin concentrations (oral glucose
tolerance test, 75 g)Increased LH/FSH ratio
![Page 25: Enfermedades de Las Gonodas](https://reader031.fdocuments.es/reader031/viewer/2022021302/577c810f1a28abe054ab4da8/html5/thumbnails/25.jpg)
8/18/2019 Enfermedades de Las Gonodas
http://slidepdf.com/reader/full/enfermedades-de-las-gonodas 25/25
Therapy of PCO
Weight reduction.Metformin against insulin resistance
Contraceptive pil ls estrogen +cyproterone-acetate antiandrogenic
protesterone derivativeInduction of ovulation (Clomiphen, LH,FSH, GnRH agonists)
Gynecologic interventions: lasercoagulation, elektrocoagulation, partialwedge resection of the ovaries