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Pediatric Gynecology Prof.dr hab.med. Izabella Rzepka-Górska.

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Presentation on theme: "Pediatric Gynecology Prof.dr hab.med. Izabella Rzepka-Górska."— Presentation transcript:

1 Pediatric Gynecology Prof.dr hab.med. Izabella Rzepka-Górska

2 R.Peter Czech Republic – Prague 1940

3 FIGIJ  International Federation of Infantile and Juvenile Gynecology  1971.

4  May 2004,  Atheny, Grecee  President : Dan Apter - Finland XIII World Congress of Pediatric Adolescent Gynecology

5 Pulse generator The nucleus arcuate cells of hypothalamus produce gonadoliberine GnRH.

6 The gonadoliberine is working when it is secreting pulsatingly.

7 Secretion of gonadotropin Fetal life autonomic negative feed-back Newborn negative feed-back Childhood Suppresion of conjugation Puberty negative positive Feed back

8 Regulation of GnRH function  Restraint  - Beta- endorphin  - NPY  - CRH  - melatonin  Simulation - adrenergic activity

9 Metabolic signal Critical body mass

10 17 – 18 % of body fat is needed at time of menarche

11 22 % of body fat is needed for normal menstruation

12

13 Leptin OB

14 Leptin

15 OB ( obesity gene) L eptin – protein produced by OB

16 L eptin gives information to brain about quantity of adipose tissue needed to begin the puberty.

17 Leptin is needed for normal functioning of pulse generator

18 S tages of puberty according to Tanner

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20 The first menstruation menarche

21 M ost menstruation abnormalities in the first years after menarche are physiological

22 Anorexia nervosa G irls may restrict food intake to serval hundred calories per day (which often induces vomiting or abuse of laxatives).

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24 Precoccious puberty

25 Groups tested Premature puberty of central origin 19 % Premature thelearche 67 % Premature adrenarche 12 % Premature axillarche 1 % Premature ovarian puberty 2 %

26 Premature thelarche

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28 Premature pubarche

29 12% of girls beetwen 6- 9 y.

30 Treatment is not necessary

31 C entral precoccious puberty ( stimulus for its development is GnRH secreted in pulses by hypothalamus.)

32

33 A ccelerated growth and advanced skeletal maturation is important beetwen true precocious and premature thelarche

34 Diagnosis USG - mucus of the uterus follicle apparatus E 2 - estradiol Bone age - significantly greater than the chronological age CT - computed tomography

35 Therapy with GnRH G nRH agonist supress LH and FSH and return of estradiol to the prepubertal level

36

37 Delayed puberty Constitutional form Diabetes melitius Leukemia therapy - ( radiation or chemiotherapy) Gonadal dysgenesis

38  Turner’s syndrome  Pure gonadal dysgenesis  Mixed gonadal dysgenesis

39 Turner’s syndrome 45X, 45X/46XX karyotype

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41 Stigmata of Turner’s syndrome  short stature  broad chest  webbed neck  low hairline  cubitus valgus  low-set ears  micrognathia  lymphedame  multiple pigmented nevi

42 Turner’s syndrome  cardiac anomalies 30 – 50 %  renal anomalies 35 – 70 %  gonadal abnormality 90 – 95 % (no follicle apparatus)

43  hormone growth < 10 years old  small doses of estrogen years old  HRT after 18 - end of growth Treatment of Turner’s syndrome

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45 Pure gonadal dysgenesis  patient of normal or tall stature  FSH, LH high  gonadal abnormality  46XX or 46 XY karyotype

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47 Presence of Y chromosome Patients have increased risk of gonadal tumors

48 Gonadoblastoma  E 2  T

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50 Gonadoblastoma neoplasms in girls PatientAgePubertalTraits(Tanner)Karyotype GonadotropinsmIU/ml E 2 pg/mlT ng/ ml USG Folliculoma apparatus FSHLH KM17 M5P3A3M5P3A3M5P3A3M5P3A346XY68,045,030,0-- BM17 M3P4A3M3P4A3M3P4A3M3P4A346XY64,866,540,5-- KD16 M2P4A2M2P4A2M2P4A2M2P4A246XY82,052,0-5,8- KA18 M 1/2 P 4 A 3 46XY89,7631,581,0--

51 Treatment of pure gonadal dysgenesis 46 XY Prophylactic gonadectomy 46 XX HRT

52 Androgen insensitivity syndrome (testicular feminisation)

53

54 Diagnosis  46 XY karyotype  the gonads ( tests intraabdominal)  good breast development  absent public and axillary hair  short vagina  uterus and cervix are absent

55 A n immediate gonadectomy is not needed in the patient with androgen insensitivity syndrome in particular if it is in its complete form

56 Treatment  gonadectomy over 18 years old  ERT

57 Arenal hyperplasia Arenal enzyme deficiency ( 21 hydroxylase) Diagnosis  46 XX karyotype  USG (normal uterus small  ovaries with follicle apparatus)  Virillisation ( clitoral enlargement,  hirsutismus)  Testosterone ( T)

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63 Treatment  Hormonal treatment (glycocortycoid)  Operative treatment (virilisation of genital tract) at the onset of puberty

64 Tumors of the ovaries  germ cell tumors  gonadoblastoma  sex cord stromal tumors  epithelial tumors  functional cysts ( follicular, corpus luteum)

65 Germ cell tumor  Gonadoblastoma  Dysgerminoma  Choriocarcinoma  Endodermal sinus tumor  Mesodermal mixed tumor

66 L.pNameAge Type of neoplasm Treatment 1CA17Dysgerminoma Adnexectomy Omentectomy.Appendectomy CR 2ŁA17Dysgerminoma Adnexectomy. Omentectomy. App 3KJ18DysgerminomaAdnexectomy.Omentectomy.App.Daughter 4NB18Dysgerminoma Gonadectomy bilateralis Karyotype 46XY 5NA13Dysgerminoma Gonadectomy bilateralis Karyotype 46XY 6KA15Dysgerminoma Gonadectomy bilateralis Karyotype 46XY 7SM18Dysgerminoma Gonadectomy bilateralis Karyotype 46XY 8WM13 Endodermal sin.tumor Adnexectomy.OmentectomyCR 9PA13 Adnexectomy.Omentectomy- 10WK17 Teratoma immaturum Adnexectomy.OmentectomyCR 11KJ17 Adnexectomy.Omentectomy CR 12SE25 Teratoma immaturum Hysterectomia abdominalis cum adnexis.Omentectomy.Appendectomy Death 13KN30 Carcinoma embrionale Adnexectomy.Omentectomy Death 14JM23Dysgerminoma Adnexectomy dex. Omentectomy.Appendectomy Son 15AB20 Teratoma immaturum Adnexectomy. Omentectomy Appendectomy. CR Germ Cell Tumors in Girls

67 Endodermal sinus tumor

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69 Neoplasms markers AFP CA 125 βHCG LDH E 2, T

70 Conclusions Fertility sparing operative treatment is prefered when the karyotype is normal

71 Gonadal neoplasms  Folliculoma  Thecoma –fibroma  Sertolioma Leydigioma

72 Folliculoma

73 L.pNameAgeSymptoms E 2 pg/ml Treatment 1KN4 Premature puberty 244,3Ovariectomydeath 2GJ6 1000,0Ovariectomy- 3SM7 98,0Ovariectomy Succesfull child birlth 4SM7 Premature puberty -Ovariectomy- Premature ovarian puberty

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76 1-2% Epithelial malignant tumor

77 L.pNameAgeDiagnosisGradeFIGOTreatment 1SB19 CystadenoCa serosum G1IA Adnexectomy.. Excisio prob. Omentectomy. Sectio ceasarea m x 2 2ŻM16 CystadenoCa mucinosum G1IIB Hysterectomia abd. cum adnexis CR 3MM19 CystadenoCa papillare serosum et mucinosum G1IV Hysterectomia abd.cum adnexis CR 4GM17 Ca metastaticum ovari (Ca ventriculi) -IV Ovariectomy sin. Excisio tumoris ovari sin. Omentectomy. Death 5SK19 CystadenoCa papillare mucinosum G1IA Adnexectomy sin. Excio prob.ovari dex. omentectomy CR 6OM22 CystadenoCa papillare serosum G1IC Amputatio corp.uteri cum adnexis. Omentectomy Appendectomy CR 7LJ19 CystadenoCa serosum G1I Adnexectomy dex. Excisio prob. ovari sin. Omentectomy CR 8WM17 CystadenoCa mucinosum GII Adnexectomy dex. Excisio prob.ovarii sin. Omentectomy. Appendectomy CR Epithelial malignant tumor

78 CA 125

79 Ca 19-9

80 CEA

81 Hormonally active neoplasms of the ovaries ESTRADIOL E 2 Folliculoma Gonadoblastoma Thecoma

82 Treatment of malignant neoplasms  operative  chemotherapy

83 Abnormalities of the female reproductive tract  imperforate hymen  vaginal agenesis  cervical atresia  uterus didelphys with obstructed hemivagina

84 Imperforate hymen

85 Vaginal agenesis with rudimentary uterine horns

86 Vaginal agenesis with agenesis of the cervix

87 Uterus didelphys with obstructed hemivagina

88 Girls with many sexual partners and girls smokers High risk group of cervix carcinoma

89 Sexualy Transmitted Diseases Pelvic Inflammatory Diesease

90 Trichomonas vaginalis

91 Chlamydia trachomatis 40 % girls of the high risk group

92 Chlamydia trachomatis

93 Virus HPV 25 % girls of the high risk group

94 HPV

95 induced or associated CIN

96 CIN I

97 CIN II

98 CIN III

99 Prevention of uterine cervix carcinoma


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