Pubertas TerlambatEDIT
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Transcript of Pubertas TerlambatEDIT
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DELAYED PUBERTYDr. H. Hakimi Sp.ADr. H Charles Darwin Siregar Sp.ADr. Melda Deliana Sp.A
PEDIATRIC ENDOCRINOLOGY MEDICAL SCHOOL USU/H. Adam Malik HOSPITALMedan
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DefenitionDelayed puberty if puberty signs are absent :Male : 14 yrs oldFemale : 13 yrs oldClassificationhypergonadotrophic hypogonadismhypogonadotrophic hypogonadism
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hypogonadismLHRHLH/FSHHypothalamusPituitaryTarget Organ(gonad)Sex Steroid Primary defecthypergonadotrophic(-)
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Hypergonadotrophic hypogonadismWith chromosomal disorderDysgenesis gonadSindrom TurnerPure gonadal dysgenesisSindrom KlinefelterAndrogen Insensitivity Syndrome *
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Turner Syndrome45,XO, mosaic (45,XO/46,XX etc)1:2500 live birth ()dismorphic : web neck, shield chest, heart disorder etcMain complaint: puberty and growth disorders (final height 142 cm)
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Hypergonadotrophic hypogonadismWithout chromosomal disordercongenitaladrenal steroid biosynthesis disorder (P450c17,P450scc,3bHSD) and gonad (17-KS, P450 aromatase)anorchia, ovary resistant syndrome, LH resistanceacquiredradiation, chemotherapy, autoimune process
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hypogonadismLHRHLH/FSHHypothalamusPituitaryTarget Organ(gonad)Sex Steroid Primary defectHypogonadotrophic(-)
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Hypogonadotrophic hypogonadismConstitutional delay (frequent etiology of delayed puberty)CNS disordersTumor (craniopharyngioma, germinoma, optic glioma, histiocytosis X)Structural (mid line defect)Kallmann syndromehypopituitarism idiopathicpost radiation, chemoterapy inflamation, infiltration - hemosiderosis)
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Hypogonadotrophic hypogonadismChronicendocrine, malnutrition/anorexia nervosa, systemic disorderExcessive physical activitySindromesPrader-Willi; Laurence-Moon-Biedl
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ManagementHistoryPhysical examinationWork upTherapy
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HistoryFamily history of delayed pubertyGrowth and development dataPrevious disease and treatment Smelling function
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Physical examinationgeneral Physical examinationNeurologic examination (funduscopy)Antropometric (height, weight, upper and lower extremity ratio, rentang arm span)puberty stageSyndromes characteristic (shoeth, obesity, mental retardation, webbed neck etc)
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Work upImaging: Bone age, head CT scan/MRI & internal genital USG (atas indikasi), Hormonal (basal/ GnRH test)LH,FSH,Prolactin, Estrogen or testosterone Etcchromosomal analysis (based on indication)Smell function test
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TherapyBased on etiology : thallasemia puberty inductionSex steroid (testosteron, estrogen)gradual : Follow natural puberty developmentPrevent early epiphiseal closurefertility induction
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ConclusionMostly normal (constitutional delay of growth and puberty)Infertility etiologyPuberty induction must be done gradually