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

DefenitionDelayed puberty if puberty signs are absent :Male : 14 yrs oldFemale : 13 yrs oldClassificationhypergonadotrophic hypogonadismhypogonadotrophic hypogonadism

hypogonadismLHRHLH/FSHHypothalamusPituitaryTarget Organ(gonad)Sex Steroid Primary defecthypergonadotrophic(-)

Hypergonadotrophic hypogonadismWith chromosomal disorderDysgenesis gonadSindrom TurnerPure gonadal dysgenesisSindrom KlinefelterAndrogen Insensitivity Syndrome *

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)

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

hypogonadismLHRHLH/FSHHypothalamusPituitaryTarget Organ(gonad)Sex Steroid Primary defectHypogonadotrophic(-)

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)

Hypogonadotrophic hypogonadismChronicendocrine, malnutrition/anorexia nervosa, systemic disorderExcessive physical activitySindromesPrader-Willi; Laurence-Moon-Biedl

ManagementHistoryPhysical examinationWork upTherapy

HistoryFamily history of delayed pubertyGrowth and development dataPrevious disease and treatment Smelling function

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)

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

TherapyBased on etiology : thallasemia puberty inductionSex steroid (testosteron, estrogen)gradual : Follow natural puberty developmentPrevent early epiphiseal closurefertility induction

ConclusionMostly normal (constitutional delay of growth and puberty)Infertility etiologyPuberty induction must be done gradually