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IN THE NAME OF GOD

IN THE NAME OF GOD. Precocios puberty in girls. By: Dr Neda Mostofizadeh Pediatric Endocrinologist. سوال.

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IN THE NAME OF GOD

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  1. IN THE NAME OF GOD

  2. Precocios puberty in girls By: Dr Neda Mostofizadeh Pediatric Endocrinologist

  3. سوال • دختر7ساله ای با شکایت از رویش موهای ناحیه پوبیس ولابیا ماژور مراجعه کرده است.در معاینه در صورت وی اکنه دیده میشود سرعت رشد قدی وی در سال گذشته 7 سانتی متردرسال بوده است درمعاینه پستان در مرحله 1تانراست:

  4. -نظر شما در مورد شروع بلوغ وتشخیص در بیمار چیست؟ • - چه ازمایشاتی برای بیمار میفرستیم؟ -در پیگیری درازمدت این بیمار به چه نکاتی باید توجه کرد؟ • -برای این بیمار چه دارویی تجویز میکنید؟

  5. سوال • دختر 8.5 ساله ای به شما مراجعه کرده است.او تا کنون 3 بار عادت ماهیانه شده است .پستانها در مرحله 5 تانر است.قد وی روی منحنی 75 پرسنتایل است در سونوگرافی حجم رحم 4 سی سی و ابعاد ان 42*23*30 میلی متراست.

  6. 1-تشخیص بیمار چیست؟ • 2-اقدام پاراکلینیک مهم در مورد این بیمار چیست؟ • 3-درمان بیمار فوق چیست؟

  7. Definition: • Onset of secondary sexual characters before the age of 8 years in girls and 9 years in boys. • In girls, the initial physical change is breast development. • In boys, the initial physical change is testicular growth.

  8. Classification: • Gonadotropin dependent (central). • Gonadotropin independent (peripheral). • Incomplete.(premature telarche,prematurepubarche ,premature menarche)

  9. CPP: • Early activation of the HPG axis. • 5- to 10-fold more often in girls than in boys . • Usually sporadic. • 90% of girls have an idiopathic form. • Structural CNS abnormality in up to 75% of boys .

  10. CPP:cont • Affect child's growth potential. • Height, weight, and osseous maturation are advanced. • Short ultimate stature. • Mental development is usually compatible with chronological age. • Emotional behavior and mood swings are common.

  11. Causes of CPP: • Idiopathic • Organic brain leision hypothalamic hamartoma brain tumors, hydrocephalus,severehead trauma,myelomeningocele • Hypothroidism(prolonged and untreated). • Previous excess androgen exposure. • Radiation

  12. Radiation: • Low-dose radiation (18-24 Gy)hastens the onset of puberty almost exclusively in girls. • High-dose radiation (25-47 Gy) appears to trigger precocious sexual development in both sexes. • Hypopituitarism with gonadotropin deficiency can subsequently develop as a late effect of high-dose CNS irradiation.

  13. Syndrome of Precocious Pubertyand Hypothyroidism: • In children with untreated hypothyroidism, the onset of pubertyis usually delayed until epiphyseal maturation reaches 12-13 yrof age. • Precocious puberty in a child with untreated hypothyroidismand a prepubertal bone age presents a strikingly unphysiologic association. • It is common and occurs in as many as 50% of children with severe hypothyroidism of long duration.

  14. Cont: • These children have the usual manifestations of hypothyroidism, including retardation of growth and of osseous maturation . • The cause of the hypothyroidism is usually Hashimotothyroiditis.

  15. Cont: • Sexual development in girls consists primarily of breast Enlargement and menstrual bleeding; the latter can occur even in girls with minimal breast enlargement. • Pelvic sonography can reveal large, multicystic ovaries. • Enlargement of the sella, which is typical of long-standing primary hypothyroidism, may be demonstrated by skull film or MRI.

  16. Cont: • TSH is markedly elevated, often >500 /lU/mL. • PRL is mildly elevated. • FSH is low and LH is undetectable but massively elevated TSH interact with the FSH receptor(specificity spillover), thus inducing FSH-like effects in the absence of LH effects on the gonads. • The precocious puberty associated with hypothyroidism behaves as an incomplete form of gonadotropin-dependent puberty.

  17. Cont: • Treatment of the hypothyroidism results in rapid return to normal of the biochemical and clinical manifestations. • Rapid bone age advancement and possible progression to central puberty could occur in the months following the initiation of thyroid hormone replacement, a complication that justifies delaying puberty with GnRH analogs.

  18. سوال • دختر 6.5ساله ای با شکایت ازکوتاه قدی مراجعه کرده کرده است .در بررسی های انجام شده قد وی زیر منحنی 5پرسنتایل و پستان درمرحله 3 تانر است .در مورد بیمار • Bone Age=2.5yrبه سوالات زیرپاسخ دهید. • 1-چه ازمایشی برای بیمار میفرستید؟ • 2- چه انتظاری دارید؟

  19. TFT • TSH:high • T4:low

  20. Patterns of CPP: • Rapidly progressive(Most girls particularly <6 yr and a large majority of boys). • Slowly progressive (girls >6 yr). • Spontaneously regressive(small percentage of girls ) • need for longitudinal observation at the onset of sexual development, before treatment is considered

  21. LABORATORY FINDINGS: • Increase Sex hormone concentrations. • Serum estradiolin girls is low or undetectable in the early phase of sexual precocity. • serum LH is detectable in 50-75% of girls.

  22. Lab:cont • GnRH stimulation test is a helpful diagnostic tool. • A brisk LH response (LH peak >5-10 lUlL) with predominance of LH over FSH tends to occur early in the course of precocious puberty.

  23. Lab:cont • In girls nocturnal LH and LH response to GnRH may be quite low at breast stages II to early III. • In girls LH: FSH ratio can remain low until mid-puberty.

  24. Lab:Cont: • Osseous maturation advanced(more than 2-3SD). • Pelvic ultrasonography in girls reveals progressive enlargement of the ovaries and uterus to pubertal size. • Upper limit of uterine length in prepubertal state is 3.5 cm , volume is 1.8 ml. • MRI scan demonstrates physiologic enlargement of the pituitary gland and also reveal CNS pathology.

  25. Indications for MRI: • Rapid breast development. • Estradiol>30 pglmL. • Girls <6 yr of age. • All boys • Some authorities recommend MRI scans for all children with central precocious puberty.

  26. Treatment of CPP: • GnRH agonist. • 60 to 120 micro/kg • Gonadotropic cells require pulsatile, rather than continuous, stimulation.

  27. When stop treatment: • We generally continue treatment until about age 11 in girls, and age 12 in boys. • The decision of when to discontinue GnRH agonist therapy is individualized. • Age of the child, bone age and height age, predicted height, and social desire are important.

  28. Adverse effects: • Except for a reversible decrease in bone density (of uncertain clinical significance), no serious adverse effects was seen. • Monitoring of bone density is not required . • We suggest ensuring adequate intake of calcium and Vit D during treatment

  29. Cont: • When therapy is discontinued, puberty resumes at a "pubertal" chronological age. • In girls, menarche and ovulatory cycles generally appear at an average of 16 mo (range 6-24 mo) of cessation of therapy. • long-term treatment with GnRH agonists does not appear to cause or exacerbate obesity

  30. سوال • دختر 8.5 ساله ای به شما مراجعه کرده است.او تا کنون 3 بار عادت ماهیانه شده است .پستانها در مرحله 5 تانر است.قد وی روی منحنی 75 پرسنتایل است در سونوگرافی حجم رحم 4 سی سی و ابعاد ان 42*23*30 • میلی متر است:

  31. -تشخیص بیمار چیست؟ • 2-اقدام پاراکلینیک مهم در مورد این بیمار چیست؟ • 3-درمان بیمار فوق چیست؟

  32. Precocious puberty • Brain MRI • Gn RH agonist(Dipherelin) in case of family agreement

  33. سوال • دختر 6 ساله ای با رشد دوطرفه پستان به شما مراجعه کرده است.در معاینه پستان وی در مرحله 3 تانر است. • در مورد این بیماربه سوالات زیر پاسخ دهید. • 1-چه ازمایشاتی برای بیمار ارسال می کنید • 2-ایا نیاز به ام ار ای دارد؟

  34. تستهای عملکرد تیرویید.گونادوتروپینها.استرادیول.سونو بله نیاز به ام ار ای دارد

  35. سوال • دختر 9ساله ای به شما مراجعه کرده است.در معاینه پستان در مرحله 2 تانر است.قد وی 140 سانتیمتر است .مادر وی نگران شروع بلوغ و قد ش میباشد. • 1- توصیه شما به مادر بیمار چیست؟ • 2-در صورت سیر طبیعی بلوغ چند سانتی متر به قد وی اضافه میشود؟

  36. با توجه به سن بیمار شروع بلوغ زودرس نمی باشد. • حدود 20سانتیمتر

  37. House cake

  38. Peripheral precocios puberty

  39. سوال دختر3 ساله ای با شکایت از خونریزی وازینال که تا کنون 3 بار تکرار شده مراجعه کرده است در معاینه پستان درمرحله سوم تانر است ولکه های پیگمانته ای روی شکم وی جلب توجه می کند در سونوگرافی کیستهای متعدد با ابعاد حدود 10میلی متر گزارش شده است.چه ازمایشاتی ارسال می کنید؟

  40. 1-تشخیص بیمار چیست؟ • 2-چه ازمایشات پاراکلینیک دیگری برای وی درخواست می کنید؟ • 3-درمان • 4-ایا در درمان این بیمارنقشی برای دیفرلین قایل هستید؟

  41. سوال • دختر 4ساله ای به درمانگاه غدد مراجعه کرده است .در سونوگرافی همراه وی 2 کیست به ابعاد 6و 8 میلیمتر گزارش شده است .در معاینه نکته مثبتی ندارد. • 1-نظر شما در مورد پیگیری و درمان این بیمار چیست؟

  42. Gonadotropin-independent p.puberty: • Excess sex hormones ,which is independent of the HPG axis. • It is isosexual or contrasexual. • May present with some or all of the physical changes of puberty. • FSH and LH levels are suppressed. • GnRH agonists are ineffective.

  43. Causes: Causes of GnRH independent isosexual precocity In girls, include: • Ovarian cysts:(most common cause) . • Ovarian tumors :Granulosa-cell tumors, Leydig cell tumors and gonadoblastoma. • Peutz-Jeghers Syndrome. • Adrenal Adenomas. • Maccune Albright syndrome

  44. Ovarian cysts in infancy and childhood • If cysts areasymptomatc,less than 4-5cm and simple,observation and serial sonographyevery 2 months is suffcient. • In functional ovarian cysts which causes psedopercocious puberty treatment with medroxyprogestrone is needed. • In case of recurrent ovarian cysts MAC should be considerd.

  45. MAC: McCune-Albright syndrome : • It is a rare disorder defined as the triad of: • Peripheral precocious puberty • Café-au-lait skin pigmentation • Fibrous dysplasia of bone.

  46. Cont: • Caused by a missensemutation in the gene encoding the a-subunit of Gs, that stimulates cAMPformation. • Activation of receptors ACTH, TSH, FSH, and LH receptors that operate via a cAMP-dependent mechanism.

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