02 kuliah semester vi_2011_1

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    Disorders of growth and puberty

    Muhammad Faizi, AY Heryana, Netty EP

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    Introduction

    Growth is the fundamental physiologic process thatcharacterizes childhood.

    Determined by genetic, psychosocial and economicfactors.

    It should be closely monitored by health providersand families alike as a benchmark of a childs health.

    Growth assessment requires an understanding of thephysiology of growth.

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    Physiology of growth

    Prenatal growth regulation Genetic, uterine size, placenta, metabolic status, nutrition.

    Hormonal: insulin, IGFs.

    Postnatal growth regulation Adequate NUTRITION.

    Caring ENVIRONMENT.

    Normal CHROMOSOMES.

    Good HEALTH.

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    Physiology of growth

    4 human growth phases:

    Fetal.

    Infantile.

    Childhood.

    Pubertal.

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    Fetal growth:

    Peaks: 10 cm/month end of 2nd trimester.

    Maximum weight gain 3rd trimester.

    Nutrient supply via the placenta is the maingrowth rate-limiting step.

    Placenta producing growth factors such asgrowth hormone variant, human placentallactogen and organ-specific hormones such asCRH, hepatic and epidermal growth factors.

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    Fetal growth:

    The fetal pancreas releases insulin inresponse to nutrient supply and this has

    direct growth-promoting effects.

    Fetus IGFs of which IGF-2 predominatesand modulates the growth factor actions

    with specific binding proteins.

    Thyroid and growth hormone dont play a

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    Fetal growth:

    Placental failure or damage results in agrowth-restricted infant. Including:abnormal implantation, vascularinsufficiency, or infarction.

    Symmetrical.

    Asymmetrical.

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    Infantile growth:

    Extension of the fetal growth phase beforegrowth becomes hormone dependent.

    Early growth in height and weight requiresadequate nutrition but also normal thyroidfunction and bone metabolism.

    HPA axis becomes increasingly active andinfants with growth hormone deficiency areshorter than may be expected even duringthe first year.

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    Infantile growth:

    The first 2 year: catch-up or catch-down.

    Catch-up growth starts soon after birth and is

    completed over 618 months.

    Catch-down growth commences between 3and 6 months and is completed by 920

    months.

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    Genetically pote

    height

    CanalizationCatch-up

    cm

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    Genetically pote

    height

    CanalizationCatch-down

    cm

    Childh d h

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    Childhood growth:

    Requires growth hormone (GH)action on epiphyseal cartilage cells

    to produce IGF-1, the majorpostnatal growth factor that

    stimulates cell division and growth.

    Childh d h

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    Childhood growth:

    By 4 years of age, average growth velocityhas declined to 7 cm/year and remains

    relatively steady until adolescence, the

    prepubertal nadir in average velocity being

    5 to 5.5 cm/year.

    During childhood, GH, in addition to thyroidhormone, is the major determinant of

    growth.

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    T f hildh d th

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    Type of childhood growth:

    T f hildh d th

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    Type of childhood growth:

    P b t l th

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    Pubertal growth

    Puberty may be defined as the transitionfrom the prepubertal state through the

    development of secondary sexual

    characteristics to the achievement of adult

    stature.

    b t l i iti ti

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    ubertal initiation

    actors:

    Genetic. Nutrition. Neurotransmitter. Hormonal.

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    Gonadotropin Patern

    Juvenile pause

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    Secondary sex Development from Tanner

    F

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    E l ti f th

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    Evaluation of growth

    Anthropometry: reliability & reproducibility.

    Training.

    Equipment.

    Plotting.

    Absolute height / Plotting. 2SD - -3SD : 80% normal variant.

    < -3SD : 80% pathologic.

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    evaluation

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    evaluation . . .

    Height velocity

    Measurement at 6 mos interval.

    Deceleration / crossing percentiles onage > 2 y - puberty: indicates

    pathologic until proven otherwise.

    Normal velocity indicates normalgrowth.

    evaluation

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    Weight vs height relationship.

    o W/H ratio orBMI: suggestiveendocrine causes.

    o W/H ratio orBMI: suggestivesystemic disease.

    evaluation . . .

    valuation

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    dy proportion.Measurement

    Sitting height and standing height.

    Arm span.

    Head circumference.

    Upper/lower segment ratio

    Birth = 1.7 and end of puberty = 1.

    valuation . . .

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    Adult size prediction Target height ( MPH )

    Boy = Fh+Mh+13

    2

    Girl= Fh-13+Mh2

    Predicted Final Height

    MPH + 8.5 cm

    t

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    . . . parameterne Age

    Greulich & Pyle

    Comparison of left wrist.

    Prediction of FH after 6 years.

    Table Bayley & Pinneau.

    Tanner Whitehouse II

    Maturation of ossification center.

    More reliable : scoring system.

    Complicated.

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    ormal height velocity:

    Familial short stature

    Constitutional delay in growth and development

    oor height velocity:

    Usually pathological

    Proportionate

    Disproportionate

    Normal growth velocity

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    Normal growth velocity

    Poor growth velocity

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    Poor growth velocity

    Diagnostic Approach to Short Statur

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    Diagnostic Approach to Short Statur

    Bone

    Dysplasi

    FSS

    Short Stature

    Abnormal

    GV

    Normal GV

    Constitutional Delay

    Proportional Dysproporti

    W/H W/H

    Endocrine

    Thank you

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    Thank you