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GROWTH AND GROWTH DISORDERS
dr. H. Hakimi, Sp.AK dr. H. Charles Darwin Siregar, Sp.A dr. Melda Deliana, Sp.AK dr. Siska Mayasari Lubis, Sp.A Pediatric Endocrinology Division H.Adam Malik Hospital/Medical School University of Sumatera Utara
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Physiology of Hormones
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CONTROL OF GROWTH
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GROWTH EVALUATION Average growth velocity at different phases :
1. Prenatal growth ,2 to 1,5 cm/wk 2. Infancy to 28 cm/yr 3. Childhood to 6,5 cm/yr 4. Puberty 8,3 cm/yr (girls) 9,5 cm/yr (boys)
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GROWTH CHART A chart that reflects the height and weight of a certain population which is specific for race, age, and sex Growth charts fall into several categories : distance charts, velocity, cross-sectional charts and longitudinal charts There are charts available for certain genetic syndromes including Turner syndrome
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PARENTAL TARGET HEIGHT
Potential genetic height: The range of adult height that should be achieved the child regarding to the biological parents final height Boys = (Fh + 13) + Mh 8.5 cm 2 Girls = (Fh - 13) + Mh 8.5 cm 2
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GROWTH VELOCITY Growth velocity is calculated using a minimum of two measurements, obtained across time, with the plotted data point representing the mean growth velocity during the interval as opposed to the measurable increment between the two time points Chronological age according to birth date Height age is the appropriate age for the height observed. Obtained by drawing a horizontal line from the observed height to cross the 50th percentile of the growth chart
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SKELETAL MATURATION AND PREDICTING OF ADULT HEIGHT
Bone Age: represents an index of maturation that relates more closely than chronological age to growth.The growth potential in tubuler bones can be assesed by evaluation of the progression of ossification within the epiphyses Useful in predicting final height (if bone age >6 years old) Methods Greulich & Pyle, TW II, RWT
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Anthropometrics General Body proportion Height Weight
Head circumference Body proportion Sitting height Arm span
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GROWTH FAILURE Definitions :
- Height below 3rd percentile (-2 SDs for age and gender) - Height significantly below genetic potential ( -2 SDs below mid-parental target) - Abnormally slow growth velocity (<2 inchies or 5 cm/yr from age 3 years to puberty) - Downwardly crossing percentile channels on growth chart (after age of 18 months)
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DIFFERENTIAL DIAGNOSED OF SHORT STATURE/GROWTH FAILURE
1. Healthy but short children - Familial short stature - Constitutional growth delay 2. Non organic etiologies - Psychosocial deprivation - Nutritional dwarfing ~ Gross deficiencies : kwashiorkor, anorexia nervosa ~ Subtle macronutriens deficiencies : non organic failure to thrive (NOFTT), picky eater, fear of obesity, fear of hypercholesterolemia ~ Micronutrient deficiencies : iron, zinc
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DIFFERENTIAL DIAGNOSED OF SHORT STATURE/GROWTH FAILURE
3. Intrinsic short stature - Small for gestational age - Genetic syndrome ~ Down syndrome ~ Turner syndrome ~ Prader-Willi syndrome - Achondroplasia/Hypochondroplasia 4. Systemic diseases - Infectious : HIV,TBC - Cardiac - Renal : Acute tubular acidosis, Chronic renal insufficiency - GIT: Cystic fibrosis, inflammatory bowel disease, Celiac disease
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DIFFERENTIAL DIAGNOSED OF SHORT STATURE/GROWTH FAILURE
5. Endocrinopathies - Early puberty - Cortisol excess : endogenous & iatrogenic - Hypothyroidism - Poorly controlled DM - Inadequate growth hormone action ~ GH deficiency : isolated vs panhypopituitarism, congenital vs aqcuired ~ GH insensitivity : Laron types 1 & 2
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DIFFERENTIAL DIAGNOSIS OF CONGENITAL GH DEFICIENCY
1. Idiopatic (the most common from) 2. Identified malformations - Congenital absence of pituitary - Associations (Holoprosencephaly, Septo-optic-dysplasia, Midline defects (cleft lip, cleft palate)) 3. Identified genetic mutations (Familial multiple anterior pituitary hormone deficiency & Familial isolated GH deficiency GH gene mutations) 4. Trauma : birth trauma/perinatal insult
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DIFFERENTIAL DIAGNOSIS OF ACQUIRED GH DEFICIENCY
1. Idiopathic (the most common) 2. Infection : viral, bacterial, fungal & TBC 3. Vascular : pituitary infection or aneurysm 4. Infiltration affecting pituitary gland or sella turcica : Histiocytosis, Sarcoidosis 5. Trauma : child abuse or other closed head injury, surgical damage of the pituitary gland/stalk 6. Tumors : Craniopharyngioma, Medulloblastoma, Glioma, Pinealoma 7. Pituitary or hypothalamic irradiation 8. Chemotherapy
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KEY ELEMENTS IN HISTORY GATHERING FOR THE EVALUATION OF GROWTH FAILURE
Familial history : parents height, parents age of puberty, familial history of short stature, familial history of delayed growth or puberty, familial history of endocrinopathies or systemic illnesses Child’s history : when did growth failure begin? How psychosocially distressed is the child about his or her growth? Perinatal history, history of systemic illnesses, any signs of puberty and age at which they commenced Medication history (non prescription drugs & health food supplements) Dentition history Psychosocial history
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INITIAL SCREENING EVALUATION OF GROWTH FAILURE
1. General tests - Chemistry (kidney and liver function tests) - Complete blood cell count with differential - Sedimentation rate - Urinalysis 2. Genetic tests : chromosome in every female 3. Endocrine tests - Thyroid function tests - Growth factors : IGF-I and IGFBP-3 4. Imaging studies : bone age (anteroposterior radiograph of left hand and wrist)
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CURRENT FOOD AND DRUG ADMINISTRATION-APPROVED INDICATIONS FOR RECOMBINANT HUMAN GH THERAPY
1. Linear growth - Pediatric growth hormone deficiency - Chronic renal insufficiency - Turner syndrome - Small for gestational age - Idiopathic short stature 2. Metabolic effects - Adult GH deficiency - AIDS cachexia - Prader-Willi syndrome
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OVERGROWTH The child whose height is greater than +2 SD is less likely to be the subject of evaluation than the one whose height is less than -2 SD A child with advanced skeletal maturation and progressive deviation > 97th percentile should be evaluated for precocious pubertal development. Screening for excessive GH secretion should be initiated with random IGF-I level
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DIFFERENTIAL DIAGNOSIS OF OVERGROWTH
1. Prenatal onset - Maternal diabetes - Sotos syndrome - Weaver syndrome - Marshall-Smith syndrome - Becwith-Wiedemann syndrome 2. Postnatal onset - Familial (constitutional) tall stature - Obesity - Precocious puberty - Hyperthyroidism - Klinefelter syndrome’ - XYY syndrome - Marfan syndrome - Homocysteinuria - Excessive GH secretion
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Wudy S et al (Pediatrics 2005;116:52–57) : Patients with Idiopathic Short Stature point to an altere eating behavior that possibly contributes to their short stature. Zadik Z et al (Pediatrics 2005;116:68–72) : The nutritional status of GH-treated patients before and throughout the course of GH treatment should be monitored closely to improve the growth response and prevent nutritional deficiencies. Special emphasis should be placed on iron nurture
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97 50 3
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Potential Genetic Height
97 Potential Genetic Height 50 3 HA CA
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INFANT PERIOD Catch-down Canalization 1 2 3 Potential Genetic Height
year 1 2 3
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Growth Velocity Curve Cm/year GH Sex steroid Infant Childhood Puberty
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NORMAL
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NORMAL
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TURNER & DOWN SYNDROME
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DOWN SYNDROME
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DOWN SYNDROME
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DOWN SYNDROME PRADER-WILLI SYNDROME LARON SYNDROME
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ACHONDROPLASIA TURNER SYNDROME
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MARFAN SYNDROME
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DE CORNELIA DE LANGE SYNDROME
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NOONAN SYNDROME
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SECKEL SYNDROME
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NORMAL GROWTH VELOCITY?
SHORT STATURE NORMAL GROWTH VELOCITY? YES NO NORMAL VARIANT PATHOLOGIC BODY PROPORTION? DYSMORPHISM W/H INDEX?
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SHORT STATURE PATHOLOGIC
PROPORTIONAL DYSPROPORTIONATE W/H W/H DYSMORPHIC ENDOCRINE GH DEFICIENCY HYPOTHYROID CORTISOL EXCESS PSEUDOHYPOPARATHYROID MALNUTRITION CHRONIC INFECTION CHRONIC DISEASE (ORGANIC) PSYCHOSOCIAL IUGR
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CRANIOSPINAL IRRADIATION
SHORT STATURE PATHOLOGIC DYSMORPHIC DYSPROPORTIONATE CHROMOSOME TRISOMI 21 TURNER SYNDROME SYNDROMES ( IUGR) FETAL ALCOHOL, RUSSELL-SILVER, PRADER-WILLI, NOONAN, SECKEL, de LANGE, LARON, COCKAYNE dll SKELETAL DYSPLASIA A/HYPO CHONDROPLASIA METABOLIC DISEASES RICKETS SPINAL DISORDERS CRANIOSPINAL IRRADIATION SPONDYLODYSPLASIA
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SHORT STATURE NORMAL VARIANT FAMILIAL SS
CONSTITUTIONAL DELAY OF GROWTH AND PUBERTY FAMILIAL SS BA = CA FINAL HEIGHT < 3RD PERCENTILE APPROPRIATE WITH PGH BA < CA FINAL HEIGHT =NORMAL APPROPRIATE WITH PGH POSITIVE FAMILY HISTORY OF CDGP
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Constitutional Delay of Growth & Puberty Familial Short Stature
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Stature and Growth Which child needs more attention?
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Quiz Data of a boy Father 172 cm, Mother 166 cm
Age 7 yrs and 4 mos : 110 cm; BA 5 yrs Age 8 yrs : 114 cm; BA 6 yrs Age 9 yrs 6 mos : 122 cm; BA 7 yrs Father 172 cm, Mother 166 cm Menarche (mother) 15 yrs WHAT IS YOUR ASSESSMENT?
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GROWTH CHART STATURE Patient 1 () Patient 2 () GROWTH NORMAL ()
ABNORMAL () CATCH UP ()
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THANK YOU
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