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35 Cards in this Set

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Length-Height measurements:
*Measure length to 24 months; measure stature 36 months to adult:

*Measure more appropriate parameter or both in 24 to 36 months olds

*In 2-3 year old stature is +/- 1.25 cm lower than length
*Measure to 0.1cm; repeat measure should be within 1 cm.
*Record average of 3 measurements; use online calculators for Z score etc
How do you do a height measurement?
*Canthus to ear canal
*Canthus to ear canal
Auxology of Height in Childhood :
*Measured length or stature (standing height) compared to appropriate reference ranges by chronologic age (“growth charts”)

*Estimation of genetic potential by reference to parental height (“midparental height”)

*Estimation of velocity of change in height by reference ranges (“growth charts”)

*Estimation of progression of process by radiographic assessment of bone maturity (“bone age”)

*Prediction of total height to be attained estimated by bone age and measured height

*Analysis of proportionality of growth (“dysmorphic features”)

*Relationship between height and weight
Growth Charts:
*WHO charts are prospective and reflect documented progressive growth of optimally healthy children

*CDC charts are cross-sectional without reference and include all children within the reference point

*Use WHO for birth to 2 years

*Use CDC for 2 years to adult

*Differences are more for weight than length
Breast Feeding and growth:
*Healthy breastfed infants typically put on weight more slowly than formula-fed infants in the first year of life.

*Formula-fed infants typically gain weight more quickly after about 3 months of age.

*Differences in weight patterns continue even after complimentary foods are introduced

*Breast Feeding significantly higher percentage in WHO data set.
components of growth charts:
*cm, inches 
*percentiles
*cm, inches
*percentiles
Midparental Height:
know how to calculate.
growth velocity:
maximum velocity in utero
maximum velocity in utero
Bone Age
top left: baby
bottom right: age 19
top left: baby
bottom right: age 19
*Bone Age-Greulich-Pyle Atlas
*most commonly used in US
*Bone Age- Greulich-Pyle Atlas
*most commonly used in US
*bone age should be within 2 SDs of actual age
Height Prediction:
*Bayley-Pinneau tables use chronological age and % of ultimate height attained by Greulich and Pyle bone age to predict final adult height

*Tanner/Whitehouse method use bone age in british children, mid-parental height, and chronologic age (also adjusted for menarche in girls) to predict adult height
Delayed: the kid catches up
GH DEFICIENCY
GH DEFICIENCY
Stature Weight Relationship:
*Short stature relative increase weight:height
hGh deficiency
hGh insensitviity
hypothyroidism
Cushing Syndrome
pseudo-hypoparathyroidism

*Short stature decreased/abnormal weight:height
“Failure to Thrive”
psychosocial deprivation
inadequate calorie intake
anorexia/bulemia
chronic illness
Indications to Formally Test for Growth Hormone Deficiency:
*DON'T MEMORIZE THESE NUMBERS*
*Major reduction in attained height
<-3.0 SD below mean
<-1.5 SD mid-parental height
<-2.0 below mean and reduction of more than
-0.5 SD over 1 year or reduction in height velocity <-1.0 SD over past year

*Reduction in height velocity
-2.5 SD over 1 year
-1.5 SD over 2 years

*Signs of intracranial lesion, hypopituitarism, or overt hGh deficiency (hypoglycemia, jaundice. craniofacial abnormalities etc.)
EVALUATION OF CHILDREN WITH SHORT STATURE:
EVALUATION OF CHILDREN WITH SHORT STATURE:
a) Height < -3.5 SD
b) Growth velocity <-2 SD chronologic age
c) Predicted height < -2.5 SD mid-parental height
Right Sided Cardiac Anomalies
Left Sided Cardiac Anomalies
Growth Hormone Deficiency IN Children:
*Continuum from insufficiency to deficiency
*Requires a clinical diagnosis based upon synthesis of historical, genetic, auxologic (growth parameters), and appropriate laboratory, and diagnostic provocative testing
*Magnetic Resonance Imaging may be extremely valuable in many circumstances

*The classical clinical phenotype, which is often present
either at birth or within the first years of life, includes:
*short stature (both in relation to peers and also parents) poor growth (Height Velocity <-2.5th centile for at least 1 year), and in severe GHD HV may be <4 cm/year
*delayed bone age (with associated delayed dentition and puberty).

*In addition the following are noted:
-increased skinfolds
-small hands and feet
-“doll-like” facies due to mid-facial hypoplasia
-micropenis in males
-hypoglycemia may occur (especially in the neonate).
Growth Hormone Deficiency in Adults:
*Abnormal body composition (↑ fat mass and ↓ lean body or muscle mass)
↓ bone mineral density (with ↑ fracture risk)
↓ exercise capacity (perhaps as a result of unfavorable body composition)

*Abnormal serum lipid profile (↑ cardiovasc risk ?)
↑ total cholesterol, ↑ low density lipoprotein [LDL] cholesterol
↑ lipoprotein a, ↑apolipoprotein B, and ↑ triglycerides,
↓ high density lipoprotein [HDL] cholesterol)

*Impaired quality-of-life (including social isolation, depression, and ↓ sense of well-being)
Isolated Growth Hormone Deficiency:
*1:3000 to 1:10,000 estimated prevalence
*Majority are sporadic
*Genetic mutations are identifiable in approximately 11%

*Genes identified include:
GH 1 which encodes GH
GHRHR which encodes GHRH receptor
SHOX 3 which encodes homeobox transcription factor
Impaired Height and Linear Growth --Genetic Causes:
*Defective genes which encode transcription factors required for pituitary growth (Prop1 etc.)

*Defects in GH-IGF-1 axis - GHRF, hGH, their receptors, signaling pathways, binding proteins, transport proteins

*Genes that effect growth directly – e.g. SHOX 3- “short stature”, Turner syndrome, FGFR3 fibrous growth factor receptor 3 (achondroplasia)
“After the ceremony (wedding Charles Stratton and Lavinia Warren Feb 1863), the hordes chased the couple’s carriage on foot to the Metropolitan Hotel, the reception site, where there awaited a treasury of lavish jewelry, furs and fine watches from the likes of the Vanderbilts, the Astors, the Lincolns and even Edwin Booth, the Shakespearean actor and brother of future Lincoln assassin, John Wilkes Booth.

Then it was time to hit the road, with stops in Philadelphia and finally Washington, where Abraham Lincoln hosted a reception at the White House for the Strattons, the president’s family and his cabinet. “
History of Growth Hormone Treatment:
*Raben used purified pituitary extract of GH (pitGH) to treat a panhypopituitary teenager 1958

*National Pituitary Agency formed 1960

*Structure of hGh identified 1977

*hGh cloned 1979

*Recombinant hGh developed (rhGh) 1981

*27,00 patients treated with extract(pitGH) 1963-1985

*Recognition of Creutsfeldt-Jakob encephalopathy and discontinuation of cadeveric sources 1985

*Synthetic rhGh FDA approved 1985
Growth Hormone and IGF-1:
*Growth hormone has direct cellular effects

*The major indirect actions of hGh are mediated by IGF-1 (insulin-like growth factor -1)

*IGF-1 is transported in the circulation by association with insulin-like binding proteins (IGBF-3), and acid labile sub-unit of IGF-1 transport (ALS) in a ternary complex

*IGF-1, IGFBP-3 and ALS are all hGh dependent
IGF-1 measurements:
*IGF-1 levels vary 30-40% day to day

*Assay is difficult; low normal ranges in infants

*Many authors recommend reference to “bone age”; normal IGF-1 for bone age excludes severe hGh deficiency but not milder forms

*IGF-1 varies with nutritional status

*IGFBP3 probably most helpful in young children

*IGFBP3 less variability with nutrition; infants with normally measurable levels
Growth Hormone Insensitivity:
*Growth failure with low IGF-1 not responsive to growth hormone treatment

*Multiple different defects in the GH-IGF-1 axis

*May be accompanied by low IGF BP-3

*mecasermin: recombinant IGF-1 linked to recombinant IGF binding protein 3 may be useful in treatment
Laron Syndrome:
*Laron Syndrome of dwarfism,
hypoglycemia, micropenis etc.
due to resisitance to HgH

*Autosomal recessive inheritance

*Defective hGh receptor

*Increased hGh levels and low
circulating levels of IGF-1.

*Classically accompanied by low levels
of GHBP-3 Response to rIGF-1 treatment
Growth charts for special syndromes?
you need em
you need em
Growth Hormone Provocative Tests:
*In many clinical instances one or even two provocative tests must be performed to support GH deficiency

*Tests Include:
-Insulin induced hypoglycemia (ITT; potential morbidity due to hypoglycemia)
-Arginine-GHRH* infusion
-Glucagon injection
-Arginine infusion or oral L-Dopa or both at once
-Ghrelin mimetic (oral) Macimorelin
Diagnosis of hGh Deficiency:
*hGh measurement is a poor test of hGh def
*IFG-1 measurement is a poor test of hGh def
*IGFB measurements are a poor test of hGh def
*Provocative tests are poor tests of hGh def
*IGF-1 Generation testing is a poor test of hGh “resistance/sensitivity”

uhhhh...so call an endocrinologist
Treatment of GROWTH HORMONE DEFICIENCY :
*Human Recombinant Growth Hormone
*Wide variety and selection of preparations

*Daily injection; other schedules and long-acting preparations under study

*Variety of injection devices

*No clear differences - patient preference

*Cost range $3,000- $10,000.00 /yr

*Wide range of physician enthusiasm for use in adults
Growth Hormone Therapy Approved Uses:
Growth hormone deficient children
Adults with severe GHD
Children with chronic renal insufficiency (CRI)
Noonan syndrome (NS)
Turner syndrome (TS)
Prader-Willi syndrome (PWS)
A history of small for gestational age (SGA)
Idiopathic short stature (ISS)
Short stature homeobox (SHOX) gene deficiency
HIV wasting
Short bowel syndrome (SBS)
Adverse Effects of GH Therapy:
*Children:
Brain swelling (“pseudotumor cerebri”)
Slipped capital head (probably)
Rare: pancreatitis, gynecomastia, ↑ nevi
Meningioma (associated with CNS radiation rx)

*Adults (think about symptoms of acromegaly):
Edema, carpal tunnel syndrome, arthralgias
Peripheral neuropathy
↑ glucose intolerance
Growth Hormone Deficiency Benefits of Treatment: Children:
*Factors which affect success rate of therapy
Severity of deficiency
Parental height
Age and height at start of treatment
Height at start of puberty
Dose and duration of treatment
Treatment compliance

*Ultimate final height with optimal treatment approaches (-1.5) to ( -0.8) SDS
Costs and effectiveness of GH therapy in kids:
pretty expensive for 3"
pretty expensive for 3"
Growth Hormone Deficiency Benefits of Treatment: Adults:
*Difficult to critically define the benefits of treatment:
-No ideal prospective studies
-Clinical picture obscured by co-incident comorbidities which include hypothalamic and pituitary disease and injury
-Ready parameters of benefit are limited to surrogate markers

*Need to re-evaluate childhood patients with isolated pituitary insufficiency as adults