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Short Stature. Hilary Suzawa (the 5 foot tall attending) November 2005. Definition. How short is “short”? Short stature is defined as height below the third percentile for age

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short stature

Short Stature

Hilary Suzawa

(the 5 foot tall attending)

November 2005

definition
Definition
  • How short is “short”?
  • Short stature is defined as height below the third percentile for age
  • The main question to be answered is whether a child is meant to be short (hereditary) or is short because of a medical disorder
growth velocity
Growth Velocity
  • Changes in height over time (the reason you took calculus in college?)
rules of thumb
Rules of Thumb
  • Birth length increased by 50% at 1 year
  • Birth length doubles by 4 years
  • Birth length triples by 13 years
  • Pubertal growth spurt is relatively early for girls and late for boys
  • Pubertal growth spurt in girls occurs 2 years earlier than for boys
  • Girls have growth spurt at Tanner 3 and boys at Tanner 4
rules of thumb5
Rules of Thumb
  • Birth weight is regained by day 10-14
  • Birth weight doubles at 4-6 months
  • Birth weight triples at 12 months
  • Birth weight quadruples at 24 months
  • After age 2 years, normal weight gain is ~5 lbs/year until adolescence
  • Largest rate of growth for FOC is birth till 2 months (0.5 cm/week)
crossing the line
Crossing the Line
  • During the first 3 years of life it is common for children to cross lines on the growth curve
  • During puberty it is also common to cross lines
  • During the time from 3 yrs until puberty crossing lines is more worrisome
  • Crossing two lines on the growth curve is more concerning that crossing just one
the apple tree
The Apple Tree
  • What are the parental heights?
  • When did each parent start puberty?
  • Calculate midparental height
  • For girls: (mom + dad -13 cm)/2
  • For boys: (mom + dad + 13 cm)/2
  • Most children achieve an adult height within 10 cm of the predicted height
genetic short stature
Genetic Short Stature
  • Family history of short stature
  • Normal birth length and weight
  • Height is ¾ height that is 3rd percentile for chronologic age
  • Normal annual growth rate
  • Predicted adult height is ¾ height that is 3rd percentile
  • Normal onset of puberty
  • No other cause of growth failure
constitutional growth delay
Constitutional Growth Delay
  • “Late Bloomer”
  • Slowing of growth during the first 2-3 years of life but then normal or low-normal
  • Family history of similar growth pattern and delayed puberty
  • Check bone age
    • Plain x-ray of left wrist and hand that is compared to established standards
bone age
Bone Age
  • Most conditions that cause poor linear growth also cause a delay in skeletal maturation and a retarded bone age
  • In constitutional growth delay, bone age is younger than chronologic age reflecting a potential for future growth
  • If bone age is the same as chronologic age, then genetic short stature is more likely
slide11
IUGR
  • Intrauterine Growth Retardation: birth weight <10th percentile for gestational age
  • ~70% are small because of genetics
  • ~30% are small because growth is restricted by pathologic conditions
  • 15-20% will have short stature at age 4 yrs
  • 8% will have short stature at age 18 yrs
turner syndrome
Turner Syndrome
  • Webbed neck
  • Low posterior hairline
  • Broad chest with widely spaced nipples
  • Short fourth metacarpals
  • Increased carrying angle of the arms (cubitus valgus)
  • Horseshoe kidney
  • Some individuals may not have any dysmorphic features besides short stature
turner syndrome13
Turner Syndrome

www.endocrineonline.org

turner syndrome15
Turner Syndrome
  • Check chromosomes
  • Check FSH at age 10 years—to check for ovarian failure
  • Check TSH—to check for autoimmune thyroiditis and hypothyroidism
proportions
Proportions
  • Measure the upper-to-lower body segment (U/L) ratio
    • Lower segment is from upper border of symphysis pubis to the floor
    • Upper segment is standing height minus lower segment
  • Skeletal dysplasia may have either increased or decreased U/L
systemic disease
Systemic Disease
  • The patient is underweight for height, ie the weight to height ratio is decreased
  • Weight decreases first and then height
  • Failure to thrive is associated with greater impairment in weight gain than linear growth
  • Systemic conditions
    • Malnutrition
    • Anorexia nervosa
    • Malabsorption
    • IBD
    • Celiac Dz
    • Diabetes
endocrine disorder
Endocrine Disorder
  • Preserved weight gain or frank obesity associated with poor linear growth suggests an endocrine disorder
  • Hypothyroidism
  • Cushing’s disease or exogenous glucocorticoid
  • Growth hormone deficiency
growth hormone deficiency
Growth Hormone Deficiency
  • Not useful to measure GH levels because it is pulsatile
  • Measure Insulin-like Growth Factors: IGF-1 and IGF-BP3
  • IGF-1 levels are age dependent
  • Low IGF-1 also in malnutrition, hypothyroidism, and chronic renal and liver dz
  • Low IGF-BP3 in chronic malnutrition
partial gh deficiency
Partial GH Deficiency
  • Presents after 6 mths age when maternal hormones decrease
  • Increased peri-abdominal fat and decreased muscle mass
  • Delayed dentition
  • Thin hair, poor nail growth
  • High-pitched voice
severe gh deficiency
Severe GH Deficiency
  • Hypoglycemia
  • Conjugated hyperbilirubinemia
  • Small phallus
  • Midline facial and CNS abnormalities
    • Septo-optic dysplasia
    • Coloboma
    • Midface hypoplasia
    • Cleft lip, palate
    • Single central incisor
short stature ddx
Short Stature Ddx
  • Genetic short stature
  • Constitutional Growth Delay
  • IUGR
  • Genetic Syndrome
    • Turner, Noonan, Prader-Willi, Russell-Silver
  • Disproportionate short stature
    • Skeletal Dysplasia
    • Spinal irradiation
short stature ddx25
Short Stature Ddx
  • Systemic Disease
    • Hypocaloric: Malnutrition, IBD, celiac, DM
    • Metabolic: Renal, hepatic, cardiac, hematologic, pulmonary
  • Endocrine Disorder
    • Hypothyroidism
    • Cushing’s
    • Growth hormone deficiency
growth hormone therapy
Growth Hormone Therapy
  • Newly approved indications for GH
    • Chronic renal insufficiency
    • Turner Syndrome
    • Prader-Willi Syndrome
    • SGA
    • Idiopathic short stature
gh therapy
GH Therapy
  • 6 brands of recombinant GH available, no generic
  • Daily subcutaneous injection
  • Mean age to start tx is 10 yrs old
  • Tx until reach final adult height
  • Contraindications
    • Active malignancies
    • Closed epiphyses
gh therapy28
GH Therapy
  • Common side FX: edema, reactions at the site of injection
  • Other side FX: Scoliosis worsening, hypothyroidism, otitis media
  • Serious events: SCFE, benign intracranial hypertension (reversible)
  • GH decreases insulin sensitivity so in children who were SGA must monitor annual fasting insulin and GLC
  • Theoretical risk of increased malignancy
summary
Summary
  • Calculate growth velocity and predicted height (midparental height)
  • Short and thin suspect systemic disease
  • Short and fat  suspect endocrine disease
  • Short and dysmorphic  suspect genetics
  • Short and disproportionate  suspect skeletal dysplasia
  • Consider Turner’s Syndrome in any short female
bibliography
Bibliography
  • Lee J and Menon R: Growth hormone for short children without a hormone deficiency: Issues and practices. Contemporary Pediatrics 2005; 22(10): 46-53.
  • Rose S, Vogiatzi M, and Copeland K: A General Pediatric Approach to Evaluating a Short Child. Pediatrics in Review 2005; 26 (11): 410-20.
  • Samuels R and Cohen L : Understanding growth patterns in short stature. Contemporary Pediatrics 2001.
  • MedStudy Pediatrics Board Review 1st edition Book 1