Short Stature

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# Short Stature - PowerPoint PPT Presentation

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

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
• 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
• Changes in height over time (the reason you took calculus in college?)
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 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
• 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
• 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
• 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
• “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
• 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
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
• 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 Syndrome

www.endocrineonline.org

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
• 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
• 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
• 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
• 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
• 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
• 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
• Genetic short stature
• Constitutional Growth Delay
• IUGR
• Genetic Syndrome
• Disproportionate short stature
• Skeletal Dysplasia
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
• Newly approved indications for GH
• Chronic renal insufficiency
• Turner Syndrome
• SGA
• Idiopathic short stature
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 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
• 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
• 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