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Short Stature

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

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  1. Short Stature Hilary Suzawa (the 5 foot tall attending) November 2005

  2. 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

  3. Growth Velocity • Changes in height over time (the reason you took calculus in college?)

  4. 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

  5. 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)

  6. 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

  7. 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

  8. 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

  9. 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

  10. 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

  11. 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

  12. 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

  13. Turner Syndrome www.endocrineonline.org

  14. Turner Syndrome

  15. Turner Syndrome • Check chromosomes • Check FSH at age 10 years—to check for ovarian failure • Check TSH—to check for autoimmune thyroiditis and hypothyroidism

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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

  21. 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

  22. 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

  23. Short Stature Ddx • Systemic Disease • Hypocaloric: Malnutrition, IBD, celiac, DM • Metabolic: Renal, hepatic, cardiac, hematologic, pulmonary • Endocrine Disorder • Hypothyroidism • Cushing’s • Growth hormone deficiency

  24. Growth Hormone Therapy • Newly approved indications for GH • Chronic renal insufficiency • Turner Syndrome • Prader-Willi Syndrome • SGA • Idiopathic short stature

  25. 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

  26. 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

  27. 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

  28. 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

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