SHORT STATURE Karen Estrella H. PGY-1

Is she short?

Definition Standing height > 2SD below the mean (< 2.5 percentile) for gender and chronological age. Compare the child’s height with that of a larger population of a similar background and mid-parental target height.

How to measure Ht? Ideally with calibrated stadiometer Wall mounted Tabletop recumbent (length)< 2 yrs old Children who can’t stand: Arm span should approximate the height (>8yrs old) **Consider: a decrease of 1.25cm in Ht measurement when standing

Aspects to consider: FHx: PMHx: Development Nutrition Parents’ and siblings’ heights, onset of puberty medical problems PMHx: Birth Hx Known diseases Development Nutrition Age of pubertal development Boys: testes > 2.5 cm Girls: breast enlargement (growth spurt 2 yrs prior to boys, peak growth velocity of 8.5 cm/yr Any current symptoms Weight

Dysmorphic features Complete PE Body proportions: Arm span: European origin: the arm span should approximate the height (>8yrs old) Asian: proportionally shorter arms Africans had significantly longer arms. Lower segment (LS): Measure from the symphysis pubis to the floor. Upper segment (US): Subtract the LS from the height. US/LS ratio is calculated by dividing the US by the LS. About 1.7 at birth and decreases to 1 at about age 10, where it remains throughout adulthood (may increase slightly in puberty)

Target height of the child BOYS: [Father’s ht (cm)+ (mother’s Ht (cm)+ 13)] 2 GIRLS: [(Father’s ht (cm) -13) + mother’s Ht(cm)] 2 Inches: change 13 for 5’’

Growth velocity Most important aspect of growth evaluation Change in standing Ht over: Infants: 4 mo Children: 6mo Normal (cm/yr) 1y: 25 2y: 12 3y: 8 Then until puberty: 4-7 cm

Bone age (skeletal maturation) Greulich and Pyle (compare epyphiseal centers in hand and wrist)

Greulich & Pyle Atlas

Causes:

Appropiate target height Common causes: Familiar (genetic) Constitutional BA=CA BA<CA N growth veloc Appropiate target height

Causes:

Additional workup

Deceleration in a well-nourished or obese child: GHD, hypothyroidism, glucocorticoid excess: TSH, T4 Karyotype IGF1, IGFBP3 GH stimulation tests ACTH test

GH stimulation test Insulin-induced hypoglycemia: most powerful, but more risk. OTHER (2 serial tests): Arginine levodopa, propranolol with glucagon, exercise, clonidine GHD: GH peak after stimulation < 10 ng/ml

Indications for GH therapy GH deficiency Turner Renal insufficiency Prader-willi SGA who haven’t reach the 5th percentile by 2 years of age ISS and are not expected to reach an adult height in the normal adult height. HIV + wasting syndrome

Deceleration in a thin child: GI, nutritional, renal or chronic systemic disease CBC , ESR Antiendomysial, transglutaminase, antigliadin Sweat chloride testing Electrolytes Albumin, transferrin UA

Dysmorphic features: Genetic: Karyotype Geneticist referral Skelelal dysplasia radiography survey

Decelaration of linear growth in Adolescence: Delayed puberty Hypogonadotropic Diseases(Klinefelter, Kallman) Serum gonadotropin levels

Kaplowitz, (AAP news, 2005)Be prudent in referring short stature to the endocrinologist At or > 3 percentile and appear to be following an established channel on the growth chart: remeasure in 6-12 mo WNL but 1 single measurement deviates for regular pattern of growth, remeasure and if confirmed value, check in 6 mo. If continue look for pathology Fall-off in Wt over time with normal linear growth: look for GI problems Healthy but sustained fall-off in Ht and Wt in 1st 2 yrs, follow and refer if persists Later pubertal children, physically mature and short and leveling off in the linear growth: near to f of growth plates

Educate the family and the child about short stature

Questions

In which of the following conditions is the BA consistent with chronological age (not delayed)? Acquired hypothyroidism Constitutional delay Familial short stature Glucocorticoid excess Psychological dwarfism

Which of the following statements regarding growth in children is true? Crossing percentiles in the first 3 yrs after birth can be normal The best indicator of the appropiateness of a child’s groth is the comparison of the child’s actual height with the target height. The pubertal growth spurt occurs later un puberty in girls than it does in boys The U/L body segment ratio is at its highest during puberty The wt-for ht ratio has little importance in the evaluation of a child who has short stature.

You are evaluating a 6yo girl for short stature You are evaluating a 6yo girl for short stature. Her growth chart reveals a birth length at 60th percentile, and a current height at 5th percentile. Her growth velocity in the last 3 yrs has been 3cm/yr. Her weight is at the 50th percentile. On PE: wnl, and her intelligence appears normal. There are no midline defects or dysmorphic features. Her BA is 4 yrs. What is the most llikely dx? Congenital hypothiroidism Crohn disease GH deficiency Spondilodysplasia Turner