short stature karen estrella h. pgy-1. is she short?

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SHORT STATURE SHORT STATURE Karen Estrella H. Karen Estrella H. PGY-1 PGY-1

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Page 1: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

SHORT STATURESHORT STATURE

Karen Estrella H.Karen Estrella H.

PGY-1PGY-1

Page 2: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Is she short?Is she short?

Page 3: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

DefinitionDefinition

Standing height > 2SD Standing height > 2SD below the mean (< below the mean (< 2.5 percentile) for 2.5 percentile) for gender and gender and chronological age.chronological age.

Compare the child’s Compare the child’s height with that of a height with that of a larger population of a larger population of a similar background similar background and mid-parental and mid-parental target height.target height.

Page 4: SHORT STATURE Karen Estrella H. PGY-1. Is she short?
Page 5: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

How to measure Ht?How to measure Ht?

Ideally with calibrated stadiometer Ideally with calibrated stadiometer – Wall mountedWall mounted– Tabletop recumbent (length)< 2 yrs oldTabletop recumbent (length)< 2 yrs old

Children who can’t stand:Children who can’t stand:– Arm spanArm span

should approximate the height (>8yrs old)should approximate the height (>8yrs old)

**Consider: a decrease of 1.25cm in Ht measurement **Consider: a decrease of 1.25cm in Ht measurement when standingwhen standing

Page 6: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

FHx: FHx: – Parents’ and siblings’ heights,Parents’ and siblings’ heights,– onset of pubertyonset of puberty– medical problemsmedical problems

PMHx: PMHx: – Birth HxBirth Hx– Known diseasesKnown diseases

DevelopmentDevelopment NutritionNutrition Age of pubertal developmentAge of pubertal development

– Boys: testes > 2.5 cmBoys: testes > 2.5 cm– Girls: breast enlargement (growth spurt 2 yrs prior to boys, peak Girls: breast enlargement (growth spurt 2 yrs prior to boys, peak

growth velocity of 8.5 cm/yrgrowth velocity of 8.5 cm/yr Any current symptomsAny current symptoms WeightWeight

Aspects to consider:Aspects to consider:

Page 7: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Dysmorphic featuresDysmorphic features Complete PEComplete PE

Body proportions:Body proportions:

Arm span: Arm span: – European origin: the arm span should approximate the height European origin: the arm span should approximate the height

(>8yrs old)(>8yrs old)– Asian: proportionally shorter armsAsian: proportionally shorter arms– Africans had significantly longer arms.Africans had significantly longer arms.

Lower segment (LS): Lower segment (LS): – Measure from the symphysis pubis to the floor. Measure from the symphysis pubis to the floor.

Upper segment (US): Upper segment (US): – Subtract the LS from the height. Subtract the LS from the height.

US/LS ratio is calculated by dividing the US by the LS. 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 About 1.7 at birth and decreases to 1 at about age 10, where it

remains throughout adulthood (may increase slightly in puberty)remains throughout adulthood (may increase slightly in puberty)

Page 8: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

BOYS:BOYS:[Father’s ht (cm)+ ([Father’s ht (cm)+ (mother’s Ht (cm)+ 13mother’s Ht (cm)+ 13)])]

22

GIRLS:GIRLS:[([(Father’s ht (cm) -13Father’s ht (cm) -13) + mother’s Ht(cm)]) + mother’s Ht(cm)]

22

Inches: change 13 for 5’’Inches: change 13 for 5’’

Target height of the childTarget height of the child

Page 9: SHORT STATURE Karen Estrella H. PGY-1. Is she short?
Page 10: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Growth velocityGrowth velocity

Most important Most important aspect of growth aspect of growth evaluationevaluation

Change in standing Change in standing Ht over:Ht over:– Infants: 4 moInfants: 4 mo– Children: 6moChildren: 6mo

Normal (cm/yr)Normal (cm/yr)– 1y: 251y: 25– 2y: 122y: 12– 3y: 83y: 8– Then until Then until

puberty: 4-7 cmpuberty: 4-7 cm

Page 11: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Bone age (Bone age (skeletal skeletal maturation)maturation)

Greulich and Pyle (compare epyphiseal Greulich and Pyle (compare epyphiseal centers in hand and wrist)centers in hand and wrist)

Page 12: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Greulich & Pyle Atlas

Page 13: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Causes:Causes:

Page 14: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Common causes: Common causes:

Familiar (genetic)Familiar (genetic) ConstitutionalConstitutional

BABA==CACA BABA<<CACA

N growth velocN growth veloc N growth velocN growth veloc

Appropiate target Appropiate target heightheight

Appropiate target Appropiate target heightheight

Page 15: SHORT STATURE Karen Estrella H. PGY-1. Is she short?
Page 16: SHORT STATURE Karen Estrella H. PGY-1. Is she short?
Page 17: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Causes:Causes:

Page 18: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Additional workupAdditional workup

Page 19: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Deceleration in a well-nourished or Deceleration in a well-nourished or obese child:obese child:

– GHD, hypothyroidism, glucocorticoid excess: TSH, T4TSH, T4 KaryotypeKaryotype IGF1, IGFBP3IGF1, IGFBP3

– GH stimulation testsGH stimulation tests– ACTH testACTH test

Page 20: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

GH stimulation testGH stimulation test

Insulin-induced hypoglycemia: most Insulin-induced hypoglycemia: most powerful, but more risk.powerful, but more risk.

OTHER (2 serial tests):OTHER (2 serial tests):– ArginineArginine– levodopa, propranolol with glucagon, levodopa, propranolol with glucagon,

exercise, clonidineexercise, clonidine

GHD: GH peak after stimulation < 10 GHD: GH peak after stimulation < 10 ng/mlng/ml

Page 21: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Indications for GH therapyIndications for GH therapy

GH deficiencyGH deficiency TurnerTurner Renal insufficiencyRenal insufficiency Prader-williPrader-willi SGA who haven’t reach SGA who haven’t reach

the 5th percentile by 2 the 5th percentile by 2 years of ageyears of age

ISS and are not ISS and are not expected to reach an expected to reach an adult height in the adult height in the normal adult height.normal adult height.

HIV + wasting syndromeHIV + wasting syndrome

Page 22: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Deceleration in a thin child:Deceleration in a thin child:

GI, nutritional, renal orGI, nutritional, renal or

chronic systemic chronic systemic diseasedisease

CBC , ESRCBC , ESR Antiendomysial, Antiendomysial,

transglutaminase, transglutaminase, antigliadinantigliadin

Sweat chloride Sweat chloride testingtesting

ElectrolytesElectrolytes Albumin, transferrinAlbumin, transferrin UAUA

Page 23: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

– Genetic:Genetic:

KaryotypeKaryotype Geneticist referralGeneticist referral Skelelal dysplasia Skelelal dysplasia

radiography surveyradiography survey

Dysmorphic features:Dysmorphic features:

Page 24: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Decelaration of linear growth in Decelaration of linear growth in Adolescence:Adolescence:

– Delayed pubertyDelayed puberty

Hypogonadotropic Diseases(Klinefelter, Hypogonadotropic Diseases(Klinefelter, Kallman)Kallman)

– Serum gonadotropin levelsSerum gonadotropin levels

Page 25: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Kaplowitz, (AAP news, 2005)Be prudent in referring short Kaplowitz, (AAP news, 2005)Be prudent in referring short stature to the endocrinologiststature to the endocrinologist

At or > 3 percentile and appear to be following an At or > 3 percentile and appear to be following an established channel on the growth chart: established channel on the growth chart: remeasure in 6-12 moremeasure in 6-12 mo

WNL but 1 single measurement deviates for WNL but 1 single measurement deviates for regular pattern of growth, remeasure and if regular pattern of growth, remeasure and if confirmed value, check in 6 mo. If continue look confirmed value, check in 6 mo. If continue look for pathologyfor pathology

Fall-off in Wt over time with normal linear growth: Fall-off in Wt over time with normal linear growth: look for GI problemslook for GI problems

Healthy but sustained fall-off in Ht and Wt in 1st 2 Healthy but sustained fall-off in Ht and Wt in 1st 2 yrs, follow and refer if persists yrs, follow and refer if persists

Later pubertal children, physically mature and Later pubertal children, physically mature and short and leveling off in the linear growth: near to short and leveling off in the linear growth: near to f of growth platesf of growth plates

Page 26: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Educate the Educate the family and the family and the

child about short child about short staturestature

Page 27: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

QuestionsQuestions

Page 28: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

In which of the following conditions In which of the following conditions is the BA consistent with is the BA consistent with chronological age (not delayed)?chronological age (not delayed)?

1.1. Acquired hypothyroidismAcquired hypothyroidism2.2. Constitutional delayConstitutional delay3.3. Familial short statureFamilial short stature4.4. Glucocorticoid excessGlucocorticoid excess5.5. Psychological dwarfismPsychological dwarfism

Page 29: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

Which of the following statements Which of the following statements regarding growth in children is true?regarding growth in children is true?

1.1. Crossing percentiles in the first 3 yrs after Crossing percentiles in the first 3 yrs after birth can be normalbirth can be normal

2.2. The best indicator of the appropiateness of a The best indicator of the appropiateness of a child’s groth is the comparison of the child’s child’s groth is the comparison of the child’s actual height with the target height.actual height with the target height.

3.3. The pubertal growth spurt occurs later un The pubertal growth spurt occurs later un puberty in girls than it does in boyspuberty in girls than it does in boys

4.4. The U/L body segment ratio is at its highest The U/L body segment ratio is at its highest during pubertyduring puberty

5.5. The wt-for ht ratio has little importance in the The wt-for ht ratio has little importance in the evaluation of a child who has short stature.evaluation of a child who has short stature.

Page 30: SHORT STATURE Karen Estrella H. PGY-1. Is she short?

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

1.1. Congenital hypothiroidismCongenital hypothiroidism

2.2. Crohn diseaseCrohn disease

3.3. GH deficiencyGH deficiency

4.4. SpondilodysplasiaSpondilodysplasia

5.5. TurnerTurner