2018 SHORT stature IPS - ipsummit.me SHORT stature IPS.pdf · Height more than 2 SD below mean for...

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Jamal Al Jubeh, MD, FAAP Consultant Pediatric Endocrinologist Chairman of Pediatrics SKMC, Abu Dhabi, UAE IPS 2018

Transcript of 2018 SHORT stature IPS - ipsummit.me SHORT stature IPS.pdf · Height more than 2 SD below mean for...

Page 1: 2018 SHORT stature IPS - ipsummit.me SHORT stature IPS.pdf · Height more than 2 SD below mean for the same age and sex compared to genetically relevant population (< 2.3 %) 8/26/18

Jamal Al Jubeh, MD, FAAPConsultant Pediatric Endocrinologist

Chairman of PediatricsSKMC, Abu Dhabi, UAEIP

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Objectives� Normal and abnormal growth patterns.� Short stature and growth failure.� Causes of growth failure.� Evaluation process.

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What is normal growth?

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25 cm

12 cm

8 cm

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Growth Velocity curves

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How to measure length and height?

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Measurement of length

www.jpeds.com Vol. 164, No. 5, Suppl. 1Rogol and Hayden8/26/18 15

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Kappy et al. ■ Pediatric Practice: Endocrinology 2010: 258/26/18 16

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Rogol and Hayden www.jpeds.com Vol. 164, No. 5, Suppl. 18/26/18 17

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Measurement accuracy

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Short Stature:Definition, causes and approach

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Short Stature� Height more than 2 SD below mean for the same age

and sex compared to genetically relevant population (< 2.3 %)

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Growth evaluation� Accurate measurement of child’s height and weight.� Accurate plotting on appropriate growth chart.� Assessment of longitudinal growth (growth velocity):

Accurate heights measured at 6 - 12 month interval

� Measuring parent’s height.

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Familial (genetic) Short stature� Parents are short� Child is growing within target height range.� Child’s bone age = Chronological age

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Midparental height (Boys)

� F: Father’s height (cm)M: Mother’s height (cm)

� Midparental height in cm for boys� = (F + M + 13) / 2

� = (F + M) / 2 + 6.5

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Midparental height (Girls)� F: Father’s height (cm)

M: Mother’s height (cm)

� Midparental height in cm for Girls� = (F + M - 13) / 2

� = (F + M) / 2 - 6.5

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Familial (genetic) Short

Stature

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Constitutional Delay of Growth and Puberty (CDGP)

� Normal growth velocity.� Delayed bone age.� Delayed puberty (Often runs in the family)� Normal adult (final) height

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Constitutional Growth Delay

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Constitutional Growth Delay

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What are the causes of short stature?

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Causes of Short Stature

Genes Nutrition

Systemic Disease

SkeletalDysplasia

Syndromes

SGA

Hormones

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Systemic Diseases

GI Renal

Hem / ONC

Metabolic Cardiac

Pulmonary

•Celiac Disease•Malabsorption•IBD

•CKD•Renal Tubular Acidosis

•Thalassemia•SCD•Malignancy

•Organic Acidemia•GSD

•Cystic Fibrosis•CLD

Congenital Heart disase

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Endocrine� Hypothyroidism� Isolated growth hormone deficiency� Hypopituitarism� GH insensitivity (Laron dwarfism)� Cushing syndrome� Poorly controlled type 1 diabetes mellitus -

Mauriac syndrome � Rickets� Idiopathic short stature

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Evaluation of Short Stature

Should be based on findings from History and Physical Exam

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Evaluation of Short stature� CBC, ESR� Electrolytes & Renal Panel, Ca, Phos.� Thyroid (TSH, FT4)� Celiac Screen:

� (Tissue transglutaminase antibodies).� Chromosomal analysis (girls).� IGF-1, IGF-BP3� Bone Age X ray.

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Endocrinol Metab Clin N Am 41 (2012) 265–282

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GH – IGF1 Axis

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GH – IGF1 axis

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Endocrinol Metab Clin N Am 36 (2007) 131–186

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When to suspect Growth Hormone Deficiency (GHD)?

� Low growth velocity.� Low IGF-1, IGF-BP3� Delayed bone age.� Other causes were excluded� ± Evidence of pituitary abnormality.

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How to confirm GHD?� Growth hormone stimulation testing:� Arginine,� Clonidine.� Glucagon� Levodopa� Insulin / hypoglycemia

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If GHD is confirmed� MRI of brain (hypothalamus / pituitary)� Start GH therapy.

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Reference� Etiologies and Early Diagnosis of Short Stature and Growth Failure in Children and

Adolescents. Alan D. Rogol, MD, PhD1, and Gregory F. Hayden, MD

� (J Pediatr 2014;164:S1-S14).

� Kappy et al. ■ Pediatric Practice: Endocrinology 2010: 25

� Endocrinol Metab Clin N Am 41 (2012) 265–282

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Thank YouIP

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