Upload
vungoc
View
234
Download
1
Embed Size (px)
Citation preview
Jamal Al Jubeh, MD, FAAPConsultant Pediatric Endocrinologist
Chairman of PediatricsSKMC, Abu Dhabi, UAEIP
S 201
8
Objectives� Normal and abnormal growth patterns.� Short stature and growth failure.� Causes of growth failure.� Evaluation process.
8/26/18 2
IPS 2
018
What is normal growth?
8/26/18 3
IPS 2
018
8/26/18 6
IPS 2
018
8/26/18 7
IPS 2
018
25 cm
12 cm
8 cm
8/26/18 8
IPS 2
018
8/26/18 9
IPS 2
018
8/26/18 10
IPS 2
018
Growth Velocity curves
8/26/18 11
IPS 2
018
8/26/18 12
IPS 2
018
8/26/18 13
IPS 2
018
How to measure length and height?
8/26/18 14
IPS 2
018
Measurement of length
www.jpeds.com Vol. 164, No. 5, Suppl. 1Rogol and Hayden8/26/18 15
IPS 2
018
Kappy et al. ■ Pediatric Practice: Endocrinology 2010: 258/26/18 16
IPS 2
018
Rogol and Hayden www.jpeds.com Vol. 164, No. 5, Suppl. 18/26/18 17
IPS 2
018
Measurement accuracy
8/26/18 18
IPS 2
018
8/26/18 19
IPS 2
018
8/26/18 20
IPS 2
018
Short Stature:Definition, causes and approach
8/26/18 21
IPS 2
018
Short Stature� Height more than 2 SD below mean for the same age
and sex compared to genetically relevant population (< 2.3 %)
8/26/18 22
IPS 2
018
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.
8/26/18 23
IPS 2
018
Familial (genetic) Short stature� Parents are short� Child is growing within target height range.� Child’s bone age = Chronological age
8/26/18 24
IPS 2
018
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
8/26/18 25
IPS 2
018
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
8/26/18 26
IPS 2
018
Familial (genetic) Short
Stature
8/26/18 27
IPS 2
018
Constitutional Delay of Growth and Puberty (CDGP)
� Normal growth velocity.� Delayed bone age.� Delayed puberty (Often runs in the family)� Normal adult (final) height
8/26/18 28
IPS 2
018
Constitutional Growth Delay
8/26/18 29
IPS 2
018
Constitutional Growth Delay
8/26/18 30
IPS 2
018
What are the causes of short stature?
8/26/18 31
IPS 2
018
Causes of Short Stature
Genes Nutrition
Systemic Disease
SkeletalDysplasia
Syndromes
SGA
Hormones
8/26/18 32
IPS 2
018
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
8/26/18 33
IPS 2
018
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
8/26/18 34
IPS 2
018
Evaluation of Short Stature
Should be based on findings from History and Physical Exam
8/26/18 35
IPS 2
018
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.
8/26/18 36
IPS 2
018
8/26/18 37
Endocrinol Metab Clin N Am 41 (2012) 265–282
IPS 2
018
GH – IGF1 Axis
8/26/18 38
IPS 2
018
GH – IGF1 axis
8/26/18 39
Endocrinol Metab Clin N Am 36 (2007) 131–186
IPS 2
018
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.
8/26/18 41
IPS 2
018
How to confirm GHD?� Growth hormone stimulation testing:� Arginine,� Clonidine.� Glucagon� Levodopa� Insulin / hypoglycemia
8/26/18 42
IPS 2
018
8/26/18 43
IPS 2
018
If GHD is confirmed� MRI of brain (hypothalamus / pituitary)� Start GH therapy.
8/26/18 44
IPS 2
018
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
8/26/18 45
IPS 2
018
8/26/18 46
Thank YouIP
S 201
8