short stature approach

1. short stature
Dr. Azad A Haleem
MBChB, DCH, FIBMS Pediatrics
Lecturer
Pediatric department
Medical college/ Duhok university
Heevi Pediatrics Teaching Hospital
azad82d@gmail.com

2. • Define short stature.
• our guideline
• another guideline
• Bone age by RUS
• some point on provocation test
• Outlines about Managements.
• I may use Kurdish language some times for
clarification and discussion ???

3.  HSDS < -2SD
 Ht velocity < 3rd percentile .
Males
Age (y )
30
34
38
42
46
50
54
58
62
66
70
74
78
Height(in)
Height(cm)
2 4 6 8 10 12 14 16 18 20
70
80
90
100
110
120
130
140
150
160
170
180
190
200
0
+2
+1
-1
-2
-2.0 SD (2.3 percentile)
Generally
accepted
definition of
normal range

4. Harpenden
$3000
Leicester
$60

5. • The normal range for height is generally considered to be
between about the 3rd and the 97th percentiles
• Normal height range differs from one country to another.
• As we don't have Iraqi growth charts so we depend on CDC
growth charts.

7. Guidelines for referral
 UK guidelines, depend on single
measurement on school entrance,
at 5years of age , no data on
sensitivity and specificity

8. Important definitions
• Chronological age – Actual age of the child.
• Height age – it’s the age at which the height of the
child is at 50th centile.
• Bone age - is an indicator of skeletal maturation.
• Target MPH: F+M/2 +6.5 for boys and -6.5 for girls.
• Then plot the result on Growth Chart at Age 20 to
form Family chart For boys ±10 & Girls ± 8.5

9. • Example: 10 Y
male,HT=115
• HT< 3RD
• HA= 6 Y

10. MPH
• Example: 3 Y – girl
• Ht=85 cm
• Father Ht= 165 cm
• Mother Ht= 155 cm
• MPH= 160-6.5=
• 153.5
• Plot on age 20
• 153.5 ±8.5
• 145 – 162
3rd
MPH= 153.5±10

11. Approach to short stature
(now in Heevi Hospital)
1- Height (CDC) ↓ 3rd centile
2- Family Chart: HT ↓ MPH
3- Bone age evaluation by greulich and pyle method.
4- BA < HA<CA (Pathological Short Stature)
5- Investigations: level 1 general then Level 2 TFT
6- Provocative tests (GH stimulation tests)&IGF
Familial Short Stature Constitutional Short Stature Pathological Short Stature
↓3rd Centile ↓3rd Centile ↓3rd Centile
↔ MPH ↓ MPH ↓ MPH
BA = CA Normal BA = Height Age < CA BA < HA<CA

12. approach to short stature (new)
1- Height SDS =(height observed – mean height reference)
SD reference
2- MPH (SDS )= Target mph – MHR (From 19 years refrences)
SDR
3- Calculate the difference between the height SDS of the
child and the midparental height SDS
4- Bone age evaluation by TW2(RUS) method.
5- Investigations: level 1 general then Level 2 TFT
6- Provocative tests (GH stimulation tests)&IGF

14. • Height SDS = (height observed – mean height reference)
SD reference
Example calculation:
A 9-year-old boy is 116 cm tall
• Mean height reference for his age = 134.7 cm
• Standard deviation reference = 6.2 cm
Height SDS = (116–134.7) = –3 SDS
6.2

16.  Target MPH: F+M/2 +6.5 for boys and -6.5 for girls.
 MPH (SDS )= Target MPH -MHR
SDR
Example calculation:
Target midparental height for a 9-year-old boy is 170 cm
• (MHR)Mean height reference for maximum age (adult height) for boys = 176
cm
• (SDR)Standard deviation reference for maximum age (adult height) for boys =
6 cm
Midparental height SDS = (170–176) = –1 SDS
6

17. Calculate the difference between the height SDS of the child
and the midparental height SDS
Example of calculation:
Height SDS of the child: –3 SDS
Midparental height SDS: –1 SDS
Difference: –2 SDS
• As the boy’s height SDS is more than 1.5 SDS below the
midparental height SDS, he is short for his genetic
potential

19. • Bone age estimated from an x-ray of the left wrist and hand
should be undertaken as part of the routine evaluation of
children with growth failure over 1 year of age.
• It is important investigation in evaluation of patient with short
stature and diagnosis of GHD in which it is usually delayed.

20. 1) the Greulich-Pyle Atlas method (GP method) in which
a left-hand wrist radiograph is compared by means of a
sequence of radiographs grouped in the atlas according to
age and gender.
2) The Tanner-Whitehouse 2 Bones method (TW2
method).
In which a maturity score is awarded to individual
epiphyses, the sum of which is then converted to a bone
age which is plotted on the growth chart as height for bone
age.

21. G & P Method
Patient’s film is
compared with the
standard of the
same sex and
nearest age
It is next
compared with
adjacent standard,
both older and
younger to get the
closest match
The Greulich-Pyle Atlas method

22. In the UK,
most centers
use the radius,
ulna and small
bone method
devised by
Tanner and
Whitehouse 2
{RUS(TW2)}.
RUS
12
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5
6
6
8
8
7
9
9

23. 1-Radus
2-Ulna
3- 1MCB
4-3&5MCB
5-P1P
6-P3&5P
7-M3&5P
8-D1P
9-D3&5P
12
344
5
6
6
8
8
7
9
9
each
scored from
B to I ( 8)
Each have
correspondi
ng Number.
Collect all
Then get
correct age
in chart.

25.  Bone age assessment provides precious information, but if not properly
used, can be misleading and it should always be considered ancillary to
the clinical and auxological examination.
- in the diagnosis of FSS and CGD;
- for interpreting hormone levels in pubertal age;
- for diagnosis of precocious puberty or hyperandrogenism;
- for deciding whether to treat or not the above mentioned conditions;
- for predicting adult height in normal children.
- in evaluating any child with growth and/or puberty disorders;
- in deciding the time to start replacement therapy in hypogonadism.
- in monitoring children on growth hormone therapy.

26. - in evaluating children with disorders of bone mineralization;
(osteochondrodysplasias).
- in predicting adult height in pathological conditions; such as in
pp & SGA.
- if considered an absolute diagnostic marker; such as in FSS and
CGD.
- if, during the follow-up of a patient or in comparing groups of
patients, different readers are involved or different methods
are employed.

28. • GH secretion occurs in discrete irregular pulses
• Between the pulses, circulating GH falls to levels that are
undetectable with current assays
• Greatest GH levels at night, generally correlating with the onset
of sleep
• GH secretion is influenced by other physiological stimuli such as
nutrition and exercise

29. • Random sampling of serum GH is insufficient to diagnose
GH deficiency; GH stimulation tests are required
• A limited number of provocative agents should be
used after an overnight fast in a well standardized
protocol.
• Insulin tolerance test (ITT)
• Glucagon test (100 microgrammes/kg BW IM(max.1 mg)
• L-dopa test
• Arginine test(0.5g/kg BW , slow IV infusion (max,30g) ,
• Clonidine test (0.1- 0.15 mg/kg BW orally).

30.  A majority of normal pre pubertal children fail to achieve
GH values >10 ng/mL with 2 pharmacological tests, so
• Sex Steroid Priming Performed in pre-pubertal male and
female patients with a bone age greater than 10 years.
• Stilboestrol 1 mg bd for 48 hours prior to test in both sexes
• or ethinyl-oestradiol 10 mg od for 3 days before Glucagon
test in girls,
• or testosterone 100 mg IM start 72 hrs before test in boys.
 The drugs are usually prescribed in advance, when the child
is assessed in out-patient.

31. •In healthy volunteers peak GH levels are 10 ng/ml (20 mU/ml).
•If peak GH level of 10 ng/ml (20 mU/ml) is detected , it exclude
classical GHD
•In a classical GHD case a GH peak is not detected or GH peak is
less than 3 ng/ml (6 mU/ml) in all these tests.
•In partial GHD cases GH peak of 8-10 ng/ml (16-20 mU/ml)
may be seen.

32. • A strong clinical suspicion is important in establishing the
diagnosis because laboratory measures of GH sufficiency lack
specificity.
, the demonstration of poor
linear growth, delayed skeletal age, and peak levels of GH
(<10 ng/mL) in each of 2 provocative tests, are compatible
with GH deficiency.
• Acquired GH deficiency can occur at any age; when it is of
acute onset, height may be within the normal range with
normal bone age but growth velocity decline, so
• , a high clinical suspicion of GH
deficiency and low peak levels of GH (<10 ng/mL) in each of 2
provocative tests are compatible with GH deficiency.

33. In addition to establishing the diagnosis of GH deficiency, it
is necessary to examine other pituitary functions, which
includes
• If the thyroid functions tests are abnormal ,treat 1st & then
do the provocative test for growth hormone.
• If cortisol and ACTH were low give hydrocortisone tab as
replacement therapy before GH replacement.

34. • IGFBP-3 and IGF-1 serum levels represent a
stable and integrated measurement of GH
production and tissue effects
• The combination of IGF-1 and IGFBP-3
measurements appears superior to
determining either analyte alone in the
diagnosis of growth hormone (GH) related
disorders

35. • If IGF-1 and IGBP-3 level are normal then it
shows that GH level is also normal (no need for
GH testing)
• If IGF-1 and IGBP-3 level are low then it may be
due to GH def or GH resistance-----
• If GH also low then GH def,
• if GH normal or high then GH resistance
( Primary IGF-1 def), (Laron syndrome).

37. • Growth hormone deficiency .(The prevalence
between 1 in 3500 and 1 in 4000 children)
• Turner syndrome. PWS
• Chronic renal insufficiency (pretransplantation)
• SGA: Growth failure at 4 years or older in those
born small for gestational age. (Approximately
10% of SGA do not reach the normal height
range)

38. The dosage of somatropin should be tailored to the needs of
each individual child& varies according to the condition being
treated:
• 23–39 microgram/kg daily or 0.7–1.0 mg/m² daily for
growth hormone deficiency.
• 45–50 microgram/kg daily or 1.4 mg/m² daily for Turner
syndrome and CRI.
• 35 microgram/kg daily or 1.0 mg/m² daily for growth
disturbance in children born small for gestational age.
• Somatropin is self-administered or given to the child
by an adult, at home, usually as a subcutaneous
injection, 6–7 times a week.The maximum
recommended daily dose should not be exceeded.

39. • Gains in final height for children treated with
somatropin compared with untreated children
ranged from approximately 3 to 11 cm
• for growth hormone deficiency 8–11 cm.
• Turner syndrome 5 cm.
• Chronic renal insufficiency 3–9 cm.
• Long-term continuous GH treatment in short
children born SGA without signs of persistent
catch-up growth leads to a normalization of
adult height.

40. Treatment with somatropin should be discontinued if any of
the following apply:
• final height is attained.
• Decision by patient that he/she is tall enough.
• growth velocity increases less than 50% from baseline in
the first year of treatment
• final height is approached and growth velocity is less than
2 cm total growth in 1 year.
• BA >14years in girls & 16years in boys.
• there are insurmountable problems with adherence

41.  required as there is risk of :primary hypo
thyroidism/adrenal insuffiency so periodic
follow up needed.
Pseudotumour cerebri, hyperglycemia, acute
pancreatitis, liver abnormalities,
gynaecomastia,

42. • In Turner syndrome and SGA children it is recommended to
measure the IGF-I level before start of treatment and twice
a year thereafter.
• If on repeated measurements IGF-I levels exceed +2 SD
compared to references for age and pubertal status, the
dose should be reduced to achieve an IGF-I level within the
normal range.

43. • Scoliosis may progress in any child during rapid
growth. Signs of scoliosis should be monitored
during treatment. However, somatropin
treatment has not been shown to increase the
incidence or severity of scoliosis.
• Because somatropin may reduce insulin
sensitivity, patients should be monitored for
evidence of glucose intolerance. Patients with
glucose intolerance should be monitored
closely during somatropin therapy.

44. • 1.NICE technology appraisals [TA188] Published
date: May 2010
• 2.Van Pareren Y et al. Adult height after long-
term, continuous growth hormone (GH)
treatment in short children born small for
gestational age: results of a randomized, double-
blind, dose-response GH trial. J Clin
Endocrinol Metab. 2003Aug;88(8):3584-90.

45. Thank You !!