evaluation of worrsisome growth

Question 1

1. Define “worrisome growth.”

Answer 1

Short stature: 
-height below -2 SD (3% is – 1.9 SD) for age 
and gender 
-height more than 2 SD below the 
midparental target height

Growth Velocity:
-Abnormally slow linear growth velocity or dropping across two major centile lines on the growth chart

Question 2

2. Recognize the difference in growth patterns between children with normal variants of short stature and pathological causes.

Answer 2

growth velocity drop off especially crossing two percentile lines is a problem

Question 3

3. List common causes of short stature and Recognize the growth patterns in it.

Answer 3

familial short stature
Constitutional growth delay
failure to thrive
nutritional growth retardation
SGA

Question 4

4. Explain how the diagnosis of growth hormone deficiency is made in children.

Answer 4

Evaluation:

 Bone age
 IGF-1 (IGFBP-3 in infant) – however low in underweight children regardless of GH status
 Stimulation testing (never draw random GH level) – clonidine, arginine, glucagon, L-dopa

signs-
 Decreased muscle build
 Increased subcutaneous fat, especially around trunk
 Face immature for age
 Prominent forehead, depressed midface
 In males, small phallus
 Other midline facial defects
 May have history of prolonged jaundice and/or hypoglycemia in newborn period

 Abnormal growth velocity with exclusion of other causes

Question 5

5. List some of the manifestations of a girl with Turner syndrome.

Answer 5

• Virtually all of girls with Turner syndrome exhibit short stature
• —-Final height is about 20 cm less than their target height if untreated

Skeletal Abnormalities: Short Stature, Increased carrying angle, Short neck, Micro or retrognathia

Cardiac abnormalities- bicuspid aortic valve, coarctation

Renal – horseshoe kidney

Ovarian insufficiency

Hypothyroidism/celiac disease  Otitis media

Hearing loss

Non-verbal learning disability

Question 6

6. Produce a strategy for the evaluation of a poorly growing child.

Answer 6

1. Bone age (left hand and wrist)
2. Screening labs: Turner Syndrome, Metabolic panel, CBC, UA, karyotype in girls, TSH & T4, (IGF-1)
3. Nutritional growth retardation: also ESR, TTG (tissue transglutaminase antibody) & IgA

Question 7

7. Identify the “FDA-approved” uses of growth hormone from 1985 to the present.

Answer 7

 1985 – Growth hormone deficiency
 1993 – Chronic renal insufficiency
 1996 – Adult growth hormone deficiency 
 1997 – Turner Syndrome
 2000 – Prader-Willi Syndrome
 2001 – Small for gestational age
 2003 – Idiopathic short stature
 2006 – SHOX deficiency
 2007 – Noonan syndrome

Question 8

8. List some of the ethical issues surrounding growth hormone treatment.

Answer 8

 How good is growth hormone testing?
 How long to treat?
 Who to treat?
 Who pays?
 Side effects- SCFE, psuedotumor cerebri

Question 9

Constitutional Growth delay

Answer 9

growth deceleration during first 2 years of life followed by normal growth paralleling lower percentile curve throughout prepubertal years

• Skeletal maturation is delayed
• Catch-up growth achieved by late puberty and delayed
  fusion of growth plates
• Generally end up along lower end of normal height range for families

Question 10

Familial Short Stature

Answer 10

• Children who have normal growth velocity and height that are within normal limits for parent’s heights
• Initially will have decrease in growth rate between 6 and 18 months of age
• Some families with short stature may have tubular bone alterations (eg, brachydactyly syndromes, SHOX haploinsufficiencies)

Question 11

Failure to Thrive

Answer 11

• Infants or young children with: Deceleration of weight gain to a point

Question 12

Nutritional Growth retardation

Answer 12

• Linear growth stunting from poor weight gain in children over 2 years of age
• May be secondary to systemic illnesses such as celiac disease, inflammatory bowel disease
• Stimulant medications
• Sometimes hard to distinguish from constitutional growth delay and constitutional thinness

Question 13

Children Born Small for Gestational Age

Answer 13

Defined as less than - 2SD for birth weight or length

Etiologies

• Maternal – infection, nutritional deficiencies, uterine abnormalities, smoking, alcohol, drugs
• Placental – Previa, abruption, infarcts, structural, multiple gestation
• Fetal – Chromosomal abnormalities, metabolic, infections, malformations

Most healthy infants born SGA achieve catch-up in height by age 2 years

-Most catch-up growth is achieved within 6 months of birth
~10-15% of children born SGA remain short as adults
- Final height may also be compromised by early puberty

Question 14

Hypothyroidism

Answer 14

 Can result in profound growth failure
Hormonal Causes of Worrisome Growth

 Generally, weight is spared

 Many clinical features that are seen in hypothyroid adults are lacking in children

Question 15

syndromic short stature

Answer 15

 Skeletal Dysplasias and other genetic syndromes
 Turner syndrome – Haploinsufficiency of SHOX genes
 Prader-Willi syndrome – GH deficient
 Noonan syndrome – abnormal GH post-receptor signaling