Transcript Basic Growth and Puberty

Common Disorders of
Growth and Puberty
Atanu Dutta
Queen Mary’s Hospital for
Children
Learning Objectives:
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Normal growth
Common Growth disorders
Puberty
Common problems with puberty
Height velocity charts
Growth charts: son of Count Phillip de
Montbeillard 1759-1777
Genetic
Environmental
Nutritional
Hormonal
The ICP model of growth
PUBERTY
CHILDHOOD
INFANCY
Height Velocity chart for Boys
and Girls in UK
Growth Assessment
Building
evidence
Growth Assessment
The Six blocks:
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History inc red book
Clinical examination
Measurement (Anthropometry)
Parental height
Bone age
Pubertal development
Common things first !!!
• Include a system check:
• Look out for
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Asthma
CF
Coeliac
IBD
Psychosocial
• Syndromes are rare
Growth Assessment
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History inc red book
Clinical examination
Measurement (Anthropometry)
Parental height
Bone age
Pubertal development
Anthropometry
• Use every opportunity
to measure height
• not done often!!
• Calibrated instrument
• Proper positioning
Growth Assessment
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History inc red book
Clinical examination
Measurement (Anthropometry)
Parental height
Bone age
Pubertal development
• Using parents height, we can calculate a
target range or 95 % tolerance limit for
their expected heights of their children
A) Fathers height
B) Mothers height
C) A + B
D) C divided by 2
E) D – 7 cm (Mid parental height)
F) E +/- 8.5 cm = Target centile range
• Using parents height, we can calculate a
target range or 95 % tolerance limit for
their expected heights of their children
A) Fathers height
B) Mothers height
C) A + B
D) C divided by 2
E) D + 7 cm (Mid parental height)
F) E +/- 10 cm = Target centile range
91st – 9th centile
Growth Assessment
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History inc red book
Clinical examination
Measurement (Anthropometry)
Parental height
Bone age
Pubertal development
Bone age
• Compare maturity of epiphyseal
centres with standard
• Growth better viewed in
relationship to their physical
maturity than chronological age
• Possible to predict early vs late
developers, final adult stature
• Advanced in girls
• Does not make a diagnosis
• Adds to the evidence
• Done where indicated
• If concerned, preferable to have BA done
• Info included in ref if possible
+ parental heights
+ growth charts
Growth Assessment
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History inc red book
Clinical examination
Measurement (Anthropometry)
Parental height
Bone age
Pubertal development
Change from childhood to adulthood
– Hormonal
– sexual maturation
– physical – body shape/image
– psychological
– Emotional
– experimentation
Puberty
Prader Orchidometer
• Also known as
“Prader balls”
• Endocrine rosary
Growth: Clinical problems
Short stature
• “ absolute height which is < - 2 SDS for
age, and or a linear growth velocity
consistently < - 1 SDS for age”
• Significant SS is ht < - 2.5 SDS and ht
velocity < - 1.0 SDS
Short stature – Normal appearance
Short for parents
Looks normal
Normal growth velocity
Thin
Systemic causes
Low growth velocity
Fat
Endocrine
Systemic causes of short stature
• CNS
– Developmental
• Cardiovascular
• Often delayed skeletal
maturation
– Heart disease
• Respiratory
– CF/ Asthma
• GI
– Coeliac / IBD
• Renal
– CRF/ RTA
• Psychosocial
– Emotional deprivation,
anorexia
• Potential to catch up
remains if underlying
cause treated
Psychosocial S S
• Psychosocial and
emotional deprivation
commonly recognised
• Short stature, skeletal
delay
• Older children may
experience delayed
puberty
• Endocrine dysfunction
may be seen
Endocrine causes
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Hypothyroidism
Isolated GH deficiency
Multiple pituitary deficiency
GH resistant states
Puedohypoparathyroidism
Cushings syndrome
SGA
Non endocrine causes
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Constitutional Growth delay
Turners syndrome
Skeletal dysplasias and bone disorders
Russell Silver Syndrome
Noonan's syndrome
Neurofibromatosis
Constitutional Growth delay
CDGP
• After 13 in girls and 14 in boys
• Growth rate and bone age usually 2 SD
below
• However, NORMAL growth rate for bone
age
• Often a family history of delayed puberty
Constitutional vs Familial
Short stature – Abnormal phenotype
Short for parents
Looks abnormal
Dysmorphic
Recognisable
syndrome
Systemic causes
Disproportionate
Skeletal dysplasia
Endocrine
Variation in Pubertal development
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Delayed Puberty
Precocious Puberty
Premature thelarche
Premature menarche
Premature adrenarche
Adolescent gynaecomastia
Delayed Puberty
• Constitutional
• Hypogonadotrophic hypogonadism
• Hypergonadotrophic hypogonadism
Hypogonadotrophic hypogonadism
» Isolated deficiency
» MPH deficiency
» PWS, LMB
» Hypothyroidism
» CNS tumours
» Anorexia, increased physical activity
Hyper gonadotrophic hypogonadism
»Klinefelters
»Anorchia/ Cryptorchidism
»Turners
»Other forms of primary
testicular/ovarian failure
»XX and XY Gonadal dysgenesis
Sexual Precocity
• Complete (True) Precocious
• Incomplete Precocious puberty
Complete Precocious Puberty
– Constitutional
– Idiopathic
– CNS disorder:
– Severe hypothyroidism
– Following androgen exposure, CAH
Incomplete Precocious puberty (1)
• MALES
»Gonadotrophin secreting tumours
»Excessive androgen production
»Premature maturation of Leydig
cells/germinal cells
Incomplete Precocious puberty (2)
• Females
» Ovarian cysts
» Oestrogen secreting neoplasms
• Secondary to exogenous gonadotrophin or
exposure to sex steroids
• Mc Cune Albright
Treatment of Sexual precocity
• Depends on
– GnRH dependent true or central precocious
puberty
» GNRH AGONISTS
– GnRH independent incomplete sexual precocity
» Medroxy progesterone acetate
» Testolactone
» Ketoconazole
» Cyprotone acetate
Variation in Pubertal development
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Delayed Puberty
Precocious Puberty
Premature thelarche
Premature menarche
Premature adrenarche
Adolescent gynaecomastia
Basic steps in growth
assessment
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Measure the height. Assess puberty
Parental height and calculate MPH
Compare Childs height with MPH
Re measure Childs height after period of
time
• Calculate present growth velocity
• If abnormally slow or rapid = Investigate
Case scenario (1)
• Paul is 8 yrs old
• Always short than his
peers
• Healthy but teased
• Parents ask
– Cant you give him
something to make
him grow better ?
• Mother = 166 cm
• Father = 169 cm
• Mothers parents
• 150 and 160 cm
• Father’s parent
• 155 and 160 cm
• Physical exam: N
• Bone age = 7.5 years
• Testis = 2 mls
• Diagnosis?
Case scenario (2)
• Steven is 14.5 yrs
• Hardly grown at all
during the last year
• Almost all are taller
than him currently
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Father = 173
Mother = 171
Father had late puberty
Physical exam = N
No pubertal development
BA = 10 yrs
Bloods = N
LHRH shows not yet in
puberty
• Diagnosis?
• Any treatment
Thank You