Common Disorders of Growth and Puberty

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Common Disorders of Growth and Puberty

• Atanu Dutta
• Queen Marys Hospital for Children

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Learning Objectives

• Normal growth
• Common Growth disorders
• Puberty
• Common problems with puberty

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Height velocity charts
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Growth charts son of Count Phillip de
Montbeillard 1759-1777
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The ICP model of growth
PUBERTY
CHILDHOOD
INFANCY
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Height Velocity chart for Boys and Girls in UK
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Growth Assessment
Building evidence
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Growth AssessmentThe Six blocks

• History inc red book
• Clinical examination
• Measurement (Anthropometry)
• Parental height
• Bone age
• Pubertal development

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Common things first !!!

• Include a system check
• Look out for
• Asthma
• CF
• Coeliac
• IBD
• Psychosocial
• Syndromes are rare

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Growth Assessment

• History inc red book
• Clinical examination
• Measurement (Anthropometry)
• Parental height
• Bone age
• Pubertal development

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Anthropometry

• Use every opportunity to measure height
• not done often!!
• Calibrated instrument
• Proper positioning

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Growth Assessment

• History inc red book
• Clinical examination
• Measurement (Anthropometry)
• Parental height
• Bone age
• Pubertal development

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• 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

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• 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

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Growth Assessment

• History inc red book
• Clinical examination
• Measurement (Anthropometry)
• Parental height
• Bone age
• Pubertal development

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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

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• Done where indicated
• If concerned, preferable to have BA done
• Info included in ref if possible
• parental heights
• growth charts

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Growth Assessment

• History inc red book
• Clinical examination
• Measurement (Anthropometry)
• Parental height
• Bone age
• Pubertal development

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• Change from childhood to adulthood
• Hormonal
• sexual maturation
• physical body shape/image
• psychological
• Emotional
• experimentation

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Puberty
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Prader Orchidometer

• Also known as
• Prader balls
• Endocrine rosary

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• Growth Clinical problems

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Short stature

• absolute height which is lt - 2 SDS for age, and
  or a linear growth velocity consistently lt - 1
  SDS for age
• Significant SS is ht lt - 2.5 SDS and ht velocity
  lt - 1.0 SDS

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Short stature Normal appearance
Short for parents
Looks normal
Normal growth velocity
Low growth velocity
Fat
Thin
Endocrine
Systemic causes
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Systemic causes of short stature

• Often delayed skeletal maturation
• Potential to catch up remains if underlying cause
  treated

• CNS
• Developmental
• Cardiovascular
• Heart disease
• Respiratory
• CF/ Asthma
• GI
• Coeliac / IBD
• Renal
• CRF/ RTA
• Psychosocial
• Emotional deprivation, anorexia

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Psychosocial S S

• Psychosocial and emotional deprivation commonly
  recognised
• Short stature, skeletal delay
• Older children may experience delayed puberty
• Endocrine dysfunction may be seen

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Endocrine causes

• Hypothyroidism
• Isolated GH deficiency
• Multiple pituitary deficiency
• GH resistant states
• Puedohypoparathyroidism
• Cushings syndrome
• SGA

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Non endocrine causes

• Constitutional Growth delay
• Turners syndrome
• Skeletal dysplasias and bone disorders
• Russell Silver Syndrome
• Noonan's syndrome
• Neurofibromatosis

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Constitutional Growth delay
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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

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Constitutional vs Familial
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Short stature Abnormal phenotype
Short for parents
Looks abnormal
Dysmorphic
Disproportionate
Recognisable syndrome
Skeletal dysplasia
Endocrine
Systemic causes
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Variation in Pubertal development

• Delayed Puberty
• Precocious Puberty
• Premature thelarche
• Premature menarche
• Premature adrenarche
• Adolescent gynaecomastia

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Delayed Puberty

• Constitutional
• Hypogonadotrophic hypogonadism
• Hypergonadotrophic hypogonadism

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Hypogonadotrophic hypogonadism

• Isolated deficiency
• MPH deficiency
• PWS, LMB
• Hypothyroidism
• CNS tumours
• Anorexia, increased physical activity

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Hyper gonadotrophic hypogonadism

• Klinefelters
• Anorchia/ Cryptorchidism
• Turners
• Other forms of primary testicular/ovarian failure
• XX and XY Gonadal dysgenesis

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Sexual Precocity

• Complete (True) Precocious
• Incomplete Precocious puberty

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Complete Precocious Puberty

• Constitutional
• Idiopathic
• CNS disorder
• Severe hypothyroidism
• Following androgen exposure, CAH

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Incomplete Precocious puberty (1)

• MALES
• Gonadotrophin secreting tumours
• Excessive androgen production
• Premature maturation of Leydig cells/germinal
  cells

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Incomplete Precocious puberty (2)

• Females
• Ovarian cysts
• Oestrogen secreting neoplasms
• Secondary to exogenous gonadotrophin or exposure
  to sex steroids
• Mc Cune Albright

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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

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Variation in Pubertal development

• Delayed Puberty
• Precocious Puberty
• Premature thelarche
• Premature menarche
• Premature adrenarche
• Adolescent gynaecomastia

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Basic steps in growth assessment

• 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

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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 ?

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• Mother 166 cm
• Father 169 cm
• Mothers parents
• 150 and 160 cm
• Fathers parent
• 155 and 160 cm
• Physical exam N
• Bone age 7.5 years
• Testis 2 mls

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• Diagnosis?

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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

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• Diagnosis?
• Any treatment

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Thank You