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Common Disorders of Growth and Puberty
Atanu Dutta Queen Mary’s 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 Assessment The 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 < - 2 SDS for age, and or a linear growth velocity consistently < - 1 SDS for age” Significant SS is ht < SDS and ht velocity < 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
CNS Developmental Cardiovascular Heart disease Respiratory CF/ Asthma GI Coeliac / IBD Renal CRF/ RTA Psychosocial Emotional deprivation, anorexia Often delayed skeletal maturation Potential to catch up remains if underlying cause treated
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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 Father’s parent
150 and 160 cm Father’s 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
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