Ch 11 - Growth and Puberty

Question 1

Fraser guidelines for contraception (5)

Answer 1

The young person must undertand the advice The young person cannot be persuaded to inform their parents (or let the professional contact them) The young person is likely to continue having sex +/- contraception Unless the young person receives contraception their physical +/- mental health is likely to suffer The young person’s best interests require them to receive contraceptive advice or treatment with or without parental consent

Question 2

Sex and child protection: regarding children younger than 13 (1) & children between 13 & 16

Answer 2

So if child

Question 3

Sequence of puberty in females (3)

Answer 3

Breast development (8.5-12.5 yrs) > pubic hair + height spurt immidiately after > menarche 2.5 yrs after start of puberty

Question 4

Breast developement stages (5)

Answer 4

B1 = prepubertal B2 = bud B3 = juvenile smooth contour B4 = areola + papilla project over breast B5 = adult

Question 5

Pubic hair developement - applies to both males and females (5)

Answer 5

1 = none 2 = sparse, pigmented, long striaght 3 = dark, coarser, curlier 4 = filling out 5 = adult in quantity, spread to medial thigh in males

Question 6

Males pubertal sequence: (3)

Answer 6

Testicular enlargement - to > 4ml (9-13yrs) > Pubic hair (10-14yrs) > Height spurt - testicular vol > 12-15 ml

Question 7

Male genitals development (5)

Answer 7

1 >2 lengthening of penis > 3 increased growth in length/ circumfrence > 4 development of glans penis & darkening of scrotum > 5 adult

Question 8

Assessment of male (1), female (1), skeletal maturation (1)

Answer 8

Male - orchidometre Female - USS pelvis - uterine size & endometrial thickness Skeletal maturation - bone age (hand & wrist xray )

Question 9

Short stature definiton (1)

Answer 9

Height below 2nd centile/ 0.4th centile

Question 10

Causes of short stature (categories - 5)

Answer 10

Familial Constitutional - also dieting/exercise > legs long compared to back (can use androgens to induce puberty) Chronic illness - e.g. Coeliac’s Endocrine - Hypothyroidism, Cushing’s, GH def Psychosocial deprivation - neglect/abuse - catch up in nurturing environment IUGR + extreme prematurity - 1/3 remain short > GH treatment Genetic - Noonan’s, Turners, Down’s,

Question 11

3 causes of extreme short stature

Answer 11

Laron syndrome - GH resistance Primordial dwarfism idiopathic

Question 12

3 causes of disproportionate short stature

Answer 12

Achondroplasia (autosomal dom, mutation in fibroblast growth factor receptor) Scoliosis storage disorders

Question 13

Ixs for short stature (6)

Answer 13

Clinical - mid parental height first (avg +/- 7), height, weight, growth chats, HEIGHT VELOCITY - 2 measurements a year apart - below 25th centile persistently is abnormal bloods - FBC (anaemia), U & C (renal failure), Ca/PO/ALP (bone), TFTs, Coeliac screen (TTG), CRP (Crohn), GH provocation - GH def Bone age, MRI for intracranial tumour

Question 14

Caused of Tall stature (6)

Answer 14

Familial - most common Obesity - puberty is advanced - however because it occurs at earlier age final height centile is lower endocrine - gigantism (GH), hyperthyroid, preocious puberty, CAH Syndromes - Long legged tall stature: Marfan, hymocystinuria, Klinefelter (47 XXY). Proportionate tall stature at birth - maternal DM, primary hyperinsulinism, Beckwith syndrome, Sotos syndrome - large head, LDs,

Question 15

Management of tall stature

Answer 15

Rarely done But if it is pre/ early pubertal may gave oestrogens/androgens to fuse epiphysis - rarely conducted

Question 16

Microcephaly: definition, causes (4)

Answer 16

Head circumfrence below 2nd centile C - congenital infection (CMV, rubella, toxo), insult to developing brain (hypoxia, hypoglycaemia), familial,

Question 17

Macrocephaly: definion, causes (4)

Answer 17

Head circ above 98th centile C - Tall stature, familial, hydrocephalus, chronic subdural haematoma, tumour, NF, Sotos, Fragile X

Question 18

Craniosynostosis: definition

Answer 18

Premature fusion of skull sutures (shouldn’t fuse till late childhood) types: Sagittal craniosynostosis - long narrow, coronal (asymmetrical), Lambdoid - flattened

Question 19

Precocious puberty: define (1), significant cause in males (1), assessment of testicles (3)

Answer 19

Puberty

Question 20

Precocious puberty in females: causes (3), McCune-Albright syndrome triad (3)

Answer 20

C - idiotpathic, familial, organic cause - e.g. CAH, Cushing, androgen secreting tumours McCune-Albright - precocious puberty + cafe au lait spots + polyostotic fibrious dysplasia

Question 21

Pseudoprecocious puberty: define, causes (2)

Answer 21

Abnormal course of puberty e.g. growth spurt without breast budding, usually GONADOTROPHIN-INDEPENDENT - extracranial e.g. Cushing, CAH, adrenal tumours

Question 22

Premature breast developement (thelarche) define, Ix if any, management

Answer 22

Breast development in females between 6 months -2 yrs (absence of other pubertal features) No Ix required, self limiting and non-progressive so reassure

Question 23

Pre mature pubarche (adrenarche): define, cause, Ix (3), Management (1)

Answer 23

D - pubic hair development

Question 24

Delayed puberty: define, causes (4)

Answer 24

>12/13 in F/M Causes: familial, constitutional, Hypogonadotropic hypogonadism: systemic disease e.g. CF/ Crohn/ starvation. Hypothalamo-pituitary e.g. IC tumours, panhypopituatrism, isolated def Acquired hypothyroidism Hypergronadotropic hypogonadism: chromosomal e.g. Turner, Klinefelter. Steroid, acquired gonadal damage e.g. chemo/trauma

Question 25

Congenital adrenal hyperplasia

Answer 25

F - clitoral hypertrophy (in F), enlarged penis + scrotal pigmentation, tall stature, muscular build, adult body odour, pubic hair, acne Ix - Serum 17a hydroxyprogesterone + hyponatraemia + hyperkalaemia + metabolic acidosis + hypoglycaemia; urine pregnanetriol