Growth and Development Flashcards

Question

Why may bone age be delayed?

Answer 1

Constitutional delay of growth Growth hormone deficiency Hypothyroidism Malnutrition/chronic illness

Answer 2

Tall stature Premature adrenarche Overweight Early puberty Congenital Adrenal Hyperplasia Overgrowth syndromes

Answer 3

- for confirming the diagnosis of the normal variants of growth, such as familial short stature (FSS) and constitutional growth delay growth (CGD), the former associated with normal skeletal maturation and the latter with delayed bone age - for interpreting of hormone blood levels in children at the pubertal age - for diagnosing precocious puberty (differential diagnosis with premature telarche and premature pubarche) or conditions of hyperandrogenism such as congenital adrenal hyperplasia (CAH) characterized by advanced bone age - for deciding whether to treat or not children with the above mentioned conditions (precocious puberty and CAH) - for predicting adult height in normal children

Answer 4

- in evaluating any child with growth retardation and advanced or delayed puberty - in deciding the right time to start a child with hypogonadism on replacement treatment - in monitoring children in replacement therapy with growth hormone

Answer 5

- in evaluating children with disorders of bone mineralization, such as osteochondrodysplasias - in predicting adult height in children with precocious puberty or born small for gestational age: adult height prediction should actually be performed in normal children only - whatever the aim, if different readers, not accurately trained to follow the same procedure, are involved in bone age assessment

Answer 6

- accessory unusual secondary ossification centers may appear in otherwise unusual ends of a bone when growth is overstimulated in rapid catch-up growth - important changes in the rate of longitudinal bone growth are registered as growth arrest lines

Answer 7

Left image showing the patient has growth delay and they have lots of growth to come Right is showing an advanced bone age, child is 6 with a bone age of around 11 Bone age refers to puberty timing Bone age gives a likely time of puberty

Answer 8

tanner method Standardised method of assessing puberty

Answer 9

B - 1 to 5 (breast development) G - 1 to 5 (genital development) PH - 1 to 5 (pubic hair) AH - 1 to 3 (axillary hair) T (testicular volume in boys) - 2ml to 20 ml

Answer 10

1 – pre pubertal 2 – beginning of puberty 3-4 different stages of puberty 5 – adult

Answer 11

Stage 1: Prepubertal Stage 2: Enlargement of scrotum and testes; scrotum skin reddens and changes in texture Stage 3: Enlargement of penis (length at first); further growth of testes Stage 4: Increased size of penis with growth in breadth and development of glans; testes and scrotum larger, scrotum skin darker Stage 5: Adult genitalia

Answer 12

Stage 1: Prepubertal (can see velus hair similar to abdominal wall) Stage 2: Sparse growth of long, slightly pigmented hair, straight or curled, at base of penis or along labia Stage 3: Darker, coarser and more curled hair, spreading sparsely over junction of pubes Stage 4: Hair adult in type, but covering smaller area than in adult; no spread to medial surface of thighs Stage 5: Adult in type and quantity, with horizontal distribution ("feminine")

Answer 13

Stage 1: Prepubertal Stage 2: Breast bud stage with elevation of breast and papilla; enlargement of areola Stage 3: Further enlargement of breast and areola; no separation of their contour Stage 4: Areola and papilla form a secondary mound above level of breast Stage 5: Mature stage: projection of papilla only, related to recession of areola

Answer 14

girl - brest development 3 out of 5 and pubic heair development 3 out of 5 so mid puberty boy - genital development 2 out of 5 and pubic hair development 2 out of 5 so at the start of puberty

Answer 15

Prader Orchidometer

Answer 16

It consists of a string of twelve numbered wooden or plastic beads of increasing size from about 1 to 25 ml. The beads are compared with the testicles of the patient, and the volume is read off the bead which matches most closely in size. Prepubertal sizes are 1–3 ml (black), pubertal sizes are considered 4 ml and up and adult sizes are 12–25 ml

Answer 17

Small testes can indicate either primary or secondary hypogonadism Testicular size can help distinguish between different types of precocious puberty Since testicular growth is typically the first physical sign of true puberty, one of the most common uses is as confirmation that puberty is beginning in a boy with delay Large testes (macroorchidism) can be a clue to one of the most common causes of inherited generalised mental disability, fragile X syndrome

Answer 18

* Birth weight and gestation * PMH * Family history/social history/schooling * Systematic enquiry - find more subtle symptoms like coeliac has more discrete symptoms than children may not go to the doctor for * Dysmorphic features * Systemic examination

Answer 19

* Height/length/weight * Growth Charts and plotting * MPH (mid-parental height) and Target centiles * Growth velocity * Bone age * Pubertal assessment

Answer 20

Precise definition difficult: – Wide range within healthy population – Different ethnic subgroups – Inequality in basic health and nutrition – Normality may relate to individuals or populations (genetic influence) Normal growth is the progression of changes in height, weight, and head circumference that are compatible with established standards for a given population. The progression of growth is interpreted within the context of the genetic potential for a particular child. Normal growth is a reflection of overall health and nutritional status Understanding the normal patterns of growth enables the early detection of pathologic deviations (eg, poor weight gain due to a metabolic disorder, short stature due to inflammatory bowel disease) and can prevent the unnecessary evaluation of children with acceptable normal variations in growth

Answer 21

**Age** **Sex** - boys are slightly taller than girls before puberty **Race** - e.g. Scandinavians are tall, Oriental races are short **Nutrition** - particularly important prenatally and in infancy. Contributes to height differences between races **Birth weight** - little effect on childhood height unless small for gestational age **Parental heights** - the underlying genetic component, a particularly strong influence **Puberty** - early developers are taller for age than late developers **Skeletal maturity (bone age)** **General health** **Chronic disease** - growth is adversely affected by chronic illness, e.g. Crohn’s disease, chronic renal failure **Specific growth disorders** - e.g. growth hormone deficiency, hypothyroidism **Socio-economic status** - children from affluent areas, are, on average, taller than children from poor areas **Emotional well-being** - can cause profound growth retardation

Answer 22

Infantile Childhood Pubertal

Answer 23

Rapid period of growth from 0 - 2 years Continuation of the rapid but decelerating intra-uterine growth phase Depends on factors such as nutrition and the insulin-like growth factors (IGF1 & IGF2) Largely independent of growth hormone In general, birth size reflects intra-uterine environment, e.g. nutrition Birth size, therefore, has little correlation with final height From birth to age 2, infant “tracks” on to his/her genetic centile, related to parental heights By age 2, around half adult height has been attained

Answer 24

Long phase of growth from 2 ~ 12 years (onset of pubertal growth) Slower, slightly decelerating curve More dependent on growth hormone & thyroxine Healthy child will stay on childhood (genetic) centile until puberty

Answer 25

From around age 12 to final height Dependent on the sex steroid that cause an increase in growth hormone secretion: testosterone in boys and oestrogen in girls Resulting growth acceleration is limited by fusion of the epiphyses (due to oestrogen in both sexes) Variable growth phase in terms of : age of onset, duration and intensity Correlation between childhood centile & final adult centile is ~60% (i.e. individual may end up on higher or lower centile) 95% of normal individuals will attain final heights within 2 standard deviations of mid-parental height (+/- 8.5 cm)

Answer 26

grow fast at start of puberty peak height velocity at 12 yr (B2-3) slow down in later stages of puberty when breast development is mature (B4 to 5) when menarche occurs (13-13.5 yr) girls are close to final height

Answer 27

grow slowly at start of puberty (G2) – still in childhood growth phase accelerate in mid-puberty (coincides with growth of penis, G3) peak height velocity at 14 yr (G4) further growth after pubertal development is complete (G5)

Answer 28

Breast budding (Tanner Stage B 2) in a girl Testicular enlargement (Tanner Stage G2-T 3- 4 ml) in boy these are the earliest objective signs of puberty and when present puberty will usually progress onwards

Answer 29

Chart showing different growth patterns in boys (solid line) and girls (dotted line) Men end up taller than women by around 12.5 cm (5”) because of 3 factors: - Pubertal growth spurt starts 2 years later than in girls (14 yr cf 12 yr) - Pubertal growth spurt is more intense in boys - Boys are slightly bigger than girls during childhood

Answer 30

Obese children will be taller Obese children go into puberty earlier More or less same height at the end Obese = taller and early puberty Obese child that isn’t tall then think about if there is an underlying pathology Don’t worry about obese tall children

Answer 31

* Extreme short or tall stature (off centiles) * Height below target height * Abnormal height velocity (crossing centiles) * History of chronic disease * Obvious dysmorphic syndrome * Early/late puberty

Answer 32

* Familial * Constitutional * SGA/IUGR (Intrauterine growth restriction (IUGR) refers to a condition in which an unborn baby is smaller than it should be because it is not growing at a normal rate inside the womb)

Answer 33

Familial Short Stature M = mother F = father Ends up short but is normal as parents are short Bone age not delayed

Answer 34

When growth and puberty are constitutionally delayed, puberty spontaneously begins at a time that is more than two standard deviations later than the mean time of breast development in girls or increase in testicular volume in boys Child short, normal but looks younger than chronological age Parent(s) not short, but may have been so in childhood Bone age delayed - know they will be a late bloomer Late puberty and catch-up growth Final height usually in lower half of target range

Answer 35

* Undernutrition (malnutrition is most ocmmon) * Chronic illness (JCA, IBD, Coeliac) * Iatrogenic (steroids) * Psychological and social * Hormonal (GHD, hypothyroidism) * Syndromes (Turner, P-W)

Answer 36

growth hormone

Answer 37

Mild GH deficiency might show no features before school age! Neonatal: hypoglycaemia, prolonged jaundice, micropenis Other anterior pituitary deficiencies Excess subcutaneous fat Mid-face hypoplasia Features of septo-optic dysplasia (rare congenital malformation syndrome featuring underdevelopment of the optic nerve, pituitary gland dysfunction, and absence of the septum pellucidum) Delayed skeletal maturation Treatment very effective - Give them growth hormone May be otherwise healthy

Answer 38

Growth hormone deficiency Thyroid deficiency

Answer 39

turner syndrome Prader-Willi syndrome Noonan’s syndrome Achondroplasia

Answer 40

Main features: Short Stature Gonadal dysgenesis Other features: Cardiac defect (coarctation of aorta) Renal anomaly Hypertension Hearing problems

Answer 41

Infantile hypotonia(decreased muscle tone)/feeding problems Hyperphagia(excessive eating)/obesity in childhood Short stature Developmental delay Hypogonadism Deletion of 15q11-q13 chromosomal region

Answer 42

turner syndrome, Prader-Willi syndrome, Noonan’s syndrome - growth hormone treatment will help them develop Achondroplasia - no treatment at the moment

Answer 43

* B2 in a girl * T 3- 4 ml in boy these are the earliest objective signs of puberty and when present puberty will usually progress onwards

Answer 44

early \< 9 years (rare) delayed \>14 (common, especially CDGP)

Answer 45

early \<8 years delayed \>13 (rare) (this is referring to the begining of puberty, periods happen at the end of puberty so not worried about them)

Answer 46

* Boys mainly * Family history in dad or brothers (difficult to obtain!) * Bone age delay * Need to exclude organic disease

Answer 47

Gonadal dysgenesis (Turner 45X (make puberty late), Klinefelter 47XXY(gonads don’t develop properly and don’t progress in puberty)) Chronic disease (Crohn’s, asthma) Impaired HPG axis (septo-optic dysplasia, craniopharyngioma, Kallman’s syndrome) Peripheral (cryptorchidism, testicular irradiation)

Answer 48

Early sexual development: \< 8 yrs ♀, \< 9yrs ♂ commoner in females

Answer 49

In girls with premature thelarche, breast tissue develops early—sometimes in the first two years of life, sometimes later, and occasionally even in the neonatal period—and then persists until the true onset of puberty Premature thelarche is an incomplete form of pubertal development without any other signs of early puberty, such as a pubertal growth spurt or marked acceleration of skeletal age

Answer 50

Exaggerated adrenarche - Adrenarche is an early stage in sexual maturation that happens in some higher primates and in humans typically peaks at around 10 to 14 years of age and is eventually involved in the development of pubic hair, body odor, skin oiliness, and acne Precocious pseudopuberty - partial pubertal development that results from autonomous (gonadotropin-independent) production of testosterone in a prepubertal boy. Affected boys have premature virilization and rapid growth, but they do not produce sperm

Answer 51

Premature pubarche/adrenarche is a further normal variant of pubertal development in which pubic and/or axillary hair develop in a girl before age 8 or in a boy before age 9 The serum levels of adrenal androgens are normal, and there is no androgeninduced growth spurt The skeletal age corresponds to the chronological age or is only mildly accelerated Premature pubarche is to be distinguished from congenital adrenal hyperplasia, and an androgen-secreting tumor with elevated levels of adrenal androgens

Answer 52

Premature menarche - defined as isolated or recurrent vaginal bleeding in a prepubertal female in the absence of appropriate secondary sexual characters or a known pathological cause

Answer 53

a condition that causes early sexual development in girls and boys. While puberty normally starts between ages 8 and 13 in girls and between ages 9 and 14 in boys, girls with central precocious puberty begin exhibiting signs before age 8, and boys with this disorder begin before age 9 Pubertal development - Breast development in girls and Testicular enlargement in boys Growth spurt Advanced bone age

Answer 54

Acne, greasy skin/hair, body odour Voice change Mood swings Vaginal discharge/bleeding Growth- acceleration? Neuro problems: Perinatal history, Headaches, Radiotherapy Drugs FHx: Parental pubertal timing

Answer 55

Fundi/ Neuro BP Skin

Answer 56

Girls: – Usually idiopathic – Pituitary imaging Boys: – Look for underlying cause, i.e. brain tumor?

Answer 57

GnRH agonist (generally used to down-regulate the secretion of pituitary gonadotropins and to inhibit ovarian steroid secretion)

Answer 58

Precocious pseudopuberty is partial pubertal development that results from autonomous (gonadotropin-independent) production of testosterone in a prepubertal boy. Affected boys have premature virilization and rapid growth, but they do not produce sperm Gonadotrophin independent (low/prepubertal levels of LH and FSH) Abnormal sex steroid hormone secretion Virilasing or feminasing Clinical picture: secondary sexual characteristics usually produces a clinical picture of incomplete puberty in which some of the milestones of puberty do not appear or fail to achieve the usual synchronicity. Development occurs despite low or prepubertal levels of FSH and LH Treatment depends on the aetiology

Answer 59

• Girls – usually **_early_** developers – watch rapid **_progression/growth_** acceleration • Boys – usually **_late_** developers • Beware abnormal patterns of **_pubertal development_**

Answer 60

* Do not guess the sex of the baby! * Multidisciplinary approach (paed endo, surg, neonatologist, geneticist, psychologist) * Exam: gonads?/internal organs * Karyotype * Exclude Congenital Adrenal Hyperplasia!- risk of adrenal crisis is first 2 weeks of life

Answer 61

1 in 4000 births

Answer 62

**Athyreosis** (abnormal condition caused by absence or functional deficiency of the thyroid gland)/**hypoplastic** (underdevelopment or incomplete development of a tissue or organ)/**ectopic** (abnormal place) Dyshormonogenic

Answer 63

* Newborn screening * Start treatment within first 2 weeks

Answer 64

Autoimmune thyroiditis

Answer 65

* Family history of thyroid/autoimmune disorders * Childhood issues: - Lack of height gain - Pubertal delay (or precocity) - Poor school performance (but work steadily)

Answer 66

* Nearly a third (31%) of children aged 2–15 are overweight or obese * The direct cost of obesity to the NHS is estimated to be £4.2bn a year * At Reception and Year 6, children in the poorest decile are almost twice as likely to be obese compared those in the most affluent decile

Answer 67

* Weight * Body mass index (BMI) (kg/m2) * Height * Waist circumference * Skin folds * History and examination * Complications

Answer 68

no, this is abnormal

Answer 69

Diet Physical activity Family history Symptoms suggestive of - Syndrome - Hypothalamic-pituitary pathology - Endocrinopathy - Diabetes

Answer 70

Acanthosis Nigricans - Velvet rash – sign of insulin resistance – not everyone who is obese has this May be short as have a hypothalamic tumour High blood pressure Problems with doing exercise

Answer 71

LOADS! ## Footnote Metabolic syndrome Fatty liver disease (nonalcoholic steatohepatitis) Gallstones Reproductive dysfunction (eg, PCOS) Nutritional deficiencies Thromboembolic disease Pancreatitis Central hypoventilation Obstructive sleep apnea Gastroesophageal reflux disease Orthopaedic problems (slipped capital femoral epiphysis, tibia vara) Stress incontinence Injuries Psychological Left ventricular hypertrophy Atherosclerotic cardiovascular disease Right-sided heart failure

Answer 72

* SIMPLE OBESITY * Drugs * Syndromes * Endocrine disorders * Hypothalamic damage

Answer 73

* Insulin * Steroids * Antithyroid drugs * Sodium Valproate

Answer 74

Prader Willi syndrome Laurence-Moon-Biedl syndrome Pseudohypoparathyroidism type 1 Down’s syndrome

Answer 75

Hypothyroidism Growth hormone deficiency Glucocorticoid excess Hypothalamic lesion (tumour/trauma/infection) Androgen excess Insulinoma Insulin resistance syndromes Leptin deficiency

Answer 76

Diet Exercise Psychological input Drugs??? - not to be used in children

Answer 77

Simple Investigations

Answer 78

Endocrine causes = growth failure Syndromes = learning difficulties Hypothalamic causes = loss of appetite control

Answer 79

Diabetic ketoacidosis (DKA) is a serious complication of type 1 diabetes and, much less commonly, of type 2 diabetes. DKA happens when your blood sugar is very high and acidic substances called ketones build up to dangerous levels in your body. Ketoacidosis shouldn't be confused with ketosis, which is harmless 1 in 4 children with type 1 diabetes are diagnosed in DKA Rising to 1 in 3 under the age of 5 years

Answer 80

DKA is preventable if diabetes is diagnosed early

Answer 81

THINK – Symptoms - Thirsty - Thinner - Tired - Using the Toilet more TEST- Immediately - Finger prick capillary glucose test. If result \>11mmol/l TELEPHONE – Urgently, contact your local specialist team for a same day review

Answer 82

Sometimes referred to as the 4T’s: **T**hirsty **T**ired **T**hinner Using the **T**oilet more

Answer 83

A return to bedwetting or day-wetting in a previously dry child is a “red flag” symptom for diabetes

Answer 84

heavier than usual nappies blurred vision candidiasis (oral, vulval) constipation recurring skin infections irritability, behaviour change

Answer 85

Nausea & vomiting Abdominal pain (Vomiting and abdominal pain in diabetics is nearly always DKA unless proven otherwise) Sweet smelling, "ketotic" Breath Drowsiness Rapid, deep “sighing” respiration Coma

Answer 86

Finger prick capillary blood glucose test Result \>11mmol/l - Diabetes Result \<11mmol/l - Other cause …………………………………… All these tetss are for when you suspect type 2 in adults: DO NOT request a returned urine specimen DO NOT arrange a fasting blood glucose test DO NOT arrange an Oral Glucose Tolerance Test DO NOT wait for lab results (urine or blood)

Answer 87

TELEPHONE – Same Day Review Call local specialist paediatric diabetes team for a same day review Diabetic Ketoacidosis (DKA) can occur very quickly in children If in any doubt about a diagnosis of Type 1 Diabetes call for advice Don’t delay the diagnosis