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4th year Paeds > Endocrine > Flashcards

Endocrine Flashcards

1
Q

Diagnosis diabetes

A

Symptomatic + random glucose >11, fasting >7 or 2hr test >11

Asymptomatic + venous sample in diabetic range + confirm with repeat test another day (random/fasting/2h)

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2
Q

The two insulin regimes

A

Twice daily (2/3 before breakfast and 1/3 pre dinner, both are split 1/3 short acting and 2/3 medium acting)

Multiple dose pen injection (basal background in eve and short acting pre meals)

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3
Q

What advice should be given about exercise in diabetic child

A

Sugary snack before

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4
Q

When should blood glucose be monitored more closely in children?

A

When ill

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5
Q

What is the dawn phenomenon

A

Increase in glucose at 4am due to GH surge

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6
Q

What is somogyi phenomenon

A

Insulin extra given to counteract dawn phenomenon- rebound hyperglycaemia

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7
Q

How do you know how dehydrated the child is in DKA

A

pH <7.1= 10%

> 7.1 = 5%

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8
Q

Paediatric DKA Rx

A

Bolus 10ml/kg if shocked

maintenance and defecit fluids 0.9% saline

Insulin 0.01-0.1u/kg/h infusion after the first hour of fluid (to reduce glucose at a rate of <5mmol/h)

Add potassium when electrolytes back (40mmol/L)

When glucose <12 change the fluid to 0.9% saline and 5% dextrose

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9
Q

Most common cause ambiguous genitalia

A

Congenital adrenal hyperplasia

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10
Q

What is true hermaphroditism

A

Genetic e.g. chimeric 46XX/XY

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11
Q

What could cause virilisation of a female foetus?

A

Maternal androgens e.g. ingested or tumour

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12
Q

How is sex determined in a foetus

A

Y chromosome has SRY gene that = gonads –> testes. If absent, they become ovaries

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13
Q

What is congenital adrenal hyperplasia

A

Autosomal recessive

Affect synthesis of adrenal steroids

Low cortisol=ant pit secretes more ACTH=more adrena androgens produced=virilisation of female foetus

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14
Q

90% congenital adrenal hyperplasia caused by what

A

21-hydroxylase deficiency

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15
Q

How might congenital adrenal hyperplasia present

A

Adrenal (salt losing) crisis 1-2w

Precocious puberty in boys

Cliteromegaly in girls

HTN

Tall stature child/short as adult

Hirsutism

Old bone age

skin hyperpigmentation

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16
Q

What type of congenital adrenal hyperplasia presents as intersex boys but non-virilised girls

A

17-hydroxylase defic

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17
Q

If Dx congenital adrenal hyperplasia antenatally what can you do?

A

Give maternal dex to reduce chance of virilisation of foetus

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18
Q

Precocious puberty ages

A

<8 girls

<9 boys

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19
Q

FSH and LH high or low in gonadotrophic dependent precocious puberty?

A

High, central control, premature activation of HPG axis

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20
Q

FSH and LH high or low in gonadotrophic independent precocious puberty?

A

Low, peripheral cause

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21
Q

Which type of precocious puberty is likely to have an unusual progression e.g. isolated thelarche

A

Pseudo/gonadotrophic independent

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22
Q

Bilaterally enlarged testes suggests what cause of precocious puberty

A

IC lesion releasing gonadotrophin

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23
Q

Unilat enarlged testes suggests what cause of precocious puberty

A

Gonadal tumour

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24
Q

Small testes suggests what cause of precocious puberty

A

Adrenal

25
Q

Female precocious puberty is usually..?

A

idiopathic, familial, not worrying

26
Q

precocious puberty cause with cafe au lait spots and polyostic fibrous dysplasia?

A

McCune Albright syndrome

27
Q

First sign of female puberty

A

Breast development

28
Q

First sign of male puberty

A

Testicular enlargement

29
Q

When do female pubertal growth spurts occur?

A

Breast stage 3 (no nipple contour separation)

30
Q

When do male pubertal growth spurts occur?

A

At testicular volume 10-12mL

31
Q

What four things are used to determine a boy’s stage of puberty?

A

Genitals 1-5

Pubic hair 1-5

Axillary hair 1-3

Testicular volume 2-25mL

32
Q

What four things are used to determine a girl’s stage of puberty?

A

Breast 1-5

Pubic hair 1-5

Axillary hair 1-3

Menarche

33
Q

What ages = delayed puberty

A

Girls by 13

Boys by 14

34
Q

Boys or girls delayed puberty more common? What is most common cause

A

Boys

Constitutional

35
Q

Causes of delayed puberty with low gonadotrophin secretion

A

Short stature:

  • Constitutional
  • Hypothyroid
  • Malnutrition
  • Prader-willi

Normal stature:

-Prolactinoma

Kallman’s (no sense of smell)

36
Q

Causes of delayed puberty with high gonadotrophin secretion

A

Short stature: Turner’s

Normal stature: Klinefelters (47XXY) or Gonadal disease e.g. torsion, trauma, RT

37
Q

Raised TSH and low thyroxine is what type of hypothyroid

A

Primary

38
Q

cause of 90% congenital hypothyroid?

A

Thyroid dysgenesis- aplasia or ectopic

39
Q

How does a baby with congenital hypothyroid present

A

Goitre

Wide spaced eyes, flat nasal bridge, macroglossia

Large fontanelle

Short, broad fingers

Dry skin

Hypotonic

Constipation and abdo distentino, unconj jaundice

Cardiomegaly/brady/murmurs

Hoarse cry, feeding probs, apnoea/noisy resp

Sleepy

Mental slowness

40
Q

How and when do you test for congenital hypothyroid

A

Guthrie test at 5days (TSH >100)

41
Q

Causes of primary acquired hypothyroid

A

Thyroiditis (hashimotos or atrophic autoimmune)

Iodine defic

RT due to leukaemia or lymphoma

42
Q

How does acquired hypothyroid present

A

Growth decel

Delayed ossification

Dry skin and hair

Lat 1/3 eyebrow lost

Low energy

Mental slowness

Constipation

Cold intolerance

43
Q

Phenylketonuria is a defect in what?

A

phenylalanine breakdown (involving phenylalanine hydroxylase)

44
Q

If untreated phenylketonuria leads to what?

A

Slowly progressive mental retardation

Spastic cerebral palsy

Hyperactivity

Athetosis

Acquired microcephaly

Fair hair, skin and blue eyes

45
Q

Test for phenylketonuria?

A

Guthrie test showing raised phenlyalanine levels

46
Q

What is the Guthrie test?

A

5 days old heel prick screening for:

S gh ickle cell

CF

Congenital hypothyroidism

6 metabolic diseases including phenylketonuria

47
Q

How do you calculate expected height from parental height?

A

Boys:

((Mothers height + 12.5) + father’s)/2

Girls:

((Father’s height - 12.5) + mother’s)/2

48
Q

How is bone age useful in short stature?

A

Idea of skeletal maturity. Delayed bone age w/o pathology means they will grow more

49
Q

What growth velocity requires investigation

A

<25th centile

50
Q

What height = short stature

A

<2nd centile

51
Q

Causes of short stature

A
  1. Familial
  2. Constitutional
  3. Psychosocial deprivation
  4. Chronic illness/malnutrition
  5. Endocrine- GH defic, hypothyroid, PHP and PPHP, cushing’s
  6. Chromosomal- Turners, downs, prader willi
  7. IUGR (Russell-Silver synd)
  8. Achondroplasia
52
Q

Which causes of short stature can be treated with GH injections

A

GH deficiency

Turners

IUGR

Prader Willi

53
Q

When is it FTT

A

height/weight <2nd centile or cross down 2 centiles

54
Q

Weight faltering suggests what?

A

Psychosocial

55
Q

Reasons for FTT

A

Inadequate intake (available food, breastfeeding technique, anorexia, meal time behaviour, low SES)

Inadequate retention (vom, gord)

Malabsorption (coeliac, CF, CMPI, short gut synd)

Can’t use the nutrients (sydromes, T21, prem, storage disorders, IUGR)

Increased requirements (thyrotoxicosis, CF, malignancy, chronic infection, renal failure, heart disease)

56
Q

History in FTT

A

Food diary

What happens at meal times

Child well? Energy?

Lethargy/D+V/cough

Prem? IUGR? birth probs? Meconium

Family growth and FHx

57
Q

Which short child should be reviewed by GP and which by paeds?

A

<2nd centile GP

<0.4 Paeds

58
Q

What tool is used to define obesity in children

A

BMI percentile charts adjusted to age and gender

59
Q

Primary amenorrhoea, no pubic hair, groin swellings suggests what?

A

Androgen insensitivity

Male karyotype but female phenotype

Groin swellings are undescended testes

4th year Paeds (13 decks)

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