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Paediatrics > Endocrine > Flashcards

Endocrine Flashcards

1
Q

Insulin start dose

A

0.5 units/kg

increase by 1-2 units/kg

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2
Q

DKA diagnosis

A

blood glucose >11.1
blood ketones >3
metabolic acidosis

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3
Q

DKA management

A

fluids resuscitation 10ml/kg
sthen slow rehydration over 48hrs
insulin 0.1units/kg/h
watch K+

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4
Q

Congenital hypothyroidism causes

A 
maldescent of thyroid 
athyrosis 
dyshormonogenesis 
iodine deficiency 
TSH deficiency
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5
Q

Descent of thyroid gland

A

sublingual position -> larynx

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6
Q

Congenital hypothyroidism clinical features

A 
faltering growth 
prolonged jaundice 
constipation 
coarse facies 
large tongue 
umbilical hernia 
delayed development 
learning difficulties
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7
Q

Congenital hypothyroidism management

A

thyroxine from neonatal period

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8
Q

PTH function

A

increased osteoblast function
if Ca low-> osteoclast activity to release ca and PO
stimulates vit D metabolism

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9
Q

Hypoparathyroidism can be congenital- what is the name of the syndrome

A 
DiGeorge 
Cardiac abnormalities
Abnormal facies
Thymus hypoplasia -> cellular immune deficiency
Cleft palate
Hypoparathyroidism w/ low Ca
22 chromosome
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10
Q

Hyperparathyroidism if presents in late childhood/adulthood, can be part of what syndrome?

A

MEN 1 and 2a

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11
Q

Term babies should double and treble their weight in what period of time?

A

Double in 4.5m

Treble in 1 year

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12
Q

Puberty: development order in females

A
  1. breast development
  2. pubic hair and growth spurt
  3. menarche
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13
Q

Puberty: development order in males

A
  1. testicular enlargement over 4ml volume
  2. pubic hair
  3. rapid height growth
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14
Q

Short stature define

A

below 2nd gentile (2SDs below the mean)

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15
Q

Investigation required below which height gentile

A

0.4 th

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16
Q

Short stature causes

A

familial
constitutional delay
small for gestational age and extreme prematurity
chromosomal disorders

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17
Q

Gene involved in pathological short stature

A

Short stature homeobox (SHOX) gene

X chromosome

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18
Q

Short stature investigations

A 
x ray l wrist 
FBC 
Creatine (CKD)
Calcium, phos, alk phos 
TSH 
Karyotyope 
Anti-endomysial and anti-tissue transglutaminase antibodies 
CRP 
GH provocation test 
IGF-1 levels (GH axis)
Morning cortisol and dexamethasone test 
MRI
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19
Q

Growth hormone treatment indications

A 
GH deficiency 
Turner syndrome 
Prader-Willi syndrome 
CKD 
SHOX deficiency 
IUGR/small for gest. age w/o catch up by 4yrs
20
Q

Tall stature causes

A 
familial/inherited 
obesity 
hyperthyroidism 
XS sex steroids
gigantism (XS GH)
Syndromes (Marfan, Homocystinuria, Kliengelter, Sotos syndrome)
21
Q

Development of undifferentiated gonad in fetes into female

A

absence of SRY gene (Y chromosome)

22
Q

Development of undifferentiated gonad in fetes into testis in male

A

SRY (sex determining region on Y chromosome)
anti-Mullerian hormone (inhibits uterus and Fallopian tube development)
testosterone drives external genitalia development and maintains internal genitalia

23
Q

XS androgens in females

A

seen in congenital adrenal hyperplasia

causes virilisation in females

24
Q

inadequate androgens in male

A 
under-virilisation
caused by 
-receptor problem (androgen insensitivity)
-5alpha reductase deficiency 
-abnorm. of synthesis from cholesterol
25
Q

Congenital adrenal hyperplasia causes…

A

insufficient cortisol and mineralocorticoid secretion

26
Q

Congenital adrenal hyperplasia caused by

A

deficiency in 21-hydroxylase

+/- aldosterone deficiency

27
Q

Congenital adrenal hyperplasia clinical features

A

virilisation in females
-> clitoral hypertrophy, fusion of labia
enlarged penis and pigmented scrotum in males
salt-loosing adrenal crisis
tall stature
precocious pubarche

28
Q

Aldosterone function

A

salt and water retention

via increased ENaC expression

29
Q

Congenital adrenal hyperplasia diagnosis

A

increased-> 17 alpha hydroxy- progesterone

30
Q

Congenital adrenal hyperplasia: salt loosing crisis management

A

IV chloride
glucose
hydrocortisone

31
Q

Congenital adrenal hyperplasia: general management

A

glucocorticoids (hydrocortisone)
-> ACTH suppression -> normal growth and maturation
mineralocorticoids (fludrocortisone)
monitor growth, skeletal maturity, plasma androgens and 17alpha hydroxy-progesterone and adjust dose

32
Q

HPA axis

A

Hypothalamus-> CRH
Ant Pit -> ACTH
Adrenal cortex-> cortisol

33
Q

Primary adrenal insufficiency (Addison’s disease) diagnosis

A

Synthacthen test

- normal response excludes adrenal insufficiency

34
Q

Precocious puberty types

A 
gonadotrophin dependent (central, true)- premature activation of the axis -> normal sequence 
independent (XS sex steroids outside of pituitary, LH and FSH suppressed) -> abnormal sequence
35
Q

Female precocious puberty causes

A

idiopathic/familial*
pituitary adenoma (gonad. dep)*
XS androgens from CAH, adrenal tumours (gonad. independent.)

36
Q

Female precocious puberty Ix

A

USS ovaries and uterus

-> progress of puberty

37
Q

Which type of precocious puberty is common in females and why?

A

Gonadotrophin dependant

Ovaries are sensitive to LH and FSH

38
Q

Which type of precocious puberty is common in males and why?

A

gonadotrophin independent

testes are relatively insensitive to LH and FSH

39
Q

Male precocious puberty causes

A

b/l testes enlargement -> gonad. dep cause
- intracration tumour
- liver ca secreting b-hCG
pre-pubertal testes (gonad. independent. cause)
-adrenal pathology
unilateral testis enlargement
- gonadal tumour

40
Q

Precocious puberty management

A

gonadotrophin releasing hormone analogue
-> delays onset of menage
Gonadotrophin independent cause
- inhibitors of androgen or oestrogen production or action
-> medroxyprogesterone acetate, cyproterone acetate, testolactone, ketoconazole

41
Q

Define precocious puberty

A

<8yrs females

<9yrs males

42
Q

Define delayed puberty

A

> 14 yrs females

>15 yrs males (more common)

43
Q

Delayed puberty causes

A 
constutianal delay 
low gonadotrophin 
-systemic disease 
-pituitary dysfunction 
-isolated gonadotrophin or GH deficiency 
-Kallmann syndrome (LH def)
-acquired hypothyroidism

high gonadotrophin

  • klinefelter’s, Turner’s
  • gonadal damage
44
Q

Delayed puberty management in males

A

not usually required
oxandrolone
androgenic anabolic steroids
testosterone

45
Q

Delayed puberty management in females

A

r/o organic cause

oestradiol

Paediatrics (9 decks)

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