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Specialties > Paeds Written - ENDO > Flashcards

Paeds Written - ENDO Flashcards

1
Q

1st sign of Male & Female puberty

A

Male = increase in testicular volume

  • usually 12 yrs old (range 10-15)
  • vol >4ml defines onset of puberty

Female = breast development

  • ~11.5 yrs
  • may be asymmetrical
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2
Q

Definition of precocious puberty

A

Development of secondary sexual characteristics before 8 years in female or 9 years in male

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3
Q

Types & mechanism of precocious puberty

A

Gonadotrophin dependent / central / true = premature activation of hypothalamic-pituitary-gonadal axis

Gonadotrophin independent / pseudo / false = excess sex hormones

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4
Q

Rare but important cause of precocious puberty

A

McCune Albright syndrome

  • disorder of bone, skin & endocrine tissue
  • abnormal scar like fibrous tissue in bones ‘polyostotic fibrous dysplasia’
  • cafe au lait spots
  • other endo issues e.g. thyrotoxicosis
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5
Q

Sex hormone results in precocious puberty

A

Central / Gonadotrophin dependent = LH, FSH raised

Pseudo / Gonadotrophin INdependent = LH, FSH low

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6
Q

Central / gonadotrophin dependent causes of precocious puberty

A

Idiopathic - 80% of girls, 40% of boys

CNS abnormality - tumour, trauma, central congenital disorder

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7
Q

Pseudo / gonadotrophin INdependent causes of precocious puberty

A

Ovarian - follicular cyst, Leydig cell tumour, granulosa cell tumour, gonadoblastoma

Testicular - Leydig cell tumour, testotoxicosis

Adrenal - Congenital adrenal hyperplasia, Cushing’s syndrome

Somatic tumour e.g. b-hCG secreting liver tumour

McCune Alright syndrome

Exogenous hormones - COCP, testosterone gel

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8
Q

Gold standard for Ix of (male) precocious puberty

A

GnRH stimulated LH/FSH

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9
Q

Size of testes in different types of precocious puberty?

A

Small = congenital adrenal hyperplasia

Bilateral enlargement = central cause - intracranial lesion e.g. optic glioma in NF1

Unilateral enlargement - sex cord gonadal stromal tumour

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10
Q

Medication options for Gonadotrophin DEPENDENT precocious puberty

A

GnRH agonist e.g. leuprolide + Growth hormone

GnRH agonist + cyproterone (anti-androgen)

Or no Tx if idiopathic

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11
Q

Medication options for Gonadotrophin INDEPENDENT precocious puberty

A

McCune Alright or Testotoxicosis
1st line = ketoconazole, cyproterone
2nd line = aromatase inhibitors

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12
Q

Causes of HYPO gonadotrophic hypogonadism

A

Hypo-thalamo pituitary disorders - panhypopituitarism, tumours

Kallman’s - LHRH deficiency, anosmia

Prader-Willi

Acquired hypothyroidism

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13
Q

Causes of HYPER gonadotrophic hypogonadism

A

Congenital

  • cryptorchidism
  • Klinefelter’s (47 XXY)
  • Turner’s (45 X0)

Acquired

  • testicular torsion
  • chemotherapy
  • infection
  • trauma
  • autoimmune
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14
Q

Functional causes of delayed puberty

A

Constitutional delay of growth & puberty

Chronic disease, malnutrition

Psych - over exercising, anorexia, depression

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15
Q

Medical management of delayed puberty

A

Pubertal induction

  • Males = IM testosterone monthly for 6 months
  • Females = transdermal oestrogen for 6 months THEN cyclical progesterone once established

+ Regular hormone replacement (only needed if primary failure NOT constitutional delay)

  • Males = regular, as above
  • Females = gradual oestrogen to avoid premature epiphyseal fusion + overdeveloped breasts
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16
Q

Key clinical features of Androgen Insensitivity syndrome

A

Phenotypic female

  • breast buds
  • sparse pubic hair

Bilateral groin swelling = immature undescended testes

Primary amenorrhoea
- absence of uterus + ovaries

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17
Q

Tx required for androgen insensitivity syndrome

A

Counselling!!

Bilateral orchidectomy - increased risk of testicular ca. if left undescended

Oestrogen therapy

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18
Q

T1 DM Diagnostic Criteria

A

Symptoms AND either fasting 7+ OR random 11.1+

No Sx AND 2 of:

  • fasting 7+
  • random 11.1+
  • OGTT 11.1+

HbA1c 6.5%+ or 48+

19
Q

Classical Triad of T1DM

A

Polyuria
Polydipsia
Weight loss

20
Q

Insulin regimens

A

1st line = multiple daily injection ‘Basal-Bolus’

  • short acting insulin with meals e.g. Lispro
  • 1 or more separate injections of intermediate/long acting insulin (analogue) e.g. Glargine

2nd line = insulin pump

  • usually rapid or short acting
  • often needs to be for older child with good control
21
Q

General principles of T1DM management

A

Same day referral to MDT paediatric diabetes team

Insulin therapy

Education

  • diet, exercise
  • insulin method
  • hypo awareness & Tx
  • DKA awareness
  • BM home monitoring

Monitoring

  • HbA1c 4+ times per year
  • after 12 yo, annual checks for retinopathy, nephropathy, HTN
22
Q

BSPED criteria for diagnosis of DKA

A

D - diabetes = BM > 11.1

K - ketones = >3

A - acidosis = pH <7.3

23
Q

How to calculate fluid deficit for child with DKA

A 
Deficit volume (to be replaced over 48 hours)
= (% deficit x weight) - any fluid bolus if NOT shocked

NOTE: in shocked child - do not minus boluses

24
Q

How to determine % fluid deficit in child with DKA

A

Mild DKA ph <7.3 = 5% fluid deficit
Moderate DKA pH <7.2 = 7% fluid deficit
Severe DKA pH <7.1 = 10% fluid deficit

25
Q

Special requirements for choice of fluid in DKA?

A

Fluid bolus - can be 0.9% NaCl

Maintenance & deficit fluids:
- 0.9% NaCl
with 20mmol potassium chloride per 500ml

26
Q

Fluid maintenance calculation in children?

A

First 10kg body weight = 100mL/kg/day
Next 10kg body weight = 50mL/lg/day
Each extra kg after that = 20mL/kg/day

E.g. 55kg child needs (10 x 100) + (10 x 50) + (35 x 20) = 2200mL over 24 hours

27
Q

Insulin - dose & when to start in DKA?

A

0.05-0.1 units / kg / hour
Start infusion 1-2 hours after IV fluid replacement

NOTE: if on insulin pump, STOP

28
Q

Monitoring needed during treatment of DKA

A 
Hourly blood glucose
Neuro status at least hourly
Hourly fluid input:output
U&E 2 hours after start of IV therapy THEN 4 hourly
1-2 hourly blood ketone levels
29
Q

Criteria for ‘resolution of DKA’

A

Clinically well
Drinking well, tolerating food
Blood ketones <1.0 OR pH normal
NOTE: Urine ketones may still be positive

30
Q

Complication seen during Tx of DKA?

A

Cerebral oedema

Tx:

  • call senior staff!
  • hypertonic saline
  • mannitol
  • restrict IV fluids by half
31
Q

Genetics of Achondroplasia

A

Autosomal dominant inheritance
BUT ~70% of mutations are sporadic

FGFR3 mutations

32
Q

Clinical features of osteogenesis imperfecta

A 
Blue sclera
Hearing loss
Short stature
Loose joints
Breathing problems
33
Q

Dwarfism definition

A

Height > 2.5 SD below the mean

34
Q

Key clinical features of Achondroplasia

A

Short limbs + shortened fingers

Large head + frontal bossing

Flattened nasal bridge

‘Trident’ hand deformity

Lumbar lordosis

35
Q

Radiological evidence of Achondroplasia

A

Metaphyseal irregularity
- inverted V metaphysis ‘chevron deformity’

Long bone flaring

Late appearing, irregular epiphyses

36
Q

Pattern of growth in Nutritional/Chronic disease OR Psychosocial (deprivation / neglect) short stature

A

Falling off height centiles
Weight centile < height centile
Delayed bone age

37
Q

Pattern of growth in Endocrine-related short stature

A

Falling of height centimes.
Weight centile > height centile (i.e. short + overweight)
Markedly delayed bone age.

38
Q

Pattern of growth in constitutional delay

A

Short stature
Accentuated by delayed puberty
Delayed bone age

39
Q

Pattern of growth in Familial short stature

A

Following growth centile

Within predicted range for mid-parental height

40
Q

Causes of congenital hypothyroidism

A

Thyroid gland defect - missing, ectopic, poorly developed

Disorder of metabolism - TSH unresponsive, defect in TG structure

Hypothalami-pituitary dysfunction - tumours, ischaemia, congenital defect

Transient hypothyroidism - maternal meds (carbimazole) or Abs (hashimoto’s

41
Q

Unique Sx of congenital hypothyroidism

A

Coarse features
Macroglossia
Umbilical hernia

42
Q

Most common cause of hypothyroidism in childhood?

A

Hashimoto’s autoimmune thyroiditis

43
Q

RF for childhood Hashimoto’s

A

Down’s syndrome

Turner’s syndrome

44
Q

Safety netting advice for Hyperthyroidism drugs

A

Carbimazole + Propylthiouracil can cause neutropenia

Seek medical attention (+ do FBC) if sore throat or fever

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