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Coovadias Paediatrics > Endocrine disorders > Flashcards

Endocrine disorders Flashcards

1
Q

Discuss the development of the endocrine system in children

A

Differentiation of cells -> hormone producing cells able to secrete
Just before birth, incr ACTH secretion -> incr cortisol -> rapid maturation of enzyme systems
Birth -> incr ADH, renin + angiotensin
Postbirth
-> incr noradrenaline -> blood glucose maintained by metabolism of brown fat
-> incr thyrotropin (birth stress) -> incr thyroid hormones
-> high GT and sex hormone output

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2
Q

Name risk factors for infants at risk of hypoglycemia

A 
Premature
IUGR
Maternal diabetes
Septicemia
Severe illness
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3
Q

Name causes of infant hypoglycemia

A 
Defects in
- hepatic glycogen release/storage
- carnitine metabolism
- fatty acid oxidation 
Hyperinsulinism 
Cortisol deficiency
GH deficiency
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4
Q

Name symptoms of hypoglycemia

A 
Poor feeding
Lethargy
Apnoea
Cyanotic spells
Hypothermia
Seizure
Coma
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5
Q

What tests should be ordered when evaluating hypoglycemia?

A

Critical

  • true blood glucose
  • LFT
  • electrolytes
  • serum bicarb
  • urinalysis

Archival

  • serum for insulin, ketones, GH, cortisol
  • C peptide
  • carnitine profile
  • amino acids
  • toxins
  • urine amino + organic acids
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6
Q

What are the 3 phases of growth and what do they depend on?

A

Infantile - nutrition
Childhood - GH
Pubertal - sex hormones

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7
Q

What investigations are important when evaluating a child with short stature?

A 
Urine dipstix
Stool microscopy
Hb
Serum albumin
Blood urea
T4 and TSH
Radiological bone age
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8
Q

Discuss a differential for familial short stature

A

Prenatal onset

  • IUGR
  • Silver-Russell syndrome
  • Turner syndrome

Postnatal onset

  • psychosocial dwarfism
  • malnutrition
  • GIT disorder
  • renal disorder
  • cardiopulmonary disease
  • chronic anemia
  • endocrine disease
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9
Q

Which children should be evaluated for a growth disturbance?

A
  1. Height
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10
Q

Discuss a differential for endocrine causes of short stature

A 
Hypopituitarism 
Hypothyroidism
Precocious puberty
CAH
Cushing's syndrome
Pseudohypoparathyroidism
Poorly controlled DM
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11
Q

Name causes of pituitary gland malfunction

A

Cranial

  • holoprosencephaly
  • septooptic dysplasia
  • midline craniocerebral abnormality
  • midfacial abnormality

Embryonic

  • pituitary hypoplasia
  • pituitary aplasia
  • congenital absence of pituitary gland

Childhood cancer (radiation)
Infectious (meningitis, encephalitis)
Infiltrative (histiocytosis)
Trauma

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12
Q

What is the clinical presentation of pseudohypoparathyroidism?

A 
Short stature
Truncal obesity
Short metacarpals
Round face
Mental retardation
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13
Q

What is Silver-Russell syndrome?

A

A rare disorder characterized by IUGR, poor postnatal growth, a relatively large head size, a triangular facial appearance, a prominent forehead (looking from the side of the face), body asymmetry and significant feeding difficulties

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14
Q

What is Mauriac syndrome?

A

Severe growth failure and HSM due to hepatic deposition of glycogen in poorly controlled diabetics

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15
Q

Give a differential diagnosis for tall statue

A 
Familial tall stature
Obesity
Thyrotoxicosis
Precocious puberty
Marfan's syndrome
Klinefelter's syndrome
Excess GH
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16
Q

Name disorders that are classified as 46, XY DSD

A

Disorder of gonadal development
Disorder of androgen synthesis/action
Cloacal extrophy
Severe hypospadia

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17
Q

Name disorders that are classified as 46, XX DSD

A

Disorder of gonadal development
Androgen excess
Cloacal extrophy
Vaginal atresia

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18
Q

What should you evaluate when approaching ambiguous genitalia?

A 
Clinical examination
U/S
Exploratory laparotomy
Chromosome analysis
Na/K balance
Plasma 17-OH
Gonadal biopsy
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19
Q

Name disorders of puberty

A 
Premature thelarce
Premature adrenarche
Adolescent gynecomastia
Precocious puberty
Pseudoprecocious puberty 
Delayed puberty
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20
Q

Name disorders of water balance

A 
SIADH
Diabetes insipidus
- central
- nephrogenic
DM type I
DM type II
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21
Q

Name causes of SIADH

A

Severe meningitis
Head injury
Pulmonary disorders
Vincristine therapy

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22
Q

Name the clinical features of SIADH

A 
N+V
Mm weakness
Neurological irritability
Convulsions
Coma
Oedema
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23
Q

How do you manage SIADH?

A

Restrict water intake
3% saline 5ml/kg
Furosemide therapy 0.5-1mg/kg

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24
Q

Name the clinical features of diabetes insipidus

A

Polyuria
Polydipsia
Chronic dehydration
Growth failure

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25
Q

How do you make the diagnosis of diabetes insipidus in children?

A

Urine osmolality <300mOsm/kg
Serum osmolality >300mOsm/kg
Water deprivation test

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26
Q

When should you not perform water deprivation tests?

A

Night time - inadequate monitoring

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27
Q

How do you manage central diabetes insipidus?

A

Lysine vasopressin/DDAVP

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28
Q

What is DDAVP?

A

Desamino D-arginine vasopressin

29
Q

What is the inheritance pattern of nephrogenic diabetes insipidus?

A

X linked dominant

30
Q

How do infants with nephrogenic diabetes insipidus present?

A 
Vomiting
Constipation
Hypotonia
FTT
Recurrent hypertonic dehydration
31
Q

How do you manage a child with nephrogenic diabetes insipidus?

A

High water intake with frequent feeds
Low salt diet
Hydrochlorothiazide 3mg/kg/d
Indomethacin 1.5-3mg/kg/d

32
Q

What is the cause of type I DM?

A

T-cell mediated pancreatic islet beta cell destruction

33
Q

Name clinical features of DM type I

A 
Polyuria
Polydipsia
Vision blurring
Weight loss
Glycosuria
Ketonuria
34
Q

How do you diagnose DM type I?

A

Clinically
Fasting glucoe >7mmol/l
2hr ppg >11.1mmol/l

35
Q

When should hyperglycemia not be regarded as diagnostic of diabetes?

A

Acute stress situation

Incidental finding

36
Q

How does diabetic ketoacidosis occur?

A

Insulin deficiency -> incr levels of catecholamines, glucagon, cortisol and GH -> catabolism

37
Q

Name the clinical features of diabetic ketoacidosis

A 
Acidotic breathing
N+V
Abdominal pain
Decr LOC
Coma
38
Q

What should you always consider in a dehydrated, acidotic patient?

A

Diabetic ketoacidosis

39
Q

What is the criteria to diagnose diabetic ketoacidosis?

A

BG >11 mmol/l
Venous pH <7.3
Serum bicarb <15mmol/l
Ketonuria

40
Q

Discuss management of a DKA child

A

0.9% saline IV 10-20ml/kg bolus
Continue infusion for 36-48hrs (replace fluid deficit)
Insulin 0.1unit/kg/hr
When glucose <11mmol/l, add dextrose
Give potassium
2 hours before discontinuing IV insulin, give 1st insulin maintenance dose (0.5-0.7units/kg/24hr)

41
Q

How do you calculate corrected Na?

A

Measure Na + 2(glucose -5.5)/5.5

42
Q

How do you calculate fluid deficit?

A

7.5% -10% body weight

43
Q

What is a serious complication of DKA?

A

Cerebral oedema

44
Q

Name clinical signs of cerebral oedema

A 
Headache
Altered mental status
Vomiting
Hypertension
Inappropriate HR decrease
45
Q

Name clinical signs of newborn hypothyroidism

A 
Open posterior fontanelle >1cm
Umbilical hernia
Coarse facial features
Poor sucking
Prolonged UC hyperbilirubinaemia
46
Q

Which score is used to recognise congenital hypothyroidism?

A

Congenital hypothyroidism score

47
Q

Name factors in the congenital hypothyroidism score

A 
Umbilical hernia
Coarse facial features
Constipation
Hypothermia
Enlarged tongue
Hypotonia
Jaundice >3days
Dry skin
Wide post fontanelle
GA >40wk
BW >3.5kg
Female
48
Q

Name causes of congenital hypothyroidism

A 
Dysgenesis
Aplasia
Maldescent
Iodine deficiency
Familial enzyme defect
Ingestion of goitrogens
49
Q

Give examples of goitrogens

A

Iodine-containing cough mixtures
Antithyroid drugs
Para-amino-salicylic acid

50
Q

What is the most common use of para-amino salicylic acid?

A

Treat XDR TB

51
Q

How is congenital hypothyroidism treated?

A

Thyroxine 10-15ug/kg/day in 1st year

52
Q

Name clinical features of congenital hyperthyroidism

A 
Emotional lability
Nervousness
Behavioural disturbances
Sweating
Nocturnal enuresis
53
Q

Name clinical features of acute adrenal insufficiency

A 
Circulatory collapse
Low Na
Hypoglycemia
High K
Metabolic acidosis
54
Q

Name causes of adrenocortical insufficiency in children

A 
Aplasia
Hypoplaia
CAH
Haemorrhage
Infection
WF syndrome
Autoimmune
X-linked adrenoleukodystrophy
Steroid therapy
Hypothalamic damage
Pituitary damage
55
Q

Discuss the acute management of adrenocortical insufficiency in children

A

Bloods (electrolytes, acid base, urea, glucose, plasma cortisol assay)
Replace fluids and electrolytes
- Ringer’s lactate/plasma/Haemaccel
- 0.9%NaCl 5% dextrose at 10-20mg/kg over 1st hour then 60ml/kg over following 24hrs
Hydrocortisone sodium succinate
- as IV bolus 50mg small child, 100mg large child, then
- 50-100mg/24hr maintenace
Florinef 0.05-0.1mg//d orally
Monitor patient

56
Q

What is CAH?

A

AR condition due to partial/severe deficiency of enzyme in the biosynthetic pathway of cortisol (and aldosterone in 50% of cases)

57
Q

Discuss management of CAH

A

Oral hydrocortisone 20-25mg/m2/d
Oral florinef 0.05-01mg/d
Dietary salt if needed

58
Q

Name clinical signs of Addison’s disease

A 
Weak
Anorectic
Vomiting
Diarrhoea
Hydration
Hypotension
Skin pigmentation
59
Q

Discuss management of Addison’s disease

A

Oral hydrocortisone 20-25mg/m2/d

Oral florinef 0.05-01mg/d

60
Q

Name the cause of Cushing syndrome

A
  1. Excess steroid therapy
  2. Adrenal tumour
  3. Cushing’s disease
61
Q

Name the clinical signs of Cushing’s syndrome

A 
Moon face
Truncal obesity
Buffalo hump
Purple striae
Mm wasting
Hypertension
Virilisation
62
Q

Name investigations you would perform in a child with signs of Cushing’s syndrome

A

24hr urinary free cortisol excretion
Overnight dexamethasone adrenal suppression test

Tumour:
U/S
IVP
CT

63
Q

Name causes of hypocalcemia in infancy

A 
Prematurity
Asphyxia
Gestational diabetes
Hypoparathyroidism
High P intake
Hypomagnesemia
Chronic alkalosis
Maternal hyperparathyroidism
64
Q

Name causes of hypocalcemia in childhood

A 
Vitamin D deficiency
Rickets
Chronic renal failure
Hypoparathyroidism
Pseudohypoparathyroidism
65
Q

Name investigations you would perform in a child with signs of hypocalcemia?

A 
Calcium
Phosphate
Alkaline phosphatase
PTH
25-hydroxyvitamin D
66
Q

Name parathyroid-related causes of hypocalcemia

A

Congenital

  • transient neonatal hypoparathyroidism
  • PTH synthesis defect
  • aplasia
  • CaSR

Acquired

  • autoimmune
  • surgical
  • magnesium deficiency
  • nutritional
  • bisphosphonate ingestion
  • PTH resistance
67
Q

Discuss management of chronic hypoparathyroidism

A

Calcium supplements

1,25 hydroxyvitamin D

68
Q

Name clinical features of Albright’s hereditary osteodystrophy

A 
Short stature
Mental retardation
Skeletal changes
Subcutaneous ossifications
Brachydactyly
69
Q

What is the Frankfort plane?

A

Line of sight (hole in ear to bottom of eye socket)

Coovadias Paediatrics (10 decks)

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