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Peadiatrics > Endocrinology > Flashcards

Endocrinology Flashcards

1
Q

What are the two endogenous chemicals that increase serum Ca2+ levels in the body?

A

1,25-(OH)2-DH

  1. Increases Ca2+ and PO4 absorption from the gut
  2. Increase Ca2+ resorption from bone
  3. Increases renal tubular Ca2+ and PO4 resorption

= primary ligand for vitamin D receptor in many tissues -> binds to vitamin D receptor in intestine, inducing expression of transport proteins

PTH

  1. Increases kidney Ca2+ resorption
  2. Decreases PO4 resorption
  3. Increases activity of kidney hydroxylase -> increased 1,25-(OH)2-D production

Main regulator of PTH release = serum Ca2+

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2
Q

What is the most common MODY type?

A

MODY3

MODY2, MODY3 and MODY5 make up for 90% all MODYs

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3
Q

What are the characteristics of MODY2, MODY3, MODY 5, MODY1?

A

MODY2
Second most common
Gene = glucokinase (mild decrease in beta-cell response to glucose)
Hyperglycaemia mild and non-progressive

MODY3
Most common
Gene = HNF-1 alhpa (hepatocyte nuclear factor)
Very sensitive to sulfonylureas

MODY5
Gene = HNF1-beta
Associated with: renal cysts/dysfunction, pancreatic exocrine dysfunction, hypospadias, joint laxity, uterine abnormalities

MODY1
Gene = HNF4-alpha
Very sensitive to sulfonylureas
Decreased levels of TAGs and apolipoprotein

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4
Q

Other than T1DM/T2DM, what are other rarer types of DM?

A
  1. Genetic defects of beta-cell function
    - > MODY
  2. Mitochondrial gene defects
    - > Wolfram syndrome 1 (diabetes insipidus, DM, optic atrophy, deafness = DIDMOAD); Wolfram 2 (no diabetes insipidus)
    - > avoid metformin (risk of severe lactic acidosis)
  3. Neonatal diabetes mellitus
    - > transient vs. permanent, IPEX (immunodysregulation, polyendocrinopathy and enteropathy, X-linked)
  4. Genetic defects of insulin action (mutation in insulin receptor)
    - > Donohue syndrome (IUGR, fasting hypoglycaemia, postprandial hyperglycaemia, profound insulin resistance), Rabson-Mendenhall syndrome (extreme insulin resistance, acanthosis nigricans, abnormalities teeth and nails, pineal hyperplasia), lipoatrophic diabetes, Stiff-Person (progressive stiffness, painful spasms of axial muscles), SLE
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5
Q

Does an ectopic posterior pituitary gland produce anterior or posterior problems?

A

Anterior

Failure of posterior pituitary to migrate and form the infundibulum causes anterior pituitary deficiencies

Normal stalk = isolated GH deficiency
Abnormal stalk = multiple pituitary hormone deficiencies

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6
Q

What are the side effects of GH therapy?

A 
SCFE 
Pseudotumour cerebri 
Transient carbohydrate intolerance 
Transient hypothyroidism 
Scoliosis
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7
Q

Which is more specific for GH-deficiency: IGF-1 or IGF-BP3?

A

IGF-BP3
Vary less with age and nutrition -> more specific (though less sensitive) for GH action

IGF-1
Can vary greatly with age and nutritional status regardless of GH secreion -> sensitive, but not specific

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8
Q

What is the most common anterior pituitary tumour?

A

Prolactin-secreting tumours

MRI of sella = best diagnostic tool

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9
Q

What is the hormone that causes testicular enlargement?

A

FSH

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10
Q

What are the causes or high vs. low thyroxine-binding globulin? What is its significance?

A

Increased TPG -> increased total T4

Decreased TPG -> decreased total T4

This is because total T4 measures a combination of free T4 and bound T4

INCREASED TPG 
Oestrogen (OCP, pregnancy) 
Tamoxifen 
Narcotics 
Hepatitis, biliary cirrhosis 
DECREASED TPG 
Androgens 
Glucocorticoids 
Nephrotic syndrome 
Inherited TPG deficiency
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11
Q

Does amiodarone cause hyper or hypothyroidism?

A

BOTH

Amiodarone is iodine-rich
-> also blocks peripheral deiodination of reverse T3 and T4 -> increased reverse T3 and T4, and decreased T3

HYPERthyroid = due to iodine

HYPOthyroid = too much iodine and peripheral deiodination

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12
Q

What type of hypothyroidism is not detected on the neonatal screening test?

A

SECONDARY hypothyroidism

Screening test does not detect low TSH levels (as seen in secondary hypothyroidism)

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13
Q

When administering thyroxine to neonates/infants with hypothyroidism, what do you NOT mix it with?

A

Do NOT mix thyroxine with: (as can be bound)

Soy protein formula
Iron
Calcium supplements

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14
Q

What change does alkalosis cause to calcium levels?

A

HYPOcalcaemia

As pH increases, albumin becomes more ionised into anions -> causes free calcium to bind more strongly with albumin

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15
Q

What are the counter-regulatory hormones?

A

= Hormones that counter the effect of insulin

Glucagon
Adrenaline
Cortisol
Growth hormone

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16
Q

Why is acanthosis nigricans seen in T2DM?

A

Marker of insulin resistance

Pathogenesis = hyperinsulinaemia thought o play key role -> ?stimulate keratinocyte and dermal fibroblast proliferation via IGFR-1

Thus, seen ALSO in hyperinsulinaemic states (e.g. insulin-producing tumour)

Peadiatrics (13 decks)

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