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RC Peds Endo 2023 > Lipids > Flashcards

Lipids Flashcards

1
Q

dyslipidemia criteria?

A

LDL-C ≥ 3.4mmol/L

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2
Q

what is a statin?

A

HMG-CoA reductase inhibitors

  • Block hepatic HMG Co-A reductase (rate limiting enzyme in cholesterol biosynthesis)
  • Leads to increased LDL receptor and decreased serum cholesterol
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3
Q

adverse effects of statins?

A

Headaches, myalgias, hepatotoxicity, myopathy, rhabdomyolysis

Most common: elevated LFTS (improves after d/c)

***Teratogenic

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4
Q

meds for hyperlipidemia besides statins

A

Resins = Bile acid sequestrants

Cholesterol absorption inhibitors (ezetimibe)

Fibric acids

Nicotinic acid

Omega 3 fatty acids

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5
Q

what to monitor once starting statin

A

Serum ALT, AST, CK and lipids 1 month after initiating therapy
Monitor every 3-6 months

Monitor for growth abnormalities and secondary sexual characteristics

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6
Q

FH - gene

A

90%: LDL receptor (LDLR)
Other:
apoB (APOB)
proprotein convertase subtilisin-kexin type 9 (PCSK9)
LDL receptor adaptor protein 1 (LDLRAP1)

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7
Q

how to dx FH

A

LDL-C >/= 4.0
PLUS ONE OF
- DNA mutation
- tendon xanthoma
- LDL-C >/= 8.5

if none of those but have FDR w elevated LDL or with early CVD, probably FH

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8
Q

goal of statin therapy?

A

An LDL-C 2.6 mmol/L or less, or a minimum of 50% reduction from baseline.

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9
Q

secondary hyperCh causes

A

● Endocrine:
○ Hypothyroidism
○ T1DM/T2DM
○ Pregnancy
○ PCOS
○ Lipodystrophy
● Renal
○ CKD
○ HUS
○ Nephrotic syndrome
● Hepatic:
○ Obstructive liver disease/cholestatic conditions
○ Biliary cirrhosis
○ Alagille syndrome
● Inflammatory disease
○ SLE
○ JIA
● Storage disease
○ Glycogen storage disease
○ Gaucher disease
○ Cystine storage disease
○ Juvenile Tay-Sachs disease
○ Niemann-Pick disease
● Others
○ Anorexia Nervosa
○ Kawasaki disease
○ Solid organ transplantation
○ Childhood cancer survivor
○ Idiopathic hypercalcemia
○ Klinefelter syndrome
○ Werner syndrome
● Drugs – corticosteroids, some OCPs

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10
Q

meds that cause hyperlipidemia

A

glucocorticosteroids
isotretinoin
OCP
insulin
thiazide diuretics
fibrates

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11
Q

if testing for familial hypercholesterolemia, what genes to test for?

A

LDLR, APOB and PCSK9

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12
Q

exam findings in hyperCh

A

i. Xanthomas – tendon, tuberous, or planar
(Tendon xanthomata in children are highly suggestive of homozygous FH)
ii. arcus corneae
iii. Xanthelasma

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13
Q

how does DM cause hyperTG

A

1) insulin def causes markedly decreased transcription of the LPL gene (administration of insulin usually helps restore normal TG levels within days, however LDL may rise due to beta effect where VLDL → LDL; this takes much longer to resolve)

2) increased flux of FFA to liver stimulates production of TGs and their secretion in VLDL

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14
Q

causes of elevated LDL with normal TG

A

i. Familial hypercholesterolemia (LDLR mutation)
ii. Autosomal dominant hypercholesterolemia (PCSK9 activating mutation)
iii. Autosomal recessive hypercholesterolemia (protein affected that allows LDLR endocytosis)
iv. ApoB100 mutation
v. Anorexia

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15
Q

what is the pathophys for Dysbetalipoproteinemia

A

genetic mutation in Apo E (most commonly E2)

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16
Q

if CK is elevated when on a statin, what do you do

A

stop the medication and consider restarting

The threshold for worrisome level of CK is 10 times above the upper limit of reported normal

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17
Q

CHILD-1 diet

A

<10% diet is fat and <300 mg cholesterol a day

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18
Q

CHILD-2 diet

A

<7% of diet is fat and <200 mg cholesterol a day

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19
Q

hyperTG and abdo pain

A

pancreatitis

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20
Q

referral to endo criteria for lipids

A
  • LDL > 3.4 mmol/L
  • HDL < 0.9 mmol/L
  • TG > 2.3 mmol/L
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21
Q

why is it so important to tx children w hyperCh?

A
  • Atherosclerosis is the basis of CV disease
  • Begins in youth
    ○ Often clinically SILENT
    ○ Only see on labs
    ○ And atherosclerotic changes seen starting in childhood, before clinically apparent signs
  • Directly linked to CV risk factors including dyslipidemia

Identification and management of dyslipidemia in childhood might serve to delay the onset and slow the progression of atherosclerotic CVD, particularly in high-risk populations.

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22
Q

what is the function of Ch in the body

A

Ch steroids
membranes
bile acids

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23
Q

what is the structure of FFA

A

3 TG bound to a glycerol backbone

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24
Q

what is the role of TG in the body

A

fuel source to
- heart, muscle

undergo B-oxidation in mitochondria
to produce acetyl CoA

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25
Q

what lipoproteins have more TG and what have more ChE?

A

more TG: chylomicrons, VLDL
more ChE: LDL, HDL

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26
Q

what apoproteins are for:
structure
ligand
to activate LPL

A

structure: B48 and B100

ligand: B100 and E

to activate LPL: CII

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27
Q

what does ApoE bind to and why

A

LRP1 to exit the endogenous pathway and go into the liver

28
Q

what binds to LDL-R

A

B-100

29
Q

when are the Apo-B’s involved?

A

endogenous pathway
(not exogenous)

30
Q

what is PCSK9

A

binds to LDL-R and causes its degradation

31
Q

where is hepatic lipase

A

in the blood

32
Q

what are apolipoproteins

A

transport proteins that bind to lipids to form lipoproteins

33
Q

what does ApoA1 do?

A

reverse pathway
cholesterol uptake from cells
activates LCAT

34
Q

what Ch does ApoA1 define

A

HDL
(A is Awesome
B is Bad)

35
Q

what is the role of LDL

A

deliver Ch to cells

36
Q

what Ch is ApoB100 in

A

LDL
IDL
VLDL

37
Q

how does HDL transfer Ch to VLDL and LDL

A

via CETP

38
Q

Lipoprotein (a)

A

strongly atherogenic

39
Q

what level of TG makes LDL not measurable

A

4.5

40
Q

LDL levels in FH

A

hetero: 2-3X UL
homo: 4-6X UL

41
Q

PSCK9 mutation

A

PSCK9 -> degradation for LDLR

GOF mutation -> increased degradation

42
Q

Ch profile in HF

A

high LDL
normal TG, HDL

43
Q

Ligand-defective apoB100
Lipid profile

features

A

Moderate to markedly high LDL normal TGs

Caused by poor binding of the LDL particle to the LDLR, because of a mutation in apoB-100
results in a decreased clearance of LDL from plasma

tendon xanthomas

44
Q

primary hyperTG DDx

A
  • = just hyperTG
    *LPL deficiency
    *Apo-CII deficiency
    *Familial chylomicronemia syndrome
    *Familial hypertriglyceridemia
  • Dysbetalipoproteinemia
  • Familial LPL inhibitor
  • Familial combined hyperlipidemia
45
Q

Causes of hyperTG

A
  • Diabetes: insufficient insulin therapy, as insulin is required for LPL activity
  • Uncontrolled T1DM
  • Uncontrolled T2DM
  • Hypopituitarism
    – GH Deficiency (incr VLDL production and dcr VLDL clearance)
    – Hypothyroidism
  • GSD
  • Renal Failure
  • Nephrotic syndrome (upregulation of cholesterol synthesis due to protein loss, upregulation of HMG Co-Reductase)
  • Pregnancy (elevated estrogen that causes incr VLDL production and dcr LPL)
  • Acute hepatitis
  • EtOH (large amounts can cause incr VLDL and impaired lipid metabolism)
  • HIV (?secondary to incr inflammation)
  • Medications:
    — Glucocorticoids (incr VLDL production)
    — PEG-asparginase (incr VLDL production and dcr LPL activity)
    — Estrogens (incr VLDL secretion and dcr hepatic lipase)
    — Anti-psychotics
46
Q

Eruptive xanthomas - what dx

A

hyperTG

47
Q

Lipemia retinalis - what dx

A

hyperTG

48
Q

mgmt hyperTG

A

fibrates
omega-3 FA

insulin in acute

49
Q

what level of TG are you at risk of pancreatisi

A

10-11

50
Q

how to dx LPL def

A

LPL activity

51
Q

physical exam in hyperTG

A

Eruptive xanthomas
Lipemia retinalis

52
Q

what is the most common cause of hyperCh?

A

Familial combined dyslipidemia

53
Q

Familial combined dyslipidemia

A

Caused by hepatic overproduction of lipoprotein particles containing apoB-100, namely VLDL and LDL

Elevated TG, LDL, low HDL

no exam findings

54
Q

Familial Dysbetalipoproteinemia

A

Abnormal ApoE which is important for uptake of TG rich chylomicrons + VLDL

labs:
Elevated TGs, low LDL & HDL

palmar and tuberous xanthomas

DIS BETA RAPPER

55
Q

hypolipoproteinemia
aka
gene

A

Tangier disease
ABCA1
very low HDL
cellular cholesterol build up

tonsils orange

56
Q

Secondary Dyslipidemia causes

A

R/O CHAN always
Cholestasis
Hypothyroidism
Anorexia nervosa
Nephrotic syndrome

Other causes:
ENDO
- DM
- Pregnancy
- PCOS
RENAL
- Nephrotic syndrome
- CKD
HEPATIC
- Obstructive liver disease/cholestatic conditions
- Biliary cirrhosis
IMMUNOLOGIC
- HIV
RHEUM
- JIA
- SLE
DRUGS
- Glucocorticoid
- Alcohol
□ XS: Increased TG
□ Mod alcohol can increased HDL
- OCP
- Atypical antipsychotics
- Isotretinoin
- Bile acid sequestrants
- Cyclophosphamide

57
Q

obesity related dyslipidemia
lipid profile

A

Mild-moderate hyperTG
Low HDL-C

58
Q

lipid profile in DM

A

high TG
low HDL
normal LDL

59
Q

universal screening for children, when?

A

between 9 and 11 years of age

again at 17-21 years of age

60
Q

what constitutes “Fhx early CVD”

A
  • parent, grandparent, aunt, or uncle
    – <55 years for men and <65 years for women
    –history of angina, myocardial infarction, coronary artery disease, or sudden cardiac death
61
Q

Corneal arcus, xanthelasmas or tendon xanthomas suggest?

A

high LDL

62
Q

Lipemia retinalis and eruptive xanthomas over extensor surfaces and buttocks

A

high TG

63
Q

palmar and tuberous xanthomas

A

Dysbetalipoproteinemia

64
Q

what is Etezimibe

A

bile acid sequestrant

65
Q

PCSK9 inhibitors
s/e

A

flu-like symptoms, site reactions, myalgias, fatigue

66
Q

meds that cause high TG

A

steroids, estrogen, beta-blockers, HAART, isotretinoin, BAS, antipsychotics

RC Peds Endo 2023 (17 decks)

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