Download Document

Chronic Care Programme
Treatment guidelines
Hyperlipidaemia
Chronic condition
Consultations protocols
Preferred treating provider
Notes
 preferred as indicated by option
 referral protocols apply
Maximum consultations per annum
 Initial consultation
 Follow-up consultation
Tariff codes
Option/plan
Provider
GMHPP
Gold Options
G1000, G500
and G200.
Blue Options
B300 and B200.
GMISHPP
General Practitioner
Pulmonologist
Physician
Gastroenterologist
Neurologist
Cardiologist
Paediatrician
Cardiology Paediatrician
Mild to Moderate
New Patient Existing Patient
New Patient
1
0
1
1
0183; 0142; 0187; 0108
1
3
Mild to Moderate
New Patient
Existing
Patient
Consultation: Dietician
 Initial consultation
 Follow-up consultation
Tariff codes
1
0
Severe
Existing Patient
1
2
Severe
New Patient
Existing
Patient
0
1
1
0
0
1
051
Mild to Moderate
New Patient
Existing
Patient
Consultation: Dietician
 Initial consultation
 Follow-up consultation
Tariff codes
1
0
Severe
New Patient
Existing
Patient
0
0
2
0
0
1
053
Investigations protocols
Type
Provider
Tariff code
Urine dipstick (per stick,
irrespective of the number of
tests on stick)
Blood glucose
ECG without effort
Chol/HDL/LDL/Trig
TSH
Serum urea
Serum creatinine
Serum potassium
GP; Specialist;
Pathologist
GP; Specialist;
Pathologist
GP or
Specialist
(see list)
Pathologist
Pathologist
Pathologist
Pathologist
Pathologist
Maximum investigations per annum
New
Existing
New
patient
patient
patient
Existing
patient
4188
1
1
2
2
4057;
4050
1
0
1
0
1232
1
1
1
1
4025
4507
4151
4032
4113
2
1
1
1
1
1
0
0
0
0
3
1
1
1
1
3
0
1
1
1
Serum sodium
Aspartate aminotransferase
(AST)
Alanine aminotransferase
(ALT)
Creatine Kinase (CK)
CXR
Pathologist
Pathologist
4114
4130
1
1
0
1
1
1
1
1
Pathologist
4131
1
1
1
1
Pathologist
Pathologist
4132
3445
1
0
1
0
1
1
1
1
E78.1-E78.5
ICD 10 coding
General
Hyperlipidemia, hyperlipoproteinemia or dyslipidemia is the presence of raised or abnormal
levels of lipids and/or lipoproteins in the blood. Lipids (fatty molecules) are transported in a
protein capsule, and the density of the lipids and type of protein determines the fate of the particle
and its influence on metabolism.
Lipid and lipoprotein abnormalities are extremely common in the general population, and are
regarded as a highly modifiable risk factor for cardiovascular disease due to the influence of
cholesterol, one of the most clinically relevant lipid substances, on atherosclerosis. In addition,
some forms may predispose to acute pancreatitis
Classification
Hyperlipidemias are classified according to the Fredrickson classification which is based on
the pattern of lipoproteins on electrophoresis or ultracentrifugation.[1] It was later adopted by the
World Health Organization (WHO). It does not directly account for HDL, and it does not
distinguish among the different genes that may be partially responsible for some of these
conditions. It remains a popular system of classification, but is considered dated by many.
Fredrickson classification of Hyperlipidemias
Problems
Labs
description
Hyperlipoproteinemia
Synonyms
Type I
Buerger-Gruetz
syndrome, Primary
hyperlipoproteinaemia,
or Familial
hyperchylomicronemia
Decreased
lipoprotein
lipase
Elevated
Diet Control
(LPL) or
Chylomicrons
altered
ApoC2
Type IIa
Polygenic
hypercholesterolaemia
or Familial
hypercholesterolemia
LDL
receptor
deficiency
Bile Acid
Elevated LDL Sequestrants,
only
Statins,
Niacin
Type IIb
Combined
hyperlipidemia
Decreased
LDL
receptor
and
Increased
ApoB
Elevated LDL
Statins,
and VLDL
Niacin,
and
Gemfibrozil
Triglycerides
Type III
Familial
Defect in
Dysbetalipoproteinemia ApoE
Increased
IDL
Treatment
Drug of
choice:
Type IV
Type V
synthesis
Gemfibrozil
Endogenous
Hyperlipemia
Increased
VLDL
production Increased
and
VLDL
Decreased
elimination
Drug of
choice:
Niacin
Familial
Hypertriglyceridemia
Increased
VLDL
production
and
Decreased
LPL
Increased
Niacin,
VLDL and
Gemfibrozil
Chylomicrons
Hyperlipoproteinemia type I
This very rare form (also known as Buerger-Gruetz syndrome, primary hyperlipoproteinaemia, or
familial hyperchylomicronemia) is due to a deficiency of lipoprotein lipase (LPL) or altered
apolipoprotein C2, resulting in elevated chylomicrons, the particles that transfer fatty acids from
the digestive tract to the liver. Lipoprotein lipase is also responsible for the initial breakdown of
endogenously made triacylglycerides in the form of very low density lipoprotein (VLDL). As
such, one would expect a defect in LPL to also result in elevated VLDL. Its prevalence is 0.1% of
the population.
Hyperlipoproteinemia type II
Hyperlipoproteinemia type II, by far the most common form, is further classified into type IIa and
type IIb, depending mainly on whether there is elevation in the triglyceride level in addition to
LDL cholesterol.
Type IIa
This may be sporadic (due to dietary factors), polygenic, or truly familial as a result of a mutation
either in the LDL receptor gene on chromosome 19 (0.2% of the population) or the ApoB gene
(0.2%). The familial form is characterized by tendon xanthoma, xanthelasma and premature
cardiovascular disease.
Type IIb
The high VLDL levels are due to overproduction of substrates, including triglycerides, acetyl
CoA, and an increase in B-100 synthesis. They may also be caused by the decreased clearance of
LDL. Prevalence in the population is 10%.

Familial combined hyperlipoproteinemia (FCH)

Secondary combined hyperlipoproteinemia (usually in the context of metabolic
syndrome, for which it is a diagnostic criterion)
Treatment
While dietary modification is the initial approach, many patients require treatment with statins
(HMG-CoA reductase inhibitors) to reduce cardiovascular risk. If the triglyceride level is
markedly raised, fibrates may be preferable due to their beneficial effects. Combination treatment
of statins and fibrates, while highly effective, causes a markedly increased risk of myopathy and
rhabdomyolysis and is therefore only done under close supervision. Other agents commonly
added to statins are ezetimibe, niacin and bile acid sequestrants. There is some evidence for
benefit of plant sterol-containing products and ω3-fatty acids[2]
Hyperlipoproteinemia type III
This form is due to high chylomicrons and IDL (intermediate density lipoprotein). Also known as
broad beta disease or dysbetalipoproteinemia, the most common cause for this form is the
presence of ApoE E2/E2 genotype. It is due to cholesterol-rich VLDL (β-VLDL). Prevalence is
0.02% of the population.
Hyperlipoproteinemia type IV
This form is due to high triglycerides. It is also known as hypertriglyceridemia (or pure
hypertriglyceridemia). According to the NCEP-ATPIII definition of high triglycerides (>200
mg/dl), prevalence is about 16% of adult population.[3]
Hyperlipoproteinemia type V
This type is very similar to type I, but with high VLDL in addition to chylomicrons.
It is also associated with glucose intolerance and hyperuricemia
Unclassified forms
Non-classified forms are extremely rare:

Hypo-alpha lipoproteinemia
 Hypo-beta lipoproteinemia (prevalence 0.01-0.1%)
Signs and symptoms
A combination of heredity and diet is responsible for the majority of fat disorders. It is not so
much the cholesterol in the diet that is the problem, because that accounts for only 10% of the
body’s store. It is the other fats in the diet that alter the way the body handles its cholesterol.
There is a convincing relation between fats in the diet and the incidence of atherosclerosis. The
guilty fats are mostly the animal fats, but palm and coconut oil are also harmful. These fats are
called saturated fats for the chemical reason that most of their carbon atoms have as many
hydrogen atoms attached as they can accommodate.
More important than the kind of fat is the amount of fat. For many people, fat is half of their diet.
A quarter to a fifth is a much healthier fraction, the rest of the diet being made up of complex
carbohydrates and protein.
This disease is silent for decades, until the first episode of heart disease or stroke.
Diagnosis
It would be easier if simple cholesterol and triglyceride tests were all it took to assess the risk of
atherosclerosis. But the important information is which package the cholesterol is in—the LDLs
or the HDLs. That takes a more elaborate testing process. To complicate matters further, the
amount of fats in the blood varies greatly in relation to the last meal—how long ago it was and
what kind of food was eaten. A true estimate of the risk comes from several tests several weeks
apart all done after at least twelve hours of fasting.
Treatment
Diet and lifestyle change are the primary focus for most cholesterol problems. It is a mistake to
think that a pill will reverse the effects of a bad diet, obesity, smoking, excess alcohol, stress, and
inactivity. Reducing the amount of fat in the diet by at least half is the most important move to
make. Much of the food eaten to satisfy a “sweet tooth” is higher in fat than in sugar. A switch
away from saturated fats is the next step, but the rush to polyunsaturated fats was ill-conceived.
These, and particularly the hydrogenated fats in margarine, have problems of their own. They
raise the risk of cancer and are considered more dangerous than animal fat by many experts.
Theory supports population studies that suggest monounsaturated olive oil may be the healthiest
of all.
There was a tremendous push at the end of the 20th century to use lipid-lowering medications.
The most popular and most expensive agents, the “statins,” hinder the body’s production of
cholesterol and sometimes damage the liver as a side effect. Their full name is 3-hydroxy-3methylglutaryl-coemzyme A (HMG-CoA) reductase inhibitors. Their generic names are
cervistatin, fluvastatin, lovastatin, pravastatin, and simvastatin. Studies show that these do lower
cholesterol. Only recently, though, has any evidence appeared that this affects health and
longevity. Earlier studies showed, in fact, an increased death rate among users of the first class of
lipid-altering agents—the fibric acid derivatives. The chain of events connecting raised HDL and
lowered LDL cholesterol to longer, healthier lives is still to be forged.
High-tech methods of rapidly reducing very high blood fat levels are performed for those rare
disorders that require it. There are resins that bind cholesterol in the intestines. They taste awful,
feel like glue and routinely cause gas, bloating, and constipation. For acute cases, there is a
filtering system that takes fats directly out of the blood.
Niacin (nicotinic acid) lowers cholesterol very effectively and was the first medication proven to
improve overall life expectancy. It can also be liver toxic, and the usual formulation causes a hot
flash in many people. This can be overcome by taking a couple of aspirins half-an hour before the
niacin, or by taking a special preparation called “flush free,” “inositol-bound” or inositol
hexanicotinate.
Omega-3 oil is a special kind found mostly in certain kinds of fish. It is beneficial in lowering
cholesterol.
An herbal alternative called gugulipid, Commiphora mukul, an extract of an Indian plant, is
supposed to work the same way as the expensive and liver toxic cholesterol-lowering
medications.
Alternative treatment
To lower cholesterol, naturopathic medicine, traditional Chinese medicine, and ayurvedic
medicine may be considered. Some herbal therapies include gugulipid, alfalfa (Medicago sativa),
Asian ginseng (Panax ginseng), and fenugreek (Trigonella foenumgraecum). Garlic (Allium
sativum) and onions are also reported to have cholesterol-lowering effects. In naturopathic
medicine, the liver is considered to be an organ that needs cleansing and rebalancing. The liver is
often treated with a botanical formula that will act as a bitter to stimulate bile flow in the liver.
Before initiating alternative therapies, medical consultation is strongly advised.
Medicine formularies
Plan or option
[Link to appropriate Mediscor formulary]
GMHPP
Gold Options
G1000, G500 and
G200
Blue Options
B300 and B00
GMISHPP
Blue Option B100
[Core]
n/a
Prognosis
The prognosis is good for Type 1 hyperlipoproteinemia with treatment; without treatment, death
may result. For Type 2 the prognosis is poor even with treatment. The prognosis for type 3 is
good when the prescribed diet is strictly followed. For types 4 and 5 the prognosis is uncertain,
due to the risk of developing premature coronary artery disease and pancreatitis, respectively.
Prevention
Genetic inheritance cannot be changed, but its effects may be modified with proper treatment.
Family members of an individual with hyperlipoproteinemia should consider having their blood
lipids assessed. The sooner any problems are identified, the better the chances of limiting or
preventing the associated health risks. Anyone with a family history of disorders leading to
hyperlipoproteinemia also may benefit from genetic testing and counselling to assist them in
making reproductive decisions
References
Frederickson DS, Lee RS. A system for phenotyping hyperlipidemia. Circulation
1965;31:321-7. PMID 14262568.
1. Thompson GR. Management of dyslipidaemia. Heart 2004;90:949-55. PMID 15253984.
2. Third Report of the National Cholesterol Education Program (NCEP) Expert Panel on
Detection, Evaluation and Treatment of High Blood Cholesterol in Adults (Adult
treatment Panel III) Final Report. Circulation 2002; 106; page 3240
OMIM
Type I
Type
IIa
Type
IIb
Online 'Mendelian Inheritance in
Man' (OMIM) 238600
Online 'Mendelian Inheritance in
Man' (OMIM) 144400
Type
III
GPnotebook
WebMD
-1389035478 at
GPnotebook
.
-1664090094 at
GPnotebook
.
Others
MeritCare
Merck
-1375338454 at
GPnotebook
.
630849560 at
GPnotebook
WebMD
Yahoo
Type
IV
Online 'Mendelian Inheritance in
Man' (OMIM) 144600
-1362100182 at
GPnotebook
WebMD
Yahoo
Type
V
Online 'Mendelian Inheritance in
Man' (OMIM) 144600
-1355481046 at
GPnotebook
.
.
Inborn error of lipid metabolism - dyslipidemia (E78 and E71.3, 272)
Hyperlipidemia
Hypercholesterolemia/Hypertriglyceridemia (Familial
hypercholesterolemia, Combined hyperlipidemia) - Xanthoma
Hypoalphalipoproteinemia/HDL (Lecithin cholesterol acyltransferase
deficiency, Tangier disease)
Hypolipoproteinemia
Hypobetalipoproteinemia/LDL (Abetalipoproteinemia, Apolipoprotein B
deficiency)
Lipodystrophy
Barraquer-Simons syndrome
transport: Carnitine (Primary, I, II, -acylcarnitine) Adrenoleukodystrophy
Fatty acid
metabolism
deficiency
beta oxidation: Acyl CoA dehydrogenase (Short-chain, Medium-chain,
Long-chain 3-hydroxy, Very long-chain) - Mitochondrial trifunctional
protein deficiency
to acetyl-CoA: Malonic aciduria
Cholesterol synthesis Smith-Lemli-Opitz syndrome
Other
Sjögren-Larsson syndrome - Lipomatosis - Adiposis dolorosa
see also lipid metabolism enzymes, lipoprotein metabolism