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35 Cards in this Set
- Front
- Back
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What is correct about fats?
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They are mainly produced in the liver.
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Which of the following is the best source of unsaturated fatty acids?
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vegetable oils
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Insulin activates hormone-sensitive lipase and triggers TAGs degradation.
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False: insulin is low when the FA is released and epinepherine activates the enzyme. high insulin we don't need FA.
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Linolenic Deficiency
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decreased vision and altered learning behaviors
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CPT-I deficiency (carnitine shuttle)
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that affects the liver’s ability to synthesize glucose during a fast
Can lead to severe hypoglycemia, coma, and death |
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CPT-II deficiency
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that occurs primarily in cardiac and skeletal muscle
Causes cardiomyopathy, muscle weakness + myoglobinemia following prolonged exercise Liver disease, malnutrition or strictly vegetarian diet Increased requirement in carnitine during pregnancy, severe infection, or trauma Hemodialysis, which removes carnitine from the blood Treatment: avoid prolonged fast, low LCFA and high carbohydrate diet, supplemented with MCFA and, if necessary, carnitine supplement |
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MCAD Deficiency
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Medium-chain fatty acyl-CoA dehydrogenase (MCAD) deficiency is the most common inborn error of FA metabolism
Decrease in FA oxidation → severe hypoglycemia Infants are particularly affected by MCAD deficiency Breast milk contains primarily MCFAs Treatment includes high-carbohydrate diet |
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Excessive ketone body formation
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High ketone bodies level in blood (ketonemia) and in urine (ketonuria)
Most often in Type 1 (insulin-dependent) diabetes High FA degradation -> high acetyl CoA and low NAD+ -> slows TCA -> forces acetyl CoA to ketone bodies Severe ketosis – urinary excretion can reach 5000mg/24hr, in blood 90 mg/dl (normal 3mg/dl) Elevated ketone body concentration -> acidemia or ketoacidosis Ketoacidosis may also happened during fasting |
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Respiratory distress syndrome (RDS)
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Deficient in Dipalmitoyl phosphotidylcholine production. in pre-term infants is associated with insufficient surfactant production
RDS can occur in adults whose surfactant-producing pneumocytes are damaged or destroyed |
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Niemann-Pick Disease
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Autosomal recessive
Deficiency of sphingomyelinase Severe infantile form (A) Deposits in the liver and brain (neurodegeneration-Mental retardation) Death in early childhood Less severe (B) Deposits in the liver, spleen, lungs and bone marrow Death in early adulthood Incidence in Ashkenazi Jewish population: A – 1:40.000, B-80.000 General population: 1:100.000 |
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Infintile form More severe (A) Niemann pick disease
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lipid Deposits in the liver and brain (neurodegeneration)
Death in early childhood |
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Less severe (B) Niemann pick disease
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lipid Deposits in the liver, spleen, lungs and bone marrow. Spleen and liver enlarged-filled with lipids
Death in early adulthood |
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Lliver is the only site of cholesterol synthesis
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false
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Which of the following agents is an activator of HMG-CoA reductase?
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Insulin-it is the product of high energy
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Sphingolipidoses
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Diseases of the degradation of glycosphingolipids.
Characteristics: Caused by deficiency of hydrolases Leads to accumulation of sphingolipids in lysosomes Can lead to neurologic deterioration and death Specific hydrolytic enzyme is deficient in each disorder Single sphingolipid accumulates in each disease Disorders are progressive and many fatal in childhood Most are autosomal recessive Fabry is X-linked Incidence is low, except Gaucher and Tay-Sachs diseases |
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Gaucher Disease
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Sphingolipidoses:
Macrophages engulfed with glucocerebroside. Most common lysosomal storage disease Results in: Hepatosplenomegaly, Osteoporosis of the long bone, CNS in Juvenile form. |
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Tay Sachs Disease
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Sphingolipidoses:
Accumulation of Gangliosides(GM), lacks hexoaminase Results in: Neurodegeneration, cherry red macula, Blindness, muscle weakness and seizures |
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Fabry Disease
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Sphingolipidoses: X-linked
Globosides accumulate in vascular lysosomes in brain, heart, kidney, and skin Results in: Kidney and heart failure, Reddish-purple skin rash, burning pain in lower extremeties |
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Niemann-Pick Disease
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Sphingolipidoses:
Accumulation of Sphingomyelin. Results In: Hepatosplenomegaly, Neurodegeneration |
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Farber Disease
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Sphingolipidoses: Accumulation of Ceremides-very rare
Results in: Painful joint deformity, granulomas(tissues) |
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Krabbe Disease
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Sphingolipidoses: Accumulation of galactocerebrosides.
Results in: Mental and motor deterioration, Blindness, deafness, Near total loss of myelin, Globoid bodies in brain |
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Metachromatic Leukodystrophy
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Sphingolipidoses: Accumulation of Sulfatides,
Results in: Cognitive deterioration, Demylenation, Progressive paralysis, Dementia, nerves are stained yellow |
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Bile acid sequestrants
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not FDA approved and are used to lower cholesterol
Prevent bile acids reabsorption in the gut The sequestered bile acids are then excreted in the feces. Causes an inability to absorb fat soluble vitamins and drugs. |
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Gall Stones
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Cholesterol solubility is maintained by phospholipids and bile salts
If cholesterol to phospholipid ratio in bile more than 1:1, it results in crystallization of excess cholesterol Gallstones occur in up to 20% of population in western countries Cholesterol stones account for about 80 percent of gallstones |
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Hyperaldosteronism
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Primary (Conn’s disease) is caused by aldosterone-secreting tumor. Secondary due to kidney or liver disease is more common
too much aldosterone |
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Primary adrenal insufficiency or Addison’s disease
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Insufficient production of aldosterone and cortisol due to atrophy of adrenal glands (autoimmune disorder, tuberculosis)
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Secondary adrenal insufficiency
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No enough production of ACTH (abrupt discontinue of taking synthetic glucocorticoids)-steriod therapy
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Cushing’s syndrome
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caused by excessive amounts of cortisol
Exogenous (iatrogenic) caused by administration of glucocorticoids Endogenous caused by excessive production of cortisol due to pituitary adenoma and adrenal adenoma/carcinoma |
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21 alpha hydroxylase deficiency
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Steroid hormone synthesis disease. No mineralcorticoids and glucocorticoids present. No aldosterone or cortisol production. Most common form of CAH. Androgen overproduction= external genetalia in females and early virilization in males
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17 alpha hydroxylase deficiency
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Steroid hormone synthesis disease. No sex hormones(androgens/estrogens) or cortisol produced. Increased minerocorticoid production=Na and fluid retention, hypertension and female like genetalia
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Anabolic steroids
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Dihydrotestosterone (DHT)
Testosterone (esters) Methandrostenolone (Dianabol, DBOL) Oxandrolone (Oxandrin) |
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Methandrostenolone (Dianabol, DBOL)
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Aromatization converts it to estrogen
Heavily masculinising effects Causes lever damage as all 17α-alkylated steroids. Androgens aromatized into estrogen=promotes muscle building and glycolysis and female like effects |
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Oxandrolone (Oxandrin)
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Absence of aromatization affect
Does not inhibit normal testosterone production Causes lever damage as all 17α-alkylated steroids. Androgens not converted to estrogens because of oxygen presence, no female like effects but shrinkage of testicles. |
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Negative Side Affects of Anabolic Steroids(male)
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Baldness
Headaches Breast development Enlarged prostate reduced sperm count testicle shrinkage liver damage strokes and blood clots |
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Negatuve Side Affects of Anabolic Steroids(female)
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Reduced breast size
enlarged clitoris facial hair and body hair increase deepened voice menstrual problems |
