Intracellualr and extracellular errors of metabolism of lipid, lipidoses, erros of carbohydrtae metabolism Flashcards
lipid staining methods
steatosis: lipid within membrane bound liposomes of perenchymal cells
SUDAN STAINS AND OSMIUM TETTROXIDE
what is fatty liver also known as
steatorrhoeic hepatosis or steatosis heptais
what is fatty liver
reversible condition where large vacouled of TAG fat accumulate in liver cells
what can oversupply come from
obesity, insulin resistance or alcoholism,
also nutrient malnutristion can cause the mobilization of fat from adipocytes and create local oversupply in liver
some other: metabolic syndrome-diabetes, hypertension, dyslipidemia
metabolic causes of steatosis
- a-betalipoproteniemia
- glycogen storage disease
-weber-christian disease - wolmans disease
-acute fatty liver of pregnancy
-lipodystrophy
nutritional causes of steatosis
- malnutrition
- total parenteral nutrition
- severe weight loss
-refeeding syndorme
-jejubo-ileal bypass
-gastric bypass - jejunal diverticulosis with bacterial overgrowth
drugs and toxins causes of steatosis
-amiodarone
methotrecte
-dilitazem
-highly active antitetrovital therapy
-glucicorticoids
-tamoxifen
-enviromantal heptatotoxins-phosphorus, toxis mushroom
other causes
inflammatory bowel disease or HIV
peripheral fatty metamorphosis
due to intocification of amanita ohaloides-death angel
central fatty metamorphosis in liver
right circulatory failure
tocix intracellular steatosis
diphteric myocarditis
lipomatosis is
fatty infiltration within interstital tissue or increase in adipos cells and infiltration into the tissues and organs
most frequent examples of lipomatosis
- atrophia lipomatosa cordis
- pseudohypertrophya lipomatosa pancreatis
- pseudohypertrophya lipomatosa renis
lipidoses is
lipid storage disease
- group of inherited metabolic disorders where harmful amount of lipids accumulate on some cells and tissues.
- dont produce enough enxymes needed for metaboliszation or the produce some that dont work correctly
lipidoses causes damage in
brain, peripheral nervous system, liver, spleen, bone marrow
most common lysosomal storage disease
gaucher disease
Gaucher disease
deficiency of glucocerebrosidase–>acc of glucocerebroside/glucosylceramide
can happen in spleen, liver, kidney, lungs, brain, BM
autosomal recessive ingeritance
symtoms of gaucher
- enlrged spleen and liver
- liver malfunction
- skeletal disorders
- bone lesions
- pain, severe neurologic complication, swelling of Lymphnodes and maybe some near joint, distended abdomen, brownish tint to skin, anemia, low blood platelets, yelloe fatty deposits on the sclera
niemann-pick disease
inheritted condition whre harmful amount of lipids accumulate in spleen, lungs, BM and brian
autosomal recessive
caused by mutation on NPC1, NPC2, SMPD1 genes (sphingomyelin phosphodiesterase)
no lysosmal liposidoses
familial hyperlipoproteinemia
xanthomatous
tangier disease
what is used to identify glycogen
PAS and water
what is diabetes
metabolic disorder characterized by hyperglycemia (^blood sygar) resulting form low lvls of insulin or resistance of insulin effects
symtomps of DM
polyuria, polydipsia(thrist and fluid intake), blurred vision
DM 1
-autoimmune disease–>destruction of insulin producing beta cells
- injection
- not primarly childhood problem
DM 2
- non insulin dependent
- obesty
- insulin resistance, insulin deficiency and hyperglycemia
hyalinosis iof istelts of langehans
Gestational diabetes (GDM
affect pregnant women
reduces insulin sensitivyt
complications of dm
dibaetic ketoacidosis
non ketoic hyperosmolar coma
hypoglycemia
diabetic coma
chronic complications of dm
-vascular disease
- ^blood glucose–>angiopathy
- endithelial cells lining the vv take in more glucosa–>surface glycoprotien –>Basement membrane grow thicker and weaker
gierke and pompe diseaee
gierke: glu-6-phos: lievr
- sudan stain
pompe: acid maltase: ehart and muscles
