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porphyria Flashcards

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1
Q

rare disorders that affect heme synthesis

A

porphyria

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2
Q

if enzyme missing/defiant=

A

no heme made or decreased amounts

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3
Q

buildup of precursors =

A

causes lots of symptoms

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4
Q

what does porphyria affect

A

nervous or cutaneous system or BOTH

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5
Q

can be inherited or acquired

A

porphyria

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6
Q

how does a porphyria begin

A

triggered by stress, antibiotics, toxic substances, pregnancy, alcohol

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7
Q

what is the most sig buildup

A

porphobilinogen
uroporphyrinogen
protoporphyrinogen 3

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8
Q

structure of porphyria

A

alternating double bonds
4 chiral rings joined together

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9
Q

porphyria absorbs visible light

A

purple or dark red

cause RBC and teeth to fluorescent

chelate metal ions and cause symptoms

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10
Q

pathological

A

many who have gene don’t express porphyria illness

can be triggered

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11
Q

how is it diagnosed?

A

excretion of metabolites

-check urine or feces for increases of porphyrin

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12
Q

where is uroporphyrin excreted

A

urine

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13
Q

where is protoporphyrinogen excreted

A

feces

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14
Q

where is coproporphyrinogen excreted?

A

urine and feces

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15
Q

where is ALA delta aminolevulinic acid

A

urine

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16
Q

enzyme early on in pathway

A

nervous system

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17
Q

enzyme further down in pathway

A

cutaneous symptoms –
photosensitivity 1st common symptoms

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18
Q

acute porphyria

A

neurological
symptoms come on acutely
-acute abdominal pain
-muscle weakness

extreme case: coma and death

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19
Q

syndrome of inappropriate antidiuretic hormone (vasopressin)

A

increases in amounts of antidiuretic hormone

decreases in Na– leads to G.I symptoms and neurological chains

-makes you retain water

last days to weeks

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20
Q

how to treatment acute porphyria

A

no medication to cure due to enzyme lack

-stay away from trigger!

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21
Q

1 type of acute porphyria

A

acute intermittent porphyria

seen in: sweden, netherlands

22
Q

what is defect in acute intermittent porphyria

A

buildup of ALA and porphobilinogen= urine

porphyrin absorb visible light= reddish/purple urine– port wine color

23
Q

cutaneous porphyria

A

buildup of porphyrin in skin

photosensitivity absorb visible light= #1 symptom

24
Q

what happens in cutaneous

A

blisters and sunburns

increase facial hair due to defect in heme synthesis chain

25
Q

most common cutaneous porphyria

A

porphyria cutanea tarda

26
Q

what happens in porphyria cutanea tarda

A

blister on skin

hypopigmentation and disfiguring of extremities (most exposed to light)

27
Q

how is porphyria cutanea tarda obtained

A

inherited and acquired

acquired- hep. c and contraceptives and iron overload

28
Q

liver involvement in porphyria cutanea tarda

A

buildup of hemosiderin and necrosis of the liver

29
Q

erythropoietic protoporphyria

A

accumulate in RBC
-look at RBC and flursconct due to absorb visible light

buildup of protoporphyrinogen(onset adults)

30
Q

congenital erythropoietic is also called

A

gunther’s disease

31
Q

congenital erythropoietic

A

-hereditary and see in infancy

in sun and UV lights babies will scream
will develop pain and blisters

vampire!!

32
Q

characteristics of congenital erythropoietic

A

teeth like a vamp
misshapen heads
erythrodontia= teeth absorb UV light and turn red

33
Q

congenital erythropoietic excret

A

copro and uro

34
Q

hemolytic anemias

A

increase in porphyrins and lead to splenomegaly

35
Q

variegate porphyria (w/ variety)

A

cutaneous and neurological symptoms independently of each other

autosomal dominant inheritance

misdiagnosed with buildup of metabolites depending on symptoms

36
Q

variegate porphyria neurological

A

porphobilinogen

37
Q

variegate porphyria cutaneous

A

proto and coproporphyrinogen

38
Q

secondary porphyrias

A

no genetic components due to exposure of certain chemicals and looks like porphyria

39
Q

what is the #1 secondary porphyria

A

lead poisoning from paint chips

40
Q

lead poisoning

A

occupational hazard for painters and being around lead based pipes

-lead can stay in bones, teeth, tissues for long time

41
Q

can a preg lady give lead poisoning to her baby

A

yes

42
Q

lead poisoning inhibits

A

heme chain at ALA and ferrochelatase enzyme and stops iron going into Hgb

HGB molecule needs something in core: replace iron with zinc

43
Q

what do you end up with in lead poisoning

A

zinc protoporphyrin

(erythrocyte zinc)??

44
Q

detect lead poisoning by

A

basophilic stippling

hypochromic, microcytic RBC’s resembles IDA– its not do not want more iron

45
Q

body during lead poisoning

A

iron increases buildup not getting in

transferrin decreases bound with iron

% saturation increases because more iron

total binding Fe capacity decreases (no room)

46
Q

how to differentiate with IDA and lead poisoning

A

% saturation (IDA will be low)

look at lead levels –chelation therapy preformed

47
Q

lab testing porphyrias

A

look at increases in analytes present in urine or stool

also ALA increased (neurological)

48
Q

urine for testing porphyrias

A

must be in dark container and not exposed to light before testing bc will absorb light

49
Q

what test helps differentiate porphyrias

A

watson-schwartz

urobilinogen- normal
porphobilinogen- porphyria

50
Q

how to perform watson-schwartz? screening

A

urine sample– add ehrlich’s reagent

screen turns red= may have uro or porp present

stays yellow= done

if light colors– add sodium acetate to enhance color

51
Q

once urine is red add

A

spilt into 2 tubes

add butanol in one
add chloroform in the other

butanol will float to top because density is lighter than urine

chloroform sinks to the bottom

52
Q

how to read watson-schwatz test

A

if in both tubes red remains in area added= urobilinogen

if red remains in urine part= porphobilinogen

chem (5 decks)

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