Chem exam 6

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ALA Dehydratase Deficiency (ADP) enzyme

ALA Dehydratase

Acute Intermittent Porphyria (AIP) enzyme

PBG Deaminase

Congenital Erythropoietic enzyme

uroporphyrinogen III synthase

Porphyria Cutanea Porphyria (PCT) enzyme

uroporphyrinogen decarboxylase

Hereditary Coproporphyria (HCP) enzyme

coproporphyrinogen oxidase

Variegate Porphyria (VP) enzyme

protoporphyrinogen oxidase

Erythropoietic Protoporphyria (EPP) enzyme

ferrochelatase

plumboporphyria enzyme

ALA dehydratase

glycine + succinyl coA -->

dALA

dALA -->

PBG

PBG -->

uroporphyrinogen I

uroporphyrinogen I -->

uroporphyrinogen III

uroporphyrinogen III -->

coproporphyrinogen

coproporphyrinogen -->

protoporphyrinogen IX

protoporphyrinogen IX -->

protoporphyrin

protoporphyrin -->

Heme

free iron produces

free radicals

Fe__ to Fe __ by HCL in stomach

3+, 2+

Hemosiderin stores a __ concentration of iron and releases it __ compared to ferritin

higher, slower

histochemical evaluation of hepatic iron in liver biopsy

diagnostic test for hemachromatosis

If you see FEP very increased in iron studies, it's probably

lead poisoning

1. transferrin-Fe3+ --> transferrin + Fe3+ 2. Fe3+ + ascorbic acid --> Fe2+ 3. Fe2+ + pyridoylazo dye --> Fe2+-pyridylazo dye (colored)

serum assay

transferrin saturation is most sensitive in

hemachromatosis

available transferrin sites are saturated with iron citrate. Unbound iron is adsorbed and the transferrin-bound iron is measured

total iron binding capacity

apotransferrin + Fe3+ --> Fe-transferrin + Fe2+

total iron binding capacity

measure iron-free apoferritin

serum ferritin

Zn2+ protoporphyrin, measurement of nonheme protoporphyrin in rbc

FEP

URO excreted in

urine

PROTO excreted in

feces

COPRO excreted in

urine and feces depending on pH

Neuropsychiatric porphyrias

PP, AIP

neurocutaneous porphyrias

VP, HCP

proto & copro increase, see PBG, ALA, Copro in urine, see proto, copro in feces:

VP

see copro in feces (maybe some in urine), see ALA & PBG in urine during an acute attack

HCP

cutaneous porphyrias

CEP, EPP, PCT, HEP

urine ALA & PBG increased in __ porphyrias

neurological

urine ALA & PBG not increased in __ porphyrias

cutaneous

see abdominal pain, constipation in __ porphyria

neurological

__ porphyrias do not have abdominal symptoms

cutaneous

increased ALA & copro in urine, normal PBG

plumporphyria

most common cutaneous porphyria

PCT

acquired secondary to liver damage, estrogens, insecticides, etc

PCT

avoid sunlight, iron, alcohol with

PCT

abnormal hair growth

PCT

urine uro increased, some corpro in feces. fecal isocopro.

PCT

do a liver biopsy (fluoresce) to confirm

PCT

deficiency of hetero uro decarboxylase

PCT

Hepatoerythropoietic porphyria enzyme

homozygous uroporphyrinogen decarboxylase

HEP see facial hair?

yes

see an increased occurance of gallstones with

EPP

will see a huge increase in FEP with ___ porphyria (rbc will fluoresce)

EPP

increase in rbc, plasma, fecal protoporphyrins

EPP

Gunthers Disease aka

CEP

Gunthers inherited

autosomal recessive

the only "true" erythropoeitic porphyria: werewolf

CEP

see a huge increase of porphyrins in rbc, BM

CEP

which porphyria will you see red-brown fluorescing teeth?

CEP

diagnostic: increase in rbc, urine, and fecal uro and copro

CEP

Lead inhibits these enzymes

ferrochelatase, ALA dehydratase

see __ in urine with lead poisoning

COPRO

screening test for __ : increased FEP, decreased ALAD

lead

analysis of urine and feces is sufficient for diagnosis of __ porphyria

neurological

Watson test: __ gives red color in aqueous

PBG

fluorescence in lower layer: pos/neg for porphyrins

pos

duodenal aspirate of bile preferred specimen for

VP

liver biopsy fluoresces under Wood's lamp:

PCT

urobilinogen and indole interfere with __ test

watson schwartz

acquired porphyrias: WS test is __

negative

WS 1st step __ layer is red

top

WS 2nd step __ layer is red

bottom

these cells regulate blood flow and store fat soluble vitamins

stellate

conjugated bilirubin bound to albumin

delta

deficiency in UDP glucoronyl transferase: disease

Crigler-Najjar

defect transport from plasma to hepatocyte:

gilbert's

defect in excreting conjugated bilirubin

Dubin-Johnson

5' nucleotidase

seen in obstruction

Alphafetoprotein in adults

diagnostic of liver tumor

copper --> ceruloplasmin defect

Wilson's

rings in eye

Wilson's

alpha 1 antitrypsin

protease inhibitor

Hb competes with binding site on __ test

diazo

Jendrasik: __ stops reaction and shifts pH

acid, alkaline tartrate

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