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82 terms

Chem exam 6

STUDY
PLAY
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