2. PorphyriaPorphyria
The porphyrias are caused byThe porphyrias are caused by
deficiencies of enzymes involved indeficiencies of enzymes involved in
heme biosynthesis which lead toheme biosynthesis which lead to
blockade of the porphyrin pathwayblockade of the porphyrin pathway
and subsequent accumulation ofand subsequent accumulation of
porphyrins and their precursors.porphyrins and their precursors.
3.
4. IntroductionIntroduction
Seven major types of porhyria areSeven major types of porhyria are
now recognized .now recognized .
They include acute and non-acuteThey include acute and non-acute
forms.forms.
6. in porphyria increased amount of uroporphyrinein porphyria increased amount of uroporphyrine
or protoporphyrine are presnt inor protoporphyrine are presnt in
urine.coproporphyrine also may be presnt ,ALLurine.coproporphyrine also may be presnt ,ALL
ARE DERIVED FROM PORPHOBILLINOGENARE DERIVED FROM PORPHOBILLINOGEN
7. Porphobilinogen is charcteristic of the diseses butPorphobilinogen is charcteristic of the diseses but
its identification in the urine is difficult.its identification in the urine is difficult.
Porphobilinogen in urin is exposed to air will bePorphobilinogen in urin is exposed to air will be
converted into dark red colored porphobillinconverted into dark red colored porphobillin
In porphyrineuria coproporphyrine perdominate inIn porphyrineuria coproporphyrine perdominate in
the urine coproporphyrineuria iii may occur afterthe urine coproporphyrineuria iii may occur after
exposure to metal(lead poisoning is a prominentexposure to metal(lead poisoning is a prominent
example)sedative,sulonamides, alchol, andexample)sedative,sulonamides, alchol, and
various organic substance ``in hypermetbolicvarious organic substance ``in hypermetbolic
states` with hepatic diseses in variousstates` with hepatic diseses in various
haemtological condation and in plegra and andhaemtological condation and in plegra and and
riboflavin deficency.riboflavin deficency.
8. in lead poisnoning the amount of coproporphyrinein lead poisnoning the amount of coproporphyrine
iii excreted in the urine is markedly increasediii excreted in the urine is markedly increased
.small or moderate increase are to be inerpreted.small or moderate increase are to be inerpreted
with cautionwith caution
9. Method for identification and quntitativeMethod for identification and quntitative
estimation of porphyins are based uponestimation of porphyins are based upon
solubility differnce in organic solventsolubility differnce in organic solvent
and chromatographic orand chromatographic or
spectrophotometric anylysis.Qualitativespectrophotometric anylysis.Qualitative
testing of urine for porphobilnogen is atesting of urine for porphobilnogen is a
simple proceduresimple procedure
11. ReagentReagent
Ehrlich s ragentEhrlich s ragent
a.paradimethy aminobenzaldehyde…..0.7ga.paradimethy aminobenzaldehyde…..0.7g
b concentrated HCL……150mlb concentrated HCL……150ml
c distelled water…..100mlc distelled water…..100ml
12. ProcedureProcedure
this test is based on differntialthis test is based on differntial
extaction.Add 3 ml of Ehrilich reagent to 3 ml ofextaction.Add 3 ml of Ehrilich reagent to 3 ml of
urin and mix. Add 3ml chloroform shake for 2urin and mix. Add 3ml chloroform shake for 2
mint and then let stand .persistence of reddishmint and then let stand .persistence of reddish
color in the aqueous supernatant undicatecolor in the aqueous supernatant undicate
porphobilinogen. MANYY SUBSTANCE CAUSE Aporphobilinogen. MANYY SUBSTANCE CAUSE A
FALSE POSITIVE REACTION .NOTABLE AREFALSE POSITIVE REACTION .NOTABLE ARE
ANTICONVULASANT DRUGSANTICONVULASANT DRUGS
PYRIDIUM.UROROSEIN.OR INDOXYL..PYRIDIUM.UROROSEIN.OR INDOXYL..
