PorphyriaPorphyriaThe porphyrias are caused byThe porphyrias are caused bydeficiencies of enzymes involved indeficiencies of enzymes involved inheme biosynthesis which lead toheme biosynthesis which lead toblockade of the porphyrin pathwayblockade of the porphyrin pathwayand subsequent accumulation ofand subsequent accumulation ofporphyrins and their precursors.porphyrins and their precursors.
IntroductionIntroductionSeven major types of porhyria areSeven major types of porhyria arenow recognized .now recognized .They include acute and non-acuteThey include acute and non-acuteforms.forms.
Classification of porphyriasClassification of porphyrias
in porphyria increased amount of uroporphyrinein porphyria increased amount of uroporphyrineor protoporphyrine are presnt inor protoporphyrine are presnt inurine.coproporphyrine also may be presnt ,ALLurine.coproporphyrine also may be presnt ,ALLARE DERIVED FROM PORPHOBILLINOGENARE DERIVED FROM PORPHOBILLINOGEN
Porphobilinogen is charcteristic of the diseses butPorphobilinogen is charcteristic of the diseses butits 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 beconverted into dark red colored porphobillinconverted into dark red colored porphobillinIn porphyrineuria coproporphyrine perdominate inIn porphyrineuria coproporphyrine perdominate inthe urine coproporphyrineuria iii may occur afterthe urine coproporphyrineuria iii may occur afterexposure to metal(lead poisoning is a prominentexposure to metal(lead poisoning is a prominentexample)sedative,sulonamides, alchol, andexample)sedative,sulonamides, alchol, andvarious organic substance ``in hypermetbolicvarious organic substance ``in hypermetbolicstates` with hepatic diseses in variousstates` with hepatic diseses in varioushaemtological condation and in plegra and andhaemtological condation and in plegra and andriboflavin deficency.riboflavin deficency.
in lead poisnoning the amount of coproporphyrinein lead poisnoning the amount of coproporphyrineiii 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 inerpretedwith cautionwith caution
Method for identification and quntitativeMethod for identification and quntitativeestimation of porphyins are based uponestimation of porphyins are based uponsolubility differnce in organic solventsolubility differnce in organic solventand chromatographic orand chromatographic orspectrophotometric anylysis.Qualitativespectrophotometric anylysis.Qualitativetesting of urine for porphobilnogen is atesting of urine for porphobilnogen is asimple proceduresimple procedure
Watson-schwartzWatson-schwartzporphobilnogen testporphobilnogen test
ReagentReagentEhrlich s ragentEhrlich s ragenta.paradimethy aminobenzaldehyde…..0.7ga.paradimethy aminobenzaldehyde…..0.7gb concentrated HCL……150mlb concentrated HCL……150mlc distelled water…..100mlc distelled water…..100ml
ProcedureProcedurethis test is based on differntialthis test is based on differntialextaction.Add 3 ml of Ehrilich reagent to 3 ml ofextaction.Add 3 ml of Ehrilich reagent to 3 ml ofurin and mix. Add 3ml chloroform shake for 2urin and mix. Add 3ml chloroform shake for 2mint and then let stand .persistence of reddishmint and then let stand .persistence of reddishcolor in the aqueous supernatant undicatecolor in the aqueous supernatant undicateporphobilinogen. MANYY SUBSTANCE CAUSE Aporphobilinogen. MANYY SUBSTANCE CAUSE AFALSE POSITIVE REACTION .NOTABLE AREFALSE POSITIVE REACTION .NOTABLE AREANTICONVULASANT DRUGSANTICONVULASANT DRUGSPYRIDIUM.UROROSEIN.OR INDOXYL..PYRIDIUM.UROROSEIN.OR INDOXYL..