Johar ali Johar ali

Trainee tech.Trainee tech.

Porpyria

PorphyriaPorphyriaThe porphyrias are caused by The porphyrias are caused by deficiencies of enzymes involved in deficiencies of enzymes involved in heme biosynthesis which lead to heme biosynthesis which lead to blockade of the porphyrin pathway blockade of the porphyrin pathway and subsequent accumulation of and subsequent accumulation of porphyrins and their precursors.porphyrins and their precursors.

IntroductionIntroduction

Seven major types of porhyria are Seven major types of porhyria are now recognized .now recognized .

They include acute and non-acute They include acute and non-acute forms. forms.

Classification of porphyriasClassification of porphyrias

in porphyria increased amount of uroporphyrine in porphyria increased amount of uroporphyrine or protoporphyrine are presnt in or protoporphyrine are presnt in urine.coproporphyrine also may be presnt ,ALL urine.coproporphyrine also may be presnt ,ALL ARE DERIVED FROM PORPHOBILLINOGENARE DERIVED FROM PORPHOBILLINOGEN

Porphobilinogen is charcteristic of the diseses Porphobilinogen is charcteristic of the diseses but its identification in the urine is difficult. but its identification in the urine is difficult. Porphobilinogen in urin is exposed to air will be Porphobilinogen in urin is exposed to air will be converted into dark red colored porphobillinconverted into dark red colored porphobillin

In porphyrineuria coproporphyrine perdominate in In porphyrineuria coproporphyrine perdominate in the urine coproporphyrineuria iii may occur after the urine coproporphyrineuria iii may occur after exposure to metal(lead poisoning is a prominent exposure to metal(lead poisoning is a prominent example)sedative,sulonamides, alchol, and example)sedative,sulonamides, alchol, and various organic substance ``in hypermetbolic various organic substance ``in hypermetbolic states` with hepatic diseses in various states` with hepatic diseses in various haemtological condation and in plegra and and haemtological condation and in plegra and and riboflavin deficency.riboflavin deficency.

in lead poisnoning the amount of coproporphyrine in lead poisnoning the amount of coproporphyrine iii excreted in the urine is markedly iii excreted in the urine is markedly increased .small or moderate increase are to be increased .small or moderate increase are to be inerpreted with caution inerpreted with caution

Method for identification and quntitative Method for identification and quntitative estimation of porphyins are based upon estimation of porphyins are based upon

solubility differnce in organic solvent solubility differnce in organic solvent and chromatographic or and chromatographic or

spectrophotometric anylysis.Qualitative spectrophotometric anylysis.Qualitative testing of urine for porphobilnogen is a testing of urine for porphobilnogen is a

simple proceduresimple procedure

Watson-schwartz Watson-schwartz porphobilnogen testporphobilnogen test

Reagent Reagent

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

ProcedureProcedure this test is based on differntial this test is based on differntial extaction.Add 3 ml of Ehrilich reagent to 3 ml of extaction.Add 3 ml of Ehrilich reagent to 3 ml of urin and mix. Add 3ml chloroform shake for 2 urin and mix. Add 3ml chloroform shake for 2 mint and then let stand .persistence of reddish mint and then let stand .persistence of reddish color in the aqueous supernatant undicate color in the aqueous supernatant undicate porphobilinogen. MANYY SUBSTANCE CAUSE A porphobilinogen. MANYY SUBSTANCE CAUSE A FALSE POSITIVE REACTION .NOTABLE ARE FALSE POSITIVE REACTION .NOTABLE ARE ANTICONVULASANT DRUGS ANTICONVULASANT DRUGS PYRIDIUM.UROROSEIN.OR INDOXYL..PYRIDIUM.UROROSEIN.OR INDOXYL..

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