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All porphyrias are semi-treatable.

Homa Homayoni on Protoporphyrin IX (PpIX)-conjugated self-lighting nanoparticles for photodynamic therapy: synthesis and characterization, part of a collection of online lectures.">

Suspect porphyria when appropriate,order the right tests,and collect the specimens properly.

Of these compounds, B12H11NH22−‐conjugated porphyrin induced a significant increase in the level of boron accumulation and PDT efficacy against HeLa cells.

DO NOT go back and memorize the porphyrin section of Stryer!

Performance of an MTT assay with each derivative revealed that the synthesized boronated porphyrins showed low cytotoxicities in a variety of cancer cells.

Of these compounds, B12H11NH22−‐conjugated porphyrin induced a significant increase in the level of boron accumulation and PDT efficacy against HeLa cells.">

Porphyria in children:Mayo Clin.


The diagram is simplistic -- PCT can show the perivascular PAS-positive stuff, and of courseerythropoietic protoporphyria can blister.ACQUIRED PORPHYRIAS AND PSEUDOPORPHYRIAS

But again, you shouldn't have biopsied in the first place.)A small minority of proroporphyria patientsget liver damage (sometimes even cirrhosis) from porphyrin crystals that accumulatein the hepatic parenchyma.

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MECHANISMS AND SYMPTOMATOLOGY IN THE PORPHYRIAS porphyria

More about "MCS", later, along with why almost no one in scientific medicinebelieves in it any more, and how its proponents operate. PORPHYRIA CUTANEA TARDA (familiar as "PCT"; Medicine 89: 69, 2010)

ACUTE INTERMITTENT PORPHYRIA (AIP -- see Neurology48: 1678, 1997)

Gung-ho clinicians now aspirateduodenal bile or cannulate the common bile duct to measure bile porphyrins(* protoporphyrin is soluble in bile but not in urine), establishing thediagnosis between attacks (NEJM 324: 1432, 1991, works great).

suggests porphyria as the cause of Vincent VanGogh's craziness (Br.


PDT works by direct injury to the target cells and tissues. While all of the exact mechanisms are not fully known yet, the basic pathway seems to involve an activated oxygen molecule that has the ability to injure or destroy nearby or specific cells.
Aminolevulinic acid is then incorporated into the body’s natural heme ( blood) biosynthesis pathway and activated to form protoporphyrin IX, a potent photosensitier. Protoporphyrin IX then becomes excited to an activated singlet state. This active singlet state is then directly toxic to cells.
Other potential pathways include directly killing abnormal or cancerous cells, damaging the blood vessels and blood supply to the tissue, causing inflammation and irritation, and possibly also activating the person’s own immune system to attack the abnormal or cancerous cells.

1: 7, 1968;case against porphyria J.

Cool finding: Because the urine is loaded with uroporphyrin,the urine is likely to be reddish or brownish, and ifyou have a fluorescent light handy, it will glow pink.

Uroporphyrinogen is elevatedin the urine, enabling the diagnosis.

* Dividing porphyrias into "hepatic" ("caused by accumulation of precursors of the P450 cytochrome") and "erythropoietic" ("caused by accumulation of precursors of hemoglobin") is becoming unfashionable.

(ERYTHROPOIETIC) PROTOPORPHYRIA

Mild versions are surely common and are missed by thestandard enzyme "normal ranges".* This unit will focus on what a general physician needs to know about the porphyrias.

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