Nisa Celik
Porphyria
Porphyrias are a group of at least eight related disorders that primarily
affect the skin or the nervous system, and sometimes the liver and other
organs.Names of the eight types of porphyrias are:
1.Delta-aminolevulinate-dehydratase deficiency porphyria
2.Acute intermittent porphyria
3.Hereditary porphyria
4.Variegate porphyria
5.Congenital erythropoietic porphyria
6.Porphyria cutanea tarda
7.Hepatoerythropoietic porphyria
8.Erythropoietic protoporphyria
Porphyria disease occurs when hemoglobin in the blood cannot be
synthesized properly. Porphyria occurs when hemoglobin in the blood
cannot be synthesized properly. Each type of porphyria is caused by low
levels of a particular enzyme needed during heme production. Heme is part
of the hemoglobin in the blood. To produce heme, your body must convert
aminolevulinic acid (ALA) and porphobilinogen (PBG), known as porphyrin
precursors, into more complex substances called porphyrins. These are
then converted from one type of porphyry to another ro form heme.
Hemoglobin transports oxygen to body tissues and gives color to red blood
cells. Heme is part of the proteins in the liver that help your liver work
properly. Porphyrin and porphyrin precursors accumulate in the liver, skin
and other bodily tissues due to low levels of any of the enzymes needed to
make heme. Most forms of porphyria are caused by a gene change passed
down from one or both parents. Inherited metabolic disorders of heme
synthesis lead to porphyrin accumulation. However Porphyria cutanea
tarda (PCT) usually is not passed down in families. In PCT, certain triggers
can cause a buildup of porphyrins that may cause symptoms. Triggers
could be smoking, heavy alcohol use and too much iron in the body.
Porphyria is a disease that affects the skin and nervous system. According
to the porphyria type, it is divided into two as acute porphyrias and
, cutaneous porphyrias. Acute porphyria affects the nervous system.
Abdominal pain, vomiting, fever. ALA and PBG excretion in the urine cause
neuropsychiatric disorders at high rates. Acute porphyrias can be
life-threatening if an attack isn’t promptly treated. During the attack, a
person will experience breathing problems or high blood pressure.
Treatment of Acute porphyria intravenously heme or glucose infusions.
These treatments reduce the number of porphyrins or porphyrin precursors
produced in the liver.
Cutaneous porphyrias, on the other hand, especially affects the skin,
causing water-filled blisters to form on the skin and gums. And skin blisters
can become infected. When your skin heals after symptoms, it may have a
changed appearance and coloring and leave scars. It creates sensitivity to
sunlight. Avoid exposure to sunlight to prevent disease. Also cutaneous
porphyrias increase the risk of liver damage or liver cancer.
Porphyria
Porphyrias are a group of at least eight related disorders that primarily
affect the skin or the nervous system, and sometimes the liver and other
organs.Names of the eight types of porphyrias are:
1.Delta-aminolevulinate-dehydratase deficiency porphyria
2.Acute intermittent porphyria
3.Hereditary porphyria
4.Variegate porphyria
5.Congenital erythropoietic porphyria
6.Porphyria cutanea tarda
7.Hepatoerythropoietic porphyria
8.Erythropoietic protoporphyria
Porphyria disease occurs when hemoglobin in the blood cannot be
synthesized properly. Porphyria occurs when hemoglobin in the blood
cannot be synthesized properly. Each type of porphyria is caused by low
levels of a particular enzyme needed during heme production. Heme is part
of the hemoglobin in the blood. To produce heme, your body must convert
aminolevulinic acid (ALA) and porphobilinogen (PBG), known as porphyrin
precursors, into more complex substances called porphyrins. These are
then converted from one type of porphyry to another ro form heme.
Hemoglobin transports oxygen to body tissues and gives color to red blood
cells. Heme is part of the proteins in the liver that help your liver work
properly. Porphyrin and porphyrin precursors accumulate in the liver, skin
and other bodily tissues due to low levels of any of the enzymes needed to
make heme. Most forms of porphyria are caused by a gene change passed
down from one or both parents. Inherited metabolic disorders of heme
synthesis lead to porphyrin accumulation. However Porphyria cutanea
tarda (PCT) usually is not passed down in families. In PCT, certain triggers
can cause a buildup of porphyrins that may cause symptoms. Triggers
could be smoking, heavy alcohol use and too much iron in the body.
Porphyria is a disease that affects the skin and nervous system. According
to the porphyria type, it is divided into two as acute porphyrias and
, cutaneous porphyrias. Acute porphyria affects the nervous system.
Abdominal pain, vomiting, fever. ALA and PBG excretion in the urine cause
neuropsychiatric disorders at high rates. Acute porphyrias can be
life-threatening if an attack isn’t promptly treated. During the attack, a
person will experience breathing problems or high blood pressure.
Treatment of Acute porphyria intravenously heme or glucose infusions.
These treatments reduce the number of porphyrins or porphyrin precursors
produced in the liver.
Cutaneous porphyrias, on the other hand, especially affects the skin,
causing water-filled blisters to form on the skin and gums. And skin blisters
can become infected. When your skin heals after symptoms, it may have a
changed appearance and coloring and leave scars. It creates sensitivity to
sunlight. Avoid exposure to sunlight to prevent disease. Also cutaneous
porphyrias increase the risk of liver damage or liver cancer.
