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In many cases, propionic acidemia can damage the brain, heart, kidney, liver, cause seizures and delays to normal development such as walking or talking. The accumulation of propionic acid is known to induce differential responses in different organs. The heart and liver are specific targets of the complication.
The increased levels of glutamine lead to an increase in osmotic pressure in the astrocytes, which become swollen. There is increased activity of the inhibitory γ-aminobutyric acid (GABA) system and the energy supply to other brain cells is decreased. This can be thought of as an example of brain edema of the "cytotoxic" type. [14]
Documented cases of Reye syndrome in adults are rare. The recovery of adults with the syndrome is generally complete, with liver and brain function returning to normal within two weeks of onset. [citation needed] In children, mild to moderate to severe permanent brain damage is possible, especially in infants.
Injury to hepatocyte and bile duct cells lead to accumulation of bile acid inside the liver. This promotes further liver damage. [38] Non-parenchymal cells such as Kupffer cells, collagen-producing stellate cells, and leukocytes (i.e. neutrophil and monocyte) also have a role in the mechanism.
Long-chain fatty acids cannot cross the blood–brain barrier, but the liver can break these down to produce ketone bodies. However, short-chain fatty acids (e.g., butyric acid, propionic acid, and acetic acid) and the medium-chain fatty acids, octanoic acid and heptanoic acid, can cross the blood–brain barrier and be metabolised by brain cells.
For these purposes, hepatocytes are usually isolated from animal or human [8] whole liver or liver tissue by collagenase digestion, which is a two-step process. In the first step, the liver is placed in an isotonic solution, in which calcium is removed to disrupt cell-cell tight junctions by the use of a calcium chelating agent.
Lead encephalopathy is a medical emergency and causes permanent brain damage in 70–80% of children affected by it, even those that receive the best treatment. [32] The mortality rate for people who develop cerebral involvement is about 25%, and of those who survive who had lead encephalopathy symptoms by the time chelation therapy was begun ...
Haemopoiesis - The formation of blood cells is called haemopoiesis. In the embryonic stage, RBCs and WBCs are formed by liver. In the first trimester fetus, the liver is the main site of red blood cell production. By the 32nd week of gestation, the bone marrow has almost completely taken over that task. [56] The liver helps in the purification ...