A
what are the type of cell injury (intoxication) caused by endogenous toxins?
A) genetic
B) Ionizing radiation
C) accumulation of metabolic by-products
What are types of Cell injury related to endogenous toxins that are caused by genetics issues?
- accumulation of normal metabolite
- activation of alternative pathway
what else can the the accumulation of PKU can contrubute?
Brain damage
what is an example of a disease cause by accumulation of metabolic by-products due to impairment of purine metabolism?
Gout
what occurs in gout?
results in accumulation of uric acid salts (usually in the form sodium urate) in the blood circulation (hyperuricemia) and tissues.
what are the type of hyperthermia cell injury?
-Fire
-Ionizing agent
-non-ionizing agent
-electric currents
which gouty arthritis condition is caused by the over production of sodium urates or a decrease of its elimination through the kidneys or both?
Hyperuricemia
what severe and dangerous condition can gout lead to?
Gouty Kidneys
what are some of the brain damage effect of PKU in children?
- severe mental retardation by 6 months of life
- about a third of these children are never able to walk
- two thirds of children cannot talk
- seizures
what are some characteristics sign of gouty arthritis?
The involved joint is red, swollen, hot
what is an example of accumulation of normal metabolite is a rare disease called "black urine"?
Alkaptonuria
what is the synovial inflammation accompanied by sever joint pain which can be amplified even by slight air movement cause by the effect of hyperuricemia known as?
Gouty arthritis
what are the 3 major clinical syndrome that characterize the course of gout?
- gouty arthritis
- formation of tophi
- gouty kidneys
what occurs when alkapton (homogentisic acid) accumulates in tissue?
Large amount of alkapton is excreted imparting a black color in the urine if allowed to stand and undergo oxidation.
what are the type of cell injury caused by trauma?
A) direct contact
B) hypothermia
C) hyperthermia
D)mechanical pressure
E)High intensity sound
F) microorganism
what effect doe the disease have on bones?
this disease leads to real destruction of the bones and joints
what are Tophi or tophus?
Tissue deposition of sodium urate into the tissue is known as tophus (in plural tophi)
what occurs in the body that leads to alkaptonuria?
The lack of the enzyme called homogentisic oxidase ( not being produced in the patient's body), blocks the metabolism of phenylalanine to tyrosine at the level of homogentisic acid(aka alkapton)
what are the 4 main type of electrical injuries?
- flash - is caused by an arc flash, usually results in superficial burns
- flame - occurs when an arc flash ignites an individual's clothing
- lightning - injuries, involving extremely short but very high voltage electrical energy
- true electrical injuries involve an individual becoming part of an electrical circuit
what is an example of hypothermia caused cell injury?
- frostbite
The temperature lower than 32° F leads to turning liquid into ice, which occupies the space larger than the one occupied by fluid, it is made of
In the accumulation of normal metabolite what is he pathways that disrupted?
Option 1: accumulation of normal metabolite
1. A substrate is converted into next intermediate product #1 under the influence of the Enzyme 1, normally producing in the body.
2. Then the intermediate product #1 is converted into the next intermediate product (#2 here) under the action of the Enzyme 2.
2. Then the intermediate product #2 has to be converted to the intermediate product #3 (or end product) by the action of the Enzyme 3.
3. However, the last one is not produced in the body due to genetic defect.
4.As a result, this intermediate product #2 being unable to be converted into the intermediate product #3, accumulates in the tissue in high concentration
If normal concentrations is non-toxic, the higher one becomes toxic.
In the case of hyperuricemia which cell in the body try to eliminate the crystal and what is the outcome?
special cells, known as phagocytic cells (whose function is the elimination of anything what is considered non-self from the body) try to engulf and digest the crystals. And, thus, to eliminate from the body.
However, these crystals are not made of organic material, and cannot be digested
the crystals being sharp, destroy the phagocytic cells, resulting in release of their lysosomal enzymes directly into the joint space
In the activation of the alternative pathway what is disrupted?
Option 2: activation of alternative pathway
1. The same consequences of events: a substrate is converted into next intermediate product #1 under the influence of the Enzyme 1, normally producing in the body.
2.Then the intermediate product #1 is converted into the next intermediate product (#2 here) under the action of the Enzyme 2.
3. Then the intermediate product #2 has to be converted to the intermediate product #3 (or end product) by the action of the Enzyme 3.
3. However, the last one is not produced in the body due to genetic defect.
4.As a result, this intermediate product #2, instead of being accumulated, is looking for another, alternative, pathway of metabolism.
This new pathway could lead to production of toxic products.
in severe secondary osteoarthritis caused by ochronosis what can be seen?
-deposits of the black pigment (alkapton) into the cartilage of the femur head, deformity of the spine
- deposition of calcium salts into the intervertebral discs (pay attention to the radiopacity of the discs, which normally are radiolucent)
What are some signs of PKU in early children?
Mental deficiency, light hair and skin (children of darker heritage may be red-haired or even brunet but lighter than normal)
-eczematoid rash
-motor performance habits such as biting arm
-banging head on the floor.
-But relatively normal physical development