Transcript Heamachromatosis - Department of Pathological Physiology

Haemochromatosis
Haeme- : ”Of Blood”
Chromo- : ”in connection to
genetics”
Subject: Current Health Problems in
Home Contries
Background: -Connection to syllaby
in Physiology
-Connection to syllaby in
Pathyphysiology
-Personal Predisposition
Charracteristics:
• Disease connected to the Iron homeostasis
• Caused by
– 1. Genetic predisposition
– 2. Anemia, Alcohol consumption
• Primary symptoms:
– Joint pain
– Lack of energy
– Abdominal pain
• Secondary symptoms:
–
–
–
–
Arthritis
Liver disease
Damage of Pancreas
Many more
• Distribution
– One of the most common genetic diseases in the U.S.
– 0,5% of Caucasion people of North Europen origin has the disease
Iron Overload
•Iron is taken up in the Small
intestine al crypts the
Enterocytes
•During physiological function
of the Iron Homestasis the
Enterocytes have a way of
monitoring the level of Iron in
the blood
•According to the Iron level,
the uptake rate is regulated
•In Heamochromatosis
however, there is an error
somewhere between the
Enterocyte and the
chemoreceptor sensing the
Iron concentration in the
blood
Iron in human
body
•In Erythorcytes
• Iron is an essential
part of hemoglobin
molecule
•In Plasma
•Iron is transported
troughput the body
connected to
Transferrin
•In Tissues
•Iron is stored in
various places in the
body, in conjugation to
the storage molucule
Ferritin
Iron in Patient with
Hemocromatosis
•Iron levels in the body are far above normal values
•In case of values of Ferritin exceeding 300 ng/L, the
condition is considered pathological and treatment
are needed
•When the Iron level are greatly increased the Iron is
stored in the form of Hemosiderin
•Most common places of hemosiderin depostis in
case of Hemocromatosis
•Liver
•Pancreas
•Boiny marrow
•Myocardium
•Can also been seen in
•Lungs
•Skin
•Brain
•Joints
Liver in Hemocromatosis
Hepatic tissue with Hemosiderin
deposit
Hemosiderin
depositis
Healthy liver
Genetics
Testing
Treatment
Sources and material