Hemochromatosis natural history, complications and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
Hemochromatosis is due to unchecked transfer of iron into the bloodstream in the absence of increased erythropoietic needs and its toxic effects in parenchymatous organs.The features of Hemochromatosis are due to presence of toxic iron in pro-oxidant form in surroundings of parenchymatous tissue cells of the liver and other organs, where it can cause oxidative damage and lead to cirrhosis, hypogonadism, diabetes, cardiomyopathy, arthropathy, and skin pigmentation. | |||
==Complications== | ==Complications== | ||
End-organ damage | End-organ damage | ||
Iron is stored in the liver, | Iron is stored in the liver, gonads, joints, brain, pancreas and the heart. | ||
'''[[Cirrhosis]]''': Permanent scarring of the [[liver]]. Along with other maladies like long-term alcoholism, haemochromatosis may have an adverse effect on the liver. The liver is a primary storage area for iron and will naturally accumulate excess iron. Over time the liver is likely to be damaged by iron overload. Cirrhosis itself may lead to additional and more serious complications, including bleeding from dilated veins in the [[esophagus]] and [[stomach]] ([[varices]]) and severe fluid retention in the [[abdomen]] ([[ascites]]). Toxins may accumulate in the blood and eventually affect mental functioning. This can lead to confusion or even [[coma]] (hepatic [[encephalopathy]]). | * Long term effects of haemochromatosis on these organs can be very serious, even fatal when untreated.<ref>[http://www.mayoclinic.com/health/hemochromatosis/DS00455/DSECTION=6 Haemochromatosis Complications]</ref> | ||
* '''[[Cirrhosis]]''': Permanent scarring of the [[liver]]. Along with other maladies like long-term alcoholism, haemochromatosis may have an adverse effect on the liver. The liver is a primary storage area for iron and will naturally accumulate excess iron. Over time the liver is likely to be damaged by iron overload. Cirrhosis itself may lead to additional and more serious complications, including bleeding from dilated veins in the [[esophagus]] and [[stomach]] ([[varices]]) and severe fluid retention in the [[abdomen]] ([[ascites]]). Toxins may accumulate in the blood and eventually affect mental functioning. This can lead to confusion or even [[coma]] (hepatic [[encephalopathy]]). | |||
'''[[Liver]] [[cancer]]''': Cirrhosis and haemochromatosis together will increase the risk of liver cancer. (Nearly one-third of people with haemochromatosis and cirrhosis eventually develop liver cancer.) | * '''[[Liver]] [[cancer]]''': Cirrhosis and haemochromatosis together will increase the risk of liver cancer. (Nearly one-third of people with haemochromatosis and cirrhosis eventually develop liver cancer.) | ||
'''[[Diabetes]]''': The [[pancreas]] which also stores iron is very important in the body’s mechanisms for sugar [[metabolism]]. Diabetes affects the way the body uses blood sugar ([[glucose]]). Diabetes is in turn the leading cause of new blindness in adults and may be involved in [[kidney failure]] and [[cardiovascular disease]]. | * '''[[Diabetes]]''': The [[pancreas]] which also stores iron is very important in the body’s mechanisms for sugar [[metabolism]]. Diabetes affects the way the body uses blood sugar ([[glucose]]). Diabetes is in turn the leading cause of new blindness in adults and may be involved in [[kidney failure]] and [[cardiovascular disease]]. | ||
'''[[Congestive heart failure]]''': If excess iron in the heart interferes with the its ability to circulate enough blood, a number of problems can occur including death. The condition may be reversible when haemochromatosis is treated and excess iron stores reduced. | * '''[[Congestive heart failure]]''': If excess iron in the heart interferes with the its ability to circulate enough blood, a number of problems can occur including death. The condition may be reversible when haemochromatosis is treated and excess iron stores reduced. | ||
'''[[Heart]] [[arrhythmias]]''': Arrhythmia or abnormal heart rhythms can cause heart palpitations, [[chest pain]] and light-headedness and are occasionally life threatening. This condition can often be reversed with treatment for haemochromatosis. | * '''[[Heart]] [[arrhythmias]]''': Arrhythmia or abnormal heart rhythms can cause heart palpitations, [[chest pain]] and light-headedness and are occasionally life threatening. This condition can often be reversed with treatment for haemochromatosis. | ||
'''Pigment changes''': Deposits of iron in skin cells can turn skin a bronze or gray color. | * '''Pigment changes''': Deposits of iron in skin cells can turn skin a bronze or gray color. | ||
; An increased susceptibility to certain [[infectious disease]]s caused by siderophilic microoganisms: | ;* An increased susceptibility to certain [[infectious disease]]s caused by siderophilic microoganisms | ||
: | |||
* ''[[Vibrio vulnificus]]'' infections from eating seafood | * ''[[Vibrio vulnificus]]'' infections from eating seafood | ||
* ''[[Listeria monocytogenes]]'' | * ''[[Listeria monocytogenes]]'' | ||
Revision as of 22:05, 3 December 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Natural History
Hemochromatosis is due to unchecked transfer of iron into the bloodstream in the absence of increased erythropoietic needs and its toxic effects in parenchymatous organs.The features of Hemochromatosis are due to presence of toxic iron in pro-oxidant form in surroundings of parenchymatous tissue cells of the liver and other organs, where it can cause oxidative damage and lead to cirrhosis, hypogonadism, diabetes, cardiomyopathy, arthropathy, and skin pigmentation.
Complications
End-organ damage Iron is stored in the liver, gonads, joints, brain, pancreas and the heart.
- Long term effects of haemochromatosis on these organs can be very serious, even fatal when untreated.[1]
- Cirrhosis: Permanent scarring of the liver. Along with other maladies like long-term alcoholism, haemochromatosis may have an adverse effect on the liver. The liver is a primary storage area for iron and will naturally accumulate excess iron. Over time the liver is likely to be damaged by iron overload. Cirrhosis itself may lead to additional and more serious complications, including bleeding from dilated veins in the esophagus and stomach (varices) and severe fluid retention in the abdomen (ascites). Toxins may accumulate in the blood and eventually affect mental functioning. This can lead to confusion or even coma (hepatic encephalopathy).
- Liver cancer: Cirrhosis and haemochromatosis together will increase the risk of liver cancer. (Nearly one-third of people with haemochromatosis and cirrhosis eventually develop liver cancer.)
- Diabetes: The pancreas which also stores iron is very important in the body’s mechanisms for sugar metabolism. Diabetes affects the way the body uses blood sugar (glucose). Diabetes is in turn the leading cause of new blindness in adults and may be involved in kidney failure and cardiovascular disease.
- Congestive heart failure: If excess iron in the heart interferes with the its ability to circulate enough blood, a number of problems can occur including death. The condition may be reversible when haemochromatosis is treated and excess iron stores reduced.
- Heart arrhythmias: Arrhythmia or abnormal heart rhythms can cause heart palpitations, chest pain and light-headedness and are occasionally life threatening. This condition can often be reversed with treatment for haemochromatosis.
- Pigment changes: Deposits of iron in skin cells can turn skin a bronze or gray color.
- An increased susceptibility to certain infectious diseases caused by siderophilic microoganisms
- Vibrio vulnificus infections from eating seafood
- Listeria monocytogenes
- Yersinia enterocolica
- Salmonella enteritidis (serotype Typhymurium)
- Klebsiella pneumoniae
- Escherichia coli
- Rhizopus arrhizus
- Mucor species