Hemochromatosis natural history, complications and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Hemochromatosis}} | {{Hemochromatosis}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}}{{SKA}} | ||
==Overview== | ==Overview== | ||
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. | |||
==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. | 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.<ref name="pmid3799437">{{cite journal| author=Rivers J, Garrahy P, Robinson W, Murphy A| title=Reversible cardiac dysfunction in hemochromatosis. | journal=Am Heart J | year= 1987 | volume= 113 | issue= 1 | pages= 216-7 | pmid=3799437 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3799437 }}</ref> | ||
==Complications== | ==Complications== | ||
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* '''[[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.<ref name="pmid8022042">{{cite journal| author=Miller M, Hutchins GM| title=Hemochromatosis, multiorgan hemosiderosis, and coronary artery disease. | journal=JAMA | year= 1994 | volume= 272 | issue= 3 | pages= 231-3 | pmid=8022042 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8022042 }}</ref> | * '''[[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.<ref name="pmid8022042">{{cite journal| author=Miller M, Hutchins GM| title=Hemochromatosis, multiorgan hemosiderosis, and coronary artery disease. | journal=JAMA | year= 1994 | volume= 272 | issue= 3 | pages= 231-3 | pmid=8022042 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8022042 }}</ref> | ||
* '''[[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.<ref name="pmid8035843">{{cite journal| author=| title=Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-1994. A 25-year-old man with the recent onset of diabetes mellitus and congestive heart failure. | journal=N Engl J Med | year= 1994 | volume= 331 | issue= 7 | pages= 460-6 | pmid=8035843 | doi=10.1056/NEJM199408183310708 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8035843 }}</ref> | ||
* '''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. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sunny Kumar MD [2]
Overview
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.
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.[1]
Complications
End-organ damage Iron is stored in the liver, gonads, joints, brain, pancreas and the heart.[2]
- Long term effects of haemochromatosis on these organs can be very serious, even fatal when untreated.[3]
- 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.[4]
- 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.[5]
- Pigment changes: Deposits of iron in skin cells can turn skin a bronze or gray color.
- Hypothyroidism: Due to deposition of pro-oxidant iron in thyroid tissue and damages it level that it is not able to produce thyroid hormone[6]
- Hypogonadism: Due to damage to pituitary gland.[7]
- Hypopitutarisum:Due to deposition of pro-oxidant iron in pituitary gland.[8]
- 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
Prognosis
References
- ↑ Rivers J, Garrahy P, Robinson W, Murphy A (1987). "Reversible cardiac dysfunction in hemochromatosis". Am Heart J. 113 (1): 216–7. PMID 3799437.
- ↑ Hsing AW, McLaughlin JK, Olsen JH, Mellemkjar L, Wacholder S, Fraumeni JF (1995). "Cancer risk following primary hemochromatosis: a population-based cohort study in Denmark". Int J Cancer. 60 (2): 160–2. PMID 7829208.
- ↑ Haemochromatosis Complications
- ↑ Miller M, Hutchins GM (1994). "Hemochromatosis, multiorgan hemosiderosis, and coronary artery disease". JAMA. 272 (3): 231–3. PMID 8022042.
- ↑ "Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-1994. A 25-year-old man with the recent onset of diabetes mellitus and congestive heart failure". N Engl J Med. 331 (7): 460–6. 1994. doi:10.1056/NEJM199408183310708. PMID 8035843.
- ↑ Walton C, Kelly WF, Laing I, Bu'lock DE (1983). "Endocrine abnormalities in idiopathic haemochromatosis". Q J Med. 52 (205): 99–110. PMID 6683854.
- ↑ Kelly TM, Edwards CQ, Meikle AW, Kushner JP (1984). "Hypogonadism in hemochromatosis: reversal with iron depletion". Ann Intern Med. 101 (5): 629–32. PMID 6435491.
- ↑ Fujisawa I, Morikawa M, Nakano Y, Konishi J (1988). "Hemochromatosis of the pituitary gland: MR imaging". Radiology. 168 (1): 213–4. doi:10.1148/radiology.168.1.3380960. PMID 3380960.