Juvenile hereditary haemochromatosis, Type 2 (Q97541): Difference between revisions
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A hemocromatose tipo 2 (juvenil) é a forma precoce e mais grave de hemocromatose hereditária rara, um grupo de doenças de origem genética caracterizadas por deposição excessiva de ferro nos tecidos. Esta forma juvenil de hemocromatose tem as características clássicas da HH, mas também é caracterizada por cardiomiopatia grave e hipogonadismo. Artropatia, fibrose hepática, intolerância à glicose e aumento da pigmentação da pele são frequentes. | |||||||||||||||
| description / en | description / en | ||||||||||||||
Hemochromatosis type 2 (juvenile) is the early-onset and most severe form of rare hereditary hemochromatosis, a group of diseases characterised by excessive tissue iron deposition of genetic origin. This juvenile form of hemochromatosis has the classical features of HH but is also characterised by severe cardiomyopathy and hypogonadism. Arthropathy, hepatic fibrosis, glucose intolerance, and increased skin pigmentation are frequent. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/303352828 / rank | |||||||||||||||
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CID11:ID_303352828 | |||||||||||||||
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dki-india-ID_303352828 | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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15 August 2026
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Latest revision as of 13:39, 16 August 2026
Hemochromatosis type 2 (juvenile) is the early-onset and most severe form of rare hereditary hemochromatosis, a group of diseases characterised by excessive tissue iron deposition of genetic origin. This juvenile form of hemochromatosis has the classical features of HH but is also characterised by severe cardiomyopathy and hypogonadism. Arthropathy, hepatic fibrosis, glucose intolerance, and increased skin pigmentation are frequent.
| Language | Label | Description | Also known as |
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| default for all languages | ID_303352828 |
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| English | Juvenile hereditary haemochromatosis, Type 2 |
Hemochromatosis type 2 (juvenile) is the early-onset and most severe form of rare hereditary hemochromatosis, a group of diseases characterised by excessive tissue iron deposition of genetic origin. This juvenile form of hemochromatosis has the classical features of HH but is also characterised by severe cardiomyopathy and hypogonadism. Arthropathy, hepatic fibrosis, glucose intolerance, and increased skin pigmentation are frequent. |
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CID11:ID_303352828
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dki-india-ID_303352828
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Concluído
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15 August 2026
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