Hereditary late-onset lymphedema.
Identifieur interne : 006650 ( PubMed/Checkpoint ); précédent : 006649; suivant : 006651Hereditary late-onset lymphedema.
Auteurs : L B Holmes ; J P Fields ; J B ZabriskieSource :
- Pediatrics [ 0031-4005 ] ; 1978.
Descripteurs français
- KwdFr :
- Aberrations des chromosomes (génétique), Adulte, Benzathine benzylpénicilline (usage thérapeutique), Canal vertébral (imagerie diagnostique), Cils (malformations), Enfant, Facteurs temps, Femelle, Gènes dominants, Humains, Infections à streptocoques (génétique), Jambe, Lymphangite (), Lymphangite (génétique), Lymphoedème (), Lymphoedème (génétique), Maladies chromosomiques, Mâle, Radiographie, Récidive, Système lymphatique (malformations).
- MESH :
- génétique : Aberrations des chromosomes, Infections à streptocoques, Lymphangite, Lymphoedème.
- imagerie diagnostique : Canal vertébral.
- malformations : Cils, Système lymphatique.
- usage thérapeutique : Benzathine benzylpénicilline.
- Adulte, Enfant, Facteurs temps, Femelle, Gènes dominants, Humains, Jambe, Lymphangite, Lymphoedème, Maladies chromosomiques, Mâle, Radiographie, Récidive.
English descriptors
- KwdEn :
- Adult, Child, Chromosome Aberrations (genetics), Chromosome Disorders, Eyelashes (abnormalities), Female, Genes, Dominant, Humans, Leg, Lymphangitis (genetics), Lymphangitis (prevention & control), Lymphatic System (abnormalities), Lymphedema (genetics), Lymphedema (prevention & control), Male, Penicillin G Benzathine (therapeutic use), Radiography, Recurrence, Spinal Canal (diagnostic imaging), Streptococcal Infections (genetics), Time Factors.
- MESH :
- chemical , therapeutic use : Penicillin G Benzathine.
- abnormalities : Eyelashes, Lymphatic System.
- diagnostic imaging : Spinal Canal.
- genetics : Chromosome Aberrations, Lymphangitis, Lymphedema, Streptococcal Infections.
- prevention & control : Lymphangitis, Lymphedema.
- Adult, Child, Chromosome Disorders, Female, Genes, Dominant, Humans, Leg, Male, Radiography, Recurrence, Time Factors.
Abstract
Two different types of hereditary late-onset lymphedema are presented. In one family the father and one son had recurrent streptococcal lymphangitis beginning in childhood. In the son there was lymphatic hypoplasia in both legs with the infection having only occurred in one. Prophylaxis with penicillin prevented the recurrent lymphangitis. Because of 30 years of untreated lymphangitis, the father has chronic severe lymphedema. The second type, lymphedema associated with extra eyelashes (distichiasis) and a wide spinal canal, occurred in a woman whose lymphedema began at age 12 but in whom the hereditary nature of the disorder was not recognized until she was 29. Both of these types of late-onset lymphedema, lymphedema with lymphangitis and lymphedema with distichiasis, are due to autosomal dominant genes. Both families would have benefited from early diagnosis of the cause of the lymphedema.
PubMed: 662482
Affiliations:
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pubmed:662482Le document en format XML
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<author><name sortKey="Fields, J P" sort="Fields, J P" uniqKey="Fields J" first="J P" last="Fields">J P Fields</name>
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<author><name sortKey="Zabriskie, J B" sort="Zabriskie, J B" uniqKey="Zabriskie J" first="J B" last="Zabriskie">J B Zabriskie</name>
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<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Chromosome Aberrations</term>
<term>Lymphangitis</term>
<term>Lymphedema</term>
<term>Streptococcal Infections</term>
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<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Aberrations des chromosomes</term>
<term>Infections à streptocoques</term>
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<term>Lymphoedème</term>
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<term>Chromosome Disorders</term>
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<term>Time Factors</term>
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<term>Facteurs temps</term>
<term>Femelle</term>
<term>Gènes dominants</term>
<term>Humains</term>
<term>Jambe</term>
<term>Lymphangite</term>
<term>Lymphoedème</term>
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<front><div type="abstract" xml:lang="en">Two different types of hereditary late-onset lymphedema are presented. In one family the father and one son had recurrent streptococcal lymphangitis beginning in childhood. In the son there was lymphatic hypoplasia in both legs with the infection having only occurred in one. Prophylaxis with penicillin prevented the recurrent lymphangitis. Because of 30 years of untreated lymphangitis, the father has chronic severe lymphedema. The second type, lymphedema associated with extra eyelashes (distichiasis) and a wide spinal canal, occurred in a woman whose lymphedema began at age 12 but in whom the hereditary nature of the disorder was not recognized until she was 29. Both of these types of late-onset lymphedema, lymphedema with lymphangitis and lymphedema with distichiasis, are due to autosomal dominant genes. Both families would have benefited from early diagnosis of the cause of the lymphedema.</div>
</front>
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<Abstract><AbstractText>Two different types of hereditary late-onset lymphedema are presented. In one family the father and one son had recurrent streptococcal lymphangitis beginning in childhood. In the son there was lymphatic hypoplasia in both legs with the infection having only occurred in one. Prophylaxis with penicillin prevented the recurrent lymphangitis. Because of 30 years of untreated lymphangitis, the father has chronic severe lymphedema. The second type, lymphedema associated with extra eyelashes (distichiasis) and a wide spinal canal, occurred in a woman whose lymphedema began at age 12 but in whom the hereditary nature of the disorder was not recognized until she was 29. Both of these types of late-onset lymphedema, lymphedema with lymphangitis and lymphedema with distichiasis, are due to autosomal dominant genes. Both families would have benefited from early diagnosis of the cause of the lymphedema.</AbstractText>
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