Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family
A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinic...
Gespeichert in:
| Veröffentlicht in: | Pediatrics (Evanston) 1983-01, Vol.71 (1), p.81-87 |
|---|---|
| Hauptverfasser: | , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 87 |
|---|---|
| container_issue | 1 |
| container_start_page | 81 |
| container_title | Pediatrics (Evanston) |
| container_volume | 71 |
| creator | Roord, J J Daha, M Kuis, W Verbrugh, H A Verhoef, J Zegers, B J Stoop, J W |
| description | A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. Biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis. |
| doi_str_mv | 10.1542/peds.71.1.81 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_80351346</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>80351346</sourcerecordid><originalsourceid>FETCH-LOGICAL-c232t-ebc3db48e59af059155eae0f22944dc875a47a873163f75c06a92ce24d6a3be33</originalsourceid><addsrcrecordid>eNqFkTtv3DAQhAkjgX1-dGkNsEplXfiUqDKwndiAgTRJTfDIpY-BRCokleR-kf-mJfiQNsVisTvfTDMIfaBkS6VgnyZwZdvRLd0qeoI2lPSqEayT79CGEE4bQYg8Q-el_CSECNmxU3TaKqF6xTfo5THuIYcKDjvwwQaI9oCTx3UPy4TssE3jlCLEur7XY4BxvUwpyQazWv-EuscZ7JzzqkyH9AwxWByiB1tDiuUG25DtPJga4jMO4zhHOIb9hUU10eHfpixEqKEsRmzw3VztHnszhuFwid57MxS4Ou4L9OPL_ffbh-bp29fH289PjWWc1QZ2lrudUCB744nsqZRggHjGeiGcVZ00ojOq47TlvpOWtKZnFphwreE74PwCfXzLnXL6NUOpegzFwjCYCGkuWhEuKRftf0HKZa96Jhbw5g20OZWSwesph9Hkg6ZErwXqtUDdUU21ogt-fcyddyO4f_CxMf4KWUObpQ</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>13598924</pqid></control><display><type>article</type><title>Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family</title><source>MEDLINE</source><source>EZB-FREE-00999 freely available EZB journals</source><creator>Roord, J J ; Daha, M ; Kuis, W ; Verbrugh, H A ; Verhoef, J ; Zegers, B J ; Stoop, J W</creator><creatorcontrib>Roord, J J ; Daha, M ; Kuis, W ; Verbrugh, H A ; Verhoef, J ; Zegers, B J ; Stoop, J W</creatorcontrib><description>A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. Biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.</description><identifier>ISSN: 0031-4005</identifier><identifier>EISSN: 1098-4275</identifier><identifier>DOI: 10.1542/peds.71.1.81</identifier><identifier>PMID: 6848983</identifier><language>eng</language><publisher>United States</publisher><subject>Adolescent ; Adult ; Antigen-Antibody Complex - immunology ; Bacterial Infections - genetics ; Bacterial Infections - immunology ; Complement C3 - deficiency ; Dermatitis - genetics ; Dermatitis - immunology ; Female ; Genes, Recessive ; Humans ; IgG Deficiency ; Male ; Middle Aged ; Pedigree ; Recurrence ; Vasculitis - genetics ; Vasculitis - immunology</subject><ispartof>Pediatrics (Evanston), 1983-01, Vol.71 (1), p.81-87</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c232t-ebc3db48e59af059155eae0f22944dc875a47a873163f75c06a92ce24d6a3be33</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/6848983$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Roord, J J</creatorcontrib><creatorcontrib>Daha, M</creatorcontrib><creatorcontrib>Kuis, W</creatorcontrib><creatorcontrib>Verbrugh, H A</creatorcontrib><creatorcontrib>Verhoef, J</creatorcontrib><creatorcontrib>Zegers, B J</creatorcontrib><creatorcontrib>Stoop, J W</creatorcontrib><title>Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family</title><title>Pediatrics (Evanston)</title><addtitle>Pediatrics</addtitle><description>A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. Biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Antigen-Antibody Complex - immunology</subject><subject>Bacterial Infections - genetics</subject><subject>Bacterial Infections - immunology</subject><subject>Complement C3 - deficiency</subject><subject>Dermatitis - genetics</subject><subject>Dermatitis - immunology</subject><subject>Female</subject><subject>Genes, Recessive</subject><subject>Humans</subject><subject>IgG Deficiency</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Pedigree</subject><subject>Recurrence</subject><subject>Vasculitis - genetics</subject><subject>Vasculitis - immunology</subject><issn>0031-4005</issn><issn>1098-4275</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1983</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkTtv3DAQhAkjgX1-dGkNsEplXfiUqDKwndiAgTRJTfDIpY-BRCokleR-kf-mJfiQNsVisTvfTDMIfaBkS6VgnyZwZdvRLd0qeoI2lPSqEayT79CGEE4bQYg8Q-el_CSECNmxU3TaKqF6xTfo5THuIYcKDjvwwQaI9oCTx3UPy4TssE3jlCLEur7XY4BxvUwpyQazWv-EuscZ7JzzqkyH9AwxWByiB1tDiuUG25DtPJga4jMO4zhHOIb9hUU10eHfpixEqKEsRmzw3VztHnszhuFwid57MxS4Ou4L9OPL_ffbh-bp29fH289PjWWc1QZ2lrudUCB744nsqZRggHjGeiGcVZ00ojOq47TlvpOWtKZnFphwreE74PwCfXzLnXL6NUOpegzFwjCYCGkuWhEuKRftf0HKZa96Jhbw5g20OZWSwesph9Hkg6ZErwXqtUDdUU21ogt-fcyddyO4f_CxMf4KWUObpQ</recordid><startdate>198301</startdate><enddate>198301</enddate><creator>Roord, J J</creator><creator>Daha, M</creator><creator>Kuis, W</creator><creator>Verbrugh, H A</creator><creator>Verhoef, J</creator><creator>Zegers, B J</creator><creator>Stoop, J W</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>198301</creationdate><title>Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family</title><author>Roord, J J ; Daha, M ; Kuis, W ; Verbrugh, H A ; Verhoef, J ; Zegers, B J ; Stoop, J W</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c232t-ebc3db48e59af059155eae0f22944dc875a47a873163f75c06a92ce24d6a3be33</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1983</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Antigen-Antibody Complex - immunology</topic><topic>Bacterial Infections - genetics</topic><topic>Bacterial Infections - immunology</topic><topic>Complement C3 - deficiency</topic><topic>Dermatitis - genetics</topic><topic>Dermatitis - immunology</topic><topic>Female</topic><topic>Genes, Recessive</topic><topic>Humans</topic><topic>IgG Deficiency</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Pedigree</topic><topic>Recurrence</topic><topic>Vasculitis - genetics</topic><topic>Vasculitis - immunology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Roord, J J</creatorcontrib><creatorcontrib>Daha, M</creatorcontrib><creatorcontrib>Kuis, W</creatorcontrib><creatorcontrib>Verbrugh, H A</creatorcontrib><creatorcontrib>Verhoef, J</creatorcontrib><creatorcontrib>Zegers, B J</creatorcontrib><creatorcontrib>Stoop, J W</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatrics (Evanston)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Roord, J J</au><au>Daha, M</au><au>Kuis, W</au><au>Verbrugh, H A</au><au>Verhoef, J</au><au>Zegers, B J</au><au>Stoop, J W</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family</atitle><jtitle>Pediatrics (Evanston)</jtitle><addtitle>Pediatrics</addtitle><date>1983-01</date><risdate>1983</risdate><volume>71</volume><issue>1</issue><spage>81</spage><epage>87</epage><pages>81-87</pages><issn>0031-4005</issn><eissn>1098-4275</eissn><abstract>A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. Biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.</abstract><cop>United States</cop><pmid>6848983</pmid><doi>10.1542/peds.71.1.81</doi><tpages>7</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0031-4005 |
| ispartof | Pediatrics (Evanston), 1983-01, Vol.71 (1), p.81-87 |
| issn | 0031-4005 1098-4275 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_80351346 |
| source | MEDLINE; EZB-FREE-00999 freely available EZB journals |
| subjects | Adolescent Adult Antigen-Antibody Complex - immunology Bacterial Infections - genetics Bacterial Infections - immunology Complement C3 - deficiency Dermatitis - genetics Dermatitis - immunology Female Genes, Recessive Humans IgG Deficiency Male Middle Aged Pedigree Recurrence Vasculitis - genetics Vasculitis - immunology |
| title | Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-29T01%3A49%3A31IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Inherited%20deficiency%20of%20the%20third%20component%20of%20complement%20associated%20with%20recurrent%20pyogenic%20infections,%20circulating%20immune%20complexes,%20and%20vasculitis%20in%20a%20Dutch%20family&rft.jtitle=Pediatrics%20(Evanston)&rft.au=Roord,%20J%20J&rft.date=1983-01&rft.volume=71&rft.issue=1&rft.spage=81&rft.epage=87&rft.pages=81-87&rft.issn=0031-4005&rft.eissn=1098-4275&rft_id=info:doi/10.1542/peds.71.1.81&rft_dat=%3Cproquest_cross%3E80351346%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=13598924&rft_id=info:pmid/6848983&rfr_iscdi=true |