Hypoimmunoglobulinemia with normal T cell function in female siblings
A distinct syndrome reported here provides evidence for an autosomal recessive counterpart to X-linked (Bruton's) hypoimmunoglobulinemia. Two sisters had humoral (B cell) immunodeficiency. One suffered from recurrent infection, the second presented with non septic arthritis. Both had markedly l...
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| Veröffentlicht in: | Clinical immunology and immunopathology 1977-05, Vol.7 (3), p.364-371 |
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| container_end_page | 371 |
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| container_issue | 3 |
| container_start_page | 364 |
| container_title | Clinical immunology and immunopathology |
| container_volume | 7 |
| creator | Hoffman, Thomas Winchester, Robert Schulkind, Martin Frias, Jaime L. Ayoub, Elia M. Good, Robert A. |
| description | A distinct syndrome reported here provides evidence for an autosomal recessive counterpart to X-linked (Bruton's) hypoimmunoglobulinemia. Two sisters had humoral (B cell) immunodeficiency. One suffered from recurrent infection, the second presented with non septic arthritis. Both had markedly lowered immunoglobulin levels in serum and secretions, deficient antibody production, and absent B-dependent regions in their lymph nodes. They also lacked surface immunoglobulin-bearing peripheral blood lymphocytes. T cell number and cell-mediated immunity were normal; lymphocytes with complement or Fc receptors were present. |
| doi_str_mv | 10.1016/0090-1229(77)90070-8 |
| format | Article |
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Two sisters had humoral (B cell) immunodeficiency. One suffered from recurrent infection, the second presented with non septic arthritis. Both had markedly lowered immunoglobulin levels in serum and secretions, deficient antibody production, and absent B-dependent regions in their lymph nodes. They also lacked surface immunoglobulin-bearing peripheral blood lymphocytes. T cell number and cell-mediated immunity were normal; lymphocytes with complement or Fc receptors were present.</description><identifier>ISSN: 0090-1229</identifier><identifier>EISSN: 1090-2341</identifier><identifier>DOI: 10.1016/0090-1229(77)90070-8</identifier><identifier>PMID: 301456</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Agammaglobulinemia - genetics ; Agammaglobulinemia - immunology ; Antibody Formation ; Child ; Child, Preschool ; Female ; Humans ; Immunity, Cellular ; Infant ; Lymph Nodes - pathology ; T-Lymphocytes - immunology</subject><ispartof>Clinical immunology and immunopathology, 1977-05, Vol.7 (3), p.364-371</ispartof><rights>1977</rights><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c271t-75941532752a0a826de16034380e6be85c299d0cfa03425fd0e3923d315902013</citedby><cites>FETCH-LOGICAL-c271t-75941532752a0a826de16034380e6be85c299d0cfa03425fd0e3923d315902013</cites></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/301456$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hoffman, Thomas</creatorcontrib><creatorcontrib>Winchester, Robert</creatorcontrib><creatorcontrib>Schulkind, Martin</creatorcontrib><creatorcontrib>Frias, Jaime L.</creatorcontrib><creatorcontrib>Ayoub, Elia M.</creatorcontrib><creatorcontrib>Good, Robert A.</creatorcontrib><title>Hypoimmunoglobulinemia with normal T cell function in female siblings</title><title>Clinical immunology and immunopathology</title><addtitle>Clin Immunol Immunopathol</addtitle><description>A distinct syndrome reported here provides evidence for an autosomal recessive counterpart to X-linked (Bruton's) hypoimmunoglobulinemia. 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T cell number and cell-mediated immunity were normal; lymphocytes with complement or Fc receptors were present.</description><subject>Agammaglobulinemia - genetics</subject><subject>Agammaglobulinemia - immunology</subject><subject>Antibody Formation</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Female</subject><subject>Humans</subject><subject>Immunity, Cellular</subject><subject>Infant</subject><subject>Lymph Nodes - pathology</subject><subject>T-Lymphocytes - immunology</subject><issn>0090-1229</issn><issn>1090-2341</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1977</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kD1PwzAQhi3EVyn8gw6eEAyBs53E8YKEqkKRKrGU2XKcSzFK4hInoP57EloxMp1074fuHkJmDO4YsPQeQEHEOFc3Ut4qAAlRdkQmbFxzEbNjMvmznJOLED4AII1BnpFTASxO0glZLHdb7-q6b_ym8nlfuQZrZ-i3695p49vaVHRNLVYVLfvGds431DW0xEFAGlw-BDbhkpyUpgp4dZhT8va0WM-X0er1-WX-uIosl6yLZKJilgguE27AZDwtkKUgYpEBpjlmieVKFWBLMyx5UhaAQnFRCJYo4MDElFzve7et_-wxdLp2YTzONOj7oDOhUiXEaIz3Rtv6EFos9bZ1tWl3moEe4emRjB7JaCn1L7whPSWzQ3-f11j8hfa0BvlhL-Pw45fDVgfrsLFYuBZtpwvv_u__AdnxfEo</recordid><startdate>197705</startdate><enddate>197705</enddate><creator>Hoffman, Thomas</creator><creator>Winchester, Robert</creator><creator>Schulkind, Martin</creator><creator>Frias, Jaime L.</creator><creator>Ayoub, Elia M.</creator><creator>Good, Robert A.</creator><general>Elsevier Inc</general><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>7X8</scope></search><sort><creationdate>197705</creationdate><title>Hypoimmunoglobulinemia with normal T cell function in female siblings</title><author>Hoffman, Thomas ; Winchester, Robert ; Schulkind, Martin ; Frias, Jaime L. ; Ayoub, Elia M. ; Good, Robert A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c271t-75941532752a0a826de16034380e6be85c299d0cfa03425fd0e3923d315902013</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1977</creationdate><topic>Agammaglobulinemia - genetics</topic><topic>Agammaglobulinemia - immunology</topic><topic>Antibody Formation</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Female</topic><topic>Humans</topic><topic>Immunity, Cellular</topic><topic>Infant</topic><topic>Lymph Nodes - pathology</topic><topic>T-Lymphocytes - immunology</topic><toplevel>online_resources</toplevel><creatorcontrib>Hoffman, Thomas</creatorcontrib><creatorcontrib>Winchester, Robert</creatorcontrib><creatorcontrib>Schulkind, Martin</creatorcontrib><creatorcontrib>Frias, Jaime L.</creatorcontrib><creatorcontrib>Ayoub, Elia M.</creatorcontrib><creatorcontrib>Good, Robert A.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical immunology and immunopathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hoffman, Thomas</au><au>Winchester, Robert</au><au>Schulkind, Martin</au><au>Frias, Jaime L.</au><au>Ayoub, Elia M.</au><au>Good, Robert A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Hypoimmunoglobulinemia with normal T cell function in female siblings</atitle><jtitle>Clinical immunology and immunopathology</jtitle><addtitle>Clin Immunol Immunopathol</addtitle><date>1977-05</date><risdate>1977</risdate><volume>7</volume><issue>3</issue><spage>364</spage><epage>371</epage><pages>364-371</pages><issn>0090-1229</issn><eissn>1090-2341</eissn><abstract>A distinct syndrome reported here provides evidence for an autosomal recessive counterpart to X-linked (Bruton's) hypoimmunoglobulinemia. 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| ispartof | Clinical immunology and immunopathology, 1977-05, Vol.7 (3), p.364-371 |
| issn | 0090-1229 1090-2341 |
| language | eng |
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| source | MEDLINE; Alma/SFX Local Collection |
| subjects | Agammaglobulinemia - genetics Agammaglobulinemia - immunology Antibody Formation Child Child, Preschool Female Humans Immunity, Cellular Infant Lymph Nodes - pathology T-Lymphocytes - immunology |
| title | Hypoimmunoglobulinemia with normal T cell function in female siblings |
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