Immunophenotypic analysis of lymphocyte subsets in newborns with biotinidase deficiency

Background/Aim Biotin is a vital micronutrient that plays a role in metabolic homeostasis and the regulation of innate and adaptive immune system functions. Biotinidase deficiency (BTD) leads to impairment in biotin‐dependent immune functions. This study focused on immunophenotypic analysis of lymph...

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Veröffentlicht in:Pediatric allergy and immunology 2021-04, Vol.32 (3), p.586-598
Hauptverfasser: Karaoglan, Murat, Nacarkahya, Gulper, Keskin, Mehmet, Keskin, Ozlem, Atanaskovic‐Markovic, Marina
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container_issue 3
container_start_page 586
container_title Pediatric allergy and immunology
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creator Karaoglan, Murat
Nacarkahya, Gulper
Keskin, Mehmet
Keskin, Ozlem
Atanaskovic‐Markovic, Marina
description Background/Aim Biotin is a vital micronutrient that plays a role in metabolic homeostasis and the regulation of innate and adaptive immune system functions. Biotinidase deficiency (BTD) leads to impairment in biotin‐dependent immune functions. This study focused on immunophenotypic analysis of lymphocyte subsets in newborns with BTD. Patients and Methods A total of 181 (95 female and 86 male; 114 had BTD and 67 were healthy) newborns underwent biotinidase enzyme activity, molecular and lymphocyte immunophenotyping analyses. BTD is classified into four biochemical phenotypes: profound, partial, heterozygous and normal. The following lymphocyte subsets were studied in all participants: total B lymphocyte (CD19), total T lymphocyte (CD3), helper T lymphocyte (CD3/CD4), cytotoxic T lymphocyte (CTL) (CD3/CD8), natural killer T lymphocyte (CD16/56) and a T‐lymphocyte activation marker (HLA‐DR). Results The percentages of lymphocyte subsets were similar in newborns with and without BTD. In all newborns with BTD, the mean CD3/CD4 levels were higher in females, while the CD3/CD8 levels were higher in males (P 
doi_str_mv 10.1111/pai.13416
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Biotinidase deficiency (BTD) leads to impairment in biotin‐dependent immune functions. This study focused on immunophenotypic analysis of lymphocyte subsets in newborns with BTD. Patients and Methods A total of 181 (95 female and 86 male; 114 had BTD and 67 were healthy) newborns underwent biotinidase enzyme activity, molecular and lymphocyte immunophenotyping analyses. BTD is classified into four biochemical phenotypes: profound, partial, heterozygous and normal. The following lymphocyte subsets were studied in all participants: total B lymphocyte (CD19), total T lymphocyte (CD3), helper T lymphocyte (CD3/CD4), cytotoxic T lymphocyte (CTL) (CD3/CD8), natural killer T lymphocyte (CD16/56) and a T‐lymphocyte activation marker (HLA‐DR). Results The percentages of lymphocyte subsets were similar in newborns with and without BTD. In all newborns with BTD, the mean CD3/CD4 levels were higher in females, while the CD3/CD8 levels were higher in males (P &lt; .001 for each). In female and male newborns, the CD3/CD4 levels were 53.83 ± 9.46 and 16.82 ± 5.19, respectively, and the CD3/CD8 levels were 48.80 ± 8.65 and 21.48 ± 6.02, respectively. A moderate negative correlation was found between CD3/CD4 and CD3/CD8 in female and male newborns (rfemale = −0.488, rmale = −0.574, P &lt; .001). Conclusion This study showed that although there were no differences in the lymphocyte subsets in newborns with BTD, the CD3/CD4 levels were higher in females, and the CD3/CD8 levels were higher in males. In addition, there was a negative correlation between the CD3/CD4 and CD3/CD8 levels in both genders. Although these results indicate sexual dimorphism between CD3/CD4 and CD3/CD8 levels, whether this dissociation is unique to BTD in newborns is not fully clear.</description><identifier>ISSN: 0905-6157</identifier><identifier>EISSN: 1399-3038</identifier><identifier>DOI: 10.1111/pai.13416</identifier><identifier>PMID: 33217065</identifier><language>eng</language><publisher>England: Wiley Subscription Services, Inc</publisher><subject>Biotin ; Biotinidase ; biotinidase deficiency ; CD16 antigen ; CD19 antigen ; CD3 antigen ; CD4 antigen ; CD8 antigen ; Cell activation ; Cytotoxicity ; Enzymatic activity ; Females ; Histocompatibility antigen HLA ; Homeostasis ; Immune system ; lymphocyte immunophenotyping ; Lymphocytes ; Lymphocytes B ; Lymphocytes T ; Males ; Neonates ; Phenotypes ; Sexual dimorphism ; sexual immune dimorphism</subject><ispartof>Pediatric allergy and immunology, 2021-04, Vol.32 (3), p.586-598</ispartof><rights>2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.</rights><rights>Copyright © 2021 John Wiley &amp; Sons A/S</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3136-b2c10693650c2040795252f7cda5e144a22aa8f8a112f26f9ee2c157dcaf49d13</cites><orcidid>0000-0002-8512-8833 ; 0000-0002-2861-3568 ; 0000-0002-5544-8991 ; 0000-0002-3061-7832</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fpai.13416$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fpai.13416$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33217065$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Atanaskovic‐Markovic, Marina</contributor><creatorcontrib>Karaoglan, Murat</creatorcontrib><creatorcontrib>Nacarkahya, Gulper</creatorcontrib><creatorcontrib>Keskin, Mehmet</creatorcontrib><creatorcontrib>Keskin, Ozlem</creatorcontrib><creatorcontrib>Atanaskovic‐Markovic, Marina</creatorcontrib><title>Immunophenotypic analysis of lymphocyte subsets in newborns with biotinidase deficiency</title><title>Pediatric allergy and immunology</title><addtitle>Pediatr Allergy Immunol</addtitle><description>Background/Aim Biotin is a vital micronutrient that plays a role in metabolic homeostasis and the regulation of innate and adaptive immune system functions. Biotinidase deficiency (BTD) leads to impairment in biotin‐dependent immune functions. This study focused on immunophenotypic analysis of lymphocyte subsets in newborns with BTD. Patients and Methods A total of 181 (95 female and 86 male; 114 had BTD and 67 were healthy) newborns underwent biotinidase enzyme activity, molecular and lymphocyte immunophenotyping analyses. BTD is classified into four biochemical phenotypes: profound, partial, heterozygous and normal. The following lymphocyte subsets were studied in all participants: total B lymphocyte (CD19), total T lymphocyte (CD3), helper T lymphocyte (CD3/CD4), cytotoxic T lymphocyte (CTL) (CD3/CD8), natural killer T lymphocyte (CD16/56) and a T‐lymphocyte activation marker (HLA‐DR). Results The percentages of lymphocyte subsets were similar in newborns with and without BTD. In all newborns with BTD, the mean CD3/CD4 levels were higher in females, while the CD3/CD8 levels were higher in males (P &lt; .001 for each). In female and male newborns, the CD3/CD4 levels were 53.83 ± 9.46 and 16.82 ± 5.19, respectively, and the CD3/CD8 levels were 48.80 ± 8.65 and 21.48 ± 6.02, respectively. A moderate negative correlation was found between CD3/CD4 and CD3/CD8 in female and male newborns (rfemale = −0.488, rmale = −0.574, P &lt; .001). Conclusion This study showed that although there were no differences in the lymphocyte subsets in newborns with BTD, the CD3/CD4 levels were higher in females, and the CD3/CD8 levels were higher in males. In addition, there was a negative correlation between the CD3/CD4 and CD3/CD8 levels in both genders. Although these results indicate sexual dimorphism between CD3/CD4 and CD3/CD8 levels, whether this dissociation is unique to BTD in newborns is not fully clear.</description><subject>Biotin</subject><subject>Biotinidase</subject><subject>biotinidase deficiency</subject><subject>CD16 antigen</subject><subject>CD19 antigen</subject><subject>CD3 antigen</subject><subject>CD4 antigen</subject><subject>CD8 antigen</subject><subject>Cell activation</subject><subject>Cytotoxicity</subject><subject>Enzymatic activity</subject><subject>Females</subject><subject>Histocompatibility antigen HLA</subject><subject>Homeostasis</subject><subject>Immune system</subject><subject>lymphocyte immunophenotyping</subject><subject>Lymphocytes</subject><subject>Lymphocytes B</subject><subject>Lymphocytes T</subject><subject>Males</subject><subject>Neonates</subject><subject>Phenotypes</subject><subject>Sexual dimorphism</subject><subject>sexual immune dimorphism</subject><issn>0905-6157</issn><issn>1399-3038</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><recordid>eNp10MFO3DAQBmALgcoWeugLIEtc2kPAY8fO5ohWtF1pJThQcYwcZ6w1SuwQJ1rl7TEN9IDEXObyzS_NT8h3YFeQ5rrX7gpEDuqIrECUZSaYWB-TFSuZzBTI4pR8jfGJMSiEgi_kVAgOBVNyRR63XTf50O_Rh3HunaHa63aOLtJgaTt3_T6YeUQapzriGKnz1OOhDoOP9ODGPa1dGJ13jY5IG7TOOPRmPicnVrcRv73tM_L31-3D5k-2u_u93dzsMiNAqKzmBpgqhZLMcJazopRccluYRkuEPNeca722aw3ALVe2REwXsmiMtnnZgDgjP5bcfgjPE8ax6lw02LbaY5hixXMlAFgu14lefqBPYRrSt0lJVnKe80Im9XNRZggxDmirfnCdHuYKWPXadpXarv61nezFW-JUd9j8l-_1JnC9gINrcf48qbq_2S6RL3efiSE</recordid><startdate>202104</startdate><enddate>202104</enddate><creator>Karaoglan, Murat</creator><creator>Nacarkahya, Gulper</creator><creator>Keskin, Mehmet</creator><creator>Keskin, Ozlem</creator><creator>Atanaskovic‐Markovic, Marina</creator><general>Wiley Subscription Services, Inc</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-8512-8833</orcidid><orcidid>https://orcid.org/0000-0002-2861-3568</orcidid><orcidid>https://orcid.org/0000-0002-5544-8991</orcidid><orcidid>https://orcid.org/0000-0002-3061-7832</orcidid></search><sort><creationdate>202104</creationdate><title>Immunophenotypic analysis of lymphocyte subsets in newborns with biotinidase deficiency</title><author>Karaoglan, Murat ; Nacarkahya, Gulper ; Keskin, Mehmet ; Keskin, Ozlem ; Atanaskovic‐Markovic, Marina</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3136-b2c10693650c2040795252f7cda5e144a22aa8f8a112f26f9ee2c157dcaf49d13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Biotin</topic><topic>Biotinidase</topic><topic>biotinidase deficiency</topic><topic>CD16 antigen</topic><topic>CD19 antigen</topic><topic>CD3 antigen</topic><topic>CD4 antigen</topic><topic>CD8 antigen</topic><topic>Cell activation</topic><topic>Cytotoxicity</topic><topic>Enzymatic activity</topic><topic>Females</topic><topic>Histocompatibility antigen HLA</topic><topic>Homeostasis</topic><topic>Immune system</topic><topic>lymphocyte immunophenotyping</topic><topic>Lymphocytes</topic><topic>Lymphocytes B</topic><topic>Lymphocytes T</topic><topic>Males</topic><topic>Neonates</topic><topic>Phenotypes</topic><topic>Sexual dimorphism</topic><topic>sexual immune dimorphism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Karaoglan, Murat</creatorcontrib><creatorcontrib>Nacarkahya, Gulper</creatorcontrib><creatorcontrib>Keskin, Mehmet</creatorcontrib><creatorcontrib>Keskin, Ozlem</creatorcontrib><creatorcontrib>Atanaskovic‐Markovic, Marina</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>Nursing &amp; Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric allergy and immunology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Karaoglan, Murat</au><au>Nacarkahya, Gulper</au><au>Keskin, Mehmet</au><au>Keskin, Ozlem</au><au>Atanaskovic‐Markovic, Marina</au><au>Atanaskovic‐Markovic, Marina</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Immunophenotypic analysis of lymphocyte subsets in newborns with biotinidase deficiency</atitle><jtitle>Pediatric allergy and immunology</jtitle><addtitle>Pediatr Allergy Immunol</addtitle><date>2021-04</date><risdate>2021</risdate><volume>32</volume><issue>3</issue><spage>586</spage><epage>598</epage><pages>586-598</pages><issn>0905-6157</issn><eissn>1399-3038</eissn><abstract>Background/Aim Biotin is a vital micronutrient that plays a role in metabolic homeostasis and the regulation of innate and adaptive immune system functions. Biotinidase deficiency (BTD) leads to impairment in biotin‐dependent immune functions. This study focused on immunophenotypic analysis of lymphocyte subsets in newborns with BTD. Patients and Methods A total of 181 (95 female and 86 male; 114 had BTD and 67 were healthy) newborns underwent biotinidase enzyme activity, molecular and lymphocyte immunophenotyping analyses. BTD is classified into four biochemical phenotypes: profound, partial, heterozygous and normal. The following lymphocyte subsets were studied in all participants: total B lymphocyte (CD19), total T lymphocyte (CD3), helper T lymphocyte (CD3/CD4), cytotoxic T lymphocyte (CTL) (CD3/CD8), natural killer T lymphocyte (CD16/56) and a T‐lymphocyte activation marker (HLA‐DR). Results The percentages of lymphocyte subsets were similar in newborns with and without BTD. In all newborns with BTD, the mean CD3/CD4 levels were higher in females, while the CD3/CD8 levels were higher in males (P &lt; .001 for each). In female and male newborns, the CD3/CD4 levels were 53.83 ± 9.46 and 16.82 ± 5.19, respectively, and the CD3/CD8 levels were 48.80 ± 8.65 and 21.48 ± 6.02, respectively. A moderate negative correlation was found between CD3/CD4 and CD3/CD8 in female and male newborns (rfemale = −0.488, rmale = −0.574, P &lt; .001). Conclusion This study showed that although there were no differences in the lymphocyte subsets in newborns with BTD, the CD3/CD4 levels were higher in females, and the CD3/CD8 levels were higher in males. In addition, there was a negative correlation between the CD3/CD4 and CD3/CD8 levels in both genders. Although these results indicate sexual dimorphism between CD3/CD4 and CD3/CD8 levels, whether this dissociation is unique to BTD in newborns is not fully clear.</abstract><cop>England</cop><pub>Wiley Subscription Services, Inc</pub><pmid>33217065</pmid><doi>10.1111/pai.13416</doi><tpages>13</tpages><orcidid>https://orcid.org/0000-0002-8512-8833</orcidid><orcidid>https://orcid.org/0000-0002-2861-3568</orcidid><orcidid>https://orcid.org/0000-0002-5544-8991</orcidid><orcidid>https://orcid.org/0000-0002-3061-7832</orcidid></addata></record>
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subjects Biotin
Biotinidase
biotinidase deficiency
CD16 antigen
CD19 antigen
CD3 antigen
CD4 antigen
CD8 antigen
Cell activation
Cytotoxicity
Enzymatic activity
Females
Histocompatibility antigen HLA
Homeostasis
Immune system
lymphocyte immunophenotyping
Lymphocytes
Lymphocytes B
Lymphocytes T
Males
Neonates
Phenotypes
Sexual dimorphism
sexual immune dimorphism
title Immunophenotypic analysis of lymphocyte subsets in newborns with biotinidase deficiency
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