{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T05:46:00Z","timestamp":1777355160438,"version":"3.51.4"},"reference-count":43,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2005,3,1]],"date-time":"2005-03-01T00:00:00Z","timestamp":1109635200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Clin Immunol"],"published-print":{"date-parts":[[2005,3]]},"DOI":"10.1007\/s10875-005-2822-2","type":"journal-article","created":{"date-parts":[[2005,4,8]],"date-time":"2005-04-08T11:24:28Z","timestamp":1112959468000},"page":"162-173","source":"Crossref","is-referenced-by-count":53,"title":["Akhil Kakroo"],"prefix":"10.1007","volume":"25","author":[{"given":"Wen-I","family":"Lee","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming-Ling","family":"Kuo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jing-Long","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Syh-Jae","family":"Lin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cheng-Jang","family":"Wu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"CR1","unstructured":"Stiehm ER, Ochs HD, Winkelstein JA: Immunologic disorders: General considerations. In Immunodeficiency Disorders in Infants & Children, ER Stiehm, HD Ochs, JA Winkelstein (eds). Philadelphia, WB Saunders, 2004, pp 289?355"},{"key":"CR2","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1542\/peds.9.6.722","volume":"9","author":"OC Bruton","year":"1952","unstructured":"Bruton OC: Agammaglobulinemia. Pediatrics 9:772?728, 1952","journal-title":"Pediatrics"},{"key":"CR3","doi-asserted-by":"crossref","unstructured":"Smith CIE, Ochs HD, Puck JM: Genetically determined immunodeficiency diseases: A perspective. In Primary immunodeficiency diseases, HD Ochs, CIE Smith, JM Puck (eds). New York, Oxford, 1999, pp 3?11","DOI":"10.1093\/oso\/9780195147742.003.0001"},{"key":"CR4","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/0090-1229(86)90070-X","volume":"40","author":"ER Stiem","year":"1986","unstructured":"Stiem ER, Chin TW, Hass A, Peerless AG: Infectious complications of primary immunodeficiencies. Clin Immunol Immunopathol 40:69?89, 1986","journal-title":"Clin Immunol Immunopathol"},{"key":"CR5","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1097\/00001432-199408000-00005","volume":"7","author":"ABD Webster","year":"1994","unstructured":"Webster ABD: Infection in primary immunodeficiency syndrome. Curr Opin Infect Dis 7:444?449, 1994","journal-title":"Curr Opin Infect Dis"},{"key":"CR6","first-page":"1","volume":"1","author":"WHO scientific group","year":"1997","unstructured":"WHO scientific group: Primary immunodeficiency disease. J Clin Exp Immunol supp 1:1?28, 1997","journal-title":"J Clin Exp Immunol supp"},{"key":"CR7","doi-asserted-by":"crossref","first-page":"1917","DOI":"10.1016\/S0264-410X(97)00127-8","volume":"15","author":"TY Lin","year":"1997","unstructured":"Lin TY, Chiang BL: Specific immune response in adult medical personnel immunized with acellular pertusis vaccine with special emphasis on T helper cell response. Vaccine 15:1917?1921, 1997","journal-title":"Vaccine"},{"key":"CR8","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/S0022-1759(00)00161-7","volume":"239","author":"K Godoy-Ramirez","year":"2000","unstructured":"Godoy-Ramirez K, Franck K, Gaines H: A novel method for the simulataneous assessment of natural killer cell conjugate formation and cytotoxicity at the single-cell level by multi-parameter flow cytometry. J Immunol Methods 239:35?44, 2000","journal-title":"J Immunol Methods"},{"key":"CR9","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1046\/j.0905-6157.2003.00103.x","volume":"15","author":"SJ Lin","year":"2004","unstructured":"Lin SJ, Cheng PL, Huang YJ, Kou ML: Evaluation of cytotoxic function and apoptosis in interleukin (IL)-12\/IL-15-treated umbilical cord or adult peripheral blood natural killer cell by a propidium-iodide based flow cytometry. Pediatr Allergy Immunol 15:79?85, 2004","journal-title":"Pediatr Allergy Immunol"},{"key":"CR10","first-page":"595","volume":"91","author":"T Futatani","year":"1998","unstructured":"Futatani T, Miyawaki T, Tsukada S, Hashimoto S, Kunikata T, Arai S, Kurimoto M, Niida Y, Matsuoka H, Sakiyama Y, Iwata T, Tsuchiya S, Tatsuzawa O, Yoshizaki K, Kishimoto T: Deficient expression of Bruton?s tyrosine kinase in monocytes from X-linked agammaglobulinemia as evaluated by a flow cytometric analysis and its clinical application to carrier detection. Blood 91:595?602, 1998","journal-title":"Blood"},{"key":"CR11","doi-asserted-by":"crossref","first-page":"2421","DOI":"10.1182\/blood.V92.7.2421","volume":"92","author":"K Seyama","year":"1998","unstructured":"Seyama K, Nonoyama S, Gangsaas I, Hollenbaugh D, Pabst HF, Aruffo A, Ochs HD: Mutations of the CD40 ligand gene and its effect on CD40 ligand expression in patients with X-linked hyper IgM syndrome. Blood 92:2421?2434, 1998","journal-title":"Blood"},{"key":"CR12","first-page":"1968","volume":"89","author":"JM Puck","year":"1997","unstructured":"Puck JM, Pepper AE, Henthorn PS, Candotti F, Isakov J, Whitwam T, Conley ME, Fischer RE, Rosenblatt HM, Small TN, Buckley RH: Mutations analysis of IL2RG in human X-linked severe combined immunodeficiency. Blood 89:1968?1977, 1997","journal-title":"Blood"},{"key":"CR13","doi-asserted-by":"crossref","first-page":"2680","DOI":"10.1182\/blood.V90.7.2680","volume":"90","author":"Q Zhu","year":"1997","unstructured":"Zhu Q, Watanabe C, Liu T, Hollenbaugh D, Blaese RM, Kanner SB, Aruffo A, Ochs HD: Wiskott-Aldrich syndrome\/X-linked thrombocytopenia: WASP mutations, protein expression, and phenotype. Blood 90:2680?2689, 1997","journal-title":"Blood"},{"key":"CR14","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1016\/S0091-6749(03)02021-9","volume":"112","author":"WI Lee","year":"2003","unstructured":"Lee WI, Zhu Q, Gambineri E, Jin Y, Welcher A, Ochs HD: Inducible CO-Stimulator molecule (ICOS), a candidate gene for defective isotype switching, is normal in patients with Hyper IgM syndrome of unknown molecular diagnosis. J Allergy Clin Immunol 112:958?964, 2003","journal-title":"J Allergy Clin Immunol"},{"key":"CR15","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1136\/jmg.30.10.813","volume":"30","author":"DA Driscoll","year":"1993","unstructured":"Driscoll DA, Salvin J, Sellinger B, Budarf ML, McDonald-McGinn DM, Zackai EH, Emanuel BS: Prevalence of 22q11 microdelletions in DiGeorge and velocardiofacial syndromes: Implications for genetic counseling and prenatal diagnosis. J Med Genet 30:813?817, 1993","journal-title":"J Med Genet"},{"key":"CR16","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1182\/blood.V91.1.165","volume":"91","author":"D David","year":"1998","unstructured":"David D, Bani L, Moreau JL, Demaison C, Sun K, Salvucci O, Nakarai T, de Montalembert M, Chouaib S, Joussemet M, Ritz J, Theze J: Further analysis of IL-2 receptor subunit expression on the different human peripheral blood mononuclear cell subsets. Blood 91:165?172, 1998","journal-title":"Blood"},{"key":"CR17","first-page":"262","volume":"6","author":"DR Freyer","year":"2004","unstructured":"Freyer DR, Gowans LK, Warzynski M, Lee WI: Flow cytometeric diagnosis of X-linked hyper-IgM syndrome: Application of an accurate and convenient procedure. J Pediatr Hematol Oncol 6:262?370, 2004","journal-title":"J Pediatr Hematol Oncol"},{"key":"CR18","doi-asserted-by":"crossref","first-page":"1881","DOI":"10.1182\/blood-2003-12-4420","volume":"105","author":"WI Lee","year":"2005","unstructured":"Lee WI, Torgerson TR, Schumacher MJ, Yel L, Zhu Q, Ochs HD: Mutation analysis in a large cohort patients with hyper IgM syndrome. Blood 105:1881?1890, 2005","journal-title":"Blood"},{"key":"CR19","doi-asserted-by":"crossref","first-page":"1598","DOI":"10.1016\/S0140-6736(99)04206-3","volume":"354","author":"HT Monis-Teisserenc","year":"1999","unstructured":"Monis-Teisserenc HT, Gadola SD, Cella M: Association of a syndrome rembling Wegener?s granulomatosis with low surface expression of HLA-class-I molecules. Lancet 354:1598?1603, 1999","journal-title":"Lancet"},{"key":"CR20","unstructured":"Report on Registeration of Births and Deaths, 2004. Registry of Births and Deaths, National Registeration Department, Taiwan"},{"key":"CR21","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1016\/S0022-3476(65)81796-6","volume":"67","author":"AM DiGerge","year":"1965","unstructured":"DiGerge AM: Discussion on a new concept of the cellular basis of immunology. J Pediatr 67:907, 1965","journal-title":"J Pediatr"},{"key":"CR22","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1016\/S0022-3476(79)80207-3","volume":"94","author":"ME Conley","year":"1979","unstructured":"Conley ME, Beckwith JB, Mancer JFK, Tenckhoff L: The spectum of the DiGerge syndrome. J Pediatr 94:883?890, 1979","journal-title":"J Pediatr"},{"key":"CR23","first-page":"579","volume":"44","author":"DL Lim","year":"2003","unstructured":"Lim DL, Thong BY, Ho SY, Shek LP, Lou J, Leong KP, Chng HH, Lee BW: Primary immunodeficiency diseases in Singapore-the last 11 years. Singapore Med J 44:579?586, 2003","journal-title":"Singapore Med J"},{"key":"CR24","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1007\/BF00916891","volume":"2","author":"A Fasth","year":"1982","unstructured":"Fasth A: Primary immunodeficiency disorders in Sweden: Cases among children. 1974?1979. J Clin Immunol 2:86?92, 1982","journal-title":"J Clin Immunol"},{"key":"CR25","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/BF00915474","volume":"1","author":"H Hayakawa","year":"1981","unstructured":"Hayakawa H, Iwata T, Yata J, Kobayashi N: Primary immunodeficiency syndrome in Japan. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol 1:31?39, 1981","journal-title":"J Clin Immunol"},{"key":"CR26","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1007\/BF00915792","volume":"3","author":"G Luzi","year":"1983","unstructured":"Luzi G, Businco L, Aiuti F: Primary immunodeficiency syndromes in Italy: A report of the national register in children and adults. J Clin Immunol 3:316?320, 1983","journal-title":"J Clin Immunol"},{"key":"CR27","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1007\/BF00916954","volume":"8","author":"O Ryser","year":"1988","unstructured":"Ryser O, Morell A, Hitzig WH: Primary immunodeficiencies in Switzerland: First report of the national registry in adults and children. J Clin Immunol 8:479?485, 1988","journal-title":"J Clin Immunol"},{"key":"CR28","first-page":"191","volume":"4","author":"HC Gooi","year":"1993","unstructured":"Gooi HC: Primary immunodeficiency register, United Kingdom: Update 1992. Immunodeficiency 4:191?192, 1993","journal-title":"Immunodeficiency"},{"key":"CR29","first-page":"193","volume":"4","author":"P Affentranger","year":"1993","unstructured":"Affentranger P, Morell A, Spath P, Seger R: Registry of primary immunodeficiencies in Switzerland. Immunodeficiency 4:193?195, 1993","journal-title":"Immunodeficiency"},{"key":"CR30","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1023\/A:1027335000994","volume":"17","author":"AS Grumach","year":"1997","unstructured":"Grumach AS, Duarte AJ, Bellinati-Pires R, Pastorino AC, Jacob CM, Diogo CL, Condino-Neto A, Kirschfink M, Carneiro-Sampaio MM: Brazilian report on primary immunodeficiencies in children: 166 Cases studied over a follow-up time of 15 years. J Clin Immunol 17:340?345, 1997","journal-title":"J Clin Immunol"},{"key":"CR31","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1023\/A:1027382916924","volume":"17","author":"FN Matamoros","year":"1997","unstructured":"Matamoros FN, Mila LJ, Espanol BT, Raga BS, Fontan CG: Primary immunodeficiency syndrome in Spain: First report of the National Registry in children and adults. J Clin Immunol 17:333?339, 1997","journal-title":"J Clin Immunol"},{"key":"CR32","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1023\/A:1023255102170","volume":"18","author":"M Zelazko","year":"1998","unstructured":"Zelazko M, Carneiro-Sampaio M, Cornejo de Luigi M, Garcia de Olarte D, Porras Madrigal O, Berron Perez R, Cabello A, Rostan MV, Sorensen RU: Primary immunodeficiency diseases in Latin America: First report from eight countries participating in the LAGID. Latin American Group for Primary Immunodeficiency Diseases. J Clin Immunol 18:161?166, 1998","journal-title":"J Clin Immunol"},{"key":"CR33","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1016\/S0031-3955(05)70270-4","volume":"47","author":"ME Elder","year":"2000","unstructured":"Elder ME: T-cell immunodeficiencies. Pediatr Clin North Am 47:1253?1274, 2000","journal-title":"Pediatr Clin North Am"},{"issue":"Suppl 1","key":"CR34","first-page":"1","volume":"118","author":"Primary immunodeficiency disease-report of an IUIS scientific committee.","year":"1999","unstructured":"Primary immunodeficiency disease-report of an IUIS scientific committee. Clin Exp Immunol 118 (Suppl 1):1?28, 1999","journal-title":"Clin Exp Immunol"},{"key":"CR35","unstructured":"Chapel HM, Webster ADB: Assessment of the immune system. In Primary Immunodeficiency Diseases, HD Ochs, CIE Smith, JM Puck (eds). New York, Oxford, 1999, pp 419?431"},{"key":"CR36","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1016\/S0022-3476(99)70246-5","volume":"134","author":"P Quartier","year":"1999","unstructured":"Quartier P, Debre M, De Blic J, de Sauverzac R, Sayegh N, Jabado N, Haddad E, Blanche S, Casanova JL, Smith CI, Le Deist F, de Saint Basile G, Fischer A: Early and prolonged intravenous immunoglobulin replacement therapy in childhood aggammaglobulinemia: A retrospetic survey of 31 patients. J Pediatr 134:589?596, 1999","journal-title":"J Pediatr"},{"key":"CR37","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1056\/NEJM199902183400703","volume":"340","author":"RH Buckley","year":"1999","unstructured":"Buckley RH, Schiff SE, Schiff RI, Markert L, Williams LW, Roberts JL, Myers LA, Ward FE: Hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency. N Engl J Med 340:508?516, 1999","journal-title":"N Engl J Med"},{"key":"CR38","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1023\/A:1026463900925","volume":"20","author":"AP Knutsen","year":"2000","unstructured":"Knutsen AP, Wall DA: Umbilical cord blood transplantation in severe T-cell immunodeficiency disorders: Two-year experience. J Clin Immunol 20:466?476, 2000","journal-title":"J Clin Immunol"},{"key":"CR39","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/S0022-3476(96)70248-2","volume":"129","author":"H Ozsahin","year":"1996","unstructured":"Ozsahin H, Le Deist F, Benkerrou M, Cavazzana-Calvo M, Gomez L, Griscelli C, Blanche S, Fischer A: Bone marrow transplantation in 26 patients with Wiskott?Aldrich syndrome from a single center. J Pediatr 129:238?244, 1996","journal-title":"J Pediatr"},{"key":"CR40","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1056\/NEJM200108023450516","volume":"345","author":"A Thrasher","year":"2001","unstructured":"Thrasher A, Goldblatt D: Hematopoietic-cell transplantation for chronic granulomatous disease. N Engl J Med 345:377?378, 2001","journal-title":"N Engl J Med"},{"key":"CR41","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1056\/NEJMoa012616","volume":"346","author":"S Hacein-Bey-Abina","year":"2002","unstructured":"Hacein-Bey-Abina S, Le Deist F, Carlier F, Bouneaud C, Hue C, De Villartay JP, Thrasher AJ, Wulffraat N, Sorensen R, Dupuis-Girod S, Fischer A, Davies EG, Kuis W, Leiva L, Cavazzana-Calvo M: Sustained correction of X-linked severe combined immunodeficiency by ex vivo gene therapy. N Engl J Med 346:1185?1193, 2002","journal-title":"N Engl J Med"},{"key":"CR42","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1038\/nri859","volume":"2","author":"A Fischer","year":"2002","unstructured":"Fischer A, Hacein-Bey S, Cavazzana-Calvo M: Gene therapy of severe combined immunodeficiencies. Nat Rev Immunol 2:615?621, 2002","journal-title":"Nat Rev Immunol"},{"key":"CR43","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1016\/j.jaci.2004.01.761","volume":"113","author":"C Cunningham-Rundles","year":"2004","unstructured":"Cunningham-Rundles C, Sidi P, Estrella L, Doucette J: Identifying undiagnosed primary immunodeficiency diseases in minority subjects by using computer sorting of diagnosis codes. J Allergy Clin Immunol 113:747?755, 2004","journal-title":"J Allergy Clin Immunol"}],"container-title":["Journal of Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-005-2822-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10875-005-2822-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-005-2822-2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,24]],"date-time":"2024-01-24T17:03:41Z","timestamp":1706115821000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10875-005-2822-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,3]]},"references-count":43,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2005,3]]}},"alternative-id":["2822"],"URL":"https:\/\/doi.org\/10.1007\/s10875-005-2822-2","relation":{},"ISSN":["0271-9142","1573-2592"],"issn-type":[{"value":"0271-9142","type":"print"},{"value":"1573-2592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,3]]}}}