{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T14:08:55Z","timestamp":1791554935428,"version":"4.3.4"},"reference-count":67,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,12,5]],"date-time":"2020-12-05T00:00:00Z","timestamp":1607126400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>There is paucity of literature on long\u2010term follow\u2010up of patients with hereditary angioedema (HAE) from developing countries.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This study was carried out to analyze the clinical manifestations, laboratory features, and genetic profile of 32 patients (21 male and 11 female) from 23 families diagnosed with HAE between January 1996 and December 2019.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data were retrieved from medical records of Paediatric Immunodeficiency Clinic, Postgraduate Institute of Medical Education and Research, Chandigarh, India.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Median age at onset of symptoms was 6.25\u00a0years (range 1\u201025\u00a0years), and median age at diagnosis was 12\u00a0years (range 2\u201043\u00a0years). Serum complement C4 level was decreased in all patients. All patients had low C1\u2010esterase inhibitor (C1\u2010INH) quantitative level (type 1 HAE).\n                      <jats:italic>SERPING1<\/jats:italic>\n                      gene sequencing could be carried out in 20 families. Of these, 11 were identified to have a pathogenic disease\u2010causing variant in the\n                      <jats:italic>SERPING1<\/jats:italic>\n                      gene. While 2 of these families had a previously reported mutation, remaining 9 families had novel pathogenic variants in\n                      <jats:italic>SERPING1<\/jats:italic>\n                      gene. Because of non\u2010availability of C1\u2010INH therapy in India, all patients were given long\u2010term prophylaxis (attenuated androgens or tranexamic acid (TA) or a combination of the 2). Life\u2010threatening episodes of laryngeal edema were managed with fresh\u2010frozen plasma (FPP) infusions. We recorded one disease\u2010related mortality in our cohort. This happened in spite of long\u2010term prophylaxis with stanozolol and TA.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We report largest single\u2010center cohort of patients with HAE from India. Attenuated androgens, fibrinolytic agents, and FPP may be used for management of HAE in resource\u2010limited settings.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.13420","type":"journal-article","created":{"date-parts":[[2020,11,21]],"date-time":"2020-11-21T14:31:43Z","timestamp":1605969103000},"page":"599-611","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Novel\n                    <i>SERPING1<\/i>\n                    gene mutations and clinical experience of type 1 hereditary angioedema from North India"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7954-0661","authenticated-orcid":false,"given":"Ankur K.","family":"Jindal","sequence":"first","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5875-2892","authenticated-orcid":false,"given":"Amit","family":"Rawat","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anit","family":"Kaur","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dhrubajyoti","family":"Sharma","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepti","family":"Suri","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anju","family":"Gupta","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ravinder","family":"Garg","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunil","family":"Dogra","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biman","family":"Saikia","sequence":"additional","affiliation":[{"name":"Department of Immunopathology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ranjana W.","family":"Minz","sequence":"additional","affiliation":[{"name":"Department of Immunopathology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surjit","family":"Singh","sequence":"additional","affiliation":[{"name":"Allergy Immunology Unit Department of Paediatrics Advanced Paediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,12,5]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1808012"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/1939-4551-4-S2-S9"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.2500\/aap.2015.36.3806"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000832"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anai.2012.10.008"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.13001"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.13384"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gendis.2019.07.002"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-018-0815-5"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/ddg.13815_g"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)60584-4"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0233-x"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jfma.2015.12.006"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1177\/0961203320935980"},{"key":"e_1_2_12_16_1","first-page":"187","article-title":"Hereditary angioneurotic edema","volume":"36","author":"Baranwal AK","year":"1999","journal-title":"Indian Pediatr"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12262-011-0255-0"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.4103\/0972-0707.181949"},{"key":"e_1_2_12_19_1","first-page":"524","article-title":"Recurrent intestinal obstruction with acquired angio\u2010oedema, due to C1\u2010esterase inhibitor deficiency","volume":"62","author":"Desai HG","year":"2014","journal-title":"J Assoc Physicians India"},{"key":"e_1_2_12_20_1","first-page":"927","article-title":"Recurrent angio\u2010oedema\u2013three cases of C1 inhibitor deficiency","volume":"61","author":"Philip A","year":"2013","journal-title":"J Assoc Physicians India"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anai.2014.03.003"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.waojou.2019.100049"},{"key":"e_1_2_12_23_1","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1111\/ddg.14036","article-title":"Hereditary angioedema in a single family with specific mutations in both plasminogen and SERPING1 genes","volume":"18","author":"Bork K","year":"2020","journal-title":"J Dtsch Dermatol Ges"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.13270"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2017.05.020"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.13869"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.2500\/aap.2019.40.4267"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1684\/ejd.2013.2105"},{"key":"e_1_2_12_29_1","first-page":"227","article-title":"Hereditary angioedema: a family study","volume":"23","author":"Huang Y\u2010T","year":"2005","journal-title":"Asian Pac J Allergy Immunol"},{"key":"e_1_2_12_30_1","first-page":"163","article-title":"Hereditary angioedema: a Taiwanese family with a novel gene mutation","volume":"25","author":"Wen D\u2010C","year":"2007","journal-title":"Asian Pac J Allergy Immunol"},{"key":"e_1_2_12_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anai.2015.03.010"},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.2012.04670.x"},{"key":"e_1_2_12_33_1","doi-asserted-by":"publisher","DOI":"10.1186\/1710-1492-6-18"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1398-9995.2010.02456.x"},{"key":"e_1_2_12_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2005.09.064"},{"key":"e_1_2_12_36_1","doi-asserted-by":"publisher","DOI":"10.1111\/pai.12168"},{"key":"e_1_2_12_37_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0604-6"},{"key":"e_1_2_12_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/pai.12444"},{"key":"e_1_2_12_39_1","doi-asserted-by":"publisher","DOI":"10.1177\/0009922815616886"},{"key":"e_1_2_12_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2014.11.012"},{"key":"e_1_2_12_41_1","doi-asserted-by":"publisher","DOI":"10.1111\/pai.12712"},{"key":"e_1_2_12_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2016.12.033"},{"key":"e_1_2_12_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61121-0"},{"key":"e_1_2_12_44_1","first-page":"139","article-title":"Hereditary angioedema due to c1\u2010inhibitor deficiency in pediatric patients in Croatia \u2013 first national study, diagnostic and prophylactic challenges","volume":"58","author":"Karad\u017ea\u2010Lapi\u0107 L","year":"2019","journal-title":"Acta Clin Croat"},{"key":"e_1_2_12_45_1","doi-asserted-by":"publisher","DOI":"10.1159\/000366276"},{"key":"e_1_2_12_46_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-2274"},{"key":"e_1_2_12_47_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-016-0423-1"},{"key":"e_1_2_12_48_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2007.03438.x"},{"key":"e_1_2_12_49_1","doi-asserted-by":"publisher","DOI":"10.18176\/jiaci.0037"},{"key":"e_1_2_12_50_1","doi-asserted-by":"publisher","DOI":"10.2147\/IJWH.S46460"},{"key":"e_1_2_12_51_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.9407-17"},{"key":"e_1_2_12_52_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13223-019-0376-8"},{"key":"e_1_2_12_53_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v16.i39.4913"},{"key":"e_1_2_12_54_1","first-page":"333","article-title":"Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis","volume":"21","author":"Caballero T","year":"2011","journal-title":"J Investig Allergol Clin Immunol"},{"key":"e_1_2_12_55_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-2233"},{"key":"e_1_2_12_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)60886-1"},{"key":"e_1_2_12_57_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0230128"},{"key":"e_1_2_12_58_1","doi-asserted-by":"publisher","DOI":"10.2500\/ar.2015.6.0112"},{"key":"e_1_2_12_59_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0142174"},{"key":"e_1_2_12_60_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0056712"},{"key":"e_1_2_12_61_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9029"},{"key":"e_1_2_12_62_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.12941"},{"key":"e_1_2_12_63_1","doi-asserted-by":"publisher","DOI":"10.1111\/ahg.12052"},{"key":"e_1_2_12_64_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molimm.2012.09.003"},{"key":"e_1_2_12_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2014.08.007"},{"key":"e_1_2_12_66_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9202"},{"key":"e_1_2_12_67_1","doi-asserted-by":"publisher","DOI":"10.1080\/07853890.2018.1449959"},{"key":"e_1_2_12_68_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-020-00753-2"}],"container-title":["Pediatric Allergy and Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.13420","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pai.13420","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.13420","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T19:52:19Z","timestamp":1693338739000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pai.13420"}},"subtitle":[],"editor":[{"given":"Marina","family":"Atanaskovic\u2010Markovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2020,12,5]]},"references-count":67,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2021,4]]}},"alternative-id":["10.1111\/pai.13420"],"URL":"https:\/\/doi.org\/10.1111\/pai.13420","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.1111\/PAI.13420\/v2\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v2\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v1\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v3\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v2\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v3\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1111\/PAI.13420\/v1\/decision1","asserted-by":"object"}]},"ISSN":["0905-6157","1399-3038"],"issn-type":[{"value":"0905-6157","type":"print"},{"value":"1399-3038","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,12,5]]},"assertion":[{"value":"2020-07-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-11-16","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-12-05","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}