{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,2]],"date-time":"2026-03-02T11:16:06Z","timestamp":1772450166079,"version":"3.50.1"},"reference-count":16,"publisher":"Wiley","issue":"5-6","license":[{"start":{"date-parts":[[2022,2,1]],"date-time":"2022-02-01T00:00:00Z","timestamp":1643673600000},"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":["Clinical Genetics"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Multinational studies have reported monogenic etiologies in 25%\u201330% of children with steroid\u2010resistant nephrotic syndrome. Such large studies are lacking in Asia. We established Deciphering Diversities: Renal Asian Genetics Network (DRAGoN) and aimed to describe the genetic and clinical spectrums in Asians. We prospectively studied a cohort of 183 probands with suspected genetic glomerulopathies from South and Southeast Asia, of whom 17% had positive family history. Using multi\u2010gene panel sequencing, we detected pathogenic variants in 26 (14%) probands, of whom one\u2010third had <jats:italic>COL4A4<\/jats:italic> or <jats:italic>COL4A5<\/jats:italic> variants (<jats:italic>n<\/jats:italic>\u00a0=\u00a09, 5%). Of those with <jats:italic>COL4A5<\/jats:italic> defects, only 25% had features suggestive of Alport syndrome. Besides traditional predictors for genetic disease (positive family history and extrarenal malformations), we identified novel predictors, namely older age (6.2 vs. 2.4\u2009years; <jats:italic>p<\/jats:italic>\u00a0=\u00a00.001), hematuria (OR 5.6; 95% CI 2.1\u201314.8; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), and proteinuria in the absence of nephrotic syndrome (OR 4.6; 95% CI 1.8\u201311.8; <jats:italic>p<\/jats:italic>\u00a0=\u00a00.001) at first manifestation. Among patients who first presented with proteinuria without nephrotic syndrome, the genetic diagnostic rates were &gt;60% when a second risk factor (positive family history or extrarenal manifestation) co\u2010existed. The genetic spectrum of glomerulopathies appears different in Asia. Collagen IV genes may be included in sequencing panels even when suggestive clinical features are absent.<\/jats:p>","DOI":"10.1111\/cge.14116","type":"journal-article","created":{"date-parts":[[2022,1,22]],"date-time":"2022-01-22T13:49:54Z","timestamp":1642859394000},"page":"541-551","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Multicenter study on the genetics of glomerular diseases among southeast and south Asians: Deciphering Diversities \u2010 Renal Asian Genetics Network (DRAGoN)"],"prefix":"10.1111","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8363-4353","authenticated-orcid":false,"given":"Liangjian","family":"Lu","sequence":"first","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yok\u2010Chin","family":"Yap","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Hospital Kuala Lumpur  Kuala Lumpur Malaysia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Duc Quang","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Nephrology and Endocrinology Department Children's Hospital 1  Ho Chi Minh Vietnam"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yiong\u2010Huak","family":"Chan","sequence":"additional","affiliation":[{"name":"Biostatistics National University of Singapore  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun\u2010Li","family":"Ng","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System  Singapore Singapore"},{"name":"Paediatrics National University of Singapore  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3220-3788","authenticated-orcid":false,"given":"Yao\u2010Chun","family":"Zhang","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System  Singapore Singapore"},{"name":"Paediatrics National University of Singapore  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chang\u2010Yien","family":"Chan","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System  Singapore Singapore"},{"name":"Paediatrics National University of Singapore  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mya","family":"Than","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System  Singapore Singapore"},{"name":"Paediatrics National University of Singapore  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isaac Desheng","family":"Liu","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sadaf","family":"Asim","sequence":"additional","affiliation":[{"name":"Paediatric Nephrology National Institute of Child Health  Karachi Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khemchand","family":"Moorani","sequence":"additional","affiliation":[{"name":"Paediatric Nephrology National Institute of Child Health  Karachi Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bilquis","family":"Naeem","sequence":"additional","affiliation":[{"name":"Paediatric Nephrology National Institute of Child Health  Karachi Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iftikhar","family":"Ijaz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics King Edward Medical University  Lahore Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thi Minh Tan","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Children Hospital 2  Ho Chi Minh Vietnam"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming\u2010Lee","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Hospital Tuanku Ja'afar Seremban  Seremban Malaysia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Eng","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Hospital Tuanku Ja'afar Seremban  Seremban Malaysia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Syed Saimul","family":"Huque","sequence":"additional","affiliation":[{"name":"Paediatric Nephrology Bangabandhu Sheikh Mujib Medical University  Dhaka Bangladesh"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yong\u2010Hong","family":"Ng","sequence":"additional","affiliation":[{"name":"Paediatrics KK Women's and Children's Hospital  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Indra","family":"Ganesan","sequence":"additional","affiliation":[{"name":"Paediatrics KK Women's and Children's Hospital  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sing\u2010Ming","family":"Chao","sequence":"additional","affiliation":[{"name":"Paediatrics KK Women's and Children's Hospital  Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siew\u2010Le","family":"Chong","sequence":"additional","affiliation":[{"name":"Paediatrics KK Women's and Children's Hospital  Singapore 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