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Most children with NS are steroid\u2010responsive and have a good prognosis following treatment with prednisolone. However, 10%\u201320% of them have steroid\u2010resistant nephrotic syndrome (SRNS) and fail to respond to treatment. A significant proportion of these children progress to kidney failure.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective study aimed to determine the underlying genetic causes of SRNS among Omani children below 13\u2009years old, over a 15\u2010year period and included 77 children from 50 different families. We used targeted Sanger sequencing combined with next\u2010generation sequencing approaches to perform molecular diagnostics.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found a high rate of underlying genetic causes of SRNS in 61 (79.2%) children with pathogenic variants in the associated genes. Most of these genetically solved SRNS patients were born to consanguineous parents and variants were in the homozygous state. Pathogenic variants in <jats:italic>NPHS2<\/jats:italic> were the most common cause of SRNS in our study seen in 37 (48.05%) cases. Pathogenic variants in <jats:italic>NPHS1<\/jats:italic> were also seen in 16 cases, especially in infants with congenital nephrotic syndrome (CNS). Other genetic causes identified included pathogenic variants in <jats:italic>LAMB2<\/jats:italic>, <jats:italic>PLCE1<\/jats:italic>, <jats:italic>MYO1E<\/jats:italic>, and <jats:italic>NUP93<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p><jats:italic>NPHS2<\/jats:italic> and <jats:italic>NPHS1<\/jats:italic> genetic variants were the most common inherited causes of SRNS in Omani children. However, patients with variants in several other SRNS causative genes were also identified. We recommend screening for all genes responsible for SRNS in all children who present with this phenotype, which will assist in clinical management decisions and genetic counseling for the affected families.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mgg3.2201","type":"journal-article","created":{"date-parts":[[2023,5,19]],"date-time":"2023-05-19T11:15:46Z","timestamp":1684494946000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Genetic analysis and outcomes of Omani children with steroid\u2010resistant nephrotic syndrome"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3698-5609","authenticated-orcid":false,"given":"Mohamed S.","family":"Al\u00a0Riyami","sequence":"first","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Child Healthy Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Intisar","family":"Al\u00a0Alawi","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Faculty of Medical Science Newcastle University  Newcastle upon Tyne UK"},{"name":"National Genetic Center, Ministry of Health  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Badria","family":"Al\u00a0Gaithi","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Child Healthy Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anisa","family":"Al\u00a0Maskari","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Child Healthy Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naifain","family":"Al\u00a0Kalbani","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Child Healthy Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Al\u00a0Hashmi","sequence":"additional","affiliation":[{"name":"Pediatric Metabolic and Genetic Disorder Unit Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aisha","family":"Al\u00a0Balushi","sequence":"additional","affiliation":[{"name":"Pediatric Metabolic and Genetic Disorder Unit Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryam","family":"Al\u00a0Shahi","sequence":"additional","affiliation":[{"name":"Pediatric Clinical Genetic Unit, Royal Hospital, Department of Child Health Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suliman","family":"Al\u00a0Saidi","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Child Healthy Royal Hospital  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muna","family":"Al\u00a0Bimani","sequence":"additional","affiliation":[{"name":"National Genetic Center, Ministry of Health  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fahad","family":"Al\u00a0Hatali","sequence":"additional","affiliation":[{"name":"National Genetic Center, Ministry of Health  Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6725-7570","authenticated-orcid":false,"given":"Holly","family":"Mabillard","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Faculty of Medical Science Newcastle University  Newcastle upon Tyne UK"},{"name":"Renal Services The Newcastle upon Tyne Hospitals NHS Foundation Trust  Newcastle upon Tyne UK"},{"name":"Newcastle Biomedical Research Center, NIHR  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1881-3782","authenticated-orcid":false,"given":"John A.","family":"Sayer","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Faculty of Medical Science Newcastle University  Newcastle upon Tyne UK"},{"name":"Renal Services The Newcastle upon Tyne Hospitals NHS Foundation Trust  Newcastle upon Tyne UK"},{"name":"Newcastle Biomedical Research Center, NIHR  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,5,19]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.40338.2"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1155\/2017\/6403985"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ekir.2019.08.012"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12882-020-02013-2"},{"key":"e_1_2_11_6_1","first-page":"263","article-title":"Kidney disease in Oman: A view of the current and future landscapes","volume":"11","author":"Al Alawi I. 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