{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T01:59:04Z","timestamp":1760234344042,"version":"build-2065373602"},"reference-count":18,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Reports"],"abstract":"<jats:p>Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous disorder. It is characterized by the presence of nevus sebaceous, ocular anomalies, neurological deficits, and convulsion. Renal involvement was not commonly reported. We report a 10-year-old girl with LNSS who had concomitant cystic kidney disease and diffuse aortopathy with bilateral renal artery stenosis, leading to hypertension requiring oral anti-hypertensive medications. The girl presented with chorioretinal coloboma and multiple nevus sebaceous at birth. She had aortic coarctation and received surgical repair at one week of life. She had persistent hypertension during her follow-up. Further investigations were performed to look for causes of hypertension apart from possible re-coarctation. Her magnetic resonance angiogram revealed diffuse aortopathy, which extended from the aortic arch to the abdominal aorta. Branches of the aorta, including the celiac trunk, superior mesenteric arteries, and renal arteries, were also narrowed. Multiple renal cysts were also identified in her right kidney. Interventional angioplasty over the renal arteries was not feasible due to diffuse narrowing of the aorta, especially at the origins of renal arteries. The blood pressure was controlled with oral anti-hypertensive medications. Our case illustrated that pediatricians should be aware of the possible renal involvements in LNSS, which impose a significant impact on the management and long-term prognosis of these patients.<\/jats:p>","DOI":"10.3390\/pediatric13020028","type":"journal-article","created":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T21:35:39Z","timestamp":1619904939000},"page":"203-209","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Renal Involvement in Linear Nevus Sebaceous Syndrome\u2014An Underrecognized Feature"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8608-350X","authenticated-orcid":false,"given":"Chonin","family":"Kuok","sequence":"first","affiliation":[{"name":"Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3763-9022","authenticated-orcid":false,"given":"Kwaiyu","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Jiang, L., Gao, F., Mao, S., Xu, J., and Jiang, K. (2017). Somatic KRAS mutation in an infant with linear nevus sebaceous syndrome associated with lymphatic malformations: A case report and literature review. Medicine, 96.","DOI":"10.1097\/MD.0000000000008016"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jaad.2010.01.017","article-title":"The group of epidermal nevus syndromes Part I. Well defined phenotypes","volume":"63","author":"Happle","year":"2010","journal-title":"J. Am. Acad Dermatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/B978-0-444-52891-9.00041-5","article-title":"Epidermal nevus syndrome","volume":"111","author":"Laura","year":"2013","journal-title":"Handb. Clin. Neurol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/j.jaad.2009.04.058","article-title":"Sebaceous lesions and their associated syndromes: Part I","volume":"61","author":"Eisen","year":"2009","journal-title":"J. Am. Acad Dermatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.sder.2008.03.006","article-title":"Epidermal nevus syndromes","volume":"26","author":"Sugarman","year":"2007","journal-title":"Semin. Cutan. Med. Surg."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/S0303-8467(98)00012-2","article-title":"The linear naevus sebaceus syndrome","volume":"100","author":"Renier","year":"1998","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_7","first-page":"220","article-title":"Ophthalmic manifestations of linear nevus sebaceous\/organoid nevus syndrome","volume":"25","author":"Kausar","year":"2015","journal-title":"J. Coll. Physicians Surg. Pak."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1302\/0301-620X.85B4.13483","article-title":"Linear sebaceous naevus syndrome and resistant rickets","volume":"85","author":"Hosalkar","year":"2003","journal-title":"J. Bone Jt. Surg. Br. Vol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1597\/06-025.1","article-title":"Schimmelpenning Syndrome: An Association with Vascular Anomalies","volume":"44","author":"Greene","year":"2007","journal-title":"Cleft Palate-Craniofacial J."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1055\/s-2000-7457","article-title":"CNS Lipoma in Patients with Epidermal Nevus Syndrome","volume":"31","author":"Mall","year":"2000","journal-title":"Neuropediatrics"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1038\/s10038-018-0539-3","article-title":"A postzygotic KRAS mutation in a patient with Schimmelpenning syndrome presenting with lipomatosis, renovascular hypertension, and diabetes mellitus","volume":"64","author":"Nagatsuma","year":"2019","journal-title":"J. Hum. Genet."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1159\/000045540","article-title":"Renal artery stenosis associated with epidermal nevus syndrome","volume":"84","author":"Aizawa","year":"2000","journal-title":"Nephron"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2081","DOI":"10.1007\/s00467-011-1949-z","article-title":"Bilateral renal artery stenosis and epidermal nevus syndrome in a child","volume":"26","author":"Alsohim","year":"2011","journal-title":"Pediatr. Nephrol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1093\/qjmed\/hcu089","article-title":"Vascular manifestations and post-treatment changes of epidermal nevus syndrome","volume":"108","author":"Juan","year":"2014","journal-title":"QJM Int. J. Med."},{"key":"ref_15","first-page":"647","article-title":"Epidermal nevus syndrome and dysplatic kidney disease","volume":"52","author":"Nickavar","year":"2014","journal-title":"Acta Med. Iran."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1136\/jmg.2009.075374","article-title":"Mosaicism for oncogenic G12D KRAS mutation associated with epidermal nevus, polycystic kidneys and rhabdomyosarcoma","volume":"47","author":"Bourdeaut","year":"2010","journal-title":"J. Med. Genet."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"600","DOI":"10.3171\/2013.1.PEDS12451","article-title":"Spinal arteriovenous malformation associated with Schimmelpenning syndrome","volume":"11","author":"Almefty","year":"2013","journal-title":"J. Neurosurg. Pediatr."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"868","DOI":"10.1001\/archpedi.1974.02110310116023","article-title":"Linear Sebaceous Nevus Syndrome in a Newborn","volume":"128","author":"Mollica","year":"1974","journal-title":"Arch. Pediatr. Adolesc. Med."}],"container-title":["Pediatric Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2036-7503\/13\/2\/28\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:56:37Z","timestamp":1760162197000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2036-7503\/13\/2\/28"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,1]]},"references-count":18,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2021,6]]}},"alternative-id":["pediatric13020028"],"URL":"https:\/\/doi.org\/10.3390\/pediatric13020028","relation":{},"ISSN":["2036-7503"],"issn-type":[{"type":"electronic","value":"2036-7503"}],"subject":[],"published":{"date-parts":[[2021,5,1]]}}}