{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T00:10:33Z","timestamp":1793405433929,"version":"4.5.0"},"reference-count":9,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Currently, there is no cure for epidermolysis bullosa (EB) but few studies have explored the role of aminoglycosides in promoting collagen 7 expression in recessive dystrophic EB (RDEB).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Materials and Methods:<\/jats:title>\n                    <jats:p>Consecutive patients aged &gt;1 year with a confirmed diagnosis of dystrophic EB (DEB) were advised to apply 0.1% w\/w gentamicin cream in a collagen base (Derbriment G\u2122) twice daily on a representative area on right lower limb (RLL) and paraffin gauze dressings on the corresponding opposite side on the left lower limb (LLL). Skin lesions were evaluated clinically during the 12-week treatment period at the end of which a repeat skin biopsy was sent for immunofluorescence antigen mapping (IFM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Twelve patients with DEB were recruited but only eight completed the study and were analyzed. The mean fluorescence intensity (MFI) of the study cohort increased from 2765 \u00b1 1732.07 (263\u20134845) at baseline to 5412.75 \u00b1 3937.64 (2100\u201313536) at 12 weeks; a 95.75% (range 5.34%\u2013775.14%) increase in the MFI of collagen 7 from baseline (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = 0.06). Among patients with a known termination codon mutation (\n                      <jats:italic toggle=\"yes\">n<\/jats:italic>\n                      = 3), the percentage increase in MFI was greater among patients with known premature termination codon (PTC) mutations compared to those with unknown mutations. The clinical severity did not change significantly in terms of the mean number of blisters, erosions, and scarring during the study period. None of the parents reported any adverse effect.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Topical gentamicin 0.1% w\/w is a safe and effective way to promote the expression of COL7A1 in DEB patients, especially those carrying PTC mutations.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/idoj.idoj_554_21","type":"journal-article","created":{"date-parts":[[2022,6,24]],"date-time":"2022-06-24T04:22:48Z","timestamp":1656044568000},"page":"480-483","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":9,"title":["Topical Gentamicin 0.1% Promotes Collagen 7 Expression in Recessive Dystrophic Epidermolysis Bullosa"],"prefix":"10.4103","volume":"13","author":[{"given":"Rahul","family":"Mahajan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seema","family":"Manjunath","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manoj G.","family":"Madakshira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dipankar","family":"De","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjeev","family":"Handa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debajyoti","family":"Chatterjee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bishan D.","family":"Radotra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-6-20240828","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1111\/bjd.18921","article-title":"Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility","volume":"183","author":"Has","year":"2020","journal-title":"Br J Dermatol"},{"key":"R2-6-20240828","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1007\/s00109-002-0363-1","article-title":"Aminoglycoside suppression of a premature stop mutation in a Cftr-\/- mouse carrying a human CFTR-G542X transgene","volume":"80","author":"Du","year":"2002","journal-title":"J Mol Med (Berl"},{"key":"R3-6-20240828","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1002\/1531-8249(200008)48:2<164::AID-ANA5>3.0.CO;2-B","article-title":"Sequence specificity of aminoglycoside-induced stop condon readthrough:Potential implications for treatment of Duchenne muscular dystrophy","volume":"48","author":"Howard","year":"2000","journal-title":"Ann Neurol"},{"key":"R4-6-20240828","doi-asserted-by":"crossref","first-page":"1741","DOI":"10.1038\/mt.2014.140","article-title":"Aminoglycosides restore full-length type VII collagen by overcoming premature termination codons:Therapeutic implications for dystrophic epidermolysis bullosa","volume":"22","author":"Cogan","year":"2014","journal-title":"Mol Ther"},{"key":"R5-6-20240828","doi-asserted-by":"crossref","first-page":"3028","DOI":"10.1172\/JCI92707","article-title":"Gentamicin induces functional type VII collagen in recessive dystrophic epidermolysis bullosa patients","volume":"127","author":"Woodley","year":"2017","journal-title":"J Clin Invest"},{"key":"R6-6-20240828","doi-asserted-by":"crossref","first-page":"E6536","DOI":"10.1073\/pnas.1803154115","article-title":"Gentamicin induces LAMB3 nonsense mutation readthrough and restores functional laminin 332 in junctional epidermolysis bullosa","volume":"115","author":"Lincoln","year":"2018","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R7-6-20240828","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1159\/000499906","article-title":"Attenuation of severe generalized junctional epidermolysis bullosa by systemic treatment with gentamicin","volume":"235","author":"Hammersen","year":"2019","journal-title":"Dermatology"},{"key":"R8-6-20240828","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1016\/j.ymthe.2020.03.006","article-title":"Gentamicin induces Laminin 332 and improves wound healing in junctional epidermolysis bullosa patients with nonsense mutations","volume":"28","author":"Kwong","year":"2020","journal-title":"Mol Ther"},{"key":"R9-6-20240828","doi-asserted-by":"crossref","first-page":"244","DOI":"10.4103\/idoj.IDOJ_287_18","article-title":"Newer treatment modalities in epidermolysis bullosa","volume":"10","author":"Bruckner-Tuderman","year":"2019","journal-title":"Indian Dermatol Online J"}],"container-title":["Indian Dermatology Online Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/idoj.idoj_554_21","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T15:02:25Z","timestamp":1787756545000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/idoj.idoj_554_21"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7]]},"references-count":9,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/idoj.idoj_554_21","relation":{},"ISSN":["2229-5178"],"issn-type":[{"value":"2229-5178","type":"print"}],"subject":[],"published":{"date-parts":[[2022,7]]}}}