{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T06:57:37Z","timestamp":1761807457466},"reference-count":19,"publisher":"Oxford University Press (OUP)","issue":"757","license":[{"start":{"date-parts":[[1988,11,1]],"date-time":"1988-11-01T00:00:00Z","timestamp":594345600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1988,11,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>Cuticular drusen and retinal pigment epithelial changes were found incidentally in a 27 year old Lebanese woman during assessment of partial lipodystrophy. Her vision was normal despite involvement of both maculae. The patient had hypocomplementaemia, but serum C3 nephritic factor was absent and renal function was normal. She had impaired glucose tolerance and a continuous infusion of glucose with model assessment (CIGMA) test revealed low normal tissue insulin sensitivity and high normal pancreatic beta cell function. Mild fasting hypertriglyceridaemia (2.0 mmol\/l) may have been secondary to impaired insulin sensitivity. Endocrine function was otherwise normal apart from a completely absent growth hormone response to adequate hypoglycaemia. The simultaneous occurrence of partial lipodystrophy and retinal pigmentary epithelial and basement membrane changes appears to be a newly recognized syndrome.<\/jats:p>","DOI":"10.1136\/pgmj.64.757.871","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T21:05:27Z","timestamp":1230584727000},"page":"871-874","source":"Crossref","is-referenced-by-count":20,"title":["Retinal pigment epithelial change and partial lipodystrophy"],"prefix":"10.1093","volume":"64","author":[{"given":"T M E","family":"Davis","sequence":"first","affiliation":[{"name":"Department of Medicine Woodstock Road, Oxford OX2 6HE , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D R","family":"Holdright","sequence":"additional","affiliation":[{"name":"Department of Medicine Woodstock Road, Oxford OX2 6HE , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W E","family":"Schulenberg","sequence":"additional","affiliation":[{"name":"Department of Surgery, Royal Postgraduate Medical School, Hammersmith Hospital , Du Cane Road, London W12 OHS"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R C","family":"Turner","sequence":"additional","affiliation":[{"name":"Diabetes Research Laboratories, Radcliffe Infirmary , Woodstock Road, Oxford OX2 6HE , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G F","family":"Joplin","sequence":"additional","affiliation":[{"name":"Department of Medicine Woodstock Road, Oxford OX2 6HE , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1988,11,1]]},"reference":[{"issue":"90","key":"ref_1","first-page":"105","article-title":"Singular case of absence of adipose matter in the upper half of the body","volume":"1885","author":"Mitchell, S.W.","unstructured":"Mitchell, S.W. Singular case of absence of adipose matter in the upper half of the body. Am J Med Sci 1885, 90: 105-109. The patient described in this report illustrates a further association which should be considered in the management of such patients. The extensive deposition of material in the basement membrane of the retina is ppobably not coincidental and might be analogous to glomerular basement membrane changes seen in the mesangiocapillary glomerulonephritis which can be associated with partial lipodystrophy. Alternatively, the combination of retinal abnormalities, partial lipodystrophy with impaired insulin sensitivity, and hypocomplementaemia might be due to deletion of a short segment of chromosome.","journal-title":"Am J Med Sci; The patient described in this report illustrates a further association which should be considered in the management of such patients. The extensive deposition of material in the basement membrane of the retina is ppobably not coincidental and might be analogous to glomerular basement membrane changes seen in the mesangiocapillary glomerulonephritis which can be associated with partial lipodystrophy. Alternatively, the combination of retinal abnormalities, partial lipodystrophy with impaired insulin sensitivity, and hypocomplementaemia might be due to deletion of a short segment of chromosome"},{"key":"ref_2","article-title":"The young woman described in this report illustrates many of the features of a 'typical' patient with progressive partial lipodystrophy. In addition, she has evidence of retinal pigment epithelial and basement membrane changes, a hitherto unpublished association.* Although little is known of the aetiology and pathogenesis of the lipoatrophic disorders, a careful characterization of their clinical, biochemical and metabolic manifestations may provide valuable clues for future study","unstructured":"The young woman described in this report illustrates many of the features of a 'typical' patient with progressive partial lipodystrophy. In addition, she has evidence of retinal pigment epithelial and basement membrane changes, a hitherto unpublished association.* Although little is known of the aetiology and pathogenesis of the lipoatrophic disorders, a careful characterization of their clinical, biochemical and metabolic manifestations may provide valuable clues for future study."},{"issue":"33","key":"ref_3","first-page":"593","article-title":"The syndromes of total lipodystrophy and of partial lipodystrophy","volume":"1964","author":"Senior, B.; Gellis, S.S.","unstructured":"Senior, B. & Gellis, S.S. The syndromes of total lipodystrophy and of partial lipodystrophy. Pediatrics 1964, 33: 593-612.","journal-title":"Pediatrics"},{"key":"ref_4","first-page":"111","article-title":"Lipodystrophy of the extremities. A dominantly inherited syndrome associated with lipoatrophic diabetes","volume":"29","author":"Kobberling, J.Willms; B., Katterman; R.; Creutzfeldt, W.","year":"1975","unstructured":"Kobberling, J. Willms, B., Katterman, R. & Creutzfeldt, W. Lipodystrophy of the extremities. A dominantly inherited syndrome associated with lipoatrophic diabetes. Humangenetik 1975, 29: 111-120.","journal-title":"Humangenetik"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1136\/jcp.20.1.52","article-title":"Blood lipid levels, thyroid status, and glucose tolerance in progressive partial lipodystrophy","volume":"20","author":"Rifkind, R.M.; Boyle, A.J.; Gale, M.","year":"1967","unstructured":"Rifkind, R.M., Boyle, A.J. & Gale, M. Blood lipid levels, thyroid status, and glucose tolerance in progressive partial lipodystrophy. 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Lancet 1973, ii: 535-538.","journal-title":"Lancet"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"388","DOI":"10.2337\/diab.22.5.388","article-title":"Increased growth hormone in partial and total lipoatrophy","volume":"22","author":"Tzagournis, M.; George, J.; Herrold, J.","year":"1972","unstructured":"Tzagournis, M., George, J. & Herrold, J. Increased growth hormone in partial and total lipoatrophy. Diabetes 1972, 22: 388-396.","journal-title":"Diabetes"},{"key":"ref_9","first-page":"33","article-title":"Familial lipoatrophic diabetes with dominant transmission","volume":"49","author":"Dunnigen, M.G.; Cochrane, M.; Kelly, A.; Scott, J.W.","year":"1974","unstructured":"Dunnigen, M.G., Cochrane, M., Kelly, A. & Scott, J.W. Familial lipoatrophic diabetes with dominant transmission. Q J Med 1974, 49: 33-48.","journal-title":"Q J Med"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1007\/BF01222107","article-title":"Metabolic studies in familial partial lipodystrophy of the lower trunk and extremities","volume":"11","author":"Davidson, M.B.; Young, R.T.","year":"1975","unstructured":"Davidson, M.B. & Young, R.T. Metabolic studies in familial partial lipodystrophy of the lower trunk and extremities. Diabetologia 1975, 11: 561-568.","journal-title":"Diabetologia"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1210\/jcem-46-5-800","article-title":"Cephalothoracic lipodystrophy with hypocomplementaemic renal disease: discordance in identical twin sisters","volume":"46","author":"Bier, D.M.; O'Donnell, J.J.; Kaplan, S.A.","year":"1978","unstructured":"Bier, D.M., O'Donnell, J.J. & Kaplan, S.A. Cephalothoracic lipodystrophy with hypocomplementaemic renal disease: discordance in identical twin sisters. J Clin Endocrinol Metab 1978, 46: 800-807.","journal-title":"J Clin Endocrinol Metab"},{"key":"ref_12","first-page":"81","article-title":"Partial lipodystrophy and insulin-resistant diabetes","volume":"4","author":"Crome, P.; Keen, H.","year":"1978","unstructured":"Crome, P. & Keen, H. Partial lipodystrophy and insulin-resistant diabetes. Diabete Metab 1978, 4: 81-84.","journal-title":"Diabete Metab"},{"issue":"28","key":"ref_13","first-page":"401","article-title":"Continuous infusion of glucose with model assessment: measurement of insulin resistance and beta cell function in man","volume":"1985","author":"Hosker, J.P.; Matthews, D.R.; Rudenski, A.S.","unstructured":"Hosker, J.P., Matthews, D.R., Rudenski, A.S. et al. Continuous infusion of glucose with model assessment: measurement of insulin resistance and beta cell function in man. Diabetologia 1985, 28: 401-411.","journal-title":"Diabetologia"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1136\/bmj.294.6577.923","article-title":"Type II diabetes of early onset: a distinct clinical and genetic syndrome?","volume":"294","author":"O'Rahilly, S.P.; Spivey, R.S.; Holman, R.R.; Nugent, Z.; Clark, A.; Turner, R.C.","year":"1987","unstructured":"O'Rahilly, S.P., Spivey, R.S., Holman, R.R., Nugent, Z., Clark, A. & Turner, R.C. Type II diabetes of early onset: a distinct clinical and genetic syndrome? Br Med J 1987, 294: 923-928.","journal-title":"Br Med J"},{"issue":"4","key":"ref_15","first-page":"561","article-title":"Lipid disorders in diabetes","volume":"1981","author":"Goldberg, R.","unstructured":"Goldberg, R. Lipid disorders in diabetes. Diabetes Care 1981, 4: 561-572.","journal-title":"Diabetes Care"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1358","DOI":"10.1016\/S0022-2275(20)34453-9","article-title":"Changes in the sensitivity of lipogenesis in rat hepatocytes to hormones and precursors over the diurnal cycle and during longer-term starvation of donor animals","volume":"25","author":"Gibbons, G.F.; Pullinger, C.R.; Bjornsson, O.G.","year":"1984","unstructured":"Gibbons, G.F., Pullinger, C.R. & Bjornsson, O.G. Changes in the sensitivity of lipogenesis in rat hepatocytes to hormones and precursors over the diurnal cycle and during longer-term starvation of donor animals. 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Impaired growth hormone response to human pancreatic growth hormone-releasing factor [GRF (1-44)] in type 2 (noninsulin dependent) diabetes. Diabetologia 1984, 27: 529-532."},{"key":"ref_19","first-page":"757","article-title":"nephritic factor and clinically inapparent mesangiocapillary glomerulonephritis","volume":"3","author":"Bennett, W.M.; Bardana, E.J.; Wuepper, K.","year":"1977","unstructured":"Bennett, W.M., Bardana, E.J., Wuepper, K. et al. Partial lipodystrophy, C3 nephritic factor and clinically inapparent mesangiocapillary glomerulonephritis. Am J Med 1977, 62: 757-760.","journal-title":"et al. Partial lipodystrophy"}],"container-title":["Postgraduate Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/64\/757\/871\/50079023\/postgradmedj-64-871.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/64\/757\/871\/50079023\/postgradmedj-64-871.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,5]],"date-time":"2023-05-05T04:49:25Z","timestamp":1683262165000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/pmj\/article\/64\/757\/871\/7047960"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1988,11,1]]},"references-count":19,"journal-issue":{"issue":"757","published-online":{"date-parts":[[1988,11,1]]},"published-print":{"date-parts":[[1988,11,1]]}},"URL":"https:\/\/doi.org\/10.1136\/pgmj.64.757.871","relation":{},"ISSN":["0032-5473","1469-0756"],"issn-type":[{"value":"0032-5473","type":"print"},{"value":"1469-0756","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[1988,11]]},"published":{"date-parts":[[1988,11,1]]}}}