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Immune-mediated acquired lecithin-cholesterol acyltransferase deficiency: A case report and literature review by Ryoichi Ishibashi; Minoru Takemoto; Yuya Tsurutani; Masayuki Kuroda; Makoto Ogawa; Hanae Wakabayashi; Noriko Uesugi; Michio Nagata; Naofumi Imai; Akiko Hattori; Kenichi Sakamoto; Takumi Kitamoto; Yoshiro Maezawa; Ichiei Narita; Sadayuki Hiroi; Ayaka Furuta; Takashi Miida; Koutaro Yokote is a Medicine article available to read on EtoBox.
What is Immune-mediated acquired lecithin-cholesterol acyltransferase deficiency: A case report and literature review about?
## Background: Recessive inherited disorder lecithin-cholesterol acyltransferase (lcat) deficiency causes severe hypocholesterolemia and nephrotic syndrome. characteristic lipoprotein subfractions have been observed in familial lcat deficiency (fld) with renal damage. ## Objective: We described a case of acquired lcat deficiencies with literature review. ## Methods: The lipoprotein profiles examined by gel permeation-high-performance liquid chromatography (gp-hplc) and native 2-dimensional electrophoresis before and after prednisolone (psl) treatment. ## Results: Here we describe the case of a 67-year-old man with severely low levels of cholesterol. the serum lcat activity was undetectable, and autoantibodies against it were detected. the patient developed nephrotic syndrome at the age of 70 years. renal biopsy revealed not only membranous glomerulonephritis but also lesions similar to those seen in fld. we initiated psl treatment, which resulted in remission of the nephrotic syndrome. in gp-hplc analysis, lipoprotein profile was similar to that of fld although lipoprotein x level was low. acquired lcat deficiencies are extremely rare with only 7 known cases including ours. patient
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- Author
- Ryoichi Ishibashi; Minoru Takemoto; Yuya Tsurutani; Masayuki Kuroda; Makoto Ogawa; Hanae Wakabayashi; Noriko Uesugi; Michio Nagata; Naofumi Imai; Akiko Hattori; Kenichi Sakamoto; Takumi Kitamoto; Yoshiro Maezawa; Ichiei Narita; Sadayuki Hiroi; Ayaka Furuta; Takashi Miida; Koutaro Yokote
- Publisher
- Elsevier ; Elsevier BV (ISSN 1933-2874)
- Published
- 2018
- Language
- EN
- Field
- Medicine (Health Sciences)