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Copyright of Cukurova Medical Journal / Çukurova Üniversitesi Tip Fakültesi Dergisi is the property of Cukurova University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Ege Journal of Medicine is the property of Ege University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Renal involvement is a common manifestation of systemic amyloidosis. Amyloid load can be predicted by histopathological grading of amyloid deposits in renal biopsy specimens. This study aimed to determine the relationship of renal amyloid deposition grade with clinical manifestations and outcomes in patients with biopsy-proven renal non-AA amyloidosis. Methods: This retrospective cohort study included 74 subjects with renal non-AA amyloidosis (52 light chain amyloidosis and 22 unclassified amyloidosis). Baseline characteristics and follow-up data were recorded. Pattern and quantity of amyloid deposition in glomeruli, interstitium, vessels, and tubulointerstitial changes were scored. Renal Amyloid Prognostic Score was obtained by addition of all scores and divided into 3 grades (Renal Amyloid Prognostic Score grades I, II, III). Results: In light chain amyloidosis group, the median follow-up was 11 (4-45) months. The baseline estimated glomerular filtration rate was significantly lower among patients with Renal Amyloid Prognostic Score grade III. Death-censored Renal survival was significantly lower among patients with Renal Amyloid Prognostic Score grade III. Renal Amyloid Prognostic Score grade III was a significant predictor of lower renal survival. Patient survival was not significantly different according to Renal Amyloid Prognostic Score grade. Receiving autologous stem cell transplantation treatment was associated with better patient survival. The type of amyloid could not be determined in 22 patients. In this group, baseline estimated glomerular filtration rate was significantly lower in patients with Renal Amyloid Prognostic Score grade III. Conclusions: In patients with light chain amyloidosis, baseline renal function is associated with Renal Amyloid Prognostic Score grade. Renal survival is significantly lower in patients with the highest Renal Amyloid Prognostic Score grade. However, patient survival is not significantly different according to Renal Amyloid Prognostic Score grade. [ABSTRACT FROM AUTHOR]

Glycogen storage disorder (GSD) IXb is characterized by liver and muscle involvement. We present a GSD IXb patient with an incidental union of nephrotic syndrome. A 4 year-old-patient was diagnosed with GSD IXb at 13 months of age with mildly elevated transaminases and hepatomegaly. During the follow-up period, there was no hypoglycemia. Development and growth were normal. In the last month, the onset of generalized edema was reported. Urinalysis showed a high protein level. He had low serum albumin, high serum triglycerides cholesterol. Complement levels were normal. The patient was diagnosed as minimal change disease with a renal biopsy. He was treated with oral prednisone. Minimal Change Disease is the most common cause of idiopathic nephrotic syndrome cases in children, and the first step for therapy is the usage of corticosteroids. This is the first report of nephrotic syndrome associated with GSD IXb disease. [ABSTRACT FROM AUTHOR]