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Background and Aims The recipients who have antibodies againts to donors’ HLA (Anti HLA- DSA) carrie a huge risk for allograf (AG) losing. In current study, it was retrospectively evaluated whether the live donor kidney recipients (LDKRs) under desensitization (DS) were exposed to more AG loss, lower AG survival and more complications. Method In 17 of 269 LDKRs had high anti-HLA DSA were found prior to kidney transplantation (KTX). DS was performed with plasmapheresis (PF) and rituximab (RTX) before KTX. In order to determine the risks of DS, LDKRs who had not PF for any reason, were admitted immediately before and after those with LDKRs under DS were used as controls (n: 31). Immucor™ luminex method was used for detecting the anti HLA DSA. DS was performed to the Rs who had more than 5000 MFI DSA against to at least one donor HLA locus with double check. Non-parametric Fisher’s exact test was used for statistical analysis because of distribution of our patients was heterogeneous. Results In 15 Rs had DSA more than 2 HLA loci (MFI range: 5880-21712), remaining 2 Rs had one HLA locus DSA (9959-7762). Mean PF number was of 7 before TX, while 3 after TX. RTX was used in 9 Rs (53%) before TX. KTX performed when anti DSA MFI below 2000 under triple immunosuppression plus anti-thymocitic globulin (ATG) induction therapy. In 4 Rs (13%) had DSA below 5000 MFI among controls (Cs). More female Rs (88% vs. 19%, p0.05), rate of indication biopsy (24 % vs 16 %, p>0.05), rate of CNI toxicity (5.5% vs. 6.5%, p>0.05) were indifferent. During whole follow-up period, creatinine levels and eGFR were compatible at both groups. Urinary infections were more frequent in Rs under DS (53% vs 6.5%, p Conclusion Although our results belong to a single center and also a small group, desensitization before kidney transplantation for high risk kidney recipients are seem to be useful, effective and successful procedure.

Background and Aims Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in the adult population. The aim of our study is to determine the demographic and clinical characteristics and biopsy findings of the patients diagnosed with MN in our country. Method The study included 991 patients with renal biopsy who were followed in 47 nephrology clinics and enrolled in the database of Turkish Nephrology Primary Glomerular Diseases (TSN-GOLD) Working Group between May 1, 2009, and June 1, 2019. The demographic and clinical characteristics and biopsy findings of all patients included in the study were analyzed. Results The mean age of patients included in our study was 46±14.8 years and the gender distribution was (M / F, 582/409). 80.3% (n = 796) of the patients had nephrotic proteinuria at the time of diagnosis. Before the biopsy, the PLAR2 antibody was examined in 137 patients and positive in 71.5% (n = 98) patients. Hypertension was present in 34.6% (n = 343) of the patients. Mean estimated glomerular filtration rate was 93.9±32.8 ml/min/1.73 m2, albumin level was 2.7 ± 0.2 mg/dL, proteinuria level in urine was 7,1±6,4 gr/day and total cholesterol level was 295±3.7 mg/dL. On light microscopy, baseline membrane thickening was found in 92% (n=907) and interstitial inflammation in 53% (n=524) specimens. In immunofluorescence staining, 3 (+) IgG accumulation was highest in 578 (58.3%) of the specimen. Conclusion This multicentre study is the largest study to analyze clinical, laboratory, and biopsy specimens of MN patients at the time of biopsy in our country. The findings are consistent with the literature. Prospective follow-up results of these patients will help us to understand the renal prognosis and treatment response better.

Alkaptonuria (AKU; McKusick No. 203500), a rare hereditary disorder of the phenylalanine catabolism, was the first disease to be interpreted as an inborn error of metabolism (A. E. Garrod, 1902, Lancet 2: 1616-1620). AKU patients are deficient for homogentisate 1,2-dioxygenase (HGO; EC 1.13.11.5). This enzymatic deficiency causes homogentisic aciduria, ochronosis, and arthritis. Recently we cloned the human HGO gene and showed that AKU patients carry two copies of a loss-of-function HGO allele. Here we describe the complete nucleotide sequence of the human HGO gene and the identification of its promoter region. The human HGO gene spans 54,363 bp and codes for a 1715-nt-long transcript that is split into 14 exons ranging from 35 to 360 bp. The HGO introns, 605 to 17,687 bp in length, contain representatives of the major classes of repetitive elements, including several simple sequence repeats (SSR). Two of these SSRs, a (CT)n repeat in intron 4 and a (CA)n repeat in intron 13, were found to be polymorphic in a Spanish population sample. The HGO transcription start site was determined by primer extension. We report that sequences from -1074 to +89 bp (relative to the HGO transcription start site) are sufficient to promote transcription of a CAT reporter gene in human liver cells and that this fragment contains putative binding sites for liver-enriched transcription factors that might be involved in the regulation of HGO expression in liver.