Medical Challenges of a Common Variable Immunodeficiency With a TNFRSF13B Gene Mutation in a Simultaneous Kidney and Pancreas Transplant Recipient

Common variable immune deficiency (CVID) is a primary immunodeficiency disorder, with hypogammaglobulinemia and increased susceptibility to recurrent infections, autoimmune disorders, granulomatous diseases and malignancy. Among the solid organ transplant (SOT) recipient population, those with primary immunodeficiency disorders under chronic immunosuppression therapy can theoretically be at higher risk of atypical infections, autoimmune complications and disease recurrence with suboptimal long term graft survival, but literature is scarce. Here, we report a 27-year-old female with type 1 diabetes mellitus, complicated with nephropathy that progressed to end-stage renal disease (ESRD), who had a history of a chronic inflammatory response dysregulation, with chronic monoarthritis, persistent elevation of inflammation markers, recurrent infections, low immunoglobulin G (IgG) and A (IgA) serum levels, a slightly decreased population of memory B cells at flow cytometric immunophenotyping, and a confirmed pathological heterozygous mutation in the tumor necrosis factor receptor superfamily 13B (TNFRSF13B), with a suspected diagnosis of CVID. Whilst on hemodialysis, she received a simultaneous kidney and pancreas transplant from a standard criteria donor (SCD), and our induction and maintenance immunosuppression protocol and prophylaxis regimen allowed for a successful transplant with immediate pancreatic function, with no evidence of renal graft rejection upon biopsy in the early post-transplant period, and no novel episodes of serious infectious complications were recorded during a follow-up period of six monthsinfo:eu-repo/semantics/publishedVersio

Sclerosing encapsulating peritonitis: a case successfully treated with immunosuppression.

Perit Dial Int. 1999 Sep-Oct;19(5):478-81. Sclerosing encapsulating peritonitis: a case successfully treated with immunosuppression. Martins LS, Rodrigues AS, Cabrita AN, Guimaraes S. SourceDepartment of Nephrology, Hospital de Santo António, Porto, Portugal

Greater Impact of Living Donation Than HLA Mismatching in Short-Term Renal Allograft Survival

Introduction: Living donor kidney transplantation (LDKT) is accepted as first-line treatment for patients with end-stage kidney disease with advantages over deceased donor kidney transplantation (DDKT). Still, how the known detrimental effect of HLA mismatch (MM) may hamper these advantages remains unsettled. We sought to determine the effect of the degree of HLA MM, separately in deceased and living donor renal allograft outcomes. Methods: We evaluated all adults submitted to LDKT and DDKT at our center between 2006 and 2018. Their HLA MM was classified according to the British Society of Transplantation system in low mismatch (LM) (level 1-2) and high mismatch (HM) (level 3-4). Acute rejection (AR) and global or censored graft survival were the outcomes of interest. Recipients were followed up from transplant until death, graft failure or the end of 2020. Results: One thousand sixty-eight kidney transplant recipients were analyzed, 815 (76%) received a DDKT whereas 253 (24%) received an LDKT. From those submitted to DDKT, 95 (12%) had an LM and 720 (88%) had an HM, whereas in LDKT 32 (13%) had an LM and 221 (87%) had an HM. The AR at one year was 9% in the full cohort. Significant risk factors for AR were HM DDKT (OR:2.3, P=0.047) or HM LDKT (OR:5.6, P=0.003) (LM DDKT as reference), calculated panel-reactive antibody (cPRA) ≥5% (OR:1.9, P=0.040) and delayed graft function (DGF), (OR:3.2, P<0.001). Censored graft survival (CGS) at five years was 96% in the full cohort. Independent predictors for censored graft failure (CGF) were HM LDKT (HR:0.2, P=0.046) (LM DDKT as reference), AR (HR:2.7, P=0.008) and DGF (HR:2.2, P=0.017). Global graft survival (GGS) at five years was 91% in the full cohort. Independent predictors for global graft failure (GGF) were HM LDKT (HR:0.2, P=0.042) (LM DDKT as reference), recipient age (HR:1.8, P<0.001) and DGF (HR:1.8, P=0.006). No AR, CGF or GGF episodes were observed in the LM LDKT group. Conclusions: In our cohort, the level of HLA MM increased the risk of AR independently of donor type. Considering short graft survival, our results support the advantage of living donor vs deceased donor even with an increased HLA MM. However, its effect on long-term graft survival remains to be settled, emphasizing the need for further studies on this matterinfo:eu-repo/semantics/publishedVersio

Pulmonary alveolar proteinosis: a rare pulmonary toxicity of sirolimus.

Transpl Int. 2007 Mar;20(3):291-6. Pulmonary alveolar proteinosis: a rare pulmonary toxicity of sirolimus. Pedroso SL, Martins LS, Sousa S, Reis A, Dias L, Henriques AC, Sarmento AM, Cabrita A. Nephrology Department, Hospital Geral de Santo António, Porto, Portugal. [email protected] Abstract The aim of our paper is to describe an unusual pulmonary toxicity of sirolimus (SRL) in a kidney transplant recipient. We present a 34-year-old woman with a second renal transplantation, complicated with steroid-resistant acute rejection and chronic allograft dysfunction. Two years after initiating SRL, she presented complaints of progressive dyspnoea, nonproductive cough, chest pain and low-grade fever of 1 month duration. She had chronic allograft nephropathy and slight elevation of lactic dehydrogenase levels. After exclusion of common reasons of this condition, a computed tomography (CT) of the thorax and bronchoscopy was performed, revealing ground-glass opacification with polygonal shapes on CT and an opaque appearance with numerous macrophages on bronchoalveolar lavage. The alveolar macrophages stained positive by Periodic acid-Schiff. Diagnosis of pulmonary alveolar proteinosis (PAP) was made and drug-induced toxicity was suspected. SRL was withdrawn with marked improvement in the patients' clinical and radiological status. PAP resolved within 3 months without further therapy. PAP is a very rare complication of SRL therapy with only a few cases described. Withdrawal of SRL with conversion to another immunosuppressant seems to be an appropriate procedure in this condition. PMID: 17291222 [PubMed - indexed for MEDLIN

Living Donors’ Age Modifies the Impact of Pre-Donation Estimated Glomerular Filtration Rate on Graft Survival

Background: The global scarcity of organs for kidney transplants (KTs) has led to the increased acceptance of living donors (LDs) with minor abnormalities to increase the donor pool.. We sought to evaluate the effects of some of these LDs' clinical characteristics (older age, borderline renal function, hypertension, dyslipidemia, smoking, and obesity) on graft outcomes. Methods: We studied 352 recipients of LDKTs (1998-2020). Firstly, considering the recipients and KT variables, we identified relevant predictors of overall and censored graft failure (GF). Then, adjusting for these predictors, we explored LD variables as predictors of overall and censored GF in a multivariable Cox model. Results: The recipients from LD with higher eGFR (≥90 mL/min/1.73 m2) had significantly better overall and censored graft survival GS) at 15 y after KT (respectively, 67 and 75% vs. 46 and 46%, p < 0.001). Importantly, none of the remaining LD factors which were evaluated (hypertension, dyslipidemia, smoking, proteinuria, and obesity) were independent predictors of GF. In recipients from LDs < 50 y, having an eGFR < 90 was an independent predictor of overall GF [adjusted HR (95%CI) of 2.578 (1.120-5.795)] and censored GF [adjusted HR (95%CI) of 3.216 (1.300-7.959)], compared to recipients from LDs with eGFR ≥ 90. Contrarily, when donors were older, no difference in the risk of GF was observed between eGFR categories. Conclusion: In our cohort, lower pre-donation eGFR had an impact on GS only in younger LDs. An age-adjusted eGFR cutoff may be pursued for improved donor admissibility.info:eu-repo/semantics/publishedVersio

Primeiro transplante renal ABO-incompatível

Kidney transplantation is the optimal treatment of end -stage renal disease (ESRD) improving survival and quality of life for most recipients. In our country, potential living donors have been refused due to the ABO incompatibility barrier. However, ABO -incompatible living donor kidney transplant is presently common practice in several countries with good outcomes. The authors describe a case of a 49 -year -old female patient, with chronic kidney disease due to autosomal dominant polycystic kidney disease, who had started haemodialysis 10 months before and with blood group O. The living donor was a 53 -year -old sister with blood group B. The desensitization protocol was based on rituximab and plasmapheresis. The induction protocol used was basiliximab, tacrolimus, mofetil mycophenolate and metilprednisolone. Five days post -transplant she presented a normal graft function that remained during the eight months follow -up. This case reveals the first ABO incompatible living donor kidney transplant performed in Portugal with excellent outcome.info:eu-repo/semantics/publishedVersio

Association of the Calcification Score of the Abdominal Aorta, Common Iliac, and Renal Arteries with Outcomes in Living Kidney Donors

Background: Vascular calcification is an ever-more-common finding in protocoled pre-transplant imaging in living kidney donors. We intended to explore whether a connection could be found between the Agatston calcification score, prior to kidney donation, and post-donation renal function. Methods: This is a retrospective analysis of 156 living kidney donors who underwent living donor nephrectomy between January 2010 and December 2016. We quantified the total calcification score (TCaScore) by calculating the Agatston score for each vessel, abdominal aorta, common iliac, and renal arteries. Donors were placed into two different groups based on their TCaScore: <100 TCaScore group and ≥100 TCaScore group. The relationship between TCaScore, 1-year eGFR, proteinuria, and risk of 1 measurement of decreased renal function (eGFR < 60 mL/min/1.73 m2) over 5 years of follow-up was investigated. Results: The ≥100 TCaScore group consisted of 29 (19%) donors, with a median (interquartile range) calcification score of 164 (117-358). This group was significantly older, 56.7 ± 6.9 vs. 45.5 ± 10.6 (p < 0.001), had a higher average BMI (p < 0.019), and had a lower preoperative eGFR (p < 0.014). The 1-year eGFR was similarly diminished, 69.9 ± 15.7 vs. 76.3 ± 15.5 (p < 0.048), while also having an increased risk of decreased renal function during the follow-up, 22% vs. 48% (p < 0.007). Conclusions: Our study, through univariate analyses, found a relationship between a TCaScore > 100, lower 1-year eGFR, and decreased renal function in 5 years. However, a higher-than-expected vascular calcification should not be an excluding factor in donors, although they may require closer monitoring during follow-up.info:eu-repo/semantics/publishedVersio