A Rare Case: Improved Heart Failure with Anti-Complement Therapy in Complement-Dependent Hemolytic Uremic Syndrome

MakaleWOS:000925209300014xtrarenal involvement occurs in approximately 20% of patients with complement-mediated hemolytic-uremic syndrome. The involvement is usually of the nervous system, and cardiac involvement occurs in 3%-10% of patients. Cardiac manifestations vary, including myocardial infarction, cardiomyopathy, and acute decompensated heart failure. Among these patients, thrombotic microangiopathy-related cardiac dysfunction is mainly due to the continuous activation of the complement system, which leads to endothelial damage and thrombosis in the coronary microvessels. We wanted to highlight the importance of cardiac evaluation at the time of diagnosis or during follow-up in thrombotic micro-angiopathy patients by presenting a case of heart failure with low ejection fraction in a 24-year-old young patient in whom we detected complement-mediated hemolytic-uremic syndrome, a secondary mutation of complement factor H receptor. It is still an unknown issue because of the rarity of cardiac involvement in complement-mediated hemolytic-uremic syndrome patients. Primary myocardial involvement is increasingly recognized as a possible concomitant feature of hemolytic-uremic syndrome. Failure to perform a detailed cardiac evaluation both at diagnosis and during follow-up in complement-mediated hemolytic-uremic syndrome patients can lead to fatal outcomes. Anti-complement therapy can also lead to good cardiac outcomes in these patients

Common variable immunodeficiency in adults requires reserved protocols for long-term follow-up

WOS:000370271100029PubMed ID: 27511507Background/aim: The aim of this study is to establish follow-up protocols for adult patients with common variable immunodeficiency (CVID) in a recently founded adult immunology clinic in the Central Anatolia Region of Turkey, where a clinical immunology center for adults was not available previously. Materials and methods: A total of 25 patients with CVID aged 18 years and older were included in this study. The file format consisted of 13 pages and was developed for the purpose of the study. Separate sections were designated for identity information, medical history, disease course, previous and current laboratory and imaging studies, follow-up plans, detection and management of complications/ comorbidities, and treatment results. Results: The mean age of the patients was 36.6 ± 13.4 years. The delay in diagnosis was 107 ± 95.6 months. In 92% of patients, initial symptoms resulting in admission to healthcare facilities were infections. Seventeen of 25 patients (68%) had bronchiectasis at the beginning of follow-up. Conclusion: Early identification of complications and comorbidities in patients with CVID will significantly improve quality of life and survival. Close observation and standardized protocols for follow-up are essential components of management

Renal transplant results of the organ transplant center of meram medical school between 2003-2011

AMAÇ: Son dönem böbrek yetmezliği (SDBY) hastalarında en iyi tedavi şekli böbrek naklidir. Çalışmamızda, son 8 yılda merkezimizde kadavra ve canlıdan yapılan böbrek nakillerinin(BN) demografik verileri ile birlikte böbrek fonksiyonları ve posttransplant medikal komplikasyonları, hasta- graft sağkalımını araştırmayı hedefledik. GEREÇ ve YÖNTEMLER: Çalışmaya 40ı kadavradan, 26sı canlıdan BN yapılan 66 hasta (kadın/ erkek: 36/30) dahil edildi. Nakil sonrası nefroloji polikliniğine başvurularda yaş, cinsiyet, böbrek yetmezliği nedeni, diyaliz türü-süresi, nakil türü, aldıkları indüksiyon tedavileri, kullandığı idame immünsupresif tedaviler, akut rejeksiyon sayısı ve verilen tedaviler, nakil sonrası 1., 6., 12., 24. ve 60. aylardaki biyokimya-hemogram parametreleri ve medikal komplikasyonlar hasta dosyalarından retrospektif olarak elde edildi. BULGULAR: Alıcıların ortalama yaşı 4111,6 yıldı. Ortalama nakil sonrası süre 32,231,4 ay, kreatinin değerleri 1,40,9 mg/dl tespit edildi. En sık uygulanan immünsüpresif tedavi protokolü ko rtikosteroidtakrolimusmikofenolat mofetil/sodyumdu. Gecikmiş graft fonksiyonu, kronik allograft disfonksiyonu ve akut rejeksiyon oranları sırasıyla %27,3, %25,7 ve %13,6 idi. 1 ve 5 yıllık hasta sağkalımı canlıdan yapılan BNlerinde sırasıyla %100 ve %100, kadavradan yapılan BNlerinde ise %85 ve %85 olarak bulundu. 1 ve 5 yıllık graft sağkalımı canlıdan BN yapılanlarda sırasıyla %100 ve %100, kadavradan BN yapılanlarda ise %80 ve %80 olarak saptandı. En sık görülen medikal komplikasyonlar yeni gelişen diyabet ve dislipidemiydi. Erken ve geç dönemde en sık karşılaşılan enfeksiyon idrar yolu enfeksiyonuydu. SONUÇ: BN, hasta-graft sağkalımının yüksek olduğu bir renal replasman tedavi seçeneğidir. Bununla birlikte metabolik komplikasyonlar açısından yakın takip gereklidir.OBJECTIVE: Renal transplantation (RTx) is the best therapeutic modality for end-stage renal disease patients. We report 8 years single-centre experience on cadaveric and living donor RTx in terms of demographic features along with graft functions, posttransplant medical complications, patients-graft survivals. MATERIAL and METHODS: We enrolled 66 RTx (female/male: 36/30) patients including 40 cadaveric and 26 living donors. At admission age, gender, causes of renal failure, dialysis type- duration, type of RTx, induction and maintenance immunosuppressive modalities, rejection episodes, biochemistry-hemogram parameters at 1, 6, 12, 24 and 60 months after transplantation and medical complications were obtained from the medical records. RESULTS: Mean recipient age was 41±11.6 years. Mean transplant duration was 32.2±31.4 months, and the mean creatinine values was 1.4±0.9 mg/dl. The most commonly used immunosuppressive protocol was corticosteroidtacrolimusmycophenolate mofetil. Delayed graft function, chronic allograft nephropathy and acute rejection were observed in 27.3%, 25.7% and 13.6% of patients, respectively. 1- and 5-year patient survival rates were 100% and 100% for living donor patients and 85% and 85% for cadaveric patients, respectively. 1- and 5-year graft survival rates were 100% and 100% for living donor RTx patients, and 80% and 80% for cadaveric RTx patients, respectively. The most common medical complications were new onset diabetes mellitus and dyslipidemiaThe most common early and late infection was urinary tract infection. CONCLUSION: RTx is the best renal replacement therapy in terms of patient-graft survival. However, patients should be closely moniterized for metabolic complications

The prevalence, clinical and pathological characteristics of primary focal segmental glomerulosclerosis in Turkish adults: The data from TSN-Gold (Turkish society of nephrology glomerular diseases) working group

...ERA; EDT

The relationship between severity of interstitial fibrosis and anemia in patient with primary glomerulonephritis: The data from TSN-Gold working group

...ERA EDT

Trends of primary glomerular disease in Turkey from 2009 to 2017: A regional registry report from TSN-Gold working group

...ERA EDT