scholarly journals P0179ECULIZUMAB USE IN A TERTIARY CARE NEPHROLOGY CENTER

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Christof Aigner ◽  
Gunar Stemer ◽  
Martina Gaggl ◽  
Natalja Haninger-Vacariu ◽  
Zoltán Prohaszka ◽  
...  

Abstract Background and Aims Practice patterns of Eculizumab use in patients with thrombotic microangiopathies (TMA) and C3-glomerulopathy (C3G) are not well described. Method We used the “Vienna TMA cohort” and the hospital pharmacy database at the Medical University of Vienna to identify adult patients with a history of eculizumab use between 2012 and 2019. We describe clinical characteristics, details of eculizumab use, and outcomes of patients with complement gene variant mediated TMA (cTMA), secondary TMA (sTMA) and C3G. Results As of December 2019, 212 individuals were enrolled in the Vienna TMA cohort comprising 51 cTMA, 144 sTMA, and 17 TTP patients. We included also our cohort of 14 patients with C3G for this analysis. 47 patients (22 TMA and 2 C3G, 23 other indications, i.e. paroxysmal nocturnal haemoglobinuria) received at least one dose of eculizumab at the Medical University of Vienna (Figure 1). Table 1 indicates demographic and clinical details of 15 cTMA (29.4% of all cTMA), 7 sTMA (4.9% of all sTMA) and 2 C3G (14.3% of all C3G) patients treated with eculizumab. 60% of cTMA patients showed a rare complement gene variant, while sTMA was ruled out in the remaining 40%. Causes of sTMA were bone marrow transplantation (BMT) (n=2), malignant hypertension, malignoma, systemic lupus erythematodes, antiphospholipid syndrome and lung transplantation (each n=1). One sTMA patient, a BMT recipient, had a donor with a thrombomodulin gene variant. Patients with cTMA had a greater delay from first diagnosis to treatment with eculizumab than the other groups and received maintenance therapy for a longer period of time. More female patients received eculizumab as compared to male patients. Chronic kidney disease stage improved in 60% and 43% of cTMA and sTMA patients, respectively. TMA relapses did not occur during administration of eculizumab. The 2 patients with C3G didn’t respond to eculizumab in our center. Eculizumab therapy was stopped in 66% of patients with cTMA and in all patients presenting with sTMA or C3G. In general, eculizumab was well tolerated and we did not observe life threatening infections of our patients. Three adverse drug reactions included exanthema, liver injury, and hypertensive emergency. Two patients died during therapy with eculizumab (1 cTMA, 1 C3G,) and two after cessation of eculizumab therapy (1 cTMA, 1 sTMA) resulting in a mortality of 16.7%. Conclusion Improvement of CKD stage was achieved in 60% of patients with cTMA and in 43% of patients with sTMA. In our patients with C3G, eculizumab did not improve kidney function. In general, therapy with eculizumab was well tolerated.

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Roberto Gordillo ◽  
Marcela Del Rio ◽  
David B. Thomas ◽  
Joseph T. Flynn ◽  
Robert P. Woroniecki

We report a child with Hermansky-Pudlak Syndrome (HPS) and chronic kidney disease (stage II) with histological diagnosis of focal segmental glomerulosclerosis (FSGS). A 15-year-old male of Puerto Rico ancestry with history of HPS, hypertension (HTN), asthma, obesity, and chronic kidney disease (CKD) stage II presented with new-onset proteinuria without edema. His blood pressure had been controlled, serum creatinine had been 0.9–1.4 mg/dL, and first morning urine protein/creatinine ratio (UPC) ranged from 0.2 to 0.38. Due to persistent nonorthostatic proteinuria with CKD, renal biopsy was performed and FSGS (not otherwise specified) with chronic diffuse tubulopathy (tubular cytoplasmic droplets) and acute tubular injury was reported. Ceroid-like material is known to infiltrate tissues (i.e., lungs, colon, and kidney) in HPS, but the reason for the renal insufficiency is unknown. Nonspecific kidney disease and in one adult case IgA nephropathy with ANCA-positive glomerulonephritis have previously been reported in patients with Hermansky-Pudlak syndrome. To our knowledge, we report the first pediatric renal pathology case of HPS associated with CKD. This paper discusses presentation and management of renal disease in HPS.


Author(s):  
Christof Aigner ◽  
Martina Gaggl ◽  
Gunar Stemer ◽  
Michael Eder ◽  
Georg Böhmig ◽  
...  

Abstract Background Practice patterns of eculizumab use are not well described. We examined indications for, and outcomes of, eculizumab therapy in a tertiary care nephrology center. Methods We used the “Vienna TMA cohort” and the hospital pharmacy database at the Medical University of Vienna to identify patients that received eculizumab treatment between 2012 and 2019. We describe clinical characteristics, details of eculizumab use, and outcomes of patients with complement gene-variant mediated TMA (cTMA), secondary TMA (sTMA) and C3 glomerulopathy (C3G). Results As of December 2019, 23 patients received complement blockade at the Division of Nephrology and Dialysis: 15 patients were diagnosed with cTMA, 6 patients with sTMA and 2 patients with C3G. Causes of sTMA were bone marrow transplantation (n = 2), malignant hypertension, malignant tumor, systemic lupus erythematosus, antiphospholipid syndrome and lung transplantation (each n = 1). Across all indications, patients had a median age of 31 and were predominantly female (78%) and the median duration of treatment was 227 days. Hematological recovery was seen in most patients, while renal response was best in patients with cTMA. Adverse events were recorded in 26%. Conclusions In summary, eculizumab is the treatment of choice for cTMA patients that do not respond to plasma therapy. In patients with sTMA and C3G, the response rates to therapy are much lower and therefore, the decision to start therapy needs to be considered carefully. Graphic abstract


Author(s):  
Stefania Sella ◽  
Luciana Bonfante ◽  
Maria Fusaro ◽  
Flavia Neri ◽  
Mario Plebani ◽  
...  

AbstractObjectivesKidney transplant (KTx) recipients frequently have deficient or insufficient levels of serum vitamin D. Few studies have investigated the effect of cholecalciferol in these patients. We evaluated the efficacy of weekly cholecalciferol administration on parathyroid hormone (PTH) levels in stable KTx patients with chronic kidney disease stage 1–3.MethodsIn this retrospective cohort study, 48 stable KTx recipients (37 males, 11 females, aged 52 ± 11 years and 26 months post-transplantation) were treated weekly with oral cholecalciferol (7500–8750 IU) for 12 months and compared to 44 untreated age- and gender-matched recipients. Changes in levels of PTH, 25(OH) vitamin D (25[OH]D), serum calcium, phosphate, creatinine and estimated glomerular filtration rate (eGFR) were measured at baseline, 6 and 12 months.ResultsAt baseline, clinical characteristics were similar between treated and untreated patients. Considering the entire cohort, 87 (94.6%) were deficient in vitamin D and 64 (69.6%) had PTH ≥130 pg/mL. Serum calcium, phosphate, creatinine and eGFR did not differ between groups over the follow-up period. However, 25(OH)D levels were significantly higher at both 6 (63.5 vs. 30.3 nmol/L, p < 0.001) and 12 months (69.4 vs. 30 nmol/L, p < 0.001) in treated vs. untreated patients, corresponding with a significant reduction in PTH at both 6 (112 vs. 161 pg/mL) and 12 months (109 vs. 154 pg/mL) in treated vs. untreated patients, respectively (p < 0.001 for both).ConclusionsWeekly administration of cholecalciferol can significantly and stably reduce PTH levels, without any adverse effects on serum calcium and renal function.


QJM ◽  
2019 ◽  
Vol 113 (6) ◽  
pp. 411-417 ◽  
Author(s):  
A Elis ◽  
M Leventer-Roberts ◽  
A Bachrach ◽  
N Lieberman ◽  
R Durst ◽  
...  

Abstract Background Familial hypercholesterolemia (FH) is an under-diagnosed condition. Aim We applied standard laboratory criteria across a large longitudinal electronic medical record database to describe cross-sectional population with possible FH. Methods A cross-sectional study of Clalit Health Services members. Subjects who met the General Population MED-PED laboratory criteria, excluding: age &lt;10 years, documentation of thyroid, liver, biliary or autoimmune diseases, a history of chronic kidney disease stage 3 or greater, the presence of urine protein &gt;300 mg/l, HDL-C&gt;80 mg/dl, active malignancy or pregnancy at the time of testing were considered possible FH. Demographic and clinical characteristics are described at time of diagnosis and at a single index date following diagnosis to estimate the burden on the healthcare system. The patient population is also compared to the general population. Results The study cohort included 12 494 subjects with out of over 4.5 million members of Clalit Health Services. The estimated prevalence of FH in Israel was found to be 1:285. These patients are notably positive for, and have a family history of, cardiovascular disease and risk factors. For most of them the LDL-C levels are not controlled, and only a quarter of them are medically treated. Conclusions By using the modified MED-PED criteria in a large electronic database, patients with possible FH can be identified enabling early intervention and treatment.


2018 ◽  
Vol 7 (11) ◽  
pp. 431 ◽  
Author(s):  
Diamantina Marouli ◽  
Kostas Stylianou ◽  
Eleftherios Papadakis ◽  
Nikolaos Kroustalakis ◽  
Stavroula Kolyvaki ◽  
...  

Background: Postoperative Acute Kidney Injury (AKI) is a common and serious complication associated with significant morbidity and mortality. While several pre- and intra-operative risk factors for AKI have been recognized in cardiac surgery patients, relatively few data are available regarding the incidence and risk factors for perioperative AKI in other surgical operations. The aim of the present study was to determine the risk factors for perioperative AKI in patients undergoing major abdominal surgery. Methods: This was a prospective, observational study of patients undergoing major abdominal surgery in a tertiary care center. Postoperative AKI was diagnosed according to the Acute Kidney Injury Network criteria within 48 h after surgery. Patients with chronic kidney disease stage IV or V were excluded. Logistic regression analysis was used to evaluate the association between perioperative factors and the risk of developing postoperative AKI. Results: Eleven out of 61 patients developed postoperative AKI. Four intra-operative variables were identified as predictors of AKI: intra-operative blood loss (p = 0.002), transfusion of fresh frozen plasma (p = 0.004) and red blood cells (p = 0.038), as well as high chloride load (p = 0.033, cut-off value > 500 mEq). Multivariate analysis demonstrated an independent association between AKI development and preoperative albuminuria, defined as a urinary Albumin to Creatinine ratio ≥ 30 mg·g−1 (OR = 6.88, 95% CI: 1.43–33.04, p = 0.016) as well as perioperative chloride load > 500 mEq (OR = 6.87, 95% CI: 1.46–32.4, p = 0.015). Conclusion: Preoperative albuminuria, as well as a high intraoperative chloride load, were identified as predictors of postoperative AKI in patients undergoing major abdominal surgery.


2020 ◽  
pp. 90-93
Author(s):  
Muhammed Mustafa Özdemir ◽  
Ayşe Seda Pınarbaşı ◽  
Neslihan Günay ◽  
Aynur Gencer-Balaban ◽  
Sibel Yel ◽  
...  

Objective: This study aimed to evaluate patients with renal transplantation in terms of clinical and laboratory parameters. Material and Methods: This study was performed retrospectively with records of 48 patients who underwent renal transplantation before 18 years of age, between June 2008 and July 2019. Results: Congenital malformations of the urinary tract were the most common underlying causes of chronic kidney disease stage 5. Surgical complications occurred in 33.4% of the patients and BK viremia was the most common opportunistic viral infection during the follow-up. At the last clinic visit, 57.4% of our patients had CKD stage 1, hypertension and nephrotic range proteinuria were seen in eight and two patients, respectively. Conclusion: Although renal transplantation is the most ideal renal replacement therapy, patients may experience various complications during the follow-up. Therefore, they should be monitored regularly


Author(s):  
Agri Febria Sari ◽  
Rikarni Rikarni ◽  
Deswita Sari

Reticulocyte hemoglobin equivalent (RET-He) represents hemoglobin content in reticulocyte. Reticulocyte hemoglobin equivalent test can be used to asses iron status of chronic kidney disease (CKD). Iron deficiency happens in 40% CKD and could lead to anemia manifestation. Level of RET-He gives real-time assesment of iron availability for hemoglobin production and the level will getting lower when iron storage for erythropoiesis decreasing. Reticulocyte hemoglobin equivalent is more stabil than feritin and transferin saturation in assessing iron status. Aim of this study is to determine RET-He level in patients with CKD stage IV and V. This study is  a cross sectional descripstive study. Subjects were 96 CKD stage IV and V patients that met inclusion and exclusion criterias. Subjects conducted blood tests at Central Laboratory Installation Dr. M. Djamil Hospital Padang from July to September 2020. Examination of RET-He level was analyzed by Sysmex XN-1000 flowcytometry fluorescense method. Data was presented in frequency distribution table. The RET-He level below cutoff (<29,2 pg) indicates the need for iron suplementation therapy for CKD stage IV and V patients. Samples with RET-He level below cutoff were 48 (50%) and 48 (50%) were above cutoff.


2020 ◽  
pp. 105477382095854
Author(s):  
Emanuele Poliana Lawall Gravina ◽  
Bruno Valle Pinheiro ◽  
Luciana Angélica da Silva Jesus ◽  
Fabrício Sciammarella Barros ◽  
Leda Marília Fonseca Lucinda ◽  
...  

To evaluate the factors associated with functional capacity in patients with chronic kidney disease (CKD). All patients were submitted to six-minute walk test (6MWT), 10-repetition sit-to-stand test (STS-10) and SF-36 health-related quality of life questionnaire (HRQoL). Patients with functional capacity ≥80% exhibited higher education level, family income, body mass index, estimated glomerular filtration rate, and lower age and STS-10 time. Multiple linear regression showed that gender, age, family income, chronic kidney disease stage, STS-10 time, and physical component summary of HRQoL were significantly associated with the 6MWT distance. Functional capacity was significantly associated with gender, age, family income, CKD stage, STS-10 time, and physical component of HRQoL. The progression of CKD has an impact on the decrease in functional capacity in these patients.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
FEDERICO DI MARCO ◽  
Umberto Capitanio ◽  
Arianna Bettiga ◽  
Riccardo Vago ◽  
Alessandra Cinque ◽  
...  

Abstract Background and Aims Radical Nephrectomy is usually associated to the risk of future development of a mild to severe chronic kidney disease stage especially for those patients who already present early stages of CKD (e.g CKD class II and IIIa). Any insight on this topic could influence the clinical decision about the surgery. But how can we know for sure the magnitude of the renal function’s decay? In this preliminary work, our aim was to identify a new model able to predict at time surgery the renal function’s variation at 1 year from the operation Method We collected prospectively clinical data of a group of consecutive 114 patients who underwent radical nephrectomy (RN) for the presence of a benign or malignant renal mass. We estimated Glomerular Filtration Rate (eGFR) with MDRD formula. We considered the following clinical varibles: AKI onset (according to RIFLE criteria), age, gender, presence of blood hypertension, diabetes type II and BMI. Moreover, to investigate a possible correlation between renal basal histology and renal functional decay, renal biopsies were performed on each on the healthy part of the removed kidney &gt; 3cm far from tumor. A pathological evaluation using a chronicity score (Remuzzi Score) was subsequently carried out evaluating damage on four parameters: (a) glomerular global sclerosis, (b) tubular atrophy, (c) interstitial fibrosis and (d) arterial narrowing. Statistical analysis were performed using generalized linear model (GLM), Kruskal-Wallis test and chi-square test. Multivariate analysis were applied using stepwise regressions method in order to select the best fitting model. Statistically significant correlations were considered for p-value&lt;0.05. Results At t0, 21% of the patients had an eGFR&gt;90ml/min/1.73m2, 45% between 60 and 90, 23% between 30 and 45, and 11% under 45. Median observed decay after 12 months was 32.8% (IQR= 17.9%:41.9%).Taking in account the eGFR decay’s percentage there was a strong correlation with AKI onset (decay increased by 22.4%, CI= 14%:30.8%, p&lt;0.0001), with Diabetes ( decay increased by 13%, CI= 2%: 24.5%, p=0.02) and with the CKD stages at t0 (p=0.0007). Considering the histology, a significative negative correlation was found with the presence of arterial narrowing (-14%, CI=-23%:-6%, p&lt;0.01) even though the whole chronicity score did not correlate (p=0.5). No significative correlations were found between the decay of eGFR and other variables such as age, gender or comorbidities. The multivariate analysis by stepwise regression, including all the significative variables from the univariate analysis, proposed as best model to predict the decay the use of AKI onset (14%, CI=6%:22%, p=0.001), arterial narrowing (-13%, CI=-22%:-5%, p=0.001) and diabetes (p=0.14) as variables. Conclusion A precise and reliable prediction of renal function decrease after RN represents a cornerstone for urologist and nephrologist in order to create a personalized medical approach and management.In our cohort of study, CKD stage I and II patients displayed a huge decrease of eGFR in respect to CKD stages III-IV over time. One possible biological explanation can be that the healthy kidney of the patients affected by moderate and severe CKD starts working with a compensatory mechanism before the entire removal of the kidney with cancer so that the surgical acute nephron loss does not represent a shock in comparison to healthy patients with an eGFR &gt;90 ml/min. Our preliminary study identified a new clinical and pathological panel of variables able to predict at time zero the magnitude of eGFR decay after 1 year from surgical operation. Further studies are needed in order to validate and improve this model.


2016 ◽  
Vol 4 (1) ◽  
Author(s):  
Medhyka S.A. Kawilarang ◽  
Arthur E. Mongan ◽  
Maya Memah

Abstract: Magnesium is a metal, one of the eight most abundant element in universe. Magnesium also is a mineral that responsible for bone metabolism control, neural transmission, cardiac excitability, neuromuscular conduction, muscular contraction, vasomotor, and blood pressure. In chronic kidney disease stage 4-5, compensation mechanism become inadequate that caused a hypermagnesemia. Objectives: This study aimed to know the picture of magnesium serum in non dialysis CKD stage 5 in Manado. Material Methods: This is a descriptive study, conducted by selecting 35 blood samples in Nephrology-Hypertension Polyclinic and IRINA of Interna of Prof. Dr. R.D Kandou Hospital and Teling Adventist Hospital. Result: There are 16 samples (45.7%) experience hypomagnesemia consisted of 8 home-care patient (22.9%) and 8 hospital-care patient (22.9%), 10 samples (28.6%) are in normal range consisted of 3 home-care patient (8.6%) and 7 hospital-care patient (20.0%), and 9 samples (25.7%) are experience hypermagnesemia consisted of 6 home-care patient (17.1%) and 3 hospital-care patient (8.6%) from total non dialysis CKD stage 5 samples result from laboratory examination. Conclusion: Patient with hypomagnesemia most frekuent than patient with hypermagnesemia.Keywords: magnesium, chronic kidney disease, non dialysisAbstrak: Magnesium merupakan logam yang masuk dalam delapan elemen paling melimpah di alam semesta. Magnesium juga merupakan mineral yang bertanggung jawab dalam pengaturan metabolisme tulang, transmisi saraf, eksitabilitas jantung, konduksi neuromuskular, kontraksi muscular, vasomotor, dan tekanan darah. Pada penyakit ginjal kronik stadium 4-5 mekanisme kompensasi ginjal menjadi inadekuat sehingga dapat menghasilkan hipermagnesemia. Tujuan: Penelitian ini bertujuan untuk mengetahui gambaran kadar serum magnesium pada pasien PGK non dialisis stadium 5 di Manado. Metode Penelitian: Penelitian ini adalah penelitian yang bersifat deskriptif yang dilaksanakan dengan cara mengambil sampel darah di Poliklinik Nefrologi-Hipertensi dan IRINA bagian Penyakit Dalam RSUP Prof. DR. R. D. Kandou Manado dan RS Advent Teling sebanyak 35 sampel. Hasil: Tercatat 16 orang yang mengalami hipomagnesemia (45,7%) diantaranya 8 orang pasien rawat jalan (22,9%) dan 8 orang pasien rawat inap (22,9%), 10 orang dalam batas nilai normal (28,6%) diantaranya 3 orang pasien rawat jalan (8,6%) dan 7 orang pasien rawat inap (20,0%), serta 9 orang mengalami hipermagnesemia (25,7%) diantaranya 6 orang pasien rawat jalan (17,1%) dan 3 orang pasien rawat inap (8,6%) dari total jumlah pasien terdiagnosis dokter PGK stadium 5 non dialisis yang didapatkan dari hasil pemeriksaan laboratorium. Simpulan: Frekuensi pasien yang mengalami hipomagnesemia lebih banyak dibandingkan pasien hipermagnesemiaKata kunci: magnesium, penyakit ginjal kronik, non dialisis


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