Chronic Renal Insufficiency in Japanese Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH): Improvement with Eculizumab Treatment in the Long-Term Follow-up of the AEGIS Study.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1980-1980 ◽  
Author(s):  
Yuzuru Kanakura ◽  
Kazuma Ohyashiki ◽  
Tsutomu Shichishima ◽  
Shinichiro Okamoto ◽  
Kiyoshi Ando ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Disease ◽  
Research Funding ◽  
Japanese Patients ◽  
Japanese Study ◽  
Ckd Stage ◽  
Impact On Survival ◽  
Life Threatening ◽  
Equity Ownership ◽  
Stage 1

Abstract Abstract 1980 Poster Board I-1002 PNH is a progressively debilitating and life-threatening disease characterized by chronic complement-mediated hemolysis. The clinical complications associated with chronic hemolysis include life-threatening thrombosis, chronic kidney disease (CKD), pulmonary hypertension, organ damage, ischemic bowel disease, and hepatic failure - all of which contribute to shortened survival of patients with PNH. CKD, a consequence of hemolysis, has significant impact on survival in Japanese patients with PNH, accounting for 18% of deaths. We previously reported results from the Japanese AEGIS study, a 12-week open-label single-arm phase II study, which demonstrated that eculizumab (ecu), a humanized monoclonal antibody against the terminal complement molecule C5, significantly reduced complement-mediated intravascular hemolysis. Ecu treatment subsequently improved anemia, fatigue and renal function in 11 Japanese patients with PNH. Given the important impact on survival of renal disease, the effects of ecu on renal function were further evaluated among 27 Japanese patients with PNH enrolled in a 26-week extension of the AEGIS study. Ecu was dosed as follows: 600mg weekly for 4 weeks; 900mg one week later; and then 900mg every other week for a total of 38 weeks of therapy. Patients received meningococcal vaccine 2 weeks prior to treatment. Consistent with the previous 12 week study report, there was a sustained reduction in intravascular hemolysis, as measured by lactate dehydrogenase (LDH), through the 38 weeks of ecu treatment. LDH decreased 87% from a median of 1,814 U/L at baseline to a median of 232 U/L at 38 weeks of treatment (P<0.001; see table). Control of hemolysis resulted in continued improvement in anemia; hemoglobin continued to increase from 7.5 g/dL at baseline to 9.75 g/dL at 38 weeks (P<0.001) despite the reduction in transfusion requirements (P=0.004). Fatigue levels, as measured by the FACIT-Fatigue instrument, continued to significantly improve with ecu treatment (P<0.001; see table). Renal function was defined by KDOQI CKD stage, recognized as objective evidence of abnormal glomerular function, determined by glomerular filtration rate (GFR), along with persistent proteinuria, from Stage 0 (normal) to Stage 5 (Kidney failure). An improvement in renal function was defined as a categorical reduction (improvement) in CKD stage. Worsening in renal function was defined as a categorical increase (decline) in CKD stage. At study entry, 65% of the Japanese group demonstrated evidence of CKD. During the 38-week treatment period, 33.3% (9/27) of all patients who entered the trial showed improvement while 66.7% (18/27) showed no change or progression from baseline and no patients worsened. Further, 52.9% (9/17) of patients with CKD at baseline demonstrated improvement. Among the 9 patients who showed improvement, 8 had stage 1-2 CKD at baseline and one had stage 3-5, underscoring the importance of early intervention with ecu in patients with mild kidney disease. The CKD results of the Japanese study were compared to the 26-week placebo-controlled TRIUMPH trial and the combined data from the multinational clinical trials (see table). In the overseas multinational trials, 64% (121/189) of patients had evidence of CKD at study entry, similar to the 65% in the Japanese cohort. The results from the 26-week placebo group in the TRIUMPH trial suggest that there is no significant change in CKD over the course of 6 months. In contrast, treatment with ecu significantly improved CKD in patients in the TRIUMPH and overseas multinational studies (P<0.001) and in the Japanese study (0% worsened, 33% improved, P<0.05). The results from both studies also demonstrate that the benefit from ecu treatment was most evident in patients with early CKD stage 1-2. In conclusion, kidney disease is an important cause of premature mortality in Japanese PNH patients. The current study suggests that evidence of CKD is common in Japanese PNH patients. Further, this study demonstrates that ecu is safe and well tolerated in Japanese patients with PNH. We see continued improvement in PNH symptoms from 12 to 38 weeks with ecu treatment. Similar to the beneficial effects of ecu in patients with PNH in overseas multinational phase III trials, reduction of hemolysis with ecu treatment was associated with clinical improvement in kidney function in Japanese patients with PNH. Disclosures: Kanakura: Alexion Pharmaceuticals: Research Funding. Ohyashiki:Alexion Pharmaceuticals: Research Funding. Shichishima:Alexion Pharmaceuticals: Research Funding. Okamoto:Alexion: Research Funding. Ando:Alexion: Research Funding. Ninomiya:Alexion: Research Funding. Kawaguchi:Alexion: Research Funding. Nakao:Alexion: Research Funding. Nakakuma:Alexion: Research Funding. Nishimura:Alexion: Honoraria, Research Funding. Kinoshita:Alexion: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees. Bedrosian:Alexion Pharmaceuticals: Employment, Equity Ownership. Valentine:Alexion: Employment, Equity Ownership. Ozawa:Alexion: Research Funding. Omine:Alexion: Consultancy, Research Funding.

scholarly journals Orosomucoid As a Biomarker for Chronic Kidney Disease Associated with Sickle Cell Anemia

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2352-2352
Author(s):  
Ammanuel Taye ◽  
Nathan Smith ◽  
Nowah Kokou Apeadoufia Afangbedji ◽  
Xiaomei Niu ◽  
James G. Taylor ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Research Funding ◽  
Urine Samples ◽  
Cell Disease ◽  
Howard University ◽  
Ckd Stage ◽  
Stage 1

Abstract BACKGROUND: Chronic kidney disease (CDK) is a serious and highly prevalent complication of Sickle cell anemia (SCA). Proteinuria and microalbuminuria can be under-detected in SCA because of the renal concentrating defect. Hence, complementary diagnostic biomarkers are necessary for early detection of CKD in patient with SCA. Our recent study identified alpha 1-acid glycoprotein (orosomucoid, ORM) (1) as potential biomarker of CKD. ORM is a major acute phase inflammatory protein synthesized by the liver and its presence in urine may indicate underlying inflammation in SCA patients. OBJECTIVES: We aimed to validate ORM as biomarker for CKD in a cohort of SCA patients recruited at Howard University. METHODS: We analyzed samples collected from patients enrolled in a sickle cell disease registry study at Center for Sickle Cell Disease, Howard University. Plasma and urine samples were collected and stored at -80°C. Plasma samples were assayed for creatinine and cystatin C. Urine samples were analyzed for albumin, creatinine and ORM. Multistix was used to determine protein, blood, pH and specific gravity (SG) in urine samples. Estimated GFR (eGFR) was calculated using CKD-EPI creatinine-cystatin equation. CKD stages were assigned according to the National Kidney Foundation, Kidney Disease Outcomes Quality Initiatives (K/DOQI) guidelines: stage 0 - eGFR>60 ml/min/1.73m2 and AL/CRE<30 mg/g; stage 1- eGFR>90 ml/min/1.73m2 and AL/CRE≥30 mg/g; stage 2 - eGFR 60-89 ml/min/1.73m2 and AL/CRE≥30 mg/g, stage 3 - eGFR 30 - 59 /1.73m2; stage 4 - eGFR 15 - 29 ml/min/1.73m2 and stage 5 - eGFR < 15 ml/min/1.73m2. Glomerular hyperfiltration was defined as eGFR>130 ml/min/1.73 m2 for females and eGFR>140 ml/min/1.73 m2 for males. RESULTS: The Howard University registry study group consisted of 96 patients (mean age 38.1 years, range 18 - 67 years, 55% females) and included 65.6% HbSS, 21.8% HbSC, 7.2% HbS β+thalassemia, 4.1% HbS β0 thalassemia. Based on urinary dipstick test, proteinurea was detected in 16.5% patient. Hyperfiltration was detected in 45 patients (56.3%). CDK (combined stage 1-4) was detected in 35.8% patients. No difference was found between females and males in CKD prevalence. Prevalence of CKD in HbSC patients was 11% whereas it was 37% in HbSS patients. Prevalence of CDK increased with the age. The overall urinary ORM/CRE levels demonstrated modest correlation with CKD stage (R2=0.22). Next we stratified samples based on the levels of ORM/CRE. The samples were separated into two groups: (i) samples with no or mild inflammation (ORM/CRE<10μg/mg) and (ii) samples that displayed significant inflammation (ORM/CRE>10 μg/mg). We observed strong inverse correlation between the percentage of samples without inflammation and CKD stage (R2=0.996). We also observed strong correlation of CDK progression with number of samples with significant inflammation (R2=0.996). Sensitivity of ORM/CRE was 53.8%, specificity 70.2% and odds ratio OR 2.72 for samples with CKD stages 1-4 compared to the samples without CKD (stage 1). CONCLUSIONS: In our cohort, determination of proteinurea evaluated by dipstick (16%) grossly underestimated CKD prevalence (35%) determined by eGFR and AL/CRE ratios. Subjects with HbSC had 3-fold less prevalence of CKD comparing to the patients with HbSS. ORM/CRE levels correlated with the stages of CKD stages (OR 2.72). Further longitudinal study is needed to determine whether ORM/CRE ratio can be used as a prognostic marker of renal disease development. ACKNOWLEDGMENTS: This work was supported by NIH Research Grants 1P50HL118006, 1R01HL125005 and 5G12MD007597. AT was supported by ASH MMSAP summer program. The content is solely the responsibility of the authors and does not necessarily represent the official view of NHLBI, NIMHD or NIH. REFERENCES: Jerebtsova, M., Saraf, S. L., Soni, S., Afangbedji, N., Lin, X., Raslan, R., Gordeuk, V. R., and Nekhai, S. (2018) Urinary orosomucoid is associated with progressive chronic kidney disease stage in patients with sickle cell anemia. American journal of hematology93, E107-E109 Disclosures Taylor: Pfizer: Research Funding; NHLBI: Research Funding. Nekhai:NIAID, NIH: Research Funding; NIMHD, NIH: Research Funding; NHLBI, NIH: Research Funding.

scholarly journals Use of Multiple Urinary Biomarkers for Early Detection of Chronic Kidney Disease in Sickle Cell Anemia Patients

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 30-30
Author(s):  
Yagahira E. Castro-Sesquen ◽  
Santosh L. Saraf ◽  
Victor R. Gordeuk ◽  
Sergei Nekhai ◽  
Marina Jerebtsova
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Early Detection ◽  
Research Funding ◽  
Urinary Biomarkers ◽  
Complete Model ◽  
Advisory Committees ◽  
Ckd Stage ◽  
Stage 1 ◽  
Hepatocyte Growth

BACKGROUND : More than 60% of sickle cell anemia (SCA) adults develop chronic kidney disease (CKD). Identification of early stages of CKD in SCA patients at high risk of complications could lead to personalized treatment and better prognosis. Recently we identified several urinary biomarkers that can differentiate CKD stage 1 in SCA patients. These biomarkers reflect pathophysiology of SCA, including iron homeostasis (ceruloplasmin, transferrin, hemoglobin, and ferritin); inflammation (orosomucoid); and glomerular hyperfiltration (hepatocyte growth factor like). HYPOTHESIS: We hypothesized that combination of novel urinary biomarkers might improve the accuracy of early detection of CKD. METHODS : We evaluated spot urine samples of 54 patients with SCA in a steady state from the University of Illinois at Chicago. Patients were classified by the stage of chronic kidney disease (CKD) based on the National Kidney Foundation, Kidney Disease Outcomes Quality Initiatives guidelines. Three groups of CKD stages were compared: Stage 0 (without CKD, n=23), stage 1 (early stage of CKD, n=19), and stages 2 to 5 (moderate to severe CKD, n=12). Urine levels of ceruloplasmin (CP), transferrin (TrF), hemoglobin (Hgb), ferritin (FrT), orosomucoid (ORM), and hepatocyte growth factor like (HGFL) were measured by ELISA and normalized to urinary creatinine (CRE) concentrations. Differences in sensitivity and specificity of biomarker combinations were compared using the McNemar's Test against the simple model, which uses urine Hgb only, and the complete model, with all the biomarkers together. A test for the equality of the Area Under the Curve (AUC) compared to the simple and complete models was done using the algorithm suggested by DeLong, DeLong, and Clarke-Pearson (1988). RESULTS: We tested the ability of each biomarker to distinguish SCA patients without CKD and with CKD stage 1. Receiver operating characteristic (ROC) curves were constructed to determine appropriate cutoffs for each biomarker. Cutoffs that provided the highest Youden Indexes were used. Three biomarkers (Hgb, CP, and ORM) had a sensitivity of 100%; however, specificity was lower than 80% (range: 65.2%-72.7%). Other biomarkers (Frt, Trf, and HGFL) had sensitivities lower than 80%, suggesting that individually these biomarkers are not accurate for early detection of CKD. We compared different combinations to the single biomarker model (Hgb only or model 0) and a complete combination of six biomarkers (model 1) (Table 1). The complete combination significantly improved specificity (from 69.6 to 82.6%) and increased AUC (from 0.86 to 0.96) compared to the single biomarker model. All combinations significantly increased the specificity (from 69.6% to 78.3-82.7%) compared to the single biomarker model. Combinations of four to five biomarkers (models 1-6) significantly increased specificity and AUC values than model 0. Combination of Hgb + CP+ ORM + Frt produced similar specificity and AUC values compared to complete model. CONCLUSION: These results demonstrate that the use of multiple biomarkers can improve the accuracy in the detection of kidney pathology in SCD patients, which has essential value for clinicians and researchers of clinical trials to target high-risk individuals for early treatment and preventive care. LIMITATION: Small cohort of patients from one center. Disclosures Saraf: Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Other: Advisory Boards, Speakers Bureau; Novartis, Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees; Pfizer, Global Blood Therapeutics, Novartis: Research Funding. Gordeuk:Imara: Research Funding; Novartis: Consultancy; Ironwood: Research Funding; CSL Behring: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding.

scholarly journals The Copenhagen chronic kidney disease echo study (COInCYDE)

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Landler ◽  
S Bro ◽  
B Feldt-Rasmussen ◽  
D Hansen ◽  
A.L Kamper ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Chronic Kidney Disease ◽  
Cohort Study ◽  
Kidney Disease ◽  
Cardiac Function ◽  
Left Ventricular ◽  
Funding Source ◽  
Ckd Stage ◽  
Significant Difference ◽  
Stage 1

Abstract Background The cardiovascular mortality of patients with chronic kidney disease (CKD) is 2–10 times higher than in the average population. Purpose To estimate the prevalence of abnormal cardiac function or structure across the stages CKD 1 to 5nonD. Method Prospective cohort study. Patients with CKD stage 1 to 5 not on dialysis, aged 30 to 75 (n=875) and age-/sex-matched controls (n=173) were enrolled consecutively. All participants underwent a health questionnaire, ECG, morphometric and blood pressure measurements. Blood and urine were analyzed. Echocardiography was performed. Left ventricle (LV) hypertrophy, dilatation, diastolic and systolic dysfunction were defined according to current ESC guidelines. Results 63% of participants were men. Mean age was 58 years (SD 12.6 years). Mean eGFR was 46.7 mL/min/1,73 m (SD 25.8) for patients and 82.3 mL/min/1,73 m (SD 13.4) for controls. The prevalence of elevated blood pressure at physical exam was 89% in patients vs. 53% in controls. Patients were more often smokers and obese. Left ventricular mass index (LVMI) was slightly, albeit insignificantly elevated at CKD stages 1 & 2 vs. in kontrols: 3.1 g/m2, CI: −0.4 to 6.75, p-value 0.08. There was no significant difference in LV-dilatation between patients and controls. Decreasing diastolic and systolic function was observed at CKD stage 3a and later: LVEF decreased 0.95% (CI: −1.5 to −0.2), GLS increased 0.5 (CI: 0.3 to 0.8), and OR for diastolic dysfunction increased 3.2 (CI 1.4 to 7.3) pr. increment CKD stage group. Conclusion In accordance to previous studies, we observe in the CPHCKD cohort study signs of early increase of LVMI in patients with CKD stage 1 & 2. Significant decline in systolic and diastolic cardiac function is apparent already at stage 3 CKD. Figure 1. Estimated GFR vs. GLS & histogram of GLS Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): The Capital Region of Denmark

scholarly journals FGF-23 and Phosphate in Children with Chronic Kidney Disease: A Cross-Sectional Study in Kazakhstan

Medicina ◽  
2020 ◽  
Vol 57 (1) ◽  
pp. 15
Author(s):  
Altynay Balmukhanova ◽  
Kairat Kabulbayev ◽  
Harika Alpay ◽  
Assiya Kanatbayeva ◽  
Aigul Balmukhanova
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Fibroblast Growth Factor 23 ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional ◽  
Ckd Stage ◽  
Advanced Stages ◽  
Fgf 23 ◽  
Stage 1

Background and objectives: Chronic kidney disease (CKD) in children is a complex medical and social issue around the world. One of the serious complications is mineral-bone disorder (CKD-MBD) which might determine the prognosis of patients and their quality of life. Fibroblast growth factor 23 (FGF-23) is a phosphaturic hormone which is involved in the pathogenesis of CKD-MBD. The purpose of the study was to determine what comes first in children with CKD: FGF-23 or phosphate. Materials and Methods: This cross-sectional study included 73 children aged 2–18 years with CKD stages 1–5. We measured FGF-23 and other bone markers in blood samples and studied their associations. Results: Early elevations of FGF-23 were identified in children with CKD stage 2 compared with stage 1 (1.6 (1.5–1.8) pmol/L versus 0.65 (0.22–1.08), p = 0.029). There were significant differences between the advanced stages of the disease. FGF-23 correlated with PTH (r = 0.807, p = 0.000) and phosphate (r = 0.473, p = 0.000). Our study revealed that the elevated level of FGF-23 went ahead hyperphosphatemia and elevated PTH. Thus, more than 50% of children with CKD stage 2 had the elevating level of serum FGF-23, and that index became increasing with the disease progression and it achieved 100% at the dialysis stage. The serum phosphate increased more slowly and only 70.6% of children with CKD stage 5 had the increased values. The PTH increase was more dynamic. Conclusions: FGF-23 is an essential biomarker, elevates long before other markers of bone metabolism (phosphate), and might represent a clinical course of disease.

scholarly journals P0949EFFECT OF DIETARY PHOSPHORUS RESTRICTION ON FIBROBLAST GROWTH FACTOR,KLOTHO AND BODY COMPOSITION IN CHRONIC KIDNEY DISEASE PATIENTS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Trisha Sachan ◽  
Anita Saxena ◽  
Amit Gupta
Keyword(s):  
Chronic Kidney Disease ◽  
Body Composition ◽  
Renal Function ◽  
Kidney Disease ◽  
Urinary Protein ◽  
Dietary Phosphorus ◽  
Ckd Stage ◽  
Significant Difference ◽  
The Mean ◽  
Fgf 23

Abstract Background and Aims Changes in dietary phosphorus regulate serum FGF-23, parathyroid hormone, 1,25(OH)(2)D and Klotho concentrations . Cardiovascular disease (CVD) is the principal killer of patients with chronic kidney disease and hyperphosphetemia is a potent risk factor it. Of many causative factors for CVD in CKD, dietary interventions involving restriction of dietary phosphorous intake can help reduce onset of CVD at early stages of CKD with other corrective measures. Muscle wasting is a consequence of uremic syndrome which alters body composition. The aim of the study was to study effect of dietary phosphorous restriction on FGF-23, iPTH, Klotho, 1,25(OH)(2)D and body composition in chronic kidney disease patients. Method This is a longitudinal study with 12 months intervention, approved by Ethics Committee of the institute. A total 132 subjects were recruited (66 healthy controls, 66 CKD patient. of 66 patients 33 were in CKD stage 1 and 33 in stage 2. GFR was calculated with the help of MDRD formula. Biochemical parameters of subjects were evaluated at baseline, 6 and 12 months along with the anthropometric measurements (body weight, height, mid upper arm circumference (MUAC), and skin folds). Three days dietary recall was taken to evaluate energy, protein and phosphorous intake. CKD patients whose dietary phosphorous intake was more than 1000 mg/day, were given intense dietary counseling and prescribed dietary modifications by restricting dietary phosphorous between 800-1000 mg/day. Results The mean age of controls and patients was 37.01±9.62 and 38.27±12.06 and eGFR of 136.94±11.77 and 83.69±17.37 respectively. One way ANOVA showed significant difference among controls and the study groups in hemoglobin (p&lt;0.001), s albumin (p&lt;0.001), FGF-23 (p&lt;0.001), klotho (p&lt;0.001), urinary protein (p&lt;0.001) and Nephron Index (p&lt;0.001).The mean energy intake (p = 0.001) and dietary phosphorous intake (p&lt;0.001) of the CKD patients decreased significantly with the decline in the renal function along with the anthropometric measures i.e. BMI (p = 0.041),WHR (p = 0.015) and all four skin folds (p&lt;0.001). On applying Pearson’s correlation, eGFR correlated negatively with urinary protein (-0.739, 0.000), FGF-23 (-0.679, 0.000) and serum phosphorous (-0.697, 0.000) and positively with klotho (0.872, 0.000). FGF-23 correlated negatively with klotho (-0.742, 0.000). Dietary phosphorous was found to be positively correlated with urinary protein (0.496, 0.000), serum phosphorous (0.680, 0.000) and FGF-23 (0.573, 0.000) and negatively with Klotho (-0.602, 0.000). Nephron index revealed a positive correlation with eGFR (0.529, 0.000). Urinary protein correlated negatively with klotho (-0.810, 0.000). A multiple linear regression was run to predict eGFR from anthropometric variables such as BMI, WHR, MUAC, skin folds thickness and handgrip strength. All anthropometric variables predicted decline in eGFR (p&lt;0.05, R2 =0.223). At 6 and 12 months; repeated ANOVAs analysis showed a statistically significant difference in serum creatinine (p=0.000), serum phosphorous (p=0.000), FGF-23(p=0.000) and klotho (p=0.000). Conclusion Elevated levels of FGF-23 and decreased Klotho levels, with the moderate decline in renal function improved with the restricted phosphorous diet at 6 and 12 months emphasizing the importance of phosphorus restriction at an early stage.

FC 012PRIMARY KIDNEY DISEASE IMPACTS OUTCOME IN CKD PREGNANCIES: COMPLICATIONS IN COL4A3-5 RELATED DISEASE (ALPORT SYNDROME) VS OTHER CKD PREGNANCIES

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Rozemarijn Snoek ◽  
Margriet Gosselink ◽  
Liffert Vogt ◽  
Margriet De Jong ◽  
Agne Cerkauskaite ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Pregnancy Outcomes ◽  
Alport Syndrome ◽  
Low Birthweight ◽  
Kidney Diseases ◽  
Related Disease ◽  
Ckd Stage ◽  
Before And After ◽  
Stage 1 ◽  
Italian Cohort

Abstract Background and Aims Chronic kidney disease (CKD) affects approximately 3% of pregnant women. CKD increases the risk of pregnancy complications such as prematurity, low birthweight and pre-eclampsia. Also, kidney function can deteriorate more quickly due to pregnancy. There is limited knowledge on pregnancy outcomes in specific kidney diseases. The aim of the ALPART network is to study pregnancy outcomes differentiated by CKD aetiology. We have started with COLA3-5 related disease (Alport syndrome), which is one of the most prevalent monogenic kidney diseases. Comparing outcomes in COLA3-5 related disease to pregnancies with other CKD aetiologies allows us to investigate whether this specific diagnosis impacts outcome in CKD pregnancies. Method The ALPART network is an international 15-center network, which aims to include ∼200 COLA3-5 related disease pregnancies. In this intermediary analysis, we present data on 109 pregnancies from 68 women with COLA3-5 related disease. We compared outcomes to a cohort of 457 CKD stage 1-2 patients (a similar CKD stage as our cohort) of diverse aetiology from a 2015 Italian study and 159,924 women from the general Dutch population. Results The main pregnancy and kidney outcomes are presented in Figure 1. Foetal outcomes were better in COLA3-5 pregnancies than in pregnancies of women with CKD stage 1-2 of diverse aetiology. We saw less prematurity (17% vs 36% respectively) and a higher mean birthweight of 3216 ± 663 gram compared to 2768 ± 680 in the Italian cohort. Maternal kidney outcomes should be interpreted with caution (&gt;30% missing data): proteinuria (73%) and hypertension (30%) were more frequent in COLA3-5 pregnancies than the Italian cohort. In the ALPART cohort, 10% developed severe hypertension. Median eGFR was not impacted by pregnancy and decline of eGFR before and after pregnancy were not significantly different between groups. Conclusion Fetal outcomes in pregnancies with COLA3-5 related disease seem to be more favorable than in a cohort with mixed cause of CKD. In this intermediary analysis, proteinuria levels and frequency of new-onset hypertension in pregnancy are higher. There is no significant eGFR loss during pregnancy or increased eGFR deterioration in the long-term. The differences between COLA3-5 and general CKD pregnancies underscore the importance of investigating pregnancy outcomes in specific kidney disease phenotypes to ensure adequate (pre-) pregnancy counselling and care.

scholarly journals Comparative analysis of physical development parameters of children with stages 1 to 3 of chronic kidney disease

2017 ◽  
Vol 98 (1) ◽  
pp. 5-9
Author(s):  
T L Nastausheva ◽  
O A Zhdanova ◽  
N S Nastausheva ◽  
L I Stahurlova ◽  
I V Grebennikova
Keyword(s):  
Chronic Kidney Disease ◽  
Comparative Analysis ◽  
Kidney Disease ◽  
Physical Development ◽  
Average Height ◽  
Time Period ◽  
Ckd Stage ◽  
Group 2 ◽  
Stage 1 ◽  
Group 1

Aim. To conduct comparative analysis of height, weight and body mass index in children with stages 1 to 3 of chronic kidney disease (CKD) caused by recurrent urinary tract infection due to congenital anomalies of kidney and urinary tract.Methods. The study was performed on 210 children: 110 patients examined in 2001-2002 (group 1) and 100 children examined in 2011-2012 (group 2). Stage 1 of CKD was observed in 94 (85.4%) children in group 1 and in 93 (93%) in group 2, stage 2 - in 16 (14.6%) and 7 (7%) patients, respectively. From both groups patients matched by sex, age, diagnosis and social status were selected: 20 patients with stage 1, 19 children with stage 2; in addition, 6 children with stage 3 were examined.Results. Nowadays children with CKD stage 1 are taller compared to patients of the beginning of the XXI century (Z-score: -0.14±1.43 and 0.20±0.98 respectively, p=0.01). Significant differences in weight were found in children with stage 1 in 2011-2012 compared to the patients in 2001-2002 (0.18±0.46 and 0.78±1.19 for groups 1 and 2, respectively, р=0.026). A tendency towards decrease of average height in children with stage 3 is observed compared to patients with stage 1, i.e. due to the progression of the disease.Conclusion. The data obtained reflect modern tendencies towards increase of children height and weight. No significant differences were found in physical development parameters of children with stages of chronic kidney disease 1 and 2 examined at the same time period but a tendency towards children’s height decrease from stages 1 to 3 of CKD of non-glomerular etiology was revealed.

scholarly journals Maternal and Fetal Outcomes of Pregnancies in Women with Atypical Hemolytic Uremic Syndrome

2017 ◽  
Vol 29 (3) ◽  
pp. 1020-1029 ◽  
Author(s):  
Martina Gaggl ◽  
Christof Aigner ◽  
Dorottya Csuka ◽  
Ágnes Szilágyi ◽  
Zoltán Prohászka ◽  
...  
Keyword(s):  
Thrombotic Microangiopathy ◽  
Alternative Pathway ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Intrauterine Fetal Death ◽  
Renal Outcome ◽  
Close Monitoring ◽  
Complement Alternative Pathway ◽  
Ckd Stage ◽  
Life Threatening ◽  
Stage 1

Atypical HUS (aHUS) is a disorder most commonly caused by inherited defects of the alternative pathway of complement, or the proteins that regulate this pathway, and life-threatening episodes of aHUS can be provoked by pregnancy. We retrospectively and prospectively investigated 27 maternal and fetal pregnancy outcomes in 14 women with aHUS from the Vienna Thrombotic Microangiopathy Cohort. Seven pregnancies (26%) were complicated by pregnancy-associated aHUS (p-aHUS), of which three appeared to be provoked by infection, bleeding, and curettage, and three individuals were considered to have preeclampsia/HELLP syndrome before the definitive diagnosis of p-aHUS was made. Mutations in genes that encode the complement alternative pathway proteins or the molecules that regulate this pathway were detected in 71% of the women, with no relationship to pregnancy outcome. Twenty-one pregnancies (78%) resulted in a live birth, two preterm infants were stillborn, and four pregnancies resulted in early spontaneous abortions. Although short-term renal outcome was good in most women, long-term renal outcome was poor; among the 14 women, four had CKD stage 1–4, five had received a renal allograft, and three were dialysis-dependent at study end. We prospectively followed nine pregnancies of four women and treated six of these pregnancies with prophylactic plasma infusions (one pregnancy resulted in p-aHUS, one intrauterine fetal death occurred, and seven pregancies were uneventful). Our study emphasizes the frequency of successful pregnancies in women with aHUS. Close monitoring of such pregnancies for episodes of thrombotic microangiopathy is essential but, the best strategy to prevent these episodes remains unclear.

Total serum magnesium in cats with chronic kidney disease with nephrolithiasis

2019 ◽  
Vol 21 (12) ◽  
pp. 1172-1180 ◽  
Author(s):  
Fernanda Chicharo Chacar ◽  
Marcia Mery Kogika ◽  
Andréa C Ferreira ◽  
Khadine K Kanayama ◽  
Archivaldo Reche
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Serum Magnesium ◽  
Magnesium Concentration ◽  
Total Serum ◽  
Serum Magnesium Concentration ◽  
Electrolyte Disturbances ◽  
Kaplan Meier ◽  
Ckd Stage ◽  
Stage 1

ObjectivesMagnesium has been ‘the forgotten ion’ for many years. Over the past decade, however, the role of magnesium in essential physiological functions and several illness conditions have been elucidated. Nevertheless, the investigation of magnesium in cats with chronic kidney disease (CKD) and nephrolithiasis is yet to be determined. The purpose of this study was to investigate whether CKD cats with nephrolithiasis have changes in total serum magnesium concentrations, and whether magnesium disorders may be associated with other electrolyte disturbances, as well as with prognosis. We also aimed to evaluate whether total serum magnesium concentration differs between CKD cats with and without nephrolithiasis.MethodsTotal serum magnesium concentrations were assessed in 42 cats with CKD with stage 1–4 nephrolithiasis. The correlation between magnesium and other electrolytes, as well as Kaplan–Meier survival analysis, were performed. We also selected 14 control cats with CKD without nephrolithiasis age-matched with 14 cats with CKD with nephrolithiasis.ResultsHypermagnesemia was observed in 16/42 (38.1%) and hypomagnesemia in 6/42 (14.3%) cats. Serum magnesium abnormalities were observed in cats of all stages, and marked hypermagnesemia was noted in cats with stage 4 CKD with nephrolithiasis ( P <0.001). There was a negative correlation between total serum magnesium and ionized calcium ( r = −0.64; P <0.01), and a positive correlation between total serum magnesium and serum phosphorus ( r = 0.58, P = 0.01). Cats with CKD with nephrolithiasis and hypomagnesemia or hypermagnesemia had higher mortality than those with normal total serum magnesium concentration ( P <0.01), regardless of CKD stage. There was no difference in total serum magnesium concentration between CKD cats with and without nephrolithiasis.Conclusions and relevanceCats with CKD with nephrolithiasis have magnesium abnormalities. Hypomagnesemia and hypermagnesemia were associated with an increase in mortality, and thus total serum magnesium abnormalities may be used as prognostic factors in these cases.

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