bluuebunny/crossref_metadata_2025_split huggingface dataset:
P1788FACTORS AFFECTING EGFR SLOPE IN KIDNEY TRANSPLANT RECIPIENTS DURING 48 MONTHS AFTER TRANSPLANTATION
Authors: Rambabova Bushljetikj Irena , Dohcev Saso , Mojsova Mijovska Maja , Stavridis Sotir , Stankov Oliver , Spasovski Goce 
Published: June 2020

Abstract

Background and Aims
After the first year following kidney transplantation approximately 3-5% of grafts fail each consecutive year. Many factors have impact on the graft function and only a few may be modifiable. Monitoring of the graft function by serum creatinine concentrations and estimated glomerular filtration rate (eGFR) are recommended methods for evaluation. The aim of the study was to assess the decline of graft function defined by change in the annual eGFR slope and factors that might affect it.

Method
A total number of 55 adult patients with living donor kidney transplant (LDKT) were included in the study. The inclusion criteria were: first transplantation of one organ - kidney, use of living donor related or unrelated (emotionally related - spouses) donor. Clinical and biochemical variables, serum creatinine, BUN, protein status, 24 hours proteinuria and body weight were analyzed at 12, 24, 36 and 48 months after transplantation. The Nankivell equation for estimating glomerular filtration rate was used to calculate the slope of renal function over time (1 to 4 years post-transplantayion) by linear regression analysis.

Results
Out of 55 kidney transplant recipients 18 (33.3%) were female. The majority of transplants had good function (eGFRNankivell ≥ 60 ml/min) at 1 year.
The average GFR at 12 months was 67.81±16.7 mL/min/1.73 m2 with majority of patients at stage 2 of chronic kidney disease classification. Thirty eight patients had eGFR slope &lt;5ml/min/year and 12 patients had a higher slope ( &gt;5ml/min/year).
Only 5 patients (∼ 9%) have graft failure during the period of follow up of 48 months. The average slope decline calculated with linear regression analysis was -3.42 ± 3.2 mL/min/year (range -12.38 – 3.29 ) (slope ± standard error). Proteinuria was predictive for worsening of the graft function. Rapid progressors have been associated with a higher proteinuria at 24 and 48 months: 0.35 ± 0.39 g/24h, median 0.24 (range 0.11 – 0.43 g/24h) and 0.49 ± 0.59 g/24h, median 0.31 (range 0.14 – 0.48 g/24h), (p=0.044 and p=0.021, respectively).
Younger patients have more rapid eGFR slope decline (slope (-7.81 ± 2.12 vs. -2.40 ± 1.3, mL/min/year, p=0,0001). The mean recipient age in the group with stabile eGFR slope was 37.59± 8.7, and in the group with rapid progression was 29.1 ± 8.6 years (p=0.0046).

Conclusion
Proteinuria after kidney transplantation has been identified as a risk factor associated with poor graft survival. Younger renal transplant recipients are associated with more rapid decline of the graft function that could be partially explained with their more potent immune response
P1789DE NOVO INFLAMMATORY BOWEL DISEASE AFTER KIDNEY TRANSPLANTATION - REPORT OF THREE CASES
Authors: Obrencevic Katarina , Petroviä‡ Marijana , Vavic Neven , Petrovic Stanko , Tufegdzic Ivana , Rabrenovic Violeta , Tadiä‡ Jelena , Mijuskovic Mirjana 
Published: June 2020

Abstract

Background and Aims
Inflammatory bowel disease (IBD) are not common after kidney transplantation. Development of IBD in the patient on immunosuppression is unexpected, because IBD is believed to be the result of inappropriate and ongoing activation of the mucosal immune system and immunosuppressive drugs are used in the treatment of IBD. The aim of our study was to evaluate the occurrence of de novo IBD, ulcerative colitis and Crohn’s disease, in the patients who received kidney transplant in our hospital, the time after transplantation and the immunosuppression therapy when diagnosis was made.

Method
We used an electronic medical archival system to identify the patients with IBD. Between 1996 and 2018 a total of 462 kidney transplantations were done in Military Medical Academy. Analysis in this study included 400 patients who were regulary controlled in our hospital. Demographic data and clinical findings including age, sex, primary renal disease, type of kidney donor, and immunosuppressive therapy were evaluated. The time of the occurrance of the IBD after transplantation, the colonoscopy and pathologic findings were also noted.

Results
de novo IBD was observed in three patients 19, 11 and 7 years after kidney transplantation respectively. In all patients end-stage renal disease was due to chronic glomerulonephritis without histology verification. Family history was negative in all. They were male, age 40, 42 i 47 years respectively when diagnosis was made. Their immunosuppressive regimen included prednisone, mycophenolate mofetil (MMF) and tacrolimus with a trough level between 5 and 7 ng/ml. They all developed constitutional symptoms with significant weight loss and fever, abdominal pain and cramps, diarrhoea which was in one case bloody. Infectious and other etiology was excluded. Colonoscopy showed ulcerative colitis in two patients and Crohn’s disease in one. The patients with ulcerative colitis responded on maintenance therapy combined with 5-aminosalicylic acid, but one of them had common flare-ups with mild clinical picture. The patient with Crohn’s disease was successfully treated with azathioprine instead of MMF with high-dose steroids.

Conclusion
We concluded that new onset of IBD can develop after kidney transplantation despite use of immunosuppressive therapy. It should be considered in all transplanted patients who develop gastrointestinal complaints. The future studies could answer why the immunosuppressive therapy is not effective for IBD in these group of patients.
P1795ASSOCIATION BETWEEN T CELL INFILTRATION IN BIOPSY PROVEN ACUTE T CELL MEDIATED REJECTION AND RESPONSE TO THERAPY
Authors: Dalili Nooshin , Ahmadpoor Pedram , Einollahi Behzad , Azhdari Tehrani Hamed 
Published: June 2020

Abstract

Background and Aims
Renal transplantation is considered as the best replacement therapy for advanced ESRD patients. An allograft rejection happens as a result of post transplant immune reactions, which change the outcome of the organ transplantation. Today a major challenge in the field of transplantation is the identification of easy, reliable and non-invasive markers or methods that being able to predict the probability of organ rejection. One of the possible methods is looking for type of infiltrated cells in tissue obtaining by biopsy stained with specific cellular markers and assesses the infiltration of these cells in different types of rejection. Here the severity of CD3, CD20, Th17 and FOXP3 infiltration in patients with biopsy proven acute cellular rejection was evaluated based on IHC staining, whether these specific infiltrations can show an association with graft outcome or not.

Method
50 patients with biopsy proven Acute T Cell Mediated Rejection (ATCMR) recruited. Previous clinical data and 1 year clinical follow up collected. The entire specimen assessed for infiltration of CD3, CD20, FOXP3 Tregs and Th17 with IHC. Patients divided into subgroups: stable graft function versus impaired graft function based on serum creatinine course in one year follow up after rejection therapy and appropriate response to treatment versus failure to response, based on allograft function throughout the course of admission.

Results
In impaired graft function arm, FOXP3 (7.88 vs. 8.02 with P-value 0.96) and Th17 cells were higher (5.01 vs. 10.2 with P-value 0.24) but with non-significant values. FOXP3/Th17 ratio was higher in stable group (1.4 vs. 1.12 with P-value 0.22). In failure to response to therapy group both FOXP3 (9.95 vs. 6.63 with P-value 0.1) and Th17 (11.3 vs. 8.3 with P-value 0.15) cells were higher. FOXP3/Th17 ratio was higher in proper response group (1.19 vs. 1.15 with P-value 0.8). No significant difference was obtained between CD3 and CD20 infiltration in these two groups.

Conclusion
Final results showed that Th17 has more important role in predicting the graft outcome and response to treatment and FOXP3 infiltration had a minor part. This may be in controversial with previous facts about the role of FOXP3 cells, which drive the allograft into stable condition.
P1798MYCOPHENOLIC ACID TROUGH CONCENTRATION AND DOSE ARE ASSOCIATED WITH HEMATOLOGIC ABNORMALITIES BUT NOT REJECTION IN KIDNEY TRANSPLANT RECIPIENTS
Authors: Jung Hee-Yeon , Lee Sukyung , Jeon Yena , Lim Jeong-Hoon , Choi Ji-Young , Cho Jang-Hee , Park Sun-Hee , Kim Yong-Lim , Kim Chan Duck 
Published: June 2020

Abstract

Background and Aims
Little is known regarding the safe fixed dose of mycophenolic acid (MPA) for preventing biopsy-proven acute rejection (BPAR) in kidney transplant recipients (KTRs). We investigated the correlation of MPA trough concentration (MPA C0) and dose with renal transplant outcomes and adverse events.

Method
This study included 79 consecutive KTRs who received MPA with tacrolimus (TAC) and corticosteroids. The MPA C0 of all the enrolled KTRs was measured, which was determined monthly by using particle-enhanced turbidimetric inhibition immunoassay for 12 months, and clinical data were collected at each time point. The clinical endpoints included BPAR, any cytopenia, and BK or cytomegalovirus infections.

Results
No differences in MPA C0 and dose were observed between KTRs with or without BPAR or viral infections under statistically comparable TAC concentrations. MPA C0 was significantly higher in patients with leukopenia (P = 0.021) and anemia (P = 0.002) compared with those without cytopenia. The MPA dose was significantly higher in patients with thrombocytopenia (P = 0.002) compared with those without thrombocytopenia. MPA C0 ≥ 3.5 µg/mL was an independent risk factor for leukopenia (adjusted odds ratio [AOR] 3.80, 95% confidence interval [CI] 1.24–11.64, P = 0.019) and anemia (AOR 5.90, 95% CI 1.27–27.51, P = 0.024). An MPA dose greater than the mean value of 1188.8 mg/day was an independent risk factor for thrombocytopenia (AOR 3.83, 95% CI 1.15–12.78, P = 0.029). However, an MPA dose less than the mean value of 1137.3 mg/day did not increase the risk of BPAR.

Conclusion
Either a higher MPA C0 or dose was associated with an increased risk of cytopenia, but neither a lower MPA C0 nor dose was associated with BPAR within the first year of transplantation. Hence, a reduced MPA dose with TAC and corticosteroids might be safe in terms of reducing hematologic abnormalities without causing rejection.
P1803EFFICACY, SAFETY AND IMPACT ON RENAL FUNCTIONALITY OF ACETAZOLAMIDE TREATMENT IN PATIENTS WITH PMM2-CDG (AZATAX CLINICAL TRIAL)
Authors: Serano Guimaré Mercedes , Arango Sancho Pedro , López Baez Víctor , Codina Sampera Elena , Calzada Baños Yolanda , Vinuesa Jaca Ana , Hernández Zúñiga Lina , Madrid Aris Álvaro 
Published: June 2020

Abstract

Background and Aims
Deficit of phosphomanomutase-(PMM2-CDG) is the most frequent congenital N-glycosylation defect, producing cerebellar syndrome with intellectual deficit and “stroke-like” episodes. It has been associated with renal alterations such as proteinuria, diffuse cortical hyperechogenicity and malformations. We evaluated efficacy, safety and renal repercussion of acetazolamide as a new therapeutic tool.

Method
First clinical trial in phase II including PMM2-CDG patients (5-21-years). First phase: 6 months treatment group with acetazolamide. Second phase: 5 weeks randomized withdrawal in responders (acetazolamide vs placebo), assessing renal functionality and effects of this medication. Dosing acetazolamide by 8 to 30mg/kg/day in 2-3 doses. Controls were performed at 3, 6, 14, 25 and 30 weeks determining acid-base balance, ionogram, renal function (creatinine) and Pr/Cr, Ca/Cr index and B2-microglobulin in first morning urine. All patients underwent in a bone densitometry study and renal ultrasound.

Results
24 patients were included (mean age 12.3 ± 4.5 years). Bicarbonate levels and plasma pH were significantly lower at week 25(p &lt;0.001). 13 patient needs to reduce acetazolamide dose due to excessive metabolic acidosis or asthenia. They showed a decrease in sodium (p=0.06), potassium (p&lt;0.001) and serum calcium(p=0.030), although maintained in low normality limit with a decrease in protein loss(p=0.019) and increase in calcium/creatinine index(p=0.025) without B2-microglobulin alterations. The previously ultrasound renal described findings; the cortical hyperechogenicity was observed in 8.4% without renal dysfunction or associated nephrocalcinosis. One patient presented microlithiasis and another, symptomatic renal lithiasis. Densitometric study 69% of patients presented values in the range of osteopenia at the end of trial (-0.9 to -4.9SD, average of -2.36SD).

Conclusion
The efficacy of acetazolamide in the neurological symptoms of PMM2-CDG, due to an enzymatic stimulation mechanism mediated by acidosis, generates the possibility of chronic treatment with the drug in this group of patients, with possible renal adverse effects associated with long-term, overshadowing the skeletal and renal prognosis.
P1805UNEXPECTD COMPLEMENT GENES ABNORALITIES IN CHILDREN WITH SECONDARY HEMOLITIC UREMIC SYNDROME
Authors: Cirillo Luigi , Becherucci Francesca , Guzzi Francesco , Errichiello Carmela , Buti Elisa , Vaglio Augusto , Materassi Marco , Romagnani Paola , Roperto Rosa Maria 
Published: June 2020

Abstract

Background and Aims
Secondary hemolytic-uremic syndrome (HUS) is currently defined in the absence of genetic defects in complement genes (primary or atypical HUS, aHUS) or Shiga-toxin producing E. Coli infection (STEC-HUS). However, the role of complement genes abnormalities in secondary HUS is still a matter of debate as results coming from studies performed in adults are controversial. Moreover, genetic defects of complement regulation have been recently described in children with STEC-HUS, thus challenging the current HUS classification. The identification of a role of complement genes abnormalities in these patients could have important clinical implications for diagnosis and treatment. In this study, we assessed the presence of complement genes abnormalities in children with secondary HUS.

Method
We describe a case series of pediatric patients diagnosed with secondary HUS. All patients were screened for the presence of ADAMTS13 auto-antibody and ADAMTS13 deficiency. We performed next-generation sequencing for a panel of complement genes reported in association with aHUS (CFH, CFI, MCP, C3, FB, and THBD). Copy number variations and hybrid genes assessment were included in the analysis.

Results
Four patients aged 1-4 years with a diagnosis of sporadic secondary HUS were included in the study. HUS was secondary to glomerulopathy, cobalamin deficiency, bone marrow transplantation, and pneumococcal pneumoniae. Two out of four patients showed hypocomplementaemia (both C3 and C4). Diarrhea was a common clinical feature in all patients at onset, and none of these patients showed neurologic involvement. Renal replacement therapy was required in two patients at onset. Only one patient did not recover kidney function, and subsequently underwent kidney transplantation. Next-generation sequencing showed genetic abnormalities in all the patients (Table 1). All but one genetic variants have already been described in association with atypical HUS. Interestingly, haplotypes in CHF and MCP were present in three patients, all of Caucasian ethnicity.

Conclusion
According to the current HUS classification, complement abnormalities are diagnostic of aHUS. However, results coming from pediatric cohorts of STEC-HUS patients already claimed a role for genetic background also outside the aHUS spectrum, suggesting that the current terminology lacks both specificity and suggestion of cause. In this case series, complement genes abnormalities were present in all patients. Whether confirmed in larger cohorts, these findings would support the role of genetic background even in secondary HUS, suggesting the need for a revision of the current HUS classification, in agreement with the pathogenic mechanisms, in order to tailor the optimal treatment.
P1807COMPARISON BETWEEN INFERIOR VENA CAVA DIAMETER, BIOIMPEDANCE SPECTROSCOPY AND CLINICAL CRITERION FOR DRY WEIGHT ESTIMATION IN HEMODIALYZED CHILDREN
Authors: Askiti Varvara , Malakasioti Georgia , Servos George , Grigoriadou Georgia , Mitsioni Andromachi 
Published: June 2020

Abstract

Background and Aims
The determination of dry weight (DW) in children on chronic hemodialysis (HD) remains problematic. No method has been shown superior for DW assessment in the few pediatric studies exploring the utility of inferior vena cava expiratory and inspiratory diameter diameter (IVCDe, IVCDi) and BIS against clinical criteria. We aimed to compare the performance of IVCD, BIS and clinical judgement in DW prediction in hemodialyzed children.

Method
IVCD and BIS were measured serially pre and post the midweek HD session. IVCDi, IVCDe and IVC contractility index (IVCCI) associations with: (1) BIS estimated (BIS-relOH) and (2) clinically assessed hydration status (OHc) based on deviation from DW were explored. The interpretation of IVC measurements was based on previously published pediatric reference values. The level of agreement between the three tools in fluid overload recognition was studied.

Results
Fifty-two sets of measurements were undertaken in 13 patients (median age 11 years). OHc and BIS-relOH were positively associated to each other (r=0,5, p&lt;0,05) and to IVCDi (r=0,4, r=0,5, p&lt;0,05) and IVCDe (r=0,4, r=0,6, p&lt;0,05). A negative association between IVCCI and relOH-BIS was observed (r= -0,4, p&lt;0,05). There was a poor agreement between the three methods in identification of fluid overload; out of 28 clinically overloaded patients, BIS identified only 7 whereas IVCD none (Cohen’s k &lt;0).

Conclusion
IVCD measurements alone are not reliable for accurate fluid status prediction in hemodialyzed children as they seem to underestimate fluid overload compared to BIS and clinical criteria. Further studies are warranted to explore the applicability of new technologies for DW assessment of dialysis patients.
P1809EARLY URINARY SYSTEM COMPLICATIONS IN CHILDREN WITH HEMATOPOIETIC STEM CELL TRANSPLANTATION
Authors: Gurbanov Anar , Gülhan Bora , Kuşkonmaz Barış , Okur Fatma Visal , Uçkan Çetinkaya Duygu , Ozdemir Gulsah , Taş Nesrin , Çelegen Kübra , Ozaltin Fatih , Duzova Ali , Topaloglu Rezan 
Published: June 2020

Abstract

Background and Aims
Urinary system complications after hematopoietic stem cell transplantation (HSCT) cause severe morbidity and mortality. The aim of the study is to investigate the incidence and risk factors of urinary system complications in patients who had HSCT during their childhood.

Method
Patients who had HSCT between January 2010-2019 with a minimum follow-up period of 6 months were included in the study. Data regarding urinary system complications were collected from the medical records of the patients. pRIFLE and KDIGO classification systems were used for the definition of acute kidney injury (AKI).

Results
167 patients (108 males and 59 females) with allogeneic (n=165) and autologous (n=2) HSCT were investigated for renal complications. In cohort, HSCT was performed in 41 patients (%24.6) secondary to malignant diseases and in 126 patients secondary to non-malignant diseases. Hemorrhagic cystitis (HC) developed in 28 patients (16.8%) after HSCT. The mean age of the patients with and without HC was 14.6±5.6 years and 10.5±6 years, respectively (p=0.044). Among patients with HC, 17 had concomitant viral infection. Presence of viral infection, gender, disease group, history conditioning regime, total body irradiation, acute graft-versus-host disease and veno-occlusive disease (VOD) did not have any effect on the development of HC in logistic regression analysis. In cohort, 126 patients (75.4%) developed AKI according to KDIGO classification (stage 1; 55 patients, stage 2; 36 patients, stage 3; 35 patients). The mean period of development of AKI after HSCT was 34±22 days. The risk of AKI (according to KDIGO) was higher in patients who had HSCT secondary to malignant diseases and/or who developed viral infections after HSCT (p=0.034 and p=0.013, respectively). Among patients, 71 patients (%42.5) developed AKI according to pRIFLE classification. The risk of AKI (according to pRIFLE) was higher in patients who had HSCT secondary to malignant diseases, who developed viral infections and/or who developed VOD during follow-up (p=0.043, p=0.001 and p=0.006, respectively). One patient developed thrombotic microangiopathy after HSCT. None of the patients had nephrotic syndrome during follow-up period.

Conclusion
Patients who had HSCT secondary to malignant disease, history of viral infections and/or VOD had higher risk of AKI and should be closely monitored.
P1823THE ROLE OF FGF-23 IN CKD PROGRESSION IN CHILDREN
Authors: Balmukhanova Altynay , Kabulbayev Kairat , Kanatbayeva Assiya , Alpay Harika , Balmukhanova Aigul , Shepetov Abay 
Published: June 2020

Abstract

Background and Aims
Chronic kidney disease (CKD) is considered a global medical and public health issue. CKD takes a special place among non-infectious diseases because of its prevalence (6-20% according to different surveys and studies) and is associated with a poor life quality, complications and high risk of mortality. In recent years, there have been new biomarkers requiring more research in this area. One of these biomarkers is Fibroblast growth factor-23 (FGF-23) which is found as a bone derived hormone and might be a predictor of progression. However, the role of FGF-23 in CKD progression in children has not been adequately studied, especially on the early stages. Nowadays, the study of FGF-23 in children and the question of the clinical importance of this marker are relevant. Therefore, the aim of our study was to establish the role of FGF-23 in CKD progression in children.

Method
A prospective study was conducted on 73 children with different stages of CKD and 14 healthy individuals (control group) matched by age and gender. There were approximately equal numbers of patients in study groups. An average age was 9.61±1.05 years. Exclusion criteria: active inflammatory, bone, infectious, oncological, immunological diseases, taking steroids and vitamin D supplements. Laboratory measurements included all common clinical and biochemical indicators. Serum concentration of intact FGF-23 was assessed by using the ELISA method (Biomedica Medizinprodukte GmbH, Austria). Statistical analysis was conducted in MS Excel 2016 and SPSS 18.0.

Results
The normal range of FGF-23 for this kit was 0.1-1.5 pmol/l. The average value of FGF-23 in the control group was 0.69±0.12 pmol/l. Further studies in the groups with different stages of CKD revealed that FGF-23 concentration gradually rose in parallel with stages of CKD, and it reached the maximum on the last stage. It should be noticed that the level of FGF-23 concentration on the first stage of CKD was normal (0.73±0.14 pmol/l) and the comparison with healthy individuals revealed no significant differences. What is remarkable, despite the fact that the average value of the second stage patients was normal (1.36±0.2 pmol/l), there was a statistically significant difference with the control group (p=0.008). The levels of FGF-23 on the next stages were 2.52±0.52 pmol/l, 5.42±1.61 pmol/l, and 12.16±1.55 pmol/l, respectively. The differences were considerable and proved by statistical analysis (p&lt;0.01).

Conclusion
Our study showed that there is an upward trend of FGF-23 as CKD progresses from early to advanced stages. The results on the second and third stages indicate that FGF-23 should be considered as one of early biomarkers of CKD progression in children. Thus, there is a need for more studies in this area.