scholarly journals Infantile nephropathic cystinosis

2011 ◽  
Vol 139 (7-8) ◽  
pp. 486-490 ◽  
Author(s):  
Amira Peco-Antic ◽  
Mirjana Kostic ◽  
Radovan Bogdanovic ◽  
Brankica Spasojevic ◽  
Maja Djordjevic ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Graft Function ◽  
Nephropathic Cystinosis ◽  
Paediatric Patients ◽  
Carrier Mediated Transport ◽  
Recent Statement ◽  
Cadaver Kidney ◽  
Male Patients ◽  
Stage Renal Disease ◽  
End Stage

Introduction. Infantile nephropathic cystinosis (INC) is a metabolic disorder due to impaired carrier-mediated transport of cystine out of cellular lysosomes. Objective. To examine the prevalence and clinical characteristics of INC in paediatric patients with end- stage renal disease (ESRD) in Serbia and give a recent statement of the disease. Methods. ESRD database of the Centre for Paediatric Renal Replacement Therapy (RRT) in Serbia was used to identify all patients with INC who started RRT before age of 19 years during the period January 1980 - December 2008; their records concerning clinical characteristics, therapy and outcome were evaluated. Results. Only three of 298 paediatric patients with ESRD had INC. The first signs of the illness were recognised during infancy. Fancony syndrome was diagnosed in the second year, but the diagnosis of cystinosis was delayed at mean 6 years. ESRD occurred in the first decade of life. All patients under- went cadaver kidney transplantation. At the end of the study period all patients were alive. A 31-year-old female patient was on maintenance chemodialysis due to graft failure after functioning for 11 years. She was growth retarded, single, unemployed, with severe signs of renal dystrophy. Two male patients (14.3 and 14.7 years old) had normal graft function, normal education, and good quality of life, although they were also severe growth retarded. Conclusion. The prevalence of infantile nephropathic cystinosis is low in Serbia. The diagnosis of cystinosis was delayed in all patients, although they exhibited the typical course of the disease.

scholarly journals AB0820 INFLUENCE OF ANCA ANTIBODIES ON DEMOGRAPHIC AND CLINICAL CHARACTERISTIC OF AAV

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1434.1-1434
Author(s):  
K. Wójcik ◽  
A. Masiak ◽  
Z. Zdrojewski ◽  
R. Jeleniewicz ◽  
M. Majdan ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Male To Female

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the broad clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Unfortunately the clinical classification, ANCA specificity or genetic characteristics alone is not able to categorize AAV patients in a satisfactory manner. As a consequence advanced statistical techniques were used to identify and stratify AAV subphenotypes [1, 2]. Here we have analyzed influence of the ANCA type on clinical manifestations and demographic characteristics in various types of AAV, based on data from the POLVAS registryObjectives:We decided to retrospectively analyze a large cohort of Polish AAV patients deriving from several referral centers – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS) – and concentrate on demographic and clinical characteristics of anti-PR3 and anti-MPO positive patients regardless of their clinical diagnosis.Methods:We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016. Patients were enrolled by 9 referral centers. We analyzed dichotomous variables: gender; ANCA status – anti-PR3+ or anti-MPO+, ANCA negative; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves and polytomous variable (number of relapses), supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever)[3].Results:MPO-positive patients (both GPA and EGPA phenotype) were older at the time of diagnosis with a substantial percentage diagnosed > 65 years of age, and with high rate of renal involvement. Interestingly, while in the whole group of patients diagnosed with EGPA male to female ratio was 1:2, the MPO+ EGPA patients showed M:F ratio of 1:1.The analysis of ANCA negative AAV reveled significant differences in GPA, ANCA negative group is characterized with significantly lower frequency of renal involvement compared to rest GPA (11,5% vs 63,7%) p<0,05 what should be emphasized ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.Conclusion:ANCA specificity is indispensable as a separate variable in any clinically relevant analysis of AAV subcategories. MPO+ group is characterized by older age at time of diagnosis, male to female ration 1:1, kidney involvement, and shows more homogenous clinical phenotype than PR3+ AAV patients. In our group ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9. https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Biedroń G, Wawrzycka-Adamczyk K, Bazan-Socha S, Ćmiel A, Zdrojewski Z et al. Subphenotypes of ANCA-associated vasculitis identified by latent class analysis. Clin Exp Rheumatol. 2020 Sep 1. Epub PMID: 32896241.[3]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:None declared

scholarly journals Laparoscopic-Assisted Recipient Nephrectomy and Recipient Kidney Procurement during Orthotopic Living-Related Kidney Transplantation

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Dimitri Mikhalski ◽  
Karl Martin Wissing ◽  
Renaud Bollens ◽  
Daniel Abramowicz ◽  
Vincent Donckier ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Antiphospholipid Antibodies ◽  
Graft Function ◽  
Renal Vessels ◽  
Short Period ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage ◽  
Living Related ◽  
Laparoscopic Assisted

Advanced atherosclerosis or thrombosis of iliac vessels can constitute an absolute contraindication for heterotopic kidney transplantation. We report the case of a 42-year-old women with end-stage renal disease due to lupus nephritis and a history of bilateral thrombosis of iliac arteries caused by antiphospholipid antibodies. Occlusion had been treated by the bilateral placement of wall stents which precluded vascular anastomosis. The patient was transplanted with a right kidney procured by laparoscopic nephrectomy from her HLA semi-identical sister. The recipient had left nephrectomy after laparoscopical transperitoneal dissection. The donor kidney was orthotopically transplanted with end-to-end anastomosis of graft vessels to native renal vessels and of the graft and native ureter. Although, the patient received full anticoagulation because of a cardiac valve and antiphospholipid antibodies, she had no postoperative complication in spite of a short period of delayed graft function. Serum creatinine levels three months after transplantation were at 1.0 mg/dl. Our case documents that orthotopical transplantation of laparoscopically procured living donor kidneys at the site of recipient nephrectomy is a feasible procedure in patients with surgical contraindication of standard heterotopic kidney transplantation.

scholarly journals CHILDRENONHEMODIALYSIS: A RETROSPECTIVESTUDYIN BENGHAZI PEDIATRIC HOSPITAL

2020 ◽  
Vol 8 (12) ◽  
pp. 827-832
Author(s):  
Narges M. Kablan ◽  
◽  
Khadija A. Ali ◽  
Kamlah J. Said ◽  
Mabroka A. Ali ◽  
...  
Keyword(s):  
Body Weight ◽  
White Blood Cells ◽  
Life Quality ◽  
Pediatric Hospital ◽  
Dialysis Unit ◽  
Major Health Problem ◽  
Drug History ◽  
Male Patients ◽  
Stage Renal Disease ◽  
End Stage

End stage renal disease (ESRD) is a major health problem worldwide. In Libya, limited studies are available on children with ESRD.Regular assessment of laboratory parameters is the only way to reduce their risk of mortality.This study aimed to determinethe demographic characteristics and evaluate the hematological profile of children on hemodialysis (HD) admitted to the dialysis unit in Benghazi Pediatric hospital, Benghazi, Libya during the period 3rd of December, 2017 to 15th of January 2018.A structured form was used to record data collected from patients files. Data includedage, gender, body weight, treatment history, drug history, duration and frequency of HD and laboratory tests results, specifically white blood cells (WBC), hemoglobin (HB), blood urea, glucose, Albumin, uric acid, serum creatinine, serum iron, calcium, phosphate, sodium and potassium.Number of patients on HD includedin this study was seven with average age of 11years,the majority (71%)were males.Average body weight of female patients was24.2kg,while male patients averagebody weight was25.52kg. Most of the patients(57%)hadhigh BP.71%of patientsstarted dialysis sincemore than one year.Patients underwentdialysisthree times a week represented(86%),while the rest of patients underwent dialysis four times a weekrepresented(14%).All patients had anemia and highcreatininelevel.Providing an appropriate care for children on maintenance dialysis in Libya is quitedifficult. Increasing the awareness ofparents about ESRD is necessary to improve the life quality of children with ESRD.

scholarly journals TO004IMPACT OF RENAL TRANSPLANT ON COGNITIVE FUNCTION AMONG ESRD PATIENTS ON DIALYSIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Anupma Kaul ◽  
Dharmendra Bhaduria ◽  
Narayan Prasad ◽  
Amit Gupta
Keyword(s):  
Cognitive Function ◽  
Kinetic Modelling ◽  
Memory Performance ◽  
Graft Function ◽  
Post Transplant ◽  
Formal Kinetic ◽  
Psychomotor Ability ◽  
Normative Comparisons ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background and Aims Kidney transplantation can help in mitigating neuropsychological (NP) impairments in end-stage renal disease (ESRD). Evidences have shown mixed responses on the the nature of cognitive function in patients who have undergone renal transplantation.Present study looked into the factors impacting cognitive domains and impact post transplant. Method 43 patients who were stable ESRD on thrice a week hemodialysis were investigated 6 months pre and post transplant using a battery of neurophysiological testing assessing attention–concentration, psychomotor ability and memory. Formal kinetic modelling of dialysis delivery ensured adequate renal replacement therapy. Transplant function was assessed on regular interval and following a stable graft function and on stable doses of immunosuppressive medication 6 months post transplant were reassessed Results Within-subject comparisons showed statistically significant improvement in memory performance after kidney transplant. Other NP measures (attention– concentration and psychomotor abilities) showed non-significant improvements. Normative comparisons showed NP impairments on dialysis, which were not apparent after transplant Conclusion These data demonstrate improvements in cognition following kidney TX and emphasize the reversibility of the memory problems evidenced in dialysis

scholarly journals Self-Care Behavior Profiles With Arteriovenous Fistula in Hemodialysis Patients

2018 ◽  
Vol 29 (6) ◽  
pp. 363-367 ◽  
Author(s):  
Clemente Neves Sousa ◽  
Patrícia Marujo ◽  
Paulo Teles ◽  
Marta Nunes Lira ◽  
Vanessa Filipa Ferreira Dias ◽  
...  
Keyword(s):  
Arteriovenous Fistula ◽  
Self Care ◽  
Dialysis Unit ◽  
Dialysis Vintage ◽  
Gender Education ◽  
Male Patients ◽  
Stage Renal Disease ◽  
End Stage ◽  
Behavior Profiles ◽  
Care Behavior

Patients with end-stage renal disease should be educated and trained to take care of their own arteriovenous fistula (AVF) with the purpose of developing self-care behaviors concerning vascular access. This was a prospective and observational study. We designed this research to identify clinically meaningful self-care behavior profiles in hemodialysis (HD) patients, and it was carried out in a private dialysis unit in the Lisbon region, Portugal, involving 101 patients. The proportion of male patients was 66.3%, the mean age was 60.9 years, and the frequency of self-care behaviors was 71%. Cluster analysis based on the subscale scores grouped patients in two clusters named “moderate self-care” and “high self-care.” Those profiles exhibit significant differences concerning gender, education, employment, dialysis vintage, AVF duration, and information on care with the AVF. Identification of self-care-behavior profiles in HD patients with AVF enables one to adjust education programs to the patients’ characteristics.

scholarly journals Clinical characteristics of bladder tumors in hemodialysis patients: Bladder tumors in end-stage renal disease patients

2019 ◽  
Vol 52 (11) ◽  
pp. 639-641
Author(s):  
Hirofumi Shimoyama ◽  
Masahiro Shimoyama ◽  
Akihisa Watanabe ◽  
Satoru Hirose ◽  
Riichi Kurane ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
Hemodialysis Patients ◽  
Bladder Tumors ◽  
Stage Renal Disease ◽  
End Stage

scholarly journals Emergency Department Use by Patients with End-stage Renal Disease in the United States

2020 ◽  
Author(s):  
Ningyuan Wang ◽  
Jiao Pei ◽  
Hui Fan ◽  
Yaseen Ali ◽  
Jian Zhao ◽  
...  
Keyword(s):  
United States ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
The United States ◽  
Adult Age ◽  
Ed Visits ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

Abstract Background: We sought to describe the national characteristics of ED visits by patients with end-stage renal disease (ESRD) in the United States in order to improve the emergency treatment and screening of ESRD patients. Methods: We analyzed data from 2014-2016 ED visits provided by the National Hospital Ambulatory Medical Care Survey. We sampled adult (age ≥ 18 years) ED patients with ESRD. By proportion or means of weighted sample variables, we quantified annual ED visits by patients with ESRD. We investigated demographics, ED resource utilization, clinical characteristics, and disposition of patients with ESRD vs. those without ESRD. Logistic regression models were used to estimate the association between these characteristics and ESRD ED visits. Results: Approximately 722,692 (7.78%) out of 92,899,685 annual ED visits were ESRD patients. ED patients with ESRD were mostly non-Hispanic whites (51.5%) and males were more likely to be ESRD patients than females (aOR: 1.48; 95% CI: 1.47–1.48). ED patients with ESRD were more likely to be admitted to the hospital (aOR: 2.22; 95% CI: 2.21–2.22) and intensive care unit (ICU) (aOR: 1.53; 95% CI: 1.52–1.54) than patients without ESRD. ED patients with ESRD were more likely to receive blood tests, get medical imaging tests.Conclusion: We described the unique demographic, socioeconomic, and clinical characteristics of ED patients with ESRD, using the most comprehensive, nationally representative study to date. These patients’ higher hospital and ICU admission rates indicate that patients with ESRD require a higher level of emergency care.

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