BMC Nephrology最新文献

筛选
英文 中文
Association between TMSE/MoCA and MIS/NAF in ESKD patients undergoing hemodialysis: a cross-sectional study. 接受血液透析的 ESKD 患者的 TMSE/MoCA 与 MIS/NAF 之间的关系:一项横断面研究。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-30 DOI: 10.1186/s12882-024-03729-1
Taksaporn Lertritdecha, Pichaya Tantiyavarong, Aphichat Chatkrailert
{"title":"Association between TMSE/MoCA and MIS/NAF in ESKD patients undergoing hemodialysis: a cross-sectional study.","authors":"Taksaporn Lertritdecha, Pichaya Tantiyavarong, Aphichat Chatkrailert","doi":"10.1186/s12882-024-03729-1","DOIUrl":"https://doi.org/10.1186/s12882-024-03729-1","url":null,"abstract":"<p><strong>Background: </strong>Both cognitive impairment and malnutrition are common in hemodialysis (HD) patients and are associated with increased hospitalization rates, infection, poor clinical outcomes, and mortality. The study investigated the association between cognitive and nutrition status among end-stage kidney disease (ESKD) patients undergoing hemodialysis.</p><p><strong>Methods: </strong>In this cross-sectional study, we enrolled 115 patients with ESKD who underwent regular hemodialysis (HD). Data collection included the use of screening tools for mild cognitive impairment (MCI), specifically Thai Mental State Examination (TMSE) and Montreal Cognitive Assessment (MoCA). In addition, we collected data using nutritional screening tools including Malnutrition Inflammation Score (MIS) and Nutrition Alert Form (NAF). Our primary outcome was to demonstrate whether there was a relationship between TMSE/MoCA and MIS/NAF scores in this population. Secondary outcomes were a prevalence of MCI and malnutrition status in ESKD patients, an association between TMSE and MoCA with other surrogate nutritional markers, and factors affecting MCI in such patients.</p><p><strong>Results: </strong>A total of 109 patients undergoing HD completed our protocol. Their mean age was 63.42 (± 15.82) years, and 51.38% were male. Mean TMSE and MoCA were 23.98 (± 5.06) points and 18.3 (± 6.40) points, respectively. The prevalence of TMSE ≤ 23 and MoCA ≤ 24 were 39.45% and 83.49%, respectively. TMSE had a statistically significant negative correlation with MIS (R<sup>2</sup> = 0.16, p < 0.001) and NAF. MoCA also negatively correlated with MIS and NAF. The age, total educational year, the status of whether having a caregiver, serum albumin, serum phosphorus level, handgrip strength, and lean mass tissue were correlated with TMSE.</p><p><strong>Conclusion: </strong>Nutritional parameters, including MIS score, NAF score, serum albumin, lean tissue mass, and lean tissue index, significantly correlate with TMSE and MoCA.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11363605/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142104170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sunitinib-induced endocapillary proliferative glomerulonephritis with IgA2 deposit in addition to thrombotic microangiopathy: a case report. 舒尼替尼诱发的内毛细血管增生性肾小球肾炎伴有IgA2沉积和血栓性微血管病:一份病例报告。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-30 DOI: 10.1186/s12882-024-03732-6
Xin Zhang, Hui Wang, Jian Li, Fude Zhou, Minghui Zhao, Tao Su
{"title":"Sunitinib-induced endocapillary proliferative glomerulonephritis with IgA2 deposit in addition to thrombotic microangiopathy: a case report.","authors":"Xin Zhang, Hui Wang, Jian Li, Fude Zhou, Minghui Zhao, Tao Su","doi":"10.1186/s12882-024-03732-6","DOIUrl":"10.1186/s12882-024-03732-6","url":null,"abstract":"<p><strong>Background: </strong>Sunitinib, a multi-targeted tyrosine kinase inhibitor, is used as a second-line therapy for gastrointestinal stromal tumors (GIST) resistant to imatinib. However, its impact on the vascular endothelial growth factor (VEGF) pathway can lead to significant toxicities, including hypertension and thrombotic microangiopathy (TMA).</p><p><strong>Case presentation: </strong>This case report describes a unique instance of a patient with metastatic GIST who developed endocapillary proliferative glomerulonephritis (EPGN) with IgA2 deposits and TMA following sunitinib treatment. The patient presented with severe hypertension, nephrotic syndrome, and acute kidney injury. Renal biopsy confirmed the diagnosis, revealing IgA2 deposits, which are not commonly associated with TMA. Discontinuation of sunitinib led to a rapid improvement in renal function and proteinuria. The potential mechanisms underlying sunitinib-induced glomerular injury may involve the blockade of VEGFR-1, affecting immune cell recruitment and function, and the disruption of the nitric oxide and endothelin systems, leading to endothelial damage and immune dysregulation. Management of these toxicities requires a personalized approach, with options ranging from symptomatic relief to drug discontinuation. The use of endothelin receptor antagonists and other therapeutic alternatives for GIST management is discussed.</p><p><strong>Conclusions: </strong>This case highlights the complex interplay between the therapeutic effects of sunitinib and its potential renal and cardiovascular toxicities, emphasizing the need for close monitoring and effective management strategies to optimize patient outcomes.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11365231/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142104239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Giant retroperitoneal liposarcoma with multiple organ involvement: a case report and literature review. 多器官受累的巨大腹膜后脂肪肉瘤:病例报告和文献综述。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-29 DOI: 10.1186/s12882-024-03701-z
Jia-Ning Sun, Rui Yang, Xiao-Lu Jiang, Feng Zhang, Hong-Wei Zhao
{"title":"Giant retroperitoneal liposarcoma with multiple organ involvement: a case report and literature review.","authors":"Jia-Ning Sun, Rui Yang, Xiao-Lu Jiang, Feng Zhang, Hong-Wei Zhao","doi":"10.1186/s12882-024-03701-z","DOIUrl":"https://doi.org/10.1186/s12882-024-03701-z","url":null,"abstract":"<p><strong>Background: </strong>Retroperitoneal liposarcoma (RPLPS) is a relatively rare disease. Liposarcomas vary in size, but sizeable RPLPS larger than 30 cm in diameter are very rare, and their diagnosis and treatment present significant challenges.</p><p><strong>Case presentation: </strong>We report a 58-year-old male patient who was admitted to the hospital with an increased abdominal circumference and was later diagnosed with a giant RPLPS. The liposarcoma was found to adhere to the right kidney and the entire ureter, invading the ascending colon. The patient underwent complete combined surgical resection. The tumor was removed intact, measured 55.0 cm × 30.0 cm × 18.0 cm, and weighed 19.8 kg. Histopathologic analysis revealed well-differentiated liposarcoma (WDLPS). The patient was successfully discharged from the hospital and followed up for 6 months with no signs of recurrence.</p><p><strong>Conclusion: </strong>RPLPS is a rare tumor with atypical clinical presentation. Surgery remains the most effective method of treatment for retroperitoneal sarcomas, with complete removal if there is local invasion. Preoperative examination, including three-dimensional (3D) reconstruction, is essential for surgical success. The role of adjuvant radiotherapy or chemotherapy remains controversial. However, clinicians should not rule them out as viable options.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11363381/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142104237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Time to death and its predictors among patients with chronic kidney disease on hemodialysis at dialysis unit in Addis Ababa, Ethiopia: a retrospective cohort study. 埃塞俄比亚亚的斯亚贝巴透析室接受血液透析的慢性肾病患者的死亡时间及其预测因素:一项回顾性队列研究。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-28 DOI: 10.1186/s12882-024-03719-3
Azeb Haile Tesfaye, Adamu Birhanu, Tufa Kolola, Leja Hamza, Diriba Etana
{"title":"Time to death and its predictors among patients with chronic kidney disease on hemodialysis at dialysis unit in Addis Ababa, Ethiopia: a retrospective cohort study.","authors":"Azeb Haile Tesfaye, Adamu Birhanu, Tufa Kolola, Leja Hamza, Diriba Etana","doi":"10.1186/s12882-024-03719-3","DOIUrl":"10.1186/s12882-024-03719-3","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease is a progressive disease that affects more than 10% of the world's population and is also the leading cause of death in the twenty-first century. Furthermore, it imposes a significant financial burden on people undergoing hemodialysis. However, there is little research, particularly in the study area, on time to death and its predicators among hemodialysis patients in Ethiopia; therefore, knowing time to death and identifying predicators that affect survival time is crucial in order to improve survival time and enhance the prognosis of hemodialysis patients. The aim of this study was to assess time to death and its predictors among patients with chronic kidney disease on hemodialysis at a dialysis unit in Addis Ababa, Ethiopia, in 2023.</p><p><strong>Methods: </strong>An institution-based retrospective cohort study was carried out among 370 chronic kidney disease patients on hemodialysis from January 1st, 2017 to December 30th, 2022. Data were extracted from April 1st-May 20th, 2023, and each variable was coded and entered into Epi Data version 3.1 and then exported into STATA version 15 software for analysis. Kaplan-Meier and the log-rank test were done. Bivariable Cox-proportional regression was done, and a variable whose p-value was < 0.25 and fulfilled the proportional hazard assumption by using graphical and Shenfield residuals was entered into multivariable Cox-proportional regression. Finally, a variable whose p-value < 0.05 and adjusted hazard ratio with its CI were declared statistically significant predictors.</p><p><strong>Result: </strong>In this study, the overall median survival time was 47 months (95% CI: 36.7, 56), with an incidence rate of death of 16.8 per 1000 people per month of observation (95% CI: 13.8-20.3). Age 64 and above (Adjusted Hazard Ration: 2.8; 95% CI: 1.67-4.98), catheter vascular access (Adjusted Hazard Ration: 3.47; 95% CI: 2.03-5.93), cardiovascular disease (Adjusted Hazard Ration: 1.88; 95% CI: 1.15-3.07), and blood group B (Adjusted Hazard Ration: 2.07; 95% CI: 1.17-3.69) were significant predictors of time to death among hemodialysis patients. CONCLUSION AND RECOMMENDATION: The median survival time was 47 months, with an interquartile range of 40. Cardiovascular disease, older adults, central venous catheters, and blood type B were significant predictors of time to death for hemodialysis patients. Therefore, in order to improve the survival of hemodialysis patients, health professionals and concerned bodies should give concern to and work on those predictors.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11360759/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142092184","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Non-tunneled haemodialysis catheter-related blood stream infections and associated factors among first time haemodialysis patients: a prospective study from a tertiary care hospital in Sri Lanka. 首次血液透析患者中与非隧道血液透析导管相关的血流感染及相关因素:斯里兰卡一家三级医院的前瞻性研究。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-28 DOI: 10.1186/s12882-024-03726-4
Chanaka Muthukuda, Vindika Suriyakumara, Thilina Samarathunga, Lakshika Liyanage, Arjuna Marasinghe
{"title":"Non-tunneled haemodialysis catheter-related blood stream infections and associated factors among first time haemodialysis patients: a prospective study from a tertiary care hospital in Sri Lanka.","authors":"Chanaka Muthukuda, Vindika Suriyakumara, Thilina Samarathunga, Lakshika Liyanage, Arjuna Marasinghe","doi":"10.1186/s12882-024-03726-4","DOIUrl":"10.1186/s12882-024-03726-4","url":null,"abstract":"<p><strong>Background: </strong>A significant number of patients require non-tunneled haemodialysis catheters (NTHCs) in the event of an urgent need for immediate haemodialysis in developing countries. Catheter-related bloodstream infections (CRBSIs) are a major concern in haemodialysis, but there is a lack of local epidemiological data. This study aimed to determine the incidence of CRBSI, causative agents and associated risk factors in a tertiary care hospital in Sri Lanka.</p><p><strong>Methods: </strong>A prospective study was conducted at the dialysis unit of Colombo South Teaching Hospital, Sri Lanka from December 2019 to August 2020. Adult patients who had haemodialysis for the first time with NTHCs were included.</p><p><strong>Results: </strong>Of 149 dialysis patients (104-jugular vein and 45-femoral vein, mean age 58 ± 13.7 years, mean duration of catheterization 7.9 ± 3.4 days), the incidence of CRBSI was 13.58 per 1000 catheter days. Serum albumin levels, capillary blood sugar levels at admission, haemoglobin levels and duration of catheterization were significantly associated with CRBSI. Prescence of diabetes and patients with ESRD who started routine haemodialysis had a significantly higher risk of CRBSI. Gram-positive bacteria were the most common microorganisms associated with CRBSI (87.5%).</p><p><strong>Conclusions: </strong>Our results show high rates of infection with temporary vascular catheters in Sri Lanka, mainly due to Gram-positive bacteria. Diabetes mellitus, duration of catheterisation, low serum albumin, haemoglobin level and CBS on admission were identified as significant risk factors for CRBSI. Management strategies tailored to specific centers should be established in the nation to optimise catheter care and to monitor local microbiology for appropriate empirical antimicrobial treatment.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11361233/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142092183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A longitudinal assessment of heat exposure and biomarkers of kidney function on heat shock protein 70 and antibodies among agricultural workers. 农业工人热暴露与热休克蛋白 70 和抗体肾功能生物标志物的纵向评估。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-28 DOI: 10.1186/s12882-024-03706-8
Jaime Butler-Dawson, Richard J Johnson, Lyndsay Krisher, Diana Jaramillo, Alex Cruz, Daniel Pilloni, Stephen Brindley, Bernardo Rodriguez-Iturbe, Laura Gabriela Sanchez-Lozada, Miranda Dally, Lee S Newman
{"title":"A longitudinal assessment of heat exposure and biomarkers of kidney function on heat shock protein 70 and antibodies among agricultural workers.","authors":"Jaime Butler-Dawson, Richard J Johnson, Lyndsay Krisher, Diana Jaramillo, Alex Cruz, Daniel Pilloni, Stephen Brindley, Bernardo Rodriguez-Iturbe, Laura Gabriela Sanchez-Lozada, Miranda Dally, Lee S Newman","doi":"10.1186/s12882-024-03706-8","DOIUrl":"10.1186/s12882-024-03706-8","url":null,"abstract":"<p><strong>Background: </strong>Exposure to extreme heat impacts millions of people worldwide and outdoor workers are among the populations most affected by hot temperatures. Heat stress induces several biological responses in humans, including the production of heat shock proteins (HSP) and antibodies against HSP (anti-HSP) which may play a central role in the body's cellular response to a hot environment.</p><p><strong>Objective: </strong>This longitudinal study investigated the impact of elevated temperatures and humidity on the presence of HSP70 and anti-HSP70 and examined relationships with markers of kidney function in an at-risk workforce under conditions of extreme heat and exertion in Guatemala.</p><p><strong>Methods: </strong>We collected ambient temperature and relative humidity data as well as biomarkers and clinical data from 40 sugarcane workers at the start and the end of a 6-month harvest. We used generalized mixed-effects models to estimate temperature effects on HSP70 and anti-HSP70 levels. In addition, we examined trends between HSP70 and anti-HSP70 levels and markers of kidney function across the harvest.</p><p><strong>Results: </strong>At the end of the harvest, temperatures were higher, and workers had, on average, higher levels of HSP70 and anti-HSP70 compared to the beginning of the season. We observed significant increasing trends with temperature indices, heat index, and HSP70 levels. Maximum temperature was associated with HSP70 increments after controlling for age, systolic and diastolic blood pressure (β: 0.21, 95% Confidence Interval: 0.09, 0.33). Kidney function decline across the harvest was associated with both higher levels of anti-HSP70 levels at the end of the harvest as well as greater increases in anti-HSP70 levels across the harvest.</p><p><strong>Conclusions: </strong>These results suggest that workplace heat exposure may increase the production of HSP70 and anti-HSP70 levels and that there may be a relationship between increasing anti-HSP70 antibodies and the development of renal injury. HSP70 holds promise as a biomarker of heat stress in exposed populations.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11351828/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142092181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effect of platelet indices on mortality and comorbidity in peritoneal dialysis: a cohort study. 血小板指数对腹膜透析患者死亡率和合并症的影响:一项队列研究。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-28 DOI: 10.1186/s12882-024-03697-6
Xiao-Qing Zhang, Xin-Kui Tian, Ling Wang, Wen Tang
{"title":"Effect of platelet indices on mortality and comorbidity in peritoneal dialysis: a cohort study.","authors":"Xiao-Qing Zhang, Xin-Kui Tian, Ling Wang, Wen Tang","doi":"10.1186/s12882-024-03697-6","DOIUrl":"10.1186/s12882-024-03697-6","url":null,"abstract":"<p><strong>Background: </strong>There were limited data investigating platelet indices in predicting peritoneal dialysis (PD) outcomes on comorbidities. The aim of this study was to evaluate the association between platelet indices and new-onset comorbidity and all-cause mortality in PD patients.</p><p><strong>Methods: </strong>A single-center, retrospective observational cohort study was conducted in incident PD patients from 28 December 2011 to 24 January 2018, and followed up until 31 December 2022. Time to the first new-onset cardiovascular disease (CVD) and time to the first new-onset infection event after PD were identified as the primary outcomes. All-cause mortality was identified as the secondary endpoint. The correlation between platelet indices and comorbidities and all-cause mortality were assessed by Cox model. Data of liver disease status was not collected and analyzed. Survival curves were performed by Kaplan-Meier method with log-rank tests.</p><p><strong>Results: </strong>A total of 250 incident PD patients with a median follow-up of 6.79 (inter-quarter range 4.05, 8.89) years was included. A total of 81 and 139 patients experienced the first new-onset CVD and infection event respectively during the follow-up period. High mean platelet volume (MPV) was independently associated with high risk of time to the first new-onset CVD (HR 1.895, 95% CI 1.174-3.058, p = 0.009) and all-cause mortality (HR 1.710, 95% CI 1.155-2.531, p = 0.007). Patients with low mean platelet volume to platelet count ratio (MPV/PC) were prone to occur the new-onset infection events (log rank 5.693, p = 0.017). Low MPV/PC (HR 0.652, 95% CI 0.459-0.924, p = 0.016) was significantly associated with the time to the first new-onset infection event on PD.</p><p><strong>Conclusions: </strong>Platelet indices were associated with the new-onset CVD, infectious comorbidities and all-cause mortality on PD. Low MPV/PC was associated with time to the first new-onset infection event in PD patients. Moreover, high MPV was associated with new-onset CVD and all-cause mortality in the incident PD patients.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11360337/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142092182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Intermediate cystinosis: a case report of 10-year treatment with cysteamine. 中间型胱氨酸病:使用半胱胺治疗 10 年的病例报告。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-27 DOI: 10.1186/s12882-024-03722-8
Mariko Kawamura, Daisuke Katagiri, Yuuka Yamamoto, Keiki Shimada, Satomi Higashi, Masako Otani, Noriko Uesugi, Hideki Takano, Yukiko Shimizu, Tadashi Okamura
{"title":"Intermediate cystinosis: a case report of 10-year treatment with cysteamine.","authors":"Mariko Kawamura, Daisuke Katagiri, Yuuka Yamamoto, Keiki Shimada, Satomi Higashi, Masako Otani, Noriko Uesugi, Hideki Takano, Yukiko Shimizu, Tadashi Okamura","doi":"10.1186/s12882-024-03722-8","DOIUrl":"10.1186/s12882-024-03722-8","url":null,"abstract":"<p><strong>Background: </strong>Cystinosis is a lysosomal storage disorder characterized by an autosomal recessive phenotype. Intermediate cystinosis, which progresses slowly and causes renal failure, accounts for approximately 5% of all cystinosis cases. Patients with intermediate cystinosis may not exhibit the typical symptoms of cystinosis, such as Fanconi syndrome and ocular symptoms. Because of its diverse clinical presentation and rarity, intermediate cystinosis can be difficult to diagnose. Additionally, few patients can tolerate cystine-depleting drugs, such as cysteamine, because of their complicated administration schedules and side effects. We report a case of intermediate cystinosis that was treated with cysteamine for 10 years.</p><p><strong>Case presentation: </strong>Urinary abnormalities were first diagnosed when the patient was 3 years of age during a health examination specifically for 3-year-old children, which is unique to Japan. Cystinosis was diagnosed when the patient was 12 years of age. Cysteamine therapy was initiated and regular cystine concentration measurements were performed. Although proteinuria persisted, the patient's renal function progressed slowly. Two renal biopsies were performed, and multinucleated podocytes and cystine crystals without focal segmental glomerulosclerosis lesions were observed in the biopsy specimens. The patient's renal function remained stable.</p><p><strong>Conclusions: </strong>This case of intermediate cystinosis was treated with cysteamine over the course of 10 years. Intermediate cystinosis requires an appropriate diagnosis and long-term treatment.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
How artificial intelligence is transforming nephrology. 人工智能如何改变肾脏病学。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-27 DOI: 10.1186/s12882-024-03724-6
Miguel Hueso, Alfredo Vellido
{"title":"How artificial intelligence is transforming nephrology.","authors":"Miguel Hueso, Alfredo Vellido","doi":"10.1186/s12882-024-03724-6","DOIUrl":"10.1186/s12882-024-03724-6","url":null,"abstract":"<p><p>Current research in nephrology is increasingly focused on elucidating the complexity inherent in tightly interwoven molecular systems and their correlation with pathology and related therapeutics, including dialysis and renal transplantation. Rapid advances in the omics sciences, medical device sensorization, and networked digital medical devices have made such research increasingly data centered. Data-centric science requires the support of computationally powerful and sophisticated tools able to handle the overflow of novel biomarkers and therapeutic targets. This is a context in which artificial intelligence (AI) and, more specifically, machine learning (ML) can provide a clear analytical advantage, given the rapid advances in their ability to harness multimodal data, from genomic information to signal, image and even heterogeneous electronic health records (EHR). However, paradoxically, only a small fraction of ML-based medical decision support systems undergo validation and demonstrate clinical usefulness. To effectively translate all this new knowledge into clinical practice, the development of clinically compliant support systems based on interpretable and explainable ML-based methods and clear analytical strategies for personalized medicine are imperative. Intelligent nephrology, that is, the design and development of AI-based strategies for a data-centric approach to nephrology, is just taking its first steps and is by no means yet close to its coming of age. These first steps are not even homogeneously taken, as a digital divide in access to technology has become evident between developed and developing countries, also affecting underrepresented minorities. With all this in mind, this editorial aim to provide a selective overview of the current use of AI technologies in nephrology and heralds the \"Artificial Intelligence in Nephrology\" special issue launched by BMC Nephrology.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348672/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The efficacy of cuff-shaving combined with negative pressure wound therapy in refractory exit-site and tunnel infections: a single center experience. 袖带剃除联合负压伤口疗法对难治性出口处和隧道感染的疗效:单中心经验。
IF 2.2 4区 医学
BMC Nephrology Pub Date : 2024-08-26 DOI: 10.1186/s12882-024-03714-8
Qinghua Yang, Xiaoying Ren, Xiaowan Fang, Jiaxiang Ding
{"title":"The efficacy of cuff-shaving combined with negative pressure wound therapy in refractory exit-site and tunnel infections: a single center experience.","authors":"Qinghua Yang, Xiaoying Ren, Xiaowan Fang, Jiaxiang Ding","doi":"10.1186/s12882-024-03714-8","DOIUrl":"10.1186/s12882-024-03714-8","url":null,"abstract":"<p><strong>Background: </strong>Refractory exit-site infections (ESIs) and tunnel infections (TIs) are challenging complications for patients undergoing peritoneal dialysis (PD). This study compared the outcomes of surgical intervention, notably the cuff-shaving (CS) procedure coupled with negative-pressure wound therapy (NPWT), and conservative management strategies for patients with refractory ESI and TI.</p><p><strong>Methods: </strong>We retrospectively reviewed patients who underwent PD at our center, focusing on the incidence and management of ESI and TI. We evaluated and compared treatment outcomes, including ESI scores, frequency of ESI and/or TI, identification of causative microorganisms, and duration of catheter survival or time until removal.</p><p><strong>Results: </strong>We identified 97 episodes of catheter-related ESI and/or TI across 71 patients with an incidence rate of 0.15 episodes per patient-year. Of the 23 patients with refractory ESI and/or TI, surgical intervention was performed in 8, while 15 chose conservative management. In the one-month follow-up, patients who underwent CS combined with NPWT showed no complications such as leakage, and their local symptoms resolved completely. The mean PD catheter survival time was significantly longer in the surgical group (29.38 ± 7.25 months) than in the conservative group (7.86 ± 2.13 months). Surgical intervention demonstrated a significantly higher therapeutic efficacy and extended catheter survival.</p><p><strong>Conclusions: </strong>The combination of CS and NPWT as a surgical approach is crucial for eradicating infectious foci and significantly improving the longevity of PD catheter function. This integrated surgical strategy offers a promising solution for the management of refractory ESI and TI in patients undergoing PD.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11346171/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142071991","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信