Efficacy and safety of Nafamostat mesylate in patients with endstage renal failure

BACKGROUND Recent studies on dialysis anticoagulation therapy in patients with renal failure have shown that Nafamostat mesylate,a broad-spectrum potent serine protease inhibitor,has strong anticoagulation and anti-fiber activity.AIM To evaluate the efficacy and safety of Nafamostat mesylate in patients with end-stage renal failure.METHODS Seventy-five patients with end-stage renal failure who received hemodialysis at our hospital between January 2020 and August 2021 were selected and divided into the observation group(Nafamostat mesylate for injection,n=33)and control group(heparin sodium injection,n=32).General patient data,indicators of clinical efficacy,dialyzer hemocoagulation parameters,coagulation function indices,and hemoglobin concentration and platelet count before and after treatment,and the occurrence of adverse reactions after treatment were compared between the two groups.RESULTS The two groups showed no significant differences in general patient data(P>0.05).The post-treatment effectiveness rate in the control group was lower than that in the observation group(P<0.05).The two groups showed no significant difference in the number of patients in grade I(P>0.05),while the number of patients in grade 0 was lower in the control group,and the number of patients in grades II and III was higher in the control group(P<0.05).The post-treatment prothrombin time,activated partial thromboplastin time,thrombin time,and international normalized ratio values in the control group were higher than those in the observation group,while the fibrinogen level in the control group was lower than that in the observation group(P<0.05).The two groups showed no significant difference in the platelet count and hemoglobin level before and after treatment(P>0.05).The total number of post-treatment adverse reactions in the observation group was lower than that in the control group(P<0.05).CONCLUSION Treatment of patients showing end-stage renal failure with Nafamostat mesylate can significantly improve therapeutic efficacy and has high safety and clinical value.

Assessment of the Level of Knowledge about Chronic Renal Failure in 271 Hypertensive Patients in Brazzaville

Background and Objectives: Chronic kidney disease (CKD) is now a global public health problem. In low- and middle-income countries such as the Congo, access to dialysis is low and inequitable. The prevention of CKD involves raising awareness among patients at risk, such as those suffering from arterial hypertension (AH), by improving their knowledge of CKD. The objectives of our work were to determine the level of knowledge about CKD among hypertensive patients and to identify the factors associated with a low level of knowledge. Methodology: We conducted a 3-month descriptive and analytical cross-sectional study from 1 August to 30 October 2023 in 3 large public hospitals in Brazzaville (capital of the Republic of Congo). We included: hypertensive patients aged 18 and over who had freely consented to participate in our study and were able to answer the questions on the survey form. Patients with known hypertension who had been followed for less than 3 years and those with known chronic renal failure were not included. Results: The mean age was 58.4 ± 14.4 years (29 - 88 years). There were 121 men and 150 women (sex ratio = 0.8). All the patients were educated;37.2% with a higher level of education and 13.6% with primary education. 24 patients (9%) had a good level of knowledge about CKD and 153 (56%) had poor knowledge. A good level of knowledge was associated with the duration of hypertension, intellectual level and the existence of associated heart disease. Conclusion: Our study reveals a significant lack of knowledge about chronic kidney disease among hypertensive patients in Brazzaville.

High Urine Retention: Experience in a Series of Patients with Renal Failure Patients

Purpose: High urinary retention (HUR) can negatively impact renal function. Our study aimed to present the epidemiological, diagnostic, and therapeutic aspects of HUR in a Senegalese academic hospital. Patients and Methods: We conducted a retrospective study of 70 patients with HUR associated with renal failure from January 2017 to December 2020. Parameters examined included: age, sex, coexisting conditions affecting renal function, clinical symptoms, diagnostic tests, causes of HUR, urinary diversion, and patient outcomes. Results: The average age was 66, with a majority of male patients (87%). Twenty-three patients had pre-existing medical conditions. Oligo-anuria was the most common reason for detecting HUR (70%). Half of the patients had an ECOG score ≥ 2. The mean creatinine level was 50.7 mg/l. Nineteen patients exhibited hydroelectrolytic disorders. Bacterial colonization was observed in 25 patients. Ultrasound and computed tomography were the most frequently performed imaging tests (100% and 62.8%, respectively). Sixty-seven patients had ureterohydronephrosis (UHN), with bilateral UHN in 88.6% of cases. Pelvic cancers (47.1%) were the primary cause of HUR, primarily bladder cancers (27.1%). Nephrostomy was the most common urinary drainage method (50%), particularly for obstructions due to pelvic cancer (88.6%). The majority of patients (52.8%) regained normal renal function after drainage. Nineteen deaths occurred among elderly patients with compromised general health. Conclusion: Urinary drainage significantly improved renal function for most patients. Pelvic cancer emerged as the leading cause of HUR. Nephrostomy was the predominant drainage method.

Morbidity and Mortality of Acute Renal Failure in COVID-19 Patients in Intensive Care According to Waves/Variant: Case of the Grand Hôpital de l’Est Francilien Site de Meaux

Introduction: The incidence of acute renal failure (ARF) varies between 20% and 40% of cases for COVID-19 patients admitted to the intensive care unit, with very high mortality, but heterogeneous according to the different epidemic waves, probably due to the genetic variant phenomenon of the virus. The aim of this study is to determine the morbidity and mortality of COVID-19 patients admitted with ARF to the intensive care unit of the Grand H?pital Est Francilien (GHEF) according to the waves and variants. Methods: Cross-sectional observational study of COVID-19 patients with ARF admitted to the intensive care unit of the GHEF site in Meaux covering the period from March 1st 2020 to December, 31st 2021. Per-hospitalisation and outcome data were collected and analysed with SPSS version 25.0 software using the Chi-square or Fischer’s exact test or Student’s t-test and logistic regression for p Results: A total of 86 patients were included. The mean age was higher (70 ± 8.5) in patients in the fourth wave than in the other waves (p = 0.015), with male predominance in all waves without significant difference. Co-morbidities: hypertension, diabetes, heart disease, dyslipidaemia and arrhythmia complete with fibrillation were present in all waves. The majority of patients were classified as KDIGO 1 for the different waves (1st: 61.9%, 2nd: 86.5%, 3rd: 80%, and 4th: 75%), with the same trend according to variant (alpha: 80%, beta: 75%, delta: 81.3%, omicron: 75%). Mortality by the wave was: 1st: 28.5%, 2nd: 37.5%, 3rd: 23% and 4th: 11%) and by variant: alpha: 24.2%, beta: 44.8%, delta: 20.7%, omicron: 10.3%). Overall mortality was 33.7%. Case fatality was higher in the fourth wave. Hypertension, shock, failure to recover renal function, acute lung oedema, ventilator-associated lung disease and hyperkalaemia were factors associated with mortality (p Conclusion: Acute renal failure is common in COVID-19 patients admitted to the intensive care unit, and mortality is not negligible. The beta variants and the second wave presented more cases of renal impairment, although the mechanism is still unknown. Further studies are needed to understand this mechanism and perhaps to be able to identify the cause.

Comparative efficacy of traditional Chinese herbal injection for chronic renal failure:A systematic review and network meta-analysis of randomized controlled trials

Objective: To conduct a systematic review and network meta-analysis(NMA) for the comparison of the efficacy and safety of Chinese herbal injection(CHI) combined with Western medicine(WM) and WM monotherapy for chronic renal failure(CRF).Methods: Eight databases were searched from inception to August 30, 2022. Randomized controlled trials(RCTs) regarding the comparison of CHI-WM combination therapy and WM monotherapy were included. Literature search, risk-of-bias assessment, and data extraction were conducted by 2 reviewers independently. NMA was performed by Stata 14.0, R 4.0.4 software, and the latest risk of bias assessment tool 2(RoB 2).Results: A total of 53 RCTs were finally included, involving 4445 participants and 16 CHIs. RoB 2 showed that 2 of these studies had a high risk of bias. Tianqi injection(TQ) + WM was the most effective in reducing serum creatinine(Scr) level. Xingding injection(XD) + WM was the most effective in reducing blood urea nitrogen(BUN) and cystatin C(Cys C) levels. Guhong injection(GH) + WM had the highest endogenous creatinine clearance rate(Ccr). Shuxuetong injection(SXT) + WM was the most effective in improving the clinical effective rate. Danhong injection(DH) + WM resulted in the lowest 24-h urinary protein quantity(24 h-UPQ), while Danshen injection(DS) + WM led to the lowest blood uric acid(UA)level. Shenfu injection(SF) + WM was the most effective in increasing hemoglobin(Hb) level.Conclusion: CHIs-WM combination therapy is more effective than WM monotherapy in treating CRF.Considering all of the indicators, SK + WM may be the optimal treatment option for improving renal function in patients with CRF.

Effects of paricalcitol combined with hemodiafiltration on bonemetabolism-related indexes in patients with diabetic nephropathy and chronic renal failure

BACKGROUND Diabetic nephropathy(DN)is frequently seen in the development of diabetes mellitus,and its pathogenic factors are complicated.Its current treatment is controversial,and there is a lack of a relevant efficacy prediction model.AIM To determine the effects of paricalcitol combined with hemodiafiltration on bonemetabolism-related indexes in patients with DN and chronic renal failure(CRF),and to construct an efficacy prediction model.METHODS We retrospectively analyzed 422 patients with DN and CRF treated in Cangzhou Central Hospital between May 2020 and May 2022.We selected 94 patients who met the inclusion and exclusion criteria.Patients were assigned to a dialysis group(n=45)and a joint group(n=49)in relation to therapeutic regimen.The clinical efficacy of the two groups was compared after treatment.The changes in laboratory indexes after treatment were evaluated,and the two groups were compared for the incidence of adverse reactions.The predictive value of laboratory indexes on the clinical efficacy on patients was analyzed.RESULTS The dialysis group showed a notably worse improvement in clinical efficacy than the joint group(P=0.017).After treatment,the joint group showed notably lower serum levels of serum creatinine,uric acid(UA)and blood urea nitrogen(BUN)than the dialysis group(P<0.05).After treatment,the joint group had lower serum levels of phosphorus,procollagen type I amino-terminal propeptide(PINP)and intact parathyroid hormone than the dialysis group,but a higher calcium level(P<0.001).Both groups had a similar incidence of adverse reactions(P>0.05).According to least absolute shrinkage and selection operator regression analysis,UA,BUN,phosphorus and PINP were related to treatment efficacy.According to further comparison,the non-improvement group had higher risk scores than the improvement group(P<0.0001),and the area under the curve of the risk score in efficacy prediction was 0.945.CONCLUSION For treatment of CRF and DN,combined paricalcitol and hemodiafiltration can deliver higher clinical efficacy and improve the bone metabolism of patients,with good safety.

Epidemiological and Clinical Aspects of Chronic Renal Failure in the Medical Department at the Hospital of Sikasso

Introduction: Chronic renal failure is a real public health problem because of its prevalence, the cost of its management and the high morbidity and mortality rate associated with it. The objective of this study was to determine the frequency, causes and main aggravating factors of chronic renal failure in the medical department of the hospital of Sikasso. Materials and Methods: This was a cross-sectional, retrospective, descriptive study conducted in the medical department of the Sikasso hospital from January 1, 2021 to November 30, 2022. It covered all the records of patients with chronic renal failure hospitalized in the department during this period. The MDRD formula was used to estimate the glomerular filtration rate. The KDIGO 2012 classification was used to stage CKD. Results: Of 820 patient records reviewed, we retained 197 records. The mean age of our patients was 42.25 ± 10 years. The male sex represented 114 (57.9%). The socio-economic level was considered low and precarious in 79.8% of cases. The frequency of chronic renal failure was 24%. The reason for hospitalization was mostly hyper creatinine > 185 μmol/l (92.4%). Herbal medicine was reported in 103 cases (52.3%). Medical history was hypertension 101 cases (51.3%), undocumented gastro duodenal ulcer (UGD) 14 cases (7.6%), hypertension and diabetes 11 cases (5.1%), diabetes 8 cases (4%), lower limb edema 9 cases (4.6%). Hypocalcemia was 147 cases (75.6%) with hyper phosphoremia was 153 cases (77.7%). Hemoglobin level was: <6 g/dl, 44 cases (22.3%);between 6 - 8 g/dl, 77 cases (39.1%), from 8 - 10 g/dl, 54 cases (27.4%). The etiologies of CKD were vascular nephropathy 106 cases (53.8%), interstitial nephropathy, 44 cases (22.3%), glomerular nephropathy, 33 cases (16.8%), diabetic nephropathy, 12 cases (6.1%) and polycystic kidney disease 2 (1%). CKD was classified as stage 5, 171 cases (86.8%), stage 4, 11 cases (5.6%), stage 3, 13 cases (6.6%) and stage 2, 2 cases (1%.) Dialysis was performed in 1095 (5.3%) of our patients. All these patients started dialysis with a central line. Conclusion: This study reveals the high prevalence of chronic renal failure in the department and above all the late diagnosis at very advanced stages. This imposes policies of prevention and effective management of the responsible diseases.

Simultaneous pancreas-kidney transplantation for end-stage renal failure in type 1 diabetes mellitus: Current perspectives

Type 1 diabetes mellitus(T1DM)is one of the important causes of chronic kidney disease(CKD)and end-stage renal failure(ESRF).Even with the best available treatment options,management of T1DM poses significant challenges for clinicians across the world,especially when associated with CKD and ESRF.Substantial increases in morbidity and mortality along with marked rise in treatment costs and marked reduction of quality of life are the usual consequences of onset of CKD and progression to ESRF in patients with T1DM.Simultaneous pancreas-kidney transplant(SPK)is an attractive and promising treatment option for patients with advanced CKD/ESRF and T1DM for potential cure of these diseases and possibly several complications.However,limited availability of the organs for transplantation,the need for long-term immunosuppression to prevent rejection,peri-and post-operative complications of SPK,lack of resources and the expertise for the procedure in many centers,and the cost implications related to the surgery and postoperative care of these patients are major issues faced by clinicians across the globe.This clinical update review compiles the latest evidence and current recommendations of SPK for patients with T1DM and advanced CKD/ESRF to enable clinicians to care for these diseases.

Acute Renal Failure in the Elderly in the Nephrology Department of Aristide Le Dantec Hospital in Senegal: About 45 Cases

Introduction: The incidence of acute renal failure (ARF) increases with age. In Senegal, few studies have described the epidemiology of ARF in the elderly. The aim of our study is to establish the epidemiological profile of elderly patients with ARF, identify the causes of ARF in the elderly and assess treatment and prognosis. Methods: We conducted a descriptive and retrospective study over a five-year period from 2011 to 2015 involving patients aged 60 and over, treated for ARI during the study period. Data entry and analysis were done on Epi info 7.3. Results: We included a total of 45 patients. The prevalence of ARF was 3.34%. The mean age was 70.31 years (60 - 83) and the sex ratio was 3.5. Phytotherapy was found in 68.9%, hypertension was found in 68.9%, and diabetes was found in 31.1%. Prostate hypertrophy was found in 53.4% of patients. Pre-renal ARF was the most common (46.6%). Most of the cases, 66.67%, were at Stage 3 of KDIGO. The most common etiologies were respectively tumor (35.5%) and infection (20%). The most common complications were respectively hyperkalemia (33.3%) and hyponatremia (33.3%). Recovery was complete in 62.6% of cases, partial in 37.8% of patients and 13.3% of patients ended up on chronic hemodialysis. Mortality was 4.4%. Conclusion: Herbal medicine, hypertension and diabetes are frequently associated with ARF in the elderly. This justifies increased monitoring of the elderly subject with these conditions in order to subject him to early and appropriate care.

Acute Renal Failure in COVID-19 Patients in Intensive Care at the CHU du Point G in Mali

Introduction: SARS-CoV-2 (Severe Acute Respiratory Syndrome Corona-virus 2) causes an acute respiratory disease with interstitial and alveolar pneumonia, which can affect several organs including the kidneys [1] [2] [3]. As Mali is no stranger to this pandemic, we report our experience of the management of cases of kidney failure observed in the COVID-19 intensive care unit at the Point G University Hospital Centre (CHU). The aim of this work was to characterise acute renal failure in COVID-19 patients in intensive care, describing the management methods used and determining the vital prognosis. Materials and Methods: This was a retrospective descriptive study, covering an 18-month period from April 2020 to September 2021. We included all patients admitted to the COVID-19 intensive care unit on the basis of a positive RT-PCR and/or the presence of ground-glass images on thoracic computed tomography. Results: We selected 232 patients admitted for COVID-19. Acute Renal Failure (ARF) developed in 71 patients (30.6%). The stages of AKI according to KDIGO were Stage 1 in 28.2%, Stage 2 in 18.3% and Stage 3 in 53.5%. The mean age was 63.96 years, with a standard deviation of 16.6, and males accounted for the majority (71.8%). Organic ARF was found in 80.3% of cases. Risk factors and comorbidities for ARF included advanced age (60.6%), male sex (71.8%), hypertension (52.1%), diabetes (21.1%), invasive mechanical ventilation (71.8%) and septic shock (56.3%). Extra renal purification (haemodialysis) was used in 29.6% of patients. Admission to intensive care ranged from 7 days to 14 days in 43.7% of cases. More than half the patients (52.1%) were in critical condition on admission. Death occurred in 76.1% of patients. Conclusion: ARF appears to occur more frequently in patients with severe COVID-19. It is associated with a poor prognosis.

Real-time Shear Wave Elastography Assessment of Muscle Elasticity in Patients with Renal Failure

Objective:To explore the value of real-time elastic shear wave in evaluating muscle elasticity in patients with renal failure.Methods:50 patients with chronic renal failure from January 2019 to December 2022 were randomly selected as the experimental group,and 50 healthy patients aged 21-61 during the same period were selected as the control group,and the basic information of the patients,including age,gender,body mass index,etc.,were collected.Besides,the Young's modulus of the two groups of patients were observed and analyzed.Results:The Young's modulus values of left and right gastrocnemius muscles in the experimental group were significantly lower than those in the control group(P<0.05),and there was no statistical difference between the left and right sides of the experimental group and the control group(P>0.05).Conclusion:Real-time shear wave elastography provides a non-invasive,real-time and effective tool for the assessment of muscle elasticity in patients with renal failure.Through further research and optimization,real-time shear wave elastography will play a greater role in the prevention and treatment of patients with renal failure,improving the quality of life and prognosis of patients.

Dental implant treatment for renal failure patients on dialysis:a clinical guideline

Chronic kidney disease （CKD） is a worldwide public health problem that is growing in prevalence and is associated with severe complications. During the progression of the disease, a majority of CKD patients suffer oral complications. Dental implants are currently the most reliable and successful treatment for missing teeth. However, due to complications of CKD such as infections, bone lesions, bleeding risks, and altered drug metabolism, dental implant treatment for renal failure patients on dialysis is more challenging. In this review, we have summarized the characteristics of CKD and previous publications regarding dental treatments for renal failure patients. In addition, we discuss our recent research results and clinical experience in order to provide dental implant practitioners with a clinical guideline for dental implant treatment for renal failure patients undergoing hemodialysis.

Doctor-led intensive diet education on health-related quality of life in patients with chronic renal failure and hyperphosphatemia

BACKGROUND Secondary hyperparathyroidism,renal osteodystrophy,and cardiovascular adverse events can occur if long-term hyperphosphatemia is not corrected,leading to the adverse prognosis of patients with chronic renal failure.Besides the use of phosphorus binders,clinical control measures for hyperphosphatemia in these patients should also incorporate diet control.AIM To observe doctor-led intensive diet education effects on health-related quality of life in patients with chronic renal failure and hyperphosphatemia.METHODS We assessed 120 patients with hyperphosphatemia and chronic renal failure on hemodialysis admitted to our hospital(July 2018 to March 2020).The control group(n=60)was given routine nursing guidance,and the observation group(n=60)was given doctor-led intensive diet education.The changes in EQ-5D-3L scores,disease-related knowledge,and compliance scores before intervention and 3 and 6 mo after intervention in the two groups were recorded.The levels of serum parathyroid hormone(iPTH),calcium(Ca),phosphorus(P),calciumphosphorus product(Ca×P),serum creatinine(Scr),and blood urea nitrogen(BUN)before intervention and 3 and 6 mo after intervention in the two groups were assessed along with patient satisfaction.RESULTS There was no significant difference in blood iPTH,Ca,P,Ca×P,Scr,or BUN levels between the groups before intervention.After 3 and 6 mo of intervention,the blood iPTH,Ca,P,and Ca×P levels in the two groups decreased gradually(P<0.05),but there were no significant differences in Scr or BUN.The blood iPTH,Ca,P,and Ca×P levels in the observation group were lower than those in the control group(P<0.05).The satisfaction rate in the observation group after 3 mo was 93.33%and after 6,90.00%,which was high compared with the 80.00%and 71.67%,respectively,in the control group(P<0.05).There was no significant difference in EQ-5D-3L score between the two groups before intervention.After 3 and 6 mo of intervention,the visual analogue scale score of the two groups increased gradually(P<0.05);and the scores of action ability,self-care,daily activities,pain and discomfort,and anxiety and depression decreased gradually(P<0.05).The overall EQ-5D-3L score in the observation group was better than that in the control group(P<0.05).There was no significant difference in diseaserelated knowledge or compliance scores between the groups before intervention.After 3 and 6 mo of intervention,the scores of disease,diet,and medication knowledge and compliance in the two groups increased gradually(P<0.05).The scores of disease-related knowledge and compliance were higher in the observation group than in the control group(P<0.05).CONCLUSION Doctor-led intensive diet education can improve patient satisfaction and the quality of life in patients with chronic renal failure and hyperphosphatemia and promote low-phosphorus diet behavior.

An Experimental Study on the Mechanism of Anisodamini Hydrobromidum in Treating Acute Ischemic Renal Failure

In order to explore the mechanism of anisodamini hydrobromidum (654-2) in treating acute ischemic renal failure, the model of acute ischemic renal failure in white New Zealand rabbits was established to dynamically observe and statistically analyze the intracellular concentration changes of free calcium([Ca(2+)]i) and inositol triphosphate (IP3). The results showed that the levels of [Ca(2+)], and IP3 in acute renal failure group were higher than those in control group (P<0. 01). However, the levels of [Ca(2+)]i and IP3 in 654-2 treated group were significantly lower than those in acute renal failure group (P<0. 001). It was concluded that 654-2 could alleviate Ca(2+)-overload in renal histocytes in acute ischemic renal failure. The protective mechanism is associated with intracellular reduction of IP3.

Detection of the Level of Urinary FPA in Chronic Nephritis with Renal Failure and Its Clinical Implication

The level of urinary FPA was assayed by high per formance liquid chromatography (HPLC ) in 42 normal controls, 57 cases of chronic glomeru-lonephritis, including 24 with normal renal function, 12 with renal insufficiency and 21 with uremia. Their levels were 24. 40± 10. 30 μg/L, 26. 99±5.77 μg/L,38. 81±6. 28 μg/L, 79. 74± 18. 76 μg/L, respectively. The level of urinary FPA in renal insufficiency function group was significantly higher than those of the con-trol group and normal renal function group (P<0.01). The patients with uremia presented dramatically higher level of urinary FPA than those in the renal insufficiency group (P<0.01). A positive correlation was found between the level of urinary FPA and the blood creatine (r= 0. 9120, P<0. 01 ). It was suggested that a hypercoagulable state existed in the patients with chronic nephritis with renal failure, in which the severity was closely related with the occurrence and development of the disease. The urinary FPA could serve as a good indicator for renal function.

Effects of chronic renal failure on the expression of connexin 43 in the rat＇s corpus cavernosum

Aim： To explore the mechanism of chronic renal failure （CRF）-related erectile dysfunction （ED）. Methods： CRF experimental models were established by 5/6 nephrectomy from male Sprague-Dawley rats. Both the rats from the control group （NCRF group, n = 6） and the experimental group （CRF group, n = 30） were injected with a low dose （80 μg/g） of apomorphine in the 12th week after resection surgery to measure corresponding penile erections. Western blot method was thereafter conducted to measure the expression of connexin 43 （CX43） in the rat corpus cavernosum in the 12th week after the resection surgery. Results： There was one death in the NCRF group and five in the CRF group. The penile erection ratio of the CRF group was 28% （7/25）, whereas that of the NCRF group was 100% （5/5）, which presents a significant difference between the two groups （P 〈 0.05）. In terms of penile erection frequency, the average of the CRF group was 1.0 ± 0.0, which was significantly different from that of the NCRF group （2.2 ± 0.8） （P 〈 0.05）. As for the expression of CX43 in the rat corpus cavernosum, a notable difference existed between the CRF group （0.21 ± 0.07） and the NCRF group （0.53 ± 0.27） （P 〈 0.01）. Conclusion： CRF significantly reduces the erectile function of rats. A close correlation exists between the expression of CX43 in rats＇ corpus cavernosum and CRF-related ED. （Asian J Andro12008 Mar; 10： 286-289）

OFD1 mutation induced renal failure and polycystic kidney disease in a pair of childhood male twins in China

BACKGROUND Oral-facial-digital syndrome type 1(OFD1) is a rare ciliopathy mainly with an Xlinked dominant pattern of inheritance, which is caused by mutations in the OFD1 gene. The OFD1 protein is located within the centrosomes and basal bodies of the primary cilia. It is reported that approximately 15%–50% cases of OFD1 progress to end-stage renal disease(ESRD) following development of polycystic kidney diseases(PKD). Here we report a pair of childhood male twins who presented only renal failure and PKD caused by an OFD1 mutation in China.CASE SUMMARY A pair of 14-year male twins were hospitalized with a complaint of abnormal renal function for nine days. They both complained of ankle pain for 3 mo vs 2 wk, respectively. They denied fever, abdominal pain, daytime or nighttime enuresis, urgency, dysuria, or gross hematuria. Laboratory tests at a local hospital showed renal failure(serum creatinine 485 μmol/L vs 442 μmol/L, blood urea nitrogen 14.7 mol/L vs 14.5 mol/L) and anemia(hemoglobin 88 g/L vs 98 g/L).The twins are monozygotic. There was no abnormal birth, past medical, or family history. Clinical data were analyzed and genetic analysis on PKD was carried out in the twins by next-generation sequencing. The results showed that the twins presented low-molecular-weight proteinuria, hyposthenuria, anemia, renal failure, and renal polycystic changes. Genetic tests showed that the twins both carried a hemizygous mutation in exon 19 c.2524 G>A(p. G842 R) of the OFD1 gene. Their mother heterozygously carried the same mutation as the twins but was without any phenotypes while their father was normal.CONCLUSION We have reported a pair of childhood male twins with an OFD1 mutation who presented ESRD and PKD but without any other phenotypes of OFD1 in China.

Effect of Heme Oxygenase-1 Inducer Hemin on Chronic Renal Failure Rats

Summary: The role of HO-1 inducer, hemin, in chronic renal failure (CRF) rats and its possible mechanism of action was studied. 5/6 subtotal nephrectomy was performed to establish chronic renal failure model. Rats were randomly assigned to 4 groups: sham-operated group, CRF group, ferrous gluconate group and hemin group. At the 10th week after operation, serum creatinine, BUN, RBC, HGB and HCT were measured. Renal pathologic changes were observed. RT-PCR and immunohistochemistry were used to detect the expression and distribution of HO-1. RT-PCR and radioimmunoassay was used to determine the expression of ET-1 in the kidney and plasma. The results showed that as compared with CRF group, serum creatinine and BUN in hemin group were reduced significantly and nephrogenic anemia was improved markedly. Glomerular mesangial proliferation and interstitial lesion were also ameliorated significantly. Hemin not only increased the expression of HO-1 but also reduced the expression of ET-1 in the kidney. The level of ET-1 protein in the plasma was also reduced after hemin treatment. Most of these indexes were not obviously changed in ferrous gluconate group. It was suggested that through inducing the expression of HO-1 and reducing the level of ET-1 in the kidney and plasma, hemin plays an important protective role in 5/6 subtotal nephrectomized rats.

Renal failure in cirrhosis: Emerging concepts

Acute renal failure, now termed acute kidney injury(AKI), is frequently found in patients with cirrhosis. The occurrence of AKI, irrespective of the underlying cause, is associated with reduced in-hospital, 3-mo and 1-year survival. Hepatorenal syndrome is associated with the worst outcome among AKI patients with cirrhosis. Several definitions for AKI that have been proposed are outlined and evaluated in this paper. Among these, the International Club for Ascites-AKI criteria substantially strengthen the quality of early diagnosis and intervention according to underlying cause of AKI.

Late Nephrology Case Management and Morbidity Due to Chronic Renal Failure, Case of Guinea

Context and Object: Late nephrology case management for patients with chronic renal failure is consistently associated with high morbidity rate. The objectives of this study were to determine the factors associated with late nephrology case management and to describe the evolution of patients depending on the time of care nephrology. Material and Methods: It was a 5-year prospective study, observational type, going from January 1st, 2010 to December 31th, 2014. We studied all the patients hospitalized with chronic renal failure with dialysis or not and we excluded the patients hospitalized for acute renal failure. Results: The study population includes 307 men (53.4%) and 268 women (46.6%) with a sex ratio of 1.14. Age population ranged between 15 and 85 years old with a mean of 44.4 ± 16.20 years old. Their profession was distributed as follows: 220 housewives (38.3%), 181 workers (31.5%), 104 civil servants (18.1%), 56 students (9.7%) and 14 unemployed (2.4%). Patients came from all regions of the country: Conakry: 389 patients (67.6%);Lower Guinea: 82 (14.3%);Middle-Guinea: 57 (9.9%);Upper Guinea: 31 (5.4%);Guinea Forest Region: 16 (2.8%). In this study population, 48 patients were admitted in emergency (8.3%);105 were scheduled (18.3%) and 422 were referred to the nephrology department (73.4%). Reasons for the emergency admission were acute pulmonary edema in 21 patients (43.7%), decompensated anemia in 13 patients (27.1%), loss of consciousness in 9 patients (18.7%) and malignant hypertension in 5 patients (10.4%). The reference patterns was uremic syndrome in 312 patients (54.3%), followed by high blood pressure in 247 patients (42.9%), an edematous syndrome in 234 patients (40.7%), oligoanuria in 222 patients (38.6%), morphological abnormalities on the renal ultrasound in 4 patients (0.7%). The functional symptoms were vomiting in 379 patients (65.9%), followed by dyspnea in 290 patients (50.4%), headaches in 287 patients (49.9%). Among them, 121 patients could handle alone, 431 were borne by their families and 23 were indigent;181 patients had consulted before 1 month of the beginning of the signs, 238 between 1 and 2 months and 156 after 2 months;48 were admitted in emergency, 105 were programmed by the Nephrology Department and 422 were referred to the other health structures of the country. Conclusion: Several factors contributed to the delay in treatment, among which: the low socioeconomic level, the delay of the reference of the patients, the long distance of the regions, the lack of Nephrology units in the country.