Primary hyperoxaluria type II and organ transplantation / 器官移植

Primary hyperoxaluria type Ⅱ (PH2) is an inherited disorder of the glyoxylate metabolism caused by the gene mutation of glyoxylate reductase/hydroxypyruvate reductase (GRHPR). PH2 is characterized by recurrent nephrolithiasis and nephrocalcinosis, which may even progress into end-stage renal disease. Currently, organ transplantation is the only treatment option for PH2, which mainly includes two strategies: kidney transplantation and combined liver and kidney transplantation. Kidney transplantation yields a high risk of recurrence of oxalate nephropathy, which may cause early graft dysfunction. Combined liver and kidney transplantation could mitigate the deficiency of oxalate metabolism, whereas it yields a high risk of graft complications. PH2 is an extremely rare disorder. No consensus has been reached on the indications, surgical selection and perioperative management of organ transplantation for PH2 patients. In this article, the pathogenesis, diagnosis, monitoring and organ transplantation experience of PH2 were reviewed, aiming to divert clinicians' attention to PH2 and provide reference for determining diagnosis and treatment regimens, especially transplantation strategy for PH2 patients.

Clinical technical operation specification of kidney transplantation for autosomal dominant polycystic kidney disease (2022 edition) / 器官移植

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease and one of the most common causes for end-stage renal disease (ESRD). Kidney transplantation is the optimal renal replacement therapy for ADPKD patients complicated with ESRD. Currently, scholars at home and abroad have a certain controversy about whether polycystic kidney resection is necessary in ADPKD patients before kidney transplantation, and the criteria and methods for polycystic nephrectomy also differ. To further standardize the clinical technical operation of kidney transplantation in ADPKD patients, experts in organ transplantation organized by Branch of Organ Transplantation of Chinese Medical Association formulated this specification from the aspects of diagnosis of ADPKD, indications and contraindications of kidney transplantation for ADPKD, preoperative evaluation and treatment, polycystic nephrectomy, and postoperative management, etc.

Alpha-Galactosidase A Levels in Colombian Males with End-Stage Renal Disease: Ten Years of Selective Screening in Dried Blood Spots

Abstract Fabry disease is a metabolic alteration linked to an enzymatic deficiency of Alpha-Galactosidase A, this disorder compromises the sphingolipid metabolism, leading to an accumulation of lysosomal globotriaosylceramide and is inherited in an X-linked recessive way. The diagnostic of this disease, in general, requires the confirmation of below-normal levels of Alpha-Galactosidase A obtained from dried blood spot (DBS) samples, followed by an assessment of the enzyme in leukocytes. We aimed to report the Alpha-Galactosidase A values obtained in Colombian males with end-stage renal disease (ESRD) screened using dried blood spot samples during ten years. This screening was performed with samples sent to the analysis center from 6156 patients between 2006- 2016. All patients with low levels in enzyme activity (compared to the control population) were sent to confirmation through enzyme analysis in isolated leukocytes. 26 males (0.42%) with low levels of Alpha-Galactosidase A were identified (Range 0.0 - 1.14 nmol/ml/hour, cut-off: 1.15), 22 patients were subsequently measured in isolated leukocytes having a confirmation of Fabry disease in 5 patients (0.08% of total male population) (Range: 0.3 -4.7 nmol/mg prot/h). These results are similar to those reported in studies with comparable characteristics being this the first reporting frequency of Fabry disease among Colombian males with end-stage renal disease.

Summary of clinical characteristics, diagnosis and treatment of primary hyperoxaluria type 1 in mainland China / 器官移植

Objective To investigate the clinical manifestations, treatment and prognosis of primary hyperoxaluria type 1 (PH1). Methods Relevant literature review was conducted from Chongqing VIP, CNKI, Wanfang Data, PubMed, Web of Science, Embase and Cochrane databases. Clinical data of 57 patients with PH1 were collected, and the clinical manifestations, diagnosis and treatment and prognosis were analyzed. Results A total of 35 eligible studies were searched, including 57 patients with PH1, 39 male and 18 female, aged 0.2-57.0 years old, and the age of onset was from date of birth to 42 years old. The specificity of clinical symptoms of 57 patients with PH1 was relatively low, including 41 cases of renal stones, 21 cases of renal calcification and/or calcium deposition, 12 cases of oxalic acid deposition outside the urinary system, 12 cases of lumbago, backache and abdominal pain, and 8 cases of ureteral stones. Besides, alternative symptoms, such as decreased urine output, metabolic acidosis, disorder of water and electrolyte, anemia and gross hematuria were also reported. Thirty-three patients were diagnosed with end-stage renal disease (ESRD) upon admission. Twenty-six patients received transplantation. Among them, 17 cases underwent kidney transplantation (2 cases repeatedly received combined liver-kidney transplantation due to recurrence of stones and resumption of dialysis, and 1 case repeatedly received liver transplantation due to resumption of dialysis), 7 cases received combined liver-kidney transplantation, 2 cases underwent liver transplantation, and 3 cases received sequential liver-kidney transplantation, respectively. Thirty-one patients did not undergo transplantation. Significant differences were observed in the survival rate between patients treated with and without transplantation (85% vs. 58%, P < 0.05). Conclusions Clinical manifestations of PH1 are diverse and lack of specificity. A majority of PH1 patients are diagnosed with ESRD upon admission. Clinical prognosis of patients undergoing transplantation is better than that of those counterparts without transplantation. Prior liver transplantation or combined liver-kidney transplantation is recommended.

Intracapsular infection of polycystic kidney disease and renal transplantation / 器官移植

Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic hereditary kidney disease, which can progress into end-stage renal disease (ESRD). Patients with ADPKD constantly suffer from recurrent intracapsular infection. The drug resistance caused by antibiotic treatment is becoming increasingly prominent. The pattern of renal transplantation should be selected according to the infection of polycystic kidney disease. In this article, the origin of renal cyst, classification and source of cystic fluid, type and drug resistance of bacteria in the cystic fluid, and intracapsular infection of patients with renal transplantation- associated ADPKD were reviewed, aiming to provide reference for the diagnosis and treatment of intracapsular infection of patients with ADPKD.

Cerebral structural abnormalities and cognitive function in end-stage renal disease patients undergoing maintenance hemodialysis / 上海交通大学学报（医学版）

Objective: To explore the patterns of cerebral structural abnormalities and cognitive function alterations in end-stage renal disease (ESRD) patients undergoing maintenance hemodialysis, and the underlying correlative factors. Methods: Thirty-seven ESRD patients undergoing maintenance hemodialysis without prior stroke in Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine were selected. All patients underwent brain magnetic resonance imaging (MRI) and assessment of cognitive function by using Mini-Mental State Examination (MMSE). Patients were divided into lacunar infarction group (n=33) and non-lacunar infarction group (n=4), or white matter hyperintensities (WMH) group (n=14) and non-WMH group (n=23). The difference of demographic characteristics, past history, blood parameter and dialysis adequacy between patients and their controls were analyzed by t test, Mann-Whitney U test, χ2 test and Fisher exact test. Spearman correlation analysis were performed to explore the relationship between clinical features, cerebral structural abnormalities and cognitive function. Results: Nineteen male and eighteen female patients participated in the study. The mean age was (59.4±12.3) years. The incidences of lacunar infarction and WMH were 89.2% and 37.8%, respectively. 24.3% of the participants were diagnosed as cognitive impairment. Patients with lacunar infarction were elder, who had lower level of hemoglobin, hematocrit, serum albumin and serum total protein, while parathyroid hormone (PTH), erythrocyte sedimentation rate, tumor necrosis factor-α and interleukin-6 were elevated. Patients with WMH were also significantly elder, lower in transferrin saturation and higher in PTH. The differences between the two groups were statistically significant (all P<0.05). Spearman correlation analysis showed MMSE score had a negative correlation with age (r=-0.471, P=0.003) and had positive correlations with education status (r=0.355, P=0.031) and hypertension (r=0.358, P=0.030). The study did not find the relationship among lacunar infarction, WMH and MMSE score; however, recall function was found negatively correlated with lacunar infarction (r=-0.357, P=0.030). Conclusion: ESRD patients undergoing maintenance hemodialysis have a high prevalence of cerebrovascular disease and cognitive impairment. Older age, anemia, inflammation status, chronic kidney disease-mineral and bone disorder may be the influencing factors of the cerebral structural abnormalities and cognition decline.

The Impact of Perioperative Factors on Changes in Diastolic Function after Kidney Transplantation: A Retrospective Analysis

PURPOSE: Cardiac changes in end-stage renal disease are the most common causes of death after kidney transplantation (KT). Chronic kidney disease presents a major risk factor for the development and progression of diastolic dysfunction. The purpose of this study was to identify the association between changes in left ventricular (LV) diastolic function and perioperative clinical factors in patients with preserved ejection fraction following KT. MATERIALS AND METHODS: We reviewed 115 patients who underwent KT between January 2011 and December 2015 with both preand post-transplant echocardiograms; patients with LV systolic dysfunction were excluded. LV diastolic function was measured using the ratio of early transmitral flow velocity to early diastolic velocity of the mitral annulus (E/e′). RESULTS: Patients with normal pre-operative LV systolic function (n=97) showed improvement in E/e′ after KT (11.9±4.4 to 10.5±3.8, p=0.023). Additionally, post-KT estimated glomerular filtration ratio was associated with changes in E/e′ (odds ratio, −0.056; 95% confidence interval, −0.014 to −0.007; p=0.026). Among patients with preexisting diastolic dysfunction (20/97 patients), the amount of intraoperative fluid administration was related to E/e′ changes (odds ratio, 0.003; 95% confidence interval, 0.000 to 0.005; p=0.029). CONCLUSION: KT is associated with improved diastolic function. Post-KT renal function was significantly related to changes in LV diastolic function. The amount of intraoperative fluid was a risk factor for worsening diastolic function after KT in patients with preexisting diastolic dysfunction.

Pyruvate dehydrogenase activity and pyruvate dehydrogenase kinase 4 expression in skeletal muscle of end-stage renal disease patients / 上海交通大学学报（医学版）

Objective: To explore the changes of pyruvate dehydrogenase (PDH) activity and pyruvate dehydrogenase kinase 4 (PDK4) expression in the end-stage renal disease (ESRD) patients' skeletal muscles. Methods: Skeletal muscle samples were collected from non-chronic kidney disease (non-CKD) patients and ESRD patients. PDH activity was detected by ELISA assay. Real-time qPCR was performed to examine gene transcription levels of PDK1-PDK4 and PDH subunits. Western blotting analysis was used to detect protein expression levels of PDK1 and PDK4. Results: There were no demographic differences between two groups of patients. Plasma creatinine and urea nitrogen were significantly elevated in ESRD group (both P<0.05), while estimated glomerular filtration rate, hemoglobin and plasma albumin in ESRD group were significantly lower than those in non-CKD group (all P<0.05). Skeletal muscle PDH activity in ESRD group was markedly lower than that in non-CKD group (P=0.014). There were no differences in PDK1-PDK4 and PDH subunits mRNA transcription levels between ESRD and non-CKD group. PDK4 protein expression was significantly higher than that in non-CKD group (P=0.000). Conclusion: The decreased PDH activity in ESRD patients' skeletal muscle may be related to up-regulation of PDK4.

Pyruvate dehydrogenase activity and pyruvate dehydrogenase kinase 4 expression in skeletal muscle of end-stage renal disease patients / 上海交通大学学报（医学版）

Objective·To explore the changes of pyruvate dehydrogenase (PDH) activity and pyruvate dehydrogenase kinase 4 (PDK4) expression in the end-stage renal disease (ESRD) patients' skeletal muscles. Methods·Skeletal muscle samples were collected from non-chronic kidney disease (non-CKD) patients and ESRD patients. PDH activity was detected by ELISA assay. Real-time qPCR was performed to examine gene transcription levels of PDK1-PDK4 and PDH subunits.Western blotting analysis was used to detect protein expression levels of PDK1 and PDK4. Results·There were no demographic differences between two groups of patients. Plasma creatinine and urea nitrogen were significantly elevated in ESRD group (both P<0.05), while estimated glomerular filtration rate, hemoglobin and plasma albumin in ESRD group were significantly lower than those in non-CKD group (all P<0.05).Skeletal muscle PDH activity in ESRD group was markedly lower than that in non-CKD group(P=0.014).There were no differences in PDK1-PDK4 and PDH subunits mRNA transcription levels between ESRD and non-CKD group.PDK4 protein expression was significantly higher than that in non-CKD group (P=0.000). Conclusion·The decreased PDH activity in ESRD patients' skeletal muscle may be related to up-regulation of PDK4.

A comparison between 9-month versus 6-month cyclophosphamide induction chemotherapy in the management of lupus nephritis in a government tertiary pediatric hospital in the Philippines

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Lupus nephritis is very common complications in SLE, with clinical symptoms of renal involvement occurring in 30%-70% of patients. Outcomes in children with proliferative lupus nephritis (PLN) show 9-15% progress to end-stage renal disease (ESRD) at 5 years.</p><p style="text-align: justify;"><strong>OBJECTIVES:</strong> This study compared the outcome of children and adolescent patients with lupus nephritis treated with 9 month versus 6 month induction of cyclophosphamide therapy. Renal frequency and adverse effects of IV cyclophosphamide during and after induction therapy were described and determined.</p><p style="text-align: justify;"><strong>DESIGN:</strong> Retrospective Cohort Study</p><p style="text-align: justify;"><strong>SETTING:</strong> Tertiary Hospital</p><p style="text-align: justify;"><strong>METHODS:</strong> Retrospective cohort study comparing 6 and 9 month protocol of IV cyclophosphamide for lupus nephritis were conducted in a government tertiary pediatric hospital in the Philippines. A total of 39 patients with lupus nephritis were gathered, 23 patients underwent 6 months and 16 patients underwent 9 months protocol.</p><p style="text-align: justify;"><strong>RESULTS:</strong> The comparison of two protocols in the administration of intravenous cyclophosphamide (IVCY) did not show significant difference between the two in terms of changes in GFR levels, but some evidence of a greater percent increase from baseline with the 6 months protocol post treatment were observed. Among 39 subjects, creatinine, albumin and urinalysis profile did not also differ between the two groups and levels within each group changed insignificantly over time up to 24 months. Proportion of subjects with renal flare ups, adverse effects and who expired during the study period were also essentially similar between the two groups.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> IV Cyclophosphamide seems efficacious if given at the very beginning of the flare and at the start after patient was diagnosed with lupus nephritis. No statistically difference between the duration of the protocol. Renal flare ups and adverse effects of cyclophosphamide such as nausea, vomiting and headache were observed similarly between two protocols. Diligent follow up is needed for further studies and specificity of the results.</p>

Role of Glycemic control and Lipid profile in management of Diabetic complications

Background: Diabetes mellitus is one of the most common endocrine disorders affecting about 6% of the world’s population. Diabetes mellitus is the leading cause of end stage renal disease (ESRD), a major cause of non-traumatic amputations, responsible for preventable blindness and a leading cause of cardiovascular mortality. Objective: The objective of the study was to assess the glycemic control by estimation of glycated hemoglobin (HbA1c), and lipid profile in patients of Diabetes mellitus without complications and in Diabetes mellitus with complications like neuropathy, retinopathy and nephropathy and compare with controls. Material and methods: The present study comprised of 100 clinically diagnosed and confirmed cases of type 2 Diabetes mellitus attending and admitted in RNT Medical College and Hospital, Udaipur, Rajasthan, India. Glycosylated hemoglobin (HbA1c), Total Cholesterol, Triglycerides, HDL-Cholesterol, LDLCholesterol and VLDL-Cholesterol were calculated in all groups using Friedewald’s formula. Result: Our study showed that HbA1c levels were significantly higher (p<0.01) in all groups of patients as compared to controls. The increase in Serum Cholesterol, Triglyceride, LDL-Cholesterol, VLDL-Cholesterol and decrease in HDL-Cholesterol levels were statistically significant (p<0.01) in Diabetic retinopathy and Diabetic nephropathy group as compared to controls, whereas in Diabetic neuropathy group and in Diabetes mellitus without complication, the increase in Serum Cholesterol, Triglyceride, LDL-Cholesterol, VLDL-Cholesterol and decrease in HDL-Cholesterol levels was not statistically significant as compared to controls. Conclusion: Our study revealed that poor glycemic control and dyslipidemia are associated with Diabetic complications like neuropathy, retinopathy and nephropathy. Estimation of glycosylated hemoglobin and lipid profile helps in predicting the development of microvascular complications.

A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

Association of MYH9 SNPs with end-stage renal disease in Chinese Han popula-tion / 中国免疫学杂志

Objective:To explore the associations of MYH9 gene polymorphisms with ESRD in Han population in the fragment between exon 23 and 24.Methods:A hospital-based case control study was carried out including 180 patients and 118 controls in this study.Single nucleotide polymorphisms of MYH9 gene were determined using PCR sequencing,and the haplotypes were calculated using phase software(version 2.0),and transcription factor binding sites were predicted using AliBaba2.Univariate analysis was conducted for exploring the associations between polymorphisms and ESRD.Results: Five newly discovered and three previously reported SNP loci [Rs4821480(MYH9-92),Rs2032487(MYH9-273) and Rs4821481(MYH9-787)]were homozygote genotyped by bidirectional se-quencing.Among newly discovered polymorphisms,two were found at the 489 locus(G→A)and the 616 locus(A→C) in the 901 bp fragment which located in the intron 23 of MYH9 gene.A G489A transversion was very likely a risk mutation contribute to the occurrence of ESRD(P=0.013).No association was observed between ESRD and three previous reported sites [Rs4821480(MYH9-92),Rs2032487(MYH9-273)and Rs4821481(MYH9-787)].The most common haplotype was TCTCGGAT,which was less frequent in the cases than that in the controls.Moreover, TCTCGGCT and TCTCAGAT haplotypes were more in the cases than that in the controls.The number of transcription binding sites increased from 82 ( wild ) to 85 ( mutation ) in the 23th intron of MYH9 gene.Conclusion:Polymorphisms of MYH9 at intron 23 may influence the prevalence of ESRD in Chinese Han population and TCTCGGAT haplotype may be one protective haplotype.TCTCGGCT and TCTCAGAT may be risk haplotypes attributed to ESRD.The polymorphism of MYH9 at the 23th intron may company with the amount alteration of transcription factor binding sites.

Transplantation Options in Type 1 Diabetes with End Stage Renal Disease / 대한이식학회지

For patients with type 1 diabetes complicated by end stage renal disease (ESRD), kidney transplantation prolongs life and frees the recipient from the substantial morbidities of dialysis. Transplantation of whole pancreas has emerged as an effective treatment modality for patients with diabetes mellitus, especially those with established end-stage renal disease. However, it is not clear that transplantation of the pancreas itself prolongs the life of the recipient. Living donor kidney transplant (LDKT) and simultaneous pancreas-kidney transplant (SPKT) provide distinct benefits over deceased donor kidney transplant (DDKT) alone. But, previous studies have not demonstrated that the SPKT approach prolongs life compared with LDKT. Patients who receive an LDKT can later opt for a pancreas after kidney transplant (PAKT) or an islet after kidney transplant (IAKT). Therefore, when a transplant doctor is counseling a patient with type 1 diabetes and ESRD and has a live donor, the decision about whether to proceed with an LDKT or to remain on the waiting list for an SPKT requires careful consideration. The aim of this review was to summarize the current status and outcomes of SPKT, LDKT, IAKT, and PAKT and their effects on survival of patients with type 1 diabetes and ESRD.

A Renal Transplantation and Hemodialysis Cost-Utility Analysis in Patients with End-Stage Renal Disease / 대한이식학회지

BACKGROUND: The purpose of this study was to examine the cost-effectiveness of renal transplantation and hemodialysis among end-stage renal disease patients. METHODS: Empirical data on treatment costs were collected from five hospitals in Korea. We used European Renal Association-European Dialysis and Transplant Association registry data for transition probability. Quality adjusted life year (QALY) values were derived from the literature. A Markov model was used for predicting the cost utility of transplantation and hemodialysis over a 10-year period. RESULTS: Renal transplantation was less costly and resulted in a better outcome than hemodialysis. The cost per QALY gained was 19,450 thousand won in transplantation patients, whereas it was 36,514 thousand won per QALY gained in hemodialysis patients. CONCLUSIONS: Although the cost of the first year after transplantation was expensive, transplantation was more effective over 2 years and was less costly than hemodialysis. The results suggest that transplantation is more cost-effective than hemodialysis in Korea.

Effects of ezetimibe on lipid profiles and hemostatic markers in end-stage renal disease / 대한내과학회지

BACKGROUND/AIMS: Dyslipidemia is one of the major causes of cardiovascular disease in end-stage renal disease (ESRD) patients. Most of them are dyslipidemic despite the use of lipid-lowering agents. Ezetimibe is a novel chemical entity that inhibits the intestinal absorption of dietary and biliary cholesterol. This study evaluated the effects of ezetimibe on the lipid profile, inflammation markers, endothelial injury, and thrombogenesis in ESRD patients. METHODS: Sixty-five patients with serum low-density lipoprotein (LDL)-cholesterol levels > or =100 mg/d were recruited: 33 patients were on hemodialysis and 32 patients were on peritoneal dialysis. They were assigned randomly to the ezetimibe (10 mg) monotherapy group and the ezetimibe (10 mg) plus simvastatin (10 mg) combination therapy group. Both drugs were administered for 8 weeks. RESULTS: There were no significant differences in the baseline demographic and laboratory characteristics between the two groups. In the monotherapy group, the total and LDL-cholesterol levels were reduced by 14.7 and 21.9%, respectively. There were no changes in the high-density lipoprotein (HDL)-cholesterol or triglyceride levels. Fibrinogen increased significantly (p=0.04). In the combination therapy group, the total and LDL-cholesterol levels were reduced by 29.8 and 42.4%, respectively. There was an additional 15.1% reduction in total cholesterol and an additional 20.5% reduction in LDL cholesterol compared with monotherapy. Several patients complained of minor adverse effects and only one patient in the ezetimibe monotherapy group discontinued medication, because of diarrhea. CONCLUSIONS: In ESRD patients, ezetimibe used as combination therapy with a statin is more effective than ezetimibe monotherapy in ESRD patients.

Depression in Patients with End-Stage Renal Disease

Depression is common in patients with end-stage renal disease(ESRD) and has a negative effect on the quality of life, functional ability, and mortality of the patients, with a prevalence rate as high as 20-25%. Especially, the increasing tendency of mortality in ESRD patients is associated with recent or current depression, and the suicide rate is also increased by depression in patients with ESRD. Therefore, accurate detection and appropriate treatment of depression is very important in ESRD patients. Also, a deferential diagnosis is needed concerning uremic symptoms and depression in ESRD patients. However, there has been little data so far particularly in terms of randomized clinical trials. This review focused on the recent knowledge of depression in ESRD, and could encourage clinical study and trials in this field.

A Case of End-Stage Renal Disease (ESRD) from Reflux Nephropathy in a patient with Situs Inversus and Duplicated Ureter / 대한신장학회지

Situs inversus is a rare congenital anomaly that occurs in adults at the rate of 1:5,000-1:10,000. In the total form, the thoracic organs, as well as the abdominal organs, are completely reversed in a "mirror image" of their normal arrangements. Ureteral duplication is the most common ureteral anomaly founded incidentally. However, there is a higher incidence of duplication in cases of urinary tract infection, and it may be associated with upper urinary tract stasis, obstruction, or reflux. But ureteral duplication has no relation to situs inversus. Vesicoureteral reflux (VUR) is the most common disorder usually detected during radiologic evaluation of children with urinary tract infection. Complications such as renal scarring, reflux nephropathy, chronic renal failure and hypertension are well known in patients with VUR. Reflux nephropathy is responsible for about 10% of all cases of treated ESRD and is the most common case of ESRD in children. Thus, if VUR exists, early diagnosis and intensive treatment is necessary. Herein, we present a case of reflux nephropathy related ESRD in a 41-year-old woman with total situs inversus and duplicated ureter.

Sleep disturbances in patients on maintenance hemodialysis: role of dialysis shift

OBJECTIVES: Subjective sleep complaints have been reported in up to 80 percent of patients with end stage renal disease (ESRD). In these patients, sleep disturbances manifesting as insomnia, sleep apnea syndrome, restless leg syndrome (RLS), periodic limb movement disorder and excessive daytime sleepiness (EDS) have been frequently reported. Moreover, studies about the role of dialysis shift on sleep abnormalities, morbidity and mortality are still scarce. The aim of this study was to investigate the influence of dialysis shift on the quality of sleep and sleep abnormalities in patients with ESRD. MÉTHODS: We studied one hundred consecutive patients from a dialysis center. Quality of sleep was assessed by the Pittsburgh Sleep Quality Index and subjective EDS by the Epworth Sleepiness Scale. Restless leg syndrome was diagnosed using the four minimum criteria defined by the International Restless Legs Syndrome Study Group. Clinical and laboratory parameters were obtained by interview and chart review. Adequacy of dialysis was evaluated by the Kt/V index. RESULTS: Poor quality sleep (PSQI>6) was found in 75 percent of cases and was associated with RLS (p=0.004) and with snoring (p=0.016). EDS (ESS>10) was present in 28 percent of cases. Patients with EDS (1.33±0.29) had lower values of the Kt/v index (P=0.01) than those without EDS (1.52±0.32). RLS was present in 48 percent of cases. Irrespective of dialysis shift, poor quality sleep, EDS and RLS were not different among patients. CONCLUSION: Poor quality sleep, EDS and RLS were common and not related to dialysis shift.

OBJETIVOS: Alterações do sono têm sido relatadas em até 80 por cento dos pacientes com Insuficiência renal crônica dialítica (IRCD). Insônia, síndrome da apnéia do sono, síndrome das pernas inquietas (SPI), movimentos periódicos de extremidades e sonolência excessiva diurna (SED) têm sido descritos. A influência que o turno da diálise exerce sobre as alterações do sono e sobre a morbidade e mortalidade ainda é desconhecida. O objetivo deste estudo foi avaliar a influência do turno da diálise sobre as anormalidades do sono em pacientes com IRCD. MÉTODOS: Estudamos 100 pacientes consecutivos provenientes de um centro de diálise. A qualidade do sono foi avaliada através do Índice de Qualidade do Sono de Pittsburgh (IQSP) e a SED através da Escala de sonolência de Epworth (ESE). A SPI foi avaliada utilizando os quatro critérios mínimos definidos internacionalmente pela International Restless Legs Syndrome Study Group. Os parâmetros clínicos e laboratoriais foram obtidos através de entrevistas e revisão de prontuários. A qualidade da diálise foi avaliada pelo índice Kt/V. RESULTADOS: Má qualidade do sono (IQSP>6), encontrada em 75 por cento dos casos, associou-se à SPI (P= 0.004) e à presença de ronco (P= 0.016). Pacientes com SED (ESE>10) (1.33±0.29) apresentaram valores do índice Kt/v menores (P=0.01) do que aqueles sem SED (1.52±0.32). Observou-se a presença de SPI em 48 por cento dos pacientes. Má qualidade do sono, SED e SPI não diferiram entre os pacientes agrupados quanto ao turno de diálise. CONCLUSÃO: Má qualidade do sono, SED e SPI são freqüentes e não se relacionam com o turno da diálise.

Impact of Insulin Resistance on Glycemic Control in Diabetic End Stage Renal Disease Patients on Hemodialysis / 대한신장학회잡지

BACKGROUND: Type 2 diabetes develops because of defects in both insulin secretion and action. The half-life of insulin in uremia is prolonged because the metabolic clearance rate of insulin in diabetic end stage renal disease (ESRD) patients is reduced with consequence that the dose of insulin and/or oral hypoglycemic agent (OHA) administered in normal renal function make them increase the risk of hypoglycemia. Therefore, we should usually reduce the dose of insulin and/or OHA, or stop administration of insulin and/or OHA if type 2 diabetic patients are progressed to ESRD. But in some patients, that is not true. The aim of this study was to test the hypothesis that insulin resistance plays an important role in (re)evaluation of optimal insulin and/or OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD. METHODS: Insulin resistance was examined in 23 type 2 diabetic ESRD patients with tight control of glycemia using the K index of the insulin tolerance test (Kitt). We divided 23 patients into three groups. Group 1 (n=10) was defined as patients who were administered neither insulin nor OHA after ESRD. Group 2 (n=9) was defined as patients who were changed from insulin to OHA as drug for glycemic control after ESRD. Group 3 (n=4) was defined as patients in whom insulin or OHA was continuously administered after ESRD without a change of them for glycemic control. We compared the degree of insulin resistance among these three groups. RESULTS: Insulin resistance determined by Kitt was significantly different between group 1 (Kitt, 2.1422/0.94-4.01%/min), group 2 (Kitt, 1.3811/0.79- 3.90%/min) and group 3 (Kitt, 0.8550/0.44-1.81%/min) by using Kruskal-Wallis test (p=0.048). Kitt in group 3 was significantly lower than in group 1 by using Mann-Whitney test (p=0.016). CONCLUSION: Although metabolic clearance of insulin is reduced by renal failure, demand of insulin/ OHA for optimal glycemic control is not reduced in higher insulin-resistant type 2 diabetic ESRD patients on hemodialysis. Insulin resistance plays an important role in determination of optimal insulin/ OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD.