Successful High-dose Chemotherapy in Combination With Autologous Peripheral Blood Stem Cell Transplantation in an Anuric Child With Neuroblastoma.

No reports describe high-dose chemotherapy (HDCT) with autologous peripheral blood stem cell transplantation (auto-PBSCT) in pediatric patients with neuroblastoma and end-stage renal disease. Here, we report the case of a patient with high-risk neuroblastoma who developed anuria during treatment. HDCT with auto-PBSCT under hemodialysis, with strict attention to the ultrafiltration volume and dose modification of alkylating agents, was performed. Although the first auto-PBSCT led to engraftment failure, the second auto-PBSCT resulted in successful myeloid engraftment 8 months after anuria. This case demonstrated that HDCT with auto-PBSCT can be safely performed in children with renal failure under hemodialysis.

Incremental Peritoneal Dialysis May be Beneficial for Preserving Residual Renal Function Compared to Full-dose Peritoneal Dialysis.

Maintaining residual renal function (RRF) is a crucial issue in peritoneal dialysis (PD). Incremental dialysis is the practice of initiating PD exchanges less than four times a day in consideration of RRF, and increasing dialysis dose in a step-wise manner as the RRF decreases. We aimed to compare the outcomes of incremental PD and full-dose PD in terms of RRF preservation and other outcomes. This was a single-center, observational study. Data were extracted retrospectively from a cohort of incident PD patients over 16 years old who started PD between 2007 and 2015 in the PD Unit of Seoul National University Hospital. We used inverse probability weighting (IPW) adjustment based on propensity scores to balance covariates between the incremental and full-dose PD groups. Multivariate, time-dependent Cox analyses were performed. Among 347 incident PD patients, 176 underwent incremental PD and 171 underwent conventional full-dose PD. After IPW adjustment, the incremental PD group exhibited a lower risk of developing anuria (hazard ratio [HR] 0.61, 95% confidence interval [CI] 0.43-0.88). Patient survival, technique survival, and peritonitis-free survival were all similar between these groups (P > 0.05 by log-rank test). Incremental PD was beneficial for preserving RRF and showed similar patient survival when compared to conventional full-dose PD.

Survival predictors in anuric patients on peritoneal dialysis: A prospective, multicenter, propensity score-matched cohort study.

Prevalent anuric peritoneal dialysis (PD) patients usually have higher mortality than PD patients with residual urine volume. We aimed to evaluate the predictors of survival in anuric PD patients. Anuric PD patients (n = 505, <100 mL of daily urine) enrolled in Korean nationwide prospective cohort were analyzed. Survived and non-survived anuric PD patients were compared by propensity score matching analysis with a ratio of two to one. The propensity method was used to adjust for patient age, dialysis duration, and presence of diabetes. Among the total anuric PD patients, non-survived patients showed a significantly older age, higher incidence of diabetes, coronary artery disease, and arrhythmia, and lower serum creatinine and albumin. After propensity score matching, multivariate Cox regression analysis for patient survival showed a decreasing risk as serum albumin increased (HR = 0.347, p = 0.0094). Analysis using the receiver-operating-characteristic (ROC) curve showed that survival could be predicted with a sensitivity of 59.4% and a specificity of 63.2% using a cutoff value of 3.6 g/dL of serum albumin in unmatched total PD patients. The beneficial impact of high albumin level on death was significantly greater for patients with older age (≥50 years), no diabetes, low ultrafiltration (UF) volume (<1000 mL/day), and low levels of serum creatinine (<10 mg/dL), total cholesterol (<177.5 mg/dL), ferritin (<100 ng/mL), and high-sensitivity C-reactive protein (hs-CRP) (<0.1 mg/dL). Survival in anuric PD patients was associated with age, comorbidities, and nutritional factors such as creatinine and albumin. After adjustment by propensity score matching, serum albumin level was an independent predictor for survival in anuric PD patients.

[Mortal poisoning by bee stings in a child: Study of a case observed in the regional hospital of Maradi (Niger)]. / Envenimation mortelle par piqûres d'abeilles chez l'enfant : à propos d'un cas observé au centre hospitalier régional de Maradi (Niger).

We report a fatal case due to a massive attack by a swarm of bees in a nine-year-old child. The accident was fatal because of two aggravating factors: the cephalic location and the large number of stings (about 300). Complications were coagulopathy, anemia, hemorrhage, coma, and oligoanuria. Support was symptomatic at a facility second level. The outcome was fatal within 14 days. In view of the encountered difficulties, we recommend to build written protocols for the management of envenomation in any health training reference.

The Role of Dialysis Membranes on Intradialytic Selenium Removal and on Selenium Status in Patients Receiving Renal Replacement Therapy.

BACKGROUND/AIMS: Dialysis membrane has been implicated in selenium (Se) deficiency in hemodialysis (HD). Intradialytic Se removal into dialysate through different membranes was investigated. METHODS: We studied 19 patients on standard HD with low-flux polysulfone membrane (group A), 10 patients on standard HD with ethylene vinyl alcohol membrane (group B), 12 patients on hemodiafiltration (HDF; group C) and 16 healthy subjects (control group D). Se was measured in blood before and after dialysis session and in effluent dialysate every hour during session. RESULTS: In all patients together, pre-dialysis serum Se levels were lower than those in control group, but, in a separate analysis, only in standard HD. In all patient groups, there was a net Se removal into dialysate but it was greater in HDF patients who, however, had similar pre-dialysis serum Se levels to those in healthy controls. CONCLUSION: An intradialytic Se loss was found with all 3 membrane types, but it is not the principal factor for Se depletion in HD.

Faster Transport Status and Mortality in Anuric Patients Undergoing Continuous Ambulatory Peritoneal Dialysis.

BACKGROUND: There are limited data regarding the relationship between transport status and mortality in anuric continuous ambulatory peritoneal dialysis (CAPD) patients. METHODS: According to the dialysate to plasma creatinine ratio (D/P Cr), 292 anuric CAPD patients were stratified to faster (D/P Cr ≥0.65) and slower transport groups (D/P Cr <0.65). The Cox proportional hazards models were used to evaluate the association of transport status with mortality. RESULTS: During a median follow-up of 22.1 months, 24% patients died, 61.4% of them due to cardiovascular disease (CVD). Anuric patients with faster transport were associated with an increased risk of all-cause mortality (HR (95% CI) = 2.16 (1.09-4.26)), but not cardiovascular mortality, after adjustment for confounders. Faster transporters with pre-existing CVD had a greater risk for death compared to those without any history of CVD. CONCLUSION: Faster transporters were independently associated with high all-cause mortality in anuric CAPD patients. This association was strengthened in patients with pre-existing CVD.

Resolution of refractory hypotension and anuria in a premature newborn with loss-of-function of ACE.

We present the investigation and management of a premature, hypotensive neonate born after a pregnancy complicated by anhydramnios to highlight the impact of early and informed management for rare kidney disease. Vasopressin was used to successfully treat refractory hypotension and anuria in the neonate born at 27 weeks of gestation. Next generation sequencing of a targeted panel of genes was then performed in the neonate and parents. Subsequently, two compound heterozygous deletions leading to frameshift mutations were identified in the angiotensin 1-converting enzyme gene ACE; exon 5:c.820_821delAG (p.Arg274Glyfs*117) and exon24: c.3521delG (p.Gly1174Alafs*12), consistent with a diagnosis of renal tubular dysgenesis. In light of the molecular diagnosis, identification, and treatment of associated low aldosterone level resulted in further improvement in renal function and only mild residual chronic renal failure is present at 14 months of age. Truncating alterations in ACE most often result in fetal demise during gestation or in the first days of life and typically as a result of the Potter sequence. The premature delivery, and serendipitous early treatment with vasopressin, and then later fludrocortisone, resulted in an optimal outcome in an otherwise lethal condition.

Ranking of factors determining potassium mass balance in bicarbonate haemodialysis.

BACKGROUND: One of the most important pathogenetic factors involved in the onset of intradialysis arrhytmias is the alteration in electrolyte concentration, particularly potassium (K(+)). METHODS: Two studies were performed: Study A was designed to investigate above all the isolated effect of the factor time t on intradialysis K(+) mass balance (K(+)MB): 11 stable prevalent Caucasian anuric patients underwent one standard (∼4 h) and one long-hour (∼8 h) bicarbonate haemodialysis (HD) session. The latter were pair-matched as far as the dialysate and blood volume processed (90 L) and volume of ultrafiltration are concerned. Study B was designed to identify and rank the other factors determining intradialysis K(+)MB: 63 stable prevalent Caucasian anuric patients underwent one 4-h standard bicarbonate HD session. Dialysate K(+) concentration was 2.0 mmol/L in both studies. Blood samples were obtained from the inlet blood tubing immediately before the onset of dialysis and at t60, t120, t180 min and at end of the 4- and 8-h sessions for the measurement of plasma K(+), blood bicarbonates and blood pH. Additional blood samples were obtained at t360 min for the 8 h sessions. Direct dialysate quantification was utilized for K(+)MBs. Direct potentiometry with an ion-selective electrode was used for K(+) measurements. RESULTS: Study A: mean K(+)MBs were significantly higher in the 8-h sessions (4 h: -88.4 ± 23.2 SD mmol versus 8 h: -101.9 ± 32.2 mmol; P = 0.02). Bivariate linear regression analyses showed that only mean plasma K(+), area under the curve (AUC) of the hourly inlet dialyser diffusion concentration gradient of K(+) (hcgAUCK(+)) and AUC of blood bicarbonates and mean blood bicarbonates were significantly related to K(+)MB in both 4- and 8-h sessions. A multiple linear regression output with K(+)MB as dependent variable showed that only mean plasma K(+), hcgAUCK(+) and duration of HD sessions per se remained statistically significant. Study B: mean K(+)MBs were -86.7 ± 22.6 mmol. Bivariate linear regression analyses showed that only mean plasma K(+), hcgAUCK(+) and mean blood bicarbonates were significantly related to K(+)MB. Again, only mean plasma K(+) and hcgAUCK(+) predicted K(+)MB at the multiple linear regression analysis. CONCLUSIONS: Our studies enabled to establish the ranking of factors determining intradialysis K(+)MB: plasma K(+) → dialysate K(+) gradient is the main determinant; acid-base balance plays a much less important role. The duration of HD session per se is an independent determinant of K(+)MB.

A case of sodium chlorite toxicity managed with concurrent renal replacement therapy and red cell exchange.

INTRODUCTION: Sodium chlorite is a powerful oxidizing agent with multiple commercial applications. We report the presentation and management of a single case of human toxicity of sodium chlorite. CASE REPORT: A 65-year-old man presented to hospital after accidentally ingesting a small amount of a sodium chlorite solution. His principal manifestations were mild methemoglobinemia, severe oxidative hemolysis, disseminated intravascular coagulation, and anuric acute kidney injury. He was managed with intermittent hemodialysis, followed by continuous venovenous hemofiltration for management of acute kidney injury and in an effort to remove free plasma chlorite. Concurrently, he underwent two red cell exchanges, as well as a plasma exchange, to reduce the burden of red cells affected by chlorite. These interventions resulted in the cessation of hemolysis with stabilization of serum hemoglobin and platelets. The patient survived and subsequently recovered normal renal function. DISCUSSION: This is only the second case of sodium chlorite intoxication reported in the medical literature and the first to report the use of renal replacement therapy in combination with red cell exchange in its management.

Pathologic findings and toxin identification in cyanobacterial (Nodularia spumigena) intoxication in a dog.

A 3-year-old Cairn Terrier dog that had been in contact with sea water containing cyanobacteria (blue-green algae) was euthanized because of acute hepatic failure and anuria after a 5-day illness. Histologic findings included lytic and hemorrhagic centrilobular hepatocellular necrosis and renal tubular necrosis. The cyanotoxin nodularin was detected in liver and kidney by high-performance liquid chromatography-mass spectrometry. Nodularin is a potent hepatotoxin produced by the algal species Nodularia spumigena. The intensity of algal blooms has increased during the past decades in the Baltic Sea region, thus increasing the risk for intoxications in domestic and wild animals. The authors describe the pathologic findings of cyanobacterial toxicosis in a dog with direct identification of the toxin from organ samples.

Lower urinary tract symptoms (LUTS) after renal transplant in non-urologic anuric patients.

We assessed LUTS at least 12 months after RTx in patients without evidence of lower urinary tract dysfunction (non-urologic) that had been anuric for at least six months before RTx. No bladder recycling was performed before RTx. LUTS were evaluated using a questionnaire. Clinical records were also reviewed. LUTS in anuric patients were compared with those in non-anuric patients. Fourteen anuric patients fulfilled the inclusion criteria. Median age at RTx was 11 (5-21) yr, median duration of anuria before RTx 24 (7-46) months, and median post-RTx follow-up 2.7 (1.9-10.2) yr. Daytime symptoms were exceptional. Nocturia was the most common symptom (10 patients). Only one patient reported symptoms to affect her quality of life. One patient experienced a febrile UTI and none graft failure. LUTS (nocturia) proved unrelated to duration of anuria, length of follow-up, and presence of (nocturnal) polyuria. LUTS were not statistically different in patients anuric and non-anuric before RTx. Non-urologic patients suffer from long-term storage symptoms, particularly nocturia. LUTS, however, do not seem to increase the risks of urinary infections or graft failure and appear to occur irrespective of the presence of anuria before RTx. Bladder recycling before RTx seems unnecessary.

A further case of renal tubular dysgenesis surviving the neonatal period.

Renal tubular dysgenesis is a critical disorder characterized by the Potter phenotype and severe hypotension in the early neonatal period. We herein report a 3-year-old female with renal tubular dysgenesis. Endocrinological studies showed a high plasma renin activity (over 49.2 ng/ml/h; normal range 2.0-15.2), high active renin concentration (1,823.5 pg/ml; normal range 2.4-21.9), and low angiotensin-converting enzyme (ACE) concentration (1.7 U/l; normal range 8.3-21.4). Taken together, these findings suggested an abnormality of the ACE gene, ACE. Direct sequencing analysis revealed two novel deletions in the coding region of ACE. We conclude that hormonal analysis of the renin-angiotensin system can aid in identifying the responsible genes and help with efficient gene analysis and pathophysiological considerations.

A systematic evaluation of bioelectrical impedance measurement after hemodialysis session.

BACKGROUND: There is still no definitive indication about the ideal point of time to perform bioimpedance analysis (BIA) in hemodialysis patients. Furthermore, the interpretation of data in this regard is difficult because there is still no comprehensive information about the fluctuations in BIA variables occurring in these subjects. The aim of this study was to assess BIA changes occurring in hemodialysis and specifically in the dry-weight state. METHODS: We studied 27 anuric patients (20 males and 7 females; age 56.1 +/- 13.7 years) on chronic hemodialysis. Single-frequency BIA (R, resistance; Xc, reactance; and PhA, phase angle) was performed (1) before and at the end of hemodialysis (dialysis period); (2) 15, 30, 60, 90, and 120 minutes after hemodialysis (postdialysis period); and (3) 24, 48, and 68 hours after hemodialysis (interdialysis period). RESULTS: Body weight decreased by 2.8 +/- 0.8 kg during hemodialysis, was unchanged during the postdialysis period, and progressively rose during the interdialysis period. At the same time, BIA variables significantly increased during hemodialysis (R, 453 +/- 74 and 542 +/- 98 ohm; Xc, 38 +/- 10 and 53 +/- 16 ohm; P < 0.05), remained stable over the 120-minute period after treatment (R, 538 +/- 94, 539 +/- 95, 538 +/- 94, 541 +/- 95, and 544 +/- 95 ohm; and Xc, 53 +/- 15, 53 +/- 15, 51 +/- 16, 52 +/- 16, and 52 +/- 16 ohm; NS), and subsequently declined [R, 471 +/- 79 (P= <0.05 vs. postdialysis), 449 +/- 71 (P= <0.05 vs. postdialysis), 424 +/- 68 (P= <0.05 vs. postdialysis) ohm; Xc, 42 +/- 13 (P= <0.05 vs. postdialysis), 37 +/- 10 (P= <0.05 vs. postdialysis), 34 +/- 13 (P= <0.05 vs. postdialysis) ohm]. The stability of BIA measures during postdialysis was confirmed by the constant relationship found between R/height and Xc/height. Also PhA increased after dialysis (4.8 +/- 1.1 degrees vs. 5.7 +/- 1.3 degrees, P < 0.05), was unchanged during the following 120 minutes and decreased in the interdialysis period (5.1 +/- 1.3 degrees, 4.8 +/- 1.0 degrees, and 4.5 +/- 1.1 degrees, P < 0.05). At the end of hemodialysis and during the postdialysis period total body water (TBW) estimated from BIA was similar on average to TBW calculated using Watson formulas (37.2 +/- 6.3 L vs. 36.2 +/- 5.7 L, NS). On the contrary, when patients were hyperhydrated BIA significantly overestimated the Watson's values. CONCLUSION: In hemodialysis patients BIA variables fluctuate to a considerable extent (with the highest values immediately after hemodialysis), but remain constant and highly reproducible over the 120 minutes after the end of hemodialysis, that is, in a dry-weight state. Thus, taking into consideration that the point in time chosen for performing BIA is crucial to properly assess body composition, BIA can be appropriately performed at anytime during the postdialysis period, provided that hydration status does not change due to food or drink consumption.

Ivemark's renal-hepatic-pancreatic dysplasia: analytic approach to a perinatal autopsy.

A case of Ivemark's renal-hepatic dysplasia syndrome is presented. The findings are suggestive of a ductal obstructive process anatomically located at the junction of ducts with the parenchyma. The differential diagnosis includes Meckel syndrome, short rib polydactyly syndromes, and glutaric aciduria type 2. The molecular basis for this syndrome remains unknown.

Left multicystic dysplastic kidney with contralateral ureteropelvic obstruction: report of one case.

Multicystic dysplastic kidney (MCDK) is a common cause of abdominal mass in neonates. It is frequently associated with malformation of the contralateral kidney, such as ureteropelvic obstruction, etc. Because MCDK is usually functionless, it is important to evaluate the condition of the contralateral kidney. The presence of severe obstruction in the contralateral ureteropelvic junction is life-threatening and prompt treatment should be made to preserve the remaining renal function. We report on a neonate with left MCDK and contralateral ureteropelvic obstruction, presenting as anuria after birth, and also we review the literature.

[Anuria after delivery]. / Anurie nach der Geburt.

During the last decade certain peripartal complications decreased dramatically in industrialized countries. Here, a case of a 27 years old Caucasian primagravida will be presented. In the presence of symptoms of a severe preeclamptic condition with signs of an impending HELP syndrome the patient underwent Caesarean section. Within hours following surgery she developed complete anuria. Nuclear magnetic resonance imaging and histological evaluation of a renal biopsy led to the diagnosis of an acute, bilateral renal cortical necrosis. Besides the preeclamptic condition no further underlying disease was present, in particular no hemolytic-uremic syndrome. Following progress made in modern perinatal management renal cortical necrosis almost disappeared. Yet, in the presence of this disease a significant maternal morbidity and mortality still remains.

[Renal tubular dysgenesis with fetal renal vein thrombosis]. / Renale tubuläre Dysgenesie mit fetaler Nierenvenenthrombose.

Renal tubular dysgenesis is a rarely recognized condition characterized by oligohydramnios, Potter's sequence and congenital anuria leading to stillbirth or neonatal death from respiratory failure. It is thought to be inherited in an autosomal recessive manner. Definitive diagnosis is based on renal histology, revealing the lack of proximal tubule differentiation. Two additional cases of affected sibs in a family with parental consanguinity are reported. Lectin and immunohistochemical studies confirmed structural and functional immaturity of the proximal tubule. Further findings include bilateral renal vein thrombosis. The clinical and morphological parameters defining this disorder and the possible mechanisms of pathogenesis are discussed.