Early Renal Failure Research Articles

One year incidence of events after definite coronary stent thrombosis: a retrospective vs prospective comparison and a conglomerate determination of prognostic factors of mortality

Abstract Background Although many studies analysed the predictive factors of coronary stent thrombosis (ST), there are few data on prognostic factors of clinical outcomes after definite ST. The purpose of the current study was to analyse clinical presentation and outcomes at one year after definite coronary ST in two distinct cohorts: a retrospective cohort and a prospective cohort and to determine the prognostic factors. Methods Among 22’039 consecutive patients treated between January 2008 and December 2021, 275 patients presented a definite ST and were included in this study (retrospective cohort n=83 and prospective cohort n=192). Baseline characteristics, clinical ST presentation and 1-year outcomes were analysed between the two cohorts using SPSS Software. According to the obtained results data of both cohorts were pooled and prognostic factors of clinical outcomes determined using multivariate analysis. Results There was no statistical difference between the two cohorts concerning baseline characteristics and clinical ST presentation. All-cause mortality and MACE rate up to 1 year follow-up did not differ either among cohorts (16.9% vs 14.6%, p=0.764 for mortality (Figure 1) and 26.5% vs 24% p=0.766 for MACE). Multivariate analysis of the aggregated data of both prospective and retrospective cohorts revealed age (OR: 1.05 95% CI [1.02;1.09]; p=0.0016), early stent thrombosis (OR: 3.3 95% CI [1.52;7.15] p=0.002), severe renal failure (OR: 3.37 95% CI [1.35;8.42] p=0.009), clinical presentation (cardiogenic shock [OR: 2.92 95% CI {1.17;7.28} p=0.02] and cardiac arrest [OR: 15.3 95% CI {6.62;35.39} p<0.0001]) as strongly associated with a higher risk of death at 1 year whereas higher LVEF was a protective factor (OR: 0.922 95% CI [0.891; 0.955] p<0.0001). Conclusion Comparing retrospectively collected data versus prospectively collected data for definite ST showed very similar data concerning qualitative variables (e.g. baseline characteristics and clinical presentation of ST) suggesting feasibility of data pooling to determine prognostic factors of clinical outcome at one year. Prognostic factors influencing one-year all-cause mortality were age, early ST thrombosis, renal failure, residual LVEF and clinical presentation.Figure 1

Impact of glucose-containing fluid on acute pancreatitis outcomes: A multicenter retrospective analysis

IntroductionFluid resuscitation reduces mortality and morbidity in acute pancreatitis (AP); however, whether glucose-containing fluids negatively impact AP remains uncertain. We aimed to examine the association between glucose-containing fluids and AP outcomes. MethodsThis multicenter retrospective cohort study included patients diagnosed with AP between January 2015 and December 2018. Glucose density was defined as total glucose content divided by total fluid volume (g/dl) on day 1, and was considered high if the level exceeded the median. Endpoints were early organ failure (OF), including cardiovascular, renal, or respiratory system failure within 7 days; 30-day OF; ICU admission; and AP-related 90-day mortality. Logistic regression models, restricted cubic spline curves, and Cox proportional hazards models were used for statistical analysis. ResultsFrom the database, 1,146 patients with AP were included. Early OF occurred in 8.8% of patients within 7 days. The high glucose-density group (>5 g/dl) had increased risk of early OF (9.7% vs. 8.2%; adjusted odds ratio [aOR], 1.69; 95% confidence interval [CI], 1.03–2.80; P = 0.039), respiratory failure (8.0% vs. 6.2%; aOR, 1.88; 95% CI, 1.09–3.24; P = 0.024), cardiovascular failure (3.4% vs. 2.4%; aOR, 3.59; 95% CI, 1.28–10.0; P = 0.015), and ICU admission (6.8% vs. 5.8%; aOR, 2.06; 95% CI, 1.08–3.94; P = 0.029), with a dose-response effect observed for cardiovascular failure and ICU admission. A significant increase 30-day OF risk (adjusted hazard ratio [aHR], 1.70; 95% CI, 1.19–2.45) was also noted. ConclusionExcess glucose-containing fluid was associated with increased risks of overall, respiratory, and cardiovascular OF and ICU admission in AP.

Investigation of the Systemic Immune Inflammation (SII) Index as an Indicator of Morbidity and Mortality in Type 2 Diabetic Retinopathy Patients in a 4-Year Follow-Up Period.

Background and Objectives: This study aimed to investigate the relationship between the systemic immune inflammation (SII) index and the development of micro and macro complications and mortality within the first year and the following three years in type 2 diabetic retinopathy patients. Materials and Methods: The retrospective study included 523 type 2 diabetic retinopathy patients seen in the endocrinology outpatient clinic of our hospital between January and December 2019. Their demographic and clinical characteristics were analyzed using descriptive statistics. The normal distribution of quantitative data was assessed by the Shapiro-Wilk test. Mann-Whitney U, McNemar-Chi-square, and Cochran's Q tests were used to analyze the SII values and complication rates over time. An ROC analysis determined the sensitivity and specificity of SII. A multiple linear regression analysis examined the relationship between variables and SII, while Spearman's test assessed the correlation between CRP and SII. p < 0.05 was accepted as significant. Results: The mean age of patients was 63.5 ± 9.3 years, with mean SII values of 821.4 ± 1010.8. Higher SII values were significantly associated with acute-chronic renal failure, peripheral arterial disease, and hospitalization rates in both the first year and the following three years (p < 0.05 for all). Significant cut-off values for SII were found for micro- and macrovascular complications and death within the first year (p < 0.05 for all). The ROC curve analysis identified an optimal SII cut-off value of >594.0 for predicting near-term (1-year) complications and mortality, with a sensitivity of 73.8% and specificity of 49.4% (area under the ROC curve: 0.629, p = 0.001). Multiple linear regression indicated that smoking of at least 20 pack-years had a significant positive effect on SII. The Spearman test showed a weak positive correlation between SII and CRP. Conclusions: High SII values predict both early and late acute-chronic renal failure, peripheral arterial disease, and hospitalizations in patients with type 2 diabetic retinopathy. The study also shows that high SII values may predict microvascular and macrovascular complications of type 2 DM and mortality risk in the early period in patients with type 2 diabetic retinopathy. In addition, comorbidities and inflammatory habits, such as long-term smoking, should be considered in the clinical use of SII.

THE RELATIONSHIP OF POSTOPERATIVE CYSTATIN C LEVEL WITH EARLY ACUTE RENAL FAILURE IN ADULT PATIENTS UNDERGOING CARDIAC SURGERY

Introduction: Cardiovascular diseases are still the most important cause of mortality and morbidity in the world. Cardiac surgery is the most frequently performed major surgical intervention in hospitals, and acute kidney injury (AKI) that develops after surgery is an important condition that is frequently encountered and increases mortality. The success of treatment may be increased by early detection of AKI that may appear following surgery. In this study, we sought to assess how early stage acute renal failure and postoperative Cystatin C levels related in patients underwent cardiac surgery. Methods: 40 adult patients over the age of 18 who underwent cardiac surgery between 2016 and 2017 participated in our study. All of the participants had baseline renal function that was normal (creatinine 1.2 mg/dL). The patients' baseline biochemistry values and demographics were noted. The link between postoperative AKI and plasma cystatin C level was examined using samples collected at 6 hours and 24 hours. Results: In our study, 12 out of 40 patients were diagnosed with AKI according to the Akut Kidney Injury Network (AKIN) classification and 14 according to the Risk-Injury-Failure-Loss-End stage (RIFLE) classification.It was determined that the duration of hospital stay increased significantly with the development of AKI, the 24th hour Cystatin C level was correlated with the development of AKI (p:0.048) and was significantly higher in the group developing AKI. Presence of baseline diabetes was significantly higher in patients who developed AKI (p:0.045). Plasma Cystatin C 24-hour AUCROC value was calculated as 0.78 (0.75-0.90 Confidence interval, p:0.002). There was no correlation between Cystatin C level measured at 6th hour and AKI (p:0.358). Conclusion: The development of AKI after cardiac surgery is associated with the frequency of adverse clinical events. Cystatin C tested at 6 hours has low sensitivity in early diagnosis, while Cystatin C at 24 hours has a strong diagnostic value in early diagnosis. Postoperative Cystatin C level can make a significant contribution to the diagnosis of AKI (especially at the 24th hour).

АУТОСОМНО-ДОМИНАНТНАЯ ПОЛИКИСТОЗНАЯ БОЛЕЗНЬ ПОЧЕК С ОЧЕНЬ РАННИМ НАЧАЛОМ

Both autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease may occur at any age starting infancy and up to adulthood. Manifestations of autosomal dominant polycystic kidney disease usually occur in adult patients with a risk of development of end-stage renal failure after the fifth decade of life. However, an increase in the number of pediatric cases of a very-early onset of the disease, in utero or during the first 18 months of life, had been registered recently, that were characterized by severe clinical manifestations which in tis turn had mimic the autosomal recessive polycystic kidney disease. The three infant cases of autosomal dominant polycystic kidney disease are observed in the Article with perinatal period features, clinical manifestations of the disease, tests’ results and its interpretation given. Children with very-early onset of autosomal dominant polycystic kidney disease are in the high-risk group for nephromegaly, severe hypertension and the early end-stage renal failure.

Medium-Term Outcomes in Pediatric Patients Undergoing Cardiac Catheterization Early After Congenital Cardiac Surgery.

Safety and acute outcomes for patients who need catheterization shortly after congenital cardiac surgery has been established; literature on mid-term outcomes is lacking. We sought to evaluate the mid-term outcomes of patients who undergo early postoperative cardiac catheterization. This is a retrospective cohort study of patients who underwent cardiac catheterization within 6weeks of congenital cardiac surgery with longitudinal follow-up and assessment of mid-term outcomes. Multivariable analysis was utilized to relate variables of interest to outcomes. 99 patients underwent cardiac catheterizations within 6weeks of cardiac surgery between January 2008 and September 2016. Forty-six (45.5%) interventional procedures were performed at a median age of 41days (IQR 21-192) and a median weight of 3.9kg (3.3-6.6). During a median follow-up duration of 4.24years (1.6-5.6) in study survivors, 61% of patients remained free from the primary endpoint (death and/or transplant). Sixty-nine patients (69.7%) underwent an unplanned surgical or catheter procedure. Renal failure at catheterization (OR 280.5, p 0.0199), inotropic medication at catheterization (OR 14.8, p 0.002), and younger age were all significantly associated with meeting the primary endpoint. Patients requiring surgical intervention as an initial additional intervention underwent more unplanned re-interventions, while patients who survived to hospital discharge demonstrated favorable mortality, though with frequent need for re-intervention. In patients requiring early postoperative cardiac catheterization, renal failure, younger age, and need for inotropic support at catheterization are significantly associated with meeting the primary endpoint.

Use of pre-operative hemoglobin a1c to predict early post-operative renal failure and infection risks in patients who are not diabetics and undergoing elective off pump coronary artery bypass graft surgery.

Recent studies have indicated that patients, both with and without diabetes with an increased HbA1c, have a higher rate of adverse outcomes following cardiac surgeries. Our study is focused on to evaluate the prognostic impact of admission value of HbA1c in non-diabetic patients for postoperative renal failure and infections. Plasma HbA1c levels were collected from 200 consecutive nondiabetic patients who got admitted for elective off pump coronary artery bypass graft (CABG) procedure over a 2-year period under two groups, Group A whose HbA1c was < 6% at admission and Group B whose HbA1c was ≥6% and ≤6.4% at admission. After surgery, patients were followed up to see if they have got infection or renal failure as postoperative complication. Student's unpaired t test was used to test the significance of difference between the quantitative variables, Yate's and Fisher's chi square tests were used for qualitative variables. We found early postoperative renal failure in 14 (3/96 in Group A and 11/104 in Group B) out of 200 patients (7%) and infection in 21 (8/96 in Group A and 13/104 in Group B) out of 200 patients (10.5%). After data analysis, it was noted that there is a positive correlation between HbA1c and postoperative renal failure (P = 0.0213) whereas no association was found between HbA1c and postoperative infections (P = 0.175) in patients undergoing off-pump CABG surgery. In patients without diabetes, a plasma HbA1c ≥6% was a significant independent predictor for early postoperative renal failure.

Is robotic assistance an added value in minimally invasive mitral valve surgery? A meta-analysis from propensity score-matched series.

There is still ongoing debate about the benefits of robotic assistance (R-MVS) in comparison with video assistance (V-MVS) in minimally invasive mitral valve surgery. This study aims to update the current evidence. Three propensity score-matched studies published from 2011 to 2021 were included with a total of 1193 patients operated on from 2005 (R-MVS: 536, V-MVS: 657). Data regarding early mortality, postoperative event, and time-related outcomes were extracted and submitted to a meta-analysis using weighted random-effects modeling. The incidence of early mortality, stroke, renal failure, conversion, atrial fibrillation, and prolonged ventilation were similar, all in the absence of heterogeneity. Reoperation for bleeding (odds ratio [OR]: 0.36, 95% confidence interval [CI] 0.16-0.81, p = 0.01) and the need for blood transfusion (OR: 0.30, 95% CI, 0.20-0.56, p = 0.001) were significantly lower in V-MVS group. Regarding time-related outcomes, there was evidence for important heterogeneity of treatment effect among the studies. Operative times were longer in R-MVS: differences in means were 20.7 min for cross-clamp time (95% CI, 9.07-32.3, p = 0.001), 20.7 min for cardiopulmonary bypass time (95% CI, 2.5-38.9, p = 0.03) and 40.2 min for total operative time (95% CI, 24.5-55.8, p < 0.001). Intensive care unit stay and hospital stay were reported in one study, and longer after R-MVS compared to V-MVS; the differences in means were 0.17 days (p = 0.005) and 0.6 days (p = 0.017), respectively. Total cost of both procedures was reported in an additional dedicated propensity score-matched series including 448 patients; it was 21% higher for R-MVS than for V-MVS. This meta-analysis showed excellent outcomes of both video and robotic techniques with low incidence of morbidity and mortality. However, there is no evidence for an added value of robotic assistance in comparison with video assistance; the drawbacks of mini access are reported higher regardless the induced over cost.

Identification and functional characterization of a novel truncating splicing variant in COL4A5 gene causes X-linked Alport syndrome with astigmatism.

Identification and functional characterization of a novel truncating splicing variant in COL4A5 gene causes X-linked Alport syndrome with astigmatism.

SÍNDROME DE PRUNE BELLY COM FÍSTULA VESICOCUTÂNEA: RELATO DE CASO RARO E REVISÃO DA LITERATURA

Prune Belly syndrome (PBS) is a rare complex malformation that preferentially affects male newborns. It presents with a wide spectrum of alterations that include abdominal muscle deficiency, varying degrees of renal dysplasia and urinary tract obstruction, in addition to bilateral cryptorchidism. In general, it is associated with other developmental anomalies, mainly of cardiac, pulmonary and gastrointestinal origin. Despite numerous advances in the treatment of complex malformations, morbidity and mortality in PBS remains high, due to early renal failure and associated malformations. This is a case report of a male newborn, inserted in a complex social context, born by vaginal delivery, premature, with a gestational age of 36 weeks and 1 day. At the initial assessment in the delivery room, he presented: male external genitalia, bilateral cryptorchidism, flabby and hollowed abdomen, parchment skin suggestive of the absence of abdominal muscles, mild respiratory effort and bradycardia. He received positive pressure ventilation and was referred to the Neonatal Intensive Care Unit (UTIn) where a vesicocutaneous fistula and renal failure were found, despite the vesicostomy, progressing to death at 1 month and 1 day of chronological age. Complex congenital malformations, such as PBS, require individualized assessment so that better postnatal results are achieved. However, the limitation of therapeutic care must be carefully evaluated, considering the great morbidity that accompanies these patients. This article aims to describe a rare case of PBS, detailing the evolution of patients with this malformation.

SÍNDROME DE PRUNE BELLY COM FÍSTULA VESICOCUTÂNEA: RELATO DE CASO RARO E REVISÃO DA LITERATURA

Prune Belly syndrome (PBS) is a rare complex malformation that preferentially affects male newborns. It presents with a wide spectrum of alterations that include abdominal muscle deficiency, varying degrees of renal dysplasia and urinary tract obstruction, in addition to bilateral cryptorchidism. In general, it is associated with other developmental anomalies, mainly of cardiac, pulmonary and gastrointestinal origin. Despite numerous advances in the treatment of complex malformations, morbidity and mortality in PBS remains high, due to early renal failure and associated malformations. This is a case report of a male newborn, inserted in a complex social context, born by vaginal delivery, premature, with a gestational age of 36 weeks and 1 day. At the initial assessment in the delivery room, he presented: male external genitalia, bilateral cryptorchidism, flabby and hollowed abdomen, parchment skin suggestive of the absence of abdominal muscles, mild respiratory effort and bradycardia. He received positive pressure ventilation and was referred to the Neonatal Intensive Care Unit (UTIn) where a vesicocutaneous fistula and renal failure were found, despite the vesicostomy, progressing to death at 1 month and 1 day of chronological age. Complex congenital malformations, such as PBS, require individualized assessment so that better postnatal results are achieved. However, the limitation of therapeutic care must be carefully evaluated, considering the great morbidity that accompanies these patients. This article aims to describe a rare case of PBS, detailing the evolution of patients with this malformation.

Predicting short- and long-term renal function following partial and radical nephrectomy

ObjectivesTo externally validate the previously published Mayo clinic model for the prediction of early (<30 days) postoperative renal failure, which relies solely on preoperative estimated glomerular filtration rate (eGFR) and develop a novel model for the prediction of long-term (>30 days) renal function after partial nephrectomy (PN) and radical nephrectomy (RN), including patient factors and nephrometry scores. Patients and methodsRetrospective, single-center cohort study on patients who underwent PN or RN for a unilateral renal tumor between 2003 and 2019 with a preoperative eGFR of at least 15 ml/min/1.73m2. Early postoperative renal failure was defined as eGFR <15 ml/min/1.73 m2 or receipt of dialysis within 30 days. We determined the area under the receiver operating characteristics curve (AUC) to assess the Mayo clinic model's discriminative power. We used hierarchical linear mixed models with backward selection of candidate variables to develop a prediction model for long-term eGFR following PN and RN, separately. Their predictive ability was quantified using the marginal and conditional R2GLMM and an internal validation. ResultsWe included 421 patients (7,548 eGFR observations) who underwent PN and 271 patients (6,530 eGFR observations) who underwent RN. The Mayo clinic model for prediction of early postoperative renal failure following PN and RN showed an AUC of 0.816 (95% CI 0.718–0.920) and 0.825 (95% CI 0.688–0.962), respectively.In multivariable models, long-term eGFR following PN was associated with age, diabetes, the presence of a solitary kidney, tumor diameter and preoperative eGFR, while long-term eGFR following RN was associated with age, body mass index, RENAL nephrometry score and preoperative eGFR. Marginal and conditional R2GLMM were 0.591 and 0.855 for the PN model, and 0.363 and 0.849 for the RN model, respectively. ConclusionsThe Mayo clinic model for short-term renal failure prediction showed good accuracy on external validation. Our long-term eGFR prediction models depend mostly on host factors as opposed to tumor complexity and can aid in decision-making when considering PN vs. RN.

Improving Treatment Options for Primary Hyperoxaluria.

The primary hyperoxalurias are three rare inborn errors of the glyoxylate metabolism in the liver, which lead to massively increased endogenous oxalate production, thus elevating urinary oxalate excretion and, based on that, recurrent urolithiasis and/or progressive nephrocalcinosis. Frequently, especially in type 1 primary hyperoxaluria, early end-stage renal failure occurs. Treatment possibilities are scare, namely, hyperhydration and alkaline citrate medication. In type 1 primary hyperoxaluria, vitamin B6, though, is helpful in patients with specific missense or mistargeting mutations. In those vitamin B6 responsive, urinary oxalate excretion and concomitantly urinary glycolate is significantly decreased, or even normalized. In patients non-responsive to vitamin B6, RNA interference medication is now available. Lumasiran® is already available on prescription and targets the messenger RNA of glycolate oxidase, thus blocking the conversion of glycolate into glyoxylate, hence decreasing oxalate, but increasing glycolate production. Nedosiran blocks liver-specific lactate dehydrogenase A and thus the final step of oxalate production. Similar to vitamin B6 treatment, where both RNA interference urinary oxalate excretion can be (near) normalized and plasma oxalate decreases, however, urinary and plasma glycolate increases with lumasiran treatment. Future treatment possibilities are on the horizon, for example, substrate reduction therapy with small molecules or gene editing, induced pluripotent stem cell-derived autologous hepatocyte-like cell transplantation, or gene therapy with newly developed vector technologies. This review provides an overview of current and especially new and future treatment options.Supplementary InformationThe online version contains supplementary material available at 10.1007/s40265-022-01735-x.

Predicting Post-Heart Transplant Composite Renal Outcome Risk in Adults: A Machine Learning Decision Tool

Predicting Post-Heart Transplant Composite Renal Outcome Risk in Adults: A Machine Learning Decision Tool

POS-111 I244T MUTATION IN PRIMARY HYPEROXALURIA TYPE 1: A MUTATION WITH ATYPICAL COURSE

Primary hyperoxaluria type 1 (PH1) is a rare genetic disorder characterized by allelic and clinical heterogeneity. It’s characterized by severe urolithiasis, nephrocalcinosis, and early renal failure. We report a unusual course in a patient with primary hyperoxaluria

NPHS2 gene polymorphism aggravates renal damage caused by focal segmental glomerulosclerosis with COL4A3 mutation.

Focal segmental glomerulosclerosis (FSGS), a type of primary glomerular disease, is the leading cause of end-stage renal disease (ESRD). Several studies have revealed that certain single-gene mutations are involved in the pathogenesis of FSGS; however, the main cause of FSGS has not been fully elucidated. Homozygous mutations in the glomerular basement membrane gene can lead to early renal failure, while heterozygous carriers develop renal failure symptoms late. Here, molecular genetic analysis of clinical information collected from clinical reports and medical records was performed. Results revealed that nephrosis 2 (NPHS2) gene polymorphism aggravated renal damage in three FSGS families with heterozygous COL4A3 mutation, leading to early renal failure in index patients. Our findings suggest that COL4A3 and NPHS2 may have a synergistic effect on renal injury caused by FSGS. Further analysis of the glomerular filtration barrier could help assess the cause of kidney damage. Moreover, a detailed analysis of the glomerular basement membrane-related genes and podocyte structural proteins may help us better understand FSGS pathogenesis and provide insights into the prognosis and treatment of hereditary glomerulonephropathy.

Comparative assessment of renal function by cystatin C level in patients with hypertension and extrasystole

Although the tight connection of the cardiovascular system and the kidneys is well known, using of cystatin C (Cys-C) opens new horizons in studying early renal failure stages. The study aimed to compare the functional status of the kidneys in patients with hypertension and extrasystole to the level of Cys-C. 156 patients with stage II hypertension (EH II) were examined. 124 of them had frequent symptomatic extrasystoles (74 – of supraventricular origin and 50 – ventricular), 32 patients had no arrhythmias, and were referred to the comparison group. The control group consisted of 30 healthy people with normal blood pressure (BP). All patients underwent a complete clinical examination, blood pressure measurement, daily blood pressure monitoring, daily electrocardiogram monitoring, echocardiography, and determination of renal function (creatinine, blood electrolytes, serum cystatin C) followed by calculation of glomerular filtration rate (GFR). The level of Cys-C in patients with hypertension was significantly higher (p&lt;0.001) compared with healthy individuals. Among patients with arrhythmias, the highest level of Cys-C was noted in patients with ventricular arrhythmias. The correlation analysis showed that the level of Cys-C was higher in the presence of frequent extrasystoles (namely of ventricular origin), smoking, high blood pressure, increased systolic and pulse blood pressure, the presence of concentric left ventricular hypertrophy, dyslipidemia, increased creatinine level and decreased GFR. All three EH II patient groups had significantly lower GFR (calculated by creatinine level) (p&lt;0.001). The lowest creatinine-based GFR was revealed in patients with ventricular extrasystole. All patients with EH II had significantly lower Cys-C based GFR than the control group (p&lt;0.001). Mean Cys-C-based GFR values in patients with extrasystole were significantly lower than in patients without extrasystole (p&lt;0.03). The analysis of GFR levels depending on the extrasystole origin was provided. The lowest level of GFR was recorded in patients with ventricular extrasystole. It was significantly different from the patients with supraventricular extrasystole (p=0.02). Our findings confirm the opinion of other researchers that Cys-C is an early marker of renal dysfunction in patients with hypertension, which is more sensitive than creatinine. Another finding is that ventricular extrasystole is more hemodynamically and metabolically unfavorable compared to supraventricular based on clinical and prognostic evaluation.

Features of the course of nephropathies in combination with CAKUT-syndrome in young children

Background . Children with CAKUT-syndrome (congenital malformations of the urinary tract) quickly develop an inflammatory process, which, in combination with dysplasia and/or urodynamic disorders, creates a high risk of developing early chronic renal failure. Purpose of the study : to identify the main factors of the formation, features of the clinical course of nephropathies, morphometric parameters and features of the organ blood flow of the kidneys in young children against the background of CAKUT syndrome. Methods . Study design: It carried out a cohort prospective study. The main group included 26 children with pyelonephritis against the background of CAKUT syndrome at the age from zero to 36 months of life, the comparison group consisted of 29 children with non-obstructive pyelonephritis of the same age. It carried out a comparative analysis of antenatal and postnatal anamnesis, clinical, laboratory and instrumental data in the considered groups of children with nephro-urological pathology. Additionally, it researches the morphometric parameters of the kidneys and the Doppler indices of the renal vessels. The research conducted during 2016–2018. Results . In children with a chronic course of nephropathy in combination with CAKUT-syndrome, possible predictors of an early onset of the disease are: anemia, damage to the urinary tract with pathological urinary syndrome during pregnancy and a combination with defects of this system in the woman herself, and the development of placental insufficiency. In the group with CAKUT syndrome, a decrease in renal filtration function (in 88.5% of children) and persistent trace proteinuria (in 65.4% of children) are recorded by the end of the first year of life. According to ultrasound, in addition to signs of obstructive nephropathy, it recorded decreases in the thickness of the parenchyma and a violation of organ hemodynamics with an increase in the resistance index. In the observed group of children, CAKUT syndrome combined in 100% of cases with connective tissue dysplasia syndrome also. Conclusion . Thus, CAKUT syndrome leads to early realization of nephropathies in children with a rapid decline in renal function.

Outcomes of tricuspid valve surgery in patients with functional tricuspid regurgitation.

Functional tricuspid regurgitation (fTR) has been amenable to tricuspid valve repair (TVr), with fewer patients needing tricuspid valve replacement (TVR). We sought to review our experience of tricuspid valve surgery for fTR. A retrospective analysis of adult patients (≥18 years) who underwent primary tricuspid valve surgery for fTR (n = 926; mean age 68.6 ± 12.5 years; 67% females) from January 1993 through June 2018 was conducted. There were 767 (83%) patients who underwent TVr (ring annuloplasty, 67%; purse-string annuloplasty, 33%) and 159 (17%) underwent TVR (bioprosthetic valves, 87%; mechanical valves, 13%). The median follow-up was 8.2 years [95% confidence interval (CI) 7.2-8.9 years]. A greater proportion of patients who underwent TVR had severe right ventricular dysfunction (P < 0.001), severe tricuspid regurgitation (P < 0.001) and congestive heart failure (P = 0.001) while the TVr cohort had a greater proportion with severe mitral valve (MV) regurgitation (P < 0.001) and concomitant cardiac procedures. Early mortality (TVR, 9% vs TVr, 3%; P = 0.004), renal failure (TVR, 10% vs TVr, 5%; P = 0.014) and hospital stay (TVR, 15 ± 15 days vs TVr, 12 ± 11 days; P < 0.001) were greater in TVR patients. The TVR cohort had worse survival [hazard ratio (HR) 1.57; 95% CI 1.23-1.99]. Multivariable analysis identified congestive heart failure (HR 1.37; 95% CI 1.10-1.72), renal failure (HR 1.79; 95% CI 1.14-2.82), previous MV surgery (HR 1.35; 95% CI 1.05-1.72) and TVR (HR 1.36; 95% CI 1.03-1.79) as independent risk factors for late mortality. Tricuspid repair for fTR appears to have better early and late outcomes. Since previous MV surgery and TVR are identified as independent risk factors for late mortality, concomitant TVr at the time of index MV surgery may be considered. Early referral before the onset of advanced heart failure may improve outcomes.

Possible PKHD1 Hot-spot Mutations Related to Early Kidney Function Failure or Hepatofibrosis in Chinese Children with ARPKD: A Retrospective Single Center Cohort Study and Literature Review.

PKHD1 mutations are generally considered to cause autosomal recessive polycystic kidney disease (ARPKD). ARPKD is a rare disorder and one of the most severe conditions leading to end-stage renal disease in childhood. With the biallelic deletion mutation, patients have difficulty in surviving the perinatal period, resulting in perinatal or neonatal death. This study retrospectively analyzed patient characteristics, imaging characteristics, laboratory examinations and family surveys from 7 Chinese children with different PKHD1 gene mutations diagnosed by high-throughput sequencing from January 2014 to February 2018. Of the 7 children, there were 3 males and 4 females. Eight missense mutations, two frameshift mutations, two deletion mutations, and two intronic slicing mutations were identified. Six of the mutations have not previously been identified. In the literature search, we identified a total of 29 Chinese children with PKHD1 mutations. The missense mutation c.2507T>C in exon 24 was found in one patient in our study, and five patients with liver fibrosis but normal renal function were reported in the literature. The missense mutation c.5935G>A in exon 37 was found in two patients in our study and three cases in the literature. Four patients had renal failure at an age as young as 1 year of those five patients with the missense mutation c.5935G>A in exon 37. It was concluded that: (1) Kidney length more than 2-3 SDs above the mean and early-onset hypertension might be associated with PKHD1-associated ARPKD; (2) The more enlarged the kidney size is, the lower the renal function is likely to be; (3) c.5935G>A may be a hot spot that leads to early renal failure in Chinese children with PKHD1 mutations; (4) c.2507T>C may be a hot-spot mutation associated with hepatic lesions in Chinese children with PKHD1.