Congenital Kidney Abnormalities Research Articles

Incidence, Risk Factors, and Outcomes Associated With Recurrent Neonatal Acute Kidney Injury in the AWAKEN Study

The incidence and associated outcomes of recurrent acute kidney injury (rAKI) in neonates remain largely unknown. To determine the incidence, risk factors, and clinical outcomes associated with rAKI in critically ill neonates. This cohort study was a secondary analysis of the multicenter, international Assessment of Worldwide Acute Kidney Injury Epidemiology in Neonates retrospective study. Comparisons were made among neonates with no AKI, a single AKI episode (sAKI), and rAKI. All neonates younger than 14 days who were admitted between January 1 and March 31, 2014, to 24 participating level II to IV neonatal intensive care units and received intravenous fluids for at least 48 hours were considered for inclusion. Neonates with congenital heart disease requiring surgery within the first week of life, lethal chromosomal anomalies, death within 48 hours of admission, or severe congenital kidney abnormalities were excluded. Data were analyzed from May 23, 2022, to December 8, 2023. Recurrent AKI using the neonatal Kidney Disease: Improving Global Outcomes criteria. Determination of each rAKI required a complete return to the baseline serum creatinine level that defined the prior AKI episode. Incidence and risk factors of rAKI and associations of rAKI with length of stay (LOS; ie, birth to hospital discharge) and mortality. The study cohort (n = 2162) included 1233 male neonates (57.0%). Gestational age distribution was less than 29 weeks for 276 neonates (12.8%), 29 to less than 36 weeks for 958 (44.3%), and 36 weeks or older for 928 (42.9%). Of 605 neonates with AKI, 133 (22.0%) developed rAKI with risk factors including younger gestational age, lower birthweight, and higher stage of initial AKI. Infants with rAKI experienced longer median LOS (no AKI, 17 [IQR, 8-34] days; sAKI, 18 [IQR, 9-45] days; rAKI, 60 [IQR, 25-109] days; P < .001). Time-varying Cox proportional hazards regression models suggest rAKI is independently associated with a lower hazard of discharge (adjusted hazard ratio, 0.7 [95% CI, 0.6-0.9]; P = .01) when compared with sAKI, but mortality did not differ between groups (adjusted hazard ratio, 1.4 [95% CI, 0.6-3.0]; P = .44). In this cohort study, neonatal rAKI was independently associated with longer LOS when compared with sAKI, suggesting that rAKI in neonates may be an important clinical distinction warranting further study and careful monitoring after an initial AKI episode.

Receipt of high-frequency ventilation is associated with acute kidney injury in very preterm neonates.

High-frequency ventilation (HFV) is frequently used in critically ill preterm neonates. We aimed to determine the incidence of acute kidney injury (AKI) in neonates less than 29weeks gestation who received HFV in the first week of life and to determine if the rates of AKI differed in those who received other forms of respiratory support. This retrospective cohort study of 24 international, level III/IV neonatal intensive care units (NICUs) included neonates less than 29weeks gestation from the AWAKEN study database. Exclusion criteria included the following: no intravenous fluids ≥ 48h, admission ≥ 14days of life, congenital heart disease requiring surgical repair at < 7days of life, lethal chromosomal anomaly, death within 48h, severe congenital kidney abnormalities, inability to determine AKI status, insufficient data on ventilation, and when the diagnosis of early AKI was unable to be made. Subjects were grouped into three groups based on ventilation modes (CPAP/no ventilation, conventional ventilation, and HFV). The incidence of AKI was highest in the CPAP/no ventilation group, followed by HFV, followed by conventional ventilation (CPAP/no ventilation 48.5% vs. HFV 42.6% vs. conventional ventilation 28.4% (p = 0.009). An increased risk for AKI was found for those on HFV compared to CPAP/no ventilation (HR = 2.65; 95% CI:1.22-5.73). HFV is associated with AKI in the first week of life. Neonates on HFV should be screened for AKI. The reasons for this association are not clear. Further studies should evaluate the relationship between ventilator strategies and AKI in premature neonates. A higher resolution version of the Graphical abstract is available as Supplementary information.

Effects of Lockdown for COVID-19 Pandemic on Chronic Kidney Disease Progression in Children with Congenital Anomalies of the Kidney and Urinary Tract: A Retrospective Pilot Study.

The coronavirus disease 2019 (COVID-19) pandemic changed adults and children's lifestyle. We focused our attention on children affected by chronic kidney disease (CKD) due to congenital abnormalities of kidney and urinary tract (CAKUT) and their behavior during the lockdown. Our aims were to evaluate the incidence of CKD progression within 6 months after the end of the first Italian lockdown and the factors associated to it. CKD progression was defined by the transition to higher CKD stage or by the drop in estimated glomerular filtration rate by a 25% or more for patients belonging to CKD stages 1 and 2. We retrospectively selected 21 children with CAKUT and CKD ≥ stage 1 observed within 3 months before and 6 months after the first Italian lockdown. We called them by phone and asked them about their lifestyle before and during lockdown focusing on physical activity, screen time, sweet/candies/sugar-sweetened beverages eaten/drunk and adherence to the Mediterranean diet (MD) (through KIDMED questionnaire). We calculated and analyzed the delta between the pre- and post- lockdown observation of all collected parameters (clinical and biochemical parameters and questionnaires scores). Analyzing the overall cohort, we found significantly increased mean BMI and mean screen time and significantly lower mean physical activity time in post- compared with pre-lockdown observations. Eleven out of twenty-one patients (52.4%) had a worsening of CKD. These patients presented higher delta of levels of uric acid and microalbuminuria and showed minor adherence to the MD and declared to have consumed more sweets or candies or sugar-sweetened beverages/week during the lockdown with a tendentially major increment of BMI compared with patients not presenting CKD progression. In conclusion, the lockdown for COVID-19 pandemic determined increase of BMI in all enrolled patients due to a "forced" negative lifestyle. About half of these patients presented CKD progression. This progression was associated to less adherence to the MD and major consumption of sweets or candies or sugar-sweetened beverages.

Evaluation of urinary acidification in children: Clinical utility.

The kidney plays a fundamental role in acid-base homeostasis by reabsorbing the filtered bicarbonate and by generating new bicarbonate, to replace that consumed in the buffering of non-volatile acids, a process that leads to the acidification of urine and the excretion of ammonium (NH4 +). Therefore, urine pH (UpH) and urinary NH4 + (UNH4 +) are valuable parameters to assess urinary acidification. The adaptation of automated plasma NH4 + quantification methods to measure UNH4 + has proven to be an accurate and feasible technique, with diverse potential indications in clinical practice. Recently, reference values for spot urine NH4 +/creatinine ratio in children have been published. UpH and UNH4 +, aside from their classical application in the study of metabolic acidosis, have shown to be useful in the identification of incomplete distal renal tubular acidosis (dRTA), an acidification disorder, without overt metabolic acidosis, extensively described in adults, and barely known in children, in whom it has been found to be associated to hypocitraturia, congenital kidney abnormalities and growth impairment. In addition, a low UNH4 + in chronic kidney disease (CKD) is a risk factor for glomerular filtration decay and mortality in adults, even in the absence of overt metabolic acidosis. We here emphasize on the need of measuring UpH and UNH4 + in pediatric population, establishing reference values, as well as exploring their application in metabolic acidosis, CKD and disorders associated with incomplete dRTA, including growth retardation of unknown cause.

MO348: Methylene Blue Beneficial Effect in Treatment of Renal Calculi in Patient With Recurrent Urinary Tract Infection

Abstract BACKGROUND AND AIMS Assessment of efficacy of fixed combination of cranberry and sage extract with methylene blue (MBC) in dose of 30 mg twice daily in the prevention and management of chronic renal calculous disease. METHOD In 87 patients who complained recurrent urinary tract infection (UTI)—55 pts with pyelonephritis and 32 with cystitis—at least three episodes in 12 months, the impact of 3 months treatment of MBC with on renal calculi was investigated. All patients were older than 18 years without any biochemical or congenital kidney abnormalities, coagulopathies with size of renal stone over 10 mm. Simultaneous administration of phenothiazine derivatives, SSRI, MAO, tricyclic or any other antidepressants we excluded. A total of 39 (45%) of the patients with a history of nephrolithotomy in the past were included. RESULTS In most cases, patients were supervised during 12 months with 2–3 courses of MBC during the year. A total of 18 pts (21%) have passed no further stones with dramatic improvement of quality of life, and 47 (54%) have improved clinically with reduction of calculi size. A total of 79 pts (91%) reported overall clinical improvement of UTI symptoms and quality of life. The main disadvantage during MBC administration could be discoloration of the urine in light blue or green color. No side or adverse effects were observed during the whole period of the trial. CONCLUSION Methylene blue could be a promising solution in the management of patients with multiple small renal calculi, especially with necessity to reduce the level of concomitant infection without antibiotic and may be useful in the prevention of new stone formation.

Congenital Abnormalities of Kidneys and Urinary Tract in Children at the Dakar University Hospital

Congenital abnormalities of the kidneys and urinary tract (CAKUT) represent all the morphological abnormalities associated with a disorder of the embryonic development of the kidneys and their excretory tract. Its prevalence is underestimated in developing countries compared to developed countries. This was a cross-sectional and descriptive study between January 1st, 2015 and February 28th, 2020, carried out in the pediatric department of Aristide Le Dantec Hospital. The prevalence of CAKUT was 8.77% for all pediatric nephrologic pathologies (n = 58), the median age at diagnosis was 77.28 months and the sex ratio was 1.6. The circumstances of the discovery of CAKUT were dominated by urinary tract infection (n = 21), the antenatal diagnosis was poor (n = 13) and confusion between cysts and calyx dilation in antenatal was noted. Ultrasound was the main postnatal imaging test requested. The most common renal abnormality was kidney cysts (n = 18) (cystic kidneys and multicystic dysplastic kidney) and the most common urological abnormality was the posterior urethral valves (n = 8). Kidney failure was a pejorative factor in the evolution of these CAKUT.

Laurence-Moon-Bardet-Biedl syndrome

Lawrence-Moon-Bardet-Biedl syndrome is a rare autosomal recessive genetic disorder, which may result in a number of multiorgan abnormalities, including impaired brain function, eye diseases, kidney and limbs’ dysfunction. The main symptoms of this syndrome include retinal degeneration, polydactyly, obesity, hypogonadism, congenital kidney abnormalities and mental retardation. However, Lawrence-­Moon­-Barde­-Biedl syndrome may also present with other secondary abnormalities, including ataxia, diabetes insipidus, and dental abnormalities. Clinical changes of the eyes include retinitis pigmentosa, low visual acuity, and vision loss, often due to photoreceptor disorders in the retinal tissue with macular degeneration, leading to night blindness and then, in most cases, can cause complete blindness. In patients with an archetypal manifestation of Lawrence­-Moon­-Barde-­Biedl syndrome, abdominal obesity is common, even if the birth weight is usually normal. In addition, this group of patients has type 2 diabetes mellitus. A distinctive feature of this syndrome is postaxial polydactyly. Hypogonadism, which is a common sign of the disease, as usual can be diagnosed at early age in men in a form of micropenis and testicular hypoplasia).The paper presents clinicalcase of Lawrence­-Moon­-Barde-­Bidle syndrome in a thirteen-year-old boy who referred to endocrinologist with complaints of excessivegain of body weight, memoryloss, visual impairment, difficulties in school, delayedsexual development. Ad ditional investigations enabled to establish the diagnosis of Laurence­-Moon­-Bardet­-Biedl syndrome.

Associated Etiologies of Enuresis in 5-to-15-Year-Old-Children

Background: Enuresis is one of the most common problems in children with physical and psychological complications that can disturb their social relationships. The main objective of this study is to evaluate the relationship between enuresis and its associated etiologies in children aged 5 to 15 years in Zahedan. The study further contributes to an early realization of the parents of children with enuresis-associated problems. Methods: Following a descriptive design, 200 children (5 to 15 years old) suffering from enuresis referred to nephrology and psychiatric clinics in Zahedan in 2019 were recruited. Data were analyzed using SPSS software. Results: Of 200 children with enuresis, 134 (67%) were girls, and 66 (33%) were boys. Also, 174 (87%) were aged 5 - 10 years, and only 26 (13%) were 10 to 15 years old. Birth weight of 172 (86%) children was 2500 - 4000 g, 20 (10%) were &lt; 2500g, and only 8 (4%) were &gt; 4000 g. Fecal incontinence was observed in 99 cases (49%), and 101 (51%) did not have fecal incontinence. Attention deficit hyperactivity disorder (ADHD) was found in 80 (40%) cases. For 144 (72%) children, we found no stress, and 56 children (28%) had stress. Urinary tract infection was observed in 142 children (71%). According to imaging studies, 34 children (17%) with enuresis had congenital kidney abnormalities and 166 (83%) had no kidney problems. Conclusions: The significant etiologies typically found in the onset of enuresis and its exacerbation can be controlled or treated. Since the complications of enuresis have not been properly clarified for families, it is of crucial importance to increase awareness about this health problem.

Hepatocyte nuclear factor 1β suppresses canonical Wnt signaling through transcriptional repression of lymphoid enhancer–binding factor 1

Hepatocyte nuclear factor-1β (HNF-1β) is a tissue-specific transcription factor that is required for normal kidney development and renal epithelial differentiation. Mutations of HNF-1β produce congenital kidney abnormalities and inherited renal tubulopathies. Here, we show that ablation of HNF-1β in mIMCD3 renal epithelial cells results in activation of β-catenin and increased expression of lymphoid enhancer-binding factor 1 (LEF1), a downstream effector in the canonical Wnt signaling pathway. Increased expression and nuclear localization of LEF1 are also observed in cystic kidneys from Hnf1b mutant mice. Expression of dominant-negative mutant HNF-1β in mIMCD3 cells produces hyperresponsiveness to exogenous Wnt ligands, which is inhibited by siRNA-mediated knockdown of Lef1. WT HNF-1β binds to two evolutionarily conserved sites located 94 and 30 kb from the mouse Lef1 promoter. Ablation of HNF-1β decreases H3K27 trimethylation repressive marks and increases β-catenin occupancy at a site 4 kb upstream to Lef1. Mechanistically, WT HNF-1β recruits the polycomb-repressive complex 2 that catalyzes H3K27 trimethylation. Deletion of the β-catenin-binding domain of LEF1 in HNF-1β-deficient cells abolishes the increase in Lef1 transcription and decreases the expression of downstream Wnt target genes. The canonical Wnt target gene, Axin2, is also a direct transcriptional target of HNF-1β through binding to negative regulatory elements in the gene promoter. These findings demonstrate that HNF-1β regulates canonical Wnt target genes through long-range effects on histone methylation at Wnt enhancers and reveal a new mode of active transcriptional repression by HNF-1β.

Acute kidney injury in children with chronic kidney disease is associated with faster decline in kidney function

BackgroundThis study aimed to investigate the association of acute kidney injury (AKI) with change in estimated glomerular filtration rate (eGFR) in children with advanced chronic kidney disease (CKD).MethodsSingle centre, retrospective longitudinal study including all prevalent children aged 1–18 years with nondialysis CKD stages 3–5. Variables associated with CKD were analysed for their potential effect on annualised eGFR change (ΔGFR/year) following multiple regression analysis. Composite end-point including 25% reduction in eGFR or progression to kidney replacement therapy was evaluated.ResultsOf 147 children, 116 had at least 1-year follow-up in a dedicated CKD clinic with mean age 7.3 ± 4.9 years with 91 (78.4%) and 77 (66.4%) with 2- and 3-year follow-up respectively. Mean eGFR at baseline was 29.8 ± 11.9 ml/min/1.73 m2 with 79 (68%) boys and 82 (71%) with congenital abnormalities of kidneys and urinary tract (CAKUT). Thirty-nine (33.6%) had at least one episode of AKI. Mean ΔGFR/year for all patients was − 1.08 ± 5.64 ml/min/1.73 m2 but reduced significantly from 2.03 ± 5.82 to − 3.99 ± 5.78 ml/min/1.73 m2 from youngest to oldest age tertiles (P < 0.001). There was a significant difference in primary kidney disease (PKD) (77% versus 59%, with CAKUT, P = 0.048) but no difference in AKI incidence (37% versus 31%, P = 0.85) between age tertiles. Multiple regression analysis identified age (β = − 0.53, P < 0.001) and AKI (β = − 3.2, P = 0.001) as independent predictors of ΔGFR/year. 48.7% versus 22.1% with and without AKI reached composite end-point (P = 0.01).ConclusionsWe report AKI in established CKD as a predictor of accelerated kidney disease progression and highlight this as an additional modifiable risk factor to reduce progression of kidney dysfunction.Graphical abstract

Incomplete distal renal tubular acidosis in children.

To describe incomplete distal renal tubular acidosis (iDRTA) in paediatric patients, a term used for the diagnosis of patients who do not develop spontaneous overt metabolic acidosis but are unable to acidify the urine in response to an ammonium chloride load. Tests used to explore urinary acidification were revised. In addition, publications in English extracted from 161 entries yielded by a PubMed database search, using 'incomplete distal renal tubular acidosis' as keyword, were reviewed. Incomplete distal renal tubular acidosis has mostly been identified in adults with autoimmune diseases, nephrolithiasis, nephrocalcinosis and/or osteopenia. iDRTA has been reported in few paediatric patients with rickets, congenital abnormalities of kidney and urological tract and/or growth failure. The pathophysiological mechanisms potentially responsible for the defect of urinary acidification are discussed as well as the clinical and biochemical findings of iDRTA described in children. The presentation of iDRTA in children differs from adults. The clinical and biochemical features of iDRTA are not well characterised in paediatric patients. The detection of iDRTA in groups of population such as heterozygous carriers of primary DRTA gene mutations and children with hypocitraturia or hypercalciuria might be of clinical interest to better know the pathophysiology and natural history of iDRTA.

Significance of third trimester ultrasound in detecting congenital abnormalities of kidney and urinary tract—a prospective study

Antenatal hydronephrosis is defined as dilatation of the renal pelvis with or without dilatation of calyces in the developing fetus. Although second trimester scan is more sensitive in detecting congenital abnormalities of kidney and urinary tract (CAKUT), it has been suggested that the third trimester scan is more predictive of postnatal outcome. This study aims to determine the incidence of antenatal hydronephrosis at our institution and the significance of third trimester scan in detecting CAKUT postnatally. It is a prospective observational study. During the 3-year study period from June 2013 to May 2016, all cases of antenatal hydronephrosis, defined as renal pelvic diameter (RPD) ≥4mm in the second trimester and ≥7mm in the third trimester scan, diagnosed in the fetal medicine unit of our hospital, were included and were followed up postnatally for 6 months. In the fetal medicine unit, 32,443 women were screened for anomalies, and hydronephrosis was detected in 269 cases. Incidence of antenatal hydronephrosis was observed to be 0.83% in our institution. In second trimester scan, of 80 cases with the left hydronephrosis, only 22 (27.5%) had postnatal CAKUT and of 70 cases with the right hydronephrosis, 18 (25.7%) had CAKUT. In the third trimester scan, it was observed that with RPD >10mm, on the left side hydronephrosis, 87% had CAKUT on postnatal scan, and 85% with the right hydronephrosis had CAKUT. Sixty percent cases of hydronephrosis detected in the second trimester scan resolve in utero. In the third trimester scan with RPD <10mm, the possibility of postnatal CAKUT is 23%, whereas if RPD is>10mm, then the possibility of postnatal CAKUT is 86%.

Whole Genome Sequencing of a Rat Model of Congenital Abnormalities of Kidney and Urinary Tract

The HSRA rat is a model of congenital abnormalities of the kidney and urogenital tract (CAKUT), with offspring exhibiting renal agenesis and other urogenital abnormalities. Our laboratory has used this model to investigate the role of nephron number in susceptibility to develop chronic kidney disease as offspring are born with a single kidney (HSRA‐S; 50%–75%) or with two‐kidneys (HSRA‐C, 25–50%). The model provides a unique advantage by allowing to directly compare congenital one‐kidney animals (~20,400 nephrons, less than comparable kidney), nephrectomized two‐kidney animals (HSRA‐UNX, ~25,100 nephrons) and two‐kidney littermates (~50,000 nephrons) in the same model, a comparison not possible in models that utilize nephrectomy. HSRA‐S rats are highly susceptible to the hypertension induced kidney injury (DOCA/1% NaCl) compared to HSRA‐UNX, suggesting that even slight nephron differences can be an important driver of elevated blood pressure, hastening kidney injury and accelerating decline in kidney function. The HSRA rat was selected and inbred (&gt;20 generation) from a single pair of outbred heterogeneous stock (HS) rats that exhibited a high incidence of CAKUT (~50% versus 1% in HS population). The HS model was originally developed from 8 inbred strains (ACI, BN, BUF, F344, M520, MR, WKY, and WN). Thus, the genetic make‐up of the HSRA model is a mosaic of these 8 inbred strains. Interestingly, the ACI progenitor of the HS model exhibits CAKUT in 10–15% of offspring with the genetic cause being attributed to presence of a long‐term repeat (LTR) within exon 1 of c‐Kit gene. Our hypothesis is that the HSRA and ACI share this common genetic cause, but other genes in the HSRA genome contribute to increase penetrance of CAKUT (75% vs 15% in ACI). To facilitate genetic studies and better characterize the model, the whole genome of the HSRA was sequenced to a depth of 50X using the Illumina NextSeq500 platform. Sequence alignment and annotation [BN reference genome (UCSC Rn5)] identified 4,692,623 SNPs and 1,438,131 INDELs. Sequence analysis of fifteen recessive and 25 dominant genes linked to CAKUT in humans identified three genes (Frem2, Hnf1b, and Six5) predicted to have high‐impact variation that could play a role in the observed CAKUT. Future studies will involve performing a linkage analysis using a segregating population [F2(HSRA x ACI)] to map the QTL/location of modifier genes, which in combination with genome sequence and annotation data will include/exclude known genes as well as identify additional candidate genes linked to CAKUT. In summary, the identification of specific gene defects, modifier genes, and signaling pathways that lead to loss of one kidney and nephron deficiency will provide a mechanistic link between the solitary kidney genotype and the development of kidney dysfunction exhibited by the HSRA rat.Support or Funding InformationThe whole genome sequencing performed through the UMMC Molecular and Genomics Facility is supported, in part, by funds from the NIGMS, including P20GM103476, P20GM104357, and P20GM121334.This abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Incidence and outcomes of neonatal acute kidney injury (AWAKEN): a multicentre, multinational, observational cohort study

BackgroundSingle-center studies suggest that neonatal acute kidney injury (AKI) is associated with poor outcomes. However, inferences regarding the association between AKI, mortality, and hospital length of stay are limited due to the small sample size of those studies. In order to determine whether neonatal AKI is independently associated with increased mortality and longer hospital stay, we analyzed the Assessment of Worldwide Acute Kidney Epidemiology in Neonates (AWAKEN) database.MethodsAll neonates admitted to 24 participating neonatal intensive care units from four countries (Australia, Canada, India, United States) between January 1 and March 31, 2014, were screened. Of 4273 neonates screened, 2022 (47·3%) met study criteria. Exclusion criteria included: no intravenous fluids ≥48 hours, admission ≥14 days of life, congenital heart disease requiring surgical repair at <7 days of life, lethal chromosomal anomaly, death within 48 hours, inability to determine AKI status or severe congenital kidney abnormalities. AKI was defined using a standardized definition —i.e., serum creatinine rise of ≥0.3 mg/dL (26.5 mcmol/L) or ≥50% from previous lowest value, and/or if urine output was <1 mL/kg/h on postnatal days 2 to 7.FindingsIncidence of AKI was 605/2022 (29·9%). Rates varied by gestational age groups (i.e., ≥22 to <29 weeks =47·9%; ≥29 to <36 weeks =18·3%; and ≥36 weeks =36·7%). Even after adjusting for multiple potential confounding factors, infants with AKI had higher mortality compared to those without AKI [(59/605 (9·7%) vs. 20/1417 (1·4%); p< 0.001; adjusted OR=4·6 (95% CI=2·5–8·3); p=<0·0001], and longer hospital stay [adjusted parameter estimate 8·8 days (95% CI=6·1–11·5); p<0·0001].InterpretationNeonatal AKI is a common and independent risk factor for mortality and longer hospital stay. These data suggest that neonates may be impacted by AKI in a manner similar to pediatric and adult patients.FundingUS National Institutes of Health, University of Alabama at Birmingham, Cincinnati Children’s, University of New Mexico.

Review of three Common Congenital Abnormalities of Kidney and their Clinical Perspective with an Elaboration on a rare case of Co-Occurrence of Horse Shoe Kidney , Unilateral Duplex Kidney And Aberrant Renal Vessel With Pelviureteric Junction Obstruction Of The Lower Moiety

Review of three Common Congenital Abnormalities of Kidney and their Clinical Perspective with an Elaboration on a rare case of Co-Occurrence of Horse Shoe Kidney , Unilateral Duplex Kidney And Aberrant Renal Vessel With Pelviureteric Junction Obstruction Of The Lower Moiety

Urinary tract infection in children after cardiac surgery: Incidence, causes, risk factors and outcomes in a single-center study.

Nosocomial urinary tract infection (UTI) increases hospitalization, cost and morbidity. In this cohort study, we aimed to determine the incidence, risk factors, etiology and outcomes of UTIs in post-operative cardiac children. To this end, we studied all post-operative patients admitted to the Pediatric Cardiac Intensive Care Unit (PCICU) in 2012, and we divided the patients into two groups: the UTI (UTI group) and the non-UTI (control group). We compared both groups for multiple peri-operative risk factors. We included 413 children in this study. Of these, 29 (7%) had UTIs after cardiac surgery (UTI group), and 384 (93%) were free from UTIs (control group). All UTI cases were catheter-associated UTIs (CAUTIs). A total of 1578 urinary catheter days were assessed in this study, with a CAUTI density rate of 18 per 1000 catheter days. Multivariate logistic regression analysis demonstrated the following risk factors for CAUTI development: duration of urinary catheter placement (p<0.001), presence of congenital abnormalities of kidney and urinary tract (CAKUT) (p<0.0041) and the presence of certain syndromes (Down, William, and Noonan) (p<0.02). Gram-negative bacteria accounted for 63% of the CAUTI. The main causes of CAUTI were Klebsiella (27%), Candida (24%) and Escherichia coli (21%). Resistant organisms caused 34% of CAUTI. Two patients (7%) died in the UTI group compared with the one patient (0.3%) who died in the control group (p<0.05). Based on these findings, we concluded that an increased duration of the urinary catheter, the presence of CAKUT, and the presence of syndromes comprised the main risk factors for CAUTI. Gram-negative organisms were the main causes for CAUTI, and one-third of them found to be resistant in this single-center study.

Chronic kidney disease in children and adolescents in Brunei Darussalam.

To determine epidemiology of Bruneian paediatric chronic kidney disease (CKD) patients and factors that affect growth and progression of disease. A cross-sectional study conducted on all children below 18 years old who were diagnosed with CKD over a ten year period (2004 to 2013). The reference population was all children (< 18 years old) suffering from CKD and attending the tertiary paediatric nephrology clinic in Brunei Darussalam. Demographic (current age, age of diagnosis, gender, ethnicity), anthropometric (weight and height), diagnosis, laboratory data (serum creatinine and haemoglobin, urinalysis) and blood pressure were extracted from the patients' clinical case notes and recorded using a data collection form. The study revealed a high national prevalence [736 per million child population (pmcp)] and incidence (91 pcmp) of CKD. If CKD was defined at Stage 1, 2, 3, 4 or 5, the associated prevalence figures were 736, 132, 83, 50 and 33 pmcp. Glomerulonephritis accounted for 69% of all prevalent cases, followed by congenital abnormalities of kidney and urinary tract (20%) and tubulointerstitial diseases (8%). Minimal change disease being the most common histological diagnosis. The median age of diagnosis was 4.5 years, with congenital disease patients experiencing an earlier onset of diagnosis. A large proportion of patients were below the 5% percentile for height and weight. Non-glomerular diseases, adolescent and female patients were significantly associated with poor growth, but not glomerular filtration rate, age of diagnosis or steroid usage. Brunei has a high prevalence of chronic kidney disease in the paediatric population with glomerulonephritis being the most common disease.

Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract.

Congenital abnormalities of the kidney and urinary tract (CAKUT) include a wide range of abnormalities ranging from asymptomatic ectopic kidneys to life threatening renal agenesis (bilateral). Many of them are detected in the antenatal or immediate postnatal with a significant proportion identified in the adult population with varying degree of severity. CAKUT can be classified on embryological basis in to abnormalities in the renal parenchymal development, aberrant embryonic migration and abnormalities of the collecting system. Renal parenchymal abnormalities include multi cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), shape and cystic renal diseases. Aberrant embryonic migration encompasses abnormal location and fusion anomalies. Collecting system abnormalities include duplex kidneys and Pelvi ureteric junction obstruction. Ultrasonography (US) is typically the first imaging performed as it is easily available, non-invasive and radiation free used both antenatally and postnatally. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful to confirm the ultrasound detected abnormality, detection of complex malformations, demonstration of collecting system and vascular anatomy and more importantly for early detection of complications like renal calculi, infection and malignancies. As CAKUT are one of the leading causes of end stage renal disease, it is important for the radiologists to be familiar with the varying imaging appearances of CAKUT on US, CT and MRI, thereby helping in prompt diagnosis and optimal management.

Congenital anomalies of the kidney and urinary tract. A vision for the paediatrician

The congenital abnormalities of kidney and urinary tract (CAKUT) are disorders with a high prevalence in the general population, with urinary tract dilations being the most frequent. CAKUT also account for the most important cause of chronic kidney disease in childhood. This paper focuses on the role of the primary care paediatrician in the diagnosis, assessment, and follow-up of children with CAKUT, with special emphasis on the associated urinary tract infections, the progression toward chronic renal failure, and the genetic basis.

Anomalías nefrourológicas congénitas. Una visión para el pediatra

The congenital abnormalities of kidney and urinary tract (CAKUT) are disorders with a high prevalence in the general population, with urinary tract dilations being the most frequent. CAKUT also account for the most important cause of chronic kidney disease in childhood.This paper focuses on the role of the primary care paediatrician in the diagnosis, assessment, and follow-up of children with CAKUT, with special emphasis on the associated urinary tract infections, the progression toward chronic renal failure, and the genetic basis.