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Intradialytic hypotension (IDH) is a common adverse event resulting in premature interruption of hemodialysis, and consequently, inadequate fluid and solute removal. IDH occurs in response to the reduction in blood volume during ultrafiltration and subsequent poor compensatory mechanisms due to abnormal cardiac function or autonomic or baroreceptor failure. Pediatric patients are inherently at risk for IDH due to the added difficulty of determining and attaining an accurate dry weight. While frequent blood pressure monitoring, dialysate sodium profiling, ultrafiltration-guided blood volume monitoring, dialysate cooling, hemodiafiltration, and intradialytic mannitol and midodrine have been used to prevent IDH, they have not been extensively studied in pediatric population. Lack of large-scale studies on IDH in children makes it difficult to develop evidence-based management guidelines. Here, we aim to review IDH preventative strategies in the pediatric population and outlay recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup. Without strong evidence in the literature, our recommendations from the expert panel reflect expert opinion and serve as a valuable guide. [ABSTRACT FROM AUTHOR]

Hemodialysis (HD) in neonates and infants poses unique challenges due to high risks of mortality attributable to obligatory small blood flow volumes. Although HD is often necessary in neonates, its effectiveness and feasibility are poorly understood. The aim of this review is to describe in detail the few studies reporting on HD in neonates and infants (<12 months old) and then dissertate more broadly on the subject with an emphasis on recent innovations with potential to overcome traditional barriers for effective HD in this population. We detail the clinical characteristics, outcomes, technical considerations, maintenance and complications associated with HD, and provide guidance for addressing challenges associated with HD in this population. [ABSTRACT FROM AUTHOR]

Abstract: Atypical hemolytic uremic syndrome (aHUS) is a rare life‐threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta‐analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations. A PubMed/Medline search was conducted for “atypical hemolytic uremic syndrome” case reports published between November 2005 and November 2015. R Version 3.2.2 was used to calculate descriptive statistics and perform univariate analyses. Wilcoxon rank‐sum test was used to compare time to symptoms resolution, creatinine and platelet count normalization across the treatment and mutation carrier groups. A total of 259 aHUS patients were reported in 176 articles between 2005 and 2015. In the last 5‐year period compared to the precedent, there was an increase in the number of aHUS cases reported (180 vs. 79 cases) and the use of eculizumab also increased (6.3% to 46.1%, P < 0.000), although plasma exchange usage did not change (P = 0.281). CFH antibodies were present in a significantly higher number of patients treated with plasma exchange therapy (19.1%, P = 0.000) while none of the non‐plasma exchange therapy group had CFH antibodies. Most common mutation was CFH (50%, 69/139) followed by CFHR1 (35%, 30/85), MCP (22.8%, 23/101) and CFI (16.6%, 17/102). Time to symptoms resolution and serum creatinine or platelet count normalization were not significantly different between eculizumab and non‐eculizumab group (P = 0.166, P = 0.361, P = 0.834), and between plasma exchange and non‐plasma exchange group (P = 0.150, P = 0.135, P = 0.784). However, both eculizumab and plasma exchange groups had early platelet recovery (22 vs. 30 days and 25.5 vs. 32.5 days), faster creatinine normalization (27 vs. 30.5 days and 27 vs. 37 days) and interestingly, a longer period for symptoms resolution (45.5 vs. 21 days and 30 vs. 18.5 days) compared to non‐eculizumab and non‐plasma exchange groups. Mortality rate decreased with the use of eculizumab significantly (P = 0.045) compared to non‐eculizumab group and there was no change in mortality rate with the use of plasma exchange therapy (P = 0.760) compared to non‐plasma exchange group. Plasma exchange continues to be the initial treatment of choice for aHUS. Although significant reduction in the mortality rate was noted with the use of eculizumab, there were no differences in time to resolution of symptoms or serum creatinine or platelet normalization with the use of either eculizumab or plasma therapy. Atypical HUS is acute and life‐threatening, so plasma exchange may be initiated before the confirmed diagnosis and in patients positive for CFH antibodies. Eculizumab therapy should be considered once aHUS is confirmed by genetic testing. [ABSTRACT FROM AUTHOR]

Background: Bartter's syndrome is a rare autosomal recessive renal tubular disorder characterized by hypokalemia, hypochloremia, metabolic alkalosis, hyperreninemia and hyperaldosteronemia with normotension. Bartter syndrome has five types; type 1 (mutation in sodium/potassium chloride transporter), type 2 (mutation in voltage gated potassium channel), type 3 (mutation on chromosome 1 that encodes Barttin and makes only kidney-specific chloride channel B non-functional), type 4 (mutation in BSND gene encoding Barttin and makes both kidney-specific chloride channels A & B non-functional) and type 5 (L125P gain in function mutation in calcium-sensing receptor). Case Presentation: A 28-year-old male was hospitalized for evaluation of nausea, vomiting, generalized weakness and persistent chronic hypokalemia. Bartter's syndrome was suspected based on clinical and laboratory evidence, however serum aldosterone level was normal. Further genetic testing confirmed the diagnosis of Bartter's syndrome type 3. Conclusions: We report a case of Bartter's syndrome type 3 with a unique finding of normal aldosterone level. [ABSTRACT FROM AUTHOR]

A clinical quiz about two siblings who were presented with complement activation, thrombotic microangiopathy and gangrene is presented.

The article presents a case study of a 07-year-old Indian boy with nephroticrange proteinuria without oedema. Topics include graft being lost and the father now back on dialysis awaiting a second kidney transplant; and peripheral smear showing numerous monomorphic giant platelets with the inset showing neutrophils with abnormal cytoplasmic inclusions.

A clinical quiz is presented regarding a rare complication of pauci-immune crescentic glomerulonephritis in a six-year-old female born of non-consanguineous marriage with no previous comorbidities.

Background: Neonates and infants undergoing heart surgery on cardiopulmonary bypass (CPB) are at high risk for significant post-operative morbidity and mortality. Hence, there is a need to identify and quantify clinical factors during the early post-operative period that are indicative of short-term as well as long-term outcomes. Multiple inotrope scores have been used in practice to quantify the amount of cardiovascular support received by neonates. Aims: The goal of this study was to determine the association between inotropic/vasoactive support and clinical outcomes in children after open cardiac surgery. Materials and Methods: This is a retrospective analysis of the 208 patients who underwent cardiac surgery for congenital heart disease at a tertiary pediatric cardiac surgery Intensive Care Unit (ICU) from January 2012 to March 2013. Multiple demographic, intra-operative and post-operative variables were recorded, including the Vasoactive Inotrope Score (VIS). Results: A total of 208 patients underwent cardiac surgery for congenital heart disease in the study period. The mean age and weight in the study were 66.94 months and 16.31 kg, respectively. Statistically significant associations were found in the various variables and VIS, including infancy, weight < 10 kg, CPB time, pump failure and post-operative variables like sepsis, hematological complications, hepatic dysfunction, acute kidney injury during admission, mortality, prolonged ventilator requirement, CPB time (in min) and hospital stay. Conclusions: Inotrope score and its adaptations are an excellent tool to measure illness severity, deciding interventions and during parental counseling in the pediatric cardiac surgery ICUs. [ABSTRACT FROM AUTHOR]

The article discusses the clinical manifestations, pathogenesis, diagnosis, prevention and treatment for the pandemic H1N1 influenza in pregnant women. Topics mentioned include the increased risk in pregnancy during seasonal influenza, increased risk on mortality and morbidity with complications including preterm birth and spontaneous miscarriage, and the need for obstetricians and health care professionals to be familiar with symptoms and treatment.

Acute kidney injury (AKI) has a significant impact on the short-term and long-term clinical outcomes of pediatric and neonatal patients, and it is imperative in these populations to mitigate the pathways leading to AKI and be prepared for early diagnosis and treatment intervention of established AKI. Recently, artificial intelligence (AI) has provided more advent predictive models for early detection/prediction of AKI utilizing machine learning (ML). By providing strong detail and evidence from risk scores and electronic alerts, this review outlines a comprehensive and holistic insight into the current state of AI in AKI in pediatric/neonatal patients. In the pediatric population, AI models including XGBoost, logistic regression, support vector machines, decision trees, naïve Bayes, and risk stratification scores (Renal Angina Index (RAI), Nephrotoxic Injury Negated by Just-in-time Action (NINJA)) have shown success in predicting AKI using variables like serum creatinine, urine output, and electronic health record (EHR) alerts. Similarly, in the neonatal population, using the "Baby NINJA" model showed a decrease in nephrotoxic medication exposure by 42%, the rate of AKI by 78%, and the number of days with AKI by 68%. Furthermore, the "STARZ" risk stratification AI model showed a predictive ability of AKI within 7 days of NICU admission of AUC 0.93 and AUC of 0.96 in the validation and derivation cohorts, respectively. Many studies have reported the superiority of using biomarkers to predict AKI in pediatric patients and neonates as well. Future directions include the application of AI along with biomarkers (NGAL, CysC, OPN, IL-18, B2M, etc.) in a Labelbox configuration to create a more robust and accurate model for predicting and detecting pediatric/neonatal AKI. [ABSTRACT FROM AUTHOR]

Distal renal tubular acidosis (RTA) with sensorineural deafness is a rare entity, inherited in an autosomal recessive manner. It is caused by mutations in the ATP6V1B1 gene, leading to defective function of H+-ATPase pump in the distal nephron, cochlea and endolymphatic sac. We report two siblings with distal RTA and sensorineural deafness having mutation C>T in the first coding exon of the gene, resulting in a non functional protein. The parents were found to be carriers for the mutation. [ABSTRACT FROM AUTHOR]

Background Acute kidney injury (AKI) is associated with increased morbidity/mortality. With artificial intelligence (AI), more dynamic models for mortality prediction in AKI patients have been developed using machine learning (ML) algorithms. The performance of various ML models was reviewed in terms of their ability to predict in-hospital mortality for AKI patients. Methods A literature search was conducted through PubMed, Embase and Web of Science databases. Included studies contained variables regarding the efficacy of the AI model [the AUC, accuracy, sensitivity, specificity, negative predictive value and positive predictive value]. Only original studies that consisted of cross-sectional studies, prospective and retrospective studies were included, while reviews and self-reported outcomes were excluded. There was no restriction on time and geographic location. Results Eight studies with 37   032 AKI patients were included, with a mean age of 65.3 years. The in-hospital mortality was 18.0% in the derivation and 15.8% in the validation cohorts. The pooled [95% confidence interval (CI)] AUC was observed to be highest for the broad learning system (BLS) model [0.852 (0.820–0.883)] and elastic net final (ENF) model [0.852 (0.813–0.891)], and lowest for proposed clinical model (PCM) [0.765 (0.716–0.814)]. The pooled (95% CI) AUC of BLS and ENF did not differ significantly from other models except PCM [Delong's test P = .022]. PCM exhibited the highest negative predictive value, which supports this model's use as a possible rule-out tool. Conclusion Our results show that BLS and ENF models are equally effective as other ML models in predicting in-hospital mortality, with variability across all models. Additional studies are needed. [ABSTRACT FROM AUTHOR]