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Infant botulism is caused by ingesting Clostridium botulinum spores and characterized by symmetric descending paralysis, which can progress to respiratory failure in severe cases (1). Specific therapies include intravenous administration [...]

Parechoviruses are small, nonenveloped, singlestranded RNA viruses belonging to the large Picomaviridae family that circulate worldwide; primary infections occur mainly in children Nineteen human parechovirus types have been classified as [...]

Three mother-baby pairs with invasive meningococcal disease occurred over 7 months in Western Australia, Australia, at a time when serogroup W sequence type 11 clonal complex was the predominant local [...]

Background Infants with chronic kidney disease (CKD) form a vulnerable population who are highly prone to mineral and bone disorders (MBD) including biochemical abnormalities, growth retardation, bone deformities, and fractures. We present a position paper on the diagnosis and management of CKD-MBD in infants based on available evidence and the opinion of experts from the European Society for Paediatric Nephrology (ESPN) CKD-MBD and Dialysis working groups and the Pediatric Renal Nutrition Taskforce. Methods PICO (Patient, Intervention, Comparator, Outcomes) questions were generated, and relevant literature searches performed covering a population of infants below 2 years of age with CKD stages 2-5 or on dialysis. Clinical practice points (CPPs) were developed and leveled using the American Academy of Pediatrics grading matrix. A Delphi consensus approach was followed. Results We present 34 CPPs for diagnosis and management of CKD-MBD in infants, including dietary control of calcium and phosphate, and medications to prevent and treat CKD-MBD (native and active vitamin D, calcium supplementation, phosphate binders). Conclusion As there are few high-quality studies in this field, the strength of most statements is weak to moderate, and may need to be adapted to individual patient needs by the treating physician. Research recommendations to study key outcome measures in this unique population are suggested. Graphical Abstract, Author(s): Justine Bacchetta [sup.1] [sup.2] [sup.3] , Claus Peter Schmitt [sup.4] , Sevcan A. Bakkaloglu [sup.5] , Shelley Cleghorn [sup.6] , Maren Leifheit-Nestler [sup.7] , Agnieszka Prytula [sup.8] , Bruno [...]

Background Continuous kidney replacement therapy (CKRT) has been expanded from simple kidney replacement therapy to the field of critical illness in children. However, CKRT is rarely used in critically ill neonates in the neonatal intensive care unit (NICU). This study aimed to describe patients' clinical characteristics at admission and CKRT initiation, CKRT effects, short-term outcomes, and predictors of death in critically ill neonates. Methods A 7-year single-center retrospective study in a tertiary NICU. Results Thirty-nine critically ill neonates received CKRT between May 2015 and April 2022 with a mortality rate of 35.9%. The most common primary diagnosis was neonatal sepsis in 15 cases (38.5%). Continuous veno-venous hemodiafiltration and continuous veno-venous hemofiltration were applied in 43.6% and 56.4% of neonates, respectively. The duration of CKRT was 44 (18, 72) h. Thirty-one patients (79.5%) had complications due to CKRT-related adverse events, and the most common complication was thrombocytopenia. Approximately 12 h after the CKRT initiation, urine volume, mean arterial pressure, and pH were increased, and serum creatinine, blood urea nitrogen, and blood lactate were decreased. In the multivariate logistic regression analysis, neonatal critical illness score [odds ratio 0.886 (0.786 ~ 0.998), P = 0.046] was an independent risk factor for death in critically ill neonates who received CKRT. Conclusions CKRT can be an effective and feasible technique in critically ill neonates, but the overall mortality and CKRT-related complications are relatively high. Furthermore, the probability of death is greater among neonates with greater severity of illness at CKRT initiation. Graphical abstract, Author(s): Jinglin Xu [sup.1] , Lingyu Fang [sup.1] , Jiangbin Chen [sup.1] , Xinhua Chen [sup.1] , Hansong Yang [sup.1] , Weifeng Zhang [sup.1] , Lianqiang Wu [sup.1] , Dongmei [...]

Background Neonatal renal vein thrombosis (NRVT) is a rare condition with little data available. Methods We retrospectively analyzed newborns diagnosed with NRVT admitted to 3 pediatric nephrology units in Paris from 2005 to 2020. Results Twenty-seven patients were analyzed (male = 59%). The median age at diagnosis was 2.5 days (1 - 4.5). Diagnosis was suspected based on at least one of the three cardinal signs of renal vein thrombosis in 93%: flank mass (67%), hematuria (67%) and thrombocytopenia (70%). In all patients, diagnosis was confirmed by ultrasound. All patients had at least one known perinatal risk factor. A prothrombotic risk factor was found in 13 patients (48%). NRVT was unilateral in 70%, involving the left renal vein in 58%. Among 25 treated patients, 19 (76%) received low molecular weight heparin (LMWH) as initial therapy, 2 (8%) received unfractionated heparin and 4 (16%) received fibrinolysis. Median duration of treatment was 8 weeks (4 - 12). Bleeding occurred significantly more often with fibrinolysis than with LMWH/supportive therapy (3 of 4: 75% vs 0 of 4: 0%, p = 0.05). Clot resolution in patients treated with fibrinolysis did not differ significantly from those treated with LMWH/supportive therapy. After a median follow-up of 5.7 years (3 years - 9.9 years), pathological kidney features were observed in 73% of the patients (19 of 26), kidney atrophy in 18 (69%), hypertension in 2 (8%), chronic kidney disease (CKD) in 1 (4%) and proteinuria in 2 (8%). Conclusions NRVT remains a challenging condition, which still requires further study because of its associated morbidity. Graphical abstract, Author(s): Bellaure Ndoudi Likoho [sup.1] , Romain Berthaud [sup.2] , Claire Dossier [sup.1] , Jean-Daniel Delbet [sup.3] , Olivia Boyer [sup.2] , Véronique Baudouin [sup.1] , Marianne Alison [sup.4] , [...]

Chikungunya is a mosquitoborne disease caused by the chikungunya virus (CHIKV), a single-stranded positive-sense RNA virus belonging to the family Togaviridae (1), which is transmitted to humans through the bite [...]

Background Hypertensive disorders of pregnancy is one of the leading causes of adverse infant outcomes. Black women are disproportionately affected by hypertensive disorders of pregnancy, and it associated adverse outcomes. Adequate prenatal care may improve adverse infant outcomes. However, the evidence on adequate prenatal care improving birth outcomes for women with hypertensive disorders of pregnancy especially for Blacks is limited. This study examined the role of adequate prenatal care and race/ethnicity as moderators of hypertensive disorders of pregnancy on infant outcomes. Methods The sample was obtained from the 2016-2019 Pregnancy Risk Assessment Monitoring Surveillance dataset from North Carolina. We compared adequate prenatal care among women with hypertensive disorders of pregnancy (n = 610) to women without(n = 2,827), and women with hypertensive disorders of pregnancy with adequate prenatal care to women hypertensive disorders of pregnancy with inadequate prenatal care. Results The weighted prevalence of hypertensive disorders of pregnancy was 14.1%. Adequate prenatal care was associated with better infant outcomes for low birth weight (AOR = 0.72; 95% CI = 0.58, 0.90) and preterm birth (AOR = 0.62; 95% CI = 0.46, 0.82). Although these effects were not moderated by Black race/ethnicity, Black women independently also had worse outcomes for preterm birth (AOR = 1.59; 95% CI = 1.11, 2.28) and low birth weight (AOR = 1.81; 95% CI = 1.42, 2.29). Conclusions Moderation of hypertensive disorders of pregnancy effects on infant outcomes by prenatal care and race/ethnicity was not found. Women with hypertensive disorders of pregnancy who received inadequate prenatal care experienced worse adverse birth outcomes compared to women without hypertensive disorders of pregnancy. Strategies to improve prenatal care, particularly among underserved populations at risk for hypertensive disorders of pregnancy, need to be a public health priority., Author(s): Forgive Avorgbedor [sup.1] [sup.2] [sup.4] , Thomas P. McCoy [sup.4] , Susan Silva [sup.1] , James A. Blumenthal [sup.2] , Elizabeth Merwin [sup.1] , Seonae Yeo [sup.3] , Diane [...]

Objectives We sought to identify the most common diagnostic categories linked to dispensed opioid prescriptions among children 1-36 months old and changes in patterns over the years 2000 to 2017. Methods This study used South Carolina's Medicaid claims data of pediatric dispensed outpatient opioid prescriptions between 2000 and 2017. The major opioid-related diagnostic category (indication) for each prescription was identified using visit primary diagnoses and the Clinical Classification System (AHRQ-CCS) software. The variables of interest were the rate of opioid prescriptions per 1,000 visits for each diagnostic category and the relative percentage of opioid prescriptions assigned to each category compared to all categories. Results Six major diagnostic categories were identified; Diseases of the respiratory system (RESP), Congenital anomalies (CONG), Injury (INJURY), Diseases of the nervous system and sense organs (NEURO), Diseases of the digestive system (GI), and Diseases of the genitourinary system (GU). The overall rate of dispensed opioid prescriptions per category declined significantly for four diagnostic categories throughout the study period, RESP by 15.13, INJURY by 8.49, NEURO by 7.33, and GI by 5.93. Two categories increased during the same time, CONG (by 9.47) and GU (by 6.98). RESP was the most prevalent category linked to a dispensed opioid prescription within 2010-2012 (almost 25%) but CONG was the most prevalent by 2014 (17.77%). Conclusions for practice Among Medicaid children 1-36 months old, annual dispensed opioid prescription rates declined for most major diagnostic categories (RESP, INJURY, NEURO, and GI). Future studies should explore alternatives to current opioid dispensing practices for GU and CONG cases., Author(s): Laksika Banu M. Sivaraj [sup.1] , William T. Jr. Basco [sup.2] , Smith F. Heavner [sup.1] [sup.3] [sup.4] , Snehal S. Lopes [sup.1] , Laura J. Rolke [sup.1] , [...]

Background Acute kidney injury (AKI) is common and is associated with poor clinical outcomes in premature neonates. Urine biomarkers hold the promise to improve our understanding and care of patients with kidney disease. Because kidney maturation and gender can impact urine biomarker values in extremely low gestational age neonates (ELGANs), careful control of gestational age (GA) and time is critical to any urine biomarker studies in neonates. Methods To improve our understanding of the potential use of urine biomarkers to detect AKI during the first postnatal weeks, we performed a nested case-control study to evaluate 21 candidate urine AKI biomarkers. Cases include 20 ELGANs with severe AKI. Each case was matched with 2 controls for the same GA week (rounded down to the nearest week), gender, and birth weight (BW) (± 50 g). Results Urine cystatin C, creatinine, ghrelin, fibroblast growth factor-23 (FGF23), tissue metalloproteinase 2 (TIMP2) and vascular endothelial growth factor A (VEGFa) concentrations were higher in ELGANs with early severe AKI compared to matched control subjects without AKI. Urine epidermal growth factor (EGF) and uromodulin (UMOD) concentrations are lower in cases than controls. Interleukin (IL)-15 was lower on day 1, but higher on day 8 in cases than controls; while VEGFa was lower on day 1, but higher on day 5 in cases than controls. Conclusion Urine biomarkers hold the promise to improve our ability to reliably detect kidney injury. Interventional studies are needed to determine the biomarkers' ability to predict outcomes, enhance AKI phenotypes, and improve timely interventions which can prevent the sequalae of AKI in ELGANs. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): David J. Askenazi [sup.1] , Brian A. Halloran [sup.1] , Patrick J. Heagerty [sup.2] , Robert H. Schmicker [sup.2] , Sandra E. Juul [sup.3] , Sangeeta Hingorani [sup.3] , [...]

Background Despite its utility, uncertainty exists on the feasibility of acute peritoneal dialysis (PD) and optimal PD catheter type for very low birth weight (VLBW < 1500 g) and extremely low birth weight (ELBW < 1000 g) infants. We hereby report our experience of acute PD among these high-risk infants and compare the outcome between stylet-based rigid catheter (SRC) and Cook Mac-Loc Multipurpose Drainage catheters® (CMMDC). Methods Case notes of infants < 1500 g undergoing PD between 2012 and 2021 in a network of five participating neonatal units supported by a tertiary paediatric nephrology centre in Kolkata, India, were retrospectively reviewed. PD was conducted either with SRC or after 2018 with CMMDC. Outcome parameters included complications, survival during PD, and survival to discharge. Results 24 infants (VLBW: n = 13 and ELBW: n = 11) underwent PD at median age 4.5 days (IQR 3-6) with either CMMDC (n = 14) or SRC (n = 10). Significant improvement in biochemical parameters and fluid removal was seen in both ELBW and VLBW infants. CMMDC was associated with significantly fewer PD-related complications 7/14 (50%) vs. 9/10 (90%) (p = 0.04) and higher survival during PD 13/14 (93%) vs. 5/10 (50%) (p = 0.02), without significant difference in survival to hospital discharge 8/14 (57%) vs. 3/10 (30%) (p = 0.25). CMMDC also enabled longer duration of PD, higher ultrafiltration, and better control of acidosis. Consumable cost was higher for CMMDC (USD$60) than SRC (USD$14). Conclusions In a low resource setting, CMMDC had lower PD complications and superior short-term survival among ELBW/VLBW infants. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Rajiv Sinha [sup.1] [sup.2] , Rana Saha [sup.1] [sup.3] [sup.4] , Deblina Dasgupta [sup.1] , Niladri Bose [sup.1] , Shamik Ghosh [sup.5] , Ashok Modi [sup.4] , Bikramjit Das [...]

Background Assessment of fluid status in neonatal dialysis has largely focused on traditional tools including clinical assessment, serial weights, and blood pressure (BP) measurements. However, in infants on kidney replacement therapy, the assessment of fluid overload is problematic due to errors in weight assessment, subtlety of physical exam findings, and inaccuracy of non-invasive BP measurements. Case summary In this presentation of a neonate with bilateral renal agenesis requiring kidney replacement therapy, the treating team assessed a number of variables in determining the ultrafiltration prescription for dialysis across 2 modalities (hemodialysis and continuous kidney replacement therapy). Complications Fluid overload, cardiomegaly, and worsened respiratory status occurred when attempting to assess the neonate's fluid status by traditional markers (weights, blood pressures, physical exam findings). B-type natriuretic peptide (BNP) was obtained and was noted to correlate with the degree of fluid overload. Key management points Compared to traditional tools for assessment of fluid status in pediatric dialysis, BNP assisted the medical team in optimizing the volume status of the subject and determining optimal daily ultrafiltration goals. Due to the rapid release in response to myocardial stretch and the lack of kidney clearance of the peptide, BNP may represent an objective, timely, and reliable index of volume status in the pediatric dialysis patient., Author(s): Noureddin Nourbakhsh [sup.1] [sup.2] , Nadine Benador [sup.1] [sup.2] Author Affiliations: (1) grid.266100.3, 0000 0001 2107 4242, Pediatric Nephrology, University of California San Diego, , La Jolla, CA, USA [...]

Antenatal steroid administration to pregnant women at risk of prematurity provides pulmonarymaturation in infants, while it has limited effects on incidence of bronchopulmonary dysplasia (BPD), the clinical expression of hyperoxia-induced lung injury (HILI). Cytidine-5'-diphosphate choline (CDP-choline) was shown to alleviate HILI when administered to newborn rats. Therefore, we investigated effects of maternal administration of CDP-choline, alone or in combination with betamethasone, on lung maturation in neonatal rats subjected to HILI immediately after birth. Pregnant rats were randomly assigned to one of the four treatments: saline (1mL/kg), CDP-choline (300 mg/kg), betamethasone (0.4mg/kg), or CDP-choline plus betamethasone (combination therapy). From postnatal day 1 to 11, pups born to mothers in the same treatment group were pooled and randomly assigned to either normoxia or hyperoxia group. Biochemical an d histopathological effects of CDP-choline on neonatal lung tissue were evaluated. Antenatal CDP-choline treatment increased levels of phosphatidylcholine and total lung phospholipids, decreased apoptosis, and improved alveolarization. The outcomes were further improved with combination therapy compared to the administration of CDP-choline or betamethasone alone. These results demonstrate that antenatal CDP-choline treatment provides benefit in experimental HILI either alone or more intensively when administered along with a steroid, suggesting a possible utility for CDP-choline against BPD. Key words: betamethasone, bronchopulmonary dysplasia, citicoline, newborn rat, 1. Introduction Bronchopulmonary dysplasia (BPD) is the most common long-term respiratory complication of premature birth. Despite improvements such as antenatal corticosteroid therapy, non-invasive ventilation practices, surfactant replacement therapy, and advanced [...]

Background Bardet-Biedl syndrome (BBS) is a rare, autosomal recessive ciliopathy characterized by early onset retinal dystrophy, renal anomalies, postaxial polydactyly, and cognitive impairment with considerable phenotypic heterogeneity. BBS results from biallelic pathogenic variants in over 20 genes that encode key proteins required for the assembly or primary ciliary functions of the BBSome, a heterooctameric protein complex critical for homeostasis of primary cilia. While variants in BBS1 are most frequently identified in affected individuals, the renal and pulmonary phenotypes associated with BBS1 variants are reportedly less severe than those seen in affected individuals with pathogenic variants in the other BBS-associated genes. Case-Diagnosis We report an infant with severe renal dysplasia and lethal pulmonary hypoplasia who was homozygous for the most common BBS1 pathogenic variant (c.1169 T > G; p.M390R) and also carried a predicted pathogenic variant in TTC21B (c.1846C > T; p.R616C), a genetic modifier of disease severity of ciliopathies associated with renal dysplasia and pulmonary hypoplasia. Conclusions This report expands the phenotypic spectrum of BBS with the first infant with lethal neonatal respiratory failure associated with biallelic, pathogenic variants in BBS1 and a monoallelic, predicted pathogenic variant in TTC21B. BBS should be considered among the ciliopathies in the differential diagnosis of neonates with renal dysplasia and severe respiratory failure., Author(s): Luke Viehl [sup.1] , Daniel J. Wegner [sup.1] , Stanley P. Hmiel [sup.1] , Frances V. White [sup.2] , Sanjay Jain [sup.3] , F. S. Cole [sup.1] , Jennifer [...]

Background Survival to hospital discharge in neonates born with kidney failure has not been previously described. Methods This was a retrospective, observational analysis of the Pediatric Health Information System (PHIS) database from 2005 to 2019. Primary outcome was survival at discharge; secondary outcomes were hospital and ICU length of stay (LOS). Univariate analysis was performed to describe the population by birth weight (BW) and characterize survival; multivariable generalized liner mixed modeling assuming a binomial distribution and logit link was performed to identify mortality risk factors. Results Of 213 neonates born with kidney failure (median BW 2714 g; GA 35 weeks; 68% male), 4 (1.9%) did not receive dialysis or peritoneal dialysis (PD) catheter placement, 152 (72.9%) received PD only, 49 (23.4%) received PD plus extracorporeal dialysis (ECD), and 8 (3.4%) were treated with an undocumented dialysis modality. Median age at dialysis initiation was 7 days; median hospital LOS and ICU LOS were 84 and 69 days, respectively. One-hundred and sixty-two patients (76%) survived to discharge. Non-survivors (n = 51) were more likely to have received ECD and mechanical ventilation, and had a longer duration of mechanical ventilation. Every day of mechanical ventilation increased the mortality odds by 2% (n = 189; adjusted OR 1.02; 1.01, 1.03); in addition, the odds of mortality were 2 times higher in those who received ECD vs. only PD (adjusted OR 2.25; 1.04, 4.86). Conclusions Survival to initial hospital discharge occurs in the majority of neonates born with kidney failure. Predictors of increased mortality included longer duration of mechanical ventilation, as well as the requirement for ECD. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Donna J. Claes [sup.1] , Troy Richardson [sup.2] , Matthew W. Harer [sup.3] , Mahima Keswani [sup.4] , Alicia Neu [sup.5] , Allison C. Redpath Mahon [sup.6] , Michael [...]

Excessive accumulation of fluid may result in interstitial edema and multiorgan dysfunction. Over the past few decades, the detrimental impact of fluid overload has been further defined in adult and pediatric populations. Growing evidence highlights the importance of monitoring, preventing, managing, and treating fluid overload appropriately. Translating this knowledge to neonates is difficult as they have different disease pathophysiologies, and because neonatal physiology changes rapidly postnatally in many of the organ systems (i.e., skin, kidneys, and cardiovascular, pulmonary, and gastrointestinal). Thus, evaluations of the optimal targets for fluid balance need to consider the disease state as well as the gestational and postmenstrual age of the infant. Integration of what is known about neonatal fluid overload with individual alterations in physiology is imperative in clinical management. This comprehensive review will address what is known about the epidemiology and pathophysiology of neonatal fluid overload and highlight the known knowledge gaps. Finally, we provide clinical recommendations for monitoring, prevention, and treatment of fluid overload., Author(s): Lucinda J. Weaver [sup.1] , Colm P. Travers [sup.1] , Namasivayam Ambalavanan [sup.1] , David Askenazi [sup.1] Author Affiliations: (1) grid.265892.2, 0000000106344187, University of Alabama at Birmingham, , Birmingham, [...]

Background To determine in vitro whether infant hemofiltration and hemodialysis devices can reliably deliver precise ultrafiltration (UF) control. Methods We tested the Prismaflex, Aquarius and NIDUS devices which have different circuit types, by in vitro testing with a bag of saline set up as a dummy patient, and monitoring fluid shifts by precise weighing. We looked for differences between the UF rates set and achieved and between the UF result the device displays to the clinician and the true volumes removed, which may lead to clinical errors. We performed short studies at UF settings of zero and 40 ml/h, and with and without simulating poor withdrawal and return lines, and simulated a 4-h treatment session. Results The Prismaflex setting vs actual errors and display vs actual errors had wide variances, with SDs of 4.1 and 14.0 ml by 15 min, respectively, at both zero and 40 ml/h UF settings. The Aquarius values were wider at 17.3 and 30.3 ml, respectively. For the NIDUS, the mean UF errors were close to zero, and the variances were 0.17 ml. Stop-alarms induced by an obstructed line produced extra UF errors of up to 0.2 ml. A limitation was that we used crystalloid and not colloid for these tests. Conclusions Hemotherapy devices with conventional circuits available in the UK do not regulate UF control sufficiently well to recommend for use in small infants, but the NIDUS volumetrically controlled circuit does. All hemotherapy devices intended for small infants should be tested for UF precision. We were unable to test the CARPEDIEM or Aquadex devices. Graphical Abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Jean Crosier [sup.1] , Mike Whitaker [sup.2] , Heather J. Lambert [sup.1] , Paul Wellman [sup.3] , Andrew Nyman [sup.3] , Malcolm G. Coulthard [sup.1] Author Affiliations: (1) grid.459561.a, [...]