Dialysis for paediatric acute kidney injury in Cape Town, South Africa.

BACKGROUND: Dialysis is lifesaving for acute kidney injury (AKI), but access is poor in less resourced settings. A "peritoneal dialysis (PD) first" policy for paediatric AKI is more feasible than haemodialysis in low-resource settings. METHODS: Retrospective review of modalities and outcomes of children dialysed acutely at Red Cross War Memorial Children's Hospital between 1998 and 2020. RESULTS: Of the 593 children with AKI who received dialysis, 463 (78.1%) received PD first. Median age was 9.0 (range 0.03-219.3; IQR 13.0-69.6) months; 57.6% were < 1 year old. Weights ranged from 0.9 to 2.0 kg (median 7.0 kg, IQR 3.0-16.0 kg); 38.6% were < 5 kg. PD was used more in younger children compared to extracorporeal dialysis (ECD), with median ages 6.4 (IQR 0.9-30.4) vs. 73.9 (IQR 17.5-113.9) months, respectively (p = 0.001). PD was performed with Seldinger soft catheters (n = 480/578, 83%), predominantly inserted by paediatricians at the bedside (n = 412/490, 84.1%). Complications occurred in 127/560 (22.7%) children receiving PD. Overall, 314/542 (57.8%) children survived. Survival was significantly lower in neonates (< 1 month old, 47.5%) and infants (1-12 months old, 49.2%) compared with older children (> 1 year old, 70.4%, p < 0.0001). Survival was superior in the ECD (75.4%) than in the PD group (55.6%, p = 0.002). CONCLUSIONS: "PD First for Paediatric AKI" is a valuable therapeutic approach for children with AKI. It is feasible in low-resourced settings where bedside PD catheter insertion can be safely taught and is an acceptable dialysis modality, especially in settings where children with AKI would otherwise not survive.

Evaluation of the implementation of a "Pediatric Feasibility Assessment for Transplantation" tool in children and adolescents at Red Cross War Memorial Children's Hospital, Cape Town, South Africa.

BACKGROUND: Kidney transplantation remains the treatment of choice for children with kidney failure (KF). In South Africa, kidney replacement therapy (KRT) is restricted to children eligible for transplantation. This study reports on the implementation of the Paediatric Feasibility Assessment for Transplantation (pFAT) tool, a psychosocial risk score developed in South Africa to support transparent transplant eligibility assessment in a low-resource setting. METHODS: Single-center retrospective descriptive analysis of children assessed for KRT using pFAT tool from 2015 to 2021. RESULTS: Using the pFAT form, 88 children (median [range] age 12.0 [1.1 to 19.0] years) were assessed for KRT. Thirty (34.1%) children were not listed for KRT, scoring poorly in all domains, and were referred for supportive palliative care. Fourteen of these 30 children (46.7%) died, with a median survival of 6 months without dialysis. Nine children were reassessed and two were subsequently listed. Residing >300 km from the hospital (p = .009) and having adherence concerns (p = .003) were independently associated with nonlisting. Of the 58 (65.9%) children listed for KRT, 40 (69.0%) were transplanted. One-year patient and graft survival were 97.2% and 88.6%, respectively. Only one of the four grafts lost at 1-year posttransplant was attributed to psychosocial issues. CONCLUSIONS: Short-term outcomes among children listed using the pFAT form are good. Among those nonlisted, the pFAT highlights specific psychosocial/socioeconomic barriers, over which most children themselves have no power to change, which should be systemically addressed to permit eligibility of more children and save lives.

Care burden and support needs of caregivers of infants and children with CHDs and dysphagia.

Dysphagia is common in children with CHDs, resulting in multiple stressors for their caregivers including having a child with a serious medical condition and coping with their child's feeding needs. However, relatively little is known about caregivers' perceptions and experiences of the burden of care and support needs for their child with a CHD and dysphagia in low-middle income contexts. This qualitative study investigated the burden of care and support needs identified by parents of children with CHDs and dysphagia in a single centre in South Africa. Semi-structured interviews took place in a tertiary hospital with seven mothers of children with CHDs and dysphagia, followed by content analysis. Participants described four main impacts of their child's condition, which included worry, the burden of caregiving, emotional responses, and acceptance and coping. The participants were well-supported by speech-language therapists and dieticians, but suggestions for additional support included support groups and using mobile messaging apps for communication with peers and professionals. The study has important implications for understanding challenges faced by caregivers of children with complex needs in low-middle income settings and will be useful to inform and improve holistic healthcare practice for families of children with CHDs and dysphagia.

A research definition and framework for acute paediatric critical illness across resource-variable settings: a modified Delphi consensus.

The true global burden of paediatric critical illness remains unknown. Studies on children with life-threatening conditions are hindered by the absence of a common definition for acute paediatric critical illness (DEFCRIT) that outlines components and attributes of critical illness and does not depend on local capacity to provide critical care. We present an evidence-informed consensus definition and framework for acute paediatric critical illness. DEFCRIT was developed following a scoping review of 29 studies and key concepts identified by an interdisciplinary, international core expert panel (n=24). A modified Delphi process was then done with a panel of multidisciplinary health-care global experts (n=109) until consensus was reached on eight essential attributes and 28 statements as the basis of DEFCRIT. Consensus was reached in two Delphi rounds with an expert retention rate of 89%. The final consensus definition for acute paediatric critical illness is: an infant, child, or adolescent with an illness, injury, or post-operative state that increases the risk for or results in acute physiological instability (abnormal physiological parameters or vital organ dysfunction or failure) or a clinical support requirement (such as frequent or continuous monitoring or time-sensitive interventions) to prevent further deterioration or death. The proposed definition and framework provide the conceptual clarity needed for a unified approach for global research across resource-variable settings. Future work will centre on validating DEFCRIT and determining high priority measures and guidelines for data collection and analysis that will promote its use in research.

Protein loss and glucose absorption in children with AKI treated with peritoneal dialysis.

BACKGROUND: Protein loss and glucose absorption in children on acute peritoneal dialysis (PD) is important to inform dietary prescription, yet data are lacking in this regard. This study was a secondary analysis of a previously published crossover randomised controlled trial, aiming to describe glucose uptake and protein loss into dialysate among children with acute kidney injury (AKI) receiving PD. METHODS: This secondary analysis described and compared dialysate albumin loss and glucose absorption in 15 children with AKI receiving PD or continuous flow peritoneal dialysis (CFPD). In addition, correlations between albumin loss, glucose absorption and other patient and dialysis factors were analysed. RESULTS: Median (range) age and weight of participants were 6.0 (0.2-14) months and 5.8 (2.3-14.0) kg, respectively. Patients received approximately 8 h of dialysis on each modality; however, results were extrapolated and expressed per day. The mean ± SD albumin loss on conventional PD and CFPD was 0.3 ± 0.19 g/kg/day and 0.56 ± 0.5 g/kg/day, respectively, and the mean ± SD glucose absorption was 4.67 ± 2.87 g/kg/day and 3.85 ±4.1 g/kg/day, respectively. There was a moderate correlation between ultrafiltration and albumin loss during CFPD only (Pearson's R = 0.61; p = 0.02). There were no significant differences between PD and CFPD for either glucose absorption or albumin loss; however, the study was not powered for this outcome. CONCLUSIONS: Protein losses and glucose absorption in children on PD with AKI are significant and should be considered when prescribing nutritional content. Protein losses on CFPD were twice as high as on conventional PD.

Global disparities in cystic fibrosis outcomes prior to CFTR modulators: A CF registries cohort study in South Africa and Canada.

BACKGROUND: Outcomes of cystic fibrosis (CF) differ between low-middle income and high-income countries, but comparative data are lacking. We compared South African (SA) and Canadian CF outcomes to explore what disparities existed prior to access of CFTR modulators in Canada. METHODS: A cross-sectional study of SA and Canadian CF registries data for period 1 January to 31 December 2018. CF registry data were harmonised between countries to compare lung function and nutrition outcomes. Poor nutrition was defined as BMIz-score < -1 in children and < 18.5 kg/m2 in adults. Standardised mean difference (SMD) >10 was considered significant. RESULTS: After excluding Canadians on CFTR modulators and lung transplant recipients, data on 4049 Canadian and 446 SA people was analysed. Compared to Canada, people in SA were younger (median age 15.8 years vs. 24.1 years: SMD 52) with fewer males (47.8% vs 54.2%; SMD 12.5) and White (70.9% vs. 93.3%; SMD 61.3). Class I-III CFTR mutation frequency was similar in SA (n = 384, 86.1%) and Canada (n = 3426, 84.9%). After adjusting for age, gender, diagnosis age, genotype, P.aeruginosa infection and pulmonary treatments, FEV1pp was 8.9% lower (95% CI 6.3% to 11.4%) and poor nutrition 1.7-fold more common (OR 1.70; 95% CI 1.19-2.41) in SA compared to Canada. CONCLUSION: Lung function and nutrition was significantly lower in SA compared to Canada. Global disparities in CF outcomes between high and low-middle income countries are likely to widen as CFTR modulators are rapidly scaled up in only high-income countries.

Pediatric Acute Respiratory Distress Syndrome in South African PICUs: A Multisite Point-Prevalence Study.

OBJECTIVES: To describe the prevalence of pediatric acute respiratory distress syndrome (pARDS) and the characteristics of children with pARDS in South African PICUs. DESIGN: Observational multicenter, cross-sectional point-prevalence study. SETTING: Eight PICUs in four South African provinces. PATIENTS: All children beyond the neonatal period and under 18 years of age admitted to participating PICUs. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Clinical and demographic data were prospectively collected on a single day of each month, from February to July 2022, using a centralized database. Cases with or at risk of pARDS were identified using the 2015 Pediatric Acute Lung Injury Consensus Conference criteria. Prevalence was calculated as the number of children meeting pARDS criteria/the total number of children admitted to PICU at the same time points. Three hundred ten patients were present in the PICU on study days: 166 (53.5%) male, median (interquartile range [IQR]) age 9.8 (3.1-32.9) months, and 195 (62.9%) invasively mechanically ventilated. Seventy-one (22.9%) patients were classified as being "at risk" of pARDS and 95 patients (prevalence 30.6%; 95% CI, 24.7-37.5%) fulfilled pARDS case criteria, with severity classified as mild (58.2%), moderate (25.3%), and severe (17.6%). Median (IQR) admission Pediatric Index of Mortality 3 risk of mortality in patients with and without pARDS was 5.6 (3.4-12.1) % versus 3.9 (1.0-8.2) % ( p = 0.002). Diagnostic categories differed between pARDS and non-pARDS groups ( p = 0.002), with no difference in age, sex, or presence of comorbidities. On multivariable logistic regression, increasing admission risk of mortality (adjusted odds ratio [aOR] 1.02; 95% CI, 1.00-1.04; p = 0.04) and being admitted with a respiratory condition (aOR 2.64; 95% CI, 1.27-5.48; p = 0.01) were independently associated with an increased likelihood of having pARDS. CONCLUSIONS: The 30.6% prevalence of pARDS in South Africa is substantially higher than reports from other sociogeographical regions, highlighting the need for further research in this setting.

Lessons learned from regional training of paediatric nephrology fellows in Africa.

BACKGROUND: Access to care for children with kidney disease is limited in less well-resourced regions of the world and paediatric nephrology (PN) workforce development with good practical skills is critical. METHODS: Retrospective review of a PN training program and trainee feedback from 1999 to 2021, based at Red Cross War Memorial Children's Hospital (RCWMCH), University of Cape Town. RESULTS: A regionally appropriate 1-2-year training program enrolled 38 fellows with an initial 100% return rate to their country of origin. Program funding included fellowships from the International Pediatric Nephrology Association (IPNA), International Society of Nephrology (ISN), International Society of Peritoneal Dialysis (ISPD), and the African Paediatric Fellowship Program (APFP). Fellows were trained on both in- and out-patient management of infants and children with kidney disorders. "Hands-on skills" training included examination, diagnosis and management skills, practical insertion of peritoneal dialysis catheters for management of acute kidney injury and kidney biopsies. Of 16 trainees who completed > 1 year of training, 14 (88%) successfully completed subspecialty exams and 9 (56%) completed a master's degree with a research component. PN fellows reported that their training was appropriate and enabled them to make a difference in their respective communities. CONCLUSIONS: This training program has successfully equipped African physicians with the requisite knowledge and skills to provide PN services in resource-constrained areas for children with kidney disease. The provision of funding from multiple organizations committed to paediatric kidney disease has contributed to the success of the program, along with the fellows' commitment to build PN healthcare capacity in Africa. A higher resolution version of the Graphical abstract is available as Supplementary information.

The Reviewer Academy of the Society of Critical Care Medicine: Key Principles and Strategic Plan.

The Society of Critical Care Medicine (SCCM) Reviewer Academy seeks to train and establish a community of trusted, reliable, and skilled peer reviewers with diverse backgrounds and interests to promote high-quality reviews for each of the SCCM journals. Goals of the Academy include building accessible resources to highlight qualities of excellent manuscript reviews; educating and mentoring a diverse group of healthcare professionals; and establishing and upholding standards for insightful and informative reviews. This manuscript will map the mission of the Reviewer Academy with a succinct summary of the importance of peer review, process of reviewing a manuscript, and the expected ethical standards of reviewers. We will equip readers to target concise, thoughtful feedback as peer reviewers, advance their understanding of the editorial process and inspire readers to integrate medical journalism into diverse professional careers.

Prevalence and Incidence of Pediatric Acute Respiratory Distress Syndrome in a Tertiary Academic PICU in South Africa.

OBJECTIVES: To determine the prevalence and incidence of pediatric acute respiratory distress syndrome (pARDS) among infants and children admitted to the PICU. DESIGN: A single-center descriptive point prevalence study with twice weekly data collection over a 6 months (August 2020 to February 12, 2021). SETTING: Red Cross War Memorial Children's Hospital, Cape Town, South Africa. PATIENTS: All infants and children admitted to the PICU on study days were included. INTERVENTIONS: Data were captured electronically on a standardized case record form using a Research Electronic Data Capture electronic database. MEASUREMENTS AND MAIN RESULTS: The Pediatric Acute Lung Injury Consensus Conference criteria were used to define pARDS cases. Prevalence was calculated as the total number of pARDS cases/1,000 PICU bed days. The study included 354 patients (median [interquartile range]) 10.1 months old (1.5-61.3 mo old), with 204 males (57.6%), who occupied 879 bed days. Of these 879 bed days, 266 (30.3%; 95% CI, 27.2-33.3%) were occupied by pARDS cases, with a calculated prevalence and incidence of 302.6 of 1,000 bed days (30.3%) and 29.7% (95% CI, 26.7-32.7%), respectively. Three cases from the cohort were defined using the oxygen saturation index calculation. In cases receiving invasive ventilation ( n = 494; 56.2%), pARDS severity was classified as mild ( n = 143; 16.3%), moderate ( n = 44; 5.0%), and severe ( n = 29, 3.3%). A further 205 beds (23.3%) were occupied by patients classified as being at risk of pARDS. CONCLUSIONS: The prevalence and incidence of pARDS in a South African PICU appears substantially higher than findings described in international reports. Further investigation of risk factors and outcomes is warranted.

Gravity-assisted continuous flow peritoneal dialysis technique use in acute kidney injury in children: a randomized, crossover clinical trial.

BACKGROUND: Our previously demonstrated continuous flow peritoneal dialysis (CFPD) technique in children with acute kidney injury (AKI), although effective, was manpower heavy and expensive due to the high-volume pumps required. The aim of this study was to develop and test a novel gravity-driven CFPD technique in children using readily available, inexpensive equipment and to compare this technique to conventional PD. METHODS: After development and initial in vitro testing, a randomised crossover clinical trial was conducted in 15 children with AKI requiring dialysis. Patients received both conventional PD and CFPD sequentially, in random order. Primary outcomes were measures of feasibility, clearance and ultrafiltration (UF). Secondary outcomes were complications and mass transfer coefficients (MTC). Paired t-tests were used to compare PD and CFPD outcomes. RESULTS: Median (range) age and weight of participants were 6.0 (0.2-14) months and 5.8 (2.3-14.0) kg, respectively. The CFPD system was easily and rapidly assembled. There were no serious adverse events attributed to CFPD. Mean ± SD UF was significantly higher on CFPD compared to conventional PD (4.3 ± 3.15 ml/kg/h vs. 1.04 ± 1.72 ml/kg/h; p < 0.001). Clearances for urea, creatinine and phosphate for children on CFPD were 9.9 ± 3.10 ml/min/1.73 m2, 7.9 ± 3.3 ml/min/1.73 m2 and 5.5 ± 1.5 ml/min/1.73 m2 compared to conventional PD with values of 4.3 ± 1.68 ml/min/1.73 m2, 3.57 ± 1.3 ml/min/1.73 m2 and 2.53 ± 0.85 ml/min/1.73 m2, respectively (all p < 0.001). CONCLUSION: Gravity-assisted CFPD appears to be a feasible and effective way to augment ultrafiltration and clearances in children with AKI. It can be assembled from readily available non-expensive equipment. A higher resolution version of the Graphical abstract is available as Supplementary information.

Burden and severity of deranged electrolytes and kidney function in children seen in a tertiary hospital in Kano, northern Nigeria.

INTRODUCTION: Derangement in serum electrolytes and kidney function is often overlooked, especially in resource-constrained settings, and associated with increased risk of morbidity and mortality. This study aimed to describe the burden of derangements in serum electrolytes and kidney function in children presenting to a tertiary hospital in Nigeria. METHODS: The laboratory records of all children who had serum electrolytes urea and creatinine ordered on their first presentation to hospital between January 1 and June 30, 2017 were retrospectively reviewed. Basic demographic data including admission status (inpatient or outpatient) were recordedandserum levels of sodium, potassium, chloride and bicarbonate were assessed for derangements usingnormal values from established reference ranges. Results of repeat samples were excluded. Kidney function was classified based on the serum creatinine relative to normal values for age and sex. RESULTS: During the study period, 1909 children (60.3% male); median (IQR) age 42 (11.9) months had serum chemistry and 1248 (65.4%) were admitted. Results of their first samples were analyzed. Electrolyte derangements were present in 78.6% of the samples most commonly hyponatraemia (41.1%), low bicarbonate(37.2%), hypochloraemia (33.5%) and hypokalemia(18.9%). Azotaemia was found in 20.1% of the results. Elevated serum creatinine levels were found in 399 children (24.7%), 24.1% of those being in the severe category. Children aged 5 years and younger accounted for 76.4% of those with derangement in kidney function. One hundred and eight outpatients (17.8%) had deranged kidney function. CONCLUSION: Deranged serum electrolytes and kidney function were common in this cohort.

Executive Summary of the Second International Guidelines for the Diagnosis and Management of Pediatric Acute Respiratory Distress Syndrome (PALICC-2).

OBJECTIVES: We sought to update our 2015 work in the Second Pediatric Acute Lung Injury Consensus Conference (PALICC-2) guidelines for the diagnosis and management of pediatric acute respiratory distress syndrome (PARDS), considering new evidence and topic areas that were not previously addressed. DESIGN: International consensus conference series involving 52 multidisciplinary international content experts in PARDS and four methodology experts from 15 countries, using consensus conference methodology, and implementation science. SETTING: Not applicable. PATIENTS: Patients with or at risk for PARDS. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Eleven subgroups conducted systematic or scoping reviews addressing 11 topic areas: 1) definition, incidence, and epidemiology; 2) pathobiology, severity, and risk stratification; 3) ventilatory support; 4) pulmonary-specific ancillary treatment; 5) nonpulmonary treatment; 6) monitoring; 7) noninvasive respiratory support; 8) extracorporeal support; 9) morbidity and long-term outcomes; 10) clinical informatics and data science; and 11) resource-limited settings. The search included MEDLINE, EMBASE, and CINAHL Complete (EBSCOhost) and was updated in March 2022. Grading of Recommendations, Assessment, Development, and Evaluation methodology was used to summarize evidence and develop the recommendations, which were discussed and voted on by all PALICC-2 experts. There were 146 recommendations and statements, including: 34 recommendations for clinical practice; 112 consensus-based statements with 18 on PARDS definition, 55 on good practice, seven on policy, and 32 on research. All recommendations and statements had agreement greater than 80%. CONCLUSIONS: PALICC-2 recommendations and consensus-based statements should facilitate the implementation and adherence to the best clinical practice in patients with PARDS. These results will also inform the development of future programs of research that are crucially needed to provide stronger evidence to guide the pediatric critical care teams managing these patients.

Nonpulmonary Treatments for Pediatric Acute Respiratory Distress Syndrome: From the Second Pediatric Acute Lung Injury Consensus Conference.

OBJECTIVES: To provide an updated review of the literature on nonpulmonary treatments for pediatric acute respiratory distress syndrome (PARDS) from the Second Pediatric Acute Lung Injury Consensus Conference. DATA SOURCES: MEDLINE (Ovid), Embase (Elsevier), and CINAHL Complete (EBSCOhost). STUDY SELECTION: Searches were limited to children with PARDS or hypoxic respiratory failure focused on nonpulmonary adjunctive therapies (sedation, delirium management, neuromuscular blockade, nutrition, fluid management, transfusion, sleep management, and rehabilitation). DATA EXTRACTION: Title/abstract review, full-text review, and data extraction using a standardized data collection form. DATA SYNTHESIS: The Grading of Recommendations Assessment, Development, and Evaluation approach was used to identify and summarize evidence and develop recommendations. Twenty-five studies were identified for full-text extraction. Five clinical practice recommendations were generated, related to neuromuscular blockade, nutrition, fluid management, and transfusion. Thirteen good practice statements were generated on the use of sedation, iatrogenic withdrawal syndrome, delirium, sleep management, rehabilitation, and additional information on neuromuscular blockade and nutrition. Three research statements were generated to promote further investigation in nonpulmonary therapies for PARDS. CONCLUSIONS: These recommendations and statements about nonpulmonary treatments in PARDS are intended to promote optimization and consistency of care for patients with PARDS and identify areas of uncertainty requiring further investigation.

Diagnostic, Management, and Research Considerations for Pediatric Acute Respiratory Distress Syndrome in Resource-Limited Settings: From the Second Pediatric Acute Lung Injury Consensus Conference.

OBJECTIVES: Diagnosis of pediatric acute respiratory distress syndrome (PARDS) in resource-limited settings (RLS) is challenging and remains poorly described. We conducted a review of the literature to optimize recognition of PARDS in RLS and to provide recommendations/statements for clinical practice and future research in these settings as part of the Second Pediatric Acute Lung Injury Consensus Conference (PALICC-2). DATA SOURCES: MEDLINE (Ovid), Embase (Elsevier), and CINAHL Complete (EBSCOhost). STUDY SELECTION: We included studies related to precipitating factors for PARDS, mechanical ventilation (MV), pulmonary and nonpulmonary ancillary treatments, and long-term outcomes in children who survive PARDS in RLS. DATA EXTRACTION: Title/abstract review, full-text review, and data extraction using a standardized data collection form. DATA SYNTHESIS: The Grading of Recommendations Assessment, Development, and Evaluation approach was used to identify and summarize evidence and develop recommendations. Seventy-seven studies were identified for full-text extraction. We were unable to identify any literature on which to base recommendations. We gained consensus on six clinical statements (good practice, definition, and policy) and five research statements. Clinicians should be aware of diseases and comorbidities, uncommon in most high-income settings, that predispose to the development of PARDS in RLS. Because of difficulties in recognizing PARDS and to avoid underdiagnosis, the PALICC-2 possible PARDS definition allows exclusion of imaging criteria when all other criteria are met, including noninvasive metrics of hypoxemia. The availability of MV support, regular MV training and education, as well as accessibility and costs of pulmonary and nonpulmonary ancillary therapies are other concerns related to management of PARDS in RLS. Data on long-term outcomes and feasibility of follow-up in PARDS survivors from RLS are also lacking. CONCLUSIONS: To date, PARDS remains poorly described in RLS. Clinicians working in these settings should be aware of common precipitating factors for PARDS in their patients. Future studies utilizing the PALICC-2 definitions are urgently needed to describe the epidemiology, management, and outcomes of PARDS in RLS.

ß-adrenergic sweat test in children with inconclusive cystic fibrosis diagnosis: Do we need new reference ranges?

BACKGROUND: Investigating inconclusive cystic fibrosis (CF) diagnosis in children is difficult without advanced cystic fibrosis transmembrane conductance regulator (CFTR) function tests. This study investigated the utility of beta (ß)-adrenergic sweat test to exclude CF in participants with inconclusive diagnosis (CF suspects) in South Africa. METHODS: ß-adrenergic sweat test and sweat chloride tests (SCT) were performed simultaneously in CF suspects and adult controls (healthy, CFTR heterozygotes and CF). Cholinergic and ß-adrenergic induced sweat rate was measured by evaporimetry (transepithelial water loss [TEWL]: g H2 O/m2 /h) following intradermal injections. Next-generation sequencing of CFTR was performed in CF suspects. CF diagnosis was defined by genotype. RESULTS: Thirty-seven controls (10 healthy, 14 CF, 13 CFTR heterozygotes) and 32 CF suspects (26 children; 6 adults) were enrolled. Six were excluded from formal analyses due to ß-adrenergic sweat test failure. In adults, evaporimetry was superior to SCT for diagnosis of CF with ß-adrenergic:cholinergic ratio TEWL ≤ 0.05 achieving 100% sensitivity and specificity. Twenty-two CF suspect children (age range: 3.4-15.6 years) completed ß-adrenergic sweat testing of which none had CF confirmed by genotyping: ß-adrenergic:cholinergic ratio > 0.05 successfully excluded CF in all but one child who was CFTR heterozygous. Median peak ß-adrenergic TEWL and ß-adrenergic:cholinergic ratio in CFTR negative and CFTR heterozygous children was significantly lower than adult controls. CONCLUSION: ß-adrenergic sweat test is more accurate than SCT for excluding CF in children with inconclusive diagnosis. Established reference ranges for ß-adrenergic sweat test may not be suitable for children due to lower ß-adrenergic sweat secretion compared to adults.

Measurement of hand grip strength: A cross-sectional study of two dynamometry devices.

Background: Grip strength has been identified as an important indicator of health status and predictor of clinical outcomes. The gold standard for measuring grip strength is the JAMAR® Hydraulic Hand Dynamometer. Less expensive dynamometers are available but have not been validated within a hospital setting. Objectives: To validate the Camry Digital Handgrip Dynamometer (Model EH101) against the validated JAMAR® Dynamometer (Model J00105) in a hospital population. Methods: A cross-sectional observational study with a randomised single-blind cross-over component was conducted on consenting adult patients admitted to general hospital wards. The best of three measurements taken using the dominant hand was used for analysis. Results: Fifty-one participants (median [interquartile range] age 42 [30-58] years; n = 27 [52.9%] female) were included. The mean difference between the Jamar® and Camry measurements was 1.9 kg ± 3.6 kg (t-value 0.9; p = 0.4). There was a strong positive correlation between the Jamar® and the Camry devices (R = 0.94; r² = 0.88; p < 0.0001). Excellent agreement was found between Jamar® and Camry measurements (interclass correlational coefficient 0.97, 95% CI 0.94-0.99, p < 0.0001). Hand dominance significantly affected the agreement between devices (p = 0.002). Conclusions: The Camry Digital Handgrip Dynamometer is a valid tool for assessing grip strength in hospitalised adult patients. Clinical implications: The Camry Digital Handgrip Dynamometer could be used as an inexpensive tool to measure grip strength.

A Core Outcome Measurement Set for Pediatric Critical Care.

OBJECTIVES: To identify a PICU Core Outcome Measurement Set (PICU COMS), a set of measures that can be used to evaluate the PICU Core Outcome Set (PICU COS) domains in PICU patients and their families. DESIGN: A modified Delphi consensus process. SETTING: Four webinars attended by PICU physicians and nurses, pediatric surgeons, rehabilitation physicians, and scientists with expertise in PICU clinical care or research ( n = 35). Attendees were from eight countries and convened from the Pediatric Acute Lung Injury and Sepsis Investigators Pediatric Outcomes STudies after PICU Investigators and the Eunice Kennedy Shriver National Institute of Child Health and Human Development Collaborative Pediatric Critical Care Research Network PICU COS Investigators. SUBJECTS: Measures to assess outcome domains of the PICU COS are as follows: cognitive, emotional, overall (including health-related quality of life), physical, and family health. Measures evaluating social health were also considered. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Measures were classified as general or additional based on generalizability across PICU populations, feasibility, and relevance to specific COS domains. Measures with high consensus, defined as 80% agreement for inclusion, were selected for the PICU COMS. Among 140 candidate measures, 24 were delineated as general (broadly applicable) and, of these, 10 achieved consensus for inclusion in the COMS (7 patient-oriented and 3 family-oriented). Six of the seven patient measures were applicable to the broadest range of patients, diagnoses, and developmental abilities. All were validated in pediatric populations and have normative pediatric data. Twenty additional measures focusing on specific populations or in-depth evaluation of a COS subdomain also met consensus for inclusion as COMS additional measures. CONCLUSIONS: The PICU COMS delineates measures to evaluate domains in the PICU COS and facilitates comparability across future research studies to characterize PICU survivorship and enable interventional studies to target long-term outcomes after critical illness.