Presentation, treatment, and outcome of renovascular hypertension below 2 years of age.

Renovascular hypertension in most cases requires endovascular treatment and/or surgery. This is technically much more difficult in small children and there is very limited published knowledge in this age group. We here present treatment and outcome of young children with renovascular hypertension at our institution. Children below 2 years of age, with renovascular hypertension between January 1998 and March 2020 were retrospectively reviewed. Demographics and treatment modalities were noted. Primary outcome was blood pressure within a week after the procedures and at last available visit. Sixty-six angiographies were performed in 34 patients. Median age at time of first angiography was 1.03 (interquartile range (IQR) 0.4-1.4) years and systolic blood pressure at presentation 130 (IQR 130-150) mm Hg. Thirty-eight percent (13/34) of children were incidentally diagnosed and 18% (6/34) presented with heart failure. Twenty-six (76%) children had main renal artery stenosis and 17 (50%) mid-aortic syndrome. Seventeen (50%) children showed intrarenal, six (18%) mesenteric, and three (9%) cerebrovascular involvement. Twenty patients underwent 45 percutaneous transluminal angioplasty procedures and seven children surgeries. In 44% of the 16 patients who underwent only percutaneous transluminal angioplasty blood pressure was normalized, 38% had improvement on same or decreased treatment and 19% showed no improvement. Complications were seen in 7.5% (5/66) of angiographies. In four of the seven (57%) children who underwent surgery blood pressure was normalized, two had improved (29%) and one unchanged (14%) blood pressure. CONCLUSION: In small children with renovascular hypertension below the age of 2 years, percutaneous transluminal angioplasty caused significant improvement in blood pressure with low complication profile. Surgery can be recommended where percutaneous transluminal angioplasty and medical treatments failed. WHAT IS KNOWN: • Renovascular hypertension is diagnosed in all age groups from a few weeks of life until adulthood. • Both angioplasty and surgery are significantly more difficult to perform in small children and the published information on short and long-term outcome in these children is very scarce. WHAT IS NEW: • Children below the age of two years can safely and successfully undergo selective renal angiography and also safely be treated with angioplasty. • We here present a large group of babies and infants where angioplasty and in some cases surgery effectively and safely improved their blood pressure.

Longitudinal Impact of a Randomized Clinical Trial to Improve Family Function, Reduce Maternal Stress and Improve Child Outcomes in Families of Children with ADHD.

Objective Evaluate the efficacy of a 12 month nursing case-management intervention over a period of 18 months, 6 months after the end of intervention, for families of children attention deficit hyperactivity disorder (ADHD). Methods Mother and child dyads were enrolled to participate in a randomized controlled clinical trial. Children were 4-18 years old. Data were collected at baseline, 6, 12, and 18 months or 6 months after the termination of direct intervention. Longitudinal analyses, using generalized estimating equations, were conducted to assess change in study outcomes relating to family function, maternal stress, and child behavior over the 18 month period. Results Compared to control families, some family function outcomes were moderately improved in the intervention group. In particular, intervention families demonstrated substantial improvement in implementing family behavior controls (p value = 0.038) and improvement in family satisfaction (not statistically significant p = 0.062). Although there was improvement in the overall family function measure there was not a statistically significant difference between groups. Maternal stress and child behavior outcomes were not significantly different between control and intervention groups by the end of the intervention. Conclusions for Practice Addressing ADHD is complex and requires the assessment of comorbidities that might exacerbate negative behavior. Our findings support the latest American Academy of Pediatrics guidelines to use behavioral therapy as the first line of treatment in young children. Nursing case-management interventions that provide direct family education and improve family function, especially with respect to providing structure and behavior control, may complement and facilitate behavioral therapy for treatment of ADHD and improving child behavior.

Non-invasive imaging cannot replace formal angiography in the diagnosis of renovascular hypertension.

OBJECTIVES: Renovascular disease (RVD) is found in about 10 % of secondary childhood hypertension. Digital subtraction angiography (DSA) is the gold standard to diagnose RVD. Non-invasive imaging methods like Doppler ultrasound (US), magnetic resonance angiography (MRA), and computed tomography angiography (CTA) are increasingly used. Our aim was to evaluate the role and accuracy of US, MRA, and CTA compared to DSA in diagnosing RVD in children. PATIENTS AND METHODS: A retrospective review of 127 children with suspected renovascular hypertension was performed in children referred to Great Ormond Street Hospital between 2006 and 2014 due to clinical suspicion of renovascular hypertension and/or findings on US and/or MRA or CTA. RESULTS: Ninety-nine of 127 children (78 %) were diagnosed with renovascular disease and 80 were treated with percutaneous transluminal angioplasty during the same procedure. The median age at presentation was 5.6 (range, 2.5-10.6) years. Thirty-six children had unilateral renal artery stenosis in major extraparenchymal vessels, 47 bilateral stenosis and 16 intrarenal small vessel disease. US had a sensitivity of 63 % and specificity of 95 %. MRA and CTA were performed in 39 and 34 children, respectively. CTA sensitivity was slightly higher than that of MRA, 88 vs. 80 %, and specificity 81 vs. 63 %. CONCLUSIONS: The sensitivity of MRA and CTA is still too low to reliably rule out renovascular disease in children with a strong suspicion of this diagnosis. DSA remains the gold standard to diagnose renovascular hypertension and is recommended when clinical and laboratory criteria are highly suggestive of renovascular disease even with normal radiological investigations from non-invasive methods.

Desirable Components for a Customized, Home-Based, Digital Care-Management App for Children and Young People With Long-Term, Chronic Conditions: A Qualitative Exploration.

BACKGROUND: Mobile apps for mobile phones and tablet devices are widely used by children and young people aged 0-18 years with long-term health conditions, such as chronic kidney disease (CKD), and their healthy peers for social networking or gaming. They are also poised to become a major source of health guidance. However, app development processes that are coproduced, rigorously developed, and evaluated to provide tailored, condition-specific, practical advice on day-to-day care management are seldom systematic or sufficiently described to enable replication. Furthermore, attempts to extrapolate to the real world are hampered by a poor understanding of the effects of key elements of app components. Therefore, effective and cost-effective novel, digital apps that will effectively and safely support care management are critical and timely. To inform development of such an app for children with CKD, a user requirements-gathering exercise was first needed. OBJECTIVE: To explore the views of children with CKD, their parents, and health care professionals to inform future development of a child-focused, care-management app. METHODS: Using age- and developmentally appropriate methods, we interviewed 36 participants: 5-10-year-olds (n=6), 11-14-year-olds (n=6), 15-18-year-olds (n=5), mothers (n=10), fathers (n=2), and health care professionals (n=7). Data were analyzed using Framework Analysis and behavior change theories. RESULTS: Of the 27 interviews, 19 (70%) interviews were individual and 8 (30%) were joint-5 out of 8 (63%) joint interviews were with a child or young person and their parent, 1 out of 8 (13%) were with a child and both parents, and 2 out of 8 (25%) were with 2 professionals. Three key themes emerged to inform development of a software requirement specification for a future home-based, digital care-management app intervention: (1) Gaps in current online information and support, (2) Difficulties experienced by children with a long-term condition, and (3) Suggestions for a digital care-management app. Reported gaps included the fact that current online information is not usually appropriate for children as it is "dry" and "boring," could be "scary," and was either hard to understand or not relevant to individuals' circumstances. For children, searching online was much less accessible than using a professional-endorsed mobile app. Children also reported difficulty explaining their condition to others, maintaining treatment adherence, coping with feeling isolated, and with trying to live a "normal" life. There was recognition that a developmentally appropriate, CKD-specific app could support the process of explaining the condition to healthy peers, reducing isolation, adhering to care-management plans, and living a "normal" life. Participants recommended a range of media and content to include in a tailored, interactive, age- and developmentally appropriate app. For example, the user would be able to enter their age and diagnosis so that only age-appropriate and condition-specific content is displayed. CONCLUSIONS: Future development of a digital app that meets the identified information and support needs and preferences of children with CKD will maximize its utility, thereby augmenting CKD caregiving and optimizing outcomes.

Clinico-pathological correlations of congenital and infantile nephrotic syndrome over twenty years.

BACKGROUND: Nephrotic syndrome (NS) presenting early in life is caused by heterogeneous glomerular diseases. We retrospectively evaluated whether histological diagnosis in children presenting with NS in the first year of life predicts remission or progression to end-stage kidney disease (ESKD). METHODS: This is a single centre retrospective review of all children diagnosed with NS before one year of age between 1990 and 2009. All subjects had a renal biopsy, which was independently blindly reviewed by a single renal pathologist for the purpose of this study. RESULTS: Forty-nine children (25 female) who presented at 0.1-11.6 (median 1.6) months were included with 31 presenting within the first three months of life. Histopathological review diagnostic categories were; 13 Mesangial proliferative glomerulopathy (MesGN), 12 Focal and segmental glomerulosclerosis (FSGS), 11 Finnish type changes, eight Diffuse Mesangial Sclerosis (DMS), three Minimal change disease (MCD) and one each of Dense Deposit Disease (DDD) and Membranous nephropathy. Two children died from haemorrhagic complications of the biopsy. Eight children achieved remission (four MesGN, one Finnish type changes, one FSGS, one MCD and one membranous) with patient and renal survival of 73 % and 43 %, respectively, at follow-up duration of 5-222 (median 73) months (with five lost to follow-up). All children with Finnish-type histopathological changes presented within five months of age. Due to the historical nature of the cohort, genetic testing was only available for 14 children, nine of whom had an identifiable genetic basis (seven NPHS1, one PLCE1 and one ITGA3) with none of these nine children achieving remission. All of them had presented within four months of age and required renal replacement therapy, and two died. CONCLUSIONS: Histopathological findings are varied in children presenting with NS early in life. Whilst groups of histological patterns of disease are associated with differing outcomes, accurate prediction of disease course in a specific case is difficult and more widespread genetic testing may improve the understanding of this group of diseases and their optimal management.

An action research study to explore the nature of the nurse consultant role in the care of children and young people.

AIMS AND OBJECTIVES: An action research study was undertaken to explore the development of the nurse consultant role when caring for children and young people. BACKGROUND: Five nurse consultants in different areas of specialist care in a tertiary paediatric hospital undertook the study when implementing the new role of nurse consultant into the hospital. METHODS: Action research meetings took place over a year. The nurse consultants then collated and analysed data using thematic analysis during the second year. A research fellow facilitated meetings, carried out participant observation, and coordinated the action research project. RESULTS: Data analysis revealed 22 subthemes grouped into four overarching themes: shaping the role; shaping child-centred care through consultancy; taking responsibility for practice; and leadership. These roles and their ease and complexity within the nurse consultant role are examined in further detail in this paper. Balancing the four key components in a newly developing role was initially complex and required support. Over time the nurse consultants developed the necessary skills to perform fully in all areas. A major challenge was developing the research role, a key function of the nurse consultant role. By the end of the study, all nurse consultants were actively embarking upon their own research either in preparation for or as part of Doctoral studies. CONCLUSIONS AND RELEVANCE TO CLINICAL PRACTICE: While there are many similarities with nurse consultants in adult practice, one major difference was the nurse consultant role in supporting families when caring for children and young people. This meant having a three-way communication style: with the family, the child/young person, and other healthcare professionals. This communication style was observed by the research fellow in participant observation of the nurse consultants undertaking clinical care and is described further in the analysis of the role.

Detection and diagnostic value of urine leucine-rich α-2-glycoprotein in children with suspected acute appendicitis.

STUDY OBJECTIVE: Previously, we used a proteomics approach for the discovery of new diagnostic markers of acute appendicitis and identified leucine-rich α-2-glycoprotein (LRG) that was elevated in the urine of children with acute appendicitis and enriched in diseased appendices. Here, we sought to evaluate the diagnostic utility of enzyme-linked immunosorbent assay (ELISA) of urine LRG in a blinded, prospective, cohort study of children being evaluated for acute abdominal pain. METHODS: Urine LRG concentration was measured with a commercially available LRG ELISA and selected ion monitoring mass spectrometry. Urine LRG test performance was evaluated blindly against the pathologic diagnosis and histologic grade of appendicitis. RESULTS: Urine LRG was measured in 49 patients. Mean urine LRG concentration measured with commercial LRG ELISA was significantly elevated in patients with acute appendicitis but exhibited an interference effect. Direct measurements using selected ion monitoring mass spectrometry demonstrated that LRG was elevated more than 100-fold in patients with acute appendicitis compared with those without, with the receiver operating characteristic area under the curve of 0.98 (95% confidence interval 0.96 to 1.0). Among patients with acute appendicitis, elevations of urine LRG measured with ELISA and selected ion monitoring mass spectrometry correlated with the histologic severity of appendicitis. CONCLUSION: Urine LRG ELISA allows for discrimination between patients with and without acute appendicitis but exhibits limited accuracy because of immunoassay interference. Direct measurements of urine LRG with selected ion monitoring mass spectrometry demonstrate superior diagnostic performance. Development of a clinical-grade urine LRG assay is needed to advance the diagnostic accuracy of clinical evaluations of appendicitis.

Renal artery revascularisation can restore kidney function with absent radiotracer uptake.

BACKGROUND: Children with renovascular hypertension often present with severe hypertension. Some children have severe obstruction of their renal arteries resulting in <10% relative function on [(99m)Tc]dimercaptosuccinic acid (DMSA) scan. Conventional treatment of these children has been nephrectomy of the poorly functioning kidney to normalise their blood pressure (BP). CASE-DIAGNOSIS/TREATMENT: We describe three children aged 20 months to 9 years with severe renal artery stenosis and severe hypertension who had radionucleotide uptake of 0% in one kidney. In one case, no renal perfusion was demonstrated by duplex ultrasound scan. Significant recovery of relative renal function of 18 to 52% was achieved after revascularisation by percutaneous angioplasty or open surgery of the obstructed renal artery. CONCLUSION: These cases illustrate that scintigraphy alone cannot be used to predict salvageable function in children with renovascular disease.

Factors Contributing to Employment Status over Time for Caregivers of Young People with Mental Health Disorders.

This study utilized the conservation of resources theory to guide the examination of employment outcomes for caregivers of children with emotional and/or behavioral disorders. The sample included 2455 caregivers whose children received services through federally funded systems of care. Of special interest was whether receiving services and supports predicted change in employment status. We examined change in employment between baseline data collection and the six-month follow-up including: (1) gaining employment, and (2) retaining employment. Findings indicated that the relationship between service/supports and caregiver employment differed depending on initial employment status, and type of service received. Accessing any service was associated with gaining employment. For families who accessed any services, receiving behavioral aide services was associated with gaining employment. Caregivers of children who used residential services were less likely to lose employment. Several child, caregiver, and demographic variables also predicted employment status over time. Taken together, the findings suggest that caregivers of children with emotional and behavioral challenges are at risk for downward cycles of resource loss, and that services and supports have the potential to mitigate that risk.

Expanding Workplace Inclusion of Employees Who Are Parents of Children with Disabilities through Diversity Training.

Employed parents raising children with disabilities manage exceptional care responsibilities along with their work careers. This study examines the effects of targeted diversity training on human resource (HR) professionals' knowledge of the work-family experiences of these parents, and on their self-efficacy in providing workplace supports. Using computer-based training in field settings, 64 U.S. human resource professionals in an international company participated in two diversity training sessions. Data related to knowledge and efficacy of dependent and disability care were collected before the first training and immediately after the second. HR participants demonstrated significant increases from pretest to posttest on the trained items: knowledge of dependent and disability care and self-efficacy regarding provision of workplace supports. There was no change in relevant but untrained variables over time. Training HR professionals on parents' exceptional care responsibilities, specific community resources, and heightened self-efficacy promoted their likelihood to grant flexible work arrangements. Results suggest HR self-efficacy is developmental, building on prior knowledge of dependent care and tenure in HR positions. This is one of the first studies that address the effects of HR diversity training regarding employees providing exceptional care. Theoretical developments and implications for inclusive practices are discussed.

Renal FMD may not confer a familial hypertensive risk nor is it caused by ACTA2 mutations.

Renal fibromuscular dysplasia (FMD) can cause hypertension, and previous reports suggest that FMD is familial. We hypothesized that, in families containing an individual with proven FMD, relatives of index cases would have an increased risk of hypertension. ACTA2 mutations cause a spectrum of extra-renal arteriopathy, leading to our second hypothesis that mutations are implicated in FMD. The blood pressure of first-degree relatives was measured using standard devices and, when indicated, with 24-h ambulatory monitoring. Leucocyte DNA was obtained from FMD index cases and ACTA2 sequenced. Thirteen unrelated index cases, aged 2-32 (median 15) years, were recruited. Blood pressure was assessed in 40 first-degree relatives, comprising 22 parents aged 28-58 (median 44) years and 18 siblings aged 3-30 (median 13) years. Hypertension was evident in six (27%) parents but in none of the eight adult siblings. Of the ten screened siblings aged less than 18 years, one teenager was pre-hypertensive (90th-95th centile), the remainder being normotensive. No ACTA2 mutations were found in 13 index cases. Hypertension was evident in 20% of all assessed adult first-degree relatives and is therefore not increased relative to 25% of the adult population. Although hypertensive parents did not undergo angiography to assign FMD status, this observation, together with the lack of hypertension in 18 siblings, indicates that FMD is unlikely to confer an excess hypertension risk in first-degree relatives up to middle-age. Furthermore, in our cohort, FMD was not caused by ACTA2 mutations.

Pre- and postcaptopril renal scintigraphy as a screening test for renovascular hypertension in children.

We studied the ability of pre- and postcaptopril renal scintigraphy to predict renovascular disease (RVD) in children. Retrospective review of medical notes and radiology reports of all hypertensive children who had had both pre- and postcaptopril renal scintigraphy with [(99m)Tc] dimercaptosuccinic acid (DMSA) and/or [(99m)Tc] mercaptoacetyltriglycine (MAG3) and digital subtraction angiography (DSA). 81 children aged 1-18 (median 10) years were studied with 62% (51) having a diagnosis of RVD. Main renal artery disease, intrarenal disease, and both main and intrarenal artery disease were present in 25, 14, and 12 patients respectively. The isotope study accurately diagnosed RVD, confirmed by DSA, in 47% (24 of 51) children, with eight false positive studies. The sensitivity, specificity, and positive and negative predictive values of the isotope study to predict RVD were 48%, 73%, 76%, and 51%, respectively. Pre- and postcaptopril renal scintigraphy was unable to predict RVD in children.

Training Needs of Peer and Non-Peer Transition Service Providers: Results of a National Survey.

Participatory action research processes guided a national online survey of service providers working with transition age youth with mental health challenges. The survey investigated transition service providers' ratings of the importance of competencies and skills, self-assessed need for training in these competencies and skills, their preferred training modalities, and obstacles to engaging in training. The 254 participants identified trauma-informed care, understanding youth culture, promoting natural supports, and using culturally responsive practices as most important training needs. Age, years in current job, years in transition work, and race/ethnicity predicted training needs regarding some competencies and skills. Peer providers expressed preferences for young adult-led training. Qualitative responses highlighted training needs for supporting specific underserved populations: youth from communities of color, LGBTQ youth, and those with co-occurring disorders. Results may guide future training initiatives for peer support and non-peer support providers and workforce development initiatives designed to improve behavioral health services for young people.

Renovascular hypertension in children.

Renovascular disease is an uncommon but important cause of hypertension in children. It is usually diagnosed after a long delay because blood pressure is infrequently measured in children and high values are generally dismissed as inaccurate. Many children with renovascular disease have abnormalities of other blood vessels (aorta, cerebral, intestinal, or iliac). Individuals suspected of having the disorder can be investigated further with CT, MRI, or renal scintigraphy done before and after administration of an angiotensin-converting-enzyme inhibitor, but angiography is still the gold standard. Most children with renovascular disease will need interventional or surgical treatment. Endovascular treatment with or without stenting will cure or reduce high blood pressure in more than half of all affected children. Surgical intervention, if needed, should be delayed preferably until an age when the child is fully grown. Modern treatment provided by a multidisciplinary team of paediatric nephrologists, interventional radiologists, and vascular surgeons offers good long-term treatment results.

Predicting Positive Education Outcomes for Emerging Adults in Mental Health Systems of Care.

Emerging adults who receive services based on positive youth development models have shown an ability to shape their own life course to achieve positive goals. This paper reports secondary data analysis from the Longitudinal Child and Family Outcome Study including 248 culturally diverse youth ages 17 through 22 receiving mental health services in systems of care. After 12 months of services, school performance was positively related to youth ratings of school functioning and service participation and satisfaction. Regression analysis revealed ratings of young peoples' perceptions of school functioning, and their experience in services added to the significant prediction of satisfactory school performance, even controlling for sex and attendance. Finally, in addition to expected predictors, participation in planning their own services significantly predicted enrollment in higher education for those who finished high school. Findings suggest that programs and practices based on positive youth development approaches can improve educational outcomes for emerging adults.

Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology. Absence of definitive diagnostic markers limits the accuracy of clinical evaluations of suspected KD with significant increases in morbidity. In turn, incomplete understanding of its molecular pathogenesis hinders the identification of rational targets needed to improve therapy. We used high-accuracy mass spectrometry proteomics to analyse over 2000 unique proteins in clinical urine specimens of patients with KD. We discovered that urine proteomes of patients with KD, but not those with mimicking conditions, were enriched for markers of cellular injury such as filamin and talin, immune regulators such as complement regulator CSMD3, immune pattern recognition receptor muclin, and immune cytokine protease meprin A. Significant elevations of filamin C and meprin A were detected in both the serum and urine in two independent cohorts of patients with KD, comprised of a total of 236 patients. Meprin A and filamin C exhibited superior diagnostic performance as compared to currently used markers of disease in a blinded case-control study of 107 patients with suspected KD, with receiver operating characteristic areas under the curve of 0.98 (95% confidence intervals [CI] of 0.97-1 and 0.95-1, respectively). Notably, meprin A was enriched in the coronary artery lesions of a mouse model of KD. In all, urine proteome profiles revealed novel candidate molecular markers of KD, including filamin C and meprin A that exhibit excellent diagnostic performance. These disease markers may improve the diagnostic accuracy of clinical evaluations of children with suspected KD, lead to the identification of novel therapeutic targets, and allow the development of a biological classification of Kawasaki disease.

Work-family fit: voices of parents of children with emotional and behavioral disorders.

Employed parents of children with emotional or behavioral disorders overcome significant challenges to fulfill their daily work-family responsibilities; however, their experiences in meeting these multiple demands have not been examined. The purpose of the present study was to describe the strategies these parents use and their perceptions about how caregiving and employment responsibilities can successfully fit together. Findings of five focus groups conducted with 41 employed parents whose children had been diagnosed with mental health problems are presented. Qualitative analysis was used to identify major themes across the areas of employment responsibilities and adaptations, child care arrangements, and achieving fit between job and home. Results revealed that parents experience a serious lack of community-based services and resources necessary to support work and family obligations in a meaningful way. The data suggest a need for more research and services development to support these families in their communities.