COVID-19: experiences of lockdown and support needs in children and young adults with kidney conditions.

BACKGROUND: During the initial COVID-19 pandemic, young United Kingdom (UK) kidney patients underwent lockdown and those with increased vulnerabilities socially isolated or 'shielded' at home. The experiences, information needs, decision-making and support needs of children and young adult (CYA) patients or their parents during this period is not well known. METHODS: A UK-wide online survey co-produced with patients was conducted in May 2020 amongst CYA aged 12-30, or parents of children aged < 18 years with any long-term kidney condition. Participants answered qualitative open text alongside quantitative closed questions. Thematic content analysis using a three-stage coding process was conducted. RESULTS: One-hundred and eighteen CYA (median age 21) and 197 parents of children (median age 10) responded. Predominant concerns from CYA were heightened vigilance about viral (68%) and kidney symptoms (77%) and detrimental impact on education or work opportunities (70%). Parents feared the virus more than CYA (71% vs. 40%), and had concerns that their child would catch the virus from them (64%) and would have an adverse impact on other children at home (65%). CYA thematic analysis revealed strong belief of becoming seriously ill if they contracted COVID-19; lost educational opportunities, socialisation and career development; and frustration with the public for not following social distancing rules. Positive outcomes included improved family relationships and community cohesion. Only a minority (14-21% CYA and 20-31% parents, merged questions) desired more support. Subgroup analysis identified greater negative psychological impact in the shielded group. CONCLUSIONS: This survey demonstrates substantial concern and need for accurate tailored advice for CYA based on individualised risks to improve shared decision making.

Low-Dose Aspirin Reduces the Rate of Renal Allograft Thrombosis in Pediatric Renal Transplant Recipients.

OBJECTIVES: Renal allograft thrombosis is an important cause of early renal allograft loss. A previous study from our unit showed thrombosis rates in patients who received heparin that were similar to those who did not receive any thromboprophylaxis. This study evaluated the impact of aspirin prophylaxis on renal allograft thrombosis rates in pediatric renal transplant recipients. MATERIALS AND METHODS: We conducted a retrospective study of 456 consecutive pediatric allografts from deceased and living related donors over age 22 years in a single center. Routine perioperative heparin was introduced in 1994 and was subsequently changed to aspirin prophylaxis in 2000. Group 1 comprised 126 patients who did not receive any thromboprophylaxis, group 2 comprised 128 patients who received heparin, and group 3 comprised 202 patients who received aspirin therapy. Variables associated with increased risk of renal allograft loss were examined using multivariable logistic regression. RESULTS: Thrombosis occurred in 11% (14/126) of grafts in group 1, 9% (11/128) of grafts in group 2, and 1% (2/202) of grafts in group 3 (odds ratio for aspirin group = 0.38, 95% confidence interval, 0.22-0.64; P = .02). In patients who received aspirin (group 3), there was only one renal allograft loss secondary to hemorrhage, and no grafts were lost in patients younger than 5 years of age. CONCLUSIONS: After our center introduced a change from heparin to aspirin prophylaxis, the thrombosis rate in pediatric renal allografts fell from 9% to 1%. Although there are a number of possible confounding variables, the introduction of aspirin has led to a reduced rate of renal allograft thrombosis.

Ready, Steady, Go - Achieving successful transition in cystic fibrosis.

Successful transfer to adult services is the paediatric team's anticipated endpoint for the care they provide to their patients. The preceding transition process needs to be well planned and designed to address young peoples' psycho-social, educational and vocational as well as their medical needs. Ready, Steady, Go is a generic programme that has been successfully implemented to make the transition process an integral part of the routine care of young people with cystic fibrosis. Used in combination with other initiatives, the programme helps achieve the more seamless transfer of young people better prepared to meet their ongoing needs.

Tolvaptan treatment for severe neonatal autosomal-dominant polycystic kidney disease.

BACKGROUND: Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful. CASE DIAGNOSIS/TREATMENT: A female infant with massive renal enlargement, respiratory compromise and hyponatraemia was treated with the arginine vasopressin receptor 2 antagonist tolvaptan. This resolved hyponatraemia, and there was no further increase in renal size. CONCLUSION: Tolvaptan may be a useful treatment for severe neonatal PKD.

Implementing transition: Ready Steady Go.

There is good evidence that morbidity and mortality increase for young persons (YP) following the move from paediatric to adult services. Studies show that effective transition between paediatric and adult care improves long-term outcomes. Many of the issues faced by young people across subspecialties with a long-term condition are generic. This article sets out some of the obstacles that have delayed the implementation of effective transition. It reports on a successful generic transition programme 'Ready Steady Go' that has been implemented within a large National Health Service teaching hospital in the UK, with secondary and tertiary paediatric services, where it is now established as part of routine care.

Eculizumab in atypical haemolytic uraemic syndrome with severe cardiac and neurological involvement.

BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) is a rare disorder usually caused by dysregulation of the alternative complement pathway. Uncontrolled complement activation results in systemic complement-mediated thrombotic microangiopathy (TMA) and subsequent multi-organ damage. The two most common extrarenal complications comprise neurological and cardiovascular involvement. Eculizumab, a humanised anti-C5 monoclonal antibody, has recently been introduced as a therapy for this condition. CASE-DIAGNOSIS/TREATMENT: A 19-month-old child suffering from aHUS with severe neurological involvement, dilated cardiomyopathy and renal impairment requiring dialysis received eculizumab as first-line treatment, initiated within 12 h of admission, resulting in significant improvements in her neurological state and normalisation of cardiac and renal function. These positive outcomes have been sustained with fortnightly eculizumab therapy (at the time of writing, on-going for 1 year). No further complications of TMA have occurred. CONCLUSION: Severe cardiac involvement in a child with aHUS is an important indication for prompt, first-line treatment with eculizumab, resulting in rapid normalisation of cardiac function.

Does dysregulated complement activation contribute to haemolytic uraemic syndrome secondary to Streptococcus pneumoniae?

We describe two patients with haemolytic uraemic syndrome (HUS) associated with invasive Streptococcus pneumoniae infection. Both patients had transiently reduced serum concentrations of complement C3. One had reduced expression of CD46 and never recovered renal function. No constitutive defect in regulation of the alternative pathway of complement activation was demonstrated in the second patient but there was an apparent improvement in her condition after administration of eculizumab. The most widely accepted mechanism for pneumococcal HUS is endothelial cell damage by pre-formed antibodies against the Thomsen-Friedenreich antigen. This explanation does not bear rigorous scrutiny. We postulate that transiently dysregulated complement activation may play a role in the pathogenesis of pneumococcal disease. We further postulate that the mechanism could be enhanced binding of factor H to the neuraminidase-altered surface of endothelial cells or reduced binding of factor H to the endothelial cell surface mediated by competitive binding of factor H by pneumococcal surface protein C (pspC).

The effect of heparin on graft thrombosis in pediatric renal allografts.

Graft thrombosis is an important cause of early (<4 weeks) renal graft loss. Reports show that heparin reduces the incidence of early renal allograft thrombosis. Routine peri-operative administration of unfractionated heparin was introduced in our unit in 1994. We conducted a retrospective study of 254 transplants, undertaken in children, between 1987 and 2000. There were 126 children who did not receive heparin (group 1) and 128 who did (group 2). Recipient characteristics and immunosuppression were similar in both groups. The incidence of graft loss secondary to thrombosis was compared between the groups. Variables previously identified with increased risk of graft loss, including donor age, recipient age, cold ischaemia time (CIT), multiple donor vessels, surgical complications, and side of graft donation, were examined using logistic regression. Thrombosis occurred in 14 grafts in group 1 and 11 grafts in group 2 (odds ratio 0.7, 95% confidence interval 0.3-1.6, P=not significant). The mean time to graft loss was similar in groups 1 and 2 (6.6, SD 3.9, range 2-12 days and 7.9, SD 4.4, range 1-14 days, respectively) ( P=0.445). Young recipient age ( P=0.006), young donor age ( P=0.009), increasing CIT ( P=0.007), and surgical complications ( P=0.002) increased the risk of graft thrombosis. A reduction in the incidence of early renal allograft thrombosis upon introduction of heparin was not demonstrated.