Rukshana Shroff | University College London (original) (raw)
Papers by Rukshana Shroff
Pediatric nephrology, Feb 13, 2024
Despite significant medical and technical improvements in the field of dialysis, the morbidity an... more Despite significant medical and technical improvements in the field of dialysis, the morbidity and mortality among patients with chronic kidney disease (CKD) stage 5 on dialysis remains extremely high. Hemodiafiltration (HDF), a dialysis method that combines the two main principles of hemodialysis (HD) and hemofiltration-diffusion and convection-has had a positive impact on survival when delivered with a high convective dose. Improved outcomes with HDF have been attributed to the following factors: HDF removes middle molecular weight uremic toxins including inflammatory cytokines, increases hemodynamic stability, and reduces inflammation and oxidative stress compared to conventional HD. Two randomized trials in adults have shown improved survival with HDF compared to high-flux HD. A large prospective cohort study in children has shown that HDF attenuated the progression of cardiovascular disease, improved bone turnover and growth, reduced inflammation, and improved blood pressure control compared to conventional HD. Importantly, children on HDF reported fewer headaches, dizziness, and cramps; had increased physical activity; and improved school attendance compared to those on HD. In this educational review, we discuss the technical aspects of HDF and results from pediatric studies, comparing outcomes on HDF vs. conventional HD. Convective volume, the cornerstone of treatment with HDF and a key determinant of outcomes in adult randomized trials, is discussed in detail, including the practical aspects of achieving an optimal convective volume.
Pediatric nephrology, Feb 13, 2024
Background Gastrostomy tube (GT) feeding is used to promote nutrition and growth in children with... more Background Gastrostomy tube (GT) feeding is used to promote nutrition and growth in children with chronic kidney disease (CKD). We explored the relationship between gastrostomy feeding and growth parameters in children with CKD, looking specifically at the nutritional composition of feeds. Methods Children with CKD stages 3-5 or on dialysis in a tertiary children's kidney unit were studied. Data on anthropometry, biochemistry, and nutritional composition of feeds were collected from the time of GT insertion for 3 years or until transplantation. Results Forty children (18 female) were included. Nineteen children were on peritoneal dialysis, 8 on hemodialysis, and 13 had CKD stages 3-5. The median (interquartile range [IQR]) age at GT insertion was 1.26 (0.61-3.58) years, with 31 (77.5%) under 5 years of age. The median duration of gastrostomy feeding was 5.32 (3.05-6.31) years. None received growth hormone treatment. Children showed a significant increase in weight standard deviation score (SDS) (p = 0.0005), weight-for-height SDS (p = 0.0007) and body mass index (BMI) SDS (p < 0.0001). None of the children developed obesity. Although not statistically significant, the median height-SDS increased into the normal range (from-2.29 to-1.85). Weight-SDS positively correlated with the percentage of energy requirements from feeds (p = 0.02), and the BMI-SDS correlated with the percentage of total energy intake as fat (p < 0.001). Conclusion GT feeding improves weight-SDS and BMI-SDS without leading to obesity. GT feeding improved height-SDS but this did not reach statistical significance, suggesting that factors in addition to nutritional optimization need to be considered for statural growth.
Pediatric Nephrology, Nov 21, 2013
Background. Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysi... more Background. Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that is associated with significant morbidity and mortality in adults. There are scarce data for children. We performed a 10-year survey to determine the prevalence, risk factors and outcome for EPS in children. Methods. Chronic PD patients in 14 dialysis units participating in the European Paediatric Dialysis Working Group between January 2001 and December 2010 were included in this study. Results. Twenty-two cases of EPS were reported (prevalence 1.5%; 8.7 per 1000 patient-years on PD). Median PD vintage was 5.
Nephrology Dialysis Transplantation, Aug 23, 2022
During conflicts, people with kidney disease, either those remaining in the affected zones or tho... more During conflicts, people with kidney disease, either those remaining in the affected zones or those who are displaced, may be exposed to additional threats because of medical and logistical challenges. Acute kidney injury developing on the battlefield, in field hospitals or in higher-level hospital settings is characterized by poor outcomes. People with chronic kidney disease may experience treatment interruptions, contributing to worsening kidney function. Patients living on dialysis or with a functioning graft may experience limitations of dialysis possibilities or availability of immunosuppressive medications, increasing the risk of severe complications including death. When patients must flee, these threats are compounded by unhealthy and insecure conditions both during displacement and/or at their destination. Measures to attenuate these risks may only be partially effective. Local preparedness for overall and medical/kidney-related disaster response is essential. Due to limitations in supply, adjustments in dialysis frequency or dose, switching between hemodialysis and peritoneal dialysis and changes in immunosuppressive regimens may be required. Telemedicine (if possible) may be useful to support inexperienced local physicians in managing medical and logistical challenges. Limited treatment possibilities during warfare may necessitate referral of patients to distant higher-level hospitals, once urgent care has been initiated. Preparation for disasters should occur ahead of time. Inclusion of disaster nephrology in medical and nursing curricula and training of patients, families and others on self-care and medical practice in austere settings may enhance awareness and preparedness, support best practices adapted to the demanding circumstances and prepare non-professionals to lend support.
Scientific Reports, May 28, 2019
Autosomal recessive polycystic kidney disease (ARpKD) is a severe pediatric hepatorenal disorder ... more Autosomal recessive polycystic kidney disease (ARpKD) is a severe pediatric hepatorenal disorder with pronounced phenotypic variability. A substantial number of patients with early diagnosis reaches adulthood and some patients are not diagnosed until adulthood. Yet, clinical knowledge about adult ARpKD patients is scarce. Here, we describe forty-nine patients with longitudinal follow-up into young adulthood that were identified in the international ARPKD cohort study ARegPKD. Forty-five patients were evaluated in a cross-sectional analysis at a mean age of 21.4 (±3.3) years describing hepatorenal findings. Renal function of native kidneys was within CKD stages 1 to 3 in more than 50% of the patients. Symptoms of hepatic involvement were frequently detected. Fourteen (31%) patients had undergone kidney transplantation and six patients (13%) had undergone liver transplantation or combined liver and kidney transplantation prior to the visit revealing a wide variability of clinical courses. Hepatorenal involvement and preceding complications in other organs were also evaluated in a time-to-event analysis. In summary, we characterize the broad clinical spectrum of young adult ARpKD patients. Importantly, many patients have a stable renal and hepatic situation in young adulthood. ARPKD should also be considered as a differential diagnosis in young adults with fibrocystic hepatorenal disease.
Nephrology Dialysis Transplantation, Jun 1, 2023
Figure 2: Lipidomic analysis in renal tissue. A) Total fatty acids profile in renal tissue of mal... more Figure 2: Lipidomic analysis in renal tissue. A) Total fatty acids profile in renal tissue of male and female animals in percentage relative area (RA %). a, M-SD vs. M-HFD p< 0.001; b, F-HFD vs. F-SD p< 0.001 and vs. F-SD-OVX p< 0.05; c, F-HFD-OVX vs. F-SD p≤ 0.001 and vs. F-SD-OVX p≤ 0.001; d, M-SD vs. M-HFD p< 0.05; e, F-HFD vs. F-SD and vs F-SD-OVX p< 0.05; f, F-HFD-OVX vs. F-SD and vs. F-SD-OVX p≤ 0.01. B) Percentage Relative Area (RA %) of fatty acid profile from phosphatidylcholine; C) phosphatidylethanolamine D) and triglycerides in renal tissue. Data are represented as mean ± standard deviation. g, F-HFD vs. F-HFD-OVX and vs. F-SD p≤ 0.05. h, F-HFD-OVX vs. F-HFD and vs. F-SD-OVX p≤ 0.05. i, F-HFD-OVX vs. F-HFD and vs. F-SD-OVX p≤ 0.05. j, F-HFD vs. F-SD p≤ 0.05. k, F-HFD vs. F-SD and vs. F-SD-OVX p≤ 0.05. l, F-HFD-OVX vs. F-SD-OVX and vs. F-SD p≤ 0.05. m, F-HFD-OVX vs. F-SD-OVX and vs. F-SD p≤ 0.05 and vs. F-HFD. p = 0.052. n, F-HFD-OVX vs. F-SD-OVX and vs. F-SD p≤ 0.05 and vs. F-HFD p = 0.058.
Pediatric Nephrology, Mar 20, 2023
The nutritional management of children with acute kidney injury (AKI) is complex. The dynamic nat... more The nutritional management of children with acute kidney injury (AKI) is complex. The dynamic nature of AKI necessitates frequent nutritional assessments and adjustments in management. Dietitians providing medical nutrition therapies to this patient population must consider the interaction of medical treatments and AKI status to effectively support both the nutrition status of patients with AKI as well as limit adverse metabolic derangements associated with inappropriately prescribed nutrition support. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPR) for the nutritional management of children with AKI. We address the need for intensive collaboration between dietitians and physicians so that nutritional management is optimized in line with AKI medical treatments. We focus on key challenges faced by dietitians regarding nutrition assessment. Furthermore, we address how nutrition support should be provided to children with AKI while taking into account the effect of various medical treatment modalities of AKI on nutritional needs. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. Statements with a low grade or those that are opinionbased must be carefully considered and adapted to individual patient needs, based on the clinical judgment of the treating physician and dietitian. Research recommendations are provided. CPRs will be regularly audited and updated by the PRNT.
Pediatric Nephrology, Jan 25, 2022
The quest for precision Precision medicine holds the promise of individualized care. Thus, while ... more The quest for precision Precision medicine holds the promise of individualized care. Thus, while traditional evidence-based medicine, with its reliance on large randomized controlled trials, can only provide prognostic and treatment information for a defined group of patients, precision medicine aims to go beyond that, by providing accurate information on treatment response and prognosis for each individual patient. Thereby, precision medicine tries to address the problem of the large spectrum of severity within a given clinical group, by identifying the genetic and environmental factors that contribute to the phenotypic diversity. Imagine that you could with reasonable accuracy predict beforehand whether a patient with, for instance, IgA nephropathy will eventually have spontaneous resolution or progressive chronic kidney disease, whether this will respond to immunosuppression and, if so, which drug will work best with the least side effects! Or, as we will discuss here, whether a patient with kidney failure is likely to do well on peritoneal dialysis (PD) or not and with what kind of PD prescription.
Pediatric Nephrology, May 15, 2020
Nephrology Dialysis Transplantation, Mar 17, 2023
People living with kidney disease are among the most vulnerable groups at the time of natural or ... more People living with kidney disease are among the most vulnerable groups at the time of natural or man-made disasters. In addition to their unpredictable course, armed-conflicts impose a major threat given disruption of infrastructure, sanitation, access to food, water, and medical care. The ongoing war in Ukraine has once more demonstrated the importance of preparedness, organization, coordination and solidarity during disasters. People living with kidney disease face serious challenges given their dependence on life-saving treatment, irrespective of whether they remain in the war zone, or are displaced internally or externally. This affects especially those requiring kidney replacement therapy (KRT), dialysis or transplantation, but also patients with other kidney diseases and the medical staff who take care of them. The European Renal Association (ERA) assigned a Renal Disaster Relief Task Force (RDRTF) dedicated to support the people living with kidney disease and the nephrology community in Ukraine, soon after the war started. This report summarizes the major challenges faced, actions taken, and lessons learnt by this Task Force. We anticipate that the experience will help to increase preparedness and to mitigate the devastating effects of armed conflicts on the kidney community in the future and propose to establish an international collaboration to extend this effort to other parts of the world facing similar challenges.
Pediatric Nephrology, Mar 17, 2021
Dyskalemias are often seen in children with chronic kidney disease (CKD). While hyperkalemia is c... more Dyskalemias are often seen in children with chronic kidney disease (CKD). While hyperkalemia is common, with an increasing prevalence as glomerular filtration rate declines, hypokalemia may also occur, particularly in children with renal tubular disorders and those on intensive dialysis regimens. Dietary assessment and adjustment of potassium intake is critically important in children with CKD as hyperkalemia can be life-threatening. Manipulation of dietary potassium can be challenging as it may affect the intake of other nutrients and reduce palatability. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPRs) for the dietary management of potassium in children with CKD stages 2-5 and on dialysis (CKD2-5D). We describe the assessment of dietary potassium intake, requirements for potassium in healthy children, and the dietary management of hypo-and hyperkalemia in children with CKD2-5D. Common potassium containing foods are described and approaches to adjusting potassium intake that can be incorporated into everyday practice discussed. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. Statements with a low grade or those that are opinion-based must be carefully considered and adapted to individual patient needs, based on the clinical judgment of the treating physician and dietitian. These CPRs will be regularly audited and updated by the PRNT.
Pediatric Nephrology, Jul 29, 2020
The nutritional prescription (whether in the form of food or liquid formulas) may be taken orally... more The nutritional prescription (whether in the form of food or liquid formulas) may be taken orally when a child has the capacity for spontaneous intake by mouth, but may need to be administered partially or completely by nasogastric tube or gastrostomy device ("enteral tube feeding"). The relative use of each of these methods varies both within and between countries. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPRs) based on evidence where available, or on the expert opinion of the Taskforce members, using a Delphi process to seek consensus from the wider community of experts in the field. We present CPRs for delivery of the nutritional prescription via enteral tube feeding to children with chronic kidney disease stages 2-5 and on dialysis. We address the types of enteral feeding tubes, when they should be used, placement techniques, recommendations and contraindications for their use, and evidence for their effects on growth parameters. Statements with a low grade of evidence, or based on opinion, must be considered and adapted for the individual patient by the treating physician and dietitian according to their clinical judgement. Research recommendations have been suggested. The CPRs will be regularly audited and updated by the PRNT.
Springer eBooks, Nov 7, 2011
ABSTRACT We describe the outcome since 1984 of all children receiving chronic dialysis in our cen... more ABSTRACT We describe the outcome since 1984 of all children receiving chronic dialysis in our centre for >3 months with a minimum follow-up of 5 (median 7.2) years. There were 98 children (61 boys), with a median age at the start of dialysis of 4.2 (range: birth to 16.2) years. Twenty-one children started dialysis at <1 year of age and 54 under <5 years. Thirty children had significant comorbidity. The median time on dialysis was 1.4 (0.3 to 14.4) years, giving a total dialysis experience of 296 patient-years. Fifty-three children received a renal transplant as their first change of treatment modality, but 31 switched between PD and HD, with a total of 54 changes of dialysis modality pre-transplantation. Twenty-one of the transplanted patients (39%) returned to dialysis. There were a total of 115 transplants in 88 patients. There was a positive increase for both the weight and height SDS for all the age groups while on dialysis, but this did not reach statistical significance. There were 17 deaths over the 20-year study period; of these, 10 died on dialysis. The overall patient survival was 83%. The mortality rate was 2.7 times greater in children who required renal replacement therapy under the age of 5 years. Of the deaths, 76% were in association with comorbid conditions. In conclusion, both a younger age at the start of renal replacement therapy and comorbidity are significant risk factors for death. The number of returnees to dialysis highlights the importance of conserving dialysis access.
Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis
[SU-FC093] Fetuin-A and Osteoprotegerin Are Associated with Vascular Stiffness and Calcification ... more [SU-FC093] Fetuin-A and Osteoprotegerin Are Associated with Vascular Stiffness and Calcification in Children on Dialysis.R. Shroff, V. Shah, M. Schoppet, L. Hofbauer, G. Hawa, L. Schurgers, A. Donald, C. Shanahan, J. Deanfield, L. Rees Nephrourology Unit, Great Ormond Street Hosp, London, United Kingdom; Department of Internal Medicine, Philipps-Univ, Marburg, Germany; Division of Endocrinology Metabolic Bone Disease, Technical Univ of Dresden, Germany; Biomedica, Vienna, Austria; CARIM and VitaK, Univ of Maastricht, Netherlands; Vascular Physiology Unit, Institute of Child Health, United Kingdom; Dept of Medicine, Univ of Cambridge, United KingdomMedial calcification of vessels occurs in the majority of patients with chronic kidney disease, but a subset of patients do not develop calcification despite exposure to a similar uraemic environment. We studied the impact of physiological inhibitors of calcification on vascular measures in children on dialysis.61 children between 5 18 yrs with dialysis vintage >3months were studied, comparing with 40 age-matched controls. Levels of Fetuin-A, osteoprotegerin (OPG), and undercarboxylated Matrix-Gla Protein (ucMGP) were measured and correlated with carotid intima media thickness (IMT), pulse wave velocity (PWV) and coronary calcification on multi-slice CT scan.Fetuin-A levels were age-dependent and unexpectedly higher in dialysis patients than controls (0.840.3 vs 0.560.1 gm/L, p<0.0001). OPG levels were higher in patients than controls (6.82.2 vs 5.31.2 pmol/L, p<0.001), particularly in those on haemodialysis, and ucMGP levels were lower in patients than controls (23211.6 vs 56028.3 nM, p=0.01). Fetuin-A positively correlated with dialysis vintage (p=0.0013), and negatively with Ca x PO4 product (p<0.05) and PTH (p=0.03).Aortic PWV showed a negative correlation with Fetuin-A (p=0.02), and a positive correlation with OPG levels (p=0.04). Patients with calcification (n=13) had lower Fetuin-A (0.690.2 vs 0.890.3 gm/L, p=0.03) and higher OPG (7.22.2 vs 6.52.1 pmol/L, p=0.002) than those without calcification.This study shows for the first time that inhibitors of calcification impact on vascular stiffness and calcification in children on dialysis. Children may have an upregulation of Fetuin-A as a protective response to the pro-inflammatory and pro-calcific uraemic environment.
Nephrology Dialysis Transplantation, 2020
Mineral and bone disorder (MBD) is widely prevalent in children with chronic kidney disease (CKD)... more Mineral and bone disorder (MBD) is widely prevalent in children with chronic kidney disease (CKD) and is associated with significant morbidity. CKD may cause disturbances in bone remodelling/modelling, which are more pronounced in the growing skeleton, manifesting as short stature, bone pain and deformities, fractures, slipped epiphyses and ectopic calcifications. Although assessment of bone health is a key element in the clinical care of children with CKD, it remains a major challenge for physicians. On the one hand, bone biopsy with histomorphometry is the gold standard for assessing bone health, but it is expensive, invasive and requires expertise in the interpretation of bone histology. On the other hand, currently available non-invasive measures, including dual-energy X-ray absorptiometry and biomarkers of bone formation/resorption, are affected by growth and pubertal status and have limited sensitivity and specificity in predicting changes in bone turnover and mineralization. ...
Pediatric nephrology, Feb 13, 2024
Despite significant medical and technical improvements in the field of dialysis, the morbidity an... more Despite significant medical and technical improvements in the field of dialysis, the morbidity and mortality among patients with chronic kidney disease (CKD) stage 5 on dialysis remains extremely high. Hemodiafiltration (HDF), a dialysis method that combines the two main principles of hemodialysis (HD) and hemofiltration-diffusion and convection-has had a positive impact on survival when delivered with a high convective dose. Improved outcomes with HDF have been attributed to the following factors: HDF removes middle molecular weight uremic toxins including inflammatory cytokines, increases hemodynamic stability, and reduces inflammation and oxidative stress compared to conventional HD. Two randomized trials in adults have shown improved survival with HDF compared to high-flux HD. A large prospective cohort study in children has shown that HDF attenuated the progression of cardiovascular disease, improved bone turnover and growth, reduced inflammation, and improved blood pressure control compared to conventional HD. Importantly, children on HDF reported fewer headaches, dizziness, and cramps; had increased physical activity; and improved school attendance compared to those on HD. In this educational review, we discuss the technical aspects of HDF and results from pediatric studies, comparing outcomes on HDF vs. conventional HD. Convective volume, the cornerstone of treatment with HDF and a key determinant of outcomes in adult randomized trials, is discussed in detail, including the practical aspects of achieving an optimal convective volume.
Pediatric nephrology, Feb 13, 2024
Background Gastrostomy tube (GT) feeding is used to promote nutrition and growth in children with... more Background Gastrostomy tube (GT) feeding is used to promote nutrition and growth in children with chronic kidney disease (CKD). We explored the relationship between gastrostomy feeding and growth parameters in children with CKD, looking specifically at the nutritional composition of feeds. Methods Children with CKD stages 3-5 or on dialysis in a tertiary children's kidney unit were studied. Data on anthropometry, biochemistry, and nutritional composition of feeds were collected from the time of GT insertion for 3 years or until transplantation. Results Forty children (18 female) were included. Nineteen children were on peritoneal dialysis, 8 on hemodialysis, and 13 had CKD stages 3-5. The median (interquartile range [IQR]) age at GT insertion was 1.26 (0.61-3.58) years, with 31 (77.5%) under 5 years of age. The median duration of gastrostomy feeding was 5.32 (3.05-6.31) years. None received growth hormone treatment. Children showed a significant increase in weight standard deviation score (SDS) (p = 0.0005), weight-for-height SDS (p = 0.0007) and body mass index (BMI) SDS (p < 0.0001). None of the children developed obesity. Although not statistically significant, the median height-SDS increased into the normal range (from-2.29 to-1.85). Weight-SDS positively correlated with the percentage of energy requirements from feeds (p = 0.02), and the BMI-SDS correlated with the percentage of total energy intake as fat (p < 0.001). Conclusion GT feeding improves weight-SDS and BMI-SDS without leading to obesity. GT feeding improved height-SDS but this did not reach statistical significance, suggesting that factors in addition to nutritional optimization need to be considered for statural growth.
Pediatric Nephrology, Nov 21, 2013
Background. Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysi... more Background. Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that is associated with significant morbidity and mortality in adults. There are scarce data for children. We performed a 10-year survey to determine the prevalence, risk factors and outcome for EPS in children. Methods. Chronic PD patients in 14 dialysis units participating in the European Paediatric Dialysis Working Group between January 2001 and December 2010 were included in this study. Results. Twenty-two cases of EPS were reported (prevalence 1.5%; 8.7 per 1000 patient-years on PD). Median PD vintage was 5.
Nephrology Dialysis Transplantation, Aug 23, 2022
During conflicts, people with kidney disease, either those remaining in the affected zones or tho... more During conflicts, people with kidney disease, either those remaining in the affected zones or those who are displaced, may be exposed to additional threats because of medical and logistical challenges. Acute kidney injury developing on the battlefield, in field hospitals or in higher-level hospital settings is characterized by poor outcomes. People with chronic kidney disease may experience treatment interruptions, contributing to worsening kidney function. Patients living on dialysis or with a functioning graft may experience limitations of dialysis possibilities or availability of immunosuppressive medications, increasing the risk of severe complications including death. When patients must flee, these threats are compounded by unhealthy and insecure conditions both during displacement and/or at their destination. Measures to attenuate these risks may only be partially effective. Local preparedness for overall and medical/kidney-related disaster response is essential. Due to limitations in supply, adjustments in dialysis frequency or dose, switching between hemodialysis and peritoneal dialysis and changes in immunosuppressive regimens may be required. Telemedicine (if possible) may be useful to support inexperienced local physicians in managing medical and logistical challenges. Limited treatment possibilities during warfare may necessitate referral of patients to distant higher-level hospitals, once urgent care has been initiated. Preparation for disasters should occur ahead of time. Inclusion of disaster nephrology in medical and nursing curricula and training of patients, families and others on self-care and medical practice in austere settings may enhance awareness and preparedness, support best practices adapted to the demanding circumstances and prepare non-professionals to lend support.
Scientific Reports, May 28, 2019
Autosomal recessive polycystic kidney disease (ARpKD) is a severe pediatric hepatorenal disorder ... more Autosomal recessive polycystic kidney disease (ARpKD) is a severe pediatric hepatorenal disorder with pronounced phenotypic variability. A substantial number of patients with early diagnosis reaches adulthood and some patients are not diagnosed until adulthood. Yet, clinical knowledge about adult ARpKD patients is scarce. Here, we describe forty-nine patients with longitudinal follow-up into young adulthood that were identified in the international ARPKD cohort study ARegPKD. Forty-five patients were evaluated in a cross-sectional analysis at a mean age of 21.4 (±3.3) years describing hepatorenal findings. Renal function of native kidneys was within CKD stages 1 to 3 in more than 50% of the patients. Symptoms of hepatic involvement were frequently detected. Fourteen (31%) patients had undergone kidney transplantation and six patients (13%) had undergone liver transplantation or combined liver and kidney transplantation prior to the visit revealing a wide variability of clinical courses. Hepatorenal involvement and preceding complications in other organs were also evaluated in a time-to-event analysis. In summary, we characterize the broad clinical spectrum of young adult ARpKD patients. Importantly, many patients have a stable renal and hepatic situation in young adulthood. ARPKD should also be considered as a differential diagnosis in young adults with fibrocystic hepatorenal disease.
Nephrology Dialysis Transplantation, Jun 1, 2023
Figure 2: Lipidomic analysis in renal tissue. A) Total fatty acids profile in renal tissue of mal... more Figure 2: Lipidomic analysis in renal tissue. A) Total fatty acids profile in renal tissue of male and female animals in percentage relative area (RA %). a, M-SD vs. M-HFD p< 0.001; b, F-HFD vs. F-SD p< 0.001 and vs. F-SD-OVX p< 0.05; c, F-HFD-OVX vs. F-SD p≤ 0.001 and vs. F-SD-OVX p≤ 0.001; d, M-SD vs. M-HFD p< 0.05; e, F-HFD vs. F-SD and vs F-SD-OVX p< 0.05; f, F-HFD-OVX vs. F-SD and vs. F-SD-OVX p≤ 0.01. B) Percentage Relative Area (RA %) of fatty acid profile from phosphatidylcholine; C) phosphatidylethanolamine D) and triglycerides in renal tissue. Data are represented as mean ± standard deviation. g, F-HFD vs. F-HFD-OVX and vs. F-SD p≤ 0.05. h, F-HFD-OVX vs. F-HFD and vs. F-SD-OVX p≤ 0.05. i, F-HFD-OVX vs. F-HFD and vs. F-SD-OVX p≤ 0.05. j, F-HFD vs. F-SD p≤ 0.05. k, F-HFD vs. F-SD and vs. F-SD-OVX p≤ 0.05. l, F-HFD-OVX vs. F-SD-OVX and vs. F-SD p≤ 0.05. m, F-HFD-OVX vs. F-SD-OVX and vs. F-SD p≤ 0.05 and vs. F-HFD. p = 0.052. n, F-HFD-OVX vs. F-SD-OVX and vs. F-SD p≤ 0.05 and vs. F-HFD p = 0.058.
Pediatric Nephrology, Mar 20, 2023
The nutritional management of children with acute kidney injury (AKI) is complex. The dynamic nat... more The nutritional management of children with acute kidney injury (AKI) is complex. The dynamic nature of AKI necessitates frequent nutritional assessments and adjustments in management. Dietitians providing medical nutrition therapies to this patient population must consider the interaction of medical treatments and AKI status to effectively support both the nutrition status of patients with AKI as well as limit adverse metabolic derangements associated with inappropriately prescribed nutrition support. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPR) for the nutritional management of children with AKI. We address the need for intensive collaboration between dietitians and physicians so that nutritional management is optimized in line with AKI medical treatments. We focus on key challenges faced by dietitians regarding nutrition assessment. Furthermore, we address how nutrition support should be provided to children with AKI while taking into account the effect of various medical treatment modalities of AKI on nutritional needs. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. Statements with a low grade or those that are opinionbased must be carefully considered and adapted to individual patient needs, based on the clinical judgment of the treating physician and dietitian. Research recommendations are provided. CPRs will be regularly audited and updated by the PRNT.
Pediatric Nephrology, Jan 25, 2022
The quest for precision Precision medicine holds the promise of individualized care. Thus, while ... more The quest for precision Precision medicine holds the promise of individualized care. Thus, while traditional evidence-based medicine, with its reliance on large randomized controlled trials, can only provide prognostic and treatment information for a defined group of patients, precision medicine aims to go beyond that, by providing accurate information on treatment response and prognosis for each individual patient. Thereby, precision medicine tries to address the problem of the large spectrum of severity within a given clinical group, by identifying the genetic and environmental factors that contribute to the phenotypic diversity. Imagine that you could with reasonable accuracy predict beforehand whether a patient with, for instance, IgA nephropathy will eventually have spontaneous resolution or progressive chronic kidney disease, whether this will respond to immunosuppression and, if so, which drug will work best with the least side effects! Or, as we will discuss here, whether a patient with kidney failure is likely to do well on peritoneal dialysis (PD) or not and with what kind of PD prescription.
Pediatric Nephrology, May 15, 2020
Nephrology Dialysis Transplantation, Mar 17, 2023
People living with kidney disease are among the most vulnerable groups at the time of natural or ... more People living with kidney disease are among the most vulnerable groups at the time of natural or man-made disasters. In addition to their unpredictable course, armed-conflicts impose a major threat given disruption of infrastructure, sanitation, access to food, water, and medical care. The ongoing war in Ukraine has once more demonstrated the importance of preparedness, organization, coordination and solidarity during disasters. People living with kidney disease face serious challenges given their dependence on life-saving treatment, irrespective of whether they remain in the war zone, or are displaced internally or externally. This affects especially those requiring kidney replacement therapy (KRT), dialysis or transplantation, but also patients with other kidney diseases and the medical staff who take care of them. The European Renal Association (ERA) assigned a Renal Disaster Relief Task Force (RDRTF) dedicated to support the people living with kidney disease and the nephrology community in Ukraine, soon after the war started. This report summarizes the major challenges faced, actions taken, and lessons learnt by this Task Force. We anticipate that the experience will help to increase preparedness and to mitigate the devastating effects of armed conflicts on the kidney community in the future and propose to establish an international collaboration to extend this effort to other parts of the world facing similar challenges.
Pediatric Nephrology, Mar 17, 2021
Dyskalemias are often seen in children with chronic kidney disease (CKD). While hyperkalemia is c... more Dyskalemias are often seen in children with chronic kidney disease (CKD). While hyperkalemia is common, with an increasing prevalence as glomerular filtration rate declines, hypokalemia may also occur, particularly in children with renal tubular disorders and those on intensive dialysis regimens. Dietary assessment and adjustment of potassium intake is critically important in children with CKD as hyperkalemia can be life-threatening. Manipulation of dietary potassium can be challenging as it may affect the intake of other nutrients and reduce palatability. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPRs) for the dietary management of potassium in children with CKD stages 2-5 and on dialysis (CKD2-5D). We describe the assessment of dietary potassium intake, requirements for potassium in healthy children, and the dietary management of hypo-and hyperkalemia in children with CKD2-5D. Common potassium containing foods are described and approaches to adjusting potassium intake that can be incorporated into everyday practice discussed. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. Statements with a low grade or those that are opinion-based must be carefully considered and adapted to individual patient needs, based on the clinical judgment of the treating physician and dietitian. These CPRs will be regularly audited and updated by the PRNT.
Pediatric Nephrology, Jul 29, 2020
The nutritional prescription (whether in the form of food or liquid formulas) may be taken orally... more The nutritional prescription (whether in the form of food or liquid formulas) may be taken orally when a child has the capacity for spontaneous intake by mouth, but may need to be administered partially or completely by nasogastric tube or gastrostomy device ("enteral tube feeding"). The relative use of each of these methods varies both within and between countries. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPRs) based on evidence where available, or on the expert opinion of the Taskforce members, using a Delphi process to seek consensus from the wider community of experts in the field. We present CPRs for delivery of the nutritional prescription via enteral tube feeding to children with chronic kidney disease stages 2-5 and on dialysis. We address the types of enteral feeding tubes, when they should be used, placement techniques, recommendations and contraindications for their use, and evidence for their effects on growth parameters. Statements with a low grade of evidence, or based on opinion, must be considered and adapted for the individual patient by the treating physician and dietitian according to their clinical judgement. Research recommendations have been suggested. The CPRs will be regularly audited and updated by the PRNT.
Springer eBooks, Nov 7, 2011
ABSTRACT We describe the outcome since 1984 of all children receiving chronic dialysis in our cen... more ABSTRACT We describe the outcome since 1984 of all children receiving chronic dialysis in our centre for >3 months with a minimum follow-up of 5 (median 7.2) years. There were 98 children (61 boys), with a median age at the start of dialysis of 4.2 (range: birth to 16.2) years. Twenty-one children started dialysis at <1 year of age and 54 under <5 years. Thirty children had significant comorbidity. The median time on dialysis was 1.4 (0.3 to 14.4) years, giving a total dialysis experience of 296 patient-years. Fifty-three children received a renal transplant as their first change of treatment modality, but 31 switched between PD and HD, with a total of 54 changes of dialysis modality pre-transplantation. Twenty-one of the transplanted patients (39%) returned to dialysis. There were a total of 115 transplants in 88 patients. There was a positive increase for both the weight and height SDS for all the age groups while on dialysis, but this did not reach statistical significance. There were 17 deaths over the 20-year study period; of these, 10 died on dialysis. The overall patient survival was 83%. The mortality rate was 2.7 times greater in children who required renal replacement therapy under the age of 5 years. Of the deaths, 76% were in association with comorbid conditions. In conclusion, both a younger age at the start of renal replacement therapy and comorbidity are significant risk factors for death. The number of returnees to dialysis highlights the importance of conserving dialysis access.
Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis
[SU-FC093] Fetuin-A and Osteoprotegerin Are Associated with Vascular Stiffness and Calcification ... more [SU-FC093] Fetuin-A and Osteoprotegerin Are Associated with Vascular Stiffness and Calcification in Children on Dialysis.R. Shroff, V. Shah, M. Schoppet, L. Hofbauer, G. Hawa, L. Schurgers, A. Donald, C. Shanahan, J. Deanfield, L. Rees Nephrourology Unit, Great Ormond Street Hosp, London, United Kingdom; Department of Internal Medicine, Philipps-Univ, Marburg, Germany; Division of Endocrinology Metabolic Bone Disease, Technical Univ of Dresden, Germany; Biomedica, Vienna, Austria; CARIM and VitaK, Univ of Maastricht, Netherlands; Vascular Physiology Unit, Institute of Child Health, United Kingdom; Dept of Medicine, Univ of Cambridge, United KingdomMedial calcification of vessels occurs in the majority of patients with chronic kidney disease, but a subset of patients do not develop calcification despite exposure to a similar uraemic environment. We studied the impact of physiological inhibitors of calcification on vascular measures in children on dialysis.61 children between 5 18 yrs with dialysis vintage >3months were studied, comparing with 40 age-matched controls. Levels of Fetuin-A, osteoprotegerin (OPG), and undercarboxylated Matrix-Gla Protein (ucMGP) were measured and correlated with carotid intima media thickness (IMT), pulse wave velocity (PWV) and coronary calcification on multi-slice CT scan.Fetuin-A levels were age-dependent and unexpectedly higher in dialysis patients than controls (0.840.3 vs 0.560.1 gm/L, p<0.0001). OPG levels were higher in patients than controls (6.82.2 vs 5.31.2 pmol/L, p<0.001), particularly in those on haemodialysis, and ucMGP levels were lower in patients than controls (23211.6 vs 56028.3 nM, p=0.01). Fetuin-A positively correlated with dialysis vintage (p=0.0013), and negatively with Ca x PO4 product (p<0.05) and PTH (p=0.03).Aortic PWV showed a negative correlation with Fetuin-A (p=0.02), and a positive correlation with OPG levels (p=0.04). Patients with calcification (n=13) had lower Fetuin-A (0.690.2 vs 0.890.3 gm/L, p=0.03) and higher OPG (7.22.2 vs 6.52.1 pmol/L, p=0.002) than those without calcification.This study shows for the first time that inhibitors of calcification impact on vascular stiffness and calcification in children on dialysis. Children may have an upregulation of Fetuin-A as a protective response to the pro-inflammatory and pro-calcific uraemic environment.
Nephrology Dialysis Transplantation, 2020
Mineral and bone disorder (MBD) is widely prevalent in children with chronic kidney disease (CKD)... more Mineral and bone disorder (MBD) is widely prevalent in children with chronic kidney disease (CKD) and is associated with significant morbidity. CKD may cause disturbances in bone remodelling/modelling, which are more pronounced in the growing skeleton, manifesting as short stature, bone pain and deformities, fractures, slipped epiphyses and ectopic calcifications. Although assessment of bone health is a key element in the clinical care of children with CKD, it remains a major challenge for physicians. On the one hand, bone biopsy with histomorphometry is the gold standard for assessing bone health, but it is expensive, invasive and requires expertise in the interpretation of bone histology. On the other hand, currently available non-invasive measures, including dual-energy X-ray absorptiometry and biomarkers of bone formation/resorption, are affected by growth and pubertal status and have limited sensitivity and specificity in predicting changes in bone turnover and mineralization. ...