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Introduction
Current institution
Publications
Publications (108)
Background
Idiopathic Overactive Bladder is the most common cause of urinary incontinence in children. Anticholinergic medications are successful in only 20% of those with daily wetting so there is a real need to find a more effective treatment for this condition. Onabotulinum toxin A injections are often used as a treatment but there have been no...
Background:
Intravesical botulinum toxin A (BtA) injection is well established in managing paediatric neuropathic detrusor overactivity (NDO). Electromotive drug administration (EMDA) is a less invasive method, which can be performed in the clinic, using pulsed electrical current for drug delivery via a urethral catheter. Few small studies report...
Cystinosis is an autosomal recessive storage disease due to impaired transport of cystine out of lysosomes. Since the accumulation of intracellular cystine affects all organs and tissues, the management of cystinosis requires a specialized multi‐disciplinary team consisting of pediatricians, nephrologists, nutritionists, ophthalmologists, endocrino...
Background
Vascular access is required for haemodialysis (HD), a form of renal replacement therapy required when a child has acute or end stage kidney disease (ESKD). Central venous catheter (CVC) line infections are the second most common cause of vascular access loss in the long term HD patient, commonly caused by poor hand hygiene practices, cli...
Introduction:
Botulinum toxin-A (BtA) has been used for refractory idiopathic overactive bladder (IOAB) in children. Data on the optimum dose success rates, duration of effect, complications and medium-term outcomes are limited. This study aims to analyse the authors' experience to provide medium-term results of BtA in symptomatic refractory patie...
In 2016, 964 children and young people aged less than 18 years were receiving long-term renal replacement therapy (RRT) for established renal failure (ERF) at UK paediatric nephrology centres. A total of 125 incident patients under 18 years commenced RRT. At the census date (31 December 2016), 77% of prevalent paediatric patients aged ,16 years had...
In 2016, the median height z-score for prevalent paediatric patients on dialysis was -1.8 and -1.1 for those with a functioning transplant (p < 0.0001). The median weight z-score for children receiving dialysis in 2016 was -1.2 compared with -0.2 for children with a functioning transplant. The median systolic blood pressure (SBP) z-score for transp...
A total of 941 children and young people aged <18 years with established renal failure (ERF) were receiving treatment at paediatric nephrology centres in 2015. At the census date (31st December 2015), 75.3% of prevalent paediatric patients aged <16 years had a functioning kidney transplant, 13.0% were receiving haemodialysis (HD) and 11.7% were rec...
The median height z-score for paediatric patients on dialysis in 2015 was-1.8 and for those with a functioning transplant-1.2. Children transplanted before the age of 12 years improved their height z-score over the subsequent five years, whereas those older than 12 years maintained their height z-score, with all transplanted patients having a simil...
Veno-occlusive disease (VOD), or sinusoidal obstruction syndrome, is a well-recognised, serious complication associated with the chemotherapy conditioning therapy used in hematopoietic stem cell transplantation (HSCT). Fluid management is typically challenging in children with this condition. We describe effective early use of peritoneal dialysis c...
A total of 917 children and young people under 18 years with established renal failure (ERF) were receiving treatment at paediatric nephrology centres in 2014. At the census date (31st December 2014), 79.3% of prevalent paediatric patients aged ,18 years had a functioning kidney transplant, 11.2% were receiving haemodialysis (HD) and 9.5% were rece...
We aimed to describe survival in European pediatric dialysis patients and compare the differential mortality risk between patients starting on hemodialysis (HD) and peritoneal dialysis (PD). Data for 6473 patients under 19 years of age or younger were extracted from the European Society of Pediatric Nephrology, the European Renal Association, and E...
The median height z-score for paediatric patients on dialysis was -2.1 and for those with a functioning transplant -1.3. Children transplanted before the age of 12 years improved their height z-score over the subsequent five years, whereas those older than 12 maintained their height z-score, with all transplanted patients having a similar median he...
The OVERT study is a pilot randomised controlled trial to compare the effectiveness of intravesical onabotulin toxin A with extended release tolterodine in the management of children aged 7-16 with Idiopathic Overactive Bladder.
The primary and secondary outcomes for this study are recorded in a bladder diary, as recommended by the International C...
Background: The Paediatric Registry analyses renal replacement
therapy (RRT) data in children. All 13 UK paediatric
nephrology centres submit electronic data. Aims: To
provide centre specific data and to determine adherence to
relevant audit standards. Methods: Data analysis to calculate
summary statistics and achievement of an audit standard.
Resu...
Introduction:
To describe the demographics of the paediatric renal replacement therapy (RRT) population under the age of 18 years in the UK and to analyse changes in demography with time.
Methods:
Data were collected from all 13 paediatric renal centres within the UK. A series of crosssectional and longitudinal analyses were performed to describ...
Introduction and Aims: The UK Renal Registry (UKRR) hosts a paediatric registry, which reports demographic, biochemical and outcomes data on all renal replacement therapy (RRT) patients under 18 years managed in UK paediatric centres. The paediatric registry was formed in 1995 and is entering its 20th year. We aim to describe the history and develo...
Urofacial syndrome (UFS) is an autosomal recessive congenital disease featuring grimacing and incomplete bladder emptying. Mutations of HPSE2, encoding heparanase 2, a heparanase 1 inhibitor, occur in UFS, but knowledge about the HPSE2 mutation spectrum is limited. Here, seven UFS kindreds with HPSE2 mutations are presented, including one with dele...
Background
Considerable disparities exist in the provision of paediatric renal replacement therapy (RRT) across Europe. This study aims to determine whether these disparities arise from geographical differences in the occurrence of renal disease, or whether country-level access-to-care factors may be responsible.
Methods
Incidence was defined as t...
Introduction and Aims: Autosomal recessive polycystic kidney disease (ARPKD) occurs in 1:20,000 live births and is the most common cystic kidney
disease in childhood. Since kidney and liver manifestations progress differently in ARPKD, the decision of the right type
of transplantation may be challenging. Most often kidney-alone transplantation (KT)...
Background:
The British Association for Paediatric Nephrology Registry was established to analyse data related to renal replacement therapy (RRT) in children. The registry receives data from the 13 paediatric nephrology centres in the UK.
Aims:
To provide centre specific data so that individual centres can reflect on the contribution that their da...
Aims:
To describe the demographics of the paediatric RRT population under the age of 16 years in the UK and to analyse changes in demography with time.
Methods:
Data were collected from all 13 paediatric renal centres within the UK. A series of cross-sectional and longitudinal analyses were performed to describe the demographics of paediatric RR...
The spectrum of indications and availability of continuous renal replacement therapy (CRRT) have increased massively over the last three decades. The availability of commercially produced equipment has reduced many of the associated risks. The mortality associated with paediatric CRRT remains significant and reflects the severity of the underlying...
Urofacial syndrome (UFS) (or Ochoa syndrome) is an autosomal-recessive disease characterized by congenital urinary bladder dysfunction, associated with a significant risk of kidney failure, and an abnormal facial expression upon smiling, laughing, and crying. We report that a subset of UFS-affected individuals have biallelic mutations in LRIG2, enc...
Aims:
To describe the demographics of the paediatric RRT population under the age of 16 years in the UK and to analyse changes in demography with time.
Methods:
Data were collected from all 13 paediatric renal centres within the UK. A series of cross-sectional and longitudinal analyses were performed to describe the demographics of prevalent pae...
Background:
The British Association for Paediatric Nephrology Registry was established to analyse data related to renal replacement therapy (RRT) for children. The registry receives data from the 13 paediatric nephrology centres in the UK.
Aim:
To provide centre specific data so that individual centres can reflect on the contribution that their...
Nephropathic cystinosis (NC) is an autosomal recessive disorder occurring in one to two per 100,000 newborns. Because of the rarity of NC, long-term outcome data are scarce.
245 NC patients from 18 countries provided data to the ESPN/ERA-EDTA registry. We matched NC patients on renal replacement therapy (RRT) to non-NC children on RRT.
Between 1979...
Hypertension is a well-known complication in children on renal replacement therapy and an important risk factor for cardiovascular disease in later life. In order to define the prevalence of and risk factors for hypertension among children, we enrolled 3337 pediatric patients from 15 countries in the ESPN/ERA-EDTA Registry of whom 464 were on hemod...
The British Association for Paediatric Nephrology Registry was established fifteen years ago to analyse data related to renal replacement therapy for children. The registry receives data from the 13 paediatric nephrology centres in the UK. In 2008 the registry was relocated to the UK Renal Registry (UKRR).
To provide centre specific data so that in...
To describe the demographics of the paediatric RRT population under the age of 16 years in the UK and to analyse changes in demography with time.
Extraction and analysis of data from the UK Renal Registry (UKRR).
There were 751 children <16 years old with established renal failure (ERF) in the UK in December 2009. The reported prevalence under the...
To evaluate the outcome of early (ER <3 months) and late (LR >3 months) episodes of corticosteroid resistant acute allograft rejection (CRR) treated with anti-thymocyte globulin (ATG) in pediatric renal allograft recipients. Retrospective study of 15 children, mean age 13.2 y, who received ATG for the treatment of biopsy proven CRR over a 15 year p...
Few studies have investigated the determinants of glomerular filtration rate (GFR) in paediatric patients starting on dialysis or with a transplant.
Data were collected as part of the European Society of Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association registry from 14 European countries and referred to...
Urinary voiding dysfunction in childhood, manifesting as incontinence, dysuria, and urinary frequency, is a common condition. Urofacial syndrome (UFS) is a rare autosomal recessive disease characterized by facial grimacing when attempting to smile and failure of the urinary bladder to void completely despite a lack of anatomical bladder outflow obs...
Established renal failure is a manageable but incurable problem. Safely transitioning young people from paediatric to adult services remains a major challenge.
The aim of our study was to determine the clinical course of children with idiopathic childhood nephrotic syndrome (ICNS) who received intravenous methylprednisolone (ivMP) following failure to achieve remission with standard oral prednisolone therapy. This study was designed as a retrospective case record review from 1993 to 2007. Sixteen children...
Purpose
Management of neuropathic bladder aims to preserve renal function, and achieve satisfactory continence. We have assessed long-term continence outcomes following bladder augmentation.
Material and Methods
We evaluated neuropathic bladder patients with bladder augmentation between 1988-2006. Patients were documented prospectively in spina bi...
To describe the demographics of the paediatric RRT population in the UK and analyse changes in demographics with time.
Extraction and analysis of data from the UK Paediatric Renal Registry and the UK Renal Registry (UKRR).
The UK paediatric established renal failure (ERF) population in December 2008 was 905 patients. The prevalence under the age of...
The British Association for Paediatric Nephrology Registry was established thirteen years ago to analyse data related to renal replacement therapy for children. The registry receives data from the 13 paediatric nephrology centres in the UK. In 2008 the registry was relocated to the UK Renal Registry (UKRR).
To provide centre specific data so that i...
To describe the demographics of the paediatric RRT population in the UK and analyse changes in demographics with time.
Extraction and analysis of data from the UK paediatric Renal Registry.
The UK paediatric established renal failure (ERF) population in April 2008 was 875 patients. The prevalence under the age of 16 years was 55 per million age rel...
Secondary hypertension in children is a rare but important cause of morbidity and mortality. Whilst severe hypertension can be symptomatic more often it is not and blood pressure measurement is mandatory in all children at risk. The vast majority of childhood secondary hypertension is related to renal disease and treatment is with angiotensin-conve...
The demography of renal failure in childhood is examined through an analysis of the UK Renal Registry data on patients in established renal failure (ERF) and studies of chronic kidney disease populations. The predominant cause is renal dysplasia and related conditions. Congenital obstructive uropathy is the third largest group overall and the secon...
Thrombosis of the inferior vena cava (IVC) has previously been considered to be a contraindication to renal transplantation in children because of the technical difficulties associated with surgery and the increased risk of graft thrombosis. We report three children with previous IVC thrombosis who underwent renal transplantation at our institution...
This retrospective study investigated the outcome of 27 children (19 male) with Henoch-Schönlein purpura nephritis (HSN) of International Study of Kidney Disease in Children (ISKDC) grade 3b or higher treated with long-term immunosuppressive therapy in a single centre over a 10-year period. The mean age at presentation was 9.7 years. The median est...
Short stature is a major problem in paediatric ERF patients with 29% of transplant patients and 41% of dialysis patients below
the second percentile for height. Only 6.5% of transplant patients and 15.5% of dialysis patients are receiving rhGH. There
is no significant difference in the height distribution of patients commencing RRT and those who ha...
The incidence and prevalence of ERF in children in the UK are relatively static at 8.0 and 47.7 per million population under the age of 15 years, respectively.
The prevalence of ERF in children from the South Asian community is almost three times that of the White population whilst the incidence is over three times that of the White population and...
Despite the universal availability of erythropoietin and intravenous iron, 14% of transplant patients and 30% of dialysis patients have a haemoglobin (Hb) <10.5 g/dl. Only 11% of anaemic transplant patients were receiving erythropoietin.
There was a linear relationship between estimated glomerular filtration rate (eGFR) and Hb with the risk of anae...
There is an ongoing debate over the radiological investigations of children with urinary tract infections (UTIs) with some authorities suggesting that ultrasound scan (USS) alone is an accurate tool to diagnose renal parenchymal scarring post-pyelonephritis. All studies on this subject have been performed at paediatric teaching centres whereas most...
Post-transplant diabetes mellitus (PTDM) has a variably reported incidence of 4-41% among adults and children. We describe our recent experience of four children with PTDM in a paediatric renal transplantation centre.
We undertook a retrospective analysis of the glycaemic status of all paediatric patients undergoing renal transplantation at our cen...
Percutaneous renal biopsy under real time ultrasound guidance is a routine procedure in pediatric nephrology and allows a histological diagnosis to be made in children with evidence of renal disease.
Retrospective case note review.
Over four years 191 renal biopsies were attempted in 116 patients; 186 biopsies were performed successfully: 102 nativ...
Setting:
Twenty-one of 209 children admitted to the intensive care unit with meningococcal septicemia developed oliguric acute renal failure necessitating renal replacement therapy.
Patients:
Twelve survivors underwent renal assessment at a median of 4.2 yrs postpresentation.
Result:
Two had abnormal glomerular filtration rate, proteinuria, an...
Complete necrosis of a transplant ureter is a rare complication that needs to be considered early in cases of severe graft dysfunction if successful surgical intervention and restoration of graft function is to be achieved. We report on two cases of this complication occurring in children and discuss the surgical management. Surgical exploration of...
Serum leptin decreases during growth hormone (hGH) treatment and pre-treatment values have been suggested as a predictor of the response to hGH in GH deficiency (GHD) but not in non-GHD syndromes. To investigate whether this holds true in children with chronic renal failure (CRF), we evaluated changes in serum leptin, insulin-like growth factor-I (...
To investigate the seroconversion rate and duration of persistence of protective antibody titres after varicella immunisation in children with renal failure.
32 children (25 end stage and 7 pre-end stage renal failure) were immunised using 2 x 2,000 plaque forming unit doses of varicella vaccine 3 months apart. Varicella antibody titres were measur...
OBJECTIVES
To investigate the seroconversion rate and duration of persistence of protective antibody titres after varicella immunisation in children with renal failure.DESIGN32 children (25 end stage and 7 pre-end stage renal failure) were immunised using 2 × 2000 plaque forming unit doses of varicella vaccine 3 months apart. Varicella antibody tit...
Few studies have investigated the incidence of seizures following renal transplantation in childhood. The aim of this study
was to determine this incidence and to identify risk factors. Retrospective casenote analysis was carried out on 119 transplants
performed in 109 children over 10 years. Twenty-one transplants (in 20 children) were complicate...
An uncharacterized circulating factor that increases vascular permeability has previously been described in childhood steroid-sensitive nephrotic syndrome (SSNS). The aim of this study was to determine whether this factor is vascular endothelial growth factor (VEGF), the recently described endothelial cell mitogen and enhancer of vascular permeabil...
Three children (two girls aged 7 and 9 years, and one boy aged 4 years) with diuretic resistant oedema in steroid resistant nephrotic syndrome were treated with a combination of intravenous mannitol and frusemide. All three responded with loss of oedema of 10% to 30% of body weight over one week. There were no complications of hypertension or hypov...
Report of chronic headache amongst children with shunted hydrocephalus is frequent, and often leads to investigation for raised intra-cranial pressure and shunt malfunction, and much debate has appeared in the literature concerning possible cause. However headache is a common neurological symptom amongst children, and studies of prevalence indicate...
The United Kingdom North West Regional Registry of patients with Neural Tube Defects was established in June 1996. The aims of the registry are to keep a record of all patients in a defined geographical location with neural tube defects and to ensure appropriate facilities and follow-up are available. During the first year of the registry 217 patie...
The frequency of bladder neuropathy and its relationship to abnormalities of the neural axis on MR scan was examined in group of 49 children with known occult spinal dysraphism and 148 children with daytime urinary incontinence.
Of the patients with known spinal dysraphism 55% had bladder neuropathy. There was no relationship between either the typ...
Management of unilateral multicystic dysplastic kidneys (MCDK) presents physicians and surgeons with a significant dilemma. Recent studies have indicated that the incidence of short term complications of MCDK is low and many authors have recommended conservative non-operative treatment. Surgery has been proposed by some because of the potential com...
Double-cuffed peritoneal catheters (DCCs) may be more effective at preventing penetrating infection than single-cuffed catheters (SCCs). The aim of this study was to see whether DCCs conferred any benefit in the pediatric population. Twenty consecutive SCCs inserted for chronic dialysis were compared with 20 subsequent DCCs. All catheters were inse...
Functional peritoneal dialysis catheter loss due to persistent or recurrent peritonitis is a common clinical problem in paediatric patients. To examine the effect of positioning of the cuff on infection and loss rate we compared catheters where the cuff was buried in the rectus sheath (deep cuffs) with those where the cuff was sutured to the extern...
Renal biopsy specimens from 51 children with steroid-sensitive nephrotic syndrome who were following a frequently relapsing or steroid-dependent course were reviewed by two histopathologists. In all cases the biopsy was performed prior to the commencement of an 8-week course of cyclophosphamide. The clinical courses of these patients both prebiopsy...
This report deals with 120 cadaveric renal transplants performed in 101 pediatric recipients in this Centre in two five-year periods, 1984 to 1988 (N = 65) and 1989 to 1993 (N = 55). In the first group transplants were allocated on the basis of best size (small donors for small recipients); in the second group priority was given to beneficial HLA m...
To document the urological problems associated with multicystic renal dysplasia (MCRD), to assess the efficacy of radiological methods for following the natural history of MCRD and to review current experience in the light of historical data regarding this condition.
From January 1982 to December 1992 data were obtained prospectively on 62 patients...
Issues raised by the recruitment of children to trials of growth hormone treatment for short stature in chronic renal failure are reported. Information needs of parents and children are discussed, the latter should take account of the children's developmental level and anticipated involvement in decision making. When the incidence of certain side e...
Objective To document the urological problems associated with multicystic renal dysplasia (MCRD), to assess the efficacy of radiological methods for following the natural history of MCRD and to review current experience in the light of historical data regarding this condition.
Patients and methods From January 1982 to December 1992 data were obtain...
A routine omentectomy is recommended in children commencing chronic peritoneal dialysis, but is not routine practice in adults. We reviewed the outcome of 66 catheters (57 straight and 9 spiral) inserted into 38 patients (median age 7.8 years, 12 patients under 2 years) over a period of 4.8 years. The aim of the study was to determine whether an om...
The case records of 72 patients attending a multidisciplinary spina bifida clinic were reviewed to discover the prevalence of renal parenchymal damage. 19.4 % of the total population had such damage.
More detailed analysis of the group according to age showed that the prevalence of parenchymal damage in the over ten year olds (27.3 %), was twice th...
In recent years this society has heard of the plight of people with spina bifida who have graduated from childrens' to adult services, and of the lack of cohesive medical and counselling services offered to them.
In the Manchester area, a joint clinic for adults has recently been set up, consisting of a Neurosurgeon, Nephrologist and Clinical Psych...
Summary— A series of 335 children with antenatally diagnosed hydronephrosis which persisted after the postnatal period was prospectively assessed. Idiopathic hydronephrosis (85 patients) and periureteric junction obstruction (73 patients) were the commonest conditions. All patients with idiopathic hydronephrosis were managed conservatively; 87% hav...
The problem of acquiring secure peritoneal access encompasses most of the history of peritoneal dialysis. We review the catheters available for children and describe a simple method of insertion of Tenckhoff catheters for acute dialysis. Two series of Tenckhoff catheters inserted in this way are presented and compared with results obtained with tro...
A new catheter for peritoneal dialysis in neonates and infants was used on 28 occasions in 17 patients. Advantages over other catheters included easy safe introduction over a guide wire, absence of early leakage of dialysate, and the ability to change the catheter without creating a second abdominal wound.
63 patients with steroid-sensitive, biopsy-proven minimal-change nephrotic syndrome were followed for between 10 and 21 years. 2 died. All the survivors had normal renal function and blood pressure, and only 2 had a single attack. Frequent relapse was more common with young age of onset and in boys. The frequency of relapse fell rapidly over the fi...