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1.
Pediatr Nephrol ; 39(7): 2131-2138, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38300268

RESUMO

BACKGROUND: Oral health conditions are common in children and young people (CYP) with kidney disorders. There is currently limited literature on how confident paediatric nephrology teams feel to identify and manage oral health concerns for their patients. METHOD: An exploratory mixed-method survey was distributed across all 13 UK specialist paediatric nephrology centres with responses received from consultants, registrars, specialist nurses and special interest (SPIN) paediatricians. RESULTS: Responses received from 109 multidisciplinary team members of 13/13 (100%) UK tertiary units. Ninety-two percent (n = 100) of respondents reported they had never received any training in oral health and 87% (n = 95) felt that further training would be beneficial to optimise care for patients and improve communication between medical and dental teams. Most respondents reported that they did not regularly examine, or enquire about, their patients' oral health. Only 16% (n = 17) reported that all their paediatric kidney transplant recipients underwent routine dental assessment prior to transplant listing. Severe adverse oral health outcomes were rarely reported and only 11% (n = 12) of respondents recalled having a patient who had a kidney transplant delayed or refused due to concerns about oral infection. Seventy-eight percent (n = 85) felt that joint working with a dental team would benefit patients at their unit; however, 17% (n = 18) felt that current infrastructure does not currently support effective joint working. CONCLUSIONS: Across the UK, paediatric kidney health professionals report lack of confidence and training in oral health. Upskilling subspecialty teams and creating dental referral pathways are recommended to maximise oral health outcomes for CYP with kidney diseases.


Assuntos
Acessibilidade aos Serviços de Saúde , Nefrologia , Saúde Bucal , Humanos , Saúde Bucal/estatística & dados numéricos , Reino Unido , Nefrologia/educação , Criança , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Atitude do Pessoal de Saúde , Equipe de Assistência ao Paciente , Inquéritos e Questionários , Transplante de Rim , Adolescente , Masculino , Feminino , Nefropatias/terapia , Nefropatias/psicologia , Assistência Odontológica/estatística & dados numéricos
2.
Pediatr Nephrol ; 39(8): 2423-2427, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38589697

RESUMO

BACKGROUND: Levamisole is a commonly used steroid-sparing agent (SSA), but the reported incidence of antineutrophil cytoplasmic antibody (ANCA) positivity has been concerning. METHODS: Observational cross-sectional study wherein children aged 2 to 18 years with frequently relapsing/steroid dependent nephrotic syndrome (FRNS/SDNS) on levamisole for ≥ 12 months were tested for ANCA. RESULTS: A total of 210 children (33% female), median age of 7.3 (IQR: 5.6-9.6) years, and a median duration of levamisole exposure of 21 (IQR: 15-30) months were tested. ANCA was positive in 18% (n = 37): 89% (n = 33) perinuclear ANCA (pANCA), 3% (n = 1) cytoplasmic ANCA (cANCA), and 8% (n = 3) both. Of ANCA-positive children, none had reduced eGFR or abnormal urinalysis. The majority of these children were asymptomatic (81%, n = 30). Rash was more common among ANCA-positive children [6/37 (16%) vs. 3/173 (2%), p = 0.0001]. On multivariate analysis, higher age (OR = 1.02, [95th CI: 1.01 to 1.03], p = 0.007) and longer duration of levamisole exposure (OR = 1.05, [95th CI: 1.02 to 1.08], p = 0.0007) were associated with ANCA positivity. Levamisole was stopped in ANCA-positive children with the resolution of any clinical manifestations if present. Repeat ANCA testing was performed in 54% (20/37), and all were ANCA negative by 18 months. CONCLUSIONS: Children with FRNS/SDNS on longer duration of levamisole were associated with increasing prevalence of ANCA positivity, but most of these children were clinically asymptomatic. Prospective studies are required to determine the chronology of ANCA positivity and its clinical implication.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos , Levamisol , Síndrome Nefrótica , Humanos , Levamisol/efeitos adversos , Criança , Feminino , Masculino , Estudos Transversais , Anticorpos Anticitoplasma de Neutrófilos/sangue , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/imunologia , Síndrome Nefrótica/sangue , Pré-Escolar , Adolescente , Estudos de Coortes
3.
Artigo em Inglês | MEDLINE | ID: mdl-38937065

RESUMO

Shared decision-making (SDM) is a collaborative approach to healthcare decision-making that involves patients and healthcare professionals working together to make decisions that are informed by the best available medical evidence, as well as the patient's values, preferences and goals. The importance of SDM and the intricate interplay among parents, children and young people (CYP), and healthcare professionals are increasingly acknowledged as the crucial aspects of delivering high-quality paediatric care. While there is a substantial evidence base for SDM improving knowledge and reducing decisional conflict, the evidence for long-term measures such as improved health outcomes is limited and mainly inconclusive. To support healthcare teams in implementing SDM, the authors offer a practical guide to enhance decision-making processes and empower CYP and their families.

4.
Pediatr Nephrol ; 38(4): 1241-1248, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-35925426

RESUMO

BACKGROUND: Despite its utility, uncertainty exists on the feasibility of acute peritoneal dialysis (PD) and optimal PD catheter type for very low birth weight (VLBW < 1500 g) and extremely low birth weight (ELBW < 1000 g) infants. We hereby report our experience of acute PD among these high-risk infants and compare the outcome between stylet-based rigid catheter (SRC) and Cook Mac-Loc Multipurpose Drainage catheters® (CMMDC). METHODS: Case notes of infants < 1500 g undergoing PD between 2012 and 2021 in a network of five participating neonatal units supported by a tertiary paediatric nephrology centre in Kolkata, India, were retrospectively reviewed. PD was conducted either with SRC or after 2018 with CMMDC. Outcome parameters included complications, survival during PD, and survival to discharge. RESULTS: 24 infants (VLBW: n = 13 and ELBW: n = 11) underwent PD at median age 4.5 days (IQR 3-6) with either CMMDC (n = 14) or SRC (n = 10). Significant improvement in biochemical parameters and fluid removal was seen in both ELBW and VLBW infants. CMMDC was associated with significantly fewer PD-related complications 7/14 (50%) vs. 9/10 (90%) (p = 0.04) and higher survival during PD 13/14 (93%) vs. 5/10 (50%) (p = 0.02), without significant difference in survival to hospital discharge 8/14 (57%) vs. 3/10 (30%) (p = 0.25). CMMDC also enabled longer duration of PD, higher ultrafiltration, and better control of acidosis. Consumable cost was higher for CMMDC (USD$60) than SRC (USD$14). CONCLUSIONS: In a low resource setting, CMMDC had lower PD complications and superior short-term survival among ELBW/VLBW infants. A higher resolution version of the Graphical abstract is available as Supplementary information.


Assuntos
Diálise Peritoneal , Recém-Nascido , Criança , Lactente , Humanos , Estudos Retrospectivos , Diálise Peritoneal/efeitos adversos , Recém-Nascido de Peso Extremamente Baixo ao Nascer , Cateteres de Demora/efeitos adversos , Drenagem/efeitos adversos
5.
Arch Dis Child Educ Pract Ed ; 108(2): 112-114, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-35264442

RESUMO

Children admitted to our hospital with cystic fibrosis had frequent medication errors due to polypharmacy and addition of specialist and high-risk medications despite an electronic prescribing and medicines administration system in place. We describe a multidisciplinary quality improvement project that combined a computerised order entry system (CPOE) with human factor process changes. Over 12 months, our run chart showed a 43% reduction in prescription errors. For medications prescribable via the CPOE, errors reaching the patient reduced from 50% to 29%. Electronic prescribing can be seen by clinicians as a fixed unalterable system contributing to rather than ameliorating errors. Improving safety requires whole team engagement and working closely with programmers to adapt function and influence human factors.


Assuntos
Fibrose Cística , Prescrição Eletrônica , Sistemas de Registro de Ordens Médicas , Criança , Humanos , Pacientes Internados , Fibrose Cística/tratamento farmacológico , Erros de Medicação/prevenção & controle
6.
Am J Med Genet C Semin Med Genet ; 190(1): 109-120, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-35289079

RESUMO

Monogenic disorders of the kidney typically affect either the glomerular or tubulointerstitial compartment producing a distinct set of clinical phenotypes. Primary focal segmental glomerulosclerosis (FSGS), for instance, is characterized by glomerular scarring with proteinuria and hypertension while nephronophthisis (NPHP) is associated with interstitial fibrosis and tubular atrophy, salt wasting, and low- to normal blood pressure. For both diseases, an expanding number of non-overlapping genes with roles in glomerular filtration or primary cilium homeostasis, respectively, have been identified. TTC21B, encoding IFT139, however has been associated with disorders of both the glomerular and tubulointerstitial compartment, and linked with defective podocyte cytoskeleton and ciliary transport, respectively. Starting from a case report of extreme early-onset hypertension, proteinuria, and progressive kidney disease, as well as data from the Genomics England 100,000 Genomes Project, we illustrate here the difficulties in assigning this mixed phenotype to the correct genetic diagnosis. Careful literature review supports the notion that biallelic, often hypomorph, missense variants in TTC21B are commonly associated with early-onset hypertension and histological features of both FSGS and NPHP. Increased clinical recognition of this mixed glomerular and tubulointerstitial disease with often mild or absent features of a typical ciliopathy as well as inclusion of TTC21B on gene panels for early-onset arterial hypertension might shorten the diagnostic odyssey for patients affected by this rare tubuloglomerular kidney disease.


Assuntos
Glomerulosclerose Segmentar e Focal , Hipertensão , Nefropatias , Feminino , Fibrose , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/genética , Glomerulosclerose Segmentar e Focal/patologia , Humanos , Hipertensão/genética , Rim/patologia , Nefropatias/genética , Masculino , Proteinúria/complicações , Proteinúria/genética , Proteinúria/patologia
7.
Br J Clin Pharmacol ; 88(12): 5128-5148, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-35849849

RESUMO

Solid oral dosage forms (SODFs) (often called pills by patients) are the default formulation to treat medical ailments. Beneficial therapeutic outcomes rely on patients taking them as directed. Up to 40% of the population experience difficulties swallowing SODFs, resulting in reduced adherence and impaired therapeutic efficacy. Often associated with children, this also presents in adults with dysphagia, and without any organic dysphagia (non-physiological-related or functional dysphagia). This review aims to identify and appraise current interventions used to screen for and overcome pill aversion in adults with functional dysphagia. A comprehensive search of the literature was conducted. Articles reporting pill aversion in adults aged ≥18 years with no underlying cause, history of, or existing dysphagia were included. Study quality was determined using the STROBE tool for observational studies. A narrative synthesis of the findings was prepared. We identified 18 relevant cohort studies, which demonstrate that pill aversion is a global problem. Perceived ease of and/or SODF swallowability appears to be influenced by female gender, younger age, co-morbidities (e.g., depression), and physical SODF properties. Patients often modify their medicines rather than raise this issue with their healthcare team. Screening for pill aversion is haphazard but controlled postural adjustments, coating SODFs and behavioural interventions appear to be successful solutions. SODF swallowing difficulties are a barrier to effective medication use. Healthcare professionals must recognise that pill aversion is a problem requiring identification through effective screening and resolution by training interventions, appropriate formulation selection or specialist referral.


Assuntos
Transtornos de Deglutição , Humanos , Adulto , Criança , Feminino , Adolescente , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/terapia , Transtornos de Deglutição/diagnóstico , Deglutição , Estudos de Coortes
8.
Acta Paediatr ; 111(9): 1808-1813, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35642352

RESUMO

AIM: To determine the prevalence of familial vesicoureteric reflux (VUR) by studying the outcomes of screening in a contemporary cohort of newborns with normal antenatal kidney scans. METHODS: A review of screening outcomes in newborns with a first degree relative with VUR, normal antenatal scans and no prior urine infections between 2014-2019 at three maternity units in the North East of England was conducted. Imaging consisted of micturating cystourethrogram (MCUG) in all and renal tract ultrasound scan (RUS) routinely in two units and by clinician preference in one unit. RESULTS: At a median age of 59 days, 265 infants underwent MCUG. High-grade VUR (Grades 3-5) was detected in 13 (4.9%) and low-grade VUR (Grades 1-2) in 24 (9.1%). In the 152 infants who had a RUS, abnormalities were detected in 21 (13.8%). An abnormal postnatal RUS has a low positive predictive value (14.3%) for high-grade VUR, but a normal RUS has a high negative predictive value (95.4%). CONCLUSION: Compared to historical cohorts from two decades ago, the yield from familial VUR screening is low and unjustifiable in the setting of normal antenatal anomaly scans.


Assuntos
Infecções Urinárias , Refluxo Vesicoureteral , Feminino , Humanos , Lactente , Recém-Nascido , Gravidez , Prevalência , Cintilografia , Ultrassonografia , Infecções Urinárias/diagnóstico por imagem , Refluxo Vesicoureteral/diagnóstico por imagem , Refluxo Vesicoureteral/epidemiologia , Refluxo Vesicoureteral/genética
9.
Pediatr Transplant ; 25(5): e13960, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33368894

RESUMO

BACKGROUND: Transplantation is the optimal modality for children with ESRD. High risk of disease recurrence and graft loss with FSGS, and its financial implications, may result in families refusing transplantation. Deceased donation is often preferred for FSGS, but access is limited in many low- and middle-income group countries (LMIC; per capita gross national income between $1026 and $3995). As FSGS secondary to an underlying genetic etiology has low recurrence risk, we hypothesized that in LMIC such as India, families with children in ESRD secondary to FSGS with proven pathogenic mutation are more likely to consent for transplantation than those with unknown etiology. METHODS: Prospective cross-sectional study with retrospective chart review was undertaken (March 2011 and February 2019) to identify children with ESRD from FSGS. The objective was to ascertain NGS uptake and findings, parental decision for transplantation, and transplant outcome. RESULTS: 28 children with FSGS started transplant workup, and 15 (54%) families agreed for NGS testing. Pathogenic mutation (NPHS1 x 2, WT1 x 2, COL4A3 x 2, CD2AP, CRB2, COL4A5, INF 2, ACTN4, NPHP4: 1 each) was identified in 12 (80%). 92% (11/12) agreed to proceed with transplantation in contrast to 13% (2/16) who either did not undergo NGS testing or had no pathogenic mutation identified (P = .001). No disease recurrences were noted in those with a known pathogenic mutation. CONCLUSION: In LMIC, NGS results are useful in transplant discussions with families for children with ESRD secondary to FSGS.


Assuntos
Testes Genéticos/métodos , Glomerulosclerose Segmentar e Focal/genética , Sequenciamento de Nucleotídeos em Larga Escala , Falência Renal Crônica/cirurgia , Transplante de Rim/psicologia , Consentimento dos Pais/psicologia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Tomada de Decisões , Países Desenvolvidos , Feminino , Seguimentos , Marcadores Genéticos , Glomerulosclerose Segmentar e Focal/fisiopatologia , Humanos , Índia , Lactente , Recém-Nascido , Falência Renal Crônica/etiologia , Masculino , Mutação , Estudos Prospectivos , Recidiva , Estudos Retrospectivos , Risco , Análise de Sequência de DNA
10.
Pediatr Nephrol ; 36(9): 2797-2810, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33742247

RESUMO

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.


Assuntos
COVID-19/psicologia , Controle de Doenças Transmissíveis/normas , Medo , Insuficiência Renal Crônica/terapia , Estresse Psicológico/epidemiologia , Adolescente , Adulto , Fatores Etários , COVID-19/epidemiologia , COVID-19/prevenção & controle , COVID-19/transmissão , Criança , Pré-Escolar , Tomada de Decisão Compartilhada , Feminino , Acessibilidade aos Serviços de Saúde , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Pandemias/prevenção & controle , Pais/psicologia , SARS-CoV-2/patogenicidade , Estresse Psicológico/diagnóstico , Estresse Psicológico/psicologia , Inquéritos e Questionários/estatística & dados numéricos , Reino Unido/epidemiologia , Adulto Jovem
11.
Arch Dis Child Educ Pract Ed ; 106(1): 23-27, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-32086340

RESUMO

Many paediatricians will be faced with a sick infant who on investigation is found to have hyponatraemia and hyperkalaemia at some time in their career. The focus of initial management includes the treatment of potentially life-threatening hyperkalaemia with concurrent investigation aiming to elucidate whether the underlying cause reflects a primarily renal or endocrine pathology. We describe the presentation of two infants who each presented with one of the more common underlying diagnoses that led to this biochemical disturbance and discuss the approach to immediate treatment, diagnostic work-up and longer term management.


Assuntos
Hiperpotassemia , Hiponatremia , Humanos , Hiperpotassemia/diagnóstico , Hiperpotassemia/terapia , Hiponatremia/diagnóstico , Hiponatremia/etiologia , Hiponatremia/terapia , Lactente , Potássio , Resolução de Problemas , Sódio
12.
Artigo em Inglês | MEDLINE | ID: mdl-33367818

RESUMO

BACKGROUND: Primary nephrogenic diabetes insipidus (NDI) is a rare disorder and little is known about treatment practices and long-term outcome. METHODS: Paediatric and adult nephrologists contacted through European professional organizations entered data in an online form. RESULTS: Data were collected on 315 patients (22 countries, male 84%, adults 35%). Mutation testing had been performed in 270 (86%); pathogenic variants were identified in 258 (96%). The median (range) age at diagnosis was 0.6 (0.0-60) years and at last follow-up 14.0 (0.1-70) years. In adults, height was normal with a mean (standard deviation) score of -0.39 (±1.0), yet there was increased prevalence of obesity (body mass index >30 kg/m2; 41% versus 16% European average; P < 0.001). There was also increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (32%) and adults (48%). Evidence of flow uropathy was present in 38%. A higher proportion of children than adults (85% versus 54%; P < 0.001) received medications to reduce urine output. Patients ≥25 years were less likely to have a university degree than the European average (21% versus 35%; P = 0.003) but full-time employment was similar. Mental health problems, predominantly attention-deficit hyperactivity disorder (16%), were reported in 36% of patients. CONCLUSION: This large NDI cohort shows an overall favourable outcome with normal adult height and only mild to moderate CKD in most. Yet, while full-time employment was similar to the European average, educational achievement was lower, and more than half had urological and/or mental health problems.

13.
Pediatr Nephrol ; 35(1): 119-126, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31673828

RESUMO

BACKGROUND: This study aimed to evaluate outcome of children on chronic peritoneal dialysis (PD) with a concurrent colostomy. METHODS: Patients were identified through the International Pediatric Peritoneal Dialysis Network (IPPN) registry. Matched controls were randomly selected from the registry. Data were collected through the IPPN database and a survey disseminated to all participating sites. RESULTS: Fifteen centers reported 20 children who received chronic PD with a co-existing colostomy. The most common cause of end stage kidney disease was congenital anomalies of the kidney and urinary tract (n = 16, 80%). The main reason for colostomy placement was anorectal malformation (n = 13, 65%). The median age at colostomy creation and PD catheter (PDC) insertion were 0.1 (IQR, 0-2.2) and 2.8 (IQR 0.2-18.8) months, respectively. The colostomies and PDCs were present together for a median 18 (IQR, 4.9-35.8) months. The median age at PDC placement in 46 controls was 3.4 (IQR, 0.2-7.4) months of age. Fourteen patients (70%) developed 39 episodes of peritonitis. The annualized peritonitis rate was significantly higher in the colostomy group (1.13 vs. 0.70 episodes per patient year; p = 0.02). Predominant causative microorganisms were Staphylococcus aureus (15%) and Pseudomonas aeruginosa (13%). There were 12 exit site infection (ESI) episodes reported exclusively in colostomy patients. Seven colostomy children (35%) died during their course of PD, in two cases due to peritonitis. CONCLUSION: Although feasible in children with a colostomy, chronic PD is associated with an increased risk of peritonitis and mortality. Continued efforts to reduce infection risk for this complex patient population are essential.


Assuntos
Colostomia/efeitos adversos , Falência Renal Crônica/terapia , Diálise Peritoneal/efeitos adversos , Peritonite/epidemiologia , Anormalidades Urogenitais/terapia , Refluxo Vesicoureteral/terapia , Antibacterianos/uso terapêutico , Estudos de Casos e Controles , Cateteres de Demora/efeitos adversos , Cateteres de Demora/estatística & dados numéricos , Criança , Pré-Escolar , Colostomia/estatística & dados numéricos , Estudos de Viabilidade , Feminino , Humanos , Lactente , Recém-Nascido , Falência Renal Crônica/etiologia , Falência Renal Crônica/mortalidade , Masculino , Diálise Peritoneal/estatística & dados numéricos , Peritonite/tratamento farmacológico , Peritonite/etiologia , Pseudomonas aeruginosa/isolamento & purificação , Estudos Retrospectivos , Staphylococcus aureus/isolamento & purificação , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/mortalidade , Refluxo Vesicoureteral/complicações , Refluxo Vesicoureteral/mortalidade
14.
Pediatr Nephrol ; 34(5): 855-863, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30564878

RESUMO

OBJECTIVE: Rituximab is an effective treatment for children with steroid dependent or frequently relapsing nephrotic syndrome. The optimum dosing schedule for rituximab has not been established. We hypothesized that a single low dose of 375 mg/m2 would have comparable outcomes to higher doses in reducing the frequency of relapse and time to B cell reconstitution. METHODS: We conducted a multicenter retrospective observational cohort study of children with steroid-sensitive frequently relapsing nephrotic syndrome. Data were extracted from clinical records including the dates of diagnosis, treatment, relapses, the use of concomitant immunosuppression, and lymphocyte subset profiling. Patients treated earlier received variable doses of rituximab, although typically two doses of 750 mg/m2. Later, patients received the current regimen of a single dose of 375 mg/m2. The primary outcome was an absence of clinically confirmed relapse 12 months following rituximab administration. Secondary outcomes were median time to relapse, probability of being relapse-free at 6 and 24 months and time to reconstitution of CD19+ B cells. RESULTS: Sixty patients received 143 courses of rituximab. Seven different dosing regimen strategies were used, ranging between 375 and 750 mg/m2 per dose, with administration of 1-4 doses. There was no significant difference in event-free survival at 12 months between dosing strategies. The median time to reconstitution of B cells was not significantly different between groups. CONCLUSIONS: Use of a single low-dose regimen of rituximab in the management of frequently relapsing nephrotic syndrome does not affect the probability of relapse at 12 months or time to B cell reconstitution compared to a conventional higher dose.


Assuntos
Glucocorticoides/administração & dosagem , Imunossupressores/administração & dosagem , Síndrome Nefrótica/tratamento farmacológico , Rituximab/administração & dosagem , Prevenção Secundária/métodos , Adolescente , Antígenos CD19/metabolismo , Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Linfócitos B/metabolismo , Criança , Pré-Escolar , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Quimioterapia Combinada/métodos , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Depleção Linfocítica/métodos , Masculino , Síndrome Nefrótica/sangue , Síndrome Nefrótica/imunologia , Síndrome Nefrótica/mortalidade , Recidiva , Estudos Retrospectivos
15.
Nephrol Dial Transplant ; 32(11): 1908-1917, 2017 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-28158862

RESUMO

BACKGROUND: Racial differences in overall mortality rates have been found in children on renal replacement therapy (RRT). We used data from the European Society for Paediatric Nephrology/European Renal Association - European Dialysis and Transplant Association Registry to study racial variation in the prevalence of cardiovascular disease (CVD) risk factors among European children on RRT. METHODS: We included patients aged <20 years between 2006-13 who (i) initiated dialysis treatment or (ii) had a renal transplant vintage of ≥1 year. Racial groups were defined as white, black, Asian and other. The CVD risk factors assessed included uncontrolled hypertension, obesity, hyperphosphataemia and anaemia. Differences between racial groups in CVD risk factors were examined using generalized estimating equation (GEE) models while adjusting for potential confounders. RESULTS: In this study, 1161 patients on dialysis and 1663 patients with a transplant were included. The majority of patients in both groups were white (73.8% and 79.9%, respectively). The crude prevalence of the CVD risk factors was similar across racial groups. However, after adjustment for potential confounders, Asian background was associated with higher risk of uncontrolled hypertension both in the dialysis group [odds ratio (OR): 1.27; 95% confidence interval (CI): 1.01-1.64] and the transplant group (OR: 1.37; 95% CI: 1.11-1.68) compared with white patients. Patients of Asian and other racial background with a renal transplant had a higher risk of anaemia compared with white patients (OR: 1.50; 95% CI: 1.15-1.96 and OR: 1.45; 95% CI: 1.01-2.07, respectively). Finally, the mean number of CVD risk factors among dialysis patients was higher in Asian patients (1.83, 95% CI: 1.64-2.04) compared with white patients (1.52, 95% CI: 1.40-1.65). CONCLUSIONS: We found a higher prevalence of modifiable CVD risk factors in Asian children on RRT. Early identification and management of these risk factors could potentially improve long-term outcomes.


Assuntos
Hipertensão/mortalidade , Falência Renal Crônica/mortalidade , Obesidade/mortalidade , Adolescente , Povo Asiático , População Negra , Criança , Pré-Escolar , Europa (Continente) , Feminino , Disparidades em Assistência à Saúde , Humanos , Hipertensão/etnologia , Lactente , Recém-Nascido , Falência Renal Crônica/etnologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Masculino , Obesidade/etnologia , Prevalência , Sistema de Registros , Diálise Renal , Fatores de Risco , Resultado do Tratamento , População Branca
16.
Pediatr Nephrol ; 31(4): 613-21, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26525201

RESUMO

BACKGROUND: Paediatric renal biopsy standards introduced in the UK in 2010 were intended to reduce variation and improve practice. A concurrent national drive was aimed at building robust paediatric nephrology networks to ensure services cater for the needs of the family and minimise time away from home. We aimed to identify current national practice since these changes on behalf of the British Association for Paediatric Nephrology. METHODS: All UK paediatric nephrology centres were invited to complete a survey of their biopsy practice, including advance preparation. From 1 January to 30 June 2012, a national prospective audit of renal biopsies was undertaken at participating centres comparing practice with the British Association for Paediatric Nephrology (BAPN) standards and audit results from 2005. RESULTS: Survey results from 11 centres demonstrated increased use of pre-procedure information leaflets (63.6 % vs 45.5 %, P = 0.39) and play preparation (90.9 % vs 9.1 %, P = 0.0001). Audit of 331 biopsies showed a move towards day-case procedures (49.5 % vs 32.9 %, P = 0.17) and reduced major complications (4.5 % vs 10.4 %, P = 0.002). Biopsies with 18-gauge needles had significantly higher mean pass rates (3.2 vs 2.3, P = 0.0008) and major complications (15.3 % vs 3.3 %, P = 0.0015) compared with 16-gauge needles. CONCLUSIONS: Percutaneous renal biopsy remains a safe procedure in children, thus improving family-centered service provision in the UK.


Assuntos
Biópsia/tendências , Atenção à Saúde/tendências , Nefropatias/diagnóstico , Rim/patologia , Nefrologia/tendências , Pediatria/tendências , Padrões de Prática Médica/tendências , Medicina Estatal/tendências , Adolescente , Biópsia/efeitos adversos , Biópsia/normas , Criança , Pré-Escolar , Atenção à Saúde/normas , Feminino , Pesquisas sobre Atenção à Saúde , Humanos , Lactente , Recém-Nascido , Nefropatias/patologia , Masculino , Auditoria Médica , Nefrologia/normas , Assistência Centrada no Paciente/tendências , Pediatria/normas , Padrões de Prática Médica/normas , Valor Preditivo dos Testes , Estudos Prospectivos , Indicadores de Qualidade em Assistência à Saúde/tendências , Medicina Estatal/normas , Reino Unido , Adulto Jovem
18.
Pediatr Nephrol ; 30(11): 2045-50, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26248471

RESUMO

BACKGROUND: Early management of congenital nephrotic syndrome invariably includes the frequent administration of intravenous human albumin solution. The safety and feasibility of intravenous administration of albumin in the patients' home setting has not previously been reported. CASE-DIAGNOSIS/TREATMENT: We report a series of seven paediatric patients whose parents were trained in the administration of albumin via a central venous catheter at home, with the aim of minimising hospital admission or attendances. We describe the clinical course of these patients and complication rates ascribed to this strategy. CONCLUSIONS: Our results demonstrate that home albumin infusion can be performed safely.


Assuntos
Albuminas/administração & dosagem , Serviços de Assistência Domiciliar , Síndrome Nefrótica/tratamento farmacológico , Cateteres Venosos Centrais , Feminino , Humanos , Lactente , Recém-Nascido , Infusões Intravenosas , Masculino
19.
Nephrol Dial Transplant ; 29(1): 196-203, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24081859

RESUMO

BACKGROUND: Childhood obesity is a significant health problem in the UK. To date, there is little known about the pattern of change in body mass index (BMI) following renal transplantation in UK paediatric patients. Our objectives in this study were to (i) describe trends in BMI seen in UK patients undergoing renal transplantation in the short and medium term and (ii) identify risk factors predisposing children to excessive weight gain following transplantation. METHODS: A retrospective case note review was performed across 12 of 13 paediatric nephrology centres in the UK, with BMI measurements recorded pre-transplantation and for 4 years thereafter. BMI% was used to assess changes in adiposity over time. International Obesity Taskforce definitions of overweight and obesity were used to identify the prevalence of excess weight pre- and post-renal transplantation. RESULTS: A total of 159 patients (113 boys) under the age of 18 with a functioning kidney transplant were included. Fifty-six patients (35.2%) were under the age of 5 at transplantation. Pre-transplantation, 31.4% of patients were classified as overweight or obese, which increased to 52.8% by the end of follow-up. The majority of patients experienced rapid increases in BMI% over the initial four months post-transplantation, which were sustained for the remainder of the follow-up period. The major risk factor for being overweight or obese at the end of follow-up was having excessive weight pre-transplantation. Four years following transplantation, the prevalence rate of overweight and obesity was much higher in our study cohort than the normal UK childhood population. CONCLUSIONS: The prevalence of patients classified as overweight or obese in the UK paediatric renal cohort is high pre-transplantation and rises subsequently. Those at risk can be identified by an unhealthy BMI pre-transplantation and will require timely intervention with close monitoring in the subsequent post-transplantation period.


Assuntos
Índice de Massa Corporal , Nefropatias/epidemiologia , Nefropatias/cirurgia , Transplante de Rim , Obesidade Infantil/epidemiologia , Adolescente , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Estudos Longitudinais , Masculino , Sobrepeso/epidemiologia , Período Pós-Operatório , Prevalência , Estudos Retrospectivos , Fatores de Risco , Reino Unido
20.
Arch Dis Child ; 109(4): 334-338, 2024 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-38336457

RESUMO

INTRODUCTION: During the COVID-19 pandemic, evidence emerged that immunosuppressed children were less affected by COVID-19 infections compared with immunosuppressed adults. The aim of our study was to investigate how COVID-19 infections affected paediatric kidney transplant recipients (pKTR) in the UK. METHODS: Questionnaires regarding COVID-19 infection data and care of pKTR during the COVID-19 pandemic were sent to all 13 UK paediatric nephrology centres examining asymptomatic and symptomatic pKTR with positive COVID-19 PCR testing from 1 April 2020 to 1 December 2021. RESULTS: 63 pKTR who were 3.1 (range 0.1-15) years post-transplantation had COVID-19 infection with positive SARS-CoV-2 PCR RNA. Classical COVID-19 symptoms were present in half of the patients; with atypical presentations including diarrhoea (13%) and lethargy (13%) also noted, while a third of patients were asymptomatic. Eighteen patients (28%) were hospitalised including five asymptomatic patients admitted for other reasons. No patients needed ventilation or intensive care admission, and one patient received supplemental oxygen. There was evidence of acute kidney injury (AKI) in 71% of patients, but no patients needed kidney replacement therapy with haemofiltration or dialysis. CONCLUSION: We report 10.4% of the UK paediatric renal transplantation population had documented COVID-19 infections with positive SARS-CoV-2 PCR RNA with 28% of those affected requiring hospitalisation. The increased incidence of AKI, particularly after the first wave of the COVID-19 pandemic, was possibly due to increased testing. There was low morbidity and mortality compared with the adult population.


Assuntos
Injúria Renal Aguda , COVID-19 , Transplante de Rim , Criança , Humanos , COVID-19/epidemiologia , Estudos Transversais , Transplante de Rim/efeitos adversos , Pandemias , Estudos Retrospectivos , RNA , SARS-CoV-2
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