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1.
Pediatr Nephrol ; 39(4): 1169-1176, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37840039

RESUMEN

BACKGROUND: When applying Pierce U25 formula for estimating glomerular filtration rate (eGFR), we observed a higher proportion of eGFR < 90 mL/min/1.73 m2 (chronic kidney disease (CKD) stage 2). We compared agreement and accuracy of the Pierce U25 (ages 2-25), Pottel (ages 2-100), and CKD-EPI (ages 18-100) formulae to GFR measurements. METHODS: Post hoc analysis of the three eGFRs compared to 367 99m technetium-diethylene-triamine penta-acetic acid (99Tc DTPA) GFR measurements (240 patients) using 3 sampling points and Brockner/Mørtensen correction (body surface area calculation based on ideal weight) on simultaneous serum creatinine and cystatin C measurements. RESULTS: Overall, the U25 formula performed well with a Spearman r of 0.8102 (95% confidence interval 0.7706 to 0.8435, p < 0.0001) while diagnostic accuracy was low in patients with normal mGFR. The U25 formula reclassified 29.5% of patients with normal mGFR as CKD stage 2; whereas the average of the modified Schwartz formula based on serum creatinine and the Filler formula based on cystatin C, only over-diagnosed CKD stage 2 in 8.5%, 24.5% within 10% and 62.7% within 30%. We therefore combined both. The average Schwartz/Filler eGFR had 36.5% of results within 10%, 84.7% within 30%, and normal mGFR accuracy was 26.8%, 63.9% for 10% and 30%, respectively, outperforming the CKD-EPI and Pottel formulae. CONCLUSIONS: The Pierce U25 formula results correlated well with mGFR < 75 mL/min/1.73 m2. Over the entire GFR range, accuracy was better for patients with a higher mGFR, when averaging the combined Schwartz/Filler formulae. More work is needed to prospectively confirm our findings in other centers.


Asunto(s)
Cistatina C , Insuficiencia Renal Crónica , Humanos , Tasa de Filtración Glomerular , Estudios Transversales , Creatinina , Insuficiencia Renal Crónica/diagnóstico
2.
Antimicrob Agents Chemother ; 67(7): e0026623, 2023 07 18.
Artículo en Inglés | MEDLINE | ID: mdl-37278655

RESUMEN

The emergence of the Omicron variant of SARS-CoV-2 represented a challenge to the treatment of COVID-19 using monoclonal antibodies. Only Sotrovimab maintained partial activity, allowing it to be used in high-risk patients infected with the Omicron variant. However, reports of resistance mutations to Sotrovimab demand efforts to better understand the intra-patient emergence of Sotrovimab resistance. A retrospective genomic analysis was conducted on respiratory samples from immunocompromised patients infected with SARS-CoV-2 who received Sotrovimab at our hospital between December 2021 and August 2022. The study involved 95 sequential specimens from 22 patients (1 to 12 samples/patient; 3 to 107 days post-infusion; threshold cycle [CT] ≤ 32). Resistance mutations (in P337, E340, K356, and R346) were detected in 68% of cases; the shortest time to detection of a resistance mutation was 5 days after Sotrovimab infusion. The dynamics of resistance acquisition were highly complex, with up to 11 distinct amino acid changes in specimens from the same patient. In two patients, the mutation distribution was compartmentalized in respiratory samples from different sources. This is the first study to examine the acquisition of Sotrovimab resistance in the BA.5 lineage, enabling us to determine the lack of genomic or clinical differences between Sotrovimab resistance in BA.5 relative to that in BA.1/2. Across all Omicron lineages, the acquisition of resistance delayed SARS-CoV-2 clearance (40.67 versus 19.5 days). Close, real-time genomic surveillance of patients receiving Sotrovimab should be mandatory to facilitate early therapeutic interventions.


Asunto(s)
COVID-19 , SARS-CoV-2 , Humanos , SARS-CoV-2/genética , Estudios Retrospectivos , Genómica , Mutación , Anticuerpos Neutralizantes
3.
Blood Purif ; 51 Suppl 1: 61-67, 2023 May 10.
Artículo en Inglés | MEDLINE | ID: mdl-37231795

RESUMEN

INTRODUCTION: Growth retardation is a common problem in pediatric patients with chronic kidney disease. It is unknown if the growth of children on peritoneal dialysis (PD) can be augmented by more dialysis. METHODS: We studied the effect of various peritoneal adequacy parameters on delta height standard deviation scores (SDSs) and growth velocity z-scores in 53 children (27 males) on PD, who underwent 2 longitudinal adequacy tests at 9-month intervals. None of the patients were on growth hormone. Intraperitoneal pressure and standard KDOQI guidelines were compared to the outcome measures delta height SDS and height velocity z-scores, using univariate and multivariate tests. RESULTS: At the time of the second PD adequacy test, their mean age was 9.2 ± 5.3 years; mean fill volume was 961 ± 254 mL/m2; and median total infused dialysate volume was 5.26 L/m2/day (range 2.03-15.32 L). The median total weekly Kt/V was 3.79 (range 0.9-9.5), and the median total creatinine clearance was 56.6 (range 7.6-133.48) L/week, higher than previous pediatric studies. The delta height SDS was a median of -0.12 (range -2 to +3.95)/year. The mean height velocity z-score was -1.6 ± 4.0. The only relationships discovered were between the delta height SDS and age, bicarbonate, and intraperitoneal pressure, but not for Kt/V or creatinine clearance. CONCLUSION: Our findings highlight the importance of normalization of bicarbonate concentrations to improve height z-score.

4.
Child Care Health Dev ; 49(6): 1046-1053, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-36905081

RESUMEN

BACKGROUND: Previous literature has explored parent/caregiver perspectives and satisfaction with the health care transition (HCT) process for their adolescents and young adults with special health care needs (AYASHCN). Limited research has explored the opinion of health care providers and researchers on parent/caregiver outcomes associated with a successful HCT for AYASHCN. METHODS: A web-based survey was distributed through the international and interdisciplinary Health Care Transition Research Consortium listserv, which at the time of the survey was composed of 148 providers dedicated to optimizing the HCT of AYAHSCN. Participants responded to the open-ended question, 'What parent/caregiver-related outcome(s) would represent a successful healthcare transition?' Respondents included 109 providers (52 health care professionals, 38 social service professionals and 19 other). Responses were coded for emergent themes, and research suggestions were identified. RESULTS: Qualitative analyses identified two major themes: emotion- and behaviour-based outcomes. Emotion-based subthemes included relinquishing control of child's health management (n = 50, 45.9%) as well as parental satisfaction and confidence in their child's care and HCT (n = 42, 38.5%). Respondents also noted that parents/caregivers should experience an improved sense of well-being and decreased stress (n = 9, 8.2%) due to a successful HCT. Behaviour-based outcomes included early preparation and planning for HCT (n = 12, 11.0%) and parental instruction on the knowledge and skills necessary for their adolescent to independently manage their health (n = 10, 9.1%). CONCLUSIONS: Health care providers can assist parents/caregivers in learning strategies for instructing their AYASHCN about condition-related knowledge and skills as well as provide support for 'letting go' of the caregiver role during the HCT to adult-focused health services and adulthood. Communication between the AYASCH, their parents/caregivers and paediatric- and adult-focused providers needs to be consistent and comprehensive to ensure continuity of care and a successful HCT. We also offered strategies to address the outcomes suggested by the participants of this study.


Asunto(s)
Transición a la Atención de Adultos , Humanos , Adolescente , Niño , Adulto Joven , Cuidadores , Transferencia de Pacientes , Atención a la Salud , Padres
5.
J Pediatr Nurs ; 71: 111-119, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36464544

RESUMEN

BACKGROUND: Adolescent/young adults (AYAs) with chronic conditions must undergo healthcare transition (HCT) preparation until their mid-twenties. Valid HCT readiness measures are lacking in China. METHODS: The present study translated, back-translated, and adapted the Self-Management and Transition to Adulthood with Rx = Treatment (STARx) Questionnaire. We examined the psychometric properties of this tool in a relatively large in-patient sample of AYAs with various chronic health conditions at a Chinese tertiary general hospital. RESULTS: We enrolled 624 AYAs aged 10-25 years (19.66 ±â€¯3.64) with various chronic health conditions. The Chinese version of the STARx Questionnaire demonstrated excellent internal consistency (Cronbach's alpha = 0.83) and reliability with a two-week test-retest (ICC = 0.88, p < .001). Furthermore, the Chinese version revealed a three-factor structure (self-management, disease knowledge, and provider communication) consistent with the revised English version of the STARx Questionnaire. In terms of discriminant validity, the total score of the Chinese STARx Questionnaire showed a significant positive correlation with age but no gender differences were found. In terms of predictive validity, the Chinese STARx Questionnaire was significantly correlated with shorter length of hospitalization and higher frequency of emergency room visit, but the correlations became insignificant after controlling for age. CONCLUSIONS: The results suggest that the Chinese version of the STARx Questionnaire is a robust HCT readiness tool in AYAs with chronic conditions and clinicians may find it useful to develop individualized interventions.


Asunto(s)
Transición a la Atención de Adultos , Humanos , Adolescente , Adulto Joven , Reproducibilidad de los Resultados , Encuestas y Cuestionarios , Traducciones , China , Enfermedad Crónica , Psicometría/métodos
6.
J Pediatr Nurs ; 71: 104-110, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36396566

RESUMEN

PURPOSE: To examine the effects of "ALL YOU NEED IS LOVE", a novel six-week, self-directed patient education manual designed to improve chronic kidney disease knowledge/self-management, health care transition readiness, self-advocacy, and mindfulness skills among adolescents with chronic kidney disease. DESIGN AND METHODS: We enrolled 49 adolescents aged 11-17 years (mean age 14.7 ± 1.9; 53.1% males) from a university hospital kidney center who had CKD stages 3 and greater, who were randomly assigned into the "ALL YOU NEED IS LOVE" patient education only group (n = 31) or the "ALL YOU NEED IS LOVE" patient education plus mindfulness training group (n = 18). Participants completed Qualtrics surveys at baseline, post-intervention, and three-month follow-up. The survey included measures of outcome variables (i.e., self-management/transition readiness, patient self-advocacy, and mindfulness), and an additional demographic questionnaire was included in the baseline survey. Multilevel model analyses were used to examine the effects of group and time on the outcome variables. RESULTS: Multilevel model analyses showed an overall significant time effect across all outcome variables in both groups. However, the group effects were not statistically significant across the outcome variables. CONCLUSIONS: Both interventions significantly increased participants' self-management/HCT readiness, self-advocacy, and mindfulness over time. Mindfulness training may not bring additional benefits to the "ALL YOU NEED IS LOVE" education manual. PRACTICE IMPLICATIONS: Pediatric nurses and clinicians may utilize the self-directed "ALL YOU NEED IS LOVE" manual to increase self-management/transition readiness, self-advocacy, and mindfulness among adolescents with chronic kidney disease.


Asunto(s)
Insuficiencia Renal Crónica , Automanejo , Transición a la Atención de Adultos , Masculino , Niño , Humanos , Adolescente , Femenino , Insuficiencia Renal Crónica/terapia , Transferencia de Pacientes , Encuestas y Cuestionarios
7.
J Pediatr ; 247: 95-101, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35598644

RESUMEN

OBJECTIVES: To evaluate national trends in health care transition preparation over a 4-year period using the National Survey of Children's Health (NSCH) and to identify and examine disparities in receipt of health care transition preparation over the study period. STUDY DESIGN: Data from the NSCH, an annual serial cross-sectional survey conducted from 2016 to 2019, were examined. Caregivers answered questions regarding one of their children within each of a random sample of households across the US. The primary analysis examined trends in health care transition preparation based on the year of survey completion. A secondary analysis examined the associations of race/ethnicity, primary household language, insurance type, and children with special health care needs (CSHCN) with receipt of health care transition preparation. RESULTS: We included data from 54 434 youths (20 708 in 2016, 8909 in 2017, 12 587 in 2018, and 12 230 in 2019) aged 12-17 years whose caregivers completed the NSCH between 2016 and 2019. The sample was weighted to be nationally representative based on weights provided by the NSCH. The proportion of youths receiving necessary health care transition preparation increased over the study period, from 14.8% in 2016 to 20.5% in 2019 (P < .001). Multivariable logistic regression demonstrated increased odds of receiving health care transition preparation in 2018 and 2019, as well as for White non-Hispanic youths, those with English or Spanish as a primary household language, those with private insurance, and CSHCN. CONCLUSIONS: Although the proportion of youths receiving health care transition preparation has increased since 2016, the need for ongoing improvement and elimination of disparities in health care transition preparation remains.


Asunto(s)
Niños con Discapacidad , Transición a la Atención de Adultos , Adolescente , Niño , Salud Infantil , Estudios Transversales , Accesibilidad a los Servicios de Salud , Humanos , Transferencia de Pacientes , Estados Unidos
8.
Am J Nephrol ; 53(1): 50-58, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35034024

RESUMEN

BACKGROUND: Cell therapies explore unmet clinical needs of patients with chronic kidney disease with the potential to alter the pathway toward end-stage kidney disease. We describe the design and baseline patient characteristics of a phase II multicenter clinical trial utilizing the novel renal autologous cell therapy (REACT), by direct kidney parenchymal injection via the percutaneous approach in adults with type 2 diabetic kidney disease (T2DKD), to delay or potentially avoid renal replacement therapy. DESIGN: The study conducted a prospective, multicenter, randomized control, open-label, phase II clinical trial between an active treatment group (ATG) receiving REACT from the beginning of the trial and a contemporaneous deferred treatment group (DTG) receiving standard of care for 12 months before crossing over to receive REACT. OBJECTIVES: The objective of this study was to establish the safety and efficacy of 2 REACT injections with computed tomography guidance, into the renal cortex of patients with T2DKD administered 6 months apart, and to compare the longitudinal change in renal function between the ATG and the DTG. SETTING: This was a multicenter study conducted in major US hospitals. PATIENTS: We enrolled eighty-three adult patients with T2DKD, who have estimated glomerular filtration rates (eGFRs) between 20 and 50 mL/min/1.73 m2. METHODS: All patients undergo an image-guided percutaneous kidney biopsy to obtain epithelial phenotype selective renal cells isolated from the kidney tissue that is then expanded ex vivo over 4-6 weeks, resulting in the REACT biologic product. Patients are randomized 1:1 into the ATG or the DTG. Primary efficacy endpoints for both study groups include eGFR measurements at baseline and at 3-month intervals, through 24 months after the last REACT injection. Safety analyses include biopsy-related complications, REACT injection, and cellular-related adverse events. The study utilizes Good Clinical and Manufacturing Practices and a Data and Safety Monitoring Board. The sample size confers a statistical power of 80% to detect an eGFR change in the ATG compared to the DTG at 24 months with an α = 0.05. LIMITATIONS: Blinding cannot occur due to the intent to treat procedure, biopsy in both groups, and open trial design. CONCLUSION: This multicenter phase II randomized clinical trial is designed to determine the efficacy and safety of REACT in improving or stabilizing renal function among patients with T2DKD stages 3a-4.


Asunto(s)
Diabetes Mellitus Tipo 2 , Nefropatías Diabéticas , Insuficiencia Renal Crónica , Tratamiento Basado en Trasplante de Células y Tejidos , Ensayos Clínicos como Asunto , Diabetes Mellitus Tipo 2/complicaciones , Diabetes Mellitus Tipo 2/terapia , Nefropatías Diabéticas/diagnóstico , Femenino , Tasa de Filtración Glomerular , Humanos , Riñón/fisiología , Masculino , Estudios Prospectivos , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/terapia , Resultado del Tratamiento
9.
J Clin Gastroenterol ; 56(9): 805-809, 2022 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-34907925

RESUMEN

OBJECTIVE: The objective of this study was to describe in detail the trends of the Transition Readiness Assessment Questionnaire (TRAQ-20) with respect to patient age and gender among a cohort of 655 youth with inflammatory bowel disease (IBD) to enhance the tool's utility in clinical and research settings. MATERIALS AND METHODS: All participants in the Crohn's and Colitis Foundation's IBD Partners and IBD Partners Kids and Teens Cohorts ages 12 to 21 who completed the TRAQ-20 at least once were included. We computed centile scores for the TRAQ-20 and applied Kernel regression to generate curves. We computed mean scores for each TRAQ-20 domain by age for the overall cohort and stratified by gender. RESULTS: TRAQ-20 scores generally increase with age. Males have a steeper trajectory from ages 17 to 19 compared with females. By age 21, median overall TRAQ-20 scores for women and men were 4.4 and 4.7, respectively. The "Talking with Providers" domain of the TRAQ-20 had high scores throughout the age range of the cohort, while others had lower scores in younger ages and higher scores in older ages. All TRAQ-20 domains had mean scores of 4 or greater (out of a possible 5) among 21-year-olds. CONCLUSIONS: TRAQ-20 scores increase with age. The rate of increase varies by gender. Rates of increase also differ across domains. The use of transition readiness growth curves can help providers and researchers identify patients who are not achieving expected levels of transition readiness and determine the level of transition readiness that a patient much achieve before transfer to adult care.


Asunto(s)
Enfermedad de Crohn , Enfermedades Inflamatorias del Intestino , Transición a la Atención de Adultos , Adolescente , Adulto , Niño , Enfermedad Crónica , Enfermedad de Crohn/diagnóstico , Femenino , Humanos , Enfermedades Inflamatorias del Intestino/diagnóstico , Masculino , Encuestas y Cuestionarios , Adulto Joven
10.
Blood Purif ; : 1-8, 2022 Aug 25.
Artículo en Inglés | MEDLINE | ID: mdl-36007503

RESUMEN

BACKGROUND: Long-term peritoneal dialysis (PD), especially with nonphysiological solutions, is afflicted with the severe complication of encapsulating peritoneal sclerosis (EPS). Physiologic PD solutions have been introduced to reduce pH trauma. Data on peritoneal biopsies in pediatrics with long-term PD using physiological solutions are scant. CASE REPORT: We report an adolescent who had been on 10-h continuous hourly cycles using mostly 2.27% Physioneal™ for 5 years. There were two episodes of peritonitis in October 2017 (Klebsiella oxytoca) and May 2018 (Klebsiella pneumoniae), which were treated promptly. This adolescent, who lost two kidney transplants from recurrent focal and segmental glomerulosclerosis, underwent a peritoneal membrane biopsy at the time of a third PD catheter placement, 16 months after the second renal transplant. Laparoscopically, the peritoneum appeared grossly normal, but fibrosis and abundant hemosiderin deposition were noted on histology. The thickness of the peritoneum was 200-900 (mean 680) µm; normal for age of 14 years is 297 [IQR 229, 384] µm. The peritoneum biopsy did not show specific EPS findings, as the mesothelial cells were intact, and there was a lack of fibrin exudation, neo-membrane, fibroblast proliferation, infiltration, or calcification. CONCLUSIONS: While the biopsy was reassuring with respect to the absence of EPS, significant histopathological changes suggest that avoiding pH trauma may not ameliorate the effects of glucose exposure in long-term PD.

11.
J Pediatr Nurs ; 62: 129-135, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-34801325

RESUMEN

PURPOSE: We evaluated disease knowledge/self-management skills among low-income Mexican young adults maintained on dialysis and to test the effectiveness of the A.L.L. Y.O.U. N.E.E.D. I.S. L.O.V.E (AYNIL) Manual - Spanish Version on patient-reported outcomes. This is a low literacy teaching tool designed with patients and educators' input. DESIGN AND METHODS: A quasi-experimental study was conducted in 17 chronic dialysis patients at Mexico City's Hospital General de México, Dr. Eduardo Liceaga. Ages 18-30-year-old completed disease knowledge/self-management and quality of life measures before the intervention and 6 weeks later. RESULTS: Significant increases were observed on disease knowledge/self-management scores in the STARx questionnaire from 47 (IQ: 40,51) to 50 (IQ: 48,54) p = 0.04. The UNC-TRxANSITION Index increased significantly from 4.8 (IQ: 3.9,5.7) to 7.7 (IQ: 7.5,8.2) p ≤0.001. Significant increases in scores were detected in the "Burden of kidney disease" (p = 0.008), "Effects of kidney disease" " (p = 0.03) and " Dialysis staff encouragement" (p = 0.027) based on the KDQoL survey. CONCLUSIONS: In this vulnerable population, the Spanish version of the A.L.L. Y.O.U. N.E.E.D. I.S. L.O.V.E. - AYNIL Manual improved CKD/ESRD disease knowledge/self-management skills and HRQoL. This study highlighted the need for low-literacy educational tools to improve patient-reported outcomes. PRACTICE IMPLICATIONS: Young adults with CKD/ESRD can benefit from patient-centered educational interventions to enhance their autonomy and the development of self-management behaviors that improve patient-reported outcomes and potential complications of the disease. Special attention is needed in low-income patients with low rates of adherence to treatments and poor self-management skills.


Asunto(s)
Automanejo , Adolescente , Adulto , Estudios de Factibilidad , Humanos , México , Medición de Resultados Informados por el Paciente , Calidad de Vida , Diálisis Renal , Adulto Joven
12.
Pediatr Transplant ; 25(5): e14062, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34076958

RESUMEN

BACKGROUND: Pre-emptive kidney transplantation for end-stage kidney disease in children has many advantages and may lead to the consideration of marginal parent donors. METHODS: Using the example of the transplant of a kidney with medullary sponge disease from a parent to the child, we review the ethical framework for working up such donors. RESULTS: The four principles of health ethics include autonomy (the right of the patient to retain control over his/her own body); beneficence (healthcare providers must do all they can do to benefit the patient in each situation); non-maleficence ("first do no harm"-providers must consider whether other people or society could be harmed by a decision made, even if it is made for the benefit of an individual patient) and justice (there should be an element of fairness in all medical decisions). Highly motivated donors may derive significant psychological benefit from their donation and may thus be willing to incur more risk. The transplantation team and, ideally, an independent donor advocate team must make a judgment about the acceptability of the risk-benefit ratio for particular potential donors, who must also make their own assessment. The transplantation team and donor advocate team must be comfortable with the risk-benefit ratio before proceeding. CONCLUSIONS: An independent donor advocacy team that focuses on the donor needs is needed with sufficient multidisciplinary ethical, social, and psychological expertise. The decision to accept or reject the donor should be within the authority of the independent donor advocacy team and not the providers or the donor.


Asunto(s)
Selección de Donante/ética , Fallo Renal Crónico/cirugía , Trasplante de Riñón/ética , Donadores Vivos/ética , Riñón Esponjoso Medular/cirugía , Padres , Adolescente , Adulto , Niño , Preescolar , Toma de Decisiones Clínicas/ética , Toma de Decisiones Clínicas/métodos , Toma de Decisiones , Selección de Donante/métodos , Femenino , Humanos , Lactante , Recién Nacido , Fallo Renal Crónico/etiología , Trasplante de Riñón/métodos , Masculino , Riñón Esponjoso Medular/fisiopatología , Defensa del Paciente/ética , Riesgo
13.
Pediatr Nephrol ; 36(11): 3621-3626, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34427794

RESUMEN

BACKGROUND: Polyuria is a common problem in patients with tubular diseases, especially for those with CKD and high-output Fanconi syndrome. There are currently no guidelines on how to treat debilitating polyuria, in children or adults, and vasopressin is usually not effective. CASE-DIAGNOSIS/TREATMENT: A 13-year-old female with idiopathic Fanconi syndrome and an eGFR of 69 mL/min/1.73 m2 was severely affected by polyuria of 5 L per day (voiding at least 11 times during the day and up to 8 times at night), impacting her mood (measured by the RCADS-child) and academic performance at school. In the absence of guidelines and with literature discouraging the use of indomethacin in this condition, we attempted indomethacin treatment at a dose of 2 mg/kg divided in two doses with substantial success. Urine output dropped to 2.5L and this was accompanied by a substantial decrease of her sodium wasting from 24.6 to 7.7 mmol/kg/day. Over the course of 18 months, the patient's eGFR dropped temporarily to 60 mL/min/1.73 m2 and was 68 mL/min/1.73 m2 at last follow-up. However, a sodium-23 (23Na) MRI of her thigh revealed ongoing moderate sodium decrease in her skin and substantial Na+ decrease in her muscle when compared to age-matched peers with normal kidney function. CONCLUSIONS: Indomethacin may be a safe and effective treatment option for polyuria in idiopathic Fanconi syndrome.


Asunto(s)
Síndrome de Fanconi , Poliuria , Adolescente , Síndrome de Fanconi/complicaciones , Femenino , Humanos , Indometacina/uso terapéutico , Poliuria/tratamiento farmacológico , Poliuria/fisiopatología , Índice de Severidad de la Enfermedad
14.
Pediatr Nephrol ; 36(5): 1307-1310, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33547529

RESUMEN

INTRODUCTION: The number of medications could serve as a surrogate for burden of care at home and may affect health-related quality of life (HRQoL) in children with chronic kidney disease (CKD). METHODS: Using baseline data from the Chronic Kidney Disease in Children (CKiD) Study, we modeled HRQoL scores, self-reported by the child (if ≥ 8 years old) and/or caregiver (all children) on unique counts and administrations of CKD- and non-CKD-related medications, using multivariate linear regression. Heterogeneity of associations between HRQoL and medication burden by age group (≥ 8 vs. < 8 years old) were explored. RESULTS: 734 participants median age 11 years, disease duration 8 years, median eGFR 53 mL/min/1.73 m2, 61% male, 22% African-American, 31% glomerular disease were prescribed median 3 unique CKD-related medications. Regarding HRQoL assessment, 201 children were < 8 years old and had only parent-proxy HRQoL score; 533 children ≥ 8 years of age had both child and parent-proxy scores. Overall, parents of children < 8 years old reported higher HRQoL scores than parents of older children: 84 vs. 76. However, in a unified multivariate regression model, HRQoL scores of children < 8 years showed greater decreases as the number of CKD-related medications increased compared to scores for children ≥ 8 years old. CONCLUSION: Average HRQoL scores reported by parents of younger CKD children were higher than those of older CKD children but decreased more with increased CKD medication counts than scores of older children. Considerations of HRQoL may be of particular importance for clinicians and caregivers when managing chronic disease comorbidities in younger children.


Asunto(s)
Calidad de Vida , Insuficiencia Renal Crónica , Adolescente , Cuidadores , Niño , Enfermedad Crónica , Femenino , Humanos , Masculino , Padres
15.
Blood Purif ; 50(4-5): 678-683, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33647913

RESUMEN

BACKGROUND: Advanced cell therapies with autologous, homologous cells show promise to affect reparative and restorative changes in the chronic kidney disease (CKD) nephron. We present our protocol and preliminary analysis of an IRB-approved, phase I single-group, open-label trial that tests the safety and efficacy of Renal Autologous Cell Therapy (REACT; NCT04115345) in adults with congenital anomalies of the kidney and urinary tract (CAKUT). METHODS: Adults with surgically corrected CAKUT and CKD stages 3 and 4 signed an informed consent and served as their "own" baseline control. REACT is an active biological ingredient acquired from a percutaneous tissue acquisition from the patient's kidney cortex. The specimen undergoes a GMP-compliant manufacturing process that harvests the selected renal cells composed of progenitors for renal repair, followed by image-guided locoregional reinjection into the patient's renal cortex. Participants receive 2 doses at 6-month intervals. Primary outcomes are stable renal function and stable/improved quality of life. Additional exploratory endpoints include the impact of REACT on blood pressure, vitamin D levels, hemoglobin, hematocrit and kidney volume by MRI analysis. RESULTS: Four men and 1 woman were enrolled and underwent 5 cell injections. Their characteristics were as follows: mean 52.8 years (SD 17.7 years), 1 Hispanic, 4 non-Hispanic, and 5 white. There were no renal tissue acquisition, cell injection, or cell product-related complications at baseline. CONCLUSION: REACT is demonstrating feasibility and patient safety in preliminary analysis. Autologous cell therapy treatment has the potential to stabilize or improve renal function in CAKUT-associated CKD to delay or avert dialysis. Patient enrollment and follow-up are underway.


Asunto(s)
Corteza Renal/citología , Insuficiencia Renal Crónica/terapia , Trasplante de Células Madre , Células Madre/citología , Trasplante Autólogo , Adulto , Tratamiento Basado en Trasplante de Células y Tejidos , Células Cultivadas , Femenino , Humanos , Masculino , Persona de Mediana Edad , Calidad de Vida , Insuficiencia Renal Crónica/complicaciones , Trasplante de Células Madre/métodos , Trasplante Autólogo/métodos , Sistema Urinario/anomalías , Adulto Joven
16.
Blood Purif ; 50(4-5): 684-695, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33706317

RESUMEN

Adolescents and young adults (AYAs) with CKD or end-stage kidney disease (ESKD) have unique medical, dental, psychosocial, neurocognitive, and academic needs and require close interdisciplinary collaboration to optimize their care. The etiology of CKD in AYAs is diverse compared to older adults. With their continuously improved survival, AYAs must start preparation for health-care transition (HCT) from pediatric- to adult-focused health care in the pediatric setting and it must continue at the adult-focused setting, given that their brain maturation and self-management skill acquisition occur until their mid-20s. While the growth and physical maturation of most visible body parts occur before 18 years of age, the prefrontal cortex of the brain, where reasoning, impulse control, and other higher executive functions reside, matures around 25 years of age. The HCT process must be monitored using patient- and caregiver-measuring tools to guide interventions. The HCT process becomes more complex when patients and/or caregivers have a language barrier, different cultural beliefs, or lower literacy levels. In this article, we discuss the unique comorbidities of pediatric-onset CKD/ESKD, provide information for a planned HCT preparation, and suggest interdisciplinary coordination as well as cultural and literacy-appropriate activities to achieve optimal patient outcomes.


Asunto(s)
Insuficiencia Renal Crónica , Transición a la Atención de Adultos , Adolescente , Adulto , Cuidadores , Humanos , Fallo Renal Crónico/terapia , Insuficiencia Renal Crónica/terapia , Automanejo , Transición a la Atención de Adultos/organización & administración , Adulto Joven
17.
Acta Paediatr ; 110(3): 773-780, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-32869283

RESUMEN

AIM: Review of current knowledge on assessing renal function in term and preterm neonates. METHODS: Literature review and analysis of own data. RESULTS: Prematurity, genetic, environmental and maternal factors may alter peak nephron endowment and life-long renal function. Nephrogenesis continues until 34-36 weeks of gestation, but it is altered with premature delivery. Variability of nephron endowment has a substantial impact on the clearance of renally excreted drugs. Postnatally, glomerular function rate (GFR) increases daily, doubles by two weeks, and slowly reaches full maturity at 18 months of age. Ideally, renal function biomarkers should be expressed as age-independent z-scores, and evidence suggests indexing these values to post-conceptual age rather than chronological age. Newborn and maternal serum creatinine correlate tightly for more than 72 hours after delivery, rendering this biomarker unsuitable for the assessment of neonatal renal function. Cystatin C does not cross the placenta and may be the preferred biomarker in the neonate. Here, we provide preliminary data on the natural evolution of the cystatin C eGFR in infancy. CONCLUSION: Cystatin C may be superior for GFR estimation in neonates, but the best approach to drug dosing of renally excreted drugs remains to be established.


Asunto(s)
Recién Nacido de Bajo Peso , Recien Nacido Prematuro , Biomarcadores , Creatinina , Femenino , Tasa de Filtración Glomerular , Humanos , Lactante , Recién Nacido , Embarazo
18.
J Pediatr Nurs ; 58: 1-8, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33278706

RESUMEN

PURPOSE: To develop and evaluate a parent-proxy measure of youth HCT readiness: the TRxANSITION Index-Parent Version. DESIGN AND METHODS: We recruited parents (77% female) and youth (ages 12 to 25) to complete transition readiness measures during outpatient clinic visits. The TRxANSITION Index-Parent Version contains two domains: the Parent Knowledge Domain assessing a parent's knowledge of their youth's illness, and the Parent Proxy Domain, which provides a parental perspective regarding a youth's transition readiness skills. We evaluated the TRxANSITION Index - Parent Version for differences between parent and youth reports of HCT readiness, associations between parent's score and youth's characteristics, and item-category, item-sub-index, and sub-index category correlations. RESULTS: Data from 93 parents-youth dyads were analyzed. Parents scored significantly higher than youth in the Parent Knowledge Domain and similarly in the Parent Proxy Domain. Parents of daughters had significantly higher scores in the Parent Knowledge Domain than parents of sons and reported similar scores to Parents of sons in the Parent Proxy Domain. Only the self-management sub-index significantly correlated with youth's age. The sub-index-domain, item-sub-index, and item-domain correlations assessed were generally large in magnitude (r > 0.5). CONCLUSIONS: The TRxANSITION Index-Parent Version shows promise as a means of assessing parent knowledge of a youth's illness and may provide an accurate proxy assessment of a youth HCT readiness skills. PRACTICE IMPLICATIONS: Obtaining parental perspective on a youth's HCT readiness may provide useful clinical information during the transition process.


Asunto(s)
Automanejo , Transición a la Atención de Adultos , Adolescente , Adulto , Niño , Enfermedad Crónica , Femenino , Humanos , Masculino , Padres , Adulto Joven
19.
J Sch Nurs ; : 10598405211053266, 2021 Dec 20.
Artículo en Inglés | MEDLINE | ID: mdl-34928723

RESUMEN

Background: Adolescents and young adults (AYA) with chronic conditions should acquire self-management skills as part of their healthcare transition (HCT) from pediatric to adult-focused care. HCT/self-management skills have the potential to help mitigate health disparities among minority AYA with chronic conditions. This study investigated school nurses' practices promoting HCT/self-management skills in urban public schools. Methods: Seventy-nine nurses from three urban school districts in Massachusetts completed a survey of 32 Likert-type questions on HCT/self-management skills, eight demographic questions, and five open-ended practice questions assessing how often they have asked students with chronic conditions about HCT/self-management skills, based on the UNC TRxANSITION IndexTM. Results: Among the 79 school nurses who participated (response rate 76%), 67% never or rarely assessed students' knowledge of HCT/self-management, and 90% would use a tool that promotes/measures HCT/self-management skills. Conclusion: In our study sample, most school nurses acknowledged the importance of assessing HCT/self-management skills. The majority favored using a tool to promote these skills.

20.
Matern Child Health J ; 24(6): 679-686, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32277385

RESUMEN

INTRODUCTION: Clinical experiences expose learners to the patient perspective, which can have a lasting impact on students' professional identity. However, in busy clinical settings where opportunities for reflection may be limited, listening to patients' stories is often neglected. We used a reflection assignment to augment a new maternal-child health integrated curriculum. METHODS: Medical students completed a brief assignment from their session in the neonatal intensive care unit (NICU), which included reflective writing, between fall 2016 and summer 2017. The Depth of Reflection rubric was used to score reflections on a scale: "Knowledge and Comprehension" (Level I), "Analysis" (Level II), and "Synthesis and Evaluation" (Level III). A constant comparison method based on grounded theory elicited prenatal and postnatal themes from medical students' reflective writing. RESULTS: All students completed narratives (n = 166); 70% (n = 116) achieved a Depth of Reflection of Level II or III. Six overarching themes emerged: (1) Conception, Pregnancy, and Delivery Experiences; (2) Positive Support Structures; (3) Barriers and Stressors to Care; (4) Future Plans; (5) Unexpected Complications; and (6) Student Career and Professional Considerations. DISCUSSION: Reflections from a novel and brief integrated maternal-child health experience demonstrated high levels on the Depth of Reflection scale. This experience exposed students to core themes central to a family's pregnancy and perinatal experience. Professional identity formation also emerged as a theme. Reflective writing assignments in a busy NICU can facilitate exploration of medical students' knowledge of maternal-child health patient experiences.


Asunto(s)
Actitud del Personal de Salud , Salud Infantil , Educación Médica/métodos , Salud Materna , Estudiantes de Medicina/psicología , Femenino , Humanos , Embarazo , Escritura
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