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2.
J Pediatr ; 248: 21-29.e1, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35660017

RESUMEN

OBJECTIVE: To test the impact of childhood adversity, including community violence exposure, on hypertension risk in Black American young adults to understand what risk factors (eg, prenatal factors, later exposures) and ages of adversity exposure increased hypertension risk. STUDY DESIGN: The study included 396 Black American participants with data from prenatal, birth, and age 7-, 14-, and 19-year visits. At age 19 years, individuals with blood pressure (BP) measures >120 mmHg systolic and/or >80 mmHg diastolic were classified as having high blood pressure (HBP), and those with BP <120/80 mmHg were classified as normal. Associations between prenatal and birth risk factors; childhood adversity at age 7, 14, and 19 years; age 19 body mass index (BMI); and both systolic and diastolic BP at age 19 were tested using logistic regression models. RESULTS: Age 19 BMI was positively associated with systolic and diastolic HBP status at age 19. Controlling for all covariates, community violence exposure at age 7 and 19 years was associated with 2.2-fold (95% CI, 1.242-3.859) and 2.0-fold (95% CI, 1.052-3.664) greater odds of systolic HBP, respectively, at age 19 years. Prenatal risk, birth risk, and other dimensions of childhood adversity were not associated with HBP in this cohort. CONCLUSION: Childhood community violence exposure is a significant risk factor for HBP in young adults. As Black American children typically experience more community violence exposure than other American children, our results suggest that racial disparities in childhood community violence exposure may contribute to racial disparities in adult hypertension burden.


Asunto(s)
Exposición a la Violencia , Hipertensión , Adolescente , Adulto , Presión Sanguínea , Niño , Estudios de Cohortes , Femenino , Humanos , Hipertensión/epidemiología , Hipertensión/etiología , Embarazo , Factores de Riesgo , Adulto Joven
3.
Pediatr Transplant ; 26(4): e14241, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35122460

RESUMEN

BACKGROUND: BKV and BKVN are common in pediatric kidney transplant, but there is limited data on treatment approaches. Our objective was to study the prevalence of BKV and BKVN utilizing only plasma qPCR and report treatment outcomes with stepwise IR and IVIG. METHODS: A retrospective study of all pediatric kidney transplants from 2013 to 2020. Excluded patients >21 years at transplant and immediate graft failure. Surveillance was conducted using only plasma BK qPCR at 1, 3, 6, 9, 12, 18, and 24 months and annually. BKV defined as ≥250 copies/ml and resolution as <250 copies/ml. Presumed BKVN as >10 000 copies/ml despite IR; and BKVN if confirmed on histology. RESULTS: Fifty-six patients were included in the study; 20 (35.7%) had BKV. BKV was associated with longer duration of stent, 40 vs. 33.5 days (p = .004). Two patients (3.5%) had confirmed, and 2(3.5%) had presumed BKVN. The first-line treatment was IR in 100% of patients. BKVN confirmed and presumed received IVIG every month for six doses. Viral resolution was achieved in 70%, and no difference was noted in estimated glomerular filtration rate between BKV and non-BKV group (p = .438). There were no rejection episodes, and graft survival was 100% over median follow-up of 3 years. CONCLUSIONS: Plasma qPCR alone is adequate for screening and monitoring treatment of BKV and BKVN. A stepwise IR and IVIG resulted in BKV resolution in the majority of patients. Larger studies are required to study the role of IVIG in the treatment of BKVN.


Asunto(s)
Virus BK , Síndromes de Inmunodeficiencia , Enfermedades Renales , Trasplante de Riñón , Infecciones por Polyomavirus , Infecciones Tumorales por Virus , Niño , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Síndromes de Inmunodeficiencia/complicaciones , Terapia de Inmunosupresión , Enfermedades Renales/complicaciones , Masculino , Infecciones por Polyomavirus/epidemiología , Estudios Retrospectivos , Infecciones Tumorales por Virus/epidemiología
4.
Pediatr Nephrol ; 37(5): 1097-1103, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-34626243

RESUMEN

INTRODUCTION: No data exist on the epidemiology of children incidentally diagnosed with advanced kidney failure (KF) during evaluation for non-specific symptoms. This is likely related to unrecognized symptoms and signs of CKD. The objective of our study was to evaluate incidentally diagnosed patients with advanced KF requiring long-term kidney replacement therapy (KRT). METHODS: An IRB-approved retrospective chart review of children who started KRT with dialysis (hemo- or peritoneal) was conducted. Included were children with no prior knowledge or diagnosis of underlying kidney disease with chronic kidney disease (CKD) disease stage 4 (GFR 15-29 mL/min/1.73 m2) or 5 (GFR < 15 mL/min/1.73 m2) at initial presentation and started on chronic KRT within 2 months of presentation. RESULTS: Of 177 patients initiating KRT during the study period, 26 (15%) were categorized as incidental advanced KF. This cohort with mean age 12.25 years consisted of 42% males, 54% African Americans included 46% with glomerular, and 54% with non-glomerular etiology for kidney failure. Vomiting (42%) and fatigue (39%) were most common, while growth failure (19%) and hyperkalemia (7%) were less frequent on initial presentation. Anemia (100%), hypertension (96%), hyperparathyroidism (96%), and hyperphosphatemia (92%) were the most frequently seen CKD comorbidities. Chronic KRT was started within 24 h in 62% and within 2 weeks in 88% of the cohort. CONCLUSION: Under-diagnosis of patients with advanced KF is most likely related to milder non-specific clinical symptoms and normal growth in the majority of patients. A higher resolution version of the Graphical abstract is available as Supplementary information.


Asunto(s)
Diálisis Renal , Insuficiencia Renal Crónica , Niño , Estudios de Cohortes , Femenino , Humanos , Masculino , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/etiología , Terapia de Reemplazo Renal , Estudios Retrospectivos
5.
Kidney Int Rep ; 6(9): 2316-2322, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34514192

RESUMEN

INTRODUCTION: The slow increase in use of telemedicine began to expand rapidly, along with reimbursement changes, during the coronavirus disease-2019 (COVID-19) pandemic. Standardized protocols for these services are lacking but are needed for effective and equitable health care. In this study, we queried pediatric nephrologists and their patients about their telemedicine experiences during the pandemic. METHODS: Surveys that were in compliance with the Health Insurance Portability and Accountability Act were deployed online to patients and physicians. RESULTS: We collected survey responses from 400 patients and 197 pediatric nephrologists. Patients reported positive experiences with telemedicine visits as it was logistically easier than in-person visits. Patients also felt that the quality of their visits were equivalent to what they would receive in person. Physicians used a wide variety of online systems to conduct synchronous telemedicine with Zoom (23%), EPIC (9%), Doxy.me (7%), services not specified (37%), or a mix of local or smaller services (24%). Most physicians' concerns were related to technological issues and the ability to procure physical exams and/or laboratory results. CONCLUSIONS: There is a paucity of published trials on telemedicine services in pediatric nephrology. Virtual care was feasible and acceptable for patients, caregivers, and providers during the COVID-19 pandemic.

6.
Int J Radiat Oncol Biol Phys ; 110(2): 412-424, 2021 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-33316362

RESUMEN

PURPOSE: Hypofractionated radiation therapy can be used to treat patients with muscle-invasive bladder cancer unable to have radical therapy. Toxicity is a key concern, but adaptive plan-of the day (POD) image-guided radiation therapy delivery could improve outcomes by minimizing the volume of normal tissue irradiated. The HYBRID trial assessed the multicenter implementation, safety, and efficacy of this strategy. METHODS: HYBRID is a Phase II randomized trial that was conducted at 14 UK hospitals. Patients with T2-T4aN0M0 muscle-invasive bladder cancer unsuitable for radical therapy received 36 Gy in 6 weekly fractions, randomized (1:1) to standard planning (SP) or adaptive planning (AP) using a minimization algorithm. For AP, a pretreatment cone beam computed tomography (CT) was used to select the POD from 3 plans (small, medium, and large). Follow-up included standard cystoscopic, radiologic, and clinical assessments. The primary endpoint was nongenitourinary Common Terminology Criteria for Adverse Events (CTCAE) grade ≥ 3 (≥G3) toxicity within 3 months of radiation therapy. A noncomparative single stage design aimed to exclude ≥30% toxicity rate in each planning group in patients who received ≥1 fraction of radiation therapy. Local control at 3-months (both groups combined) was a key secondary endpoint. RESULTS: Between April 15, 2014, and August 10, 2016, 65 patients were enrolled (SP, n = 32; AP, n = 33). The median follow-up time was 38.8 months (interquartile range [IQR], 36.8-51.3). The median age was 85 years (IQR, 81-89); 68% of participants (44 of 65) were male; and 98% of participants had grade 3 urothelial cancer. In 63 evaluable participants, CTCAE ≥G3 nongenitourinary toxicity rates were 6% (2 of 33; 95% confidence interval [CI], 0.7%-20.2%) for the AP group and 13% (4 of 30; 95% CI, 3.8%-30.7%) for the SP group. Disease was present in 9/48 participants assessed at 3 months, giving a local control rate of 81.3% (95% CI, 67.4%-91.1%). CONCLUSIONS: POD adaptive radiation therapy was successfully implemented across multiple centers. Weekly ultrahypofractionated 36 Gy/6 fraction radiation therapy is safe and provides good local control rates in this older patient population.


Asunto(s)
Radioterapia Guiada por Imagen , Neoplasias de la Vejiga Urinaria/radioterapia , Anciano de 80 o más Años , Algoritmos , Tomografía Computarizada de Haz Cónico/efectos adversos , Tomografía Computarizada de Haz Cónico/métodos , Estudios de Factibilidad , Femenino , Humanos , Masculino , Estadificación de Neoplasias , Medición de Resultados Informados por el Paciente , Hipofraccionamiento de la Dosis de Radiación , Planificación de la Radioterapia Asistida por Computador/métodos , Radioterapia Guiada por Imagen/efectos adversos , Radioterapia Guiada por Imagen/métodos , Factores de Tiempo , Resultado del Tratamiento , Reino Unido , Neoplasias de la Vejiga Urinaria/patología
7.
Kidney Int ; 100(1): 225-237, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-33307104

RESUMEN

Ravulizumab, a long-acting complement C5 inhibitor engineered from eculizumab, allows extending maintenance dosing from every 2-3 weeks to every 4-8 weeks depending on bodyweight. Here, we evaluated the efficacy and safety of ravulizumab in complement inhibitor-naïve children (under 18 years) with atypical hemolytic uremic syndrome. In this phase III, single-arm trial, ravulizumab was administered every eight weeks in patients 20 kg and over, and four weeks in patients under 20 kg. The primary endpoint was a complete thrombotic microangiopathy response (normalization of platelet count and lactate dehydrogenase, and a 25% or more improvement in serum creatinine) through 26 weeks. Secondary endpoints included change in hematologic parameters and kidney function. 18 patients with a median age of 5.2 years were evaluated. At baseline, symptoms of atypical hemolytic uremic syndrome outside the kidney were present in 72.2% of patients and 38.9% had been in intensive care. Baseline estimated glomerular filtration rate was 22 mL/min/1.73 m2. By week 26, 77.8% of patients achieved a complete thrombotic microangiopathy response; 94.4%, 88.9% and 83.3% of patients achieved platelet normalization, lactate dehydrogenase normalization and a 25% or more improvement in serum creatinine, respectively. By week 50, 94.4% patients had achieved a complete thrombotic microangiopathy response. Median improvement in platelet count was 246 and 213 x109/L through week 26 and week 50, respectively. The median increase above baseline in estimated glomerular filtration rate was 80 and 94 mL/min/1.73m2 through week 26 and week 50, respectively. No unexpected adverse events, deaths, or meningococcal infections occurred. Thus, ravulizumab rapidly improved hematologic and kidney parameters with no unexpected safety concerns in complement inhibitor-naïve children with atypical hemolytic uremic syndrome.


Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico Urémico Atípico , Inactivadores del Complemento/uso terapéutico , Microangiopatías Trombóticas , Adolescente , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Niño , Preescolar , Complemento C5 , Humanos , Microangiopatías Trombóticas/diagnóstico , Microangiopatías Trombóticas/tratamiento farmacológico
8.
Front Pediatr ; 8: 588911, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33194923

RESUMEN

Hypertensive crisis can be a source of morbidity and mortality in the pediatric population. While the epidemiology has been difficult to pinpoint, it is well-known that secondary causes of pediatric hypertension contribute to a greater incidence of hypertensive crisis in pediatrics. Hypertensive crisis may manifest with non-specific symptoms as well as distinct and acute symptoms in the presence of end-organ damage. Hypertensive emergency, the form of hypertensive crisis with end-organ damage, may present with more severe symptoms and lead to permanent organ damage. Thus, it is crucial to evaluate any pediatric patient suspected of hypertensive emergency with a thorough workup while acutely treating the elevated blood pressure in a gradual manner. Management of hypertensive crisis is chosen based on the presence of end-organ damage and can range from fast-acting intravenous medication to oral medication for less severe cases. Treatment of such demands a careful balance between decreasing blood pressure in a gradual manner while preventing damage end-organ damage. In special situations, protocols have been established for treatment of hypertensive crisis, such as in the presence of endocrinologic neoplasms, monogenic causes of hypertension, renal diseases, and cardiac disease. With the advent of telehealth, clinicians are further able to extend their reach of care to emergency settings and aid emergency medical service (EMS) providers in real time. In addition, further updates on the evolving topic of hypertension in the pediatric population and novel drug development continues to improve outcomes and efficiency in diagnosis and management of hypertension and consequent hypertensive crisis.

9.
J Clin Hypertens (Greenwich) ; 22(6): 1059-1069, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32472978

RESUMEN

The morbidity and mortality of adult and pediatric chronic kidney disease (CKD) and end-stage renal disease (ESRD) populations are mainly driven by cardiovascular disease (CVD). Improving CVD outcomes focuses on risk assessment of factors including diastolic blood pressure (DBP), systolic blood pressure (SBP), left ventricular mass index (LVMI), pulse pressure (PP), and pulse pressure index (PPi), which is calculated as PP/SBP. These markers are also proven predictors of CKD progression; however, their role in children has not been established. This study aims to evaluate the relationship between PP, PPi, ambulatory arterial stiffness index (AASI), and proteinuria with kidney function in pediatric CKD patients; it is a retrospective analysis of 620 patients (1-16 years) from the NIDDK Chronic Kidney Disease in Children (CKiD) registry. The authors analyzed data for three separate cohorts: an overall CKD as well as immunological versus non-immunological cause for CKD groups. An inverse relationship was found between SBP, DBP, and PP with iGFR and LVMI in the overall CKD group. Our immunological CKD subgroup showed significantly higher serum creatinine, SBP, DBP, and PP values with significantly lower serum albumin levels compared to the non-immunological group. There were no significant differences with iohexol-based glomerular filtration rate (iGFR), LVMI, PPi, or high-sensitivity C-reactive protein (hs-CRP) between the two groups. A subgroup analysis demonstrated that SBP, DBP, and PP all correlated significantly with LVMI in the immunological CKD patients but not the non-immunological subgroup. Additionally, AASI data in the overall CKD population were significantly correlated with PP, PPi, and DBP. This study is one of the first to correlate noninvasive measurements of vascular compliance including PP, PPi, and AASI with iGFR and LVMI in a pediatric CKD cohort. Improving our understanding of surrogate markers for early CVD is integral to improving the care of pediatric CKD population as these patients have yet to develop the hard end points of ESRD, heart failure, myocardial infarction, or stroke.


Asunto(s)
Hipertensión , Insuficiencia Renal Crónica , Rigidez Vascular , Adulto , Presión Sanguínea , Niño , Estudios Transversales , Humanos , Hipertensión/patología , Hipertensión/fisiopatología , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/patología , Insuficiencia Renal Crónica/fisiopatología , Estudios Retrospectivos , Factores de Riesgo
10.
Pediatr Nephrol ; 35(4): 581-594, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-30603807

RESUMEN

Pheochromocytomas and paragangliomas (PPGLs) generally grouped together are rare catecholamine-secreting endocrine tumors. Symptoms of catecholamine excess are non-specific and therefore a high index of suspicion in children with sustained hypertension, family history of endocrine tumors, or features of syndromes associated with PPGLs leads to a timely diagnosis and treatment. Free metanephrines in the plasma or 24-h urine are the preferred tests to establish catecholamine excess. Considerations for false-positive conditions improve diagnostic yield and accuracy. Functional imaging, targeting either specific cell membrane transporters or vesicular catecholamine transport systems, is indicated for incidental lesions suspicious for PPGLs with inconclusive biochemical testing, assessment of regional extension or multifocality, and exclusion of metastases. Surgery is the mainstay of treatment for PPGLs. Preoperatively, sequential use of alpha adrenergic receptor blockade and volume expansion followed by beta blockade is mandatory to reduce intraoperative intravascular instability and blood pressure fluctuation due to tumor manipulation. Since genetic mutations have been reported in tumor susceptibility genes in nearly 50% of patients with PPGLs, genetic counselling and testing should be considered in all patients with a confirmed tumor.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico , Paraganglioma/diagnóstico , Feocromocitoma/diagnóstico , Adolescente , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/fisiopatología , Neoplasias de las Glándulas Suprarrenales/terapia , Catecolaminas/análisis , Niño , Femenino , Pruebas Genéticas , Mutación de Línea Germinal , Humanos , Masculino , Paraganglioma/genética , Paraganglioma/fisiopatología , Paraganglioma/terapia , Feocromocitoma/genética , Feocromocitoma/fisiopatología , Feocromocitoma/terapia
11.
Pediatr Nephrol ; 34(11): 2427-2448, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31446483

RESUMEN

BACKGROUND: Intentional or unintentional ingestions among children and adolescents are common. There are a number of ingestions amenable to renal replacement therapy (RRT). METHODS: We systematically searched PubMed/Medline, Embase, and Cochrane databases for literature regarding drugs/intoxicants and treatment with RRT in pediatric populations. Two experts from the PCRRT (Pediatric Continuous Renal Replacement Therapy) workgroup assessed titles, abstracts, and full-text articles for extraction of data. The data from the literature search was shared with the PCRRT workgroup and two expert toxicologists, and expert panel recommendations were developed. RESULTS AND CONCLUSIONS: We have presented the recommendations concerning the use of RRTs for treatment of intoxications with toxic alcohols, lithium, vancomycin, theophylline, barbiturates, metformin, carbamazepine, methotrexate, phenytoin, acetaminophen, salicylates, valproic acid, and aminoglycosides.


Asunto(s)
Lesión Renal Aguda/terapia , Consenso , Intoxicación/terapia , Guías de Práctica Clínica como Asunto , Terapia de Reemplazo Renal/normas , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/etiología , Adolescente , Niño , Preescolar , Conferencias de Consenso como Asunto , Femenino , Humanos , Lactante , Masculino , Nefrología/normas , Intoxicación/diagnóstico , Intoxicación/etiología , Adulto Joven
12.
J Adolesc Health ; 65(3): 430-432, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-31227385

RESUMEN

PURPOSE: The aim of the study was to examine changes in systolic blood pressure (SBP) and whether physical activity and obesity status predicted SBP change for African-American adolescents (n = 181) participating in a behavioral weight control trial. METHODS: Data were collected at baseline, 7 months (end-of-treatment), and 9 months (2-month follow-up). RESULTS: Nearly half of adolescents achieved clinically significant SBP reductions at 7 and 9 months. Significantly, fewer adolescents had elevated SBP at 7 and 9 months compared with baseline (both p < .001). Changes in percent overweight and moderate-to-vigorous activity predicted changes in SBP over time. CONCLUSIONS: Obesity reduction and increases in moderate-to-vigorous physical activity may predict short-term, clinically meaningful reductions in SBP for African American adolescents with obesity.


Asunto(s)
Presión Sanguínea/fisiología , Ejercicio Físico/fisiología , Obesidad/terapia , Adolescente , Negro o Afroamericano/estadística & datos numéricos , Femenino , Humanos , Masculino
13.
Pediatr Crit Care Med ; 20(9): 809-816, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-31107377

RESUMEN

OBJECTIVES: To develop a novel device to predict systolic and diastolic blood pressure based on measured heart sound signals and evaluate its accuracy in comparison to intra-arterial blood pressure readings. STUDY DESIGN: Prospective, observational pilot study. SETTING: PICU. PATIENTS: Critically ill children (0-18 yr) undergoing continuous blood pressure monitoring via radial artery intra-arterial catheters were enrolled in the study after informed consent. The study included medical, cardiac, and surgical PICU patients. INTERVENTIONS: Along with intra-arterial blood pressure, patient's heart sounds were recorded simultaneously by a highly sensitive sensor taped to the chest. Additional hardware included a data acquisition unit and laptop computer. Subsequently, advanced signal processing technologies were used to minimize random interfering signals and extract and separate S1 and S2 signals. A computerized model was then developed using artificial neural network systems to estimate blood pressure from the extracted heart sound analysis. MEASUREMENTS AND MAIN OUTCOMES: We found a statistically significant correlation for systolic (r = 0.964; R = 0.928) and diastolic (r = 0.935; R = 0.868) blood pressure readings (n = 491) estimated by the novel heart-sound signal-based method and those recorded by intra-arterial catheters. The mean difference of the individually paired determinations of the blood pressure between the heart-sound-based method and intra-arterial catheters was 0.6 ± 7 mm Hg for systolic blood pressure and -0.06 ± 5 mm Hg for diastolic blood pressure, which was within the recommended range of 5 ± 8 mm Hg for any new blood pressure devices. CONCLUSIONS: Our findings provide proof of concept that the heart-sound signal-based method can provide accurate, noninvasive blood pressure monitoring.


Asunto(s)
Determinación de la Presión Sanguínea/métodos , Enfermedad Crítica , Ruidos Cardíacos/fisiología , Procesamiento de Señales Asistido por Computador , Adolescente , Presión Sanguínea/fisiología , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Redes Neurales de la Computación , Proyectos Piloto , Estudios Prospectivos
14.
Pediatr Nephrol ; 34(8): 1435-1445, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-30945006

RESUMEN

BACKGROUND: Renal damage is a progressive complication of sickle cell disease (SCD). Microalbuminuria is common in children with SCD, while a smaller number of children have more severe renal manifestations necessitating kidney biopsy. There is limited information on renal biopsy findings in children with SCD and subsequent management and outcome. METHODS: This is a multicenter retrospective analysis of renal biopsy findings and clinical outcomes in children and adolescents with SCD. We included children and adolescents (age ≤ 20 years) with SCD who had a kidney biopsy performed at a pediatric nephrology unit. The clinical indication for biopsy, biopsy findings, subsequent treatments, and outcomes were analyzed. RESULTS: Thirty-six SCD patients (ages 4-19 years) were identified from 14 centers with a median follow-up of 2.6 years (0.4-10.4 years). The indications for biopsy were proteinuria (92%) and elevated creatinine (30%). All biopsies had abnormal findings, including mesangial hypercellularity (75%), focal segmental glomerulosclerosis (30%), membranoproliferative glomerulonephritis (16%), and thrombotic microangiopathy (2%). There was increased use of hydroxyurea, angiotensin-converting-enzyme inhibitors, and angiotensin receptor blockers following renal biopsy. At last follow-up, 3 patients were deceased, 2 developed insulin-dependent diabetes mellitus, 6 initiated chronic hemodialysis, 1 received a bone marrow transplant, and 1 received a kidney transplant. CONCLUSIONS: Renal biopsies, while not commonly performed in children with SCD, were universally abnormal. Outcomes were poor in this cohort of patients despite a variety of post-biopsy interventions. Effective early intervention to prevent chronic kidney disease (CKD) is needed to reduce morbidity and mortality in children with SCD.


Asunto(s)
Albuminuria/etiología , Anemia de Células Falciformes/complicaciones , Riñón/patología , Insuficiencia Renal Crónica/etiología , Adolescente , Albuminuria/sangre , Albuminuria/patología , Albuminuria/orina , Anemia de Células Falciformes/sangre , Biopsia , Niño , Preescolar , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Tasa de Filtración Glomerular , Humanos , Masculino , Medio Oeste de Estados Unidos , Insuficiencia Renal Crónica/patología , Insuficiencia Renal Crónica/prevención & control , Insuficiencia Renal Crónica/orina , Estudios Retrospectivos , Adulto Joven
15.
Pediatr Nephrol ; 34(5): 925-941, 2019 05.
Artículo en Inglés | MEDLINE | ID: mdl-30734850

RESUMEN

Intradialytic hypotension (IDH) is a common adverse event resulting in premature interruption of hemodialysis, and consequently, inadequate fluid and solute removal. IDH occurs in response to the reduction in blood volume during ultrafiltration and subsequent poor compensatory mechanisms due to abnormal cardiac function or autonomic or baroreceptor failure. Pediatric patients are inherently at risk for IDH due to the added difficulty of determining and attaining an accurate dry weight. While frequent blood pressure monitoring, dialysate sodium profiling, ultrafiltration-guided blood volume monitoring, dialysate cooling, hemodiafiltration, and intradialytic mannitol and midodrine have been used to prevent IDH, they have not been extensively studied in pediatric population. Lack of large-scale studies on IDH in children makes it difficult to develop evidence-based management guidelines. Here, we aim to review IDH preventative strategies in the pediatric population and outlay recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup. Without strong evidence in the literature, our recommendations from the expert panel reflect expert opinion and serve as a valuable guide.


Asunto(s)
Consenso , Terapia de Reemplazo Renal Continuo/normas , Hipotensión/prevención & control , Fallo Renal Crónico/terapia , Factores de Edad , Presión Sanguínea/efectos de los fármacos , Determinación de la Presión Sanguínea , Niño , Terapia de Reemplazo Renal Continuo/efectos adversos , Terapia de Reemplazo Renal Continuo/métodos , Hemodiafiltración/efectos adversos , Hemodiafiltración/métodos , Soluciones para Hemodiálisis/efectos adversos , Humanos , Hipotensión/diagnóstico , Hipotensión/etiología , Midodrina/administración & dosificación , Diálisis Renal/efectos adversos , Diálisis Renal/normas , Temperatura
16.
Indian J Med Microbiol ; 36(3): 422-425, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30429398

RESUMEN

Scrub typhus caused by Orientia tsutsugamushi presents as an acute febrile illness with a varied presentation from mild illness to fatal disease in the absence of appropriate antibiotic treatment. Performing polymerase chain reaction (PCR) on eschar sample acts a rapid diagnostic tool in the early stage of scrub typhus when blood is negative. A total of eight patients from whom both whole blood and eschar samples were collected and tested by nested PCR targeting 56 kDa trichostatin A (TSA) gene to detect O. tsutsugamushi DNA. All (100%) eschar samples and three whole blood samples tested positive. Genetic analysis of the 56 kDa TSA gene sequences showed that the majority were related to Karp reference strains, while one clustered with Kawasaki strain. When present, eschar should be favoured as a diagnostic sample over whole blood in the early phase of infection.


Asunto(s)
Genotipo , Técnicas de Diagnóstico Molecular/métodos , Orientia tsutsugamushi/clasificación , Reacción en Cadena de la Polimerasa/métodos , Tifus por Ácaros/diagnóstico , Tifus por Ácaros/microbiología , Heridas y Lesiones/microbiología , Adolescente , Adulto , Proteínas Bacterianas/genética , Sangre/microbiología , Niño , ADN Bacteriano/genética , Femenino , Humanos , Masculino , Persona de Mediana Edad , Orientia tsutsugamushi/genética , Orientia tsutsugamushi/aislamiento & purificación , Manejo de Especímenes/métodos , Adulto Joven
17.
J Pediatr Hematol Oncol ; 40(7): 560-562, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-28991131

RESUMEN

Chemotherapy-associated myelosuppression and renal dysfunction is not uncommon during childhood acute lymphoblastic leukemia (ALL) therapy. Here we report 2 cases of atypical hemolytic uremic syndrome (aHUS) presenting with pancytopenia and renal dysfunction that developed during maintenance chemotherapy characterized by hypocomplementemia. Both cases experienced recurrence after resolution of the initial aHUS episode upon resumption of chemotherapy, raising a possible contributory role for chemotherapy in the disease pathogenesis.


Asunto(s)
Síndrome Hemolítico Urémico Atípico/inducido químicamente , Quimioterapia de Mantención/efectos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicaciones , Antineoplásicos/efectos adversos , Niño , Humanos , Enfermedades Renales/inducido químicamente , Quimioterapia de Mantención/métodos , Pancitopenia/inducido químicamente , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Recurrencia
18.
Semin Dial ; 31(3): 289-299, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29105839

RESUMEN

Hemodialysis (HD) in neonates and infants poses unique challenges due to high risks of mortality attributable to obligatory small blood flow volumes. Although HD is often necessary in neonates, its effectiveness and feasibility are poorly understood. The aim of this review is to describe in detail the few studies reporting on HD in neonates and infants (<12 months old) and then dissertate more broadly on the subject with an emphasis on recent innovations with potential to overcome traditional barriers for effective HD in this population. We detail the clinical characteristics, outcomes, technical considerations, maintenance and complications associated with HD, and provide guidance for addressing challenges associated with HD in this population.


Asunto(s)
Lesión Renal Aguda/terapia , Unidades de Cuidado Intensivo Neonatal , Diálisis Renal/métodos , Insuficiencia Renal Crónica/terapia , Lesión Renal Aguda/diagnóstico , Factores de Edad , Toma de Decisiones Clínicas , Femenino , Humanos , Recién Nacido , Masculino , Pronóstico , Diálisis Renal/efectos adversos , Insuficiencia Renal Crónica/diagnóstico , Medición de Riesgo , Resultado del Tratamiento
19.
Health Qual Life Outcomes ; 15(1): 166, 2017 Aug 23.
Artículo en Inglés | MEDLINE | ID: mdl-28835233

RESUMEN

BACKGROUND: Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome. METHODS: One hundred twenty seven children with nephrotic syndrome between the ages of 8 and 17 years participated in this prospective cohort study. Patients completed a baseline assessment while their nephrotic syndrome was active, a follow-up assessment at the time of their first complete proteinuria remission or study month 3 if no remission occurred, and a final assessment at study month 12. Participants completed six PROMIS measures (Mobility, Fatigue, Pain Interference, Depressive Symptoms, Anxiety, and Peer Relationships), the PedsQL version 4.0, and two global assessment of change items. RESULTS: Disease status was classified at each assessment: nephrotic syndrome active in 100% at baseline, 33% at month 3, and 46% at month 12. The PROMIS domains of Mobility, Fatigue, Pain Interference, Depressive Symptoms, and Anxiety each showed a significant overall improvement over time (p < 0.001). When the PROMIS measures were compared to the patients' global assessment of change, the domains of Mobility, Fatigue, Pain Interference, and Anxiety consistently changed in an expected fashion. With the exception of Pain Interference, change in PROMIS domain scores did not correlate with changes in disease activity. PROMIS domain scores were moderately correlated with analogous PedsQL domain scores. CONCLUSION: This study demonstrates that the PROMIS Mobility, Fatigue, Pain Interference, and Anxiety domains are sensitive to self-reported changes in disease and overall health status over time in children with nephrotic syndrome. The lack of significant anchoring to clinically defined nephrotic syndrome disease active and remission status may highlight an opportunity to improve the measurement of HRQOL in children with nephrotic syndrome through the development of a nephrotic syndrome disease-specific HRQOL measure.


Asunto(s)
Estado de Salud , Síndrome Nefrótico/psicología , Medición de Resultados Informados por el Paciente , Calidad de Vida , Autoinforme/normas , Adolescente , Ansiedad/psicología , Niño , Depresión/psicología , Fatiga/psicología , Femenino , Humanos , Relaciones Interpersonales , Masculino , Dolor/psicología , Estudios Prospectivos
20.
Pediatr Nephrol ; 32(7): 1243-1250, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28391546

RESUMEN

BACKGROUND: There is a paucity of data on blood pressures (BP), urinary albumin, and mineral excretion in early childhood in contemporary cohorts of extremely low gestational age (GA) neonates. Our aim was to compare BPs and the urinary excretion of albumin, calcium, and phosphate in preterm and term-born cohorts in early childhood. METHODS: This was a prospective observational study conducted at a single center, involving children <5 years age, born preterm (GA <30 weeks) or at term (≥37 weeks' GA). Urinary albumin (mg/L), calcium and phosphate levels indexed to creatinine (mg/dL), and BP were measured. RESULTS: The median (IQR) follow-up age of our cohort (n = 106) was 30 (16-48) months. Preterm-born children (n = 55) had a significantly lower mean GA and birth weight and higher mean systolic, diastolic, and mean BPs, compared with term (n = 51) controls. A significantly higher proportion of preterm-born children weighed <10th centile and had systolic BP >95th centile at follow-up. Albumin and calcium excretion did not differ between the groups; median urine-phosphate creatinine ratios were higher in the preterm group. On logistic regression, lower GA and younger age at follow-up were significantly associated with an increased risk of systolic and diastolic BP above the 95th centile; male gender was associated with decreased risk of diastolic hypertension. CONCLUSIONS: Even in early childhood, children born preterm had significantly elevated BP, compared with their term-born counterparts. Closer monitoring of BPs in this population may be warranted.


Asunto(s)
Peso al Nacer/fisiología , Presión Sanguínea/fisiología , Calcio/orina , Hipertensión/epidemiología , Nacimiento Prematuro/fisiopatología , Eliminación Renal , Albúminas/análisis , Albuminuria/orina , Determinación de la Presión Sanguínea , Preescolar , Creatinina/orina , Femenino , Estudios de Seguimiento , Edad Gestacional , Humanos , Hipertensión/etiología , Lactante , Recién Nacido , Recien Nacido Prematuro/fisiología , Masculino , Fosfatos/orina , Embarazo , Estudios Prospectivos , Factores de Riesgo , Factores Sexuales
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