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1.
J Cyst Fibros ; 22(4): 745-755, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37032248

RESUMEN

BACKGROUND: This study examines whether heterogeneous (HTG) pattern on liver ultrasound (US) identifies children at risk for advanced cystic fibrosis liver disease (aCFLD). METHODS: Prospective 6-year multicenter case-controlled cohort study. Children with pancreatic insufficient cystic fibrosis (CF) aged 3-12 years without known cirrhosis underwent screening US. Participants with HTG were matched (by age, Pseudomonas infection status and center) 1:2 with participants with normal (NL) US pattern. Clinical status and laboratory data were obtained annually and US bi-annually for 6 years. Primary endpoint was development of nodular (NOD) US pattern consistent with aCFLD. RESULTS: 722 participants underwent screening US, with 65 HTG and 592 NL. Final cohort included 55 HTG and 116 NL with ≥ 1 follow-up US. ALT, AST, GGTP, FIB-4, GPR and APRI were higher, and platelets were lower in HTG compared to NL. HTG had a 9.5-fold increased incidence (95% confidence interval [CI]:3.4, 26.7, p<0.0001, 32.7% vs 3.4%) of NOD versus NL. HTG had a sensitivity of 82% and specificity of 75% for subsequent NOD. Negative predictive value of a NL US for subsequent NOD was 96%. Multivariate logistic prediction model that included baseline US, age, and log(GPR) improved the C-index to 0.90 compared to only baseline US (C-index 0.78). Based on survival analysis, 50% of HTG develop NOD after 8 years. CONCLUSIONS: Research US finding of HTG identifies children with CF with a 30-50% risk for aCFLD. A score based on US pattern, age and GPR may refine the identification of individuals at high risk for aCFLD. CLINICAL TRIAL REGISTRATION: Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF: NCT 01,144,507 (observational study, no consort checklist).


Asunto(s)
Fibrosis Quística , Hepatopatías , Humanos , Niño , Estudios Prospectivos , Estudios de Cohortes , Fibrosis Quística/complicaciones , Fibrosis Quística/epidemiología , Fibrosis Quística/patología , Recuento de Plaquetas , Hígado/diagnóstico por imagen , Hígado/patología , Cirrosis Hepática/diagnóstico por imagen , Cirrosis Hepática/epidemiología
2.
J Clin Invest ; 133(5)2023 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-36637914

RESUMEN

The molecular mechanisms of sodium-glucose cotransporter-2 (SGLT2) inhibitors (SGLT2i) remain incompletely understood. Single-cell RNA sequencing and morphometric data were collected from research kidney biopsies donated by young persons with type 2 diabetes (T2D), aged 12 to 21 years, and healthy controls (HCs). Participants with T2D were obese and had higher estimated glomerular filtration rates and mesangial and glomerular volumes than HCs. Ten T2D participants had been prescribed SGLT2i (T2Di[+]) and 6 not (T2Di[-]). Transcriptional profiles showed SGLT2 expression exclusively in the proximal tubular (PT) cluster with highest expression in T2Di(-) patients. However, transcriptional alterations with SGLT2i treatment were seen across nephron segments, particularly in the distal nephron. SGLT2i treatment was associated with suppression of transcripts in the glycolysis, gluconeogenesis, and tricarboxylic acid cycle pathways in PT, but had the opposite effect in thick ascending limb. Transcripts in the energy-sensitive mTORC1-signaling pathway returned toward HC levels in all tubular segments in T2Di(+), consistent with a diabetes mouse model treated with SGLT2i. Decreased levels of phosphorylated S6 protein in proximal and distal tubules in T2Di(+) patients confirmed changes in mTORC1 pathway activity. We propose that SGLT2i treatment benefits the kidneys by mitigating diabetes-induced metabolic perturbations via suppression of mTORC1 signaling in kidney tubules.


Asunto(s)
Diabetes Mellitus Tipo 2 , Inhibidores del Cotransportador de Sodio-Glucosa 2 , Animales , Ratones , Diabetes Mellitus Tipo 2/tratamiento farmacológico , Diabetes Mellitus Tipo 2/genética , Diabetes Mellitus Tipo 2/metabolismo , Riñón/metabolismo , Glomérulos Renales/metabolismo , Transportador 2 de Sodio-Glucosa/genética , Inhibidores del Cotransportador de Sodio-Glucosa 2/farmacología , Humanos , Niño , Adolescente , Adulto Joven , Diana Mecanicista del Complejo 1 de la Rapamicina
3.
J Cyst Fibros ; 22(2): 248-255, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-35985930

RESUMEN

BACKGROUND: Nodular liver (NOD) in cystic fibrosis (CF) suggests advanced CF liver disease (aCFLD); little is known about progression of liver disease (LD) after detection of sonographic NOD. METHODS: Clinical, laboratory, and ultrasound (US) data from Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in CFLD Study participants with NOD at screening or follow-up were compared with normal (NL). Linear mixed effects models were used for risk factors for LD progression and Kaplan-Meier estimator for time-to-event. RESULTS: 54 children with NOD (22 screening, 32 follow-up) and 112 NL were evaluated. Baseline (BL) and trajectory of forced expiratory volume, forced vital capacity, height/BMI z-scores were similar in NOD vs NL. Platelets were lower in NOD at BL (250 vs 331×103/microL; p < 0.001) and decreased by 8600/year vs 2500 in NL. Mean AST to Platelet Ratio Index (1.1 vs 0.4; p < 0.001), Fibrosis-4 Index (0.4 vs 0.2, p < 0.001), and spleen size z-score (SSZ) [1.5 vs 0.02; p < 0.001] were higher in NOD at BL; SSZ increased by 0.5 unit/year in NOD vs 0.1 unit/year in NL. Median liver stiffness (LSM) by transient elastography was higher in NOD (8.2 kPa, IQR 6-11.8) vs NL (5.3, 4.2-7, p < 0.0001). Over 6.3 years follow-up (1.3-10.3), 6 NOD had esophageal varices (cumulative incidence in 10 years: 20%; 95% CI: 0.0%, 40.0%), 2 had variceal bleeding, and 2 underwent liver transplantation; none had ascites or hepatic encephalopathy. No NL experienced liver-related events. CONCLUSIONS: NOD developed clinically evident portal hypertension faster than NL without worse growth or lung disease.


Asunto(s)
Fibrosis Quística , Diagnóstico por Imagen de Elasticidad , Várices Esofágicas y Gástricas , Hipertensión Portal , Humanos , Niño , Estudios de Seguimiento , Fibrosis Quística/complicaciones , Fibrosis Quística/epidemiología , Fibrosis Quística/patología , Várices Esofágicas y Gástricas/patología , Hemorragia Gastrointestinal/patología , Hígado/diagnóstico por imagen , Hígado/patología , Cirrosis Hepática/diagnóstico por imagen , Cirrosis Hepática/etiología
4.
Kidney Int ; 102(6): 1345-1358, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36055599

RESUMEN

Hyperfiltration is a state of high glomerular filtration rate (GFR) observed in early diabetes that damages glomeruli, resulting in an iterative process of increasing filtration load on fewer and fewer remaining functional glomeruli. To delineate underlying cellular mechanisms of damage associated with hyperfiltration, transcriptional profiles of kidney biopsies from Pima Indians with type 2 diabetes with or without early-stage diabetic kidney disease were grouped into two hyperfiltration categories based on annual iothalamate GFR measurements. Twenty-six participants with a peak GFR measurement within two years of biopsy were categorized as the hyperfiltration group, and 26 in whom biopsy preceded peak GFR by over two years were considered pre-hyperfiltration. The hyperfiltration group had higher hemoglobin A1c, higher urine albumin-to-creatinine ratio, increased glomerular basement membrane width and lower podocyte density compared to the pre-hyperfiltration group. A glomerular 1240-gene transcriptional signature identified in the hyperfiltration group was enriched for endothelial stress response signaling genes, including endothelin-1, tec-kinase and transforming growth factor-ß1 pathways, with the majority of the transcripts mapped to endothelial and inflammatory cell clusters in kidney single cell transcriptional data. Thus, our analysis reveals molecular pathomechanisms associated with hyperfiltration in early diabetic kidney disease involving putative ligand-receptor pairs with downstream intracellular targets linked to cellular crosstalk between endothelial and mesangial cells.


Asunto(s)
Diabetes Mellitus Tipo 2 , Nefropatías Diabéticas , Humanos , Nefropatías Diabéticas/genética , Nefropatías Diabéticas/complicaciones , Diabetes Mellitus Tipo 2/complicaciones , Diabetes Mellitus Tipo 2/genética , Diabetes Mellitus Tipo 2/patología , Glomérulos Renales/patología , Tasa de Filtración Glomerular , Hemoglobina Glucada/metabolismo
5.
J Pediatr Gastroenterol Nutr ; 75(5): 635-642, 2022 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-36070552

RESUMEN

OBJECTIVES: Cystic fibrosis liver disease (CFLD) begins early in life. Symptoms may be vague, mild, or nonexistent. Progressive liver injury may be associated with decrements in patient health before liver disease is clinically apparent. We examined Health-Related Quality of Life (HRQOL) in children enrolled in a multi-center study of CFLD to determine the impact of early CFLD on general and disease-specific QOL. METHODS: Ultrasound (US) patterns of normal (NL), heterogeneous (HTG), homogeneous (HMG), or nodular (NOD) were assigned in a prospective manner to predict those at risk for advanced CFLD. Parents were informed of results. We assessed parent/child-reported (age ≥5 years) HRQOL by PedsQL 4.0 Generic Core and CF Questionnaire-revised (CFQ-R) prior to US and annually. HRQOL scores were compared by US pattern at baseline (prior to US), between baseline and 1 year and at 5 years. Multivariate analysis of variance (MANOVA) with Hotelling-Lawley trace tested for differences among US groups. RESULTS: Prior to US, among 515 participants and their parents there was no evidence that HTG or NOD US was associated with reduced PedsQL/CFQ-R at baseline. Parents of NOD reported no change in PedsQL/CFQ-R over the next year. Child-report PedsQL/CFQ-R (95 NL, 20 NOD) showed improvement between baseline and year 5 for many scales, including Physical Function. Parents of HMG children reported improved CFQ-R scores related to weight. CONCLUSIONS: Early undiagnosed or pre-symptomatic liver disease had no impact on generic or disease-specific HRQoL, and HRQoL was remarkably stable in children with CF regardless of liver involvement.


Asunto(s)
Fibrosis Quística , Hepatopatías , Humanos , Preescolar , Calidad de Vida , Estudios Prospectivos , Estado de Salud , Fibrosis Quística/complicaciones , Fibrosis Quística/diagnóstico por imagen , Encuestas y Cuestionarios , Hepatopatías/etiología , Hepatopatías/complicaciones
6.
Hepatol Commun ; 5(8): 1362-1372, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34430781

RESUMEN

Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (rs =  - 0.28, P = 0.0071) and positive correlation with aspartate aminotransferase-to-platelet ratio index (rs = 0.38, P = 0.0002), Fibrosis-4 index (rs = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; rs = 0.35, P = 0.0017), GGT-to-platelet ratio (rs = 0.35, P = 0.003), and US spleen size z-score (rs = 0.27, P = 0.0073). Conclusion: VCTE is associated with US patterns and conventional markers in patients with liver disease with CF.

7.
J Pediatr ; 219: 62-69.e4, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-32061406

RESUMEN

OBJECTIVE: To assess if a heterogeneous pattern on research liver ultrasound examination can identify children at risk for advanced cystic fibrosis (CF) liver disease. STUDY DESIGN: Planned 4-year interim analysis of a 9-year multicenter, case-controlled cohort study (Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF). Children with pancreatic insufficient CF aged 3-12 years without known cirrhosis, Burkholderia species infection, or short bowel syndrome underwent a screening research ultrasound examination. Participants with a heterogeneous liver ultrasound pattern were matched (by age, Pseudomonas infection status, and center) 1:2 with participants with a normal pattern. Clinical status and laboratory data were obtained annually and research ultrasound examinations biannually. The primary end point was the development of a nodular research ultrasound pattern, a surrogate for advanced CF liver disease. RESULTS: There were 722 participants who underwent screening research ultrasound examination, of which 65 were heterogeneous liver ultrasound pattern and 592 normal liver ultrasound pattern. The final cohort included 55 participants with a heterogeneous liver ultrasound pattern and 116 participants with a normal liver ultrasound pattern. All participants with at least 1 follow-up research ultrasound were included. There were no differences in age or sex between groups at entry. Alanine aminotransferase (42 ± 22 U/L vs 32 ± 19 U/L; P = .0033), gamma glutamyl transpeptidase (36 ± 34 U/L vs 15 ± 8 U/L; P < .001), and aspartate aminotransferase to platelet ratio index (0.7 ± 0.5 vs 0.4 ± 0.2; P < .0001) were higher in participants with a heterogeneous liver ultrasound pattern compared with participants with a normal liver ultrasound pattern. Participants with a heterogeneous liver ultrasound pattern had a 9.1-fold increased incidence (95% CI, 2.7-30.8; P = .0004) of nodular pattern vs a normal liver ultrasound pattern (23% in heterogeneous liver ultrasound pattern vs 2.6% in normal liver ultrasound pattern). CONCLUSIONS: Research liver ultrasound examinations can identify children with CF at increased risk for developing advanced CF liver disease.


Asunto(s)
Fibrosis Quística/complicaciones , Hepatopatías/etiología , Hígado/patología , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Hígado/diagnóstico por imagen , Hepatopatías/diagnóstico , Masculino , Estudios Prospectivos , Medición de Riesgo , Ultrasonografía
8.
J Surg Res ; 233: 256-261, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30502256

RESUMEN

BACKGROUND: Lymphatic malformations (LMs) are congenital and arise from errors in vascular embryogenesis. LMs are categorized by cyst size as microcystic, macrocystic, or combined. Abdominal LMs are rare. Surgical resection of abdominal LMs has been the mainstay of therapy, but recurrence and morbidity are high. We sought to determine the effectiveness of sclerotherapy treatment for abdominal LM. METHODS: A single-center, retrospective review from 2014 to 2018 was conducted evaluating pediatric patients with abdominal LM. RESULTS: Ten patients were included, n = 9 had macrocystic LM and one patient had combined disease. The average age at first treatment was 6.8 y. The most common presenting symptoms were abdominal distention, pain, infection, and anemia. Preprocedural imaging was performed for all patients; median pretreatment volume was 1572.9 cm3 (range, 67.2-13,226.4). LMs were accessed using ultrasound guidance and injected with opacified doxycycline. Patients received a mean of 7.1 sclerotherapy injections. Complications included intraperitoneal doxycycline extravasation (n = 1), managed conservatively, and LM infection (n = 1), treated with intravenous antibiotics and drainage. One patient went on to surgical resection due to inability gain stable intracystic access; follow-up ultrasonography showed no recurrence. Postprocedural imaging was available in n = 8. Volume decreased by 96.7% after sclerotherapy. The median remaining volume was 0 cm3 (range, 0-599.7) (P = 0.016). Postsclerotherapy magnetic resonance imaging was obtained in n = 6, with complete resolution in 83.3%. All patients had resolution of presenting symptoms. Follow-up duration was 12.3 mo. CONCLUSIONS: Initial results demonstrate that sclerotherapy is an effective and durable treatment for symptom resolution and volume reduction of abdominal LM.


Asunto(s)
Doxiciclina/administración & dosificación , Anomalías Linfáticas/terapia , Escleroterapia/métodos , Prevención Secundaria/métodos , Cavidad Abdominal/diagnóstico por imagen , Adolescente , Niño , Preescolar , Extravasación de Materiales Terapéuticos y Diagnósticos/epidemiología , Extravasación de Materiales Terapéuticos y Diagnósticos/etnología , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Anomalías Linfáticas/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Recurrencia , Estudios Retrospectivos , Escleroterapia/efectos adversos , Resultado del Tratamiento , Ultrasonografía Intervencional
9.
AJR Am J Roentgenol ; 211(4): 740-743, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-30063371

RESUMEN

OBJECTIVE: Pediatric interventional radiology (PIR) is a dynamic and growing subspecialty. We will detail our need to evolve like the emerging therapies and innovative imaging options that we provide to children. CONCLUSION: New interventional radiology training pathways, maintaining competency with small volumes of complex procedures, limited availability of pediatric-specific equipment, questions about the safety of sedation in the developing brain, and the dearth of PIR opportunities outside of North America provide challenges and opportunities for the vibrant community of PIR practitioners.


Asunto(s)
Pediatría/tendencias , Radiología Intervencionista/tendencias , Competencia Clínica , Congresos como Asunto , Humanos , Objetivos Organizacionales , Pediatría/educación , Radiología Intervencionista/educación , Radiología Intervencionista/instrumentación , Sociedades Médicas
10.
Pediatr Radiol ; 47(6): 651-656, 2017 May.
Artículo en Inglés | MEDLINE | ID: mdl-28265695

RESUMEN

BACKGROUND: Pediatric interventional radiology is a distinct subspecialty differing from both pediatric diagnostic radiology and adult interventional radiology. We conducted a workforce survey in 2005 to evaluate the state of pediatric interventional radiology at that time. Since then there have been many advancements to the subspecialty, including the founding of the Society for Pediatric Interventional Radiology (SPIR). OBJECTIVE: To evaluate the current state of the pediatric interventional radiology workforce and compare findings with those of the initial 2005 workforce survey. MATERIALS AND METHODS: We sent a two-part survey electronically to members of SPIR, the Society for Pediatric Radiology (SPR), the Society of Chairmen of Radiology in Children's Hospitals (SCORCH) and the Society of Interventional Radiology (SIR). Part 1 focused on individual practitioners (n=177), while part 2 focused on group practices and was answered by a leader from each group (n=88). We examined descriptive statistics and, when possible, compared the results to the study from 2005. RESULTS: A total of 177 individuals replied (a 331% increase over the first study) and 88 pediatric interventional radiology (IR) service sites responded (a 131.6% increase). Pediatric IR has become a more clinically oriented specialty, with a statistically significant increase in services with admitting privileges, clinics and performance of daily rounds. Pediatric IR remains diverse in training and practice. Many challenges still exist, including anesthesia/hospital support, and the unknown impact of the new IR residency on pediatric IR training, although the workforce shortage has been somewhat alleviated, as demonstrated by the decreased mean call from 165 days/year to 67.2 days/year. CONCLUSION: Pediatric interventional radiology practitioners and services have grown significantly since 2005, although the profile of this small subspecialty has changed and some challenges remain.


Asunto(s)
Pediatría , Radiología Intervencionista , Estudios de Seguimiento , Humanos , Internacionalidad , Encuestas y Cuestionarios , Recursos Humanos
11.
J Pediatr ; 167(4): 862-868.e2, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26254836

RESUMEN

OBJECTIVE: To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF). STUDY DESIGN: Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multicenter study of ultrasound to predict hepatic fibrosis. Consensus ultrasound patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and US or Toronto CF registries. χ(2) or ANOVA were used to compare variables among ultrasound groups and between normal and abnormal. Logistic regression was used to study risk factors for having abnormal ultrasound. RESULTS: Findings in 719 subjects were normal (n = 590, 82.1%), heterogeneous (64, 8.9%), homogeneous (41, 5.7%), and cirrhosis (24, 3.3%). Cirrhosis (P = .0004), homogeneous (P < .0001), and heterogeneous (P = .03) were older than normal. More males were heterogeneous (P = .001). More heterogeneous (15.0%, P = .009) and cirrhosis (25.0%, P = .005) had CF-related diabetes or impaired glucose tolerance vs normal (5.4%). Early infection with Pseudomonas aeruginosa (<2 years old) was associated with a lower risk (OR 0.42, P = .0007) of abnormal. Ursodeoxycholic acid use (OR 3.69, P < .0001) and CF-related diabetes (OR 2.21, P = .019) were associated with increased risk of abnormal. CONCLUSIONS: Unsuspected cirrhosis is seen in 3.3% of young patients with CF, heterogeneous in 8.9%. Abnormal ultrasound is associated with CF-related diabetes, and early P aeruginosa is associated with normal ultrasound. Prospective assessment of these risk factors may identify potential interventional targets. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01144507.


Asunto(s)
Fibrosis Quística/diagnóstico por imagen , Cirrosis Hepática/diagnóstico por imagen , Análisis de Varianza , Índice de Masa Corporal , Niño , Preescolar , Fibrosis Quística/complicaciones , Femenino , Humanos , Cirrosis Hepática/complicaciones , Masculino , Estado Nutricional , Estudios Prospectivos , Infecciones por Pseudomonas/complicaciones , Pseudomonas aeruginosa , Factores de Riesgo , Ultrasonografía , Ácido Ursodesoxicólico/química
12.
Pediatr Radiol ; 45(7): 1026-30, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25712453

RESUMEN

BACKGROUND: Lumbar spondylolysis, a unilateral or bilateral fracture at pars interarticularis, is a common cause of low back pain in children. The initial imaging study in the diagnosis of lumbar spondylolysis has historically been lumbar spine radiographs; however, radiographs can be equivocal or false-negative. Definitive diagnosis can be achieved with computed tomography (CT), but its use has been limited due to the dose of ionizing radiation to the patient. OBJECTIVE: By limiting the z-axis coverage to the relevant anatomy and optimizing the CT protocol, we are able to provide a definitive diagnosis of fractures of the pars interarticularis at comparable or lower radiation dose than commonly performed lumbar spine radiographs. As there is no gold standard for the diagnosis of spondylolysis besides surgery, we compared interobserver agreement and degree of confidence to determine which modality is preferable. MATERIALS AND METHODS: Sixty-two patients with low back pain ages 5-18 years were assessed for the presence of spondylolyis. Forty-seven patients were evaluated by radiography and 15 patients were evaluated by limited field-of-view CT. Both radiographic and CT examinations were assessed anonymously in random order for the presence or absence of spondylolyisis by six raters. Agreement was assessed among raters using a Fleiss Kappa statistic for multiple raters. RESULTS: CT provided a significantly higher level of agreement among raters than radiographs (P < 0.001). The overall Kappa for rater agreement with radiographs was 0.24, 0.34 and 0.40 for 2, 3 or 4 views, respectively, and 0.88 with CT. CONCLUSION: Interobserver agreement is significantly greater using limited z-axis coverage CT when compared with radiographs. Radiologist confidence improved significantly with CT compared to radiographs regardless of the number of views.


Asunto(s)
Dosis de Radiación , Espondilólisis/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adolescente , Niño , Femenino , Humanos , Vértebras Lumbares/diagnóstico por imagen , Región Lumbosacra/diagnóstico por imagen , Masculino , Reproducibilidad de los Resultados
13.
Pediatr Radiol ; 42(11): 1316-21, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-22854847

RESUMEN

BACKGROUND: Physician credentialing is a complex process driven by the demand for quality improvement in health care. In the U.S., the Joint Commission Standard of 2007 has tied hospital accreditation to credentialing through mandated use of the Focused Professional Practice Evaluation (FPPE) and Ongoing Professional Practice Evaluation (OPPE). OBJECTIVE: To assess pediatric interventional radiologists' knowledge of how institutions grant them privileges. MATERIALS AND METHODS: Members of the Society for Pediatric Interventional Radiology (SPIR) were sent a web-based survey regarding credentialing. RESULTS: Of 122 members from 19 countries, 81 (66%) responded, and of these 81, 59 (73%) were familiar with their hospital's privileging process. Of 49 U.S. respondents and 32 non-U.S. respondents, 37 (76%) and 17 (53%), respectively, stated that interventional radiology credentialing was different from diagnostic radiology credentialing. Of the 49 U.S. respondents, 24 (49%) reported an OPPE, and of the 32 non-U.S. respondents, 8 (25%) reported an ongoing evaluation. The U.S. OPPE is performed at shorter intervals than its international equivalent. CONCLUSION: Four years after the Joint Commission defined the FPPE and OPPE, separate credentialing of pediatric interventional radiology from pediatric diagnostic radiology is more likely in the U.S. than internationally, and U.S. pediatric interventional radiologists are more likely to have a defined ongoing professional evaluation and to be evaluated every 6 months or more frequently. There are many SPIR members who do not know how they obtain privileges and/or are not knowingly subject to an OPPE. This lack of knowledge may affect future education of interventional radiologists as well as the definition of pediatric interventional radiology practices within individual institutions.


Asunto(s)
Habilitación Profesional/estadística & datos numéricos , Conocimientos, Actitudes y Práctica en Salud , Pediatría/normas , Médicos/estadística & datos numéricos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Pautas de la Práctica en Medicina/normas , Radiografía Intervencional/normas , Recolección de Datos , Internacionalidad , Pediatría/estadística & datos numéricos , Radiografía Intervencional/estadística & datos numéricos
14.
Pediatr Blood Cancer ; 50(2): 354-6, 2008 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-17091481

RESUMEN

A 6-year-old female with homozygous sickle cell disease had a central venous access device (CVAD) placed to facilitate chronic erythrocytapheresis. Erythrocytapheresis was ineffective due to the rare pinch-off syndrome causing communication between the dual lumen tubing. Awareness of and monitoring for the pinch-off syndrome is indicated in people with sickle cell disease and a CVAD for chronic erythrocytapheresis.


Asunto(s)
Anemia de Células Falciformes/terapia , Cateterismo Venoso Central , Catéteres de Permanencia , Anemia de Células Falciformes/sangre , Eliminación de Componentes Sanguíneos , Niño , Transfusión de Eritrocitos , Femenino , Humanos , Vena Subclavia
15.
J Neurol Sci ; 260(1-2): 279-82, 2007 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-17568613

RESUMEN

Fenestration of vertebral arteries has been reported in association with thromboembolic brain infarctions. However, few cases have been reported in which recurrent infarction occurred in spite of adequate anticoagulation. We report a young man with fenestrated vertebral arteries and stroke who failed to respond to standard anticoagulation therapy but did well with angiographic coil obliteration of an abnormal vertebral segment. An 18-year-old left-handed man presented with acute onset of dizziness and headache. No trauma or other stroke risk factors were identified. Left cerebellar infarction was seen on CT, but the cause could not be identified by brain and neck MRI, MRA, or CTA. Bilateral fenestrated vertebral arteries were identified with conventional angiography. Although the patient recovered fully and was treated with anticoagulation, he suffered a recurrent stroke 1 month later involving the right cerebellum while he was on a therapeutic dose of warfarin. Repeat arteriography showed a spontaneous dissection within one of the fenestrated vertebral segments. Since receiving angiographic coil obliteration of the pathologic segment, he has been free of all symptoms. We conclude that the patient sustained recurrent thromboembolic events in his posterior circulation due to spontaneous dissection within a fenestrated vertebral artery segment. Conventional angiography and emergent interventional embolization were essential to his diagnostic evaluation and therapeutic intervention.


Asunto(s)
Embolización Terapéutica/métodos , Prótesis e Implantes , Accidente Cerebrovascular/fisiopatología , Disección de la Arteria Vertebral/fisiopatología , Disección de la Arteria Vertebral/terapia , Arteria Vertebral/fisiopatología , Adolescente , Anticoagulantes/uso terapéutico , Angiografía Cerebral , Embolización Terapéutica/instrumentación , Humanos , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Recurrencia , Accidente Cerebrovascular/etiología , Resultado del Tratamiento , Arteria Vertebral/anomalías , Arteria Vertebral/patología , Disección de la Arteria Vertebral/patología
18.
Pediatr Radiol ; 35(12): 1277-88, 2005 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-16151789

RESUMEN

In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis.


Asunto(s)
Abdomen/diagnóstico por imagen , Quistes/diagnóstico por imagen , Radiografía Abdominal , Adolescente , Niño , Preescolar , Diagnóstico Diferencial , Sistema Digestivo/diagnóstico por imagen , Enfermedades Urogenitales Femeninas/diagnóstico por imagen , Hamartoma/diagnóstico por imagen , Humanos , Enfermedades Renales/diagnóstico por imagen , Enfermedades Urogenitales Masculinas , Bazo/diagnóstico por imagen , Ultrasonografía
20.
J Pediatr Surg ; 37(7): 970-3; discussion 970-3, 2002 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-12077751

RESUMEN

BACKGROUND/PURPOSE: Although thoracoscopy in children is a well-accepted technique, there are limitations to this approach. Small lung nodules or those not located on the pleural surface often require an open thoracotomy. The purpose of this report is to evaluate the utility of computed tomography (CT) guided needle localization of lung nodules in children followed by thoracoscopic resection. METHODS: From 1997 to 2000, 13 thoracoscopic procedures were performed on 12 children who presented with small pulmonary nodules (<1 cm) or nodules deep to the pleural surface. Preoperative needle localization was performed by injecting methylene blue under CT guidance. Patients then were taken directly to the operating room for thoracoscopic resection. RESULTS: All 13 procedures were completed successfully thoracoscopically. Twelve yielded diagnostic pathologic findings as well as therapeutic resections in 7. All patients underwent extubation in the operating room, and chest tubes were removed within 36 hours. No thoracotomies were performed, and there were no complications. CONCLUSIONS: Preoperative localization utilizing CT guidance is safe and beneficial in children with pulmonary nodules less than 1 cm in size and those located deep to the pleural surface. This technique allows the surgeon to perform thoracoscopy for diagnosis and therapeutic resection of these lesions rather than submitting patients to the morbidity of a thoracotomy.


Asunto(s)
Neoplasias Pulmonares/patología , Nódulo Pulmonar Solitario/patología , Toracoscopía/métodos , Adolescente , Adulto , Biopsia con Aguja/métodos , Niño , Preescolar , Humanos , Pulmón/patología , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/cirugía , Nódulo Pulmonar Solitario/diagnóstico por imagen , Nódulo Pulmonar Solitario/cirugía , Tomografía Computarizada por Rayos X/métodos
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