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1.
Pediatr Nephrol ; 37(11): 2667-2677, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35211790

RESUMEN

BACKGROUND: X-linked hypophosphatemic rickets (XLH) is the most common cause of inherited rickets. Historically, XLH was treated with oral phosphate and calcitriol (conventional treatment). Burosumab, a fibroblast growth factor 23 (FGF-23) monoclonal antibody, was approved by the United States Food and Drug Administration (FDA) in 2018 for XLH treatment. Nevertheless, conventional treatment of XLH continues to be recommended by some specialists due to lack of published experience with burosumab in the clinical setting. We compared laboratory and radiographic changes observed following transition from conventional therapy to burosumab in pediatric XLH patients as part of routine care. METHODS: This retrospective single-center study identified and retroactively studied twelve patients aged 1-18 years old with XLH previously treated with conventional therapy and transitioned to burosumab. Laboratory studies and radiographs were obtained routinely as standard of care during two treatment periods: (1) conventional therapy and (2) burosumab treatment. Laboratory values and radiologic rickets severity scores were compared between periods. RESULTS: All laboratory values demonstrated improvement following 1 month of burosumab treatment, findings which were sustained over the 2-year study period. Rickets severity scores and height z-scores also improved with burosumab. There were no serious adverse events with burosumab, and adverse events overall were very infrequent and mild. One patient developed an asymptomatic mild elevation of serum phosphate while taking burosumab resulting in a temporary pause in therapy. CONCLUSIONS: Safety and effectiveness of burosumab in treatment of XLH were demonstrated as burosumab yielded statistically significant improvement in laboratory and radiographic markers of rickets and height compared to conventional therapy. A higher resolution version of the Graphical abstract is available as Supplementary information.


Asunto(s)
Raquitismo Hipofosfatémico Familiar , Adolescente , Anticuerpos Monoclonales , Anticuerpos Monoclonales Humanizados , Calcitriol/uso terapéutico , Niño , Preescolar , Raquitismo Hipofosfatémico Familiar/tratamiento farmacológico , Factores de Crecimiento de Fibroblastos , Humanos , Lactante , Fosfatos , Estudios Retrospectivos
2.
Am J Med Genet A ; 182(7): 1664-1672, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32369272

RESUMEN

Vertebral malformations (VMs) are caused by alterations in somitogenesis and may occur in association with other congenital anomalies. The genetic etiology of most VMs remains unknown and their identification may facilitate the development of novel therapeutic and prevention strategies. Exome sequencing was performed on both the discovery cohort of nine unrelated probands from the USA with VMs and the replication cohort from China (Deciphering Disorders Involving Scoliosis & COmorbidities study). The discovery cohort was analyzed using the PhenoDB analysis tool. Heterozygous and homozygous, rare and functional variants were selected and evaluated for their ClinVar, HGMD, OMIM, GWAS, mouse model phenotypes, and other annotations to identify the best candidates. Genes with candidate variants in three or more probands were selected. The replication cohort was analyzed by another in-house developed pipeline. We identified rare heterozygous variants in KIAA1217 in four out of nine probands in the discovery cohort and in five out of 35 probands in the replication cohort. Collectively, we identified 11 KIAA1217 rare variants in 10 probands, three of which have not been described in gnomAD and one of which is a nonsense variant. We propose that genetic variations of KIAA1217 may contribute to the etiology of VMs.


Asunto(s)
Proteínas/genética , Enfermedades de la Columna Vertebral/genética , Adolescente , Vértebras Cervicales/anomalías , Niño , Codón sin Sentido , Bases de Datos Genéticas , Femenino , Heterocigoto , Homocigoto , Humanos , Masculino , Enfermedades de la Columna Vertebral/etiología , Vértebras Torácicas/anomalías
3.
Curr Sports Med Rep ; 17(12): 425-432, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30531459

RESUMEN

Children with chronic medical conditions face many challenges when considering sport participation. Compared with their healthy counterparts, they are often discouraged from physical activity or sports participation because of real or perceived limitations imposed by their condition. Prescribed exercise should be based on the demands of the sport, the effect of the disease on performance, and the potential for exercise-induced acute or chronic worsening of the illness or disability. This article will focus on several examples of chronic medical conditions and the clinician's role in providing advice about sport participation.


Asunto(s)
Enfermedad Crónica/terapia , Personas con Discapacidad , Ejercicio Físico , Promoción de la Salud/métodos , Artritis Juvenil/fisiopatología , Artritis Juvenil/terapia , Trastorno por Déficit de Atención con Hiperactividad/fisiopatología , Trastorno por Déficit de Atención con Hiperactividad/terapia , Trastorno del Espectro Autista/fisiopatología , Trastorno del Espectro Autista/terapia , Parálisis Cerebral/fisiopatología , Parálisis Cerebral/terapia , Niño , Fibrosis Quística/fisiopatología , Fibrosis Quística/terapia , Epilepsia/fisiopatología , Epilepsia/terapia , Hemofilia A/fisiopatología , Hemofilia A/terapia , Humanos , Enfermedades Neuromusculares/fisiopatología , Enfermedades Neuromusculares/terapia , Rasgo Drepanocítico/fisiopatología , Rasgo Drepanocítico/terapia , Medicina Deportiva
4.
Clin Orthop Relat Res ; 474(7): 1543-52, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-26847455

RESUMEN

BACKGROUND: Placement and removal of fiberglass casts are among the more-common interventions performed in pediatric orthopaedic surgery offices. However, cast removal is associated with abrasive injuries and burns from the oscillating cast saw, and these injuries can occur even when the cast is removed by experienced personnel. It is unknown whether an added barrier, such as a safety strip, can mitigate injuries from blade-to-skin contact during cast removal with the oscillating saw. QUESTIONS/PURPOSES: We asked: (1) Can a safety strip provide a physical barrier during cast removal, decreasing blade-to-skin contact? (2) Does the safety strip lessen heat transfer? (3) Will the use of the safety strip prevent cast pressure from being released when the cast is split? METHODS: Standard long-arm fiberglass casts were removed by experienced and inexperienced healthcare personnel (n = 35) from life-sized pediatric models. A commercially available woven cast saw safety strip, commonly incorporated in waterproof cast constructs, was chosen as the protective strip. Each participant removed a cast with and without the safety strip present. All participants were blinded to the presence or absence of the safety strip at the time of cast removal. The number of touches was compared between cast removal with and without protective strips. A separate model was designed to assess prevention of heat transfer. Temperatures were recorded, using thermocouples, for three designated temperatures. Five to six trials were conducted at each designated temperature for each of two conditions, with and without the safety strip. Finally, to assess if the safety strip would prevent cast pressure from being released, a third model was used. Thirty standard short-arm casts were applied and removed from the arm models by one of the authors. Pressure data were collected from between the padding layers, in casts with and without the safety strip present, after application, univalving and bivalving each cast. RESULTS: Use of the safety strip reduced the number of simulated skin touches compared with casts removed without the safety strip, among experienced users (mean, 9.0 [range, 1-28] versus 0.1 [range, 0-1], mean ratio, 0.0012; 95% CI, 0.002-0.063; p < 0.001) and inexperienced users (mean, 8.5 [range, 0-31] versus 0.6 [range, 0-3], mean ratio, 0.07; 95% CI, 0.03-0.15; p < 0.001). The safety strips decreased heat transfer, preventing temperatures at the cast-skin interface from reaching 50 °C. Finally, after splitting the cast, with the numbers available, there was no increase in the pressure beneath the casts in those with the safety strip present (mean without, 0.23 [SD, 0.070] versus safety strip in the padding 0.20 [SD, 0.091] and safety strip on top padding, 0.21 [SD, 0.090]; p = 0.446 and p = 0.65 respectively). CONCLUSIONS: Our study showed the effectiveness of a safety strip in reducing simulated touches with the oscillating cast saw during cast splitting. Additional studies are warranted to investigate the clinical use and utility of the safety strip in practice. CLINICAL RELEVANCE: The findings of this study suggest that using safety strips in clinical practice could decrease blade-to-skin contact and therefore minimize cast saw injuries. However, validation of these findings in the clinical setting is necessary before drawing a definitive conclusion.


Asunto(s)
Quemaduras/prevención & control , Moldes Quirúrgicos , Remoción de Dispositivos/efectos adversos , Remoción de Dispositivos/instrumentación , Equipos de Seguridad , Instrumentos Quirúrgicos/efectos adversos , Extremidad Superior/lesiones , Heridas Penetrantes/prevención & control , Quemaduras/etiología , Niño , Transferencia de Energía , Femenino , Humanos , Maniquíes , Ensayo de Materiales , Presión , Factores Protectores , Factores de Riesgo , Heridas Penetrantes/etiología
5.
Pediatr Exerc Sci ; 27(2): 203-7, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25875854

RESUMEN

OBJECTIVE: Although fitness and obesity have been shown to be independent predictors of cardiometabolic disease risk in obese children, this interaction is not well defined in nonobese children. The purpose of this study was to define the relationships between peak aerobic capacity, body composition, and fasting insulin levels in nonobese middle school children. STUDY DESIGN: 148 middle school children (mean age 11.0 ± 2.1 years, 49% male) underwent determination of body mass index (BMI) z-score, fasting glucose, fasting insulin, body composition by DXA scan (lean body mass and body fat percentage), and peak oxygen uptake per kg of lean body mass (VO2peak). Univariate correlations and multivariate regression analysis were used to identify independent predictors of fasting insulin using age, sex, percent body fat, body mass index z-score, and VO2peak. RESULTS: fasting insulin was significantly related to VO2peak (r =-0.37, p < .001), percent body fat (r = .27, p < .001), and BMI z-score (r = .33, p = .002). After inclusion in the multivariate model, VO2peak (p = .018) and body mass index z-score (p = .043) remained significant predictors of fasting insulin, while age (p = .39), sex (p = .49), and percent body fat (p = .72) did not. CONCLUSIONS: Among nonobese middle school children, fasting insulin is independently related to aerobic fitness after accounting for age, sex, and body composition. Public health efforts to reduce cardiometabolic disease risk among all adolescents should include exercise programs to increase cardiovascular fitness.


Asunto(s)
Índice de Masa Corporal , Ayuno/sangre , Peso Corporal Ideal/fisiología , Insulina/sangre , Aptitud Física/fisiología , Adiposidad , Adolescente , Niño , Femenino , Humanos , Masculino , Sobrepeso/sangre , Sobrepeso/fisiopatología , Consumo de Oxígeno , Delgadez/sangre , Delgadez/fisiopatología
6.
J Pediatr Orthop ; 35(6): 571-5, 2015 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-26251959

RESUMEN

INTRODUCTION: Children with cerebral palsy undergoing soft tissue and bony procedures often experience pain and spasticity postoperatively. Differentiation of pain from spasticity complicates management, so controlling spasticity with a continuous infusion of baclofen, an antispasmodic, through an already present indwelling epidural catheter holds interest. METHODS: A retrospective chart review was performed of patients with cerebral palsy undergoing single event, multilevel lower extremity surgery at a single institution who received epidural analgesia with or without continuous baclofen infusion. Primary outcomes included need for supplemental narcotic analgesics and benzodiazepines postoperatively. Duration of hospitalization, pain scores, and complications were also evaluated. RESULTS: Forty-four patients were identified, ranging in age from 3 to 17 years, 19 of whom received epidural baclofen. No differences were found in use of supplemental narcotic analgesia, benzodiazepines, or duration of hospitalization. Differences in pain scores were not statistically significant (0.82±0.95 for baclofen vs. 1.48±0.99 for controls) (P=0.391). Mean arterial pressure was lower in patients receiving baclofen (P=0.004). No potential side effects attributable to baclofen were noted. CONCLUSIONS: Continuous epidural baclofen infusion seems unlikely to alter the pain-spasm cycle experienced by patients with cerebral palsy following orthopaedic surgery to a clinically significant degree. More effective, and cost-effective, measures at assessing and controlling pain and muscle spasm should be explored to benefit cerebral palsy patients postoperatively. LEVEL OF EVIDENCE: Level III-therapeutic study.


Asunto(s)
Analgesia Epidural , Baclofeno/administración & dosificación , Parálisis Cerebral/cirugía , Mialgia , Procedimientos Ortopédicos/efectos adversos , Dolor Postoperatorio/tratamiento farmacológico , Espasmo , Adolescente , Analgesia Epidural/efectos adversos , Analgesia Epidural/métodos , Niño , Preescolar , Femenino , Humanos , Masculino , Relajantes Musculares Centrales/administración & dosificación , Mialgia/diagnóstico , Mialgia/tratamiento farmacológico , Mialgia/etiología , Procedimientos Ortopédicos/métodos , Manejo del Dolor , Dimensión del Dolor , Estudios Retrospectivos , Espasmo/diagnóstico , Espasmo/tratamiento farmacológico , Espasmo/etiología , Resultado del Tratamiento
7.
J Pediatr Orthop ; 35(7): 734-8, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25393569

RESUMEN

BACKGROUND: The aim of this study was to determine if radiographic measurements, taken before tenotomy, can predict outcome in children with idiopathic clubfoot treated by the Ponseti method. METHODS: A retrospective chart and radiographic review was performed on children with idiopathic clubfoot treated with the Ponseti method over a 10-year period with minimum 2-year follow-up that had a forced dorsiflexion lateral foot radiograph before tenotomy. All angles were measured in duplicate on the pretenotomy radiographs, including: foot dorsiflexion (defined as the 90 minus the angle between the tibial shaft and a plastic plate used to dorsiflex the foot), tibio-calcaneal, talo-calcaneal, and talo-first metatarsal angles. Clinical review of patient records identified different patient outcomes: no additional treatment required, relapse (additional casting and/or surgery required), recurrence (any additional surgery required), or reconstruction (surgery not including repeat tenotomy). RESULTS: Forty-five patients (71 feet) were included in the study. The median age at follow-up was 4.6 years. The intrareader reliability was acceptable for all measures. Thirteen of the 71 (18%) feet required additional surgery, occurring at a median age of 3.6 years. Of the 4 radiographic measures, only pretenotomy foot dorsiflexion predicted recurrence (hazard ratio=0.96, P=0.03). Youden's method identified 16.6 degrees of dorsiflexion as the optimal cutoff. Feet with at least that amount of dorsiflexion pretenotomy (n=21) experienced no recurrences; feet with less than that amount of dorsiflexion (n=50) experienced 13 recurrences (P=0.007). CONCLUSIONS: Reduced foot dorsiflexion on lateral forced dorsiflexion pretenotomy radiograph was associated with an increased risk of recurrence. Radiographic dorsiflexion to 15 degrees past neutral before tenotomy appears to predict successful treatment via the Ponseti method.


Asunto(s)
Calcáneo/cirugía , Moldes Quirúrgicos , Pie Equinovaro/cirugía , Tenotomía/métodos , Preescolar , Pie Equinovaro/diagnóstico por imagen , Femenino , Estudios de Seguimiento , Humanos , Masculino , Radiografía , Recurrencia , Reproducibilidad de los Resultados , Estudios Retrospectivos , Resultado del Tratamiento
8.
Phys Sportsmed ; 42(1): 36-44, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24565820

RESUMEN

BACKGROUND: Cast-saw injuries are sustained during cast removal or splitting of a cast when a hot cast-saw blade touches the patient's skin inadvertently during cast removal. Other studies have evaluated risk factors associated with saw-blade temperature, however, none have documented the number and duration of blade-to-skin contacts during cast removal. METHODS: Using a pediatric long-arm model capable of detecting cast-saw blade contact, we tested the ability of health care providers to apply and remove casts before and after a brief education module. The total number and duration of "touches" between the saw and the model's "skin" were recorded. Correlations between user "touches," and experience and comparisons between pre- and post-education "touches" were performed. RESULTS: Of the 18 study participants, 16 touched the model surface with the cast saw; 7 of the 18 participants maintained blade contact with the skin for > 1 second 22 times during the testing process. Participants with less experience averaged 20 (± 16) touches, whereas more experienced participants averaged 24 (± 19) touches (P = 0.7). Average number of touches was similar-before 22 (± 20) and after 25 (± 22); P = 0.5-participants completed an education module. No correlation between experience or participation in the education program was found with decreased number of blade-to-skin touches. CONCLUSION: Nearly all clinicians inadvertently contacted the underlying skin with the cast-saw blade. In our limited sample size, experience and education did not prevent this; therefore, minimizing time of contact and blade temperature may be more important factors in minimizing cast-saw injuries.


Asunto(s)
Moldes Quirúrgicos , Personal de Salud/educación , Enfermedad Iatrogénica/prevención & control , Competencia Profesional , Piel/lesiones , Adulto , Brazo/anatomía & histología , Niño , Femenino , Humanos , Masculino , Modelos Anatómicos , Seguridad del Paciente , Reproducibilidad de los Resultados
9.
J Pediatr Orthop ; 33(2): 212-5, 2013 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-23389578

RESUMEN

INTRODUCTION: It is common for pediatric patients with a history of hip dysplasia to undergo anteroposterior (AP) and frog-leg (FL) lateral radiographs to monitor development of the hip. Because of sensitivity of pediatric tissues to ionizing radiation, we sought to document the relationship between AP and FL radiographs and to determine which image was better to assess acetabular dysplasia. METHODS: An Institutional Review Board-approved, retrospective review was conducted on 33 patients screened for hip dysplasia at a single institution. We sought to determine whether either film was more likely to be qualitatively better in position and whether there were differences in acetabular measures between the 2 radiographs. Standard measurements on AP and FL radiographs were made by 5 observers on 2 different occasions to assess interobserver and intraobserver variability. RESULTS: The mean age was 23+1 months, and 80% were female; none of the children had hip dysplasia. There was no statistical significance in the quotient of pelvic rotation (AP 1.2 + 0.1 degrees and FL 1.1 + 0.1 degrees; P = 0.84). There was a statistical difference in obturator height in mm (AP 16.0 + 0.3 and FL 17.8 + 0.2; P = 7.2E-10). The mean intraobserver variability for the acetabular index was 1.8 + 0.2 and 1.7 + 0.2 degrees for AP and FL alignments, respectively (P = 0.58), and the mean interobserver variability was 1.7 + 0.1 and 1.7 + 0.1 degrees, respectively (P = 0.75). The measurements of linear variability between the AP and FL showed no statistical significant difference with the exception of the left Hilgenreiner distance in mm (AP 1.00 + 0.08 and FL 0.81 + 0.08; P = 0.02). DISCUSSION: These results demonstrate that there is little clinical difference in variability of positioning and rotational variables between AP and FL radiographs for hip dysplasia. These results indicate that either image could be used assess to acetabular dysplasia thus decreasing risks of ionizing radiation. LEVEL OF EVIDENCE: Level III.


Asunto(s)
Acetábulo/diagnóstico por imagen , Luxación de la Cadera/diagnóstico por imagen , Articulación de la Cadera/diagnóstico por imagen , Acetábulo/anomalías , Preescolar , Femenino , Articulación de la Cadera/patología , Humanos , Lactante , Masculino , Variaciones Dependientes del Observador , Pelvis , Radiografía , Estudios Retrospectivos , Rotación
10.
J Pediatr Orthop ; 32(8): 799-804, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23147623

RESUMEN

STUDY DESIGN: A retrospective case-comparison study. OBJECTIVE: Compare efficacy and safety of combined intrathecal morphine (ITM) and epidural analgesia (EPI) to that of conventional intravenous patient-controlled analgesia (IV-PCA) after posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS). SUMMARY OF BACKGROUND DATA: Pain control after PSF in AIS has been managed traditionally with IV-PCA. More recently studies have shown improvement in pain control with the use of continuous EPI or intraoperative ITM. No studies to our knowledge have compared the use of both ITM and EPI analgesia to that of IV-PCA. METHODS: An Institutional Review Board-approved retrospective case-comparison study was performed from 1989 to 2009 of all patients undergoing PSF for AIS. Patients received either IV-PCA or ITM/EPI. Daily pain scores were recorded along with total opioid and benzodiazepine use. Adverse events were recorded for all the patients. RESULTS: A total of 146 patients were initially included in the study; 95 patients received ITM/EPI and 51 received IV-PCA as a historical control. Eight patients from the ITM/EPI group were excluded from the pain comparison portion of the study. There were no statistical differences in age, sex, weight, or hospital stay between the 2 groups. The ITM/EPI group had, on average, 1 additional level of fusion (P = 0.001). Daily average pain scores were lower in the ITM/EPI group on all hospital days, and statistically lower in days 1 and 3 to 5. Total opioid requirement was significantly lower in the ITM/EPI patients, although oral opioid use was higher among this group. Total benzodiazepine use was lower among the IV-PCA group. A total of 15.7% of the IV-PCA patients had bladder hypotonia, compared with 1.1% of the ITM/EPI group (P = 0.002). The rate of illeus was 15.7% in the IV-PCA patients and 5.7% in the ITM/EPI (P = 0.071). Respiratory depression was reported in 4 ITM/EPI patients, 0 in our PCA group. Technical catheter malfunction was reported in 8.5% of the EPI group. CONCLUSIONS: The use of ITM/EPI after PSF for AIS is safe and effective, this methodology provided significantly lower pain scores and lowers total opioid use which can lead to urinary and bowel dysfunction.


Asunto(s)
Dolor Postoperatorio/tratamiento farmacológico , Escoliosis/cirugía , Fusión Vertebral/métodos , Adolescente , Analgesia Epidural/efectos adversos , Analgesia Epidural/métodos , Analgesia Controlada por el Paciente/efectos adversos , Analgesia Controlada por el Paciente/métodos , Analgésicos Opioides/administración & dosificación , Analgésicos Opioides/efectos adversos , Niño , Quimioterapia Combinada , Femenino , Humanos , Inyecciones Espinales , Masculino , Morfina/administración & dosificación , Morfina/efectos adversos , Dimensión del Dolor , Dolor Postoperatorio/etiología , Estudios Retrospectivos
11.
J Pediatr Orthop ; 31(4): 450-4, 2011 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-21572284

RESUMEN

BACKGROUND: Spinal muscle atrophy (SMA) is a progressive neuromuscular disease predominantly presenting in infancy and early childhood. Scoliosis is the most common spinal deformity in these patients and treatment in SMA patients is controversial. Treatment is usually definitive fusion. The purpose of this study is to evaluate a novel growing rod technique used to treat more involved children with SMA types I and II with scoliosis at an earlier age. METHODS: An Institutional Review Board approved retrospective medical record review was performed of children with SMA who were treated for scoliosis with the growing rod construct. Chart and radiographic data were reviewed. Eleven patients met the inclusion criteria, 4 male and 7 female patients. No patients were lost to follow-up. Mean follow-up was 43 months (range, 24 to 76 mo). The average age at time of surgery was 6 years. Five patients had a diagnosis of SMA I, 6 patients with SMA II. The 11 children underwent 45 surgical procedures, 12 growing rod implantations with 34 lengthenings. TECHNIQUE: 4.5 mm titanium rods (Medronic Memphis, TN) were implanted obtaining a stable anchor point proximally with 4 pedicle screws and a cross link, and distally with 2 iliac bolts and 2 pedicle screws. Fusion is obtained at both anchor points by decortications and the use of bone graft. Subcutaneous low profile rods span between both anchor points using axial connectors. RESULTS: The average preoperative Cobb angle measurement of the major curve was 51.5 degrees (range, 38 to 76 degrees), postoperatively, 21.6 degrees (range, 2 to 34 degrees), and follow-up 18.7 degrees (range, 5 to 34 degrees). No surgical complications were identified or unplanned return to surgery. Medical complications were seen in 2 patients for postoperative pneumonia and anemia. CONCLUSIONS: Growing rod construct is an effective option in the treatment of scoliosis in SMA patients with scoliosis.


Asunto(s)
Procedimientos Ortopédicos/métodos , Escoliosis/cirugía , Fusión Vertebral/métodos , Atrofias Musculares Espinales de la Infancia/complicaciones , Niño , Femenino , Estudios de Seguimiento , Humanos , Masculino , Procedimientos Ortopédicos/efectos adversos , Procedimientos Ortopédicos/instrumentación , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Escoliosis/etiología , Escoliosis/fisiopatología , Fusión Vertebral/instrumentación , Atrofias Musculares Espinales de la Infancia/fisiopatología , Titanio , Resultado del Tratamiento
12.
J Pediatr Orthop ; 30(2): 199-205, 2010 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-20179570

RESUMEN

BACKGROUND: Recurrent unicameral bone cysts (UBCs) can result in significant morbidity during a child's physical and emotional development. Multiple treatment options are available and a review of the literature fails to clearly define the optimal treatment for UBCs. Recombinant bone morphogenetic protein (BMP) has been used with success in other disorders of poor bone formation. This manuscript is the first to report on the use of recombinant BMP in the treatment of UBCs. METHODS: Three patients with recurrent UBCs underwent revision surgery with recombinant BMP. Radiographic and medical review was performed and is reported here. RESULTS: In these patients, the use of BMP failed to fully resolve their UBC; 2 patients had complete recurrence that required further surgery. In addition to poor radiographic results, all patients developed exaggerated inflammatory responses in the acute postoperative period. Each child developed clinically significant limb swelling and pain that mimicked infection. CONCLUSIONS: On the basis of our poor radiographic results and a paradoxical clinical result, we no longer recommend the use of recombinant BMP in the manner reported here for the treatment of recurrent UBCs. LEVEL OF EVIDENCE: Level IV, case series.


Asunto(s)
Quistes Óseos/tratamiento farmacológico , Proteínas Morfogenéticas Óseas/efectos adversos , Inflamación/inducido químicamente , Proteínas Recombinantes/efectos adversos , Factor de Crecimiento Transformador beta/efectos adversos , Quistes Óseos/cirugía , Proteína Morfogenética Ósea 2 , Proteínas Morfogenéticas Óseas/uso terapéutico , Niño , Femenino , Humanos , Masculino , Proteínas Recombinantes/uso terapéutico , Recurrencia , Estudios Retrospectivos , Factor de Crecimiento Transformador beta/uso terapéutico , Insuficiencia del Tratamiento
13.
Pediatrics ; 145(3)2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-32094289

RESUMEN

Physical activity plays an important role in children's cardiovascular health, musculoskeletal health, mental and behavioral health, and physical, social, and cognitive development. Despite the importance in children's lives, pediatricians are unfamiliar with assessment and guidance regarding physical activity in children. With the release of the 2018 Physical Activity Guidelines by the US Department of Health and Human Services, pediatricians play a critical role in encouraging physical activity in children through assessing physical activity and physical literacy; providing guidance toward meeting recommendations by children and their families; advocating for opportunities for physical activity for all children in schools, communities, and hospitals; setting an example and remaining physically active personally; advocating for the use of assessment tools and insurance coverage of physical activity and physical literacy screening; and incorporating physical activity assessment and prescription in medical school curricula.


Asunto(s)
Consejo , Ejercicio Físico , Pediatras , Rol del Médico , Niño , Conductas Relacionadas con la Salud , Promoción de la Salud , Humanos , Padres , Guías de Práctica Clínica como Asunto , Instituciones Académicas
14.
Curr Probl Pediatr Adolesc Health Care ; 50(10): 100884, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-33069588

RESUMEN

Musculoskeletal illness represents a significant portion of office visits to primary and urgent care clinicians. Despite this, little emphasis is placed on learning pediatric orthopaedics during medical school or residency. The focus of this paper is to provide a systematic approach to this general musculoskeletal physical exam and to assist in the recognition of what conditions are normal development and what conditions require observation, workup and referral to an experienced pediatric orthopaedist.


Asunto(s)
Enfermedades Musculoesqueléticas/diagnóstico , Enfermedades Musculoesqueléticas/terapia , Atención Primaria de Salud/organización & administración , Adolescente , Factores de Edad , Niño , Deformidades del Pie/diagnóstico , Deformidades del Pie/terapia , Humanos , Examen Físico , Derivación y Consulta
15.
J Pediatr ; 154(5): 677-81, 2009 May.
Artículo en Inglés | MEDLINE | ID: mdl-19167724

RESUMEN

OBJECTIVE: To demonstrate the ability of a submaximal test to predict VO(2max) in overweight children. STUDY DESIGN: A total of 130 children, 11 to 14 years old, with body mass index >85 percentile for age and sex performed a submaximal walking test. VO(2max) was measured by using open circuit spirometry during a graded exercise test to volitional fatigue. An equation to predict VO(2max) was modeled by using the variables of sex, weight (kg), height (cm), heart rate (HR) after 4 minutes during the submaximal test (4minHR), HR difference (4minHR - resting HR), and submaximal treadmill speed (miles per hour [mph]) in 75% of the subjects. Validation was performed by using the remaining 25% of subjects. RESULTS: A total of 113 subjects achieved a maximal effort and was used in the statistical analysis. Development and validation groups were similar in all aspects. On validation, the mean square error was 241.06 with the predicted VO(2max) within 10% of the observed value in 67% of subjects. CONCLUSION: VO(2max) was accurately predicted in this cohort of overweight children by using a submaximal, treadmill-based testing protocol.


Asunto(s)
Prueba de Esfuerzo , Sobrepeso/fisiopatología , Consumo de Oxígeno/fisiología , Adolescente , Estatura , Índice de Masa Corporal , Peso Corporal , Niño , Femenino , Frecuencia Cardíaca/fisiología , Humanos , Masculino , Modelos Biológicos , Factores Sexuales , Espirometría
16.
J Pediatr ; 150(4): 383-7, 2007 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-17382115

RESUMEN

OBJECTIVE: We studied the relationship between % body fat (%BF), cardiovascular fitness (CVF), and insulin resistance (IR) in overweight middle-school children. STUDY DESIGN: Middle school children (n = 106, body mass index [BMI] > 95th percentile for age) underwent evaluation of body composition, maximal volume of oxygen utilization (VO2) uptake/kg lean body mass (VO2max/kgLBM), and fasting glucose and insulin (FI) concentrations and derived homeostasis model assessment index (HOMA(IR)). RESULTS: Both %BF (r = .33, P < .001) and VO2max/kgLBM (r = -0.42, P < .0001) were significantly correlated with FI. Bivariate regression analysis revealed %BF (P = .008 vs FI, P = .035 vs HOMA(IR)) and VO2max/kgLBM (P < .001 vs FI, P = .009 vs HOMA(IR)) to be independent predictors of insulin sensitivity. In males, VO2max/kgLBM was a better predictor of FI and HOMA(IR) than %BF. CONCLUSIONS: In obese middle-school children, both %BF and VO2max/kgLBM are independent predictors of FI levels. The relationship between CVF and FI levels was significant in both sexes but was particularly profound and stronger than %BF in males. Efforts to reduce risk of type 2 diabetes mellitus in an increasingly obese child population should include exercise intervention sustained enough to improve CVF.


Asunto(s)
Adiposidad , Insulina/sangre , Obesidad/metabolismo , Aptitud Física , Glucemia/metabolismo , Índice de Masa Corporal , Niño , Ayuno , Femenino , Humanos , Resistencia a la Insulina , Modelos Logísticos , Masculino , Análisis Multivariante , Obesidad/diagnóstico , Consumo de Oxígeno , Factores Sexuales
18.
Orthopedics ; 29(8): 724-6, 2006 08.
Artículo en Inglés | MEDLINE | ID: mdl-16924867

RESUMEN

The intraobserver and interobserver variability of common radiographic measurements associated with hip dysplasia from plain and digital radiographs was assessed. Only the interobserver reliability of measuring Hilgenreiner's distance was found to be potentially clinically significant. The average Spearman correlation coefficients between groups were 0.81 acetabular index, 0.80 medial teardrop to medial metaphysis distance, and 0.77 Hilgenreiner's distance. The average difference between digital and plain radiographs was acetabular index 0.97 +/- 0.6 degrees, medial teardrop to medial metaphysis distance 0.5 +/- 0.56 mm, and Hilgenreiner's distance 0.41 +/- 0.78 mm. These values are clinically insignificant; therefore, digital radiographs are as reliable as conventional radiographs in assessing hip dysplasia.


Asunto(s)
Acetábulo/anomalías , Acetábulo/diagnóstico por imagen , Enfermedades del Desarrollo Óseo/diagnóstico por imagen , Articulación de la Cadera/anomalías , Articulación de la Cadera/diagnóstico por imagen , Intensificación de Imagen Radiográfica , Preescolar , Humanos , Lactante , Variaciones Dependientes del Observador , Reproducibilidad de los Resultados , Estudios Retrospectivos
19.
Arch Pediatr Adolesc Med ; 159(10): 963-8, 2005 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-16203942

RESUMEN

BACKGROUND: Obesity and poor physical fitness constitute a health problem affecting an increasing number of children. Causes include a pervasive "toxic" environment that facilitates increased caloric intake and reduced physical activity. An effective strategy for prevention and treatment of childhood obesity likely includes a collaborative effort in the school setting. OBJECTIVE: To determine whether a school-based fitness program can improve body composition, cardiovascular fitness level, and insulin sensitivity in overweight children. DESIGN: Fifty overweight middle school children with a body mass index (BMI) above the 95th percentile for age were randomized to lifestyle-focused, fitness-oriented gym classes (treatment group) or standard gym classes (control group) for 9 months. Children underwent evaluation of fasting insulin and glucose levels, body composition by means of dual energy absorptiometry, and maximum oxygen consumption (V0(2)max) treadmill testing at baseline (before the school year) and at end of the school year. SETTINGS: Rural middle school and an academic children's hospital. MAIN OUTCOME MEASURES: Baseline test results for cardiovascular fitness, body composition, and fasting insulin and glucose levels. RESULTS: At baseline, there were no differences between groups before intervention (values for age, 12 +/- 0.5 years [all results, mean +/- SD]; BMI [calculated as weight in kilograms divided by the square of height in meters], 31.0 +/- 3.7; percentage of body fat, 36.5% +/- 4.6%; lean body mass, 41.4 +/- 8.6 kg; and V0(2)max, 31.5 +/- 5.1 mL/kg per minute). Compared with the control group, the treatment group demonstrated a significantly greater loss of body fat (loss, -4.1% +/- 3.4% vs -1.9% +/- 2.3%; P = .04), greater increase in cardiovascular fitness (V0(2)max, 2.7 +/- 2.6 vs 0.4 +/- 3.3 mL/kg per minute; P<.001), and greater improvement in fasting insulin level (insulin level, -5.1 +/- 5.2 vs 3.0 +/- 14.3 microIU/mL [-35.4 +/- 36.1 vs 20.8 +/- 99.3 pmol/L]; P = .02). CONCLUSIONS: Children enrolled in fitness-oriented gym classes showed greater loss of body fat, increase in cardiovascular fitness, and improvement in fasting insulin levels than control subjects. The modification to the school physical education curriculum demonstrates that small but consistent changes in the amount of physical activity has beneficial effects on body composition, fitness, and insulin levels in children. Partnering with school districts should be a part of a public health approach to improving the health of overweight children.


Asunto(s)
Composición Corporal/fisiología , Ejercicio Físico/fisiología , Insulina/sangre , Obesidad/terapia , Educación y Entrenamiento Físico , Aptitud Física/fisiología , Servicios de Salud Escolar , Niño , Femenino , Humanos , Masculino , Wisconsin
20.
J Child Orthop ; 9(2): 99-104, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25777178

RESUMEN

PURPOSE: The aim of this study was two-fold: (1) to determine if radiographic measures can be reliably made in infants being treated with the Ponseti method and (2) to document radiographic changes before and after Achilles tenotomy. METHODS: A retrospective radiographic and chart review was performed on children with clubfoot treated by the Ponseti method at a single institution over a 10-year period. Five independent reviewers measured a series of angles from a lateral forced dorsiflexion radiograph taken prior to and following Achilles tenotomy. These measures were taken in triplicate to determine the intra- and inter-reader reliability of dorsiflexion, tibio-calcaneal, talo-calcaneal, and talo-first metatarsal angles. RESULTS: Thirty-six subjects (56 feet) were treated with the Ponseti method and met the inclusion criteria. The median (range) age of patients at the time of tenotomy was 52 (34-147) days. The intra-reader reliability [intra-rater correlation coefficient (ICC)] for each of the measured angles pre- and post-tenotomy ranged from 0.933 to 0.995 and 0.864 to 0.995, respectively. Similarly, the inter-reader reliabilities (ICC) ranged from 0.727 for the pre-tenotomy (talo-calcaneal) to 0.950 for the post-tenotomy (talo-first metatarsal) angles. The mean differences between pre- and post-tenotomy radiographs were: dorsiflexion increase of 17°, tibio-calcaneal angle increase of 19°, talo-calcaneal angle increase of 9°, and talo-first metatarsal angle increase of 10° (p-value ≤0.001 for all measurements except the talo-first metatarsal angle, with a p-value of 0.001). CONCLUSIONS: Reliable radiographic measures can be made from lateral dorsiflexion radiographs of clubfeet treated with the Ponseti method before and after Achilles tenotomy.

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