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1.
J Neuromuscul Dis ; 8(2): 235-249, 2021.
Article in English | MEDLINE | ID: mdl-33361606

ABSTRACT

With more widespread prolonged survival, Duchenne muscular dystrophy patients progressively experience multisystem complications. We retrospectively reviewed the charts of 132 Duchenne patients (112 alive/20 dead, age 3.5÷32.3 years) with the aims: 1) to provide a comprehensive description of the clinical status considering different aspects of the disease; 2) to propose a new scoring tool able to consider and pool together heterogeneous different functional. Five functions were analyzed: cardiac, respiratory, nutritional, ambulation and scoliosis. For each function, different items were considered and classified according to clinical severity (as indicated by international guidelines) and an incremental scoring was assigned. In addition, a global score incorporating all functions was defined. The scoring system confirmed that despite the significant protective role of steroids, all functions deteriorated with age. The severity of the global score became significantly higher since the age of 13 years. The severity of cardiac, respiratory and nutritional dysfunction was higher since 18 years. Deceased patients were characterized by significantly worse cardiac function, absence of steroid therapy and later use of respiratory assistive devices. The index proposed in this pilot study is a promising tool able to aggregate and correlate heterogeneous functions. It could become either an individual prognostic indicator of decline or a global score to evaluate changes in clinical trials therefore allowing multicenter studies, optimizing the management of both the primary and the secondary complications of the disease and understanding their relative impact.


Subject(s)
Muscular Dystrophy, Duchenne/physiopathology , Adolescent , Adult , Child , Child, Preschool , Humans , Italy , Male , Pilot Projects , Retrospective Studies , Tertiary Care Centers , Young Adult
2.
AJNR Am J Neuroradiol ; 37(7): 1347-53, 2016 Jul.
Article in English | MEDLINE | ID: mdl-26988814

ABSTRACT

BACKGROUND AND PURPOSE: Cerebral palsy is frequently associated with both motor and nonmotor symptoms. DTI can characterize the damage at the level of motor tracts but provides less consistent results in nonmotor areas. We used a standardized pipeline of analysis to describe and quantify the pattern of DTI white matter abnormalities of the whole brain in a group of children with chronic bilateral cerebral palsy and periventricular leukomalacia. We also explored potential correlations between DTI and clinical scale metrics. MATERIALS AND METHODS: Twenty-five patients (mean age, 11.8 years) and 25 healthy children (mean age, 11.8 years) were studied at 3T with a 2-mm isotropic DTI sequence. Differences between patients and controls were assessed both voxelwise and in ROIs obtained from an existing DTI atlas. Clinical metrics included the Gross Motor Function Classification System, the Manual Ability Classification System, and intelligence quotient. RESULTS: The voxel-level and ROI-level analyses demonstrated highly significant (P < .001) modifications of DTI measurements in patients at several levels: cerebellar peduncles, corticospinal tracts and posterior thalamic radiations, posterior corpus callosum, external capsule, anterior thalamic radiation, superior longitudinal fasciculi and corona radiata, optic nerves, and chiasm. The reduction of fractional anisotropy values in significant tracts was between 8% and 30%. Statistically significant correlations were found between motor impairment and fractional anisotropy in corticospinal tracts and commissural and associative tracts of the supratentorial brain. CONCLUSIONS: We demonstrated the involvement of several motor and nonmotor areas in the chronic damage associated with periventricular leukomalacia and showed new correlations between motor skills and DTI metrics.


Subject(s)
Brain/diagnostic imaging , Cerebral Palsy/diagnostic imaging , Diffusion Tensor Imaging/methods , White Matter/diagnostic imaging , Anisotropy , Brain/pathology , Cerebral Palsy/etiology , Cerebral Palsy/pathology , Child , Female , Humans , Leukomalacia, Periventricular/complications , Leukomalacia, Periventricular/diagnostic imaging , Leukomalacia, Periventricular/pathology , Male , White Matter/pathology
3.
Methods Inf Med ; 55(1): 84-8, 2016.
Article in English | MEDLINE | ID: mdl-26640835

ABSTRACT

INTRODUCTION: This article is part of the Focus Theme of Methods of Information in Medicine on "Methodologies, Models and Algorithms for Patients Rehabilitation". OBJECTIVES: The great potential of robots in extracting quantitative and meaningful data is not always exploited in clinical practice. The aim of the present work is to describe a simple parameter to assess the performance of subjects during upper limb robotic training exploiting data automatically recorded by the robot, with no additional effort for patients and clinicians. METHODS: Fourteen children affected by cerebral palsy (CP) performed a training with Armeo®Spring. Each session was evaluated with P, a simple parameter that depends on the overall performance recorded, and median and interquartile values were computed to perform a group analysis. RESULTS: Median (interquartile) values of P significantly increased from 0.27 (0.21) at T0 to 0.55 (0.27) at T1 . This improvement was functionally validated by a significant increase of the Melbourne Assessment of Unilateral Upper Limb Function. CONCLUSIONS: The parameter described here was able to show variations in performance over time and enabled a quantitative evaluation of motion abilities in a way that is reliable with respect to a well-known clinical scale.


Subject(s)
Cerebral Palsy/rehabilitation , Rehabilitation/methods , Robotics , Upper Extremity/physiopathology , Adolescent , Cerebral Palsy/diagnosis , Child , Humans , Inpatients , Physical Therapy Modalities , Reproducibility of Results , Sample Size , Upper Extremity/physiology
4.
Annu Int Conf IEEE Eng Med Biol Soc ; 2015: 3590-3, 2015 Aug.
Article in English | MEDLINE | ID: mdl-26737069

ABSTRACT

Cerebral palsy (CP), one of the most common neurological disorders in childhood, features affected individual's motor skills and muscle actions. This results in elevated heart rate and rate of oxygen uptake during sub-maximal exercise, thus indicating a mean energy expenditure higher than healthy subjects. Rehabilitation, currently involving also robot-based devices, may have an impact also on these aspects. In this study, an ecological setting has been proposed to evaluate the energy expenditure of 4 children with CP before and after a robot-assisted gait training. Even if the small sample size makes it difficult to give general indications, results presented here are promising. Indeed, children showed an increasing trend of the energy expenditure per minute and a decreasing trend of the energy expenditure per step, in accordance to the control group. These data suggest a metabolic benefit of the treatment that may increase the locomotion efficiency of disabled children.


Subject(s)
Cerebral Palsy/therapy , Gait Disorders, Neurologic/therapy , Activities of Daily Living , Adolescent , Case-Control Studies , Cerebral Palsy/metabolism , Cerebral Palsy/physiopathology , Child , Disabled Children , Energy Metabolism , Exercise Therapy/instrumentation , Female , Gait , Gait Disorders, Neurologic/metabolism , Gait Disorders, Neurologic/physiopathology , Humans , Male , Monitoring, Ambulatory , Motor Skills , Robotics , Walking
5.
Article in English | MEDLINE | ID: mdl-26738003

ABSTRACT

The Gait Real-time Analysis Interactive Lab (GRAIL) is an instrumented multi-sensor platform based on immersive virtual reality for gait training and rehabilitation. Few studies have been included GRAIL to evaluate gait patterns in normal and disabled people and to improve gait in adults, while at our knowledge no evidence on its use for the rehabilitation of children is available. In this study, 4 children suffering from acquired brain injury (ABI) underwent a 5 session treatment with GRAIL, to improve walking and balance ability in engaging VR environments. The first and the last sessions were partially dedicated to gait evaluation. Results are promising: improvements were recorded at the ankle level, selectively at the affected side, and at the pelvic level, while small changes were measured at the hip and knee joints, which were already comparable to healthy subjects. All these changes also conveyed advances in the symmetry of the walking pattern. In the next future, a longer intervention will be proposed and more children will be enrolled to strongly prove the effectiveness of GRAIL in the rehabilitation of children with ABI.


Subject(s)
Brain Injuries/rehabilitation , Gait/physiology , Rehabilitation/methods , User-Computer Interface , Walking/physiology , Ankle/physiology , Child , Humans , Pelvis/physiology
6.
Respir Med ; 106(2): 276-83, 2012 Feb.
Article in English | MEDLINE | ID: mdl-22083092

ABSTRACT

In the respiratory management of DMD patients it is still under debate what parameter should indicate the correct timing for institution of nocturnal non-invasive ventilation (NIV), in addition to forced vital capacity, which is generally considered as a prognostic marker of disease progression. The aim of this study was to determine if volume variations of rib cage and abdominal compartments measured by Opto-Electronic Plethysmography can be helpful to distinguish between those patients who are in the early stages of nocturnal oxygen desaturation development and those who do not yet. Pulmonary function, abdominal contribution to tidal volume and to inspiratory capacity (%Abd IC) and a set of breathing pattern indexes were assessed in 40 DMD patients older than 14 years and not yet under nocturnal NIV. ROC analysis revealed that among all the considered parameters, %Abd IC in supine position was the best discriminator between DeSat (at least 10% of the night time with SpO(2) < 95%) and NonDeSat patients, providing an area under the curve with 95%CI equal to 0.752. In conclusion, in adolescents and adults DMD patients who present either no sign or only mild nocturnal oxygen desaturation, a reduced abdominal contribution to inspiratory capacity is a marker of the onset of diaphragm weakness and should be considered to identify the correct timing for the institution of nocturnal NIV.


Subject(s)
Abdomen/physiopathology , Hypoxia/physiopathology , Muscular Dystrophy, Duchenne/physiopathology , Respiration , Respiratory Muscles/physiopathology , Sleep Apnea Syndromes/physiopathology , Adolescent , Adult , Child , Female , Humans , Hypoxia/etiology , Hypoxia/therapy , Male , Muscular Dystrophy, Duchenne/complications , Muscular Dystrophy, Duchenne/therapy , Oxygen Inhalation Therapy , Plethysmography , ROC Curve , Sleep Apnea Syndromes/etiology , Sleep Apnea Syndromes/therapy , Thoracic Wall/physiopathology , Tidal Volume , Vital Capacity , Young Adult
7.
J Neurosci Methods ; 201(1): 239-50, 2011 Sep 30.
Article in English | MEDLINE | ID: mdl-21816172

ABSTRACT

Brain-Computer Interfaces (BCIs) are systems which can provide communication and environmental control to people with severe neuromuscular diseases. The current study proposes a new BCI-based method for psychometric assessment when traditional or computerized testing cannot be used owing to the subject's output impairment. This administration protocol was based on, and validated against, a widely used clinical test (Raven Colored Progressive Matrix) in order to verify whether BCI affects the brain in terms of cognitive resource with a misstatement result. The operating protocol was structured into two phases: phase 1 was aimed at configuring the BCI system on the subject's features and train him/her to use it; during phase 2 the BCI system was reconfigured and the test performed. A step-by-step checking procedure was adopted to verify progressive inclusion/exclusion criteria and the underpinning variables. The protocol was validated on 19 healthy subjects and the BCI-based administration was compared with a paper-based administration. The results obtained by both methods were correlated as known for traditional assessment of a similarly culture free and reasoning based test. Although our findings need to be validated on pathological participants, in our healthy population the BCI-based administration did not affect performance and added a further control of the response due to the several variables included and analyzed by the computerized task.


Subject(s)
Brain/physiology , Cognition/physiology , Neuropsychological Tests/standards , Psychomotor Performance/physiology , User-Computer Interface , Adolescent , Adult , Humans , Male , Young Adult
8.
J Neurol Sci ; 306(1-2): 54-61, 2011 Jul 15.
Article in English | MEDLINE | ID: mdl-21529845

ABSTRACT

We studied respiratory function and Chest Wall kinematics in a large population of adult patients affected by slow course muscular dystrophies such as Limb-Girdle Muscular Dystrophy (LGMD, n=38), Becker Muscular Dystrophy (BMD, n=20) and Facio-Scapulo Humeral Dystrophy (FSHD, n=30), through standard spirometry and through the Optoelectronic Plethysmography, to measure the thoraco-abdominal motion during Quiet Breathing and Slow Vital Capacity maneuvers. Within the restrictive pulmonary syndrome characterizing LGMD and FSHD, several different thoraco-abdominal patterns compared to those of healthy subjects were present in the more advanced stages of the disease. These differences were present in the seated position, during the execution of a maximal maneuver such as Slow Vital Capacity. A global respiratory (both inspiratory and expiratory) muscle involvement was more pronounced in the LGMD and FSHD than in the BMD patients, and a significant reduction of abdominal contribution in wheelchair bound patients was observed. In conclusion, OEP technique is able to reveal mild initial modifications in the respiratory muscles in FSHD and LGMD patients, which could be helpful for functional and new therapeutic strategy evaluation.


Subject(s)
Lung Diseases/etiology , Muscular Dystrophies/complications , Respiration , Adult , Female , Heart Diseases/etiology , Humans , Lung Diseases/diagnosis , Male , Middle Aged , Muscle Strength/physiology , Muscle, Skeletal/physiopathology , Muscular Dystrophies/classification , Muscular Dystrophies/diagnosis , Muscular Dystrophies/pathology , Plethysmography/methods , Respiratory Function Tests , Scoliosis/etiology , Vital Capacity/physiology , Young Adult
9.
Med. infant ; 18(1): 18-22, mar. 2011. tab, graf
Article in Spanish | LILACS | ID: lil-774194

ABSTRACT

El propósito de este estudio fue evaluar la progresión de la disfunción crónica del injerto en receptores pediátricos de un TxR que recibían CsA como parte de su inmunosupresión y ésta fue reemplazada por SRL, a causa de un lento y progresivo aumento de la creatinina sérica, signos histológicos de nefropatía crónica del trasplante y ausencia de rechazo agudo en la biopsia renal. Se empleó la inversa la de creatinina sérica (1/CrS) para estimar la velocidad de progresión de la disfunción crónica del injerto, durante el año previo al cambio de la medicación y dos años después del mismo. Analizamos 26 pacientes trasplantados a los 9.76 ± 3.9 años que recibían CsA y en quienes se reemplazó ésta por SRL a los 57.07 ± 39.1 meses post trasplante. La pendiente de la 1/CrS disminuyó de -0.016 mg/dl/mes antes del cambio a -0.002 mg/dl/mes durante el primer año después del mismo y a -0.001 mg/dl/mes durante el segundo año (p= 0.007). Cuando separamos los pacientes según el grado de NCT que mostraban las biopsias renales, aquellos pacientes que tenían NCT grado I (n= 11), la pendiente de la 1/CrS mejoró de -0.012 mg/dl/mes basal a -0.006 mg/dl/mes durante el primer año y a +0.001 mg/dl/mes durante el segundo año (p= 0.01). En cambio, los pacientes que tenían NCT grado II en sus biopsias, mostraron mejoría de la pendiente de la 1/CrS durante el primer año luego del cambio, pero la misma no fue sostenida ni significativa durante el segundo año de observación. Entre ambos grupos, las únicas variables que mostraron diferencias significativas fueron el filtrado glomerular y el tiempo post trasplante al momento del cambio de la inmunosupresión. Conclusión: nuestros resultados muestran una significativa disminución de la velocidad de progresión de la disfunción crónica del injerto cuando se reemplaza la CsA por SRL en pacientes con mínimos cambios morfológicos en la biopsia renal y con un filtrado glomerular cercano a 60 ml/min/1.73.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Cyclosporine/therapeutic use , Graft Rejection , Kidney Transplantation , Sirolimus/therapeutic use , Tacrolimus/therapeutic use , Kidney Transplantation/adverse effects , Argentina
10.
Eur J Phys Rehabil Med ; 46(3): 369-76, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20927003

ABSTRACT

BACKGROUND: In the last decades, the world of rehabilitation has been more and more calling for clear evidence to support intervention and numerous research programs have been developed. At stake, relatively little research on opinions and attitude of rehabilitation personnel involved in research conducted in real clinical settings has been carried out. AIM: To explore the opinion of professionals involved in a national clinical trial on research. DESIGN: Multicentre cross-sectional study. SETTING: 19 rehabilitation centres/services (4 research institutes, 15 local rehabilitation services). POPULATION: All professional participating to a multi-centre clinical trial on the effects of Constraint Induced Movement Therapy on children with hemiplegic cerebral palsy. METHODS: A 15-questions questionnaire inquiring feasibility, usefulness, products, costs, judgement and perceptions about clinical research in rehabilitation was administered. RESULTS: Among those working in one of the 19 rehabilitation centres part of the multicentric study, 76 professionals were asked to fill in the questionnaire. 68 professionals answered (89.4% of response rate). More than 75% of the sample thinks that its rehabilitation centre is suited to develop clinical research. Research results useful for the development of their daily activities (new tools for the assessment of children, to demonstrate the efficacy of a new treatment option and to learn a new way of working, and to strengthen the ties within the working team). Research is costly in terms of personal time and effort, but it can modify the rehabilitation praxis (assessment tools, the relationship with colleagues/patients). 98% of the interviewees declared the willingness to participate to other research projects. CONCLUSION AND CLINICAL REHABILITATION IMPACT: This survey highlights the importance of conducting research in local rehabilitation services, not only in terms of generation of new evidences, but also in terms of building networks, sharing experiences and knowledge, connecting with centers of excellence and providing a specific training for research conduction.


Subject(s)
Attitude of Health Personnel , Clinical Trials as Topic , Health Services Research , Hemiplegia/rehabilitation , Motion Therapy, Continuous Passive/methods , Adult , Age Factors , Cross-Sectional Studies , Feasibility Studies , Female , Humans , Italy , Male , Middle Aged , Multicenter Studies as Topic , Pilot Projects , Rehabilitation Centers , Restraint, Physical/instrumentation , Restraint, Physical/methods , Sex Factors , Surveys and Questionnaires , Workforce , Young Adult
11.
Respir Physiol Neurobiol ; 172(3): 184-91, 2010 Jul 31.
Article in English | MEDLINE | ID: mdl-20510388

ABSTRACT

To investigate the effects of posture and gender on thoraco-abdominal motion and breathing pattern, 34 healthy men and women were studied by Opto-Electronic Plethysmography during quiet breathing in five different postures from seated (with and without back support) to supine position. Chest wall kinematics and breathing pattern were significantly influenced by position and gender. The progressively increased inclination of the trunk determined a progressive reduction of rib cage displacement, tidal volume, and minute ventilation and a progressive increase of abdominal contribution to tidal volume. Female subjects were characterized by smaller dimensions of the rib cage compartment and during quiet breathing by lower tidal volume, minute ventilation and abdominal contribution to tidal volume than males. The effect of posture on abdominal kinematics was significant only in women. The presence of a back support in seated position determined differences in breathing pattern. In conclusion, posture and gender have a strong influence on breathing and on chest wall kinematics.


Subject(s)
Abdomen/physiology , Posture/physiology , Respiration , Respiratory Mechanics/physiology , Thorax/physiology , Adult , Anthropometry , Biomechanical Phenomena , Body Weight/physiology , Female , Humans , Male , Middle Aged , Plethysmography , Reference Values , Respiratory Rate/physiology , Ribs/physiology , Sex Characteristics , Supine Position/physiology , Tidal Volume/physiology , Xiphoid Bone/physiology , Young Adult
12.
Med. infant ; 17(2): 151-155, Junio 2010. ilus, Tab
Article in Spanish | LILACS, UNISALUD, BINACIS | ID: biblio-1248033

ABSTRACT

El retardo de crecimiento es un importante problema clínico aun no resuelto ni correctamente manejado en niños con insuficiencia renal crónica (IRC). La optimización de todos los parámetros metabólicos y nutricionales no siempre lleva a una mejoría del crecimiento en estos pacientes. Desde hace aproximadamente 20 años se utiliza el tratamiento con rhGH para mejorar la talla en este grupo de niños. La bibliografía internacional muestra mejoría de la velocidad de crecimiento en estos pacientes sin embargo la experiencia publicada en la talla final (TF) alcanzada por los mismos es escasa. Los objetivos de este estudio fueron:1) evaluar la talla final alcanzada por pacientes transplantados renales(TxR) que recibieron tratamiento con rhGH (GrGH) comparándolos con un grupo control (GrC) con similares características clínicas, 2) evaluar los factores predictores de la TF, y 3) la repercusión de dicho tratamiento en la función renal. La TF en el GrGH fue significativamente mayor que la TF del GrC (-1.96 ± 1.13 vs -3.48 ± 1.19 SDS respectivamente, p <0.05). La talla (SDS) al inicio del tratamiento con rhGH fue la única variable significativa para predecir la respuesta al tratamiento (p= 0.001). Se observó una disminución significativa ClCr final en ambos grupos (GrGH: 76 ± 18 vs 66 ± 14 ml/min/m2 sup p<0.05; GrC: 72 ± 19 vs 56 ± 9 ml/min/m2 sup, p<0.05) lo que sugiere una caída similar del filtrado glomerular en ambos grupos independiente del tratamiento. Conclusión: Nuestros hallazgos permiten confirmar que el tratamiento con rhGH es efectivo para mejorar la talla final en pacientes TxR sin afectar la función renal (AU)


Growth retardation is a common and significant clinical problem that is not adequately managed in children with chronic renal disease. Despite optimization of metabolic parameters the growth of this patients not always amelioreted. About 20 years ago rhGH treatment became to be used for this group of children to optimization final height.The international experience show that rhGH treatment improve growth velocity but the results about final heigth are scarse. The aims of our trial were: 1) to evaluate final height in renal transplant patients treated with rhGH (n=23) comparing with a control group not treated with rhGH (n=14) with similar characteristics, 2) to evaluate the effect of rhGH on creatinine clearance,3) to establish predictive variables for final height. Final Heigth was significantly greater in treated group vs control group (-1.9±1.1 vs -3.5±1.2, p<0.05). Initial height was the only significant variable to predict final height (p=0.001). We described a significantly decrease of creatinine clearence in both groups during follow up (GH Group 76±9 vs 66±14 ml/min/m2 sup, p<0.05 and Control Group 72.5±19 vs 56±9 ml/min/m2 sup, p= p<0.05).This suggest a similar decrese of creatinine clearence in both groups. Conclution: Our data confirm that rhGH treatment was effective in improving final height in renal transplant patients and did not decline allograft function (AU)


Subject(s)
Humans , Child , Adolescent , Body Height/drug effects , Recombinant Proteins/therapeutic use , Kidney Transplantation , Human Growth Hormone/therapeutic use , Renal Insufficiency, Chronic/complications , Growth Disorders/drug therapy , Case-Control Studies , Chronic Disease , Treatment Outcome
13.
Eur Respir J ; 35(5): 1118-25, 2010 May.
Article in English | MEDLINE | ID: mdl-19840972

ABSTRACT

Duchenne muscular dystrophy (DMD) is characterised by progressive loss of muscular strength that leads to an increasingly restrictive pulmonary syndrome. However, it is still not clear whether this determines alterations in the breathing pattern. We studied: 66 DMD patients at different stages of the disease (mean+/- sem age 12.6+/-0.6 yrs, range 5-22 yrs of age), subdivided into four groups according to age; and 21 age-matched healthy male controls. Spirometry, lung volumes and nocturnal oxygen saturation were measured in all DMD patients. Ventilatory pattern and chest wall volume variations were assessed by optoelectronic plethysmography during spontaneous breathing both in seated and supine positions. Whilst in a seated position, no significant differences were found between patients and controls or between different age groups. In the supine position, the average contribution of abdominal volume change (DeltaV(AB)) to tidal volume progressively decreased with age (p<0.001). The patients who showed nocturnal hypoxaemia showed significantly lower Delta V(AB). In conclusion, chest wall motion during spontaneous breathing in awake conditions and in supine position is an important indicator of the degree of respiratory muscle impairment in DMD. DeltaV(AB) is not only an important marker of the progression of the disease but is also an early indicator of nocturnal hypoxaemia.


Subject(s)
Abdomen/physiology , Muscular Dystrophy, Duchenne/physiopathology , Respiratory Mechanics/physiology , Respiratory Muscles/physiopathology , Adolescent , Analysis of Variance , Case-Control Studies , Child , Child, Preschool , Female , Humans , Male , Plethysmography , Respiratory Function Tests , Statistics, Nonparametric , Tidal Volume , Young Adult
14.
Med. infant ; 16(2): 118-125, jun. 2009. tab, graf
Article in Spanish | BINACIS | ID: bin-124839

ABSTRACT

Evaluación retrospectiva de 575 trasplantes (TX), 64,3 por ciento con donante cadavérico (DC), en 550 pacientes (311 varones) edad por x: 10.8 más menos 4.2 años, efectuados entre 1988 y 2008. edad por de donante: DC 22.5 más menos 14 años y DVR: 37.3 más menos 7.7 años. Principales causas de IRC: nefropatía por reflujo: 34,1 por ciento, hipo-displasia: 15.1 por ciento, SUH; 12.9 por ciento, GSF: 9.82 por ciento, glomerulonefritis varias: 16.4 por ciento. Inmunosupresion: en la mayoría de los pacientes, Cicloporina A; Azatioprina o micofenolato mofetil o ácido micofenólico y esteroides con linfo o timoglobulina secuencia en TXDC y profilaxis con gaciclovir en riesgo de infección por CMV. La sobrevida actuarial funcional renal (SA) a 1.3 a 5 años fue 96.5 por ciento, 94.4 por ciento y 86,2 por ciento TX DVR y 90,1 por ciento, 85,5 por ciento y 77.6 por ciento TX DC, p= 0.04, similar a resultados en EEUU (NAPRCTS 1999 - 2002). La GSF con 45.5 por ciento de recurrencia del síndrome nefrótico, tuvo inferior SA al 5to año, p= 0.001, comparado con otras etiologías de IRC. Los TX sin diálisis (D) previa, p= 0.003. Tuvieron trombosis 2.61 por ciento de los TX, más frecuentes con DPCA pre tx que con hemo D o sin diálisis, p= 0.01, con TXDC, p= 0.02 y con TX de donantes < de 6 años, p = 0.02. Los pacientes que requirieron diálisis post trasplante, tuvieron mayor creatinina al año D: 1.8 más menos 2.27 mg/dl.SD: 1.19 más menos 1.2, p < 0.01, e inferior SA al quinto año, p=0.001. Con tiempo de isquemia fria superior a 24 horas, 31,6 por ciento de los DC necesitaron diálisis. El rechazo celular agudo se dianosticó en el 14,8 por ciento de los pacientes. Las causas más frecuentes de fracaso del trasplante fueron: nefropatía crónica (69,8 por ciento) asociado a inadecuada adherencia en 54.7 por ciento, trombosis (12.6 por ciento), recurrencia (5.9 por ciento), ausencia de función (5 por ciento) rechazo severo (5 por ciento)Desarrollaron enfermedad. (AU)


Subject(s)
Infant , Child, Preschool , Kidney Transplantation/adverse effects , Kidney Transplantation/statistics & numerical data , Renal Insufficiency, Chronic/diagnosis , Kidney Transplantation/mortality , Graft Rejection/etiology , Renal Insufficiency, Chronic/etiology , Survival , Retrospective Studies , Data Interpretation, Statistical
15.
Med. infant ; 16(2): 157-162, jun. 2009. tab, graf
Article in Spanish | BINACIS | ID: bin-124833

ABSTRACT

La glomerulosclerosis focal y segmentaria (GSFS) es en Argentina la 3er cuasa de insuficiencia Renal Crónica (IRCT) en niños luego de las malformaciones y el SUH. Se ha señalado una peor sobrevida del injerto renal en pacientes con IRCT secundaria a GSFS post TX. El objeto primario de este trabajo fue evaluar la sobrevida alejada de injerto y paciente en niños con IRCT secundaria a GSFS trasplantad os y compararla con la de niños con IRCT de otras causas trnasplantados. También se evaluó el impacto de la recidiva del sindrome nefrótico, el tipo de donante y la necesidad hemodiálisis en la sobrevida del paciente de injerto. Se incluyeron los 534 trasplantes renales efectuados en el Hospital Juan P. Garrahan desde el 1/12/1988 hasta el 31/12/2007. Se definió como recurrencia del SN luego del TX a una proteinuria de 24 horas > a 50 mg/kg/día e hipoalbuminemia (< a 2.5 g/d). Para analizar la sobrevida de injerto y paciente se utilizó método de Kaplan Meier y test de log Rank para determinar significación estadística de causa una de las categorías evaluadas. Se estimó a Hazar Ratio (HR) de pérdida de injerto para: recurrencia GSFS, tipo de donante, necesidad de dialisis (dos primeras semanas post TX), y remisión de la proteinura. Se efectuaron 56 TX en 51 pacientes (48 por ciento mujeres) con GSFS, como causa de IRCT con una edad promedio TX de 10.97 más menos 4.28 años; 34 recibieron un injeto DC (64 por ciento). Se observó recurrencia del SN en 23/53 (43 por ciento). En el grupo con GSFS más la sobrevida del injerto a 1 ,3,5, y 10 años fue del 79 por ciento, 71 por ciento, 62 por ciento y 31 por ciento respectivamente, significativamente peor que la del grupo sin GSFS: 93 por ciento, 86 por ciento, 82 por ciento y 60 por ciento. En el grupo GSFS más se perdieron el 49 por ciento de los injertos comprado el 24.7 por ciento del grupo GSFS (p<0.05). Los pacientes con GSFS trasplantados con DC tuvieron peor sobrevida del injerto.(AU)


Subject(s)
Male , Female , Infant , Child, Preschool , Kidney Transplantation/adverse effects , Kidney Transplantation/mortality , Glomerulosclerosis, Focal Segmental/complications , Renal Insufficiency, Chronic/etiology , Nephrotic Syndrome , Graft Survival , Observational Studies as Topic , Retrospective Studies , Cohort Studies
18.
Med. infant ; 16(2): 118-125, jun. 2009. tab, graf
Article in Spanish | LILACS | ID: lil-538113

ABSTRACT

Evaluación retrospectiva de 575 trasplantes (TX), 64,3 por ciento con donante cadavérico (DC), en 550 pacientes (311 varones) edad por x: 10.8 más menos 4.2 años, efectuados entre 1988 y 2008. edad por de donante: DC 22.5 más menos 14 años y DVR: 37.3 más menos 7.7 años. Principales causas de IRC: nefropatía por reflujo: 34,1 por ciento, hipo-displasia: 15.1 por ciento, SUH; 12.9 por ciento, GSF: 9.82 por ciento, glomerulonefritis varias: 16.4 por ciento. Inmunosupresion: en la mayoría de los pacientes, Cicloporina A; Azatioprina o micofenolato mofetil o ácido micofenólico y esteroides con linfo o timoglobulina secuencia en TXDC y profilaxis con gaciclovir en riesgo de infección por CMV. La sobrevida actuarial funcional renal (SA) a 1.3 a 5 años fue 96.5 por ciento, 94.4 por ciento y 86,2 por ciento TX DVR y 90,1 por ciento, 85,5 por ciento y 77.6 por ciento TX DC, p= 0.04, similar a resultados en EEUU (NAPRCTS 1999 - 2002). La GSF con 45.5 por ciento de recurrencia del síndrome nefrótico, tuvo inferior SA al 5to año, p= 0.001, comparado con otras etiologías de IRC. Los TX sin diálisis (D) previa, p= 0.003. Tuvieron trombosis 2.61 por ciento de los TX, más frecuentes con DPCA pre tx que con hemo D o sin diálisis, p= 0.01, con TXDC, p= 0.02 y con TX de donantes < de 6 años, p = 0.02. Los pacientes que requirieron diálisis post trasplante, tuvieron mayor creatinina al año D: 1.8 más menos 2.27 mg/dl.SD: 1.19 más menos 1.2, p < 0.01, e inferior SA al quinto año, p=0.001. Con tiempo de isquemia fria superior a 24 horas, 31,6 por ciento de los DC necesitaron diálisis. El rechazo celular agudo se dianosticó en el 14,8 por ciento de los pacientes. Las causas más frecuentes de fracaso del trasplante fueron: nefropatía crónica (69,8 por ciento) asociado a inadecuada adherencia en 54.7 por ciento, trombosis (12.6 por ciento), recurrencia (5.9 por ciento), ausencia de función (5 por ciento) rechazo severo (5 por ciento)Desarrollaron enfermedad.


Subject(s)
Infant , Child, Preschool , Renal Insufficiency, Chronic/diagnosis , Renal Insufficiency, Chronic/etiology , Graft Rejection/etiology , Survival , Kidney Transplantation/adverse effects , Kidney Transplantation/mortality , Kidney Transplantation , Data Interpretation, Statistical , Retrospective Studies
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