RESUMEN
Moyamoya disease (MMD) is an uncommon cerebrovascular disorder characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. Direct and indirect bypass techniques have been devised with the aim of promoting neoangiogenesis. The current study aimed to investigate the role of multiple cranial burr hole (MCBH) operations in the prevention of cerebral ischemic attacks in children with MMD. Seven children suffering from progressive MMD were submitted to the MCBH and arachnoid opening technique. Ten to 20 burr holes were drilled in the fronto-temporo-parieto-occipital area of each hemisphere in each patient, depending on the site and extent of the disease. All patients were evaluated pre- and postoperatively by means of Barthel index (BI), CT, MR, angio-MR, and angiography. Patients had no recurrence of ischemic attacks postoperatively. Neoangiogenesis was observed in both hemispheres. One patient developed a persistent subdural collection after surgery, thus requiring placement of a subdural-peritoneal shunt. Postoperative BI was statistically significantly improved (P=0.02). This report suggests that MCBH for revascularization in MMD is a simple procedure with a relatively low risk of complications and effective for preventing cerebral ischemic attacks in children. In addition, MCBH may be placed as an adjunct to other treatments for MMD.
Asunto(s)
Revascularización Cerebral/métodos , Ataque Isquémico Transitorio/etiología , Ataque Isquémico Transitorio/prevención & control , Enfermedad de Moyamoya/cirugía , Trepanación/métodos , Adolescente , Niño , Preescolar , Craneotomía/métodos , Femenino , Humanos , Angiografía por Resonancia Magnética/métodos , Imagen por Resonancia Magnética/métodos , Masculino , Enfermedad de Moyamoya/complicaciones , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos , Resultado del TratamientoRESUMEN
Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH.
Asunto(s)
Enfermedades del Sistema Endocrino/patología , Histiocitosis de Células de Langerhans/patología , Edad de Inicio , Niño , Preescolar , Progresión de la Enfermedad , Enfermedades del Sistema Endocrino/diagnóstico por imagen , Enfermedades del Sistema Endocrino/tratamiento farmacológico , Femenino , Histiocitosis de Células de Langerhans/diagnóstico por imagen , Histiocitosis de Células de Langerhans/tratamiento farmacológico , Terapia de Reemplazo de Hormonas , Hormonas/sangre , Humanos , Lactante , Pulmón/diagnóstico por imagen , Pulmón/patología , Imagen por Resonancia Magnética , Masculino , Hipófisis/diagnóstico por imagen , Hipófisis/patología , Radiografía , Estudios RetrospectivosRESUMEN
OBJECTIVE: To determine the prognostic value of exercise testing, valve area, and maximum transaortic pressure gradient in asymptomatic patients with aortic valve stenosis. SETTING: The outpatient service of a tertiary referral centre for cardiology. DESIGN: Prospective clinical study. PATIENTS: 66 consecutive patients with isolated severe aortic stenosis (aortic valve area /= 0.7 cm(2) (p = 0.0021). There was no relation between the end points and transaortic gradient (p = 0.6882). In multivariate analysis, a hazard ratio of 7.43 was calculated for patients with a positive versus a negative exercise stress test. Although asymptomatic in daily life, 6% of the patients (4/66) experienced sudden death; all these had a positive exercise test and an aortic valve area of
Asunto(s)
Estenosis de la Válvula Aórtica/diagnóstico , Prueba de Esfuerzo/métodos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Estenosis de la Válvula Aórtica/patología , Estenosis de la Válvula Aórtica/fisiopatología , Presión Sanguínea , Estudios de Cohortes , Contraindicaciones , Toma de Decisiones , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Medición de Riesgo , Factores de RiesgoRESUMEN
PURPOSE: to evaluate the exercise test as a prognostic factor for asymptomatic patients with aortic valve stenosis. METHODS: During 60 months 70 patients with aortic valve area < or = 1 cm2 without any other heart lesion were studied. The patients underwent Dopplerechocardiograms exercise tests (considered positive in the presence of electrocardiographic changes or symptoms) and clinical follow-up in order to determine the onset of symptoms or sudden death. RESULTS: The event-free probability was 50% after 16 months of follow-up. There was no relationship between the occurrence of events and the sex or to the transvalvar gradient. However, there was a statistically significant association between the incidence of events (p = 0.0124), valvar area (p = 0.0003) and exercise test results (p = 0.0001). CONCLUSION: Patients with significant aortic stenosis even without symptoms, have a low survival rate and the exercise test is a good prognostic factor during follow-up.