Three-Dimensional Contrast-Enhanced Magnetic Resonance Pulmonary Angiography in the Study of Pulmonary Vascular Pathology

OBJECTIVES: To assess the feasibility of performing pulmonary angiography using MRI with contrast enhancement in patients with pulmonary vascular disease. METHODS: We present our experience in ten individuals, two controls and eight patients who underwent the exam after injection of a gadolinium-based contrast agent on a 1 Tesla MR scanner using a time-of-flight sequence and breath-holding during injection of contrast. RESULTS: Pathology in the main pulmonary artery and its major branches was detected easily while resolution at the segmental and subsegmental levels was inadequate. CONCLUSION: Contrast-enhanced magnetic resonance pulmonary angiography is feasible on a 1 Tesla MR scanner for the study of pathology of the main pulmonary artery and its major branches, like massive pulmonary embolism. However its ability to detect and define distal vessel pathology as found in chronic thromboembolic pulmonary hypertension and small pulmonary emboli is limited.

Effects of a Shortage of Imiglucerase on Three Patients with Type I Gaucher Disease

Background: Children with Gaucher disease type I (GD1) are usually treated with enzyme replacement therapy (ERT) at a dose of 30-60U/Kg/2W. Recently, due to an acute shortage supply of imiglucerase, a reduced dose or a reduced infusion frequency was recommended. Objective: To evaluate the effects of a reduced infusion frequency of imiglucerase over 15 months of follow-up. Patients and Methods: Three patients (1M:2F) were treated with ERT since a median age of 7 years (range 5-12). Only one had bone crisis and Erlenmeyer deformations. Median duration of treatment before dose reduction was 3 years (range 1-8). ERT resulted in total regression of symptoms, normalization of hematological parameters and progressive improvement of chitotriosidase in all patients. In August 2009 infusion schedule was changed from a media 45U/Kg every two weeks to every four weeks. Results: All patients remained asymptomatic and with no major change on hematological parameters except for the patient with bone crisis who presented subnormal platelet count. All patients showed an upward trend in chitotriosidase values. Comments: Although a longer follow-up is needed, is probable that even children completely stabilized can probably not be kept on lower doses even though the reduction of frequency of the infusions represent a lower social burden.

Diagnosic Classification: Results from a Clinical Experience of Three Years with DC: 0–3

Infancy and early childhood are characterized by a dynamic and ever changing process. Since the beginning of their clinical work at the Infancy Unit, the authors were concerned with individual assessment and the questions about the role played by parents as well as by babies in pathology and intervention.In this article, the authors begin with a description of the path that led them to the selection of DC 0–3 as a diagnostic classification system and how this has been instrumental in helping them to better define infant psychopathology and guide them in treatment orientations. Next, they present the results of the applicationof Axis I and II of DC: 0–3 in their clinical population in the years 1997, 1998, and 1999. The objectives of this study were to learn more about the distribution of mental disorders in a clinical population up tofour years of age. The authors attempted to separate infants at risk for developing psychic disorders from those presenting current psychopathology as well as the possible influence of demographic features on this distribution, to define a target population and design adapted therapeutic measures. The identification of these objectives provides the rationale for the use of a diagnostic tool, like DC: 0–3, which is essential to plan clinical activity, to evaluate therapeutic efficacy, and to develop specific programs.