RESUMO
Primary cardiac tumors are rare in children. The benign forms constitute 90% of cases, with often fatal clinical course due to the origin site. Here is reported a case of fibroelastic hamartoma in a four month baby, with multiple malformations, in which the initial signs have been alterations of ECG and of the cardiac profile at Rx chest. Bidimensional echocardiography suggested the correct diagnosis, revealing the mass in the interventricular septum. Cardiac catheterization excluded connections with coronary arteries. The baby died before cardiosurgical operation, probably because of a hyperkinetic ventricular arrhythmia. Anatomo-pathologic examination discusses the diagnosis of this tumor versus other kinds, defining our case as a fibroelastic hamartoma.
Assuntos
Hamartoma/patologia , Neoplasias Cardíacas/patologia , Feminino , Septos Cardíacos , Humanos , Lactente , UltrassonografiaRESUMO
Two pediatric cases are described presenting a conjunction of Addison's disease and hypoparathyroidism. One of the two cases also exhibits moniliasis of the oral cavity (Whitaker's disease). A full report is given of the symptoms and of the laboratory analyses which have motivated the diagnosis, and of the endocrinological function tests which were intended to confirm the diagnosis. The clinical process and the therapeutic criteria adopted are subsequently reported. A discussion follows focusing on the possible causes of the coexistence of the immunological defect and of the endocrinous deficiencies which are typical of the syndrome. Pathogenetic priority is attributed to the immunological defect which induces a autoimmune lesion of the endocrinous glands concerned.
Assuntos
Doença de Addison/complicações , Candidíase/complicações , Hipotireoidismo/complicações , Doença de Addison/tratamento farmacológico , Doença de Addison/imunologia , Criança , Desoxicorticosterona/uso terapêutico , Feminino , Humanos , Hidrocortisona/uso terapêutico , Hipotireoidismo/tratamento farmacológico , Hipotireoidismo/imunologia , Imunoglobulinas/análise , Masculino , Formação de RosetaRESUMO
A case of cardiac echinococcosis is reported on a 7 year old girl, whose clinical presenting picture was an embolism of the lower limbs. M-mode echocardiography didn't reveal pathological data, whereas two-dimensional (2D) examination in apical position (four-chamber view) diagnosed a cystic structure in the left ventricular free wall. Cardiac surgery confirmed the echocardiographic findings. The necessity to perform all the 2-D sections when there is a clinical suspicion, the possibility to avoid cardiac catheterization and the use of 2-D echocardiography as a screening test in the exposed subjects are emphasized.
Assuntos
Equinococose/diagnóstico , Ecocardiografia/métodos , Cardiopatias/diagnóstico , Criança , Equinococose/patologia , Feminino , Cardiopatias/parasitologia , Cardiopatias/patologia , HumanosRESUMO
Studies have been carried out in insulin-dependent diabetics of porcine Des-pheB1 insulin, which is an insulin analogue obtained by removal of the N-terminal aminoacid of the B chain. The therapeutic activity of Des-phe insulin (regular and semilinte preparations) was tested in a group of 24 insulin-dependent diabetics and compared with the unmodified parent compound. Both types of insulin, Des-phe and unmodified were chromatographically purified preparations. The mean daily blood glucose profile obtained with Des-phe insulin was slightly higher than that of unmodified preparations, while the mean blood glucose and the "M" index of Schlichtkrull were similar. The biological activity of regular Des-phe and its unmodified parent compound was evaluated in 6 further insulin-dependent diabetics, with the aid of an artificial endocrine pancreas. The insulin requirement to achieve an optimal metabolic control was 10% less with Des-phe insulin than with the unmodified preparation. The immunogenicity of Des-phe and unmodified insulins, tested by measuring plasma insulin antibody titres in diabetic patients either newly or already insulin treated, was comparable. However the binding capacity of 125I-Des-phe insulin to preexisting antibodies seemed to be less than that of unmodified 125I-insulin in patients previously treated with unmodified insulin. Finally Des-phe appeared able to correct promptly and completely the insulin-induced lipodystrophy of three insulin-dependent diabetics.
Assuntos
Diabetes Mellitus/tratamento farmacológico , Insulina/análogos & derivados , Insulina/uso terapêutico , Adulto , Idoso , Animais , Glicemia/metabolismo , Ensaios Clínicos como Assunto , Feminino , Humanos , Insulina/efeitos adversos , Insulina/imunologia , Metabolismo dos Lipídeos , Masculino , Pessoa de Meia-Idade , SuínosRESUMO
Three patients with corrected transposition of the great arteries (CTGA) have been studied by means of single plane (M-mode) and two-dimensional (2-D) echocardiography: the first study was performed after surgical closure of a VSD and the remaining two before cardiac catheterization. The following M-mode findings can suggest the diagnosis in CTGA: the interventricular septum (IVS) may not be visualized, there is lack of continuity between the posterior a-v valve (tricuspid) echoes and the anterior great artery (aorta), the posterior a-v valve leaflets may show some abnormalities in shape. The measurement of systolic time intervals of both semilunar valves can help in distinguishing the pulmonary artery from the aorta; further information can be obtained by means of peripheral venous contrast injection, which, in absence of right-to-left shunt, opacifies the posterior great vessel (pulmonary). The 2-D view of the heart allows the identification of the morphological type of ventricle and of the spatial great arteries relationship. The posteriorly and left located ventricle is recognized as morphologically right, because of the tricuspidal shape of its a-v valve: its attachment to IVS is lower than that of the mitral valve (4-chambers view) and three leaflets may be evident (short-axis); 3 papillary muscles and gross trabeculations can be identified (short axis and 4-chambers views, respectively). In long-axis the anterior vessel (aorta) runs parallel to the sternum; in short-axis both vessels are imaged as adjacent circles; by means of peripheral contrast injection, in conditions without right-to-left-shunt, the right and posterior vessel is recognized as a pulmonary artery. The differential diagnosis is discussed with d-transposition, Fallot's tetralogy, Taussig-Bing anomaly, common trunk, univentricular heart. According to our experience, the diagnosis of CTGA and associated defects can be made by M-mode and 2-D echocardiography.
Assuntos
Ecocardiografia/métodos , Transposição dos Grandes Vasos/diagnóstico , Adulto , Criança , Feminino , HumanosRESUMO
BACKGROUND: Three-dimensional transesophageal echocardiography is a new diagnostic tool and its potential has been investigated mainly in international centers dealing with research in the field of cardiac pathologies. The clinical usefulness and the potential additional information over multiplane transesophageal echocardiography in daily clinical practice have not been exstensively studied. OBJECTIVES: This study sought to assess the feasibility and to define the potential role of three-dimensional technique in a clinical cardiology department. POPULATION AND METHODS: One hundred-fifty patients (73 males, 77 females) aged 17-82 underwent a three-dimensional transesophageal echocardiographic study. Indications for the study were the following: 39 mitral (26%), 13 aortic (8%) and 4 tricuspidal (2%) valvulopathies, 23 valvular prostheses (15%), 6 aortic diseases (4%), 16 sources of embolism (10%), 16 congenital heart diseases (10%), 14 ischemic heart diseases (9.3%), 14 cardiomyopathies (9%), 5 other pathologies (3%). The 3 D examination quality was graded as insufficient, sufficient and good. The information obtained by "volume rendered" and "anyplane" three-dimensional echocardiography were compared with the traditional two-dimensional images to determine whether they provided additional information. RESULTS: A total of 288 acquisitions were obtained in the 150 patients (1.9 acquisitions per patient). Examinations were graded of good quality in 99 patients (61%), sufficient in 36 (24%) and insufficient in 15 patients (10%). Additional informations were obtained in 33 patients (22%) by "volume rendered" echocardiography and by "anyplane echocardiography", including mitral regurgitation or repair for valvular prolapse (11 patients), aortic valve malformations and endocarditis (4 patients), congenital heart diseases (9 patients), right ventricular dysplasia (6 patients) or hypertrophic cardiomyopathy (1 patient), tricuspid regurgitation (2 patients). The additional information were obtained in patients in the group of good 3 D reconstructions quality in all but two cases. CONCLUSIONS: The diagnostic use of the transesophageal technique with 3 D facilities permitted to obtain an overall 22% of additional information. These results will stimulate further study to evaluate the advantages of the three-dimensional technique in specific clinical fields of application.