[Hypophosphatemic vitamin D resistant rickets. Therapeutic management].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to V Popescu.
Explore the source record for details and available documents.
The paper reports on the development of the diagnosis algorithm in jaundices with conjugated bilirubinemia in children. The clinical hepatobiliary semeiology, the data of the objective clinical examination and the laboratory and paraclinical examinations of hepatic exploration are analyzed. The paper also presents the diagnosis phases in the main clinical biological syndromes met in the child with jaundice and conjugated bilirubinemia: acute hepatic syndrome, chronic hepatic syndrome and cholestatic syndrome.
Syndrome of Neonatal hepatitis and atresia of extrahepatic bile ducts are the most frequent causes of jaundice with conjugated bilirubin in the small infant. The neonatal hepatitis is diagnosed by its plurietiologic character, onset in the first 3 months, subacute or chronic, potentially cirrhogenous, evolution, conjugated hyperbilirubinemia and mainly, gigantocellular hepatic transformation--the essential characteristic of the diagnosis. Etiology of neonatal hepatitis has extremely diverse causes: infectious causes, genetic diseases of metabolism, toxic causes, post-hemolytic states, neonatal acute hepatic necroses, parenteral nutrition, chromosomal anomalies, familial syndromes, etc.; there exists also a form with nonspecific cause (idiopathic form). Practically, neonatal hepatitis might often be mistaken for atresia of the extrahepatic bile ducts. In the latter case, the temporization of the surgery (bilidigestive anastomosis) for more than 2 months of cholestasis evolution leads to the appearance of hepatic cirrhosis lesions. The authors analyze various clinical, biological and histopathological elements which permit the differentiation in due time of the two situations that require different therapeutic attitudes. Except for certain situations, which allow an etiologic treatment, the main therapeutic element (pathogenic) is corticotherapy and several additional measures. The paper concludes with appreciations on the evolution, prognosis and prophylaxis possibilities.
The authors approach a notion of great present day interest--namely risk factors determining infantile morbidity and mortality. They review the methods for study and identification of risk factors and analyse the relative frequency and importance depending on the maternal-infantile biosystem and exogenous, environmental factors, showing the strategy of the control (reduction) of infantile mortality based upon the identification and knowledge of risk factors. The study is of practical value allowing pediatricians at all levels of the maternal-infantile network to perfect medical surveillance of the population at risk and to plan differentiated individual care for infants and children whose health status exposes them especially to risk.
An analysis and updating is made, of the main supraventricular heart rhythm disturbances encountered in children, excepting paroxysmal supraventricular tachycardia, which remains the major clinical entity in this field, but was presented by the same authors in a previous issue of this journal. A review is made of vasovagal syncope, of sinusal tachy- and bradycardia, of atrial extrasystolic arrhythmia, of the sinus node disease, of atrial flutter, and of atrial fibrillation. On the basis of modern studies in the field of electrophysiology, including endocavitary electrophysiology, the major mechanisms are presented, involved in triggering all types of heart tachyarrhythmias, namely: abnormal automatism (automatic ectopic focuses), the "re-entry mechanism" and the "triggered dysrhythmia". Particular attention is given to extrasystolic arrhythmias of atrial origin, and to the sinus node disease, of which the first is important because of its frequency while the second one is remarkable because of the considerable mass of new data that have been published in the literature. A review is made of etiologic aspects, as well as clinical, electrocardiographic, and therapeutical data related to heart rhythm disturbances of supraventricular origin, as well as a series of considerations in relation to evolution and prognosis. The authors stress certain difficulties of diagnosis, especially with electrocardiograms, as is the case, for instance, with blocked bigeminal atrial extrasystoles, with the sinus node disease, etc. In the final part of the paper two more rarely encountered disturbances are presented; atrial fibrillation and atrial flutter. These two entities are also defined on the basis of clinical and electrocardiographic data, and their analysis is also made from the viewpoint of their etiology. A discussion is made concerning the therapeutic aspects, and their evolution and prognosis.
A comparison was made between the results of an analysis of humoral immunological changes in 64 cases of inflammatory diseases of the connective tissue of children (32 cases of ACJ, 5 cases of systemic lupus erythematosus, 2 cases of polymyositis, and 25 cases of systemic vasculitis), and data reported in recent literature, the authors conclude as follows:--changes of the immunological parameters that were tested support the hypothesis of a humoral immunopathogenic mechanism in systemic lupus erythematosus, in some of the manifestations of ACJ, and in systemic vasculitis;--of particular value for the immunological diagnosis of ACJ is the detection of FR in the patients' serum (and especially of FR from the IgG and the IgA class), as well as changes of immunological parameters of the articular fluid (the presence of FR, of immune complexes, a reduction of the titer intra-articular complement, the presence of ragocytes);--demonstration of humoral immunological changes (polyclonal hypergammaglobulinemia, the presence in the serum of circulating immune complexes, and of cryoglobulins, the low titer of serum complement in active stages, the presence of antinuclear antibodies, and especially of antinative DNA, the presence of LE cells in peripheral blood, and of a varied range of autoantibodies) is mandatory for the diagnosis of systemic lupus erythematosus;--in the present stage of our knowledge humoral immunologic changes are not an absolutely certified criterion for the diagnosis of polymyositis, and of some cases of systemic vasculitis (Henoch-Schoenlein purpura).
This study was prepared on the basis of the recent data published in the specialized literature, as well as on the personal experience of the authors, and is considered as a necessary updating in the field of heart rhythm disturbances which occur in the child. A review is presented of sinoatrial blocks, of atrioventricular conduction disturbances, and of intraventricular conduction abnormalities (branch blocks). A modern classification is used in the discussion of sinoatrial, and of atrioventricular blocks, with the three distinctive degrees (first degree block, second degree block--with type I and type II variants, and the third degree block). The authors have attempted, in their effort to present the data concerning each of the entities, to include a series of anatomical and electrophysiological notions, some of which represent modern acquisitions obtained by endocavitary electrocardiographic exploration, and which are considered to be essential for a good understanding of the material. An important place is given--for each of the entities discussed--to the etiological study, as well as to clinical semiology, and electrocardiography, to problems related to the positive and differential diagnosis, and to therapeutical means, as well as to evolution and prognosis. The study is concluded by a presentation of intraventricular conduction disturbances (branch blocks) which are evaluated in their distinct variants: the monofascicular block (single bundle block), the bi- and trifascicular bundle blocks, major or minor blocks, and functional rate-dependent blocks. In these cases also the authors have analysed etiologic aspects, the clinical picture, and the electrocardiographic aspects, as well as therapeutical, evolutive and prognostic features for each of the different forms.
The present study, based on the recent data in the literature and the authors' own experience, is a complex and up-to-date analysis of the three entities closely related, forming the "ventricular dysrhythmias": ventricular extrasystoles, ventricular paroxysmal tachycardia and ventricular fibrillation. For the ventricular extrasystoles--the most frequently met of these anomalies--the authors present the etiologic context (metabolic disturbances, drugs, myocardial lesions) and then analyze at length the ECG aspects (complex premature QRS, with modified morphology, enlarged, not preceded by P waves, etc.). The importance of studying the "coupling interval", usually fixed in the child, on the ECG tracing, is underlined. This shows, together with the monomorphous aspect of the extrasystolic QRS complexes, the presence of a single ectopic focus, and indicates a benign situation, making useless, in most of these cases, some specific treatment measures. The possible diagnosis errors (atrial or junction extrasystoles mainly with aberrant ventricular leads) are also discussed. After presenting the etiopathogenic data of the ventricular paroxysmal tachycardia the authors show that it is a rate rhythm disturbance in paediatrics (3% of the ectopic tachycardias), and may appear both on a normal heart (about 25% of the cases), and mainly in the presence of some preexisting cardiac lesions. The paper reports on the clinical aspects (partly, similar to those in the supraventricular paroxysmal tachycardia, but more severe) and especially on the aspects of electrocardiographic diagnosis. The necessity of differentiating it from other tachycardias with enlarged QRS complexes (mainly atrial and junction tachycardias with aberrant ventricular leads) is emphasized. The ventricular fibrillation, although rare in children (6% of the forms of terminal electrical activity), is the most severe disturbance of cardiac rhythm, making a clinical picture of "heart rest", that requires a maximum therapeutic emergency. The paper concludes with short references on a ventricular arrhythmia, very rarely met in children, the peak torsade, a clinical disease of syncopal type, electrocardiographically intermediary between paroxysmal tachycardia and ventricular fibrillation.
Genetic cardiac dysrhythmias, although rare in pediatrics, may be often the first manifestation of the main disease or may determine the vital prognosis in some of these diseases (e. g. Friedreich's ataxia and Duchenne's progressive muscular dystrophy). This is more so, as at present, technical possibilities of implanting pacemakers, at ever smaller ages, are ever so great. The first part of the paper deals with these aspects, reviews, and analyses a large variety of genotypic diseases (metabolism genetic diseases, cardio-skeletal syndromes, neuromuscular diseases, cardio-auditory syndromes, etc.) and also a series of tachydysrhythmias or isolated familial conduction disturbances (blocks). Within each entity, the authors describe the main elements allowing the diagnosis, with special references on the rhythm and cardiac conduction disturbances that may appear, and with the necessary therapeutical considerations. The second part of the material presented is devoted to the cardiac dysrhythmias following the surgical intervention on heart, frequently used at present in the congenital heart diseases, at ever smaller ages due to the special progress in anesthesia and intensive care techniques. Correlation of various types of surgeries with the anatomical lesions of the excitation-conduction system they can involve, makes easier the understanding of the nature and appearance risks of some postsurgical rhythm and cardiac conduction disturbances. The distinction is made between early postsurgical dysrhythmias--that are often episodic--and tardy dysrhythmias that may relapse or become chronic, thus raising difficult treatment problems and sometimes require a secondary pacemaker implantation, given the major handicap they represent for the child.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.