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Biomedical subjects

I Malcić

Publications and source records attributed to I Malcić.

At least 19 recordsLinked to original sources
PubMed2001

[Juvenile dermatomyositis].

Basic aim of this paper is presentation of probable epidemiological characteristics of juvenile dermatomyositis (JDM) in children of Croatia, due to the fact that severity of condition in majority of patients require treatment in our Department of Pediatrics, KBC Zagreb. Our intention is to present guidelines of current diagnostic approach from recent literature, with special accent on therapy. JDM was previously considered as infaust disease, while current therapy has favourable prognosis for complete resolution, as the only one mesenchymopathy with such prognosis. In period between 1988 and 1999 we treated 18 patients with JDM in our Department of Pediatrics. Thirteen cases were male and 5 female (2.6:1). Thirteen patients had clinical course of isolated JDM (72%), while five had JDM inside overlap syndrome (with other mesenchymopathies) (28%). Average age at time of diagnosis for whole group was 10 years (6-14), for girls 10.6 years (6-14) and boys 8.4 years (7-10). At the present time 5 patients (28%) are considered as cured, 7 patients are in remission with low dosis of steroids (39%) and one girl had relaps of JDM after therapy stopage. Three children die (16%), one girl due to respiratory failure and two girls with pulmonary embolia. Two girls have some signs of JDM with dominant clinical signs of sistemic sclerodermia and one girl has signs of generalized morphea. One boy has dominant muscular calcinosis with contractures of large joints, despite treatment and normal laboratory findings. JDM was not discussed in rheumatic literature in Croatia for more then 15 years.

Adolescent↗
PubMed1997

The limitations of decision trees and automatic learning in real world medical decision making.

The decision tree approach is one of the most common approaches in automatic learning and decision making. The automatic learning of decision trees and their use usually show very good results in various "theoretical" environments. But in real life it is often impossible to find the desired number of representative training objects for various reasons. The lack of possibilities to measure attribute values, high cost and complexity of such measurements, and unavailability of all attributes at the same time are the typical representatives. For this reason we decided to use the decision trees not for their primary task--the decision making--but for outlining the most important attributes. This was possible by using a well-known property of the decision trees--their knowledge representation, which can be easily understood by humans. In a delicate field of medical decision making, we cannot allow ourselves to make any inaccurate decisions and the "tips," provided by the decision trees, can be of a great assistance. Our main interest was to discover a predisposition to two forms of acidosis: the metabolic acidosis and respiratory acidosis, which can both have serious effects on child's health. We decided to construct different decision trees from a set of training objects. Instead of using a test set for evaluation of a decision tree, we asked medical experts to take a closer look at the generated trees. They examined and evaluated the decision trees branch by branch. Their comments show that trees generated from the available training set mainly have surprisingly good branches, but on the other hand, for some, no medical explanation could be found.

Acidosis↗
PubMed1996

[Polyarteritis nodosa--cutaneous or systemic form? Possible role of bacterial superantigens in the onset of systemic disease].

A boy with diagnostically unclear vasculitis was described, in which development of the disease points out the presence of cutaneous form of poliarteritis nodosa. Nevertheless, there are also signs for systemic form of the disease. His symptoms are marked cutaneous eruptions of the livedo reticularis type, recidives of erythema nodosum on the limbs and trunk, even on the cheeks, sometimes accompanied with fever, arthralgias, myalgias and cutaneous ulcera on the places of mechanical pressure (elbows), but also with monotopic ventricular premature beats and sporadic microhaematuria. Clinical development of the disease and differential diagnosis exclude other autoimmune disorders with great probability. The positive finding of cANCA has pointed out the diagnosis of vasculitis. Microscopical analysis of the skin didn't reveal any pathological changes. In the boy's disease is interposed a streptococcal infection, and maybe, tuberculosis. The paper deals with wide spectrum of diseases in differential diagnosis, and also with a possible role of bacterial superantigens in the genesis of autoimmunity.

Child↗
PubMed1995

[Incipient pericardial tamponade as the first symptom of systemic lupus erythematosus in 2 children].

Two patients (a boy and a girl), with systemic lupus erythematosus (SLE), in which pericarditis with threatening tamponade was an initial symptom of disease, are presented. Pericardial tamponade is very rare initial manifestation, described in only 1-3% of all the cases of SLE. The typical clinical features of tamponade are described (tachycardia, hypotension, venous congestion) and the importance of echocardiography in early diagnosis of pericarditis and pericardial tamponade is pointed out. Therefore, the echocardiography is considered as the complementary method in diagnosis of SLE.

Cardiac Tamponade↗
PubMed1994

Decision trees based on automatic learning and their use in cardiology.

Computerized information systems, especially decision support systems, have become an increasingly important role in medical applications, particularly in those where important decision must be made effectively and reliably. But the possibility of using computers in medical decision making is limited by many difficulties, including the complexity of conventional computer languages, methodologies and tools. Thus a conceptual simple decision making model with the possibility of automating learning should be used. In this paper we introduce a cardiological knowledge-based system based on the decision tree approach supporting the mitral valve prolapse determination. Prolapse is defined as the displacement of a bodily part from its normal position. The term mitral valve prolaps (PMV), therefore, implies that the mitral leaflets are displaced relative to some structure, generally taken to the mitral annulus. The implications of the PMV are the following: disturbed normal laminar blood flow, turbulence of the blood flow, injury of the chordae tendinae, the possibility of thrombus's composition, bacterial endocarditis, and finally hemodynamic changes defined as mitral insufficiency and mitral regurgitation. Uncertainty persists about how it should be diagnosed and about its clinical importance. It is our deep belief that the echocardiography enables properly trained experts armed with proper criteria to evaluate PMV almost 100%. But unfortunately, there are some problems concerned with the use of echocardiography. In that manner we have decided to start a research project aimed at finding new criteria and enabling the general practitioner to evaluate PMV using conventional methods and to select potential patients from the general population.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Mitochondrial cardiomyopathy and scapuloperoneal spinal muscular atrophy in a child.

A 14-year old boy was admitted for signs of heart failure and scapuloperoneal muscle weakness. He fulfilled the clinical, functional and diagnostic criteria for dilated cardiomyopathy. There was also a moderate increase in pulmonary vascular resistance. The immunohistochemical examination of the heart muscle revealed a slightly positive phytohemagglutinin reaction and minimal IgM deposits without complement. The electron microscopy examination disclosed increased numbers of abnormal mitochondria disrupting the usual cell structure; the mitochondria were of various sizes with irregular and abnormal structure of the cristae. The scapuloperoneal spinal muscular atrophy was mild and diagnosed according to clinical and electromyographic findings. Light microscope examination of the skeletal muscle revealed hypotrophic fibers. This patient is presumed to have postinflammatory mitochondriopathy and is currently being managed on low-dose digitalis, diuretics and captopril.

Adolescent↗
PubMed1992

Cor triatriatum dextrum.

Cor triatriatum dextrum was an incidental echocardiographic finding in a 5 year old boy and a 9 year old girl, who were evaluated for the presence of a heart murmur. Apart from slightly enlarged right atria, they had otherwise normal hearts and were symptomless. Most of the patients reported until then had been symptomatic due either to the persistent right sinus venosus valve or to commonly associated structural heart anomalies. Therefore, the clinical significance of asymptomatic cor triatriatum dextrum remains coniectural, but might lie in the possibility of development of arrhythmia, progressively worsening interference with the systemic venous return and thrombus formation.

Child↗
PubMed1991

Cardiomyopathies in children with neuromuscular disorders.

In this paper eight patients with myogenic or neurogenic muscle disorders are presented, in whom cardiomiopathy was also found. Six patients developed a dilated cardiomiopathy associated with neurogenic atrophies or progressive muscular dystrophy. In patients with Mb. Friedreich and HSNM type II together with the total dilatation of the septum hypertrophy was observed and in patients with spinal muscular amyotrophy of scapuloperoneal type atriomyopathy dominated. In two patients with mitochondrial disorders a hypertrophic cardiomyopathy was found. One of them had mitochondrial encephalomyoneuropathy and the other mitochondrial encephalopathy with myoclonic epilepsy. In none of them a restrictive cardiomyopathy was found. From the presentation could be concluded that in neurogenic muscle diseases and progressive muscular dystrophy respectively most frequently dilated cardiomyopathies have been developed. Hypertrophic cardiomyopathies are usually found in children with mitochondrial disorders.

Adolescent↗
PubMed1985

Verapamil in primary pulmonary hypertension.

An 8 year old girl with primary pulmonary hypertension and signs of heart failure was given 17.5 mg of verapamil injected into the pulmonary artery over 25 minutes to test for the reversibility of pulmonary arteriolar obstruction. Pulmonary artery pressure fell from 70/50 to 35/25 mm Hg. Treatment with 40 mg verapamil by mouth four times daily was started, and she was discharged. She gradually became asymptomatic and resumed the activities of a normal child of school age. On recatheterisation seven months later her pulmonary artery pressure was 30/10 mm Hg. She was still doing well 12 months after the start of treatment.

Blood Pressure↗
PubMed1979

[Pelger-Huet anomaly].

A homozygot carrier of a congenital nuclear anomaly of blood cells/Pelger-Huet's anomaly: abnormal segmentation of granulocytic leukocytes) is described. The morphologic analysis of blood samples in 4 family members showed similar Pelger-Huet's anomalies. The phagocytic activity, the "killing test" and the inhibition test of leucocyte migration of the patients neutrophils were in normal ranges.

Child↗
PubMedDate not supplied

Radioangioscintigraphy and Doppler echocardiography in the quantification of left-to-right shunt.

The magnitude of left-to-right shunt in 55 children with isolated congenital heart disease [atrial septal defect (ASD) or ventricular septal defect (VSD) (muscular and perimembranous)] was estimated by two methods: radionuclide quantification and Doppler echocardiography [flow (L/min) = mean velocity x area x ejection time x heart rate]. We found little difference between the magnitude of left-to-right shunt obtained with Doppler echocardiography and that with radioangioscintigraphy for a whole group of patients (N = 55, -11.42% to 12.04%) and for subgroups of ASD (n = 24, -12.49% to 12.19%) and VSD (n = 31, -10.69% to 12.23%). These results indicate that Doppler echocardiography, in comparison with radioangioscintigraphy, is sufficiently accurate for clinical estimation of the Q(p)/Q(s) ratio in isolated congenital heart disease with left-to-right shunt.

Adolescent↗
PubMedDate not supplied

Intracardiac juvenile xanthogranuloma in a newborn.

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.

Heart Diseases↗
PubMedDate not supplied

[Myocarditis and dilated cardiomyopathy in children. Old questions-- new answers].

The purpose of this article is to discuss the dilemmas in differential diagnosis of myocarditis and dilated cardiomyopathy of different etiologies. According to the definition of the WHO and the ISFC the dilated cardiomyopathy is a myocardial disease recognized by the severe enlargement of the left and/or the right ventricle causing the decrease in systolic function of the heart (decrease of contractility) with the development of congestive heart failure. In order to distinguish primary cardiomyopathies defined as a muscle heart disease sui generis on the one hand from cardiomyopathies as the outcome of chronic inflammatory myocarditis (chronic persistent myocarditis, chronic immune myocarditis, chronic viral heart disease) on the other hand, it is necessary to follow a complicated diagnostic flow diagram. Methods that are to become routine in diagnostic procedure are described. Etiologic diagnosis of dilated cardiomyopathies is not possible without heart muscle biopsy. The bioptic specimens must be analyzed using light microscope (according to Dallas criteria), electron microscope, and afterwards immunohistologically and immunohistochemically (in situ hybridization). The total result of these investigations leads to the final conclusion about etiological diagnosis of dilated cardiomyopathy. The directions in the treatment of dilated cardiomyopathies and acute myocarditis are stated, as well as the importance of immunosuppressive therapy in these conditions. In the treatment of acute myocarditis digoxin should be avoided and other inotropic substances used instead.

Cardiomyopathy, Dilated↗