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Biomedical subjects

K Kádár

Publications and source records attributed to K Kádár.

At least 19 recordsLinked to original sources
PubMed2001

Successful treatment of chronic disseminated candidiasis with fluconazole and a granulocyte-macrophage colony-stimulating factor combination.

We report a case of chronic disseminated candidiasis in a patient with acute non-lymphoid leukaemia. After 12 months of combined therapy with fluconazole and granulocyte-macrophage colony-stimulating factor, lesions in the liver and spleen resolved completely. The patient has given birth to a healthy baby and has been in complete hematological remission for 3 years.

Adult↗
PubMed2000

Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract.

We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant. The infant is asymptomatic 18 months after the second surgery. The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combination. The inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology. It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes. To our knowledge only 9 cases have been published of such tumor arising within the heart.

Cardiopulmonary Bypass↗
PubMed1999

[Primary reconstruction of the common truncus arteriosus in infants, using modified pulmonary homograft].

Truncus arteriosus communis (TAC) is a complex heart disease, it presents 1% of the congenital heart defects. A 7 weeks old infant with cardiac failure was operated on with TAC. The bicuspidized (14 mm diameter) pulmonary homograft valved conduit was used to reconstruct the right ventricular outflow tract (RVOT). The sternum closure was delayed. The important points of the indications for the surgical management of TAC: 1. Management or prevention of the pulmonary hypertensive crisis. 2. Patient's age. 3. Reconstruction of the RVOT (choices: homograft, dacron valve conduit, autologous pericardial valve conduit, porcine aortic root, direct anastomosis of the pulmonary trunc to the right ventricle). The surgical management is based on the optimal combination of these three important points. This is the first successfully performed primary surgical repair of an infant with TAC using modified pulmonary homograft in Hungary.

Echocardiography↗
PubMed1998

[Rhabdomyoma as a first manifestation of childhood tuberous sclerosis].

Two dimensional echocardiography seems to be the best diagnostic tool for diagnosis of cardiac tumors. In our practice using 2 dimensional echocardiography in pediatric patients from 1984 11 cardiac tumors were diagnosed and followed-up. Four of them the cardiac rhabdomyomata (one of them diagnosed in utero) was the first manifestation of tuberous sclerosis. We summarized the follow-up data from our patients with cardiac rhabdomyomata. The detection of cardiac tumor in the fetal or infant period is of outmost importance in the early diagnosis of tuberous sclerosis and suggest careful follow-up and management. To the best of our knowledge no report of tuberous sclerosis based on echocardiographic diagnosis of cardiac rhabdomyomata has been described in our country.

Child, Preschool↗
PubMed1997

[Genetic diagnosis of Williams syndrome].

Williams syndrome is a complex developmental disorder. The major cardiovascular component of Williams syndrome is supravalvular aortic stenosis, a progressive disease that may need surgical repair. Williams syndrome is associated with heterozygous microdeletion in the chromosomal region 7q11.23 encompassing the elastin gene. We have identified a new, highly informative tetranucleotide repeat polymorphism within the human elastin gene. This marker together with other, previously described elastin gene markers was used to show deletion of the elastin gene in nine sporadic Williams syndrome patients from Hungary. Application of polymorphisms within and flanking the elastin gene on chromosome 7 provides a fast, polymerase chain reaction based method for mutational analysis of Williams syndrome patients.

Aortic Valve Stenosis↗
PubMed1997

[Successful multi-step management of developmental heart defects after intrauterine diagnosis].

At 28th week of gestation a conotruncal malformation with ventricular septal defect was diagnosed by fetal echocardiography. Postnatal echocardiographic and angiocardiographic examinations confirmed the diagnosis of conotruncal malformation (pulmonary atresia, ventricular septal defect, patent ductus arteriosus, aortopulmonary collateral arteries). The unifocalization (age: 11 months) and total correction with aortic homograft (age: 7 years) were performed. To our knowledge our case is the first whose intrauterine diagnosis of complex congenital heart disease was confirmed after delivery and had successful two-stage surgical management.

Abnormalities, Multiple↗
PubMed1997

[Intraoperative epicardial echography in infancy and childhood for the evaluation of the results of surgical correction of congenital heart defects].

Intraoperative epicardial echocardiography (IEE) is very useful to assess the result of the surgical procedure. In infancy and childhood the authors performed 32 IEE (using high frequency transducer) out of 193 operations with congenital heart diseases. As a result of IEE study the surgical procedure was extended in 5 cases, as follows: closure of patent ductus arteriosus, exchange of transanular patch, repeated mitral repair, prosthetic mitral valve implantation, and correction of residual right to left shunt. No early or late reoperation was necessary in the remaining cases. Follow up echocardiography and autopsy in 4 cases confirmed the diagnosis of the IEE. The results suggest, that IEE will be the standard method for quality control of surgical procedures in congenital heart disease.

Child↗
PubMed1997

Aortico-left ventricular tunnel: late reoperations.

Aortico-left ventricular tunnel is a rare congenital anomaly that presents as aortic regurgitation in infancy or childhood. Information on late results of surgery, especially with reoperations for progressive aortic regurgitation and recurrences are limited. The case histories of two patients with reoperations following correction of aortico-left ventricular tunnel are reported.

Adolescent↗
PubMed1997

Mitral valve anomalies obstructing left ventricular outflow.

This paper reports on two cases of more uncommon types of subaortic stenosis. A 2-year-old boy was found with accessory mitral valve leaflet (AMVL) attaching to the anterior leaflet, ballooning into the subaortic ventricular septum associated with a discrete subaortic membrane. The obstruction was successfully relieved by removal of the AMVL and resection of the membrane. A 19-day-old newborn with accessory tissue on the mitral valve (AMVT) causing subaortic stenosis, subaortic ventricular septal defect (VSD) and patent ductus arteriosus was operated on successfully. Accessory tissue excision through the VSD, VSD patch closure and ductus ligation were performed.

Abnormalities, Multiple↗
PubMed1996

[Total cavo-pulmonary anastomosis in heart surgery for the correction of univentricular heart].

The total cavopulmonary anastomosis is one of the alternative surgical procedures which can be performed in the management of the most complicated congenital heart diseases. It was the first time in Hungary that this surgical management was performed successfully in a girl with univentricular heart, pulmonary valve stenosis, malposition of the great arteries, who was operated on palliative procedure previously.

Abnormalities, Multiple↗
PubMed1995

[Non-invasive and invasive assessment of the function of aortic valve homografts in infancy and childhood].

Study population involved 21 pts with complex congenital heart disease after corrective surgery using homografts between the years 1986 and 1992. Diagnoses included double outlet right ventricle, tetralogy of Fallot, transposition of great arteries, truncus arteriosus, pulmonary atresia with VSD, corrected transposition with pulmonary stenosis, and absent pulmonary valve. Pts age at surgery ranged from 18 days--to 15 yrs mean, 6.7 yrs. Time interval between surgery and diagnostic procedures was 8 days--6 yrs (mean 11 months). All pts were studied by Echo/Doppler. 2 pts by TEE and 3 pts by MRI. 6 pts had cardiac catheterisation. Distal (5) or proximal (1) stenosis was present in 6 cases. The severity and the type of stenosis was correctly identified by noninvasive technique compared to invasive findings. Homograft valve regurgitation was mild (4) or moderate (3). In two pts severe insufficiency was associated to homograft endocarditis. Postoperative residual shunts were found in 8 pts. Homograft stenosis can correctly be diagnosed using Echo/Doppler technique. Distal stenosis was more frequent than proximal. Homograft tends to become insufficient, but severe incompetence did not occur except in endocarditis.

Adolescent↗
PubMed1994

[Fetal and neonatal endocarditis].

Two infants with endocarditis were diagnosed at our Institute within a six month period. One infant died due to severe aortic valve destruction and ruptured sinus Valsalvae aneurysm both of which developed during fetal life. The other one with tricuspid valve endocarditis was successfully treated after the early echocardiographic diagnosis. To our knowledge this patient is the only survivor with neonatal endocarditis in our country.

Endocarditis, Bacterial↗
PubMed1994

[Carnitine-deficient cardiomyopathy].

The case of a 10 month old girl with carnitine deficiency caused neuromuscular symptoms and cardiomyopathy is reported. After oral carnitine therapy dramatic improvement in neurological status and in cardiac function was confirmed. This case is the first infant our country with dilated cardiomyopathy successfully treated by oral L-carnitine.

Administration, Oral↗
PubMed1993

[Giant coronary thrombus as a complication of Kawasaki disease].

We report the case of a 3 yrs old boy with the most serious complication of Kawasaki disease. 2 dimensional echocardiography detected multiple coronary aneurysms and in the giant left coronary aneurysm a thrombus formation. The thrombus disappeared after one week anticoagulant therapy. In addition the aneurysm were proved by coronarography. During the 2 years follow-up period the child is on anticoagulant therapy and he is symptom free. This is the first case in our country with a huge thrombus in a giant coronary aneurysm due to Kawasaki disease who was successfully treated using anticoagulant therapy.

Anticoagulants↗
PubMed1993

[Diagnosis and treatment of fetal and neonatal tachycardia].

Severe intrauterine cardiac failure due to supraventricular tachycardia in a 29 week fetus was diagnosed using 2 dimensional and Doppler echocardiography. The prenatal tachycardia was successfully treated, the postnatal finding suggested Wolff-Parkinson-White syndrome. The fetal echocardiography by providing information on cardiac structure and haemodynamics may provide diagnostic and therapeutic useful informations. For the optimal pre- and perinatal management of this life-threatening condition absolutely essential a brief collaboration between the pediatric cardiologist and obstetricians.

Adult↗
PubMed1991

[Coronary artery anomalies studied by Doppler echocardiography in infancy and childhood--possibilities and limitations].

We investigated infants with Kawasaki disease, congenital coronary artery fistulas and anomalous origin of the left coronary artery from the pulmonary trunc (Bland-White-Garland syndrome) by 2-dimensional and Doppler echocardiography. We describe the systematic approach for visualizing in detail coronary anatomy by two-dimensional echocardiography. Our results suggest that this echocardiographic technique is useful for detecting coronary anomalies and has a great rolbefore angiocardiography especially in sick babies. We recommend performing 2-dimensional echocardiography in the acute and chronic stage of Kawasaki disease for evaluating coronary arterial aneurysms.

Child↗