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Biomedical subjects

A Lindinger

Publications and source records attributed to A Lindinger.

At least 19 recordsLinked to original sources
PubMed2005

Reference values of NT-proBNP serum concentrations in the umbilical cord blood and in healthy neonates and children.

BACKGROUND: N-terminal brain natriuretic peptide (NT-proBNP) is a neurohormonal substance secreted mainly by the cardiac myocytes of the left ventricle and to a less degree of the left atrium. The releasing mechanism is induced by an increased wall stress on the base of volume or pressure load of the ventricle. The physiologic actions of BNP are prohibition of the sympathetic activity, vasodilatation, natriuresis, diureses and inhibition of the renin-angiotensin system. Because of its high sensitivity and specifity in relation to the left ventricular incompetence, BNP as well as NTproBNP are well accepted markers of ventricular dysfunction. It was the aim of the study to establish reference values of NT-proBNP serum concentrations throughout childhood, in particular in the newborn age group. METHODS: In a cross sectional study, serum NT-proBNP concentrations were measured by an ElectroChemiLuminescenceImmunoAssay ("ECLIA" Roche) in the umbilical cord blood of 62 healthy full-term neonates and in 222 healthy probands from birth up to the age of 18 years. RESULTS: The concentration of NT-proBNP in the cord blood samples ranged from 281 to 2595 pg/ml (mean: 818 pg/ml). There was a significant increase in the first days of life followed by a rapid decrease during the first year and a further gradual decrease throughout infancy; beyond the 10(th) year of age normal adult values were approached. CONCLUSIONS: The knowledge of the normal range of plasma NT-proBNP levels in healthy subjects during childhood serves as a non-invasive marker for differentiation of pathologic conditions such as acute myocarditis, hypertrophic or dilated cardiomyopathy, tachyarrhythmias and pulmonary hypertension.

Adolescent↗
PubMed2005

A family exhibiting arterial tortuosity syndrome displays homozygosity for markers in the arterial tortuosity locus at chromosome 20q13.

Arterial tortuosity associated with hyperextensible skin and hypermobility of joints, features that are characteristics of Ehlers-Danlos syndrome (EDS), has been described in several families. An arterial tortuosity locus has recently been mapped to chromosome 20q13. Here, we report a consanguineous Kurdish family in which an affected child manifested elongation and severe tortuosity of the aorta, carotid, and other arteries. Additional clinical symptoms include loose skin, hypermobile joints, hernias, and facial features that resemble EDS individuals. To examine whether the arterial tortuosity locus was involved in this child, homozygosity analysis was performed using microsatellite markers on 20q13. The affected child was found homozygous, whereas the unaffected parents and three siblings were heterozygous. Additional typing defined the genomic interval to a 37-cm region within which the arterial tortuosity locus is located. Three functional candidate genes (B4GALT5, KCNB1, and PTGIS) were sequenced. No mutations were discovered in the coding regions of these three genes and the promoter regions of B4GALT5 and KCNB1 genes. Moreover, the B4GALT5 mRNA expression was unaltered in patient-derived lymphoblastoid cells. In the PTGIS gene promoter, the affected child was homozygous for eight variable number of tandem repeats, while parents and unaffected siblings carried six repeats.

Angiography↗
PubMed2004

The effects of isotope substitution and nuclear spin modifications on the spectra of complexes of tetracene with hydrogen molecules in ultracold 0.37 K He droplets.

The van der Waals complexes consisting of single tetracene chromophore molecules with an attached H(2), HD, or a D(2) molecule have been assembled inside cold (0.37 K), large ( approximately 1.5 x 10(4) atoms) helium droplets. Their laser-induced fluorescence spectra exhibit typically three well isolated fairly sharp [deltanu(full width at half maximum) approximately 0.5 cm(-1)] bands in the spectral region 22220-22300 cm(-1). Their positions differ for each isotopomer and also are different for each of the ortho- and para-spin modifications. The common feature (except for D(2)) with the largest redshift at about 30 cm(-1), found also in other related free complexes, is attributed to a strongly bound site above one of the two central benzene rings. The other major features come in pairs spaced 3 cm(-1) apart and are not found in similar gas phase studies. This doublet is assigned to a less tightly bound peripheral site with either slightly different configurations or states of the aduct or possibly the He atoms which are stabilized by the surrounding helium bath. The common feature and one branch of the doublet exhibit a pronounced narrow fine structure with spacings of only 0.1 cm(-1), which is nearly the same for all complexes as well as for the bare chromophore, and maybe be due to partially resolved rotational structure of the bands.

Journal Article↗
PubMed2000

Safety and efficacy of interventional occlusion of patent ductus arteriosus with detachable coils: a multicentre experience.

UNLABELLED: In order to define the safety and efficacy of Cook detachable coils for interventional closure of patent ductus arteriosus, we performed a retrospective analysis of all patients who underwent cardiac catheterization in seven centres for intended interventional occlusion of patent arterial duct. From January 1995 until March 1998, cardiac catheterization for intended interventional occlusion of patent arterial duct was performed in 317 consecutive children. Successful placement of at least one coil was achieved in 282 children (89%). The mean diameter of the ductus in children treated with Cook detachable coils was 1.65 mm, mean fluoroscopy time was 10.7 min. Occlusion rates were 62% 10 min after the procedure, 82% at the time of discharge, 91% at 4 months and 95% at late follow-up (2 years). In children with a ductus diameter of < or = 2.5 mm the rate of successful coil deployment was 94% with a 98% occlusion rate at late follow-up. Complications occurred in 11 procedures (3.5%) including haemolysis (3 patients), embolization of a coil to the pulmonary artery (7 patients, 2.3%) and inability to release a coil (1 patient). CONCLUSION: In our opinion, Cook detachable coils are safe and effective especially in the treatment of persistent ductus arteriosus with a diameter < or = 2.5 mm. Due to the low costs these coils appear to be superior to other devices in this subgroup of patients.

Adolescent↗
PubMed1999

Double-switch operation for congenitally corrected transposition and Ebstein's malformation.

An infant is described with congenitally corrected transposition and Ebstein's malformation. Banding of the pulmonary trunk had been previously performed because of a muscular ventricular septal defect. The patient underwent the double-switch procedure with the intention of unloading the morphologically right ventricle and the malformed tricuspid valve. This resulted in prompt postoperative functional and haemodynamic improvement.

Cardiac Surgical Procedures↗
PubMed1998

Permanent junctional re-entry tachycardia. A multicentre long-term follow-up study in infants, children and young adults.

AIMS: Permanent junctional re-entry tachycardia is a relatively uncommon form of re-entry tachycardia with antegrade conduction occurring through the atrioventricular node and retrograde conduction over an accessory pathway usually located in the postero-septal region. It was the aim of the study to investigate the course of permanent junctional re-entry tachycardia with particular regard to the effectiveness of pharmacological treatment and ablation procedures; evaluation was performed with respect to the patient's symptoms, tachycardia rate, frequency of the tachycardia and left ventricular function. METHODS AND RESULTS: The long-term follow-up of 32 patients with permanent junctional re-entry tachycardia was evaluated. The first presentation with supraventricular tachycardia occurred between the 27th week of gestation and 27 years. The tachycardia rate ranged from 100 to 250 beats.min-1. During Holter-ECG, permanent junctional re-entry tachycardia was documented as present for over 50% of the time in 24 h in 22 patients (69%). Left ventricular performance was impaired in nine patients (28%) due to a tachycardia-related cardiomyopathy. Symptoms or signs of heart failure were mild to moderate in eight and severe in four patients; 20 patients showed no clinical impairment. Follow-up time was 1 to 31 (mean 10) years; current age of the patients ranged from 1.5 months to 35 (mean = 15 x 3) years. Four patients needed no therapy because of the infrequency of permanent junctional re-entry tachycardia episodes. Twenty-five patients initially received antiarrhythmic drugs, which were effective or partially effective in 14 (56%). Eight of them are still on medical therapy; in five treatment was discontinued because of absence of symptoms. Eleven patients had ablation of the accessory pathway during follow-up, three underwent ablation as a primary procedure. CONCLUSION: Permanent junctional re-entry tachycardia in our experience is an arrhythmia with a large variety of clinical symptoms. Patients with a slow tachycardia rate and infrequent episodes of tachycardia may never develop symptoms and therefore do not need any therapy. Patients with frequent permanent junctional re-entry tachycardia, a fast tachycardia rate and impaired left ventricular function need effective therapy. In infancy and early childhood medical therapy is recommended as a first option, whereas in older and symptomatic patients catheter ablation is an effective and safe procedure.

Adolescent↗
PubMed1997

Progressive hydrocephalus in two members of a family with autosomal dominant Noonan phenotype.

We present a family with an autosomal dominant phenotype characterized by pulmonary valve stenosis, craniofacial dysplasia with marked hypertelorism and, as a variable feature, progressive hydrocephalus. This phenotype is manifested in four patients from three subsequent generations of a family, with expression ranging from mild pulmonary stenosis combined with slight but characteristic facial dysmorphism to severe cyanotic pulmonary valve stenosis with multiple malformations lethal in early childhood. We consider this to be an unusual variant of Noonan syndrome.

Adult↗
PubMed1995

Ambulatory blood pressure monitoring in healthy schoolchildren.

Ambulatory blood pressure monitoring (ABPM) was performed in 564 healthy schoolchildren during normal circadian activities. The data of two cohorts (155 boys and 139 girls aged 9-13 years and 184 boys and 168 girls with a body height between 120 and 155 cm) are presented. From the age of 9 to 13 years the mean 24-h systolic/diastolic blood pressure (SBP/DBP) increases from 107 +/- 9/66 +/- 7 mmHg to 115 +/- 13/68 +/- 9 mmHg in boys and from 104 +/- 5/64 +/- 6 mmHg to 109 +/- 8/65 +/- 9 mmHg in girls. When related to body height the values rise from 105 +/- 6/64 +/- 6 mmHg at 120 cm to 113 +/- 8/67 +/- 7 mmHg at 155 cm in boys and from 100 +/- 7/65 +/- 7 mmHg to 112 +/- 9/66 +/- 9 mmHg in girls. In comparison with the causal blood pressure data obtained from European studies, the presented ABPM values (daytime BP) are higher throughout, which may be explained by the increased activity during daytime with ABPM. There is a mean difference of 4.4 mmHg in boys and of 3.0 mmHg in girls for SBP and of 10.8 mmHg in boys and of 9.0 mmHg in girls for DBP when related to age. In relation to body height, there is a mean difference of 4.4 mmHg in boys and of 3.5 mmHg in girls for SBP and of 10.9 mmHg in boys and of 10.5 mmHg in girls for DBP. We conclude that standards derived from causal blood pressure measurements should not be used for the evaluation of ABPM data.

Adolescent↗
PubMed1992

Atrial automatic tachycardia in infancy and childhood.

Twenty-one cases (13 male, eight female) of atrial automatic tachycardia (AAT) assessed by standard and Holter-ECG in otherwise healthy infants and children have been documented. AAT was incessant in 12 patients, repetitive in seven, and of undetermined type in the remaining two. The frontal P wave axis suggested an ectopic focus in the high right atrium or right atrial appendage in 13 patients, in the low right atrium in one patient, and in the left atrium in seven patients. Thirteen out of 14 patients with the incessant or undetermined type of AAT were symptomatic, in contrast to only two of seven patients with the repetitive type. All patients were treated with between one and eight (median three) antiarrhythmic drugs. The most effective drug was amiodarone, followed by the class I C antiarrhythmic drugs, propafenone and flecainide. At present, all patients are alive 4 months to 21 years (median 2.5 years) after diagnosis of AAT. Twelve patients are in sinus rhythm, five of them without any medication. Nine patients still have AAT, which, however, is repetitive or intermittent in all but one. In conclusion, AAT is an unusual, and in its incessant form often severely symptomatic arrhythmia, which is resistant to conventional antiarrhythmic medication. However, amiodarone and class I C antiarrhythmic drugs are frequently effective. Since medical treatment with these drugs is often successful, and AAT may resolve completely, a conservative approach is indicated in many cases.

Adolescent↗
PubMed1989

Balloon valvuloplasty of pulmonary stenosis in infants and children--co-operative study of the German Society of Pediatric Cardiology.

Balloon valvuloplasty (BVP) of pulmonary stenosis (PST) was performed in 305 infants and children in 20 heart centres of the Federal Republic of Germany. 273 patients suffered from isolated PST. Their age ranged from 3 days to 18 years (mean = 5.85 +/- 4.82 years), their weight from 2.9 to 60 kg. The pressure gradient was measured invasively in the sedated or anaesthetized child, partly by CW Doppler. BVP was performed by mono- or trefoil catheters, mostly oversized. As a result, mean pressure gradient fell from 71.7 +/- 32 mmHg before to 31.8 +/- 25 mmHg immediately after BVP (P less than 0.0001). The gradient was reduced by less than 30% in 13% of the patients, by 30-49% in 23%, and by greater than or equal to 50% in 64%. Among 33 patients with a gradient reduction of less than 30%, nine were aged less than 1 year, nine had dysplastic valves, and nine had a short-term infundibular reaction and good late results. Gradient reduction was not improved with oversized balloons and was independent of the initial gradient. Follow-up for a mean 11.2 +/- 8 months showed a further slight improvement, probably due to regression of infundibular hypertrophy. In the whole group we observed infundibular reaction in 12%, serious complications with one late death in 4%. From the start, BVP proved to be a secure and effective tool for elimination of pulmonary stenosis.

Adolescent↗
PubMed1988

Emery-Dreifuss muscular dystrophy: disease spectrum and differential diagnosis.

We report six patients with Emery-Dreifuss muscular dystrophy (EDMD) and four patients including one female with EDMD phenotype (EDMDP). This series includes one sporadic case who had previously been reported in this journal under the diagnosis of "rigid spine syndrome" in 1977. Time of observation ranged from three to ten years. Detailed cardiological assessment was performed in all patients, skeletal muscle biopsies were obtained from 9 out of 10 and cardiac muscle biopsies from 2 out of 10 patients. One patient showed evidence of cardiomyopathy in the absence of clinically apparent neuromuscular disease and one sibling of another EDMD patient reportedly had a similar combination of symptoms which, to our knowledge, has not yet been reported. Cardiac involvement was found to consist of four independent, albeit often combined features: 1) impairment of impulse generating cells; 2) conduction defects with atrial preponderance; 3) increased atrial and ventricular heterotopia; and 4) functional impairment of ventricular myocardium. Ventricular involvement as apparent from ventricular heterotopia, abnormal enddiastolic diameter, decrease of contractility and/or morphological evidence of ventricular myocardial disease was found in 7 out of 10 patients and confirmed by myocardial histopathology in two EDMD patients. In one myocardial biopsy extensive accumulations of intermediate filaments were observed, a rare finding, which has not been linked to EDMD before. Skeletal muscle biopsies showed evidence of myopathy throughout but several equivocal features such as fibre type grouping in EDMD and fibre type disproportion in EDMDP were also observed. The variability of clinical manifestation of both cardiac and neuromuscular disease encompassed a broader spectrum than apparent from the literature. The consequences for the inherent differential diagnosis are discussed.

Adolescent↗
PubMed1986

Multiple extra-adrenal and thoracic pheochromocytomas.

On the basis of a case of multiple, extra-adrenal intrathoracic pheochromocytomas (total of 6 tumors), the value of dynamic computerized tomography investigations, venous sampling, scintigrams with 131I-meta iodobenzylguanide, and selective arteriography is discussed.

Adolescent↗
PubMed1984

[Long-term ECG findings in children with healthy hearts].

Ambulatory monitoring of the ecg was performed in 100 healthy children aged between 6 and 16 years for the period of 24 hours. On the same day all children studied had normal standard 12 lead ecg. The maximal heart rates ranged from 96 to 176 beats and minimal rates were 36 to 79 beats per minute. Sinus arrhythmia, what we called variation of frequency, was found very often (55 per cent). A wandering atrial pacemaker occurred in 47 per cent, while true intermitted junctional rhythm (duration more than 3 beats) was seen in 20 per cent. Premature atrial beats we have seen in 11 per cent, ventricular extrasystoles occurred in 20 per cent. Sinus bradycardias were found nearly almost during sleep with a rate under 40 beats per minute in one child and under 60 beats per minute in 49 per cent. We could not find sinuatrial blocks at all. AV-Blocks I0 and II0 were seen in two children, respectively. There was a clear advantage in the diagnostic of arrhythmias with using the 24-ecg-ambulatory monitoring in comparing with the standard ecg. Further advantage of this method is it's non invasive character.

Adolescent↗
PubMed1981

[Septo-optical dysplasia: oligosymptomatic form (author's transl)].

It is reported on a nine year old boy with the oligosymptomatic form of the septo-optical dysplasia de Morsier: congenital nystagmus with nearly complete loss of visus due to a hypoplasia of the opticus-nerve of both sides, growth retardation (dwarfism) because of STH-deficiency and lack of the gonadotropins. The septum pellucidum is present. The cause of this embryologic malformation within the prosencephalon is not known.

Abnormalities, Multiple↗
PubMed1979

[Hypothyreotic connatal struma after amniofetography (author's transl)].

It is reported on a newborn suffering from a pronounced hypothyreosis and a big goitre caused by an amniofetography during the 22. week of pregnancy using a hydrous and a oily iodiferous drug. The big goitre impeded the respiration, so that intubation was indicated. After treatment with thyroid hormones for 8 months the goitre decreased. The thyroid hormone-levels and function tests were in normal range after interruption of the thyroid drugs. Etiology and pathogenesis of this disease are discussed. It is warned of using drugs containing high iodine doses for amnio- or fetography.

Contrast Media↗