The optimal method with which to assess right ventricular function.
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Cardiac Volume↗
Biomedical subjects
M Vogel
Publications and source records attributed to M Vogel.
Transthoracic 3-dimensional echocardiography in the assessment of subaortic stenosis due to a restrictive ventricular septal defect in double inlet left ventricle with discordant ventriculoarterial connections.
UNLABELLED: To evaluate the accuracy and clinical utility of three-dimensional echocardiography in the assessment of the size and shape of the ventricular septal defect in double inlet left ventricle. METHODS: We validated the technique in an autopsy study, and then performed a clinical investigation. Six autopsied hearts were immersed in a waterbath and examined with 3-dimensional echocardiography. We identified the cross-section within the dataset which optimally displayed the ventricular septal defect "en face", and compared its smallest and largest diameters, as well as its area. The ventricular septal defect was then filled with a silicone sealant and a section prepared for direct measurement. In patients, we measured the diameters and area of the ventricular septal defect in endsystole nad computed the aortic valvar area in endsystole from the cross-section showing the aortic valve "en face". Ten patients with double inlet left ventricle, aged between 2 and 15 years, were studied using rotational or parallel scanning. All patients had undergone banding of the pulmonary trunk at a mean age of 7 (3-36) days, usually at the time of repair of the coarctation. Two patients had undergone surgical enlargement of the ventricular septal defect prior to echocardiographic examination. RESULTS: The correlation between the areas of the ventricular septal defect in the specimens measured directly and by 3-dimensional echocardiography was r=0.98, with limits of agreement between -0.1-0.08 cm2. In the patients, the area of the defect was measured as 3.9+/-2 cm2, whereas the aortic valvar area was 2.6+/-0.9 cm2. The ratio between the areas was 1.5 (0.5-2.3). Three patients with areas of the ventricular septal defect smaller than those of the aortic valve had resting Doppler gradients between double inlet left ventricle and the aorta of 16, 20 and 30 mm Hgs, respectively. CONCLUSIONS: 3-dimensional echocardiography provides accurate assessment of the area of the ventricular septal defect in double inlet left ventricle, and is helpful in identifying patients with subaortic stenosis caused by restrictive defects.
Adolescent↗
Transcatheter closure of atrial septal defects under echocardiographic guidance without X-ray: initial experiences.
BACKGROUND: Transcatheter closure of atrial septal defects is performed under fluoroscopy, but echocardiography has gained an important role in the procedure. With the new Amplatzer Septal Occluder a device has become available which is easy to implant with minimal fluoroscopy time. We developed an interventional procedure with this device under transesophageal echocardiography alone without fluoroscopy. METHODS AND RESULTS: Four patients (3 to 16 years of age, bodyweight 14 to 60 kg) with atrial septal defects centrally located in the oval fossa were elected for transcatheter closure. After sedation with midazolam and propofol a diagnostic and interventional catheterization was performed in all cases without fluoroscopy. Oxymetric shunt was Qp: Qs = 1.7 (1.5 to 2.1). Under transesophageal echocardiography, the defects were sized over the wire with a balloon catheter. Mean balloon stretched diameter was 10 mm (7 to 14 mm). Under transesophageal echocardiography an Amplatzer Septal Occluder was placed into the defect. In two patients this was achieved with a 5 MHz monoplane pediatric transducer, in two patients a 10mm 5 MHz multiplane probe was used. Complete closure was achieved in all patients and no complications were encountered. CONCLUSION: We conclude that in selected cases with an atrial septal defect located in the oval fossa and clear-cut echocardiographic findings, an Amplatzer Septal Occluder can be safely deployed under echocardiographic guidance alone.
Adolescent↗
[The value of magnetic resonance tomography (MRT) for evaluating ventricular and anastomotic functions in patients with an extra- or intracardiac total cavopulmonary connection (TCPC)-modified Fontan operation].
PURPOSE: To evaluate different MR methods (ventricle and flow measurements) for the postoperative follow-up of hemodynamics in patients with extra- or intracardial TCPC. MATERIALS AND METHODS: Twenty-eight consecutive patients (14 female, 14 male) within the ages of two to thirty-eight years were examined using a 1.5 T Gyroscan ACS-NT scanner (Philips, Best, Netherlands). 7 patients had an extracardial (eTCPC), and 21 an intracardial (iTCPC) tunnel. The calculation of the ventricular function and muscle mass was performed using "multislice-multiphase" technique by summing up the end-diastolic and end-systolic areas; the flow measurements were evaluated by phase shift velocity mapping in the superior vena cava (SVC), inferior vena cava (IVC), right (RPA) and left (LPA) pulmonary artery. Besides peak and mean velocity, the mean and maximal flow volumes (ml/min) were calculated. RESULTS: Ejection fraction (EF) of the functionally single ventricle was within the normal range (mean 57%) in 22/28 patients while mean muscle mass was elevated in the group with eTCPC (mean 121 g/m2). The mean flow volumes and the peak velocities in all vessels were higher in the group with iTCPC as compared to the one with eTCPC. Clinically relevant retrograde flows in the IVC were only found in the group with iTCPC (7/21), as well as a significant predominant flow distribution towards the RPA (p < 0.05; Wilcoxon signed-rank test); in the group with eTCPC towards the LPA (n.s.). CONCLUSIONS: MRI is a useful method for the assessment of ventricular function and muscle mass in the follow-up after the modified Fontan operation. MRI flow measurements additionally provided clinically relevant information about the hemodynamics in Fontan patients.
Adolescent↗
[An attempt to extract an intraretinal nematode located in the papillomacular bundle].
BACKGROUND: The nematode infection of the retina is a rare disease with sight threatening prognosis. Untreated eyes usually become blind. There are different types of clinical progression: granuloma causing tractional detachment chronic inflammation called diffuse unilateral subacute neuroretinitis (DUSN) and rapid loss of vision named "ocular wipe-out syndrome". Efficient drugs are not available. The progression of the disease can be stopped by killing the nematode by means of photocoagulation. For parasites in the periphery of the fundus this is the therapy of choice. Photocoagulation of the nematode close to the posterior pole can cause severe damage to the visual functions. There is a need for a less detrimental treatment. PATIENT AND METHODS: We report on a 48-year-old woman with an intraretinal nematode inside the papillomacular bundle. At an early stage of the disease with normal visual acuity the eye was vitrectomized and a posterior vitreous detachment performed. It was intended to extract the worm out of the retina in toto. The retina over the parasite was incised parallel to the nerve fibers, a tiny hook inserted and the worm pulled out. RESULTS: Only one half of the parasite could be removed since the adhesion of the parasite to the retina was stronger than its own cohesion. Nevertheless the inflammation rapidly regressed. Finally a small area of pigment mottling persisted around the former location of the parasite corresponding to a relative scotoma in the visual field. Visual acuity remained unchanged at full vision. CONCLUSION: We demonstrated that nematode larvae can be removed successfully from the central retina using vitrectomy techniques. By that means the damage to the visual functions can be limited. Near the posterior pole surgical extraction of the worm may be favorable compared to photocoagulation.
Animals↗
Specular and diffuse scattering of highly aligned phospholipid membranes.
We present a quantitative study of specular and diffuse (non-specular) x-ray and neutron reflectivity from highly aligned phospholipid membranes deposited on solid substrates. The height-height correlation function could be obtained from the diffuse scattering without further model assumptions. The results differ significantly from the linear theory of smectic elasticity. We argue that the diffuse scattering is dominated by static liquid-crystalline defects, rather than thermal fluctuations.
1,2-Dipalmitoylphosphatidylcholine↗
Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects.
OBJECTIVE: To examine the incidence of raised pulmonary artery pressure and resistance in adults with isolated atrial septal defect within the oval fossa (so called secundum defect) or sinus venosus defect. DESIGN: A historical, retrospective, unrandomised study. SETTING: A tertiary referral centre. METHODS: Cardiac catheterisation was performed in all patients, with measurement of pulmonary artery pressure and resistance. Pulmonary to systemic flow ratio was calculated using the Fick principle. Pulmonary hypertension was defined as mean pulmonary artery pressure > 30 mm Hg, and increased resistance as an Rp/Rs ratio > 0.3. PATIENTS: All patients with a secundum atrial septal or sinus venosus defect who presented between July 1988 and December 1997 were enrolled in the study. RESULTS: Pulmonary artery pressure and resistance in the patients with sinus venosus defect (n = 31) was higher than in patients with atrial septal defect (n = 138). Pulmonary hypertension was present in 26% of patients with sinus venosus and in 9% of patients with atrial septal defect. The incidence of raised pulmonary vascular resistance was 16% in patients with sinus venosus and 4% in patients with atrial septal defect. The increase in resistance occurred at a younger age in sinus venosus defect than in atrial septal defect. CONCLUSIONS: Patients with sinus venosus defect have higher pulmonary pressures and resistances and develop these complications at younger age than patients with atrial septal defects. Thus they should be managed differently than patients with "simple" atrial septal defects.
Adult↗
Effect of C1-inhibitor in a rat model of necrotizing enterocolitis.
Our aim was to investigate the effect of C1-inhibitor (C1-INH) in a rat model of necrotizing enterocolitis (NEC). Twenty-four anesthetized and artificially ventilated rats received 0.1 mg/kg endotoxin. Fifteen minutes later, the animals in the study group (n = 12) were treated with 200 IU/kg C1-INH whereas the control animals (n = 12) received normal saline. In all animals, FiO(2) was reduced after 90 min from 0.21 to 0.05 and ventilation continued until 180 min or death. All animals developed shock symptoms. Drop in mean arterial blood pressure was more pronounced and survival time was shorter in the control group. Whereas the C1-INH activity increased in the study group, it decreased in the control group. The extent of macroscopic intestinal lesions did not differ between the groups. In conclusion, C1-INH did not prevent shock, but mitigated and delayed its course.
Animals↗
Endothelin-1- and endothelin-receptors in lung biopsies of patients with pulmonary hypertension due to congenital heart disease.
Endothelin-1 (ET-1), with its vasoconstrictive and proliferation-stimulating effects, could play a role in the pathogenesis of primary pulmonary hypertension. We investigated the relationship between the ET-1 like immunoreactivity and the ET-receptor density, the grade of the pulmonary vasculopathy, and properties of the pulmonary circulation in patients with pulmonary hypertension due to congenital heart disease. Twenty-six patients with a median age of 1 year and 1 month (6 weeks - 17 years - 9 months) were assigned to group I (n = 15) with a pulmonary to systemic flow ratio (Qp/Qs) > or = 1.5 and a pulmonary to systemic resistance ratio (Rp/Rs) < or = 0.3 ("high flow - low resistance group") and to group II (n = 11) with a Qp/Qs < 1.5 and an Rp/Rs > 0.3 ("low flow - high resistance group"). Patients belonging to group II showed a higher ET(A)-receptor density in lung arteries (p < 0.05) and parenchyma (p < 0.01) than patients in group I. Patients with the highest ET-1 like immunoreactivity in lung artery walls also showed a trend towards a higher ET(A)-receptor density. The ET(B)-receptor expression was low and not related to any of the above factors. Our results suggest that the paracrine lung ET-1 system is up-regulated in pediatric patients with secondary pulmonary hypertension associated with congenital heart disease.
Adolescent↗
Feglymycin, a novel inhibitor of the replication of the human immunodeficiency virus. Fermentation, isolation and structure elucidation.
The novel peptide feglymycin has been isolated from cultures of Streptomyces sp. DSM 11171 by solid phase extraction, size exclusion chromatography and repeated reversed-phase chromatography. The molecular weight was found to be 1900.90 g/mol and the molecular formula is C95H97Nl3O30. Feglymycin contains 13 amino acids of which four are 3-hydroxyphenylglycine and five are 3,5-dihydroxyphenylglycine residues. The structure of the linear peptide has been determined by 1H and 13C NMR spectroscopy. The sequence was confirmed by the observed mass spectroscopic fragmentation pattern. As well as having weak antibacterial activity, feglymycin inhibits the replication of the human immunodeficiency virus (HIV) in vitro.
Antiviral Agents↗
Ala(0)-actagardine, a new lantibiotic from cultures of Actinoplanes liguriae ATCC 31048.
The actagardine-producing strain Actinoplanes liguriae ATCC 31048, forms an additional lantibiotic when it is cultured on mannitol and soya meal. The new compound, Ala(0)-actagardine (1), has been isolated by solid-phase extraction followed by a two-step chromatographic separation. The molecular formula of 1 is C84H129N21O25S4. Its chemical structure was determined by 2D-NMR analysis and was further confirmed by an amino acid analysis, Edman degradation, and partial synthesis from actagardine. 1 exhibits a slightly higher biological activity than the parent compound actagardine. The synthetic analogs Lys(0)-actagardine (2) and Ile(0)-actagardine (3) demonstrate also antibacterial activities and emphasize the importance of the N-terminus for further derivatization.
Actinomycetales↗
Ellis-van Creveld syndrome: examination at 15 weeks' gestation.
In 1940, Ellis and van Creveld defined a syndrome they referred to as chondro-ectodermal dysplasia. This autosomal recessive condition, now usually referred to as Ellis-van Creveld syndrome (EVC), comprises bilateral postaxial polydactyly, a chondrodysplasia, characterized by shortness of limbs, and ectodermal dysplasia. Congenital heart defects are also common. There are many reports in medical literature describing affected newborns and even, older children. Here, we report the clinical, radiological and histological findings in a 15-week-old affected fetus. The diagnosis of Ellis-van Creveld syndrome in this fetus is based on a positive family history (an affected sib) and shortness of long bones as well as hexadactyly diagnosed by prenatal ultrasonography. On post-mortem examination, bilateral postaxial hexadactyly and symmetrical shortness of the long bones was noted. Histologically, there was too short a zone of cartilagineous columns in the metaphyses, a reduced number of chondrocytes and an irregularly structured spongiosa within the ossification zone. In addition, the fetus was found to have an atrio-ventricular canal. This heart defect is presumably rare in this syndrome. Other characteristic features such as small and dysplastic nails, sparse hair and abnormalities of the teeth were, of course, not yet present in this early developmental stage. In addition to EVC, the fetus had a 47,XXY chromosome constitution.
Adult↗
Epitope-specific antibody response to IgE by mimotope immunization.
We have previously described a mouse monoclonal anti-human IgE antibody (BSW17) capable of recognizing receptor-bound IgE without inducing mediator release from human basophils or mast cells. Moreover, immune complexes of IgE and BSW17 are not able to bind to the IgE receptor. An initial attempt to map the precise epitope recognized by this mAb by using Fc epsilon-derived peptides of variable length was unsuccessful. However, by screening random peptide phage display libraries we isolated circular nona- and octapeptides specifically recognized by BSW17. These constrained peptides mimic at least a part of a conformational epitope and are thus called mimotopes. These mimotopes, either phage displayed or synthetically synthesized, did not react with any other anti-human IgE antibody tested, but efficiently inhibited the binding of human IgE to BSW17 only. The use of Rhodol-Green-labeled free cyclic peptide proved that these interactions were not carrier dependent. Immunization of rabbits with phage clones displaying the specific peptides on the surface induced an anti-human IgE response specific for the epitope of BSW17. Therefore, we conclude that such mimotopes or mimotope-derived peptides might be used for vaccination to induce in vivo a beneficial anti-IgE response as a novel immunotherapy.
Amino Acid Sequence↗
A mimotope defined by phage display inhibits IgE binding to the plant panallergen profilin.
Birch pollen and mugwort pollen allergies are often associated with hypersensitivity to plant foods. This clinical and serological cross-reactivity is mediated by IgE antibodies reacting with homologous proteins in pollen and food. Cross-reacting homologs of the important birch pollen allergen Bet v 2 (profilin) could be detected in other pollen, fruits, nuts, and vegetables, such as celery tuber. We purified IgG/IgE antibodies from the serum of an exclusively profilin-allergic patient using affinity columns either coupled with protein extracts from mugwort pollen, birch pollen, or celery tuber. Constrained and unconstrained random nonapeptide libraries were pooled and screened with the anti-profilin antibody preparations to define cross-reactive ligands. Specific ligands were enriched by successive panning rounds using the profilin-specific antibodies in series. After the last panning round enriched phage clones were screened with purified profilin-specific antibodies and IgE-binding clones were sequenced. Five out of eight positive clones (62.5 %) displayed the same circular peptide CAISGGYPVC. This peptide was synthesized and examined for its ability to inhibit IgE binding to blotted mugwort pollen, birch pollen, or celery tuber profilin. Inhibition studies showed reduction of IgE binding to profilins in all three protein extracts. As the sequence of the mimotope did not show any homology to the known birch profilin sequence this peptide is considered to mimic a common conformational IgE epitope for these examined profilins.
Allergens↗
[Initial clinical results with the Amplatzer septal occluder--a self-centering double disc for occlusion of atrial septal defects].
UNLABELLED: In recent years many different systems for transcatheter closure of an atrial septal defect (ASD) have been developed and tested. However, all systems presently available have some special disadvantages. The recently introduced Amplatzer Septal Occluder (ASO), though, appears promising. It is a self-expanding and self-centering double disc made from a Nitinol mesh, which is tightly woven to give mechanical strength. Both discs are separated by a connecting cylindrical portion. Its diameter may be chosen, so that it corresponds to that of the ASD. Discs of polyester patches are sewn into the retention discs as well as into the cylindrical portion of the device in order to augment thrombogenicity. After measuring the diameter of the ASD with a balloon, an appropriate ASO is selected and advanced into the left atrium through a 7 or 8 French sheath. Then the distal disc and part of the connecting cylindrical portion is developed in the left atrium and pulled against the atrial septum, so that the cylindrical portion is occluding the ASD. Thereafter, proximal disc is deployed and the delivery cable disconnected. As long as the cable is connected to the device repositioning is easily achieved by pulling the device back into the sheath. Within a time period of 4 months in 29 out of 31 patients (median age: 12.1 years, median weight: 45.0 kg) complete closure of the ASD with a mean diameter of 11.0 mm (6-20 mm) was achieved without complications, the average fluoroscopy time being 8.3 min (2.9-21.5 min). Mean Qp:Qs was 1.5 (0.9-2.2). During a mean follow-up period of 2.1 months post implantation fixed seating of the ASO without residual shunt, arrhythmias, thrombembolic events and impairment of A-V valves was observed in all patients. CONCLUSION: The Amplatzer septal occluder allows quick, safe, and complete closure of atrial septal defects without complications if one adheres to strict implantation criteria. For a final judgement, however, long-term follow-up studies are necessary.
Adolescent↗
Genetic and immunohistochemical detection of mutations inactivating the keratinocyte transglutaminase in patients with lamellar ichthyosis.
Autosomal recessive lamellar ichthyosis is a clinically heterogeneous group of severe congenital keratinization disorders that is characterized by generalized hyperkeratosis and variable erythema. About half of the patients have mutations in the TGM1 gene, which encodes the keratinocyte transglutaminase. Linkage studies have shown that at least two further loci for autosomal recessive lamellar ichthyosis must exist. We present here two patients with lamellar ichthyosis caused by mutations in the TGM1 gene. The first patient is compound heterozygous for the novel missense mutation C53S and the splice mutation A3447G. The second patient, a child of consanguineous parents from Tunisia, is homozygous for the unknown nonsense mutation W263X. This is the first report of a mutation, C53S, that affects the region of the keratinocyte transglutaminase that is essential for anchorage of the enzyme to the plasma membrane. A novel, rapid in situ transglutaminase activity assay revealed the absence of keratinocyte transglutaminase activity in both patients. The mutations described are hence causative for the ichthyosis phenotype.
Adolescent↗
Regulation of the Epstein-Barr viral immediate early BRLF1 promoter through a distal NF1 site.
The immediate early BRLF1 and BZLF1 promoters of Epstein-Barr virus are crucial for triggering the replicative cycle of the virus. To better understand the cell type dependence of the lytic cycle we conducted an analysis of the BRLF1-promoter in the epithelial cell line HeLa and the lymphoid cell line IM9. To analyze promoter activities, transient transfections with 5'-deletions of the BRLF1-promoter in front of luciferase as reporter gene were conducted. Besides the already known cis-acting elements of the promoter close to the TATA-box, more distal elements were located and functionally tested. A nuclear factor 1 consensus site was found to act positively in HeLa cells, but did not in lymphoid IM9 cells. The NF1 site was shown to bind protein by electrophoretic mobility shift assays, antibody-supershifts and in vitro footprinting. Thus, a protein belonging to the nuclear factor 1 family of proteins was identified as additional cellular trans-acting factor for the BRLF1-promoter besides the already described factors Sp1, Zta and Zif268.
Binding Sites↗
3-D sonographic volume measurement of the cerebral ventricular system: in vitro validation.
This in vitro study evaluates the accuracy and observer dependency of a freehand three-dimensional (3-D) ultrasound system for measuring cerebral ventricular volume in infants. A sphere, a cylinder, and three cerebral ventricle phantoms were made of agarose and embedded in an echogenic matrix after measuring true volumes by water displacement. Volumes of the models were calculated by 3-D software after manual contour marking on ultrasound cross-sections. Mean +/- SD sonographic volume was 94.6%+/-7.3% (n = 130) of true volumes. Intraobserver variation (n = 10) was 2.3%-5.3% for complete investigation (scanning and marking), and 1.8%-4.1% for marking alone. Difference of means (n = 10) between two observers was 7.6% to 10.8% for complete investigation, and 0.6% to 1.6% for the marking process. We conclude that 3-D freehand ultrasonography may be useful for examining ventricle dilatation in infancy.
Anthropometry↗