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Biomedical subjects

H H Kramer

Publications and source records attributed to H H Kramer.

At least 37 records · Page 2Linked to original sources
PubMed1991

Beta-adrenoceptor density on mononuclear leukocytes and right atrial myocardium in infants and children with congenital heart disease.

Sympathetic regulation of myocardial performance has been shown to be altered in congestive heart failure. Right atrial tissue of children with severe acyanotic and cyanotic congenital heart disease (CHD) showed a significantly lower beta-receptor density than that of children with less severe defects. Since mononuclear leukocytes (MNL) contain a homogeneous population of beta 2-adrenoceptors which have similar properties to those of cardiac beta 2-adrenoceptors, they are frequently used for studying the beta-adrenergic system. In a group of 37 children with CHD of different types and severity who underwent cardiac surgery, we compared the MNL beta-adrenoceptor density to the type and severity of CHD and looked for a possible relationship to plasma catecholamine levels and to the right atrial beta-adrenoceptor density. Membranes of MNL and myocardial cells were radiolabeled with (-)3-[125I]Iodocyanopindolol [( 125I]ICYP). A significantly higher beta-adrenoceptor density on MNL was found in patients with moderate acyanotic CHD (group I) than in those with severe acyanotic (group II) and cyanotic CHD (group III). Patients of group I showed approximately 50% higher myocardial beta-receptor density than those of groups II and III. ICI 118.551-[125I]ICYP competition studies revealed that in groups II and III significantly lower proportions and densities of beta 1-receptors were found compared to group I. Noradrenaline (NA) plasma levels in group II and group III were significantly higher than those in group I. The adrenaline plasma levels were found to be very high in all children with CHD.(ABSTRACT TRUNCATED AT 250 WORDS)

Binding, Competitive↗
PubMed1991

Left ventricular thrombus in a 2-year-old boy with cardiomyopathy: lysis with recombinant tissue-type plasminogen activator.

A 2-year-old boy with cardiomyopathy and clinical signs of cardiac failure presented with an echodense structure in the left ventricle. This structure was seen from different echocardiographic views adjacent to a hypokinetic area of the apex and lateral free wall. It was different in texture and motion from the underlying myocardium and thus met the diagnostic criteria of a left ventricular thrombus. This thrombus protruded into the cavum and was partly mobile. In view of a high embolic risk, thrombolytic therapy with recombinant tissue plasminogen activator was started. The thrombus resolved within 72 h without any embolic or bleeding complications. No recurrence of the thrombus was observed during a 3-month follow up period.

Cardiomyopathies↗
PubMed1991

Myocardial beta-adrenoceptor density and the distribution of beta 1- and beta 2-adrenoceptor subpopulations in children with congenital heart disease.

Twenty-six infants and children with congenital heart disease (CHD) undergoing cardiac surgery were investigated for alterations in myocardial beta-adrenoceptor density. The patients were divided into three groups according to type and severity of CHD: group I consisted of 6 patients with acyanotic shunt lesions of moderate severity; group II comprised 13 children with severe acyanotic shunt and valve lesions and group III included 7 children with cyanotic CHD. The myocardial beta-adrenoceptor density was determined using (-)3-[125I]Iodocyanopindolol [( 125I]ICYP) and was reduced by approximately 50% in severe acyanotic CHD (33.6 fmol/mg protein) and cyanotic CHD (35.3 fmol/mg protein) in comparison with the group with less severe acyanotic shunt defects (64.4 fmol/mg protein). The affinity dissociation constant (Kd.ICYP) did not differ statistically between the groups. The proportion of beta 1- and beta 2-subpopulations was evaluated by ICI 118,551-[125I]ICYP competition studies. In group II (61.5%) and group III (69.1%) significant lower portions of beta 1-adrenoceptors were found compared with group I (78.2%). This shift of subpopulations was due to a decreased beta 1-receptor density while beta 2-receptor density was unchanged in all groups. While the plasma noradrenaline levels of group I were similar to those of a control group of 13 healthy children, respective values of group II and III were significantly elevated. A significant negative correlation was found between plasma noradrenaline levels and myocardial beta-adrenoceptor density. It is concluded that exposure of these receptors to increased circulating catecholamines, due to an enhanced sympathetic tone, leads to a reduction of their density.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Cardiac rhythm after Mustard repair and after arterial switch operation for complete transposition.

We compared the prevalence of arrhythmias among the first consecutive 45 patients with complete transposition (concordant atrioventricular and discordant ventriculo-arterial connexions) after arterial switch operation and the last 47 patients after Mustard repair in infancy. Both groups had 24-hour Holter electrocardiographic studies at similar periods of follow up (24 +/- 14 and 25 +/- 18 months). A second group of patients undergoing the Mustard procedure had been repaired at an older age before 1981. They were studied to determine the frequency of disturbances of rhythm during later postoperative follow-up (85 +/- 24 months). Symptomatic brady-/tachyarrhythmia syndrome never occurred after the arterial switch and only once in the group of patients repaired by the Mustard procedure in infancy, but developed at a late stage (69 +/- 28 months); five times in the group of patients having Mustard's repair at an older age. In addition, Holter monitoring did not detect bradyarrhythmias indicating sinus node dysfunction in a single patient after the arterial switch, but did so to a similar extent in both groups having the Mustard procedure (recent: n = 14; older: n = 18). Three cases of the group of older patients undergoing a Mustard operation developed complete atrioventricular block during follow-up. Normal findings were present in 93% of the cases after arterial switch, but in only 51% of the cases with a similar follow-up repaired by the Mustard procedure, and in 29% of the group having the Mustard repair at an older age.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1991

The arterial switch-operation: early and midterm (6 years) results with particular reference to technical problems.

Since February 1985 the arterial switch operation (ASO) has become the surgical treatment of choice for newborns with simple TGA, appropriate forms of complex TGA and double outlet right ventricle (DORV) as well at our institution. Between 1985 and 1990 a total of 87 patients underwent surgery. In 60 patients with simple TGA and 8 patients with complex TGA or DORV, respectively, an arterial switch-operation was performed. Because of coronary artery anomalies (n = 13), dysplastic pulmonary valves (n = 3) or pressure drop in the left ventricle (n = 1), the initially planned arterial switch operation was discarded and a Mustard type procedure was in 17 patient. Finally there were two primarily performed Mustard operations. The hospital mortality after arterial switch for simple TGA was 15% (9/60), 0/8 in patients with complex TGA. Late mortality was calculated to be 12% (1/8) in patients with complex TGA and 3/60 in patients with simple TGA. Within the Mustard group there were 2/19 hospital deaths and one late death. Causes of early death after arterial switch were: intraoperative myocardial infarct (n = 3) low cardiac output syndrome (n = 2), intractable bleeding (n = 2), metabolic acidosis (n = 1), and septicemia (n = 1). Late after surgery there was one death due to chylothorax after thrombotic obstruction of the SVC, and 3 more deaths secondary to intraoperative infarct, progressive LV dysfunction and meningitis, respectively. Among the long-term survivors 2 patients developed a severe supravalvulary pulmonary stenosis. There were no significant arrhythmias, supravalvulary pulmonary aortic stenoses, aortic insufficiency or myocardial perfusion disturbances.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1990

Birth weight of children with congenital heart disease.

Birth weights of 843 children with congenital heart disease (CHD) were compared to the respective data of a normal West German population. On average, the CHD group had significantly lower birth weights, but the weight deficit was far less pronounced than in previous studies. The decrease in birth weight was distinct only in children with tetralogy of Fallot and atrial septal defect. Compared to normal newborns, patients with CHD were more often small for gestational age (15.0%) or had a low (less than 2500 g) birth weight (8.6%). The prevalence of prematurity was not increased. Extracardiac malformations were not significantly more common in CHD patients, who were small for gestational age, than in CHD patients with normal birth weight.

Birth Weight↗
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↗
PubMed1989

Development of personality and intelligence in children with congenital heart disease.

One hundred and twenty-eight children with congenital heart disease (CHD) and 89 healthy controls were compared according to their development of personality and intelligence. Presence of physical disability in daily life was the independent variable for dividing children with CHD into two groups: children with physical handicap due to CHD (n = 77) have lower IQ scores than healthy children. Unexpectedly, in the parents' opinion they show no difference in behaviour patterns. On introspective examination, these cardiac patients showed an increased feeling of inferiority and of basic anxiety and a more impetuous behaviour as their way of self-protection, but a reduced need for independence due to parental overprotection was not confirmed. None of these observations could be detected in children with CHD who are physically fit (n = 51).

Adolescent↗
PubMed1989

[Normal values of the growth of the pulmonary arteries in children. An angiography study].

Systolic and diastolic diameters of the pulmonary artery anulus (PA-A), pulmonary artery sinus (PA-S), pulmonary artery trunc (PA-St), as well as those of the right and left pulmonary arteries (RPA, LPA) were measured from cineangiograms of 51 infants, children and adolescents without heart disease (n = 16) or with cardiovascular malformations which had no hemodynamic relevance (n = 35). The following diagnostic categories were included in the normal group: pulmonary stenosis (n = 16), bicuspid aortic valve (n = 4) or subvalvular aortic stenosis (n = 2), all with a systolic gradient lower than 15 mm Hg, aberrant innominate artery (n = 5), small PDA (n = 3) or small VSD (n = 2) and patients with Kawasaki disease without coronary aneurysms (n = 3). The patients were sedated and studied in a fasting state and in the supine position. Angiocardiographies were performed in the right ventricle or/and in the pulmonary artery. A grid or the known diameter of the catheter was used for calibration purposes. The corresponding systolic and diastolic diameter values were averaged in order to obtain a mean diameter of each vascular segment. All measurements were correlated with the body length and the body surface area (BSA). A root exponential function [square root of y = a(1 - e-bx) + c] was used, the regressions coefficients of which can be explained by physiological means. In this function c2 is the theoretical minimal diameter and (a + c)2 the theoretical maximal diameter of a cardiovascular structure; in this way the growth of the cardiovascular dimensions could be well defined mathematically and correlated strongly (r = 0.99) with body length and BSA. Different exponents were found for the pulmonary artery anulus, sinus, trunc as well as right and left pulmonary arteries. All the diameters showed a systolic increase between 8 to 10% above diastolic measurements. The anulus had an average diameter 16% smaller than the sinus and 9% than the distal region of the pulmonary artery; the right pulmonary artery was 7% larger than the left. A limitation of the study was that the diameters were measured in single projections only, necessitating the assumption of a circular cross section of the cardiovascular structures. The angiographically determined PA-A was larger than those of formaldehyd-fixed hearts. This finding indicates that the fixation method may alter the anatomic structures probably due to shrinkage process. PA-S measurements were closest to echocardiographic diameters measured by other authors.

Adolescent↗
PubMed1989

[Normal values of the growth of the aorta in children. An angiography study].

Angiocardiography is a standard procedure for quantification and evaluation of congenital heart disease. Knowledge of the diameters at the level of aortic valve ring, ascending and descending aorta can contribute to the decision in repair of congenital heart disease. Moreover, they are helpful for postoperative follow-up studies. In order to establish normal standards, analysis was performed on 51 cineangiograms obtained from infants, children and adolescents with no evidence of heart disease (n = 16) or only hemodynamically irrelevant cardiovascular malformations (n = 35). The individual diagnoses in these patients appear in the accompanying publication in this issue. Patients with bicuspid aortic valve or subvalvular aortic stenosis were excluded from measurements of the aortic valve ring, the sinus and the ascending aorta. Angiocardiography was performed in a fasting state, after sedation and in the supine position with injections of contrast material in the pulmonary artery and or in the left ventricle and ascending aorta. For purposes of calibration, a grid or the known diameter of the catheter was used. Systolic and diastolic diameters of the aortic valve ring (AO-R), sinus (AO-S) and distal region, between sinus valsalvae and ascending aorta (AO-D), as well as those of the isthmus (DAO-I), postisthmic region (DAO-PI) and descending thoracoabdominal aorta (DAO-T) were measured. To obtain a mean value for each vascular structure, the corresponding systolic and diastolic diameters were averaged. The mean diameter was referenced to body length and body surface area (BSA).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1987

Malformation patterns in children with congenital heart disease.

We prospectively examined 1016 infants and children with congenital heart disease (CHD) to detect additional malformation patterns. They were divided into two major groups. In the first group (n = 881), the CHD either occurred alone or was accompanied by a major extracardiac malformation (n = 68, 7.7%). In the second group (n = 135, 13.3%), the CHD was part of a malformation syndrome, embryopathy, association, or complex. In one fourth of the 56 chromosomal syndromes, the underlying cytogenetic anomaly differed from trisomy 21. Fourteen of the 30 children with non-chromosomal malformation syndromes had Noonan's syndrome. Thirteen of the 27 embryopathies were due to rubella infection, but alcohol embryopathy occurred nearly as often (n = 10). In those children with malformation associations (n = 16) seven showed cardiofacial association. Three of the five children with malformation complexes had Ivemark disease. In this study, underlying disorders were found in one of eight children with CHD, a considerably higher percentage than that reported in earlier studies.

Abnormalities, Multiple↗
PubMed1986

[Parental and childhood height development. Corrected percentile standards for German children between the age of 2 and 9].

The conventional centile standards allow a judgement of children's height according to age-groups. It is equally important, however, to take the parental height into consideration. In 1970 Tanner ascertained for the British population the correlation between children's height and the mid-parent height (i.e. the average of father's and mother's height). From these results he set up centile standards that allow the judgement of children's height at successive ages in terms of the mid-parent height. Several conditions should be fulfilled for their application within other populations: identical correlation coefficients of parents' and children's heights which were shown in Tanner's international study; the accordance of the average height and the standard deviations, respectively, of children in each of the age groups as well as the conformity of the mid-parent height. Our investigations showed that the 3 parameters which were last mentioned differed from those of the British population. Therefore, proper standards for correction were set up. The calculations were worked out for children at ages 2 to 9 years. The methodical procedure will be shown in detail. The correction standards allow a statement as to whether the height of a child can be classified as normal or not within the framework of his family. This is particularly advantageous in regards to the diagnostic clarification of short stature, since it is often due to constitutional, genetic factors; if, for example, according to age the height of a five-year-old boy as well as the mid-parent height coincide at the 3rd centile, then the child's height centile will be 32nd. in consideration of these hereditary components. So the differential diagnosis of genetic short stature is made considerably more straightforward by the use of the present charts.

Body Height↗
PubMed1984

[Longtime care of spina bifida patients. Therapy and prognosis in the last 20 years].

206 patients with spina bifida were followed up during the last 20 years; they were examined from the pediatric, neurosurgical, urological and orthopedic point of view. Evaluation of the intellectual development was also performed. Most of the 92 children of the first group were born after Lorber in 1971 proposed a selective policy of treatment of newborn infants, 144 (group 2) prior to this time. Shunting devices to alleviate hydrocephalus were necessary in two thirds of all patients. The incidence of shunt obstruction most commonly of the ventricular end was very high during the first two years of age but decreased sharply thereafter. Radiological screening disclosed pathological changes of the upper urinary tract in more than one fourth of all children, especially in girls. In the older group 28% of the patients over 5 years of age were mainly bound to a wheelchair, as opposed to 14% in the younger group. Epileptic seizures occurred in 27 cases. Children with thorakolumbar lesions proved to have a poorer intellectual development than those with lumbosacral lesions. Low IQs (less than 90) were also evaluated in every second patient with a shunt dependent hydrocephalus. Since the adoption of selective treatment the percentage of patients with severe handicap has decreased significantly.

Adolescent↗
PubMed1981

[Moyamoya like vascular disease in tuberous sclerosis (author's transl)].

A 12 years and 8 months old girl with tuberous sclerosis developed focal motor and sensory seizures and hemiplegias initially right-sided and later left-sided. Cerebral angiography showed bilateral and symmetrical vascular network with teleangiectasias in the region of the basal ganglia and bilateral stenoses and ectasias of the middle cerebral artery. It is discussed whether this is a case of atypical Moyamoya disease or whether the bilateral basal networks are blood vessel dysplasias and part of the neurocutaneous phakomatosis.

Arterial Occlusive Diseases↗