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Biomedical subjects

H Löser

Publications and source records attributed to H Löser.

At least 19 recordsLinked to original sources

[Eye manifestations of fetal alcohol syndrome].

Alcohol is the most frequent and most important teratogenic agent causing mental and physical retardation in childhood. The alcohol fetal syndrome is characterized by pre- and postnatal growth retardation, hypotonia, hyperactivity, microcephalus, mental retardation and typical craniofacial malformations. The latter includes short palpebral fissures, a poorly developed philtrum, thin upper lip vermillion, short mandibles, a flattened midface structure and dysplastic ears. Ophthalmological signs occur in 90% and include epicanthus, ptosis, myopia, optic nerve hypoplasia and tortuous retinal vessels. Microphthalmus, coloboma and Peters' anomaly have also been described. Five children with alcohol embryopathy are presented. The histopathological findings of the enucleated eye of a 6th child with alcohol embryopathy which was sent to us for examination and which revealed an anterior staphyloma with Peters' anomaly is also described. Ophthalmologists should be aware of alcohol in pregnancy as a preventable cause of eye malformation.

Child, Preschool

[Mild forms of alcoholic embryopathy following excessive use of alcohol].

Within 15 years 171 children with fetal alcohol syndrome were diagnosed. Eight children showed an unusual low susceptibility to the harmful effects of alcohol in utero with mild expression of fetal alcohol syndrome or so-called alcohol effects, inspite of excessive maternal drinking (more than 180 gs alcohol per day). Increased tolerance of alcohol might be explained by two mechanisms: 1. By metabolic adaptation, mainly by swift increase of alcohol metabolizing; 2. by biochemical and structural adaptation at cellular membranes after chronic alcohol ingestion. The grade of severity in fetal alcohol syndrome does not depend on the amounts of alcohol but mainly on the development of metabolic and morphological tolerance and adaptation.

Adolescent

[Congenital alcoholic cardiomyopathy in 3 children].

Three cases of congenital alcoholic cardiomyopathy and cardiac defects in children are described. The mothers were heavy spirits drinkers. One child had a ventricular septal defect, two had Fallot's tetralogy, all requiring operation. At open-heart surgery left-ventricular myocardial biopsies were obtained in all three children. Histological and electronmicroscopic examination revealed primary toxic and hypotrophic changes, which differed from those seen in alcoholic cardiomyopathy of the adult. The cells, their nuclei and the myofibrils had reduced diameters. The mitochondria were damaged to differing extent. The myofibrils were arranged in parallel and some had contraction bands and ruptures. The sarcoplasmic reticulum was dilated and had vacuoles. The cell surface penetrated into the interstitial tissue. All these changes could have resulted from inhibited embryofetal cell growth and cytotoxic damage by alcohol during pregnancy. It would seem that alcoholic cardiomyopathy occurs only in extreme forms of alcohol abuse during pregnancy. The prognosis is as yet unknown.

Cardiomyopathy, Alcoholic

Type and frequency of cardiac defects in embryofetal alcohol syndrome. Report of 16 cases.

Within a period of 3 years, 56 infants and children with embryofetal alcohol syndrome have been detected and examined for heart defects. All children were from mothers who had been addicted to alcohol even during pregnancy and they showed a typical pattern of malformations, as described by Lemoine et al. (1968) and Jones et al. (1973). In 16 cases cardiovascular malformations were confirmed by heart catheterisation or pathological examination. The overall incidence of heart defects in this syndrome was 29 per cent. The incidence rises to nearly 50 per cent in the more severe types of this syndrome. Atrial septal defects were found to be the most common heart defect (10 out of 16 cases); ventricular septal defects and other variable malformations occurred less frequently. The high incidence of heart defects indicates that alcoholism during pregnancy has to be considered as a serious and preventable cause of congenital heart disease.

Abnormalities, Drug-Induced

[Peripheral stenoses of the pulmonary arteries: possible causes and syndromic relationships (author's transl)].

The catheterization results and additional malformations and diseases in 20 infants and children with pulmonary artery stenosis are reported. In 17 cases additional cardiovascular malformations were found. In 15 cases extracardiac malformations or developmental retardation were observed. Peripheral stenoses of the pulmonary arteries are frequently established in rubella embryopathy, in the syndrome of supravalvular aortic stenosis, in Down s syndrome, in different types of nanism, in thalidomide embryopathy and in the socalled arteriohepatic dysplasia (Watson and Miller) associated with hypoplasia of the bile ducts. In 3 cases an arteriophepatic dysplasia was confirmed by angiocardiography and biopsy. Coarctations of pulmonary arteries rarely need operative interventions.

Adolescent

[Propranolol in Fallot's tetralogy: hemodynamic investigations and their clinical use (author's transl)].

During heart catheization in 32 non-selected patients with Fallot's Tetralogy the reaction of O2-saturation, right-to-left-shunt and of cineangiocardiography to 0.2 mg/kg propranolol i.v. was tested. Nine of 32 patients reacted favourably to propranolol. Of these 6 were put on a long term-medication of 2 to 3 mg/kg propranolol orally. Only 1 patient did not respond satisfatorily. The effect of propranolol and hemodynamic results are compared with the literature and discussed. In Fallot's Tetralogy propranolol medication without previous testing ist not recommended: without previously established exact diagnosis it can be dangerous even.

Adolescent

[Clinical aspects of pathogenesis of alcohol embryopathy (author's transl)].

68 cases of alcohol embryopathy are reported. The main symptoms are intrauterine and postnatal growth retardation (91%), microcephaly (87 per cent), psychomotor and mental retardation (84 per cent) and a typical craniofacial dysmorphism. Other malformations are frequently found such as cardiac defects (31 per cent), anomalies of joints (23 per cent) and genitalia (50 per cent). There is a marked variation in the intensity of the malformations. Taking into account the extent of the craniofacial dysmorphism and the cerebral damage, a classification into three types (I-111) of alcohol embryopathy is proposed. That ethanol has a teratogenic effect seems to be confirmed. The mother's clinical history suggests that the quantity of alcohol consumed has no marked influence on birth weight, length of gestation and severity of the symptoms. Possibly a defective ethanol metabolism in the severely affected mothers may account for the dysplasias.

Abnormalities, Drug-Induced

[Risks and results of banding surgery (author's transl)].

A Muller-Dammann banding operation was performed in 64 infants and small children within 5 years. The diagnosis and the surgical risk (between 5.9% and 65% depending on the degree of intracardial malformation) were presented. Complications and cause of death were described and compared with data presented in the literature. Twenty-three patients were given a follow-up examination with special cardiac diagnostic methods. The hemodynamic results were compared with those from 9 children who were operated on late. As other authors, were also found a pronounced reduction in pulmonary flow in those children operated on early and not in those operated on late.

Follow-Up Studies

[Cardiovascular malformations in embryofetal alcohol syndrome (author's transl)].

17 infants and children with embryofetal alcohol syndrome were examined of cardiovascular malformations. In 8 cases heart catheterization revealed malformations, mainly atrial septum defects (in 5 of 8 cases), only in one case a ventricular septum defect, in another case an aplasia of the right pulmonary artery and in one case a mild outflow tract obstruction of the left ventricle. Congenital heart diseases can be expected in nearly 50% of the cases in embryofetal alcohol syndrome.

Alcoholism

[Aberrant pulmonary vessels from the abdominal aorta in children with congenital heart disease (author's transl)].

Ten children are described in whom aberrant pulmonary vessels arising from the abdominal aorta were found in the course of cardiac investigations. In five, sequestrated lung, as described Pryce, was demonstrated; in the others, these vessels were found incidentally without any demonstrable abnormality in the lung. Our findings indicate that one must distinguish between aortic pulmonary vessels with and without pulmonary sequestration during childhood. The need for deliberate opacification of the aorta is pointed out. This is also necessary in arriving at the indications for sergery. In the presence of congenital heart defects, these arterial vessels are found particularly in the presence of hypoplastic lungs and with the scimitar syndrome (3 cases).

Abnormalities, Multiple

[Measurements of blood volume in children with orthostatic syndrome (author's transl)].

The blood volume in 12 children with primary orthostatic syndrome was determined. For six cases it was measured in vertical and horizontal position of the body. Plasma volume was measured by 125J human albumin, erythrocyte volume by 51Cr erythrocytes. In seven cases the values of total blood volume were below the normal values of 68--80 ml/kg body weight when determined in horizontal position. Diminished values were found especially in children suffering from severe orthostatic syndrome. The results indicate the diminution of total blood volume to be an important pathogenetic factor in orthostatic syndrome. In primary orthostatic syndrome it should be differentiated between a hypovolemic type, and a type of predominantly vascular dysregulation. It is suggested that the development of blood volume regulation is delayed. The reduced capacity of the vascular system associated with reduced physical activity could be an additional factor. The active total blood volume increased in all six cases with the change from an upright to a recumbent position. The mean of the difference (3.8 ml/kg body weight) was found to be lower than that in adults. This suggests that sequestration of blood volume is of no evident significance in childhood.

Blood Volume Determination