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Biomedical subjects

F Majewski

Publications and source records attributed to F Majewski.

At least 73 records · Page 4Linked to original sources

[Teratogenicity of anticonvulsant drugs (author's transl)].

Investigations were done on 111 children of epileptic mothers who used anticonvulsants in 93 pregnancies and none in 18 pregnancies. Hydantoinbarbiturate embryopathy was found in 7.1% after hydantoin monotherapy, in 17.6% after combination of hydantoin and barbiturates or primidone. No embryopathy was seen in children of untreated epileptic mothers. Children of untreated and treated epileptic mothers had an approximately equal frequency of marked single malformations and cerebral damage without dysmorphia. However, malformation and cerebral damage without dysmorphia was found significantly more frequently in children of mothers on anticonvulsant drugs with convulsions during pregnancy as compared to children of mothers without convulsions. Single manifestations and cerebral damage without dysmorphia are probably not caused by anticonvulsants but by convulsions during pregnancy.

Abnormalities, Drug-Induced

Anomalies of the kidneys and genitourinary tract in alcoholic embryopathy.

in addition to the well known major signs of growth retardation, microcephaly, mental retardation and typical craniofacial dysmorphism, malformations of the urinary tract were found in 9 of 110 patients with alcohol embryopathy. Of the 110 patients 21 have been examined adequately. An extensive urologic investigation of children with alcohol embryopathy and symptoms suggesting kidney or genitourinary tract disease is essential for early diagnosis and correction of malformations. Awarness of alcohol embryopathy in a child referred for correction of urinary tract anomalies may help to establish the diagnosis and prevent it in further offspring.

Child, Preschool

[Craniofacial amniogenic malformations within the Adam complex (author's transl)].

Four children are described with amniotic deformities in the craniofacial region. Besides encephaloceles, hydrocephalus, facial clefts, amniotic bands and furrows we observed in three of these children the deformities of the extremities which are characteristic of amniogenic malformations, such as syndactylias and mutilations, associated with ring constructions. The article describes the state of knowledge with regard to pathogenesis. Epidemiology of the disease permits to assume a very low risk of repeat occurrence with the parents concerned.

Abnormalities, Multiple

[Obstetrical and postnatal complications in children of chronically alcoholic mothers (author's transl)].

The histories of mothers addicted to chronic abuse of alcohol always present severe complications during pregnancy and for most of their children during the peri- and postnatal period as well. The question arises to what extent peri- and postnatal complications may influence the clinical aspect of the alcoholembryopathy (AE) in these children. In 35 children with AE all details of their histories could be traced. By using Prechtl's concept of optimal conditions two different populations could be found amongst the children with AE, one with and the other without severe peri- and postnatal complications (exclusively peri- and postnatal asphyxia). No correlation could be found between the severity of AE and perinatal asphyxia. The result suggests reservation in rating peri- and posnatal asphyxia as an always potent factor causing brain damage.

Asphyxia Neonatorum

Alcohol embryo- and fetopathy. Neuropathology of 3 children and 3 fetuses.

Maternal chronic ethanol abuse during pregnancy causes malformations of the offspring. Three children (aged 6 months, 9 months, 4 1/2 years) and 3 fetuses (17th, 18th, and 20th gestational week) showed a wide spectrum of disorders ranging from severe dysraphic state, arhinencephaly, porencephaly, agenesis of corpus callosum, a range from hydranencephaly to microdysplasias (p.e. reduced gyration of dentate nucleus and inferior olives), and a range from gastrochisis or congenital heart defects to craniofacial dysmorphogenesis and palmar crease anomalies. The patterns of the cerebral malformations were not as uniform as the clinical phenotype of the alcohol embryopathy. The observations did not support the assumption that there exists a specific period for alcohol teratogenicity.

Abnormalities, Drug-Induced

Alcohol embryopathy and diabetic fetopathy in the same newborn.

Both alcohol embryopathy and diabetic fetopathy were observed in the same female child. The mother was known to be alcoholic as well as diabetic. At birth the signs of diabetic fetopathy predominated: the child showed edematous subcutaneous fat, birth weight was 3650 g. The heart was enlarged. The patient's blood sugar levels ranged from 0 to 1.4 mMol/1 (0-25 mg/dl). Features of alcohol embryopathy were typical craniofacial dysmorphy, hypotonia of muscles and hyperexcitability. Later on the features of alcohol embryopathy predominated: the child became dystrophic with pronounced microcephaly, and the craniofacial dysmorphy clearly resembled other patients with alcohol embryopathy. This observation is in favour of the hypothesis, that alcohol induces cell hypoplasia in the embryo resulting in postnatal growth retardation. Maternal and consequently embryonic and fetal hyperglycemia induced cell hypertrophy in the embryo and fetus, which compensated the effect of alcohol on birth weight in our patient.

Abnormalities, Multiple

No elevation of exchange type aberrations in lymphocytes of children with alcohol embryopathy.

The lymphocyte chromosomes from 23 children with the 'fetal alcohol syndrome' or 'alcohol embryopathy' (AE) were analyzed with respect to exchange type aberrations. These aberrations were not more frequent in AE than in controls. The possibility that AE results from unspecific suppression of RNA and consequently of protein synthesis in the developing embryo is discussed.

Abnormalities, Multiple

Furrows and dermal ridges of the hand in patients with alcohol embryopathy.

Palmar creases and dermal ridge patterns of 34 patients with alcohol embryopathy are compared with 470 healthy individuals. In alcohol embryopathy several typical deviations were noted. Palmar Creases. The interdigital part of the distal palmar crease is generally sharply bent, the proximal transverse crease is hypoplastic or missing, the thenar crease is commonly well marked. Simian creases and bridged palmar creases are more common in patients with alcohol embryopathy than in healthy individuals. Ridge Patterns of the Palm. The main line D coming from triradius d in patients with alcohol embryopathy mostly shows a low type of ending in the fourth interdigital area; in this area loops are twice as common as in healthy individuals. Patterns of the Fingertips. No deviations were noted in the distribution of whorls and loops, but virtually no arches were observed in patients with alcohol embryopathy. These anomalies suggest embryonic damage in the twelfth week of gestation.

Abnormalities, Multiple

[Interruption of pregnancy in alcoholic women (author's transl)].

Basing on previous experience, with alcohol embryopathy the authors recommended interruption of pregnancy in three chronic alcoholics, two of whom were in the chronic phase and one in the critical phase of alcohol addiction. All the three fetuses were hypertrophic, two severely malformed. In the authors' opinion there is eugenically speaking an absolute indication of interruption of pregnancy in alcoholics in the chronic phase of addiction. In women who are in the critical phase of addiction, each case requires close scrutiny, whereas interruption is not indicated from the eugenic aspect in women in the prodromal stage.

Abnormalities, Drug-Induced

[Familiary arhinia combined with peters' anomaly and maxilliar deformities, a new malformation syndrome (author's transl)].

A report is given on two sisters with arhinia, hypertelorism, Peters' anomaly and deformities of the maxilla. The lacrimal sacs in both patients were extirpated because of recurrent dacryoadenitis due to bilateral aplasia of the nasolacrimal ducts. One eye showing Peters' anomaly with microphthalmus was enucleated in the elder patient 12 years ago. Differential diagnosis includes frontonasal dysplasia and the different types of holoprosencephaly-syndromes. Probably this new malformation syndrome is a recessive inherited.

Abnormalities, Multiple

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

[On certain embryopathies induced by teratogenic agents (author's transl].

In a survey of the literature the teratogenic effects of radiation and some drugs are discussed. Teratogenicity is proved for thalidomide, aminopterin, busulfan, cyclophosphamide, chlorambucil, mercaptopurin and diphenylhydantoin, trimethadione and warfarin. After the thalidomide-tragedy drug-induced malformations of the embryo are extremely rare, whereas malformations due to alcohol are rather frequent. Own experiences with more than 70 patients with alcoholembryopathy are reported. Nicotin seems not to be teratogenic, but due to nicotin the perinatal mortality is elevated. The questionable teratogenic effects of Heroin and LSD are discussed.

Abnormalities, Drug-Induced

[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