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Biomedical subjects

K Strömland

Publications and source records attributed to K Strömland.

12 recordsLinked to original sources

Refractive evaluation in thalidomide embryopathy.

To evaluate the ocular findings associated with thalidomide embryopathy, we examined 86 of 100 Swedes who had a proven correlation between birth defects and the mother's intake of thalidomide during pregnancy. Cycloplegic refraction, keratometry, and axial length measurements were performed. The subjects were divided into four groups according to their physical malformations, giving a time frame for when in gestation the insult occurred (the sensitive phase for thalidomide is 20-36 days after conception). The results indicate a trend toward shorter and longer axial lengths, high refractive errors, and corneal astigmatism in thalidomide embryopathy compared to controls, and in addition there was a tendency for those anomalies to occur in the group with the earliest thalidomide-induced defects. It is suggested that thalidomide disturbs the growth and shape of the eye and that this effect is exerted early in its teratogenic period.

Abnormalities, Drug-Induced

Goldenhar syndrome and autistic behaviour.

Two girls with concomitant Goldenhar syndrome (oculo-auriculovertebral spectrum disorder) and autistic disorder are described. One was diagnosed as having Goldenhar syndrome in the first few weeks of life and as having autistic disorder in her fifth year; the other was diagnosed as having Goldenhar syndrome when she was referred for evaluation of autistic symptoms at seven years of age. The type of physical abnormalities encountered in Goldenhar syndrome suggests damage to neural structures in the second or late stages of the first trimester. The two cases described in this report suggest that autistic disorder sometimes can result from neural damage during the second trimester.

Autistic Disorder

[Alcohol during pregnancy damages eye and vision development].

Maternal alcohol abuse during pregnancy causes malformations of the eyes with serious consequences to the vision of the affected children. A high percentage (up to 90 per cent) of children suffering from the fetal alcohol syndrome have eye abnormalities of different kinds. The most typical are hypoplasia of the optic nervehead and increased tortuosity of the retinal vessels, especially of the arteries, both occurring in up to half of the cases. Visual acuity is often reduced. Considering the developmental timing of different tissues of the eye, one can presume that there is a risk for deleterious effects of alcohol on eye structures at any point of time from early gestation until development is completed.

Alcoholism

The study of malformations "by the company they keep".

Our study of individuals with thalidomide embryopathy reaffirms many of the principles of clinical teratology concerning sensitivity of developing structures and specificity of action. It also highlights important features of ocular teratology that differ from those of other organ systems. Because malformations of the eye and associated structures do not significantly threaten the survival of the embryo, fetus, or neonate, any variation in prevalence in older individuals is not due to the nature of the ocular malformation but may be significantly modified by "the company they keep," the systemic malformations occurring at the same time as the original insult, whether it be an environmental, a genetic, or a local disturbance. Additionally, the presence of clusters of malformations caused by a teratogen at a specific time supports the concept of a common pathogenic mechanism and provides suggested avenues for further research into the cause of these malformations.

Abnormalities, Drug-Induced

Full-field electroretinograms in patients with the carbohydrate-deficient glycoprotein syndrome.

We examined five patients who had carbohydrate-deficient glycoprotein syndrome with full-field electroretinograms. Only two of the patients showed fundus changes typical for retinitis pigmentosa, whereas abnormal electroretinograms were seen in all patients. There was no recordable rod response; however, a delay in the cone b-wave implicit time was noted. All patients had nyctalopia. These observations suggest that patients with the carbohydrate-deficient glycoprotein syndrome have a progressive tapetoretinal degenerative disorder of the retinitis pigmentosa type with defined alterations in the electroretinogram.

Adolescent

Contribution of ocular examination to the diagnosis of foetal alcohol syndrome in mentally retarded children.

An ophthalmological study was conducted on a series of 28 Swedish children suffering from mild mental retardation without an obvious aetiological diagnosis. The eye examination supported a diagnosis of foetal alcohol syndrome in five out of six suspected cases and revealed two additional not previously suspected (25%). Eye anomalies were observed in 16 patients (57%) with fundus abnormalities (optic nerve hypoplasia, retinal dystrophy) being most frequent, occurring in 11 cases (39%). Four patients had substantially reduced visual acuity in both eyes. Eight children had abnormal features of the face and outer eye region. Ocular motility disturbances (strabismus or nystagmus) were seen in five patients. The ophthalmological study disclosed that at least 10% of this group of children with mild mental retardation suffered from the foetal alcohol syndrome. It is concluded that, in children with mental retardation, an ocular examination is a valuable diagnostic tool for revealing prenatal origin.

Child

Ocular pathology in disialotransferrin developmental deficiency syndrome.

Disialotransferrin developmental deficiency (DDD) syndrome is a recently described disease consisting of hepatopathy, mental retardation and neuropathy. The biochemical findings indicate a defect in the assembly of the carbohydrate moiety that is common to the secretory glucoproteins. It is believed to be of autosomal recessive inheritance. An ophthalmological examination of ten children suffering from this syndrome showed that all had ocular involvement. Esotropia (and deficient abduction) was found in all ten patients. Seven children had retinitis pigmentosa which was verified by an ERG in three. One patient had retinal signs suggestive of retinitis pigmentosa. The high incidence of ocular findings in the DDD syndrome, which are reported for the first time, indicate that an ophthalmological examination is a helpful diagnostic tool in this disease.

Adolescent

Ocular abnormalities in the fetal alcohol syndrome.

Thirty children suffering from the fetal alcohol syndrome (FAS) were compared with 22 matched controls regarding malformations of the eyes and the visual function. All mothers to the FAS children had a documented abuse of alcohol during pregnancy. It was not possible to get reliable, detailed data on drinking habits, nor could it be ruled out that some mothers might have used psychopharmaceutic drugs during periods of the pregnancy. Defects of the outer eye region, or intraocular abnormalities, or both, were found in 27 FAS children (90%). Anomalies of the outer region like ptosis and strabismus were frequently found in the FAS children but were not common in the controls. Abnormalities in the anterior segments and media were found in six eyes. Malformations of the fundus were the most frequently occurring abnormalities in the FAS children. Forty-eight per cent of the eyes showed hypoplasia of the optic nervehead and 49% had an abnormal tortuosity of the retinal arteries. These variables were studied quantitatively by a new photogrammetric method. There were serious consequences to the vision of the FAS children. Nineteen per cent of the eyes has a visual acuity of 0.2 (20/100) or less, and 46% had 0.3-0.6 (20/60-20/30). Almost all controls had normal vision. As maternal alcohol abuse during pregnancy was the only variable which could be traced in every case of FAS, it is appears very likely that alcohol caused the abnormalities of the eyes, possibly through non-specific, intra-uterine growth retardation. It that were the case, ocular abnormalities should be common in children with non-FAS growth retardation. A control study did not support this possibility.

Abnormalities, Multiple

Ocular involvement in the fetal alcohol syndrome.

Maternal alcohol abuse during pregnancy causes malformations of the eyes with serious consequences to the vision of the affected children. A high percentage (up to 90%) of children suffering from the fetal alcohol syndrome have eye abnormalities, including malformation in the outer eye region, disorders of motility, and defects of different intraocular structures. Two kinds of malformations stand out as most typical--hypoplasia of the optic nervehead (up to 48%) and increased tortuosity of the retinal vessels, especially of the arteries (up to 49%). Visual acuity is often moderately or severely reduced. Considering the developmental timing of different tissues of the eye, one can presume that there is a risk for deleterious effects of alcohol on eye structures at any point of time from early gestation until development is completed.

Adolescent

Ocular teratology.

The principles of teratology are described, and animal models for research in abnormal ocular development and clinical studies of human teratogens are surveyed. A review is made of presumed ocular teratogenic agents: radiation; external environmental teratogens; maternal conditions such as infections, diabetes, and epilepsy; alcohol and drugs such as thalidomide, retinoic acid, and coumarin anticoagulants; and other agents, such as cigarettes.

Abnormalities, Drug-Induced

Ocular motility in thalidomide embryopathy.

Twenty-one individuals, aged 28 to 29 years, with documented thalidomide embryopathy were examined in Sweden. The most noteworthy ophthalmologic finding was the high prevalence of incomitant horizontal strabismus, primarily of the Duane syndrome type. Less frequently seen were cases of both marked abduction and adduction limitation similar to a gaze paresis with no observable fissure changes, as well as a few cases of isolated abduction weakness or inferior oblique under-action. There were many examples of aberrant lacrimation, facial nerve palsy, and ear anomalies combined with horizontal incomitant forms of strabismus. Ear and facial nerve palsies are known to occur with ingestion of thalidomide early in the sensitive period (approximately days 21 to 26 after conception).

Abnormalities, Drug-Induced