PubMed Health⌕ Search

Biomedical subjects

H L Spohr

Publications and source records attributed to H L Spohr.

At least 19 recordsLinked to original sources
PubMed1995

Antiepileptic drugs alter endogenous retinoid concentrations: a possible mechanism of teratogenesis of anticonvulsant therapy.

The major antiepileptic drugs used for the control of seizures can induce developmental toxicity when administered during pregnancy. Vitamin A and retinoids are thought to control many processes of embryonic development including growth, differentiation and morphogenesis. We have therefore studied if the teratogenic action of antiepileptic agents could be mediated via alteration of the endogenous vitamin A--retinoid metabolism. Retinol and its oxidative metabolites all-trans-, 13-cis- and 13-cis-4-oxo-retinoic acid were measured in the plasma of 75 infants and children treated with various antiepileptic drugs for the control of seizures, and in 29 untreated controls of comparable age. Retinol levels increased with age, while the concentrations of retinoic acid compounds did not exhibit age-dependency. Valproic acid monotherapy increased retinol levels in the young age group and a trend toward increased retinol concentrations was also observed in all other patient groups. The plasma levels of the oxidative metabolites 13-cis- and 13-cis-4-oxo-retinoic acids were strongly decreased in all patient groups treated with phenytoin, phenobarbital, carbamazepine and ethosuximide, in combination with valproic acid, to levels which were below 1/3rd and 1/10th of corresponding control values, respectively. Little changes were observed with all-trans-retinoic acid except in one patient group treated with valproic acid/ethosuximide cotherapy where increased levels of this retinoid were found. Our study indicates that therapy with antiepileptic agents can have a profound effect on the endogenous retinoid metabolism. Because of the importance of retinoids for the signaling of crucial biological events during embryonic development, such altered retinoid metabolism may be highly significant in regard to antiepileptic drug teratogenesis.

Abnormalities, Drug-Induced↗
PubMed1994

The fetal alcohol syndrome in adolescence.

At present, alcohol is recognized as the leading teratogenic agent in long-lasting CNS dysfunction. Little is known about the long-term development and outcome of children with fetal alcohol syndrome (FAS). Forty-four FAS patients who were diagnosed in early childhood were followed up for 10-14 years. This study documents the developmental changes of the manifestations of FAS from childhood to adolescence and describes a characteristic "juvenile" pattern of FAS, which may help to identify this syndrome even in adolescence. This is especially relevant for patients who were not diagnosed earlier.

Adolescent↗
PubMed1993

Prenatal alcohol exposure and long-term developmental consequences.

Fetal alcohol syndrome (FAS) is a leading cause of congenital mental retardation but little is known about the long-term development and adolescent outcome of children with FAS. In a 10-year follow-up study of 60 patients diagnosed as having FAS in infancy and childhood, we investigated the long-term sequelae of intrauterine alcohol exposure. We found that the characteristic craniofacial malformations of FAS diminish with time, but microcephaly and, to a lesser degree, short stature and underweight (in boys) persist; in female adolescents body weight normalises. Persistent mental retardation is the major sequela of intrauterine alcohol exposure in many cases, and environmental and educational factors do not have strong compensatory effects on the intellectual development of affected children.

Adolescent↗
PubMed1992

[Alcohol drinking and intrauterine dystrophia. Effects and significance in infancy].

In a prospective study intrauterine growth retardation (IUGR) (less than 10. growth percentile at birth) was used as a predictor to diagnose intrauterine alcohol exposure. An interview about maternal alcohol consumption was performed prenatally--when IUGR was diagnosed by ultrasound--or postnatally. The children were followed up to 18 months of age. In 6/47 children we diagnosed various degrees of the Fetal Alcohol Syndrome FAS, with only one patient showing a full blown syndrome at birth. 5 patients could not be identified until the pediatric reexamination at the age of 8-18 months. IUGR and a maternal history of even moderate drinking during pregnancy should emphasize the possibility of an intrauterine alcohol damage, even in a normal child at birth.

Alcohol Drinking↗
PubMed1991

[GM2 gangliosidosis, O variant (Sandhoff disease): suspected diagnosis of a storage disease by ultrasound].

We report on a 3.6 year old boy who suffers from Sandhoff disease. The diagnosis was suspected because of striking ultrasound findings: Parts of the thalamus were more echogenic, the cortical gyri were sharp and accentuated. The CT- and MRI features were similar to the US-findings. The diagnosis was confirmed by demonstrating the typical enzyme deficiency in leucocytes.

Brain Diseases, Metabolic↗
PubMed1987

Follow-up studies of children with fetal alcohol syndrome.

Data coming from a prospective multidisciplinary study with repeated examinations of children with fetal alcohol syndrome (FAS) are reported. These patients underwent pediatric, neurological and psychiatric assessment, EEG-recordings and psychological testing. After a period of 3-4 years various subgroups of these children were re-examined. Follow-up examinations revealed that with increasing age dysmorphic signs became less apparent in children with FAS. Furthermore, neurologic performance improved and EEG-recordings revealed less pathological patterns. These positive findings were confirmed by the observation that these patients also experienced an improvement with regard to psychiatric status and cognitive functions. But it must be stated that the affected children did not become normal in all psychiatric areas. Hyperactivity and distractibility seem to be the major handicap for a normal school career of these children. In general biological maturation seems to be the main factor responsible for the outcome of FAS.

Child↗
PubMed1984

Clinical, psychopathological and developmental aspects in children with the fetal alcohol syndrome: a four-year follow-up study.

Paediatric, neurological and psychiatric examinations were done in 71 children with the fetal alcohol syndrome (FAS). A subgroup of these patients underwent psychiatric assessment and psychological testing. Psychopathology was also studied in a matched control group. After three to four years various subgroups were re-examined. With increasing age dysmorphic signs were less apparent. Neurological performance improved and EEG recordings revealed fewer pathological patterns. The psychiatric status and cognitive functions of the patients also improved, but they did not become normal in all psychiatric areas. Hyperactivity and distractibility seem to be the major handicaps preventing these children from having a normal school career.

Child Development↗
PubMed1984

[The course of alcoholic embryopathy].

Pediatric examinations were performed in 71 children with fetal alcohol syndrome (FAS). A subgroup of these patients underwent neurological and psychiatric assessment and psychological testing. Psychopathology was also studied in a matched control group. After a period of 3-4 years various subgroups of these children were re-examined. Follow-up examinations revealed that with increasing age dysmorphic signs became less apparent in children with FAS. Furthermore, neurologic performance improved and EEG-recordings revealed less pathological patterns. These positive findings were confirmed by the observation that these patients also experienced an improvement with regard to psychiatric status and cognitive functions. But it must be stated that the affected children did not become normal in all psychiatric areas. Hyperactivity and distractability seem to be the major handicaps for a normal school career of these children.

Attention Deficit Disorder with Hyperactivity↗
PubMed1983

Fetal alcohol syndrome and mental retardation: spine distribution of pyramidal cells in prenatal alcohol-exposed rat cerebral cortex; a Golgi study.

Among the various possible effects of the fetal alcohol-syndrome (FAS), mental retardation can be considered the most deleterious. In animal studies, prenatal alcohol exposure has been shown to result in increased neonatal mortality, retarded cerebellar development and a significant decrease in neonatal brain weight. In a Golgi study on Wistar rats that were prenatally exposed to alcohol the spine distribution in proximal apical dendrites of layer V pyramidal cells of the parietal cortex was examined. As compared with controls, a distinct spine abnormality could be demonstrated at 12 days and at 40 days of postnatal age: a persistent predominance of long, thin and entangled spines, and a decreased number of normal stubby and mushroom-shaped spines. These abnormal dendritic patterns show a striking resemblance to those described by Purpura in mentally retarded children of normal karyotype.

Animals↗
PubMed1983

[The incomplete fetal alcohol syndrome].

In contrast to the wellknown picture of the "fetal alcohol syndrome" (FAS), clinical symptoms of the incomplete FAS are less frequently recognized compared to the high incidence of the syndrome as stated in the literature. The diagnostic problem is a clear differentiation between mild forms of the incomplete FAS and clinical normality. Post-natal growth deficiency and microcephaly of unknown aetiology, clinical symptoms of hyperactivity, learning disabilities or mild mental retardation are the main features of the incomplete FAS. The diagnosis is established only by a positive history of maternal alcohol abuse.

Child↗
PubMed1982

Development of psychopathology of children with the fetal alcohol syndrome.

Extended psychiatric and pediatric examinations were performed on patients with the fetal alcohol syndrome (FAS). A subgroup of these patients underwent psychological testing to measure intelligence, visual perception, and psycholinguistic abilities. Psychopathology was also studied in a matched control group. The investigation revealed a broad scale of developmental hazards and psychopathologic symptoms indicating the negative long-range effects of alcohol abuse during pregnancy. The extent of morphologic damage was found to be a good predictor of psychopathology and mental impairment. Social environment and socioeconomic status appear less important as predictors.

Adolescent↗
PubMed1982

Steroid treatment of pseudo-croup.

In a randomized, controlled investigation, 349 children with pseudo-croup stage I or II were given 6 mg of dexamethasone or placebo. Although the rate of recovery was similar in both groups, there was a significant difference when only the more seriously affected children were considered: steroid treated patients recovered quicker. Steroids appear to be useful in the routine treatment of simple, uncomplicated pseudo-croup, even if their effect apparently is not very impressive. Thus, close supervision remains the most important measure in this nearly always benign but potentially dangerous condition.

Acute Disease↗
PubMed1982

Development and psychopathology of children with the fetal alcohol syndrome.

Extended psychiatric and pediatric examinations were performed on patients with the fetal alcohol syndrome (FAS). A subgroup of these patients underwent psychological testing to measure intelligence, visual perception, and psycholinguistic abilities. Psychopathology was also studied in a matched control group. The investigation revealed a broad scale of developmental hazards and psychopathologic symptoms indicating the negative long-range effects of alcohol abuse during pregnancy. The extent of morphologic damage was found to be a good predictor of psychopathology and mental impairment. Social environment and socioeconomic status appear less important as predictors.

Abnormalities, Drug-Induced↗
PubMed1981

[Reye's syndrome (author's transl)].

After a viral infection (Coxsackie-A-9) a twelve-year-old boy developed symptoms of acute encephalopathy and fatty degeneration of the liver. A fatal outcome could not be averted. Clinical course, laboratory data, light and electron microscopic examinations were typical for Reye's syndrome, a rare diagnosis in Central Europe.

Adolescent↗
PubMed1981

[Studies on alcohol embryopathy (author's transl)].

Data from pediatric, neurological, psychiatric and psychological examinations of 71 children suffering from alcoholembryopathy (AE) are reported. In comparison to a matched control group history revealed an increased rate of developmental retardation during the neonatal period and the toddler age. Besides the frequently described morphological characteristics neurological examinations frequently revealed deficits of fine motor coordination. Psychiatric examinations led to various symptoms indicating a marked psychoorganic syndrome. Impairment of cognitive functions was detected by psychological testing. Finally there were positive correlations between morphological and psychological damage.

Child↗