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[Chromosome abnormalities, tumours and developmental disorders (author's transl)].

Clonal chromosome disorders occurring or acquired at any postnatal age are often closely related with the origin of tumours. In man the Ph1-chromosome (9; 22) anomaly in CML or the 8; 14 translocation in the African malignant Burkitt Non-Hodgkin lymphoma are, among other cases, prominent examples. On the other hand, constitutive, inherited or novel chromosome anomalies conveyed from the zygote to all tissues of the organism may cause a higher risk for the origin of tumours. Rarely, inheritable minor structural chromosome mutations are known to determine the occurrence of dysontogenetic tumours, as e.g., nephroblastoma, but it is assumed that more such cases will become elucidated in the future. As a special phenomenon, true hydatiform mole is a tumour of the placental tissue due to a disorder of intragenome regulation. Constitutive or numerical structural chromosome anomalies of man are a frequent cause of early or late abortion or of abnormal development and malformation. Despite the predominating principle of selective fetal elimination, a few anomalies such as Down's syndrome, may escape to longer survival due to the relatively mild effects of chromosome 21 triplication. Trisomies which represent in man the most frequent type of chromosome disorders, can be induced, and systematically studied in an experimental model of the mouse. This allows the elaboration of the developmental profiles of all trisomies (and monosomies) of the mouse. Also, the above mentioned principle of selective elimination of abnormal implants can be analysed experimentally. Although the developmental span of a trisomic zygote is limited, there is evidence that cells and tissues isolated from the chromosomally abnormal organism can survive much longer. Thus, haemopoietic stem cells, at least in Ts 12 and 19 of the mouse, can be rescued from trisomic fetuses by transferring them to lethally irradiated adult mice, whose blood forming organs may eventually become permanently repopulated by the trisomic cell lineage. This type of experiments is suited for closer analyses of potential functions vs. defects of chromosomally abnormal cellular systems, e.g., with regard to growth and development.

Animals↗

Attention deficit disorder with developmental coordination disorders.

AIMS: To analyse the contribution of certain social, familial, prenatal, perinatal, and developmental background factors in the pathogenesis of deficits in attention, motor control, and perception (DAMP). METHODS: A population based case-control study was carried out with 113 children aged 6 years, 62 diagnosed with DAMP and 51 controls without DAMP. The children's health and medical records were studied and their history with regard to background factors was taken at an interview with the mother using a standardised schedule. Familial factors, possible non-optimal factors during pregnancy (including smoking), developmental factors (including early language development), and medical and psychosocial data were scored in accordance with the reduced optimality method. RESULTS: Low socioeconomic class was common in the group with DAMP. Familial language disorder and familial motor clumsiness were found at higher rates in the DAMP group. Neuropathogenic risk factors in utero were also more common in the children with DAMP. Maternal smoking during pregnancy appeared to be an important risk factor. Language problems were present in two thirds of the children with DAMP. Sleep problems and gastrointestinal disorders, but not atopy or otitis media, were significantly more common in the DAMP group. CONCLUSIONS: Prenatal familial and neuropathogenic risk factors contribute to the development of DAMP. Primary prevention, such as improved maternal health care and early detection or treatment, or both, of associated language problems appear to be essential.

Attention Deficit Disorder with Hyperactivity↗

Annotation: the cognitive neuroscience of face recognition: implications for developmental disorders.

Face recognition is often considered to be a modular (encapsulated) function. This annotation supports the proposal that faces are special, but suggests that their identification makes use of general-purpose cortical systems that are implicated in high-level vision and also in memory and learning more generally. These systems can be considered to function within two distinct cortical streams: a medial stream (for learning and salience of faces encountered) and a lateral stream (for distributed representations of visual properties and identities of faces). Function in the lateral stream, especially, may be critically dependent on the normal development of magnocellular vision. The relevance of face recognition anomalies in three developmental syndromes (Autism, Williams syndrome, and Turner syndrome) and the two-route model sketched above is considered.

Autistic Disorder↗

[Follow-up of psychiatric disorders and specific developmental delay disorders in special education students].

In a prospective longitudinal study 72 children who were attending a special school for children with learning disabilities in Erlangen were examined in 1990 and again in 1992/93. At the first examination the children's mean age was 7 years 1 month and at the second 8 years 10 months. The sample contained many more boys than girls. Reported here are the findings on the course of the psychiatric disorders and the specific developmental disorders of speech and language and motor function. There was a marked decrease in the prevalence of psychiatric disorders. Of the 17 children with a psychiatric disorder at follow-up, 13 (76%) also had a disorder at the start of the study and of the 23 with a psychiatric disorder at the start 13 (57%) still had a disorder at follow-up. Children with enuresis and those with disturbances of activity or attention had the best prognosis. The special school had a positive effect on conduct. The prevalence of specific developmental disorders also decreased over the two-year period. As expected, specific speech articulation disorders had the best prognosis. All other disorders had a poor prognosis. Increases in prevalence were mainly the result of different cutoff points for a given diagnosis at different ages. The high number of specific disorders of motor function at both time points is especially noteworthy.

Child↗

[The role of developmental disorders of the respiratory, digestive and vascular systems in pathology of the lungs in children].

The results of diagnosis and treatment of bronchopulmonary pathology in 680 children were analysed. It was found that besides developmental anomalies of the lungs and trachea, those of the esophagus, mediastinal organs, diaphragm, and the aorta and its vessels play an important role in the pathogenesis of pulmonary diseases in infants and young children. Traditional and special methods of examination must be used in the diagnosis of these developmental anomalies. Timely correction of the developmental anomalies makes it possible to arrest the bronchopulmonary complications which are associated with them.

Animals↗