Ruggieri Victor, Arberas Claudia
Servicio de Neurología, Hospital de Pediatría Juan P. Garrahan, Combate De Los Pozos 1881, Buenos Aires, Argentina.
Medicina (B Aires). 2007;67(6 Pt 1):569-85.
Pervasive developmental disorders (PDD) encompass a heterogeneous group of children with deficits of verbal and non-verbal language, social communication, and with a restricted repertoire of activities or repetitive behaviours. The frequency in general population is considered 27.5/10,000. In this study, we analyzed the clinical and genetic aspects of Autism, Asperger Syndrome, PDD Not Otherwise Specified, Rett Syndrome and Childhood Disintegrative Disorder. We analyzed clinical, behavioural and neuropsychological features. We revised different medical genetics associated conditions and divided the genetics aspects of pervasive developmental disorders into two groups: Syndromic forms (around 20%) and non syndromic forms (currently proposed to be 80%). The early recognition of pervasive developmental disorders and the diagnosis of specific associated syndromes allow early therapy, correct genetic counselling, and follow up anticipating possible complications related to the entity. Finally, although the genetic bases of autism have not yet been identified, the following candidate genes have been proposed: 15q, 2q, 17q, 7q, 12q, and X related genes, among others; which are analyzed in this study and will allow a better understanding of these disorders in the future.
广泛性发育障碍(PDD)涵盖了一组异质性儿童,他们存在言语和非言语语言缺陷、社会交往障碍,以及活动范围受限或重复行为。一般人群中的发病率被认为是27.5/10000。在本研究中,我们分析了孤独症、阿斯伯格综合征、未特定的广泛性发育障碍、雷特综合征和儿童瓦解性精神障碍的临床和遗传学方面。我们分析了临床、行为和神经心理学特征。我们回顾了不同的医学遗传学相关病症,并将广泛性发育障碍的遗传学方面分为两组:综合征形式(约20%)和非综合征形式(目前认为是80%)。对广泛性发育障碍的早期识别以及对特定相关综合征的诊断有助于早期治疗、正确的遗传咨询,并对与该疾病相关的可能并发症进行随访。最后,尽管孤独症的遗传基础尚未确定,但已提出以下候选基因:15q、2q、17q、7q、12q以及X相关基因等;本研究对这些基因进行了分析,未来将有助于更好地理解这些疾病。