[Genetic counseling and ultrasonographic prenatal diagnosis of skeletal dysplasias in monogenic and chromosomal syndromes]

Akush Ginekol (Mosk). 1990 Nov:(11):20-6.
[Article in Russian]

Abstract

The paper reports sonographic prenatal diagnosis of monogenic and chromosomal syndromes (congenital malformations) which are associated with skeletal abnormalities: autosomal recessive syndromes (shortribs--polydactyly, type II; dyssegmental dwarfism, type II; Pen-Shokeir disease type I), autosomal dominant syndromes (Larsen's syndrome, achondroplasia, Schmidt's metaphyseal dysplasia of segregating partial chromosome of 7 long-arm trisomy). All cases were detected in the second trimester; diagnosis of chromosomal diseases employed the amniocentesis. In aborted pregnancies, diagnoses were confirmed at autopsy. Pathognomonic sonographic markers were described for every syndrome; numerous conditions were differentiated from similar syndromes. Genetic counseling identified recurrence risks and offspring prognosis. Potentials of sonographic diagnosis of systemic skeletal dysplasia and a need for a syndrome-oriented approach to prenatal diagnosis are discussed.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnostic imaging*
  • Abnormalities, Multiple / etiology
  • Abnormalities, Multiple / genetics
  • Adult
  • Bone Diseases, Developmental / diagnostic imaging*
  • Bone Diseases, Developmental / etiology
  • Bone Diseases, Developmental / genetics
  • Chromosome Aberrations / genetics*
  • Chromosome Disorders
  • Female
  • Genetic Counseling
  • Humans
  • Male
  • Pedigree
  • Pregnancy
  • Ultrasonography, Prenatal*