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T I Ostrovskaia

Publications and source records attributed to T I Ostrovskaia.

9 recordsLinked to original sources
PubMed1985

[CNS pathology in hereditary syndromes of congenital developmental defects].

Analysis of the incidence and diagnostic importance of the congenital central nervous system (CNS) defects in 334 children with multiple congenital developmental defects (MCDD) dying under 1 year allowed the author to distinguish 4 groups of defects according to their informative value for establishing the nosological diagnosis: defects of a high, moderate, small informative value and noninformative ones. Special microscopic study of the CNS performed in 116 stillborns and children of the first year of life with various MCDD showed the disturbance of main morphogenetic processes (mitotic cell activity, cell migration, differentiation and maturation) as a basis of the observed pathological conditions. General rules of the CNS developmental defects in chromosomal and non-chromosomal syndromes may point to the common routes of the genetic material realization.

Abnormalities, Multiple↗
PubMed1984

[Pathological anatomy of monogenic syndromes of multiple congenital developmental defects].

Morphological study, with no selection, is performed of 1106 stillborns and children died before one year of age with congenital malformations (CM), 536 (46.9 +/- 1.5%) among them having multiple CM. Syndrome diagnosis is made in 44.1 +/- 2.1% cases: in 26.9 +/- 1.9% among them these were syndromes of chromosomal and in 17.9 +/- 1.7% syndromes of non-chromosomal etiology. Monogenic hereditary forms among non-chromosomal syndromes were in 42.1 +/- 4.8%. Comparative analysis of frequency and types of CM of various systems in monogenic and chromosomal syndromes and unclassified CM showed that the monogenic syndromes by their phenotypic manifestations are close to the chromosomal syndromes. The uniformity of alterations of certain morphological structures in these syndromes appear to be the consequence of common ways of realisation of both mutant genes in monogenic syndromes and their imbalance in chromosomal syndromes.

Abnormalities, Multiple↗
PubMed1983

[Pathology of hydranencephaly].

Data on pathological manifestations of hydranencephaly (3 cases) consisting of complete or nearly complete absence of cerebral hemispheres with the intact stem, cerebellum, spinal cord, meninges and skull bones are presented. A number of structural disorders were found in the remaining structures of the brain. A hypothesis on the origin of this central nervous system developmental defect is proposed: hydranencephaly is associated with disorders in the development of leptomeninges at the early stages of embryogenesis.

Adult↗
PubMed1980

[Bowen-Conrad syndrome].

An observation of multiple malformation defects (Bowen-Conradi syndrome) is presented. The main morphological manifestations included: marked prenatal hypoplasia, facial dysplasias, microgeny, clinodactylia of little fingers, hypospadia, cryptorchism, changes in the central nervous system. All the patients with this syndrome die within the first year of life. The disease is inherited by the autosome-recessive type.

Abnormalities, Multiple↗
PubMed1979

[Pathologic anatomy of the Wolf-Hirschhorn syndrome (partial monosomy 4p--)].

An analysis of phenotypic manifestations of Wolf-Hirschhorn syndrome from the 60 cases most thoroughly described in the literature and 3 own observations was done. Most characteristic malformations of this syndrome were shown to include coracoid nose and hypertelorism, coloboma of the eyes, hypospadia, aplasia, hypoplasia and polycystosis of the kidneys, dystopia and dysplasia of the cerebellar gyri, shortening of H2 field of the Ammon's horn with imparied orientation of its neurons, sacral sinus, and retarded bone maturation.

Abnormalities, Multiple↗
PubMed1978

[Multiple developmental defects. The Neu-Povýsilová syndrome].

An observation of Neu-Povýsilová lethal syndrome of multiple congenital malformations and data on 6 cases of this syndrome described in the literature are presented. The main morphological manifestations of the syndrome include markedly manifest prenatal hypoplasia, defects of the CNS and the extremities. The presumed type of heredity is autosomal-recessive.

Abnormalities, Multiple↗