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Biomedical subjects

P Naumoff

Publications and source records attributed to P Naumoff.

5 recordsLinked to original sources

Ultrasonography of partial hydatidiform mole.

Partial hydatidiform mole differs from complete mole by its focal distribution, its slower transformation, the presence of an embryo or fetus, and the triploid karyotype. Nineteen pathologically proved cases are presented. Partial mole can be diagnosed by a combination of the following sonographic findings: (a) greatly enlarged placenta relative to the size of the uterine cavity, (b) cystic spaces within the placenta ("molar placenta"), which may not always be present, (c) an amniotic cavity (gestational sac), either empty or containing amorphous fetal echoes, and (d) a well-formed but growth-retarded fetus, either dead or alive.

Abortion, Spontaneous↗

Thoracic dysplasia in spondyloepiphyseal dysplasia congenita.

Spondyloepiphyseal dysplasia congenita (SED congenita) can involve the ribs. The result is rib shortening, a dysplastic thorax that is not only short but also narrowed, and respiratory failure. This case involves an infant who died of respiratory failure 36 hours after birth. A dysplastic small volume thorax in infancy, leading to serious respiratory embarrassment, must be included into the clinical and radiological pictures of SED congenita. To my knowledge, rib shortening and thoracic dysplasia in SED congenita have not been previously described.

Humans↗

Short rib-polydactyly syndrome type 3.

Three infants died perinatally of asphyxia due to thoracic narrowing. Their uniform radiographic findings are different from previously described and recognized chondrodysplasias and possibly represent a distinct syndrome. Three identical or very similar cases are found in the literature.

Bone Diseases, Developmental↗

Heterogeneity of metatropic dysplasia.

Metatropic dysplasia is a neonatally manifest entity that is characterized clinically by a rapidly progressing kyphoscoliosis leading to severe shortening of the originally long trunk ("metatropism"). Major radiographic features include flattening and defective ossification of the vertebral bodies, a narrow thorax and a marked hypoplasia of the basilar portions of the ilia with crescent-shaped iliac crests. There is some evidence of genetic heterogeneity. From five personal observations and from a review of the literature we conclude that metatropic dysplasia comprises at least three genetic entities: (1) a nonlethal type with autosomal recessive transmission; (2) a nonlethal dominant type and (3) a lethal type with death before or shortly after birth and possibly autosomal recessive inheritance. A careful clinical and radiologic analysis permits their classification and differentiation from similar, not yet well-delineated disorders. Attention is also drawn to the possibility of atlanto-axial instability in metatropic dysplasia which may cause severe neurologic defects.

Adult↗