Thanatophoric dysplasia in identical twins.

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Thanatophoric dysplasia in identical twins.

Female twins concordant for thanatophoric dysplasia are presented. Monozygosity was confirmed using minisatellite DNA genetic fingerprinting. The evidence supporting new dominant mutations as the likely cause of thanatophoric dysplasia is reviewed.

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Thanatophoric dysplasia.

Two autopsy cases of thanatophoric dysplasia in male newborn infant are described. They have markedly short-limbs and narrow thoraxes. Histologically, generalized disruption of endochondral ossification with normal resting cartilage was seen at the epiphyseal region of femur. Differential diagnosis from other 3 types of lethal chondrodysplasia is described and the histogenesis of thanatophoric ...

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Thanatophoric dysplasia: a rare entity.

Thanatophoric dysplasia is the lethal skeletal dysplasia characterized by marked underdevelopment of the skeleton and short-limb dwarfism. The child will be having a short neck, narrow thoracic cage and protuberant abdomen. Other anatomical features include a relatively enlarged head with frontal bossing, prominent eyes, hypertelorism and the depressed nasal bridge. The diagnosis is usually mad...

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Thanatophoric Dysplasia in Newborn Twins:Case Report and Literature Review

  Thanatophoricdysplasia (TD) isanosteochondrodysplasiaalwayslethalintheneonatalperiod. Thevastmajorityofcasesareduetodenovomutations. Itisdividedintotwotypes: ashortcurvedfemurcharacterizestype 1, whileastraighterfemurwithcloverleafskullcharacterizestype 2. Inthanatophoricdysplasiathelimbsareveryshort. Theribcageissmall. Thevertebralbodiesofthespinearegreatlyreducedinheightwithwidespacesbetwee...

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Narcolepsy in identical twins.

Narcolepsy has been observed in chronic encephalitis, cerebral arteriosclerosis, brain tumour, and following trauma, but when these cases are excluded there remains a group without demonstrable brain pathology. The existence of this idiopathic group has been a controversial subject. Among the more impressive evidence is that suggesting a hereditary factor in idiopathic narcolepsy. To date this ...

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ژورنال

عنوان ژورنال: Journal of Medical Genetics

سال: 1989

ISSN: 1468-6244

DOI: 10.1136/jmg.26.4.276