Literature DB >> 32347827

Thanatophoric Skeletal Dysplasia: A Case Report.

Firoz Anjum1, Sunil Kumar Daha1, Ganesh Shah1.   

Abstract

Thanatophoric skeletal dysplasiais the most lethal, rare, sporadic birth defect due to de novo mutation in the fibroblast growth factor receptor-3. Clinically this is characterized by shortening of the limbs (micromelia), small conical thorax, flat vertebral bodies and macrocephaly at birth. We encountered a similar case with ultrasonographic findings suggestive of Thanatophoric Skeletal Dysplasia which resulted in the death of the baby within an hour of birth. Almost all cases of this condition have been reported to have died interuterinally or a few days after birth.

Entities:  

Keywords:  oligohydramnios; Potter Sequence; Potter’s facies; pulmonary hypoplasia.

Mesh:

Year:  2020        PMID: 32347827      PMCID: PMC7580312     

Source DB:  PubMed          Journal:  JNMA J Nepal Med Assoc        ISSN: 0028-2715            Impact factor:   0.406


  7 in total

1.  Thanatophoric dysplasia: a rare entity.

Authors:  N S Naveen; B V Murlimanju; Vishal Kumar; Thejodhar Pulakunta
Journal:  Oman Med J       Date:  2011-05

2.  A case of thanatophoric dysplasia: the early prenatal 2D and 3D sonographic findings and molecular confirmation of diagnosis.

Authors:  H S Wong; A Kidd; J Zuccollo; J Tuohy; L Strand; J Tait; K C Pringle
Journal:  Fetal Diagn Ther       Date:  2008-05-27       Impact factor: 2.587

3.  Long-term survival in typical thanatophoric dysplasia type 1.

Authors:  K M Baker; D S Olson; C O Harding; R M Pauli
Journal:  Am J Med Genet       Date:  1997-06-27

4.  Neural arch stenosis and spinal cord injury in thanatophoric dysplasia.

Authors:  O M Faye-Petersen; A S Knisely
Journal:  Am J Dis Child       Date:  1991-01

5.  Thanatophoric Dysplasia: A Case Report.

Authors:  Manisha Sharma; Rekha Jain
Journal:  J Clin Diagn Res       Date:  2015-11-01

6.  Molecular, radiologic, and histopathologic correlations in thanatophoric dysplasia.

Authors:  W R Wilcox; P L Tavormina; D Krakow; H Kitoh; R S Lachman; J J Wasmuth; L M Thompson; D L Rimoin
Journal:  Am J Med Genet       Date:  1998-07-07

Review 7.  Nosology and classification of genetic skeletal disorders: 2010 revision.

Authors:  Matthew L Warman; Valerie Cormier-Daire; Christine Hall; Deborah Krakow; Ralph Lachman; Martine LeMerrer; Geert Mortier; Stefan Mundlos; Gen Nishimura; David L Rimoin; Stephen Robertson; Ravi Savarirayan; David Sillence; Juergen Spranger; Sheila Unger; Bernhard Zabel; Andrea Superti-Furga
Journal:  Am J Med Genet A       Date:  2011-03-15       Impact factor: 2.802

  7 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.