Literature DB >> 27677752

Grebe dysplasia - prenatal diagnosis based on rendered 3-D ultrasound images of fetal limbs.

Luis F Goncalves1,2, Julie A Berger3, Jacqueline K Macknis4, Samuel T Bauer3, David A Bloom5,6.   

Abstract

Grebe dysplasia is a rare skeletal dysplasia characterized by severe acromesomelic shortening of the long bones in a proximal to distal gradient of severity, with bones of the hands and feet more severely affected than those of the forearms and legs, which in turn are more severely affected than the humeri and femora. In addition, the bones of the lower extremities tend to be more severely affected than the bones of the upper extremities. Despite the severe skeletal deformities, the condition is not lethal and surviving individuals can have normal intelligence. Herein we report a case of Grebe dysplasia diagnosed at 20 weeks of gestation. Rendered 3-D ultrasound images of the fetal limbs, particularly of the characteristic tiny and globular-looking fingers and toes, were instrumental in accurately characterizing the phenotype prenatally.

Entities:  

Keywords:  Fetus; Grebe dysplasia; Limbs; Prenatal diagnosis; Skeletal dysplasia; Three-dimensional; Ultrasound

Mesh:

Year:  2016        PMID: 27677752     DOI: 10.1007/s00247-016-3705-9

Source DB:  PubMed          Journal:  Pediatr Radiol        ISSN: 0301-0449


  6 in total

1.  A NEW TYPE OF DWARFISM WITH VARIOUS BONE APLASIAS AND HYPOPLASIAS OF THE EXTREMITIES.

Authors:  A QUELCE-SALGADO
Journal:  Acta Genet Stat Med       Date:  1964

2.  Prenatal sonographic diagnosis of Grebe syndrome.

Authors:  Dwight R Cordero; Yael Goldberg; Donald Basel; Michael W Kilpatrick; Susan Klugman; Petros Tsipouras; Susan Gross
Journal:  J Ultrasound Med       Date:  2006-01       Impact factor: 2.153

3.  Grebe syndrome: clinical and radiographic findings in affected individuals and heterozygous carriers.

Authors:  T Costa; G Ramsby; F Cassia; K R Peters; J Soares; J Correa; A Quelce-Salgado; P Tsipouras
Journal:  Am J Med Genet       Date:  1998-02-17

4.  Grebe-Quelce-Salgado chondrodystrophy: prenatal diagnosis of two new cases in unrelated families in Southern Brazil.

Authors:  María Verónica Muñoz Rojas; Luís Flávio Gonçalves
Journal:  Am J Med Genet       Date:  2002-11-22

5.  Nosology and classification of genetic skeletal disorders: 2015 revision.

Authors:  Luisa Bonafe; Valerie Cormier-Daire; Christine Hall; Ralph Lachman; Geert Mortier; Stefan Mundlos; Gen Nishimura; Luca Sangiorgi; Ravi Savarirayan; David Sillence; Jürgen Spranger; Andrea Superti-Furga; Matthew Warman; Sheila Unger
Journal:  Am J Med Genet A       Date:  2015-09-23       Impact factor: 2.802

6.  Suspected skeletal dysplasias: femur length to abdominal circumference ratio can be used in ultrasonographic prediction of fetal outcome.

Authors:  A Rahemtullah; B McGillivray; R D Wilson
Journal:  Am J Obstet Gynecol       Date:  1997-10       Impact factor: 8.661

  6 in total

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