Literature DB >> 8213919

Hydrops-ectopic calcification-moth-eaten skeletal dysplasia (Greenberg dysplasia): prenatal diagnosis and further delineation of a rare genetic disorder.

D Chitayat1, H Gruber, B J Mullen, D Pauzner, T Costa, R Lachman, D L Rimoin.   

Abstract

An offspring of consanguineous parents of East-Indian origin was found prenatally to have hydrops fetalis, short limb dwarfism, polydactyly and chondro-osseous changes consistent with Greenberg hydrops-ectopic calcification-moth-eaten skeletal dysplasia (HEM) dysplasia. The radiological changes include platyspondyly with multiple extra ossification centers, extraneous calcification in the ribs, sternum, pelvis, and epiphysis, and moth-eaten long bones. The histopathological changes included chondrocytes with dilated rough endoplasmic reticulum and inclusion bodies with homogeneous material of intermediate electron density. These findings further delineate the spectrum of this rare autosomal recessive skeletal dysplasia.

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Mesh:

Year:  1993        PMID: 8213919     DOI: 10.1002/ajmg.1320470226

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  9 in total

Review 1.  Malformation syndromes caused by disorders of cholesterol synthesis.

Authors:  Forbes D Porter; Gail E Herman
Journal:  J Lipid Res       Date:  2010-10-07       Impact factor: 5.922

Review 2.  Radiographic features of the skeleton in disorders of post-squalene cholesterol biosynthesis.

Authors:  Massimiliano Rossi; Christine M Hall; Raymonde Bouvier; Sophie Collardeau-Frachon; Frédérique Le Breton; Martine Bucourt; Marie Pierre Cordier; Christine Vianey-Saban; Giancarlo Parenti; Generoso Andria; Martine Le Merrer; Patrick Edery; Amaka C Offiah
Journal:  Pediatr Radiol       Date:  2015-02-03

3.  Mutations causing Greenberg dysplasia but not Pelger anomaly uncouple enzymatic from structural functions of a nuclear membrane protein.

Authors:  Peter Clayton; Björn Fischer; Anuska Mann; Sahar Mansour; Eva Rossier; Markus Veen; Christine Lang; Sevjidmaa Baasanjav; Moritz Kieslich; Katja Brossuleit; Sophia Gravemann; Nele Schnipper; Mohsen Karbasyian; Ilja Demuth; Monika Zwerger; Amparo Vaya; Gerd Utermann; Stefan Mundlos; Sigmar Stricker; Karl Sperling; Katrin Hoffmann
Journal:  Nucleus       Date:  2010-05-21       Impact factor: 4.197

4.  Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in the lamin B receptor gene.

Authors:  Hans R Waterham; Janet Koster; Petra Mooyer; Gerard van Noort Gv; Richard I Kelley; William R Wilcox; Ronald J A Wanders; Raoul C M Hennekam; Jan C Oosterwijk
Journal:  Am J Hum Genet       Date:  2003-02-28       Impact factor: 11.025

Review 5.  At the nuclear envelope of bone mechanobiology.

Authors:  Scott Birks; Gunes Uzer
Journal:  Bone       Date:  2021-05-26       Impact factor: 4.626

6.  Pelger-Huët anomaly and Greenberg skeletal dysplasia: LBR-associated diseases of cholesterol metabolism.

Authors:  Elizabeth M Turner; Christian Schlieker
Journal:  Rare Dis       Date:  2016-09-27

7.  Greenberg Skeletal Dysplasia: first reported case in the Democratic Republic of Congo.

Authors:  Toni Kasole Lubala; Nina Lubala; Arthur Ndundula Munkana; Adonis Muganza Nyenga; Augustin Mulangu Mutombo
Journal:  Pan Afr Med J       Date:  2013-02-11

8.  The Lamin B receptor is essential for cholesterol synthesis and perturbed by disease-causing mutations.

Authors:  Pei-Ling Tsai; Chenguang Zhao; Elizabeth Turner; Christian Schlieker
Journal:  Elife       Date:  2016-06-23       Impact factor: 8.140

Review 9.  A new case of Greenberg dysplasia and literature review suggest that Greenberg dysplasia, dappled diaphyseal dysplasia, and Astley-Kendall dysplasia are allelic disorders.

Authors:  Pernille A Gregersen; Victoria McKay; Maie Walsh; Erica Brown; George McGillivray; Ravi Savarirayan
Journal:  Mol Genet Genomic Med       Date:  2020-04-18       Impact factor: 2.183

  9 in total

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