Literature DB >> 11343321

Hajdu--Cheney syndrome: evolution of phenotype and clinical problems.

A M Brennan1, R M Pauli.   

Abstract

Hajdu-Cheney syndrome is a rare, autosomal dominant disorder comprising acroosteolysis of the distal phalanges with associated digital abnormalities, distinctive craniofacial and skull changes, dental anomalies, and proportionate short stature. The clinical and radiologic characteristics of Hajdu-Cheney syndrome develop and progress with age. Many of the medical problems that arise in this syndrome cluster in specific age ranges. Case reports of six affected individuals in two additional families and a summary of the English literature is presented with emphasis on the changing physical findings and medical sequelae over time.

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Year:  2001        PMID: 11343321     DOI: 10.1002/1096-8628(20010515)100:4<292::aid-ajmg1308>3.0.co;2-4

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


  38 in total

Review 1.  Notch and the regulation of osteoclast differentiation and function.

Authors:  Jungeun Yu; Ernesto Canalis
Journal:  Bone       Date:  2020-06-08       Impact factor: 4.398

2.  [Young woman with back pain and acro-osteolysis].

Authors:  S Schawo; M-A Weber; M Libicher
Journal:  Radiologe       Date:  2006-10       Impact factor: 0.635

Review 3.  Specific entities affecting the craniocervical region: osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management of basilar impression.

Authors:  Arnold H Menezes
Journal:  Childs Nerv Syst       Date:  2008-04-10       Impact factor: 1.475

4.  NOTCH2 Hajdu-Cheney Mutations Escape SCFFBW7-Dependent Proteolysis to Promote Osteoporosis.

Authors:  Hidefumi Fukushima; Kouhei Shimizu; Asami Watahiki; Seira Hoshikawa; Tomoki Kosho; Daiju Oba; Seiji Sakano; Makiko Arakaki; Aya Yamada; Katsuyuki Nagashima; Koji Okabe; Satoshi Fukumoto; Eijiro Jimi; Anna Bigas; Keiichi I Nakayama; Keiko Nakayama; Yoko Aoki; Wenyi Wei; Hiroyuki Inuzuka
Journal:  Mol Cell       Date:  2017-11-16       Impact factor: 17.970

Review 5.  Notch Signaling and the Skeleton.

Authors:  Stefano Zanotti; Ernesto Canalis
Journal:  Endocr Rev       Date:  2016-04-13       Impact factor: 19.871

Review 6.  Extreme proximal junctional kyphosis-a complication of delayed lambdoid suture closure in Hajdu-Cheney syndrome: a case report and literature review.

Authors:  Colin Y L Woon; Steven M Mardjetko
Journal:  Eur Spine J       Date:  2017-11-04       Impact factor: 3.134

7.  Mutations in NOTCH2 cause Hajdu-Cheney syndrome, a disorder of severe and progressive bone loss.

Authors:  Michael A Simpson; Melita D Irving; Esra Asilmaz; Mary J Gray; Dimitra Dafou; Frances V Elmslie; Sahar Mansour; Sue E Holder; Caroline E Brain; Barbara K Burton; Katherine H Kim; Richard M Pauli; Salim Aftimos; Helen Stewart; Chong Ae Kim; Muriel Holder-Espinasse; Stephen P Robertson; William M Drake; Richard C Trembath
Journal:  Nat Genet       Date:  2011-03-06       Impact factor: 38.330

8.  Serpentine fibula polycystic kidney syndrome is part of the phenotypic spectrum of Hajdu-Cheney syndrome.

Authors:  Mary J Gray; Chong Ae Kim; Debora Romeo Bertola; Paula Ricci Arantes; Helen Stewart; Michael A Simpson; Melita D Irving; Stephen P Robertson
Journal:  Eur J Hum Genet       Date:  2011-06-29       Impact factor: 4.246

9.  Mutations in NOTCH2 in patients with Hajdu-Cheney syndrome.

Authors:  W Zhao; E Petit; R I Gafni; M T Collins; P G Robey; M Seton; K K Miller; M Mannstadt
Journal:  Osteoporos Int       Date:  2013-02-07       Impact factor: 4.507

Review 10.  Hajdu-Cheney syndrome associated with serpentine fibulae and polycystic kidney disease.

Authors:  Guido Currarino
Journal:  Pediatr Radiol       Date:  2008-09-25
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