Literature DB >> 6682547

Radiological findings in the hand in Seckel syndrome (bird-headed dwarfism).

A K Poznanski, G Iannaccone, A M Pasquino, B Boscherini.   

Abstract

Two patients with severe intrauterine growth retardation and bird-headed appearance are described. These two children had most of the clinical features of the so-called Seckel dwarfism. The radiological findings included: (1) ivory epiphyses affecting all phalanges in one patient and many phalanges in another; (2) cone-shaped epiphyses in the proximal phalanges; (3) marked disharmonic bone maturation between carpals and phalanges, between individual carpals, and from side to side; (4) alteration in the length of the hand bones, with considerable similarity of the metacarpophalangeal pattern between the two children; (5) relatively small carpals, which have an angular configuration; and (6) relatively normal or increased cortical thickness of the metacarpals.

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Mesh:

Year:  1983        PMID: 6682547     DOI: 10.1007/bf00975661

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


  11 in total

1.  Irregularities of bone maturation in congenitally malformed children.

Authors:  H P SECKEL
Journal:  Ann Paediatr       Date:  1953-09

2.  Disharmonic skeletal maturation in the congenital malformation syndromes.

Authors:  A K Poznanski; S M Garn; L R Kuhns; H A Shaw
Journal:  Birth Defects Orig Artic Ser       Date:  1977

3.  Skeletal measurement in the evaluation of congenital malformation syndromes.

Authors:  A K Poznanski; S M Garn
Journal:  Birth Defects Orig Artic Ser       Date:  1974

4.  Metacarpophalangeal pattern profiles in the evaluation of skeletal malformations.

Authors:  A K Poznanski; S M Garn; J M Nagy; J C Gall
Journal:  Radiology       Date:  1972-07       Impact factor: 11.105

5.  Bone measurement in the differential diagnosis of osteopenia and osteoporosis.

Authors:  S M Garn; A K Poznanski; J M Nagy
Journal:  Radiology       Date:  1971-09       Impact factor: 11.105

6.  Ivory epiphyses of the hands.

Authors:  L R Kuhns; A K Poznanski; H A Harper; S M Garn
Journal:  Radiology       Date:  1973-12       Impact factor: 11.105

7.  Metacarpophalangeal length in the evaluation of skeletal malformation.

Authors:  S M Garn; K P Hertzog; A K Poznanski; J M Nagy
Journal:  Radiology       Date:  1972-11       Impact factor: 11.105

8.  The spectrum of x-ray manifestations in Cockayne's syndrome.

Authors:  A Bensman; C Frauré; H J Kaufmann
Journal:  Skeletal Radiol       Date:  1981       Impact factor: 2.199

9.  Cone-shaped epiphyses of the hands and their diagnostic value. The tricho-rhino-phalangeal syndrome.

Authors:  A Giedion
Journal:  Ann Radiol (Paris)       Date:  1967

10.  Intrauterine growth retardation. A report of two cases with bird-headed appearance, skeletal changes and peripheral GH resistance.

Authors:  B Boscherini; G Iannaccone; C La Cauza; G Mancuso; F Girotti; G Finocchi; A M Pasquino
Journal:  Eur J Pediatr       Date:  1981-10       Impact factor: 3.183

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  4 in total

1.  Ivory and dense epiphyses of the hand: Thiemann disease in three sisters.

Authors:  J G van der Laan; C J Thijn
Journal:  Skeletal Radiol       Date:  1986       Impact factor: 2.199

2.  Neuropathology of Seckel syndrome in fetal stage with evidence of intrauterine developmental retardation.

Authors:  A Hori; K Tamagawa; S W Eber; M Westmeier; I Hansmann
Journal:  Acta Neuropathol       Date:  1987       Impact factor: 17.088

3.  Microcephalic osteodysplastic primordial dwarfism, type II. Report of a case with characteristic skeletal features.

Authors:  T E Herman; N J Mendelsohn; S B Dowton; W H McAlister
Journal:  Pediatr Radiol       Date:  1991

4.  Central nervous system vasculopathy and Seckel syndrome: case illustration and systematic review.

Authors:  Osama Khojah; Saeed Alamoudi; Nouf Aldawsari; Mohammed Babgi; Ahmed Lary
Journal:  Childs Nerv Syst       Date:  2021-08-03       Impact factor: 1.475

  4 in total

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