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Gene Review

ZLS  -  Zimmerman-Laband Syndrome

Homo sapiens

 
 
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Disease relevance of ZLS

  • Zimmermann-Laband syndrome (ZLS) is characterised by findings of coarse facial appearance, hepatosplenomegaly, and hirsutism often first observed in infancy, followed by the evolution during childhood of gingival fibromatosis, small joint hyperextensibility, and hypoplasia of the finger- and toenails [1].
  • A young man was diagnosed as having Zimmerman-Laband syndrome (ZLS) on the basis of gingival fibromatosis and absence of nails on thumbs and halluces in addition to other anomalies [2].
  • Zimmermann-Laband syndrome (ZLS) is a very rare autosomal dominant inherited condition characterized by 3 major clinical findings of which gingival hyperplasia are always present [3].
  • A male with 46,XY,t(3;17)(p14.3;q24.3) presented with gingival hyperplasia, hypertrichosis, unusually large ears and marked hypertrophy of the nose, characteristic of the Zimmermann-Laband syndrome (ZLS) [4].
  • Zimmermann-Laband syndrome in a patient with severe mental retardation: The Zimmermann-Laband syndrome (ZLS) is a rare autosomal dominant disorder characterized by gingival hyperplasia or fibromatosis, various skeletal anomalies including dysplasia of the distal phalanges of thumbs and halluces, vertebral defects, and hepatosplenomegaly [5].
 

Psychiatry related information on ZLS

 

High impact information on ZLS

  • We report on a four decade follow-up of a male with ZLS who developed a cardiomyopathy and dilatation of the aortic root and arch, anomalies hitherto undescribed in this syndrome [1].
  • The purpose of the study was to examine a patient diagnosed with ZLS and to describe possible new characteristics of this rare syndrome, including the ultrastructural morphology using a transmission electron microscope (TEM) of the gingival and dermal fibroblasts [3].

References

  1. Zimmermann-Laband syndrome in an adult. Long-term follow-up of a patient with vascular and cardiac complications. Robertson, S.P., Lipp, H., Bankier, A. Am. J. Med. Genet. (1998) [Pubmed]
  2. Zimmerman-Laband syndrome and profound mental retardation. Chodirker, B.N., Chudley, A.E., Toffler, M.A., Reed, M.H. Am. J. Med. Genet. (1986) [Pubmed]
  3. Ultrastructural investigation of Zimmermann-Laband syndrome. Hoogendijk, C.F., Marx, J., Honey, E.M., Pretorius, E., Christianson, A.L. Ultrastructural pathology (2006) [Pubmed]
  4. Candidate loci for Zimmermann-Laband syndrome at 3p14.3. Kim, H.G., Higgins, A.W., Herrick, S.R., Kishikawa, S., Nicholson, L., Kutsche, K., Ligon, A.H., Harris, D.J., Macdonald, M.E., Bruns, G.A., Morton, C.C., Quade, B.J., Gusella, J.F. Am. J. Med. Genet. A (2007) [Pubmed]
  5. Zimmermann-Laband syndrome in a patient with severe mental retardation. Van Buggenhout, G.J., Brunner, H.G., Trommelen, J.C., Hamel, B.C. Genetic counseling (Geneva, Switzerland) (1995) [Pubmed]
 
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