“…The embryological development of the upper limb proceeds along three axes: proximal-distal, anterior-posterior (referred to postnatally as radial-ulnar by clinicians, because the fetal upper limb rotates during development), and dorsal-ventral 1 …”
Section: Classification and Developmental Biology Of Congenital Anomamentioning
“…The embryological development of the upper limb proceeds along three axes: proximal-distal, anterior-posterior (referred to postnatally as radial-ulnar by clinicians, because the fetal upper limb rotates during development), and dorsal-ventral 1 …”
Section: Classification and Developmental Biology Of Congenital Anomamentioning
“…The formation of the embryonic limb bud is the first step toward normal development of the upper extremity (Daluiski et al 2001). The apical ectodermal ridge (AER) represents a layer of surface ectodermal cells at the distal end of the embryonic limb bud that is responsible for cell signaling and limb differentiation.…”
Section: Incidence and Etiologymentioning
confidence: 99%
“…AER-deficient limb buds treated with FGF-4 were capable of developing normal patterns of limb outgrowth. Niswander and Martin also showed that bone morphogenic protein two (BMP-2) was capable of inhibiting this growth (Niswander and Martin 1993a, b;Niswander et al 1993;Daluiski et al 2001). Studies such as these help to elucidate the role fibroblast growth factors play in the etiology of RLD.…”
Radial longitudinal deficiency (RLD) is a congenital disorder characterized by hypoplasia of both the radius and soft tissue structures on the preaxial side of the forearm. Clinical presentation can vary within a spectrum of severity ranging from mild cosmetic deformity to complete absence of the radius and thumb. Despite over 250 years of investigation, current surgical treatment of this disorder is still inadequate in restoring "normal" function and appearance within the wrist. Shortcomings in surgical management have made a standardized treatment algorithm for RLD controversial; however centralization and radialization remain the mainstay of surgical treatment. This chapter will attempt to provide an overview of the deformity and present a review of treatment options.
“…Several factors are involved in the process of programmed cell death, including the normal interaction between FGFs and their receptors. 13 Transcription factor Msx2 is highly expressed in the interdigital mesenchyme and is thought to be a regulator in the BMP4-mediated, programmed cell death pathway. 14 Similarly, the transcription factor Hox-7 is expressed in the interdigital zones having programmed cell death.…”
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