dislocation of the thumb carpometacarpal joint is a rare injury, and only 38 cases have been definitively described in the english-speaking literature. all the reported dislocations have been dorsal. we present a volar form of the thumb carpometacarpal dislocation which was managed surgically after three months from the injury with open reduction and volar ligament reconstrction, with satisfact...

We report eight cases of a lethal association of failure to thrive, facial dysmorphism, ambiguous genitalia, syndactyly, postaxial polydactyly, and internal developmental anomalies (Hirschsprung's disease, cardiac and renal malformation). This syndrome is likely to be autosomal recessive and resembles Smith-Lemli-Opitz (SLO) syndrome. However, the lethality, the common occurrence of polydactyly...

UNLABELLED To report the frequencies of congenital hand diseases in patients who underwent surgery on a collective mobilization basis at SOS Hand, Recife, Pernambuco, between 2005 and 2009. METHODS Information was collected from 833 children and adolescents who were examined in eight missions. RESULTS Among the patients, 306 (36.7%) underwent surgery: 240 (78.4%) because of congenital malfo...

short rib polydactyly syndrome (srps) is a very rare congenital anomaly that is classified into four subtypes. it is an autosomal recessive inherited disease. we report a case of this syndrome without a previous family history of congenital defects.

This study evaluated the generalization, maintenance, and negative side-effects of Habit Reversal (HR) and Differential Reinforcement of Other Behaviour (DRO) in the elimination of thumb-sucking. Thirty children who sucked their thumbs or fingers excessively were randomly assigned to a HR, DRO or wait-list control group. Observation data were collected in three observation settings. Both proced...

Gli3 is a major regulator of Hedgehog signaling during limb development. In the anterior mesenchyme, GLI3 is proteolytically processed into GLI3R, a truncated repressor form that inhibits Hedgehog signaling. Although numerous studies have identified mechanisms that regulate Gli3 function in vitro, it is not completely understood how Gli3 function is regulated in vivo. In this study, we show a n...

The homeobox-containing genes Msx1 and Msx2 are highly expressed in the limb field from the earliest stages of limb formation and, subsequently, in both the apical ectodermal ridge and underlying mesenchyme. However, mice homozygous for a null mutation in either Msx1 or Msx2 do not display abnormalities in limb development. By contrast, Msx1; Msx2 double mutants exhibit a severe limb phenotype....