Two sisters, the children of first cousin Pakistani Moslem parents, have unusual facies, tongue hamartomata, pre- and postaxial polydactyly, severe talipes, and mesomelic limb shortening associated with tibial dysplasia. Homozygosity for a recessive gene defect is probable. The phenotype resembles, but is distinct from, the orofaciodigital syndromes delineated to date. We suggest that this cond...

Adams-Oliver syndrome (AOS) is a rare congenital disorder, characterized by aplasia cutis congenita (ACC) of the scalp and variable degrees of terminal transverse limb defects. In this article, a newborn infant diagnosed as AOS for a large scalp defect, acrania, and finger malformations is presented. The patient was hospitalized and the scalp defect was successfully repaired with several surgic...

patients and methods previously recorded data of 231 patients who underwent surgical closure of vsd between january 2009 and january 2012 at the rajaie cardiovascular medical and research center were retrospectively reviewed. vsd surgical repair was performed using continues suturing technique in group a patients (n = 163, 70.6%) and interrupted suturing technique in group b patients (n = 68, 2...

We have identified limB, a gene encoding a novel LIM domain-containing protein, LIM2, in a screen for genes required for morphogenesis. limB null cells aggregate, although poorly, but they are unable to undergo morphogenesis, and the aggregates arrest at the mound stage. limB null cells exhibit an aberrant actin cytoskeleton and have numerous F-actin-enriched microspikes. The cells exhibit poor...

We are reporting a case of a 43-year-old woman, who developed a soft tissue defect of the knee region following complex lower limb trauma. The defect was covered with a microvascular latissimus dorsi (LD) flap. The patient required a total knee replacement and developed a defect at the anterior aspect of the knee. This defect was covered by a second free LD flap from the ipsilateral side with a...

The formation and maintenance of the apical ectodermal ridge (AER) is critical for the outgrowth and patterning of the vertebrate limb. In the present work, we have investigated the role of Epiprofin (Epfn/Sp6), a member of the SP/KLF transcription factor family that is expressed in the limb ectoderm and the AER, during limb development. Epfn mutant mice have a defective autopod that shows meso...

The aim of this study is to analyze the morphologic and functional change of human bone defect after its grafting with mixture of platelet gel and autologous cancellous bone. For one year, we have prospectively studied nine consecutive pa- tients, aged 25-73 y, with pseudoarthrosis of long bones, after unsuccessful initial surgeries. We have harvested can- cellous bone from patients' iliac cres...

BACKGROUND The abnormal ventriculo-arterial connections in atrio-ventricular concordance and situs solitus with two well developed ventricles include the range from tetralogy of Fallot throughout the different forms of double outlet right ventricle to transposition of great arteries.The infundibular septum and the trabecula septomarginalis are the fundamental anatomical landmarks for the segmen...

A study of nerve regeneration through a 1cm defect in the peroneal component of the sciatic nerve was performed on sixteen rabbits. Either silicone or polytetrafluoroethylene (PTFE) tubes or nerve graft were used to bridge the defect and the opposite limb was not operated upon. The rabbits that underwent nerve grafting had favourable findings. In the PTFE group, a nerve-like structure was seen ...

Introduction: Traditionally in reconstruction of lower limb soft tissue defects, muscle flaps have been the ‘Gold Standard’ for Gustillo Anderson Grade III B fracture involving upper and middle third defects. Lower third defects were usually reconstructed with free flaps. Evolution in flap surgery has enabled fasciocutaneous, adipofascial and superthin flaps to be harvested for the purpose of r...