Wide-awake anesthesia in Dupuytren's contracture treated with collagenase.

The injection of collagenase followed by cord manipulation is one of the most popular treatments for Dupuytren's contracture. This is traditionally performed under local anesthesia or regional nerve block potentially with sedation. Neither the treatment with collagenase, nor the wide-awake anesthesia are novel techniques for hand surgeons. Nevertheless, we report the first experience of cord manipulation using the wide-awake approach. In this prospective study, we compared the pain perception of patients who underwent wide-awake anesthesia versus traditional local anesthesia. We recorded the pain sensation on a visual analog scale (VAS) (0 to 10) during anesthetic injection, during cord manipulation and before discharge. Wide-awake anesthesia significantly reduced pain levels during anesthetic injection (p=0.003) and cord manipulation (p=0.0009). Pain levels did not differ significantly right before discharge in the two groups (p=0.54). Wide-awake anesthesia can be successfully applied to cord manipulation after collagenase injection in Dupuytren's contracture. This way, it is possible to improve the patient's subjective perspective of the procedure.

Evidence of induced non-tolerance in HLA-identical twins with hemoglobinopathy after in utero fetal transplantation.

Fetus-to-fetus transplantation has been suggested for the treatment of hemoglobinopathies in utero. However, dissimilar results have to date been obtained by different groups. We describe a case in which fetus-to-fetus transplantation in HLA-identical twins was performed at the 19th week of gestation by infusion of 0.8 ml of fetal blood from normal to beta-thalassemia affected fetus with the main aim of inducing tolerance. No evidence of engraftment, determined by KM19 polymorphism, was present after 2 years of the procedure. Moreover, an alloreactive cytotoxic T lymphocyte precursor (CTLp) study of affected fetus vs donor and other different stimulators showed that immunization vs tolerance was the real effect of the procedure.

The great heterogeneity of thalassemia molecular defects in Sicily.

This paper reports the results of 1428 beta-thalassemia chromosomes studied in Sicily during a hemoglobinopathy control program starting in 1983. Molecular screening was performed by direct restriction enzyme analysis, allele specific oligonucleotide (ASO) hybridization, reverse dot blot analysis (RDB) and, for the rare or new mutations, by direct sequencing of polymerase chain reaction (PCR) products. Using these approaches 1410 (98.7%) out of 1428 beta-globin gene defects were characterized, involving 22 different beta-thalassemia mutations. Three of these were present at high frequency (beta(0)39, IVS1, 110 and IVS1,6); the other beta-globin gene defects were found at lower frequency. In the latter, we found a smaller group of mutations at a frequency lower than 10% (IVS1, 1, IVS2, 745, beta S) and a larger one at a frequency lower than 2% [-87, IVS1,2, IVS2,1, fr 6, fr 8 (-AA), fr 44, fr 76, -101, IVS1, 116, IVS1, 3'end G-C, IVS1,5 G-A, IVS1,5 G-C, cod 30, Lepore, delta beta, beta C]. The possible origin of this very large number of mutations is discussed, taking into account the historical point of view. Moreover, this approach has made a first trimester prenatal diagnosis program possible in our region in practically all cases, with a great improvement in general thalassemia management.