ABSTRACT
Haemangiomas usually can be identified by their clinical course. They are characterised by presentation at birth or shortly thereafter, and a rapid proliferative phase over the first 12 months. The haemangioma then usually stabilises and slowly involutes over a period of 5-7 years. For a long time, surgery has been limited to complicated cases, and correcting after-effects following involution. Nevertheless, aesthetic, psychological or functional prejudices may justify early surgery. We conducted a retrospective study of patients treated between 1995 and 2001. A total of 31 patients with facial and cervical haemangiomas were studied. For each, the type of lesion and its topography, age and operative indications, surgery, postoperative complications and aesthetic and functional results have been considered. Thirty-one haemangiomas were operated. The average age was 30 months (1-60 months). After an average follow-up of 3 years, the results were very good in 20%, good in 66%, and fair in 14% of cases. Early curative surgery of haemangioma before spontaneous involution, and before school-age is justified because of social and psychological considerations in infants and their family.
Subject(s)
Head and Neck Neoplasms/surgery , Hemangioma/surgery , Child, Preschool , Facial Neoplasms/surgery , Female , Humans , Infant , Male , Postoperative Complications , Reoperation , Retrospective Studies , Treatment OutcomeABSTRACT
Duchenne muscular dystrophy (DMD) is characterized by the absence of dystrophin and an elevated intracellular calcium level. Single-channel recordings were performed with the cell-attached configuration of the patch-clamp technique. The present study shows, on human co-cultured normal and dystrophic muscle cells, the evidence for an increased activity of calcium permeant cationic mechano-sensitive channels under hypotonic medium stimulation. This activity was particularly enhanced in DMD cells. The hypotonic medium induced drastic changes in the single-channel activity characteristics, which are: a large increase of the calcium over potassium permeability ratio; and a great enhancement of the quantity of current crossing through these channels. These channels could contribute to a significant calcium entry, which could participate in the abnormal calcium homeostasis observed in DMD muscle.
Subject(s)
Calcium Channels/metabolism , Calcium/metabolism , Cell Membrane/metabolism , Muscle Fibers, Skeletal/metabolism , Muscle, Skeletal/metabolism , Muscular Dystrophy, Duchenne/metabolism , Adolescent , Adult , Calcium Channels/drug effects , Cation Transport Proteins/drug effects , Cation Transport Proteins/metabolism , Cations/pharmacology , Cell Membrane/drug effects , Cells, Cultured , Child , Child, Preschool , Gadolinium/pharmacology , Humans , Hypotonic Solutions/pharmacology , Membrane Potentials/drug effects , Membrane Potentials/physiology , Middle Aged , Muscle Fibers, Skeletal/drug effects , Muscle Fibers, Skeletal/pathology , Muscle, Skeletal/pathology , Muscle, Skeletal/physiopathology , Muscular Dystrophy, Duchenne/pathology , Muscular Dystrophy, Duchenne/physiopathologyABSTRACT
The aim of this study was to provide a set of data on mechanisms involved in the calcium homeostasis of facioscapulohumeral muscular dystrophy (FSHD) co-cultured myotubes. In fact, abnormal regulation of calcium have been shown in deficient dystrophin cells like Duchenne muscular dystrophy (DMD) cells, and it seemed interesting to study the calcium regulation in a pathologic cellular model which express dystrophin. T- and L-type calcium currents and contractile responses induced by membrane depolarisations as well as intracellular calcium transients induced by three kinds of stimulus (superfusions of acetylcholine, high K+ or caffeine containing media) were recorded by means of whole-cell patch-clamp and ratiometric cytofluorimetry in co-cultured FSHD myotubes which presented a sarcolemmal localisation of dystrophin. As judged from calcium currents properties, voltage-dependency of contractile responses or amplitude of evoked calcium transients, no clear difference in the calcium handling or calcium signalling was observed between this type of cell and the control cells, at least with the means and the conditions used in the present study. Since FSHD cells, contrary to DMD (Duchenne muscular dystrophy) cells, seemed to display both dystrophin expression and unaltered calcium regulation, the FSHD co-cultured cells appeared as a useful model of dystrophin-expressing pathological muscle cells to further investigate the link between dystrophin expression and intracellular calcium level regulation.
