Ateljeevaatteen valmistuksen työtavat Salon Kaarlo Forsmanin tuotannossa

The objective of the present study was to increase knowledge about the atelier culture of recent history, especially about the ways in which atelier clothes were made. I look at the ways of dress-making in the production of a renowned atelier, Salon Kaarlo Forsman. I also give a general outline of the atelier. The studying method I used was triangulation, which is a typical approach in case studies of recent history. My data include 23 dresses by the Salon Forsman, theme interviews of four of the Salon workers and one mannequin, data from my research work, as well as press material and archives. The basis of the analysis of these materials was a theme frame that I had put together with the help of pre-understanding. I then completed and defined the theme frame on the basis of the analysis of the data. I also analyzed the dresses in the fashion photos in the press material. Salon Kaarlo Forsman represents a certain cultural period, the years 1937-1986, and a place where a woman could have individual clothes made for her, from hats to fur coats. The atelier was particularly known for embroidery with beads, draping, and fantastic cuttings designed by the owner, fashion designer Kaarlo Forsman. I draw an outline of the work and practices of the atelier, but also that of Kaarlo Forsman’s life work, as he had a great influence on the sewing methods atelier clothes. Mr. Forsman was able to stretch the first period of modern fashion well into the third period by refusing new, labor-saving methods and sticking to individually designer clothes to the end of his enterprise. The crucial practices in the atelier that I present in this study are fitting, designing, finishing and sewing, as well as beading and the decoration of dresses. I compare the activity, practices and dress-making methods in the Forsman atelier to that of Haute Couture in Paris, which served as model for Finnish fashion houses. I point out the similarities and differences.

Leukoencephalopathies in childhood : Delineation of phenotypes

Within the last 15 years, several new leukoencephalopathies have been recognized. However, more than half of children with cerebral white matter abnormalities still have no specific diagnosis. Our aim was to classify unknown leukoencephalopathies and to identify new diseases among them. During the study, three subgroups of patients were delineated and examined further. First, we evaluated 38 patients with unknown leukoencephalopathy. Brain MRI findings were grouped into seven categories according to the predominant location of the abnormalities. The largest subgroups were myelination abnormalities (n=20) and periventricular white matter abnormalities (n=12). Six patients had uniform MRI findings with signal abnormalities in hemispheric white matter and in selective brain stem and spinal cord tracts. Magnetic resonance spectroscopy (MRS) showed elevated lactate and decreased N-acetylaspartate in the abnormal white matter. The patients presented with ataxia, tremor, distal spasticity, and signs of dorsal column dysfunction. This phenotype - leukoencephalopathy with brain stem and spinal cord involvement and elevated white matter lactate (LBSL) - was first published elsewhere in 2003. A new finding was development of a mild axonal neuropathy. The etiopathogenesis of this disease is unknown, but elevated white matter lactate in MRS suggests a mitochondrial disorder. Secondly, we studied 22 patients with 18q deletions. Clinical and MRI findings were correlated with molecularly defined size of the deletion. All patients with deletions between markers D18S469 and D18S1141 (n=18) had abnormal myelination in brain MRI, while four patients with interstitial deletions sparing that region, had normal myelination pattern. Haploinsufficiency of myelin basic protein is suggested to be responsible for this dysmyelination. Congenital aural atresia/stenosis was found in 50% of the cases and was associated with deletions between markers D18S812 (at 18q22.3) and D18S1141 (at q23). Last part of the study comprised 13 patients with leukoencephalopathy and extensive cerebral calcifications. They showed a spectrum of findings, including progressive cerebral cysts, retinal telangiectasias and angiomas, intrauterine growth retardation, skeletal and hematologic abnormalities, and severe intestinal bleeding, which overlap with features of the previously reported patients with "Coats plus" syndrome and "leukoencephalopathy with calcifications and cysts", suggesting that these disorders are related. All autopsied patients had similar neuropathologic findings showing calcifying obliterative microangiopathy. Our patients may represent an autosomally recessively inherited disorder because there were affected siblings and patients of both sexes. We have started genealogic and molecular genetic studies of this disorder.