[Psoriasis as an immunological disease: pathogenic and therapeutic aspects].
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Cytokines↗
Biomedical subjects
R Bergman
Publications and source records attributed to R Bergman.
Dynamics of supercooled water in confined geometry
As with most liquids, it is possible to supercool water; this generally involves cooling the liquid below its melting temperature (avoiding crystallization) until it eventually forms a glass. The viscosity and related relaxation times (tau) of glass-forming liquids typically show non-Arrhenius temperature (T) dependencies. Liquids with highly non-Arrhenius behaviour in the supercooled region are termed 'fragile'. In contrast, liquids whose behaviour is close to the Arrhenius law (In tau infinity 1/T) are termed 'strong'. A unique 'fragile-strong' transition around 228 K has been proposed for supercooled water; however, experimental studies of bulk supercooled water in this temperature range are generally hampered because crystallization occurs. Here we use broad-band dielectric spectroscopy to study the relaxation dynamics of supercooled water in a wide temperature range, including the usually inaccessible temperature region. This is possible because the supercooled water is held within a layered vermiculite clay-the geometrical confinement and presence of intercalated sodium ions prevent most of the water from crystallizing. We find a relaxational process with an Arrhenius temperature dependence, consistent with the proposed strong nature of deeply supercooled bulk water. Because water that is less supercooled has been established as highly fragile, our results support the existence of a fragile-strong transition.
Journal Article↗
DNA based prenatal testing for the skin blistering disorder epidermolysis bullosa simplex.
Epidermolysis bullosa simplex (EBS) is a skin fragility disorder in which mild physical trauma leads to blistering. The phenotype of the disorder is variable, from relatively mild affecting only the hands and/or feet, to very severe with widespread blistering. For the severest forms of EBS there is a demand for prenatal diagnosis which until now has involved a fetal skin biopsy in the second trimester. The identification of mutations in the genes encoding keratins K5 and K14 as the cause of EBS opens up the possibility of much earlier diagnosis of the disease. We report here four cases in which prenatal testing was performed. In three of the cases the genetic lesions were unknown at the start of the pregnancy, requiring the identification of the causative mutation prior to testing fetal DNA. In two of the four cases novel mutations were identified in K14 and in the two remaining families, a previously identified type of mutation was found. Fetal DNA, obtained by chorionic villus sampling or amniocentesis, was analysed for the identified mutations. Three of the DNA samples were found to be normal; a mutant K14 allele was identified in the fourth case and the pregnancy was terminated. These results demonstrate the feasibility of DNA-based prenatal testing for EBS in families where causative mutations can be found.
Amino Acid Sequence↗
Skin granulomas after liver transplantation: unusual presentation of recurrent primary biliary cirrhosis.
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Biopsy↗
Acoustic waves in thin-walled elastic tube with polymeric solution
Acoustic waves in a pipe with polymeric liquid are investigated within a quasi-one-dimensional approach. Analysis of the dispersion equation has shown that rheological features lead to essential changes in both attenuation and speed of the sound. The results may find application in acoustic rheometry of polymeric liquids and for modeling of fast dynamic processes in polymer production technology.
Journal Article↗
A comparative immunohistochemical study of MART-1 expression in Spitz nevi, ordinary melanocytic nevi, and malignant melanomas.
BACKGROUND: The histopathologic differential diagnosis of Spitz nevus (SN) from malignant melanoma (MM) may be difficult. OBJECTIVE: We attempted to elucidate the pattern of expression of a newly recognized melanocyte-specific melanosomal protein MART-1 in routinely processed specimens of SNs, MMs, and ordinary melanocytic nevi (MNs) and to see whether it can help to differentiate between them. METHODS: Twenty SN, 22 MM, and 27 ordinary MN were immunostained with anti-MART-1 monoclonal antibody (clone A103). RESULTS: All SNs, MNs, and MMs demonstrated cytoplasmic staining for MART-1 in some of their tumor cells, of which 17 of 20 (85%) and 24 of 27 (89%) of SN and MN, respectively, demonstrated positive stainings in more than half of their tumor cells, as compared with only 10 of 22 (45%) of the MM (P <.05). The majority of lesions in all 3 types of tumors showed a homogeneous mode of staining, although MM tended to show a more heterogeneous pattern. A consistent pattern of stratification of staining with progressive descent into the dermis was not demonstrated in these tumors. CONCLUSION: MART-1 does not differentiate between SN, MM, and ordinary MN in a consistent pattern, but it may be used as a marker for these tumors.
Antigens, Neoplasm↗
Unilesional mycosis fungoides: a study of seven cases.
BACKGROUND: Unilesional mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma (CTCL), characterized by a solitary lesion clinically and by histopathological features indistinguishable from those of MF, and typically having a benign course. OBJECTIVE: To describe the clinicopathological features of a series of patients with unilesional MF. METHODS: The records of cases of unilesional MF identified during a 10-year period in two medical departments were reviewed. RESULTS: There were 7 patients: 6 males, 1 female; mean age at the time of diagnosis: 32 years; age range: 12-58 years; 3 were below the age of 18 years. The mean pretherapy follow-up period was 9 years (range: 2-20 years). In 5 patients, the eruption consisted of a characteristic patch or plaque of MF located on the trunk or upper extremity; in 2 it was atypical - in 1, a hypopigmented patch, and in 1, a plaque indistinguishable from MF-associated follicular mucinosis. Histopathologically all the lesions exhibited features characteristic of MF, with CD3+ lymphoid cells. In 6 cases (with available fresh frozen tissue) there was a predominance of CD3+ CD4+ cells; in 1 of 5 there was deletion of CD7, and in 3 of 5 there was an overexpression of IL-2 receptor. T-cell receptor gamma gene rearrangement was found in 1 of 4 cutaneous lesions tested; in 2 cases it was found in the blood but not in the skin. Treatment modalities included localized electron beam, excision, topical nitrogen mustard or topical steroids and sunbathing, resulting in all cases in a sustained complete clinical response. In 1 patient, however, there were 2 local recurrences and in yet another patient there was distant cutaneous spread 3.5 years after therapy. CONCLUSIONS: Unilesional MF is a rare variant of CTCL, has heterogeneous clinical manifestations and can affect any age group, including children. The histopathological and immunophenotypical features are in general indistinguishable from those observed in multilesional MF. Although it is a unifocal event, there may occasionally be cutaneous spread with the appearance of noncontiguous lesions, even a long time after therapy. Whether all cases represent minimal-stage IA MF or whether some are actually T-cell pseudolymphoma remains to be clarified.
Adolescent↗
Androgenetic alopecia in heterozygous carriers of a mutation in the human hairless gene.
BACKGROUND: Androgenetic alopecia is considered to be genetically determined. Recently, a rare autosomal recessive form of hereditary alopecia, termed atrichia with papular lesions (APL), was found to result from mutations in the human hairless gene. OBJECTIVE: Our aim was to assess the pattern of androgenetic alopecia in heterozygous carriers of a deleterious mutation in the human hairless gene. METHODS: Healthy male second-degree relatives (n = 31) of patients affected with APL and belonging to a large consanguineous kindred were interviewed and given a Hamilton score of baldness. DNA was obtained from each subject and analyzed for the presence of a mutation in the human hairless gene known to affect this family. The age at onset and extent of baldness were compared in healthy homozygotes and heterozygous carriers of the mutation. RESULTS: Statistical analysis of the results revealed no differences in age at onset and extent of androgenetic alopecia between the two groups of subjects. CONCLUSION: The present study reports the first attempt to characterize the phenotype of heterozygous carriers of a mutation in the human hairless gene. It indicates that the presence of a deleterious mutation in one allele of the hairless gene does not affect the pattern of androgenetic hair loss.
Adult↗
Erythema elevation diutinum--a rare cause of nodulosis in a patient with rheumatoid arthritis.
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Arthritis, Rheumatoid↗
Dynamic Surface Tension of Dilute Aqueous Solutions of Nonionic Cellulose Derivatives in Relation to Other Macromolecular Characterization Parameters.
The dynamic surface tension of 10 nonionic water soluble cellulose derivatives with molecular weights (M(w)) in the range 100,000-1,300,000 has been measured by the pendant drop method at the water/air interface. The surface tension measured after 11.7 h, gamma*, was taken as an apparent steady state surface tension. The gamma* at the highest concentration, 500 ppm, gamma*(500), showed values from 63 to 37 mN/m, and the value of gamma*(500) depends on the hydrophilic/hydrophobic balance of the polymer. The aim of the present work was to discuss the dynamic surface tension in relation to some other characterization parameters, like M(w), diffusion coefficient, intrinsic viscosity ([eta]), cloud point, and degree of hydrophilic/hydrophobic substitution. Dynamic surface tension was shown in a concentration range of 2-10 ppm. The time to reach (gamma(H2O)-gamma*)/2, defined as t(1/2), was found to be inversely correlated both to the diffusion coefficient and to the square of the bulk concentration, which can be expected for a diffusion controlled process. The time t(1/2) showed an approximately linear relationship against [eta](1/3). The pendant drop was found to decrease in volume with time due to the high vapor pressure caused by the curvature of the drop (DeltaP = 2gamma/r(drop)). Copyright 1999 Academic Press.
Journal Article↗
Primary persistent autoimmune disorder in a neonate.
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Adult↗
Soft tissue cephalometric analysis: diagnosis and treatment planning of dentofacial deformity.
This article will present a new soft tissue cephalometric analysis tool. This analysis may be used by the orthodontist and surgeon as an aid in diagnosis and treatment planning. The analysis is a radiographic instrument that was developed directly from the philosophy expressed in Arnett and Bergman "Facial keys to orthodontic diagnosis and treatment planning, Parts I and II" (Am J Orthop Dentofacial Orthod 1993; 103:299-312 and 395-411). The novelty of this approach, as with the "Facial Keys" articles, is an emphasis on soft tissue facial measurement.
Adult↗
Multivariate parameter evaluation of pharmaceutically important cellulose ethers.
A set of nonionic cellulose ethers with varying hydrophobicity and molecular weight has been investigated by principal component analysis (PCA). Several experimental variables such as dynamic surface tension, diffusion coefficient, microviscosity as monitored by a fluorescence probe technique, and intrinsic viscosity are included in the analysis. The experimental variables and observations (polymer fractions) are analyzed in models with good predictive capacities. The apparent equilibrium surface tension correlates to the cloud point and to the critical aggregation concentration in the presence of surfactant. The microviscosity is shown to be a predictive parameter for the degree of hydrophobic substitution. The irreversible process of dynamic surface tension is dependent on the diffusion coefficient but to an even larger degree on the polymer concentration, which is well illustrated by the PCA models.
Algorithms↗
Follicular cutaneous T-cell lymphoma: a clinicopathological study of nine cases.
Nine patients with follicular cutaneous T-cell lymphoma (CTCL), a recently described variant of lymphoma, are presented. On the basis of clinical manifestations and disease course, three groups of patients were distinguished: (i) two patients with follicular CTCL not associated with conventional lesions of mycosis fungoides (MF) and showing no evolution towards MF in follow-up periods of 3 and 6 years; (ii) one patient with follicular CTCL that evolved into conventional MF within 3 years; (iii) six patients showing conventional MF lesions either before or concurrently with the follicular lesions and thus representing follicular CTCL of the true MF type. The follicular lesions included hair-devoid patches or plaques with spiky hyperkeratotic papules (four patients), keratosis pilaris-like lesions (four), comedo-like lesions (four), follicular papules with alopecia (three) and milia-like lesions (three). Histopathological examination showed perifollicular and intrafollicular lymphocytes, without mucin deposition and with minimal or no involvement of the overlying epidermis. Significant syringotropism was also observed in three cases. Immunohistochemical analysis showed the predominance of CD4 + T cells, deletion of CD7 in some cases, Ki-67 + lymphocytes confined mainly to the follicular epithelium, and expression of keratinocyte intercellular adhesion molecule-1 exclusively in the hair follicle. T-cell receptor gamma gene rearrangement was positive in the one case studied from each group. Different treatment modalities were employed, the most commonly used as monotherapy being phototherapy: psoralen ultraviolet A in four patients, two of whom showed a complete clinical and histopathological remission, and ultraviolet B in one patient, who showed a complete remission (both clinical and histopathological). This study indicates that follicular CTCL is more common than reflected in the literature, has heterogeneous clinical manifestations, and is either an expression of or closely related to MF. The influence of the follicular involvement on the therapeutic response remains to be clarified. However, our therapeutic experience clearly suggests that some patients with follicular CTCL can benefit from phototherapy.
Adult↗
Almost 80. Aged population: expectations and roles.
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Adaptation, Psychological↗
Atrichia with papular lesions resulting from a nonsense mutation within the human hairless gene.
Atrichia with papular lesions is a rare autosomal recessive form of alopecia characterized by hair loss soon after birth and the development during childhood of a diffuse papular rash. We have previously shown that this disorder results from a deleterious mutation in the human hairless gene, a gene also involved in the pathogenesis of a related but clinically distinct form of congenital alopecia, termed alopecia universalis congenita. In this report, we describe a novel nonsense mutation in exon 4 of the human hairless gene in a consanguineous kindred affected with atrichia with papular lesions. This report provides additional evidence for phenotypic heterogeneity among inherited atrichias and for an association between the papular rash of atrichia with papular lesions and nonsense mutations in the human hairless gene.
Alopecia↗
Identification of a genetic defect in the hairless gene in atrichia with papular lesions: evidence for phenotypic heterogeneity among inherited atrichias.
Recently, we showed that atrichia with papular lesions (APL), a rare inherited form of alopecia, is transmitted as an autosomal recessive trait in a large inbred kindred of Israeli-Arab origin. Furthermore, we mapped the APL locus to a 5-cM region of chromosome 8p12 in this family. The human "hairless" gene is a candidate target gene for the disease mutation because it maps to the APL locus and because it was recently found to be mutated in a related but clinically distinct form of alopecia known as "alopecia universalis" or "congenital alopecia." In the present study, the coding sequence of the hairless gene was compared by reverse transcription-PCR in fibroblast cell lines derived from an affected patient and an unrelated individual. We identified a single-base deletion (3434delC) in the hairless gene that cosegregated with the disease phenotype in the family. This deletion is predicted to cause a frameshift mutation in the highly conserved C-terminal part of the hairless protein, a region putatively involved in the transcription factor activity of the hairless gene product. The present results are indicative of phenotypic heterogeneity in inherited atrichias caused by mutations in the hairless gene, suggesting different roles for the regions mutated in APL and in other forms of congenital atrichia during hair development.
Age of Onset↗