Increased frequency of HLA-DR2 in patients with Darier's disease.
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Darier Disease↗
Biomedical subjects
J U Prause
Publications and source records attributed to J U Prause.
Transvitreal retinochoroidal biopsy.
Biopsies from retina and/or choroid were performed through a transvitreal approach in 14 patients during the 2-year period 1984-1986. A 20-gauge fine needle was used. The transvitreal approach was chosen because exact location of the biopsy could be documented by video recording and/or by photography. The needle was guided either by a stereotactic micromanipulator or by hand. Immediately after biopsy laser burns were placed around the biopsy hole. Light microscopical and, in selected cases, transmission electron microscopical examination of the material obtained showed malignant melanoma, choroidal haemorrhage, leukaemic infiltration of the choroid, intraretinal fibrosis in detached retina, atrophic retina after vasculitis and malignant lymphoma of the uvea. More than 2 years after biopsy, no retinal detachment due to the biopsy or any tumour-seeding has been observed. Two cases of vitreous haemorrhage cleared spontaneously. No visual impairment was related to the biopsies.
Adolescent↗
Conjunctival cell glycoprotein pattern of healthy persons and of patients with primary Sjögren's syndrome--light microscopical investigation using lectin probes.
Formalin-fixed biopsies of conjunctival tissue from the lower fornix of 10 healthy persons and of 10 patients with primary Sjögren's syndrome (primary SS) were examined by light microscopy. Deparaffinized, rehydrated sections of 5 microns were incubated overnight, each with one of 15 different biotinylated lectins. Bound lectins were visualized using avidin-HRP-substrate complexes. Five lectins did not bind to conjunctival cells, and further six lectins bound in an unspecific manner to conjunctival cells of both healthy persons and of primary SS patients. Jacalin lectin bound selectively to goblet cells of all specimens. Peanut agglutinin (PNA) and wheat germ agglutinin (WGA) bound significantly stronger to basal conjunctival epithelial cells of patients with primary SS. This binding pattern may be of diagnostic value.
Adult↗
Rose bengal score--a possible key parameter when evaluating disease level and progression in primary Sjögren's syndrome.
The rose bengal score is one of the most commonly used tests for evaluation of ocular surface epithelial damage. The test is used in most Sjögren's syndrome criteria. We examined 24 female and four male patients with primary Sjögren's syndrome (primary SS) in order to evaluate possible correlation between the various tests for keratoconjuncivitis sicca (KCS), and for possible correlations to xerostomia and p-IgG levels. Among the KCS tests a high rose bengal score appeared to be the key parameter, being correlated to low break-up time (P less than 0.01), low tear lysozyme (P less than 0.01), appearance of snake-like chromatin in conjunctival imprints (P less than 0.05), low sialometry (P greater than 0.01) and high p-IgG (P less than 0.01). We followed another group of patients with primary SS (30 females and four males) for a mean period of 53 (range 27-76) months. The patients were divided according to their initial response to systemic treatment with bromhexine. KCS parametres and p-IgG were measured repeatedly during the observation period. Patients responding to and continuously treated with bromhexine (2/3 of patients) improved significantly (P less than 0.05) in rose bengal score, but had increasing levels of p-IgG. Non-responders kept their low tear-production rate and had also increasing p-IgG levels. However, when subdivided according to p-IgG level, the group of patients with relatively low p-IgG improved in rose begal score, whereas the high p-IgG-group increased in rose bengal score. The rose bengal score appears to be a useful key parameter when evaluating disease level and progression.
Adult↗
Polyclonal B-cell activation is related to exocrine manifestations of primary Sjögren's syndrome.
Twenty-four female and four male patients with primary Sjögren's syndrome were examined in order to evaluate possible correlations between the exocrine disease manifestations and polyclonal B-cell activation parameters. All patients were examined three times over a 4-month period and none of them received any systemic medical treatment. Van Bijsterveld scores were negatively correlated to unstimulated whole salivary flow rate (sialometry) values (P less than 0.01) and break-up times (BUT) (P less than 0.01), while unrelated to Schirmer-1 values. Unstimulated sialometry values were, in addition, positively correlated to Schirmer-1 values (P less than 0.01), while BUT were unrelated to Schirmer-1 and unstimulated sialometry values. P-IgG levels were positively correlated to van Bijsterveld scores (P less than 0.01) and negatively correlated to break-up times (P less than 0.05), while unrelated to unstimulated sialometry and Schirmer-1 values. P-IgG levels were furthermore positively correlated to levels of P-IgM (P less than 0.05), P-IgA (P less than 0.05), serum IgM-RF (P less than 0.001) and serum ANA (P less than 0.01). Levels of P-IgM-RF were positively correlated to van Bijsterveld scores (P less than 0.01), but to none of the other exocrine manifestations. We conclude that the plasma levels of B-cell products seem to correlate positively with the degree of ocular involvement in patients with primary Sjögren's syndrome, indicating a relationship between the immunoinflammatory processes and the mucosal damage.
Adult↗
Sialochemistry in Sjögren's syndrome: a review.
Sjögren's syndrome (SS) is an autoimmune exocrinopathy. The salivary glands are the site of activated T- and B-lymphocytes, along with gradual parenchymal destruction, diminished flow and altered composition of the secretory product. At present, sialochemistry has achieved no significance for the evaluation of SS patient. However, the number of sialochemical publications is steadily growing. This study review current sialochemical findings in patients with SS and relate the observations to the present concept of diagnosis, pathogenesis and prognosis of SS. An ideal combination of the collection of low-stimulated pure secretion, measurements of absolute flow-rates, and biopsy from the same glands seem to be unobtainable in SS patients. But two procedures may be appropriate: stimulated parotid secretion combined with parotid biopsies, or absorbance of low-stimulated labila saliva combined with labial gland biopsy. Sufficient data on disease-specific alterations in salivary composition in SS are still lacking. However, detection of specific changes in protein synthesis or in glycosylation as well as the detection of inflammatory cell products should be possible with the use of sensitive biochemical assays.
Adult↗
A follow-up study of the progress of keratoconjunctivitis sicca and response to treatment in primary Sjögren's syndrome.
Thirty-four patients with Sjögren's syndrome were retrospectively examined in order to evaluate longitudinal alterations in objective ocular disease parameters and their possible relation to systemic bromhexine treatment. Twenty-three patients (68%) were initially found to respond to peroral bromhexine treatment and were subsequently treated with this agent in addition to tear substitutes. The other 11 patients (32%) were considered bromhexine non-responders and were treated with topical agents only. The bromhexine non-responders had a significantly (p less than 0.02) more reduced tear gland function, evaluated by the Schirmer-1 test, than the responder group. At the end of the follow-up period the conjunctival surface cells were significantly (p less than 0.02) more damaged in the bromhexine non-responders than in the responders. The bromhexine non-responders improved in both break-up time and van Bijsterveld score in the course of time while the responders improved in the van Bijsterveld score only. No differences as regards extraglandular disease manifestations, serological abnormalities or treatment with other systemic agents were found between the bromhexine responders and non-responders.
Adult↗
Structure of the vitread face of the monkey optic disc (Macaca mulatta). SEM on frozen resin-cracked optic nerveheads supplemented by TEM and immunohistochemistry.
The authors applied frozen resin cracking after hexamethyldisilazane (HMDS) desiccation on the monkey optic disc region. The cracked face through the central part of the optic disc showed that the inner limiting membrane of the retina continued into the limiting membrane of Elschnig, and this, in turn, continued into the central meniscus of Kuhnt. At the disc margin the membrane was about 70 nm in thickness, due to a large fibrillar component. Elschnig's membrane was about 50 nm in thickness and was composed of both fibrils and flocculent material. The membrane covering the central meniscus of Kuhnt was about 20 nm in thickness. The number of fibrils here was very low, and the membrane consisted of flocculent material. The positive immunohistochemical stainings for GFA and vimentin of Elschnig's membrane and Kuhnt's meniscus were noteworthy. The positive staining disappeared when the membrane continued into the inner limiting membrane of the retina, supporting the different structural composition.
Animals↗
[Pseudoneuritis pigmentosa in malignant melanoma of the choroid].
In a 65-year-old woman with a choroidal melanoma of the choroid a unilateral pseudoretinitis pigmentosa was detected by ophthalmoscopy. Histological examination revealed a mixed-cell type of malignant melanoma with melanophages and proliferation of pigment epithelium spreading over the inner surface of the retina, with seedings into the vitreous.
Aged↗
Acanthamoeba keratitis, clinico-pathological report of 2 cases.
Acanthamoebae have in the last 14 years been reported to be responsible for severe keratitis in an increasing number of cases. To our knowledge this is the first report of acanthamoeba keratitis (AK) in Scandinavia. Two young males, both wearing soft contact lenses for extended wear, developed long lasting, therapy resistant keratitis. As the keratitis progressed to risk of perforation, keratoplasty was performed on the clinical, and in the one case biopsy-proven diagnosis of AK. Histopathology on the removed discs demonstrated the presence of typical acanthamoebae in the corneal stroma. The observation period after keratoplasty is at present 5 and 20 months, respectively. Both grafts have remained clear and visual acuity is 6/9-6/12 with glasses.
Acanthamoeba↗
Predictive value of flow cytometric DNA-analysis on fresh retinoblastoma tissue.
The cellular DNA content and the distribution of tumour cells in different phases of the cell cycles has been analysed in 8 consecutive enucleated eyes with retinoblastoma. All tumours had abnormal ploidy levels. The analysis did not reveal any specific pattern in 2 tumours which had metastasized compared to 6 local tumours. The flow cytometric analysis alone or in combination with histopathology appeared not to improve the classification of large retinoblastomas.
Child, Preschool↗
Keratoconjunctivitis sicca in patients with primary Sjögren's syndrome. A longitudinal study of ocular parameters.
Thirty-four patients, all fulfilling the Copenhagen criteria for Primary Sjögren's Syndrome, were examined retrospectively in order to evaluate possible longitudinal alterations in Schirmer-1-test results, Rose-bengal score, break-up time and level of ocular score. Twenty-three of the patients were characterized as Bromhexine responders according to their initially positive response to systemic treatment (16 mg x 3 daily) and 11 patients were Bromhexine non-responders. All patients were treated with tear substitutes during the entire observation period of 27 to 76 months (mean 53 months), and all eye examinations were carried out by the same ophthalmologist. The responder group had, both in the start as well as at the end of the observation period, a better ocular status compared to the non-responder group. The latter group had a significantly (P less than 0.02) lower Schirmer-1-test at the start and at the end of the period, and a significantly (P less than 0.02) higher Rose-bengal score at the end of the period. Moreover, the responder group improved in Rose-bengal score (P less than 0.001), whereas the non-responder group improved both in break-up time (P less than 0.05) and Rose-bengal score (P less than 0.05). The use of a score combining results from all three tests, i.e. the ocular score, seems to be a useful tool when evaluating longitudinal variations i dry eye states. Considerable variation was seen between successive results of each ocular test, also in the periods without systemic treatment.
Adult↗
Light microscopical and scanning electron microscopical examinations of deposits on disposable constant wear lenses.
An inexpensive, disposable soft contact lens for extended wear (DanaLens) has been developed. The lens is polymerized moulded in the wet state from hydroxyethyl methylacrylate and hydroxyethyl acrylate. Twenty-two pairs of the lens have in an open study been worn permanently from 1 to 43 days. Light microscopical and scanning electron microscopical examinations of the used lenses showed: superficial deposits on the front surfaces of the lenses of a mucous-like, easily removable material. Neither bacteria nor fungi were demonstrated.
Adolescent↗
Isolated leukemic choroidal relapse in a child with acute lymphoblastic leukemia one year off therapy, diagnosed through transvitreal retino-choroidal biopsy.
A serous retinal detachment and choroidal hemorrhagic infiltration developed in a 17-year-old girl with acute lymphoblastic leukemia one year after cessation of therapy without concurrent bone marrow (BM) or central nervous system relapse. A choroidal biopsy through a sclerotomy with simultaneous release of subretinal fluid revealed no malignant cells. Accordingly, she was treated with prednisone, topical corticosteroid and atropine with normalisation of vision and clinical findings. Five months later the retinal detachment and choroidal infiltration recurred. In order to re-attach the retina once more and obtain a representative choroidal biopsy, a pars plana vitrectomy followed by a retinotomy with removal of subretinal tissue was done revealing leukemic infiltration of the choroid. Simultaneously a BM relapse was diagnosed.
Adolescent↗
Amplification of the human putative oncogene INT1 in primary retinoblastoma tumors.
We report the amplification of the putative oncogene INT1 in two of four retinoblastomas. In one case, the INT1 signal was amplified 10 to 15-fold, in the other 100-fold. In both cases there were signs of increased tumor aggressiveness with invasion of the choroid and development of metastases. The two cases without INT1 amplification had neither metastases nor locally invasive growth. Our findings indicate that INT1 amplification may be a feature of increased malignant potential in retinoblastomas.
Blotting, Southern↗
Tear fluid electrolytes and albumin in persons under environmental stress.
Sixty-two subjects selected among 287 persons with indoor air complaints were exposed to a standard mixture of 22 different organic gases and vapors normally found in Danish houses. Persons were randomly assigned to one of four exposure groups, and each subject stayed during the test day from 10:00 AM to 4:00 PM under standardized conditions in a climate chamber. During exposure the blink frequency was recorded, and after exposure the tear fluid contents of serum albumin, potassium, and sodium were measured. It was found that the persons had an increased concentration of serum albumin in the tear fluid, and that exposure to high concentrations of organic gases and vapors induced a tear reflex-mediated dilution of the tears.
Adult↗
Immunohistochemical characterization of intraepidermal in vivo IgG deposits in patients with primary Sjögren's syndrome.
68% of patients with primary Sjögren's syndrome have previously been found to have intra-epidermal in vivo IgG deposits in clinically unaffected skin. In this investigation, we examined immunohistologically skin biopsies from 5 patients with primary Sjögren's syndrome and from 5 normal controls in order to characterize further the intra-epidermal IgG deposits. Employing direct immunofluorescence and peroxidase-antiperoxidase (PAP) techniques, IgG was localized to epidermal cell surfaces. Double-labelling immunofluorescence experiments showed IgG to be bound to OKT6-positive Langerhans cells, and to some degree also to keratinocytes. Only IgG1, IgG3 and in one patient IgA were deposited, whereas IgG2, IgG4, IgM, IgD, IgE, C1q, C3c, C3d, C4, beta-2 microglobulin, albumin, fibrinogen and C-reactive protein were not found deposited in the intra-epidermal area. The epidermal IgG deposits were reactive with anti-Fc-fragment antibodies and with staphylococcal protein-A. These results are compatible with, although not definitive proofs of, the hypothesis that the in vivo deposited IgG is found in the form of IgG-containing immune complexes.
Adult↗
Characterization of an intraocular retinoblastoma-like tumour.
A transplantable intraocular retinoblastoma-like tumour growing in F 344 rat eyes and tissue cultures suitable for therapeutic experiments is characterized. The tumours grew in the vitreous and infiltrated the internal layers of the retina. Feeder vessels from the retina supplied the tumours. Few and often incomplete rosettes were seen, and calcification was sparse in areas of necrosis. Stainings for glial fibrillary acidic protein, S-100 protein, neuron-specific enolase and desmin were negative. Ultrastructural analysis brought out a close correspondence between the tumour cell and the human retinoblastoma and other virus-induced retinoblastoma-like tumours. The experimental tumour cells lacked cilia, but had neurosecretory granules. Chromosome analysis showed a modal chromosome number in the triploid range, and 6 marker chromosomes were demonstrated. Flow-cytometric analysis showed a S-phase cell fraction of 42%, and chromosome stability was suggested by a tumour-cell DNA index which remained stable for more than 3 months.
Animals↗