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Biomedical subjects

W Muss

Publications and source records attributed to W Muss.

14 recordsLinked to original sources
PubMed2002

[Amniotic membrane transplantation with limbal stem cell transplantation as a combined procedure for corneal surface reconstruction after severe thermal or chemical burns].

BACKGROUND: Severe thermal and chemical burns may result in limbal deficiency leading to persistent epithelial defects, complete conjunctival epithelial ingrowth and vascularisation of the cornea. If sufficiently severe, these burns may lead to very significant visual impairment. Amniotic membrane transplantation with limbal transplantation has recently been proposed as a new method for corneal surface reconstruction. PATIENTS AND METHODS: A total of 14 patients (age 18-62 years, mean age 42 years) with limbal deficiency resulting from thermal ( n=1) or chemical burns ( n=13) underwent surgery. The corneal pannus was completely removed and the amniotic membrane was grafted onto the cornea. Limbal transplantation using autografts obtained from contralateral eyes was performed simultaneously in seven cases. Allografts from a donor were transplanted in seven cases with bilateral involvement. These patients received oral cyclosporin A postoperatively. The mean follow-up time was 18 months. RESULTS: In all cases of limbal autografts the corneal surface showed a complete and stable epithelialisation within a few weeks. Out of seven patients with limbal allografts three displayed recurrent epithelial defects in the long term. The initially semitransparent amniotic membrane became more translucent and biomicroscopically invisible within several months after surgery. There was an increase in visual acuity in most cases, limited mostly by irregular astigmatism due to the initial stromal loss. CONCLUSIONS: Amniotic membrane transplantation with limbal transplantation allows reconstruction ocular surfaces severely damaged by chemical or thermal burns. In most cases, however, additional surgical procedures such as lamellar or penetrating keratoplasty are required for adequate visual rehabilitation.

Administration, Oral↗
PubMed2001

A compound heterozygous one amino-acid insertion/nonsense mutation in the plectin gene causes epidermolysis bullosa simplex with plectin deficiency.

Plectin is a cytoskeleton linker protein expressed in a variety of tissues including skin, muscle, and nerves. Mutations in its gene are associated with epidermolysis bullosa simplex with late-onset muscular dystrophy. Whereas in most of these patients the pathogenic events are mediated by nonsense-mediated mRNA decay, the consequences of an in-frame mutation are less clear. We analyzed a patient with compound heterozygosity for a 3-bp insertion at position 1287 leading to the insertion of leucine as well as the missense mutation Q1518X leading to a stop codon. The presence of plectin mRNA was demonstrated by a RNase protection assay. However, a marked reduction of plectin protein was found using immunofluorescence microscopy of the patient's skin and Western blot analysis of the patient's cultured keratinocytes. The loss of plectin protein was associated with morphological alterations in plectin-containing structures of the dermo-epidermal junction, in skeletal muscle, and in nerves as detected by electron microscopy. In an in vitro overlay assay using recombinant plectin peptides spanning exons 2 to 15 the insertion of leucine resulted in markedly increased self-aggregation of plectin peptides. These results describe for the first time the functional consequences of an in-frame insertion mutation in humans.

Base Sequence↗
PubMed2001

Eurosol versus fetal bovine serum-containing corneal storage medium.

PURPOSE: To evaluate the usability of Eurosol, a new medium-term corneal storage medium without components of bovine origin. METHODS: Ten pairs of human donor corneas were placed in tissue culture at 31 degrees C for 7, 14, 21, 28, or 35 days. One cornea of each pair was cultivated in conventional storage medium on Earls' minimum essential medium base containing 2% fetal bovine serum; the other one was stored in Eurosol. Corneas were examined with inverse light microscopy; corneal thickness was measured; and scanning electron microscopy was performed. RESULTS: No significant difference in corneal thickness and endothelial cell count was found at any time. Scanning electron microscopy showed a complete endothelial cell layer on all corneas. CONCLUSION. The findings indicate a potential clinical applicability of the tested serum-free medium-term storage medium, offering a safer alternative to conventional media containing fetal bovine serum.

Cell Count↗
PubMed1999

Ocular involvement in IgA-epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous disease with frequent ocular involvement, but visual loss is rare. In contrast, EBA patients with predominant IgA autoantibodies more frequently develop severe ocular involvement, which tends to be refractory to therapy. We report two patients with 'IgA-EBA' with ocular involvement. Both initially presented with a generalized bullous disease, and direct immunofluorescence microscopy demonstrated IgA in the basement membrane zone of the skin, and in the conjunctiva and cornea of patient 1. On salt-split patient skin, IgA was found predominantly on the dermal side of the artificial split in both patients. Direct immunoelectron microscopy demonstrated IgA below the lamina densa in close association with the anchoring fibrils in both patients. In patient 1, who had a prolonged course of the disease, the skin disorder responded well to treatment with cyclosporin, but the ocular involvement ended in bilateral blindness despite repeated surgical treatment. In patient 2, the blister formation and scarring conjunctivitis was stopped by a combination of prednisolone and colchicine. These patients show that in subepithelial blistering diseases, early delineation of disease nosology is critical to detect subtypes with severe ocular involvement such as 'IgA-EBA'. In addition, colchicine may be a valuable alternative in the treatment of EBA with ocular involvement.

Adult↗
PubMed1993

Autonomic and peptidergic innervation of human nasal mucosa.

The localization and distribution of vasoactive intestinal polypeptide (VIP), peptide histidine methionine (PHM), the novel peptide helospectin, neuropeptide tyrosine (NPY) and its C-flanking peptide (C-PON), substance P and calcitonin gene-related peptide (CGRP) were studied in the middle and inferior turbinate of the human nose using sensitive immunocytochemical and radioimmunological methods. For light microscopy, double immunofluorescence and immunogold-silver staining methods were applied. Ultrastructural immunoelectronmicroscopy was performed using a pre-embedding method. In addition, semithin Epon resin sections were immunostained. The concentrations of VIP, NPY, CGRP, substance P and neurokinin A were measured using radioimmunological methods. A dense network of autonomic and peptidergic nerve fibers in the normal human nasal mucosa was demonstrated. Colocalization studies showed the coexistence of peptides with components of the autonomic nervous system. Scattered chromogranin A-, CGRP and bombesin-flanking peptide (BFP)-immunoreactive endocrine-like cells were detected within the lamina propria and in groups within exocrine ducts. Highest radioimmunoassay (RIA) tissue concentrations were detected for VIP, followed by NPY, substance P, CGRP and neurokinin A.

Autonomic Nervous System↗
PubMed1989

Immunocytochemical localization of estrogen and progesterone receptors in human thyroid.

The presence of steroid hormone receptors has previously been suggested in thyroid tissue by biochemical means. Our studies were designed to confirm these results and to localize the specific receptor-containing cell type using a novel immunocytochemical method. Monoclonal antibodies specific to estrogen receptors (ER) and progesterone receptors (PgR) were used to localize these steroid hormone receptors in the human thyroid gland. Frozen tissue sections from surgical specimens excised from 22 patients of both sexes with benign thyroid disease were studied. The sections were incubated with rat antiestrophilin and antiprogesterone receptor antibodies and were then exposed to rabbit anti-rat IgG and to rat peroxidase-antiperoxidase complex. The reaction product was visualized with diaminobenzidine tetrahydrochloride and hydrogen peroxide. Four specimens were positive for both ER and PgR, 16 were ER-positive and PgR-negative, and two were negative for both ER and PgR. Positive reactivity was limited to the follicular lining cell nuclei and varied from focal to diffuse. The immunohistochemical findings confirmed the presence of ER and PgR in the thyroid tissue and demonstrated for the first time that these receptors are present only in the nuclei of the lining cells of the thyroid follicle. The role of steroid hormone receptors in the thyroid in health and disease remains to be explained.

Antibodies, Monoclonal↗
PubMedDate not supplied

A Turner-like phenotype in a girl with an isodicentric fluorescent Y chromosome mosaicism.

BACKGROUND: The Ullrich-Turner syndrome (UTS) demonstrates a great clinical variability according to the cytogenetic and molecular genetic findings in various tissues. In few cases the karyotype reveals the presence of an additional Y-bearing cell line which is referred to as a borderline case of mixed gonadal dysgenesis. In this condition, Turner specific stigmata occur in about half of the cases. PATIENT: A 10 year-old girl with short stature and only a few other signs of Turner syndrome and hypertrophic clitoris revealed 45,X/46,X,idic(Yq) mosaicism with 41% 46,X,idic(Yq) cells in a blood lymphocyte culture. METHODS AND RESULTS: Fluorescence in situ hybridisation (FISH) technique, using alpha-satellite Y-chromosome specific probe for locus DYZ3, confirmed the isodicentric character of this structurally abnormal Y chromosome. Polymerase chain reaction (PCR) analysis using primers for eight loci along the Y chromosome including SRY (Sex determining Region, Y gene) were positive for all loci tested, indicating that sequences from the long arm, centromere and most of the short arm of the Y chromosome are present. CONCLUSIONS: As patients with normal or rearranged Y chromosome have an increased risk of developing gonadal neoplasia prophylactic gonadectomy was performed in our patient. No evidence for gonadoblastoma was found on her streak-like gonads, but they showed some evidence of tubular formation. This paper points out the impact of cytogenetic and molecular genetic investigations in the definition of mosaicism in Turner's syndrome.

Child↗
PubMedDate not supplied

Corneal lathing using the excimer laser and a computer-controlled positioning system.

PURPOSE: To present the excimer laser corneal shaping system (ELCS-S), an add-on device to the Keratom, a commercially available 193-nm excimer laser built by Schwind. METHODS: The system is designed for the preparation of donor corneas under sterile conditions using the ultraviolet laser to offer greatest possible flexibility. Lenticules for planolamellar grafting and refractive epikeratoplasty, as well as donor buttons for penetrating keratoplasty can be computer-designed by the surgeon or technician and lathed with the system. RESULTS: Using the excimer laser corneal shaping system (ELCS-S) on human donor corneas, the central surface of the epikeratoplasty lenticule exhibited only narrow, flat concentric notches corresponding to the single lathing steps. Transmission electron microscopy revealed a damage zone of less than 0.3 microm in close approximation to the treated surface. The final thickness revealed a difference of less than +/-53 microm from the intended, initially programmed value. Ultrastructural studies showed the perpendicular stromal surface of the penetrating keratoplasty buttons to be smooth with minimal protrusion of Descemet's membrane. Endothelial injury was observed in a zone averaging between 40 and 100 microm adjacent to the cutting edge only. CONCLUSION: The excimer laser corneal shaping system (ELCS-S) allows a computer-controlled, surgeon-designed, sterile preparation of lamellar and penetrating corneal grafts with the use of the excimer laser. This could offer significant advantages in comparison to presently available systems for lamellar dissection and trephination.

Computers↗
PubMedDate not supplied

[Vascularization of the matrix of hyaline rib cartilage in the human: changes with increasing age].

A correlative light microscopic/transmission electron microscopic study was performed on human hyaline costal cartilage material of variable age (group I: first postnatal month; group II: second decade, and group III: fifth, sixth and seventh decades of life; total: nine cartilage samples) in order to clarify and evaluate the existence and dimensions of a vascular supply in young hyaline cartilage matrix and to compare with alterations or changes, respectively, occurring with aging. Human postnatal hyaline costal cartilage (4th to 6th rib) is vascularized very early by centripetally running venous/arterial vessels invading from the perichondrium. This process seems to be an active one since endothelial spurs and capillary sprouts could be demonstrated. During the middle decades of life a drastic/substantial decrease in amount/appearance of vessels or vascular supply, respectively, of the cartilage generally seems to occur. From the third/fourth decade on up to higher decades neovascularization seems to be a constant finding, especially in degeneration areas: so-called marrow spaces of cartilage matrix. With increasing age the fibrillar perivascular matrix becomes thicker, resulting in a so-called collagen-collar which consists of highly oriented collagen fibrils/fibers (as compared with the attaching extracellular cartilaginous matrix), most probably newly synthesized by (mesenchymal/fibrocytic/specialized?) cells accompanying the invading vessels. Incidence and possible function of the structures found in aged human costal cartilage are not fully understood; the ultrastructural changes compared with postnatal costal cartilage, however, are evident and demonstrated.

Adolescent↗