Search PubMed⌕ Search

Biomedical subjects

C Cursiefen

Publications and source records attributed to C Cursiefen.

52 records · Page 3Linked to original sources

Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome: clinical and neuropathological observations in a 33-year-old man.

The syndrome of ichthyosis follicularis, alopecia, and photophobia (IFAP) is an uncommon neuroichthyosis described in only 10 males so far. We report on a man with congenital ichthyosis and alopecia with apparently normal development in early infancy. Photophobia and generalized myoclonicastatic seizures began during or after the first year of age and were associated with progressive impairment of motor skills and mental abilities. He died at 33 years of age. Neuropathological findings showed an unusual deformation of the temporal lobes and olivocerebellar atrophy. Cytogenetic and molecular studies did not uncover deletions in either Xp22.2 to 3 or in Xq27.3 to qter.

Abnormalities, Multiple↗

[Research activities of medical students in Germany using as an example the Würzburg University Clinic].

BACKGROUND AND OBJECTIVE: Many medical students in Germany take part in research at their university, as part of their work on a doctoral dissertation. There are alternatives to this model in other countries. To have a basis for comparison, the research activities of medical students at the University of Würzburg were analysed. METHOD: A questionnaire was sent to all members of the teaching staff of the medical faculty of the University Würzburg (n = 238). It contained 20 questions about the number of students whose research had been supervised, duration of the research, number of uncompleted research studies, cost of materials, resulting publications and estimate of value of the research done. Among those questioned were two groups of professors (50 and 66 respectively, 122 senior lecturers or titular professors). RESULTS: 106 faculty members answered the questionnaire sent to them (45%), 66 working clinically, 26 in a clinic-related institute and 14 in basic research. The students' research usually started in their 4th year and on average took up 216 full-time days. The average training period had lasted for 3 months, 10% of student broke off their research, and each faculty member supervised a mean of 4.5 students. Nonexperimental work (48.3%) cost on average DM 2300, experimental work (51.7%) DM 15,000. Generally two publications resulted and one or two posters/oral communications per student. Two-thirds of the faculty members thought that research undertaken by medical students was important for maintaining a qualitatively and quantitatively high publication level at a university clinic. CONCLUSION: Work on a doctoral dissertation not only promotes scientific thinking of the future doctor but also contributes towards maintaining scientific standards.

Academic Dissertations as Topic↗

Bilateral zonular cataract associated with the mitochondrial cytopathy of Pearson syndrome.

PURPOSE: To report a child with the mitochondrial cytopathy of Pearson syndrome and zonular cataract. METHOD: Case report. We describe a 6-year-old boy with Pearson syndrome. RESULTS: At age 3 years, the boy developed secondary strabismus caused by bilateral zonular cataract. Subsequently, he underwent successful bilateral cataract extraction with intraocular lens implantation. Postoperative visual acuity with best correction was RE, 20/25 and LE, 20/40. CONCLUSIONS: Children with Pearson syndrome should be examined ophthalmologically to rule out zonular cataract and possible amblyopia. Mitochondrial cytopathies such as Pearson syndrome should be included in the differential diagnosis of congenital and early juvenile cataract.

Anemia, Sideroblastic↗

Contribution of medical student research to the Medline-indexed publications of a German medical faculty.

Medical students in Germany have to write a research thesis to acquire the title of medical doctor. This study evaluates the contribution of student research to the Medline-indexed publications of a German medical faculty. A 1993-1995 Medline-publication list, on which medical students among authors should be marked, was sent to medical faculty staff of the University of Würzburg, Germany (n = 238). Faculty members responded (106, 45%), 66 were working at a clinic, 26 at a clinic-associated institute and 14 at a basic science institute. Between 1993 and 1995, 1128 Medline-indexed papers were published by these faculty members, who on average supervised 4.5 medical students (n = 477). Medical students were among the authors of 316 (28%) and were the first authors of 88 papers (7.8%). For 66% of medical students their research resulted in a Medline-indexed publication. Medical student research activity can significantly influence the published output of a medical faculty.

Faculty↗

[Molluscum contagiosum in immunosuppression with methotrexate: multiple warts with central depressions of the eyelids].

BACKGROUND: Poxvirus induced molluscum contagiosum is known to occur in children, elderly people and in patients with the acquired immune deficiency syndrome AIDS. PATIENT AND METHODS: We report on a 49-year-old patient who presented with a three-month history of multiple skin-coloured prominent warts on his right upper and lower eye-lid. On examination, the warts showed a central cavity and measured 2-6 mm in diameter. For his "mixed connective tissue disease" (MCTD) the patient took methotrexate in a weekly dosage of 7.5 mg since 3 years. Diagnosis of MCTD was made 7 years before. The warts were excised and studied histopathologically. One year postoperatively, there was no recurrence of the lesions. CONCLUSION: Drug induced immunosuppression as with methotrexate may be associated with multiple periocular lesions due to molluscum contagiosum.

Biopsy↗

[Glaucoma and pregnancy--review of the literature with a case report].

BACKGROUND: Intraocular pressure usually decreases during pregnancy. Pregnancy-related physiological changes do not seem to cause progression of glaucoma. PATIENT AND METHODS: The literature regarding glaucoma, ocular hypertension and pregnancy indexed in Medline is reviewed. In addition, we report on a 35-year-old patient with ocular hypertension and a known glaucoma family history, who was thoroughly examined before, during and after pregnancy (visual fields, optic disc measurements, 24-hours tension profile, sense physiology ["Erlanger flicker-test", pattern-reversal-ERG, blue-yellow-VEP], ocular perfusion). RESULTS: Visual fields and optic disc measurements did not become worse during pregnancy. Also the other sensory parameters ("Erlanger flicker-test", pattern-reversal-ERG, blue-yellow-VEP) did not deteriorate. Intraocular pressure was reduced by 7 mm Hg during and 2 months after pregnancy. Systolic and diastolic blood velocity of the ophthalmic artery measured by dopplersonography was increased by 60% during pregnancy. CONCLUSIONS: According to present knowledge pregnancy is associated with decreased intraocular pressure and does not seem to constitute a risk factor for progression of ocular hypertension or primary open angle glaucoma. Whether antiglaucomatous drug therapy during pregnancy is necessary awaits further evaluation.

Adult↗

Changing indications for penetrating keratoplasty: histopathology of 1,250 corneal buttons.

PURPOSE: To analyze recent trends in the indications for penetrating keratoplasty. METHODS: After keratoplasty, 2,557 corneal buttons were sent to and analyzed in the ophthalmopathology laboratory of the University of Erlangen-Nürnberg, Erlangen, Germany, between 1992 and 1996. Of these, 1,250 random corneal buttons were evaluated in this study. RESULTS: Histopathologic diagnoses were (a) keratoconus (20.9%), (b) corneal scarring after keratitis/trauma (20.4%), (c) pseudophakic/aphakic bullous keratopathy (17%), (d) regraft (15.5%), (e) Fuchs' corneal endothelial dystrophy (14.9%), (f) necrotizing/ulcerative keratitis (5.3%), and (g) corneal dystrophies (1.7%). CONCLUSION: Compared with a previous report from our laboratory (1964-1986), the relative frequencies of pseudophakic/aphakic bullous keratopathy, regraft, and Fuchs' dystrophy increased, whereas corneal scarring decreased. Keratoconus is now the most frequent indication for penetrating keratoplasty.

Adolescent↗

Angiogenesis in corneal diseases: histopathologic evaluation of 254 human corneal buttons with neovascularization.

PURPOSE: Angiogenesis in corneal diseases can necessitate corneal transplantation and induce graft rejection. The aim of this study was to find out the histopathologic prevalence of angiogenesis in human corneas removed during keratoplasty, to establish the histopathologic diagnoses most commonly associated with angiogenesis, and to evaluate the anatomic location of new corneal vessels. METHODS: Corneal buttons (2,557) after keratoplasty were sent to and analyzed in our ophthalmopathology laboratory between 1992 and 1996. Of these, 1,278 full-thickness and lamellar corneal buttons were randomly retrieved and evaluated in this study. RESULTS: Of 1,278 human corneal buttons obtained by keratoplasty, 254 (19.9%) showed angiogenesis. Associated histopathologic diagnoses were (a) scarring after keratitis (109, 45.4%); (b) graft rejection and insufficiency (73, 30.4%); (c) acute necrotizing ulcerative keratitis (30, 12.5%); and (d) scarring after mechanical or chemical injuries (28, 11.7%). Histopathologically, new vessels were usually associated with corneal edema or inflammatory cells or both (76%). The most common locations of new vessels were the upper and middle third of the corneal stroma. Only 11% of new vessels were located solely in the deep stromal layers. CONCLUSION: Angiogenesis is a common histopathologic feature of corneal diseases leading to corneal transplantation and was found in 19.9% of excised human corneal buttons. Systemic or topical antiangiogenic therapy could reduce the need for corneal transplantation and retransplantation.

Adolescent↗

[Angiogenesis and inhibition of angiogenesis in the eye].

BACKGROUND: Angiogenesis is the formation of new blood vessels from preexisting vessels. Angiogenesis plays an important physiologic and pathologic role in the eye. Pathologic angiogenesis can be found in major causes of human blindness as in diabetic retinopathy and age-related-maculopathy. In recent years great progress has been made in recognizing the mechanisms and regulation of angiogenesis. RESULTS: The general mechanism and the regulation of angiogenesis with proliferative diabetic retinopathy as an example of ocular angiogenesis are reviewed according to recent literature. Angiogenic and antiangiogenic factors regulate the neovascularization. Recent research has identified the vascular endothelial growth factor as the most important mediator of ocular angiogenesis. As well in diabetic retinopathy as in age-related-maculopathy the vascular endothelial growth factor plays a role. New knowledge about ocular angiogenesis is linked to the chance of new antiangiogenic therapies in neovascularizing eye diseases. There exist two ways of ocular antiangiogenic therapy: the first way is to block ocular angiogenic factors such as vascular endothelial growth factor, the other way is to influence the interaction between endothelial cells and extracellular matrix of the newly forming vessels. CONCLUSION: Recent progress in angiogenesis research could result in new causal antiangiogenic drug therapy in ophthalmology. There are some promising animal experiments of local and systemic antiangiogenic therapy. Because of its anatomic localisation the eye is especially suitable for topic antiangiogenic therapy.

Cell Hypoxia↗

[Chloroquine-induced bull's eye maculopathy without electrophysiologic changes].

BACKGROUND: Electrophysiologic findings are usually pathologic in patients with chloroquine-induced bull's-eye maculopathy. To avoid maculopathy the daily dosage of chloroquine is estimated not from the actual but from the ideal body weight and should not exceed 3.5 mg/kg/day. PATIENT AND METHODS: A 59-year-old housewife took a daily dosage of 250 mg chloroquine for her rheumatoid arthritis over a period of 5 years up to a total dose of 450 g. With the height of 160 cm she weighed 68 kg. In 1990, two years after cessation of treatment she complained about blurred vision. Her visual acuity then was 0.8 and fell to 0.3 (right eye) and 0.4 (left eye) in 1996. No vortex keratopathy was observed. A central scotoma was present and fundus-examination showed a typical bull's-eye maculopathy. The mid hypopigmented ring correlated with an increased background fluorescence in the fluorescence-angiogram. Color vision and the retinal nerve fiber photo were normal. In spite of the prominent fundoscopic changes the electrophysiologic examination of this patient (ERG, EOG and pattern-ERG) was normal. The relative smallness of affected retina might explain the normal electrophysiology. CONCLUSION: This case of a patient with typical chloroquine-induced bull's-eye maculopathy with normal electrophysiology points to the importance of ophthalmoscopic and visual fields examination in patients under long-term chloroquine treatment. The correct daily dosage of chloroquine below 3.5 mg/kg/day should be given to avoid maculopathy.

Antirheumatic Agents↗

[Pseudoexfoliation syndrome in patients with retinal vein branch and central vein thrombosis].

BACKGROUND: Pseudoexfoliation syndrome is a frequent cause of secondary open-angle glaucoma due to deposition and in situ-production of abnormal extracellular matrix proteins in the trabecular meshwork. Glaucomas itself are a known risk factor for retinal vein thrombosis. In this retrospective study the prevalence of pseudoexfoliation syndrome in patients with branch and central retinal vein thrombosis was investigated. PATIENTS AND METHODS: The records of 332 unselected patients with branch retinal vein thrombosis and 159 with central retinal vein thrombosis, which were seen in our laser out-patient clinic between January 1990 and July 1996, were analysed retrospectively. RESULTS: 6.0% of patients with branch and 6.9% of patients with central retinal vein thrombosis revealed pseudoexfoliation syndrome. The full picture of pseudoexfoliation syndrome was seen in 4.2% of patients with branch and in 4.4% of patients with central retinal vein thrombosis. The prevalence of pseudoexfoliation syndrome increased with age. Twenty percent of patients with branch and 27% of patients with pseudoexfoliation syndrome and branch or central retinal vein thrombosis. The branch retinal vein thrombosis was significantly more often localised at the disk in patients with glaucoma and in patients with pseudoexfoliation syndrome. CONCLUSIONS: Pseudoexfoliation syndrome is a risk factor for retinal vein thrombosis. Pathogenetically the secondary open-angle glaucoma caused by pseudoexfoliation syndrome could be causative for the venous thrombosis.

Adult↗

Breast carcinoma metastatic to the eyelid presenting as the first sign of insufficient chemotherapy.

Metastatic eyelid tumors are uncommon. Their presentation may vary markedly. We describe a 61-year-old female who presented with a tender nodule in her right upper eyelid shortly after chemotherapy for metastatic disease. She had undergone right mastectomy and radiotherapy because of an adenocarcinoma 16 years ago. Histopathologic findings were consistent with metastatic carcinoma. Knowledge of the diverse clinical and histopathologic features of metastatic breast carcinoma to the eyelids is essential for the appropriate management of these patients. Breast carcinoma metastatic to the eyelid represented in this patient the only sign of insufficient chemotherapy.

Adenocarcinoma↗

Biomolecular markers of malignancy in human uveal melanoma: the role of the cadherin-catenin complex and gene expression profiling.

In recent years there has been a trend towards conservative management of uveal melanoma (UM), aimed at preserving the eye and vision. Despite improvements with this approach, recurrent tumour and metastatic disease still occur, and the management remains problematic. As a result of these limitations, there is interest in gaining a greater understanding of molecular changes associated with aggressive disease patterns in UM. This might result in new, more effective and less toxic therapies as well as provide prognostic information for defining subgroups of patients with a less favourable prognosis as potential candidates for adjuvant therapies. Accumulating evidence over the past decade suggests that disturbance in the cadherin-catenin adhesion complex is critical in the process leading to invasion and metastasis of many cancers. The recent advent of DNA micro-array technology now offers an unprecedented ability to study these molecules and others associated with malignant transformation. In this mini-review, the aspects of tumour progression in which cadherin-catenin may be involved are dealt with along with the potential application of DNA micro-array technology to the problem in UM.

Biomarkers, Tumor↗