Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0086543 (cataract)
 29,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A typical case of Werner's syndrome was described. All characteristic features were present i.e.: atrophy of muscle and subcutaneous tissue, especially in distal parts of limbs, reduced and gray hairs, numerous atrophic ulcers and hyperkeratotic lesions in points under the pressure (elbow, feet), diffused osteoporosis in all bones, the most intensive in hands and feet, activity dental caries, bilateral cataract, high pitched, horse voice, caused by atrophy of voice cords, endocrinologic disturbances especially concerning testosterone, follicle stimulating hormone, and prolactin, hypoplasia of external genitals, the abnormal blood glucose curve. Far reaching resemblance of the Werner's syndrome to the physiological senility was emphasized. Good reaction to anabolic drugs was described.
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PMID:[Werner's Syndrome]. 228 45

In ten of 18 eyes from nine patients with Werner's syndrome, cataract surgery was complicated by wound dehiscence and its consequences: peripheral anterior synechiae (4), secondary epiretinal gliosis (4), cystoid macular edema (3) in the framework of Hruby-Irvine-Gass syndrome, unplanned filtering bleb (2), and post-operative anterior ischemic optic neuropathy (1). Additionally, corneal endothelial decompensation occurred in eight eyes. In view of the fibroblasts' reduced growth potential, we suggest small surgical incisions, extracapsular cataract surgery using phacoemulsification, intraocular irrigation solutions protecting corneal endothelium, nonabsorbable single knot sutures not removed before 1 year after surgery, and no local or systemic use of cortisone.
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PMID:Ophthalmological aspects in patients with Werner's syndrome. 264 84

We examined twin sisters with a clinical picture typical of Werner's syndrome. Both had undergone bilateral cataract extraction, one at 39 and one at 36 years of age, and had subsequently developed bilateral corneal metastatic calcification within a period of one to two years. In one twin, this keratopathy was associated with hypercalcemia. Each of the twins underwent penetrating keratoplasty in one eye, which was complicated by recurrence of metastatic calcification in a previously normal and clear corneal graft.
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PMID:Corneal metastatic calcification in Werner's syndrome. 304 39

We present two cases of Werner's syndrome associated with intracranial meningioma. Characteristic clinical features of Werner's syndrome include short stature with slender extremities, premature senility, juvenile cataract, skin changes, a tendency to diabetes mellitus and familial occurrence. A 44-year-old female, who had been treated for diabetes mellitus, was diagnosed as having Werner's syndrome because of various characteristic features. A falx meningioma was incidentally found on CT scan, and was surgically removed. Her diabetes mellitus improved. The second case was a 28-year-old male was diagnosed as having Werner's syndrome, diabetes mellitus, juvenile cataract, together with diabetes insipidus, and liver dysfunction. He developed severe headache, gait disturbance and then became unconscious with right hemiparesis. He was found to have a parasagittal meningioma by CT scan and angiography. After removal of the tumor, diabetes mellitus, diabetes insipidus and liver dysfunction improved. The reported incidence of neoplasms associated with Werner's syndrome is about 10%. The majority of associated tumors were mesenchymal in origin. Ten meningiomas, 1 neurinoma and 2 gliomas are reported as associated tumors in the central nervous system. Most of the associated meningiomas were asymptomatic and found incidentally at autopsies or CT scans. Diabetes mellitus associated with Werner's syndrome is generally mild with high immunoreactive insulin value and is controllable by diet therapy and oral antidiabetic drugs. Daily profile of blood sugar improved after the removal of tumor in our cases. In 50 gm glucose tolerance test, tendency of delayed appearance of peak value, which is common in Werner's syndrome, was not altered in our cases. Discussion is made as to the association of Werner's syndrome with meningioma and diabetes mellitus.
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PMID:[Werner's syndrome associated with meningioma: case report]. 328 33

In ten of 18 eyes from nine patients with Werner's syndrome cataract surgery was complicated by wound dehiscence and its consequences: peripheral anterior synechiae (4X), secondary epiretinal gliosis (4X), cystoid macular edema (3X) in the framework of Hruby-Irvine-Gass syndrome, unplanned filtering bleb (2X), and postoperative anterior ischemic optic neuropathy (1X). Additionally, corneal endothelial decompensation occurred in eight eyes. In view of the fibroblasts' reduced growth potential, we suggest small surgical incisions, extracapsular cataract surgery using phacoemulsification, intraocular irrigation solutions protecting corneal endothelium, nonabsorbable single knot sutures not removed before 1 year after surgery, and no local or systemic use of cortisone.
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PMID:Ophthalmic surgical complications in Werner's syndrome: report on 18 eyes of nine patients. 343 6

Data on the clinical features of the Werner syndrome in 102 patients in Japan were collected by sending questionnaires to major hospitals and analyzed. The male-to-female ratio was 3 to 2 and the incidences of consanguinity and familial occurrence were 51% and 39.4%, respectively. These patients were divided into 3 subgroups; group 1, 2, and 3 lacked short stature, cataract, and hypogonadism, respectively. Each group had somewhat different clinical features. Endocrine and metabolic abnormalities in the Werner syndrome patients were compared with those in normal aged subjects. Impaired plasma growth-hormone responses to insulin and arginine were more common and impaired plasma thyrotropin responses to TRH were less common in the Werner syndrome patients than in aged subjects. Plasma LH and FSH levels were higher in most patients than those in age- and sex-matched controls; also, their serum testosterone concentrations were lower than those in age-matched controls and testicular biopsy revealed more marked atrophy than in aged subjects. Serum triiodothyronine levels tended to be lower than in age-matched controls. Oral glucose tolerance test revealed diabetic glucose tolerance in 55% and impaired glucose tolerance in 22%, although fasting blood glucose levels were elevated only in 20%. Plasma insulin response to glucose was more exaggerated in those with the Werner syndrome than in normal aged subjects. The euglycemic glucose clamp method revealed lower glucose disposal rates and insulin sensitivity indices in the Werner syndrome than in normal subjects of similar age. The number of erythrocyte insulin-binding sites was normal in the Werner syndrome patients. These results suggest a postreceptor defect in insulin resistance in the Werner syndrome.
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PMID:Clinical, endocrine and metabolic aspects of the Werner syndrome compared with those of normal aging. 390 66

We present a case of Werner's syndrome associated with intracranial meningioma and a cerebrovascular disorder. A 49-year-old male was transferred to this hospital with a head injury as a result of a car accident, and a CT scan revealed a traumatic intracerebral hemorrhage. The patient displayed the characteristic clinical features of Werner's syndrome, including premature senility, juvenile cataract, atrophic skin, and a tendency to ward familial occurrence was present. MRI and cerebral angiography revealed multiple intracerebral hemorrhages, perhaps due to amyloid angiopathy, multiple lacunar infarctions and parasagittal meningioma. This is the first report on the MRI findings in the brain of a patient with Werner's syndrome. We suspect that of Werner's syndrome also shows evidence of premature aging on MR images.
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PMID:[Werner's syndrome associated with meningioma and a cerebrovascular disorder]. 787 80

We describe a 42-year old female with Werner's syndrome, an autosomal recessive connective tissue disorder, characterised, in her case, by juvenile cataract, chronic ulcers on the feet, characteristic skin changes, a typical appearance of the face ('bird-like face'), severe deformities of the feet, generalised osteoporosis, soft tissue calcifications and a high pitched hoarse voice. Werner's syndrome has to be distinguished from systemic sclerosis in particular. No causal therapy is available.
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PMID:[A female patient with Werner's syndrome]. 832 11

Cataract is one of the typical ocular manifestations of Werner's syndrome. In contrast to cataract in normal elderly persons, cataract in patients with Werner's syndrome is known to be associated with degenerative corneal changes after cataract surgery. Among the corneal changes bullous keratopathy occurs in almost 100% of patients with Werner's syndrome after cataract surgery. Although penetrating keratoplasty appears to be the best treatment for bullous keratopathy, there have been few reports on bullous keratopathy in patients with Werner's syndrome. Here we report two patients with Werner's syndrome who underwent penetrating keratoplasty for the treatment of bullous keratopathy. In both cases, visual acuity was improved, suggesting that penetrating keratoplasty is a recommendable treatment for bullous keratopathy in patients with Werner's syndrome.
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PMID:[Two cases of Werner's syndrome treated with penetrating keratoplasty]. 836 Oct 67

A 26 year-old woman, whose parents were consanguineously married, was admitted to our center because of bilateral juvenile cataract. The patient exhibited short stature, sclerodermalike appearance of the skin with a typical bird-like facies, thinning and graying of hair, high pitched voice and hypogonadism. Werner's syndrome, was diagnosed. History, pathogeny, clinical features, diagnosis and cataract surgery are discussed.
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PMID:[Werner syndrome. Apropos of a case]. 975 41


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