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 case of primary familial amyloidosis of the cornea in a 13-year-old boy was confirmed by histopathologic examination of material obtained by penetrating keratoplasty. Elevated subepithelial nodules of amyloid were present centrally. Extensive nonelevated subepithelial amyloid deposition was present in areas where either clinically gray, nonelevated subepithelial opacities or apparently normal cornea was present. Nonelevated subepithelial corneal deposits clinically similar to amyloid deposits in the proband were present in two siblings. The genetic pattern of the corneal amyloid deposits in this family appears to be autosomal recessive. Cataracts were present in two of the three affected members. Aspirated lens material from two of the affected members did not contain amyloid.
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PMID:Primary familial amyloidosis of the cornea. 94 79

The senescence accelerated mouse (SAM) has recently been characterized as a unique model to investigate age-related disorders, including amyloidosis, cataract, osteoporosis and dementia. However, little is known as to the properties of the lung in these animals. Tobacco smoke is also associated with enhanced loss of elastic recoil and the development of emphysema. We have attempted to examine morphological as well as biochemical changes of the distal lung in SAM-P/2, as the senescence-prone series and SAM-R/1, as the senescence-resistant series. The animals were intermittently exposed to tobacco smoke or air by Hamburg II machines for 5 weeks. Then both groups of animals were killed for histologic and biochemical study. Compared with SAM-R/1, SAM-P/2, even with air exposure, showed a higher value of the mean linear intercept without alveolar wall destruction. It became even greater due to tobacco exposure with emphysematous change. Tobacco exposure accumulated inflammatory cells into alveoli in SAM-P/2, but not in SAM-R/1. Oxygen radical generation by those cells was also higher in SAM-P/2. Analysis of bronchoalveolar lavage fluid in SAM-P/2 after tobacco exposure disclosed increases in albumin content, total protein content and elastase-like activity. There were decreases in the ratio of elastase inhibitory capacity (EIC) to trypsin inhibitory capacity (TIC), contents of glutathione and total free thiol groups. Moreover, SAM-P/2 showed significantly lower EIC/TIC ratio in serum, even with air exposure, than that of SAM-R/1. These results indicate that SAM-P/2 can be a good model for the study of natural evolution of the aging lung as well as its susceptibility to tobacco smoke in the development of emphysema.
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PMID:A new murine model of aging lung: the senescence accelerated mouse (SAM)-P. 179 64

Five senescence-prone series of mice (P-1, P-2, P-3, P-4 and P-5) and three senescence-resistant series (R-1, R-2 and R-3) were obtained by continuous sister-brother breeding from five original litters of AKR mice with severe deterioration, and the three original litters of AKR mice with normal aging, respectively. A grading score system was adopted to evaluate the degree of senescence of these mice and a steady and irreversible increase in this grading score was seen with advancing age in both the R and P series. The high grading score in the P series was due to an earlier onset of loss of passivity and reactivity, loss of skin glossiness and increased coarseness, hair loss, periophthalmic lesions, increased lordokyphosis of the spine and a more marked increase in their severity with advancing age as compared to the R series. Among the P series, P-2 showed a 100% incidence of systemic amyloidosis after 6 months of age and P-3 a 70% incidence of cataract over 16 months of age. The life span in the P series was shortened by about 26% of that of the R series. In view of the evidence obtained from the survivors, the growth rate and Gompertz function, the aging pattern in the P series was considered to be an acceleration of senescence. The P series has been named "SAM" ("Senescence Accelerated Mouse").
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PMID:A new murine model of accelerated senescence. 731 23

Clinical renal abnormalities, including haematuria, proteinuria, abnormal urinary sediment, decreased renal functions and hypertension are relatively common in children with juvenile rheumatoid arthritis (JRA). These findings may be due to renal amyloidosis or administration of drugs that are potentially nephrotoxic. The case of an 11 years old boy diagnosed as JRA at 4.5 months of age and treated with steroids for 10 years is presented. In his history he had hypertension for 5 years and cataract for one year. Renal biopsy was done to evaluate the aetiology for proteinuria, which was overlooked before his admission to our Department. Secondary renal amyloidosis due to JRA was found at biopsy. The importance of investigation for amyloidosis during the long-term follow-up of JRA is reemphasized.
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PMID:Juvenile rheumatoid arthritis and renal amyloidosis (case report). 759 86

A murine model of accelerated aging, the senescence accelerated mouse (SAM), has been developed. There are three accelerated senescence-resistant (SAM-R) strains and eight senescence-prone (SAM-P) strains. The SAM-P strains have an earlier onset and more rapid advancement of senescence resulting from a significantly shorter lifespan compared with the SAM-R strains. Spontaneous cataracts have been found in some individuals of the SAM-P/9 strain. The SAM-P/1 strain, which was used in the present study, has such systemic senescent characteristics as senile amyloidosis and alopecia, but it was previously thought that cataract does not occur in this strain. However, we found cataractous changes in the lens of these animals at early stages of their life. The earliest change was the appearance of a ripple-mark body at about 3 months of age. The number of rippled rings increased with age. These changes later induced refractive distortion of retinal vessels. Whole-mount flat preparations of the epithelium showed that the number of cells was markedly decreased at the advanced stages of cataract. At the late stages of life the lens cortex became liquefied and developed into a mature cataract. Cataract formation in this strain may be related to reduced viability of the lens epithelium.
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PMID:Morphological study of the cataractous lens of the senescence accelerated mouse. 829 81

Alzheimer disease is a type of cerebral amyloidosis, which is most frequently recognized after the age of 80. In pathophysiology of Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis and age-related cataract changes in protein aggregation play important role, for that reason they form common group of diseases, called conformational diseases. Patients with Alzheimer disease have more common pseudoexfoliation syndrome (PEX), characterized by the accumulation of an abnormal pathognomonic material in the anterior segment of the eye. Regarding common feature of the structure of fibrillar pseudoexfoliation and amyloid material, it is considered that eye examination could be useful in the early diagnosis of Alzheimer disease.
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PMID:[Coexistence of Alzheimer's disease with pseudoexfoliation syndrome PEX]. 1564 2

Senescence-Accelerated Mouse (SAM) has been under development by our research team at Kyoto University since 1970, based on the AKR/J strain donated by the Jackson Laboratory in 1968. The SAM mouse has an accelerated senescence and age-associated pathologies such as senile amyloidosis, senile osteoporosis, degenerative joint disease, cataract, deficits in learning and memory, brain atrophy, hyperinflation of lungs, hearing impairment and so on. SAM research is advancing world-wide and attempts are being made to clarify fundamental mechanisms involved in primary aging processes, pathogenesis of age-associated pathologies and effective methods to modulate or ameliorate the advance of senescence and disease processes involved in age-associated pathologies.
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PMID:A novel murine model of aging, Senescence-Accelerated Mouse (SAM). 1537 84

Glycation, a deleterious form of post-translational modification of macromolecules has been linked to diseases such as diabetes, cataract, Alzheimer's, dialysis related amyloidosis (DRA), atherosclerosis and Parkinson's as well as physiological aging. This review attempts to summarize the data on glycation in relation to its chemistry, role in macromolecular damage and disease, dietary sources and its intervention. Macromolecular damage and biochemical changes that occur in aging and age-related disorders point to the process of glycation as the common event in all of them. This is supported by the fact that several age-related diseases show symptoms manifested by hyperglycemia. Free radical mediated oxidative stress is also known to arise from hyperglycemia. There is evidence to indicate that controlling hyperglycemia by antidiabetic biguanides prolongs life span in experimental animals. Caloric restriction, which appears to prolong life span by bringing about mild hypoglycemia and increased insulin sensitivity further strengthens the idea that glucose via glycation is the primary damaging molecule.
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PMID:Glucose, glycation and aging. 1560

We describe a case of vitreous amyloidosis without systemic symptoms in familial amyloidotic polyneuropathy (FAP) associated with Val30Met transthyretin mutation. A healthy 74-year-old woman noticed left blurred vision and floaters in 1992. Severe vitreous opacities were identified in the left eye. The patient displayed no systemic symptoms, and Congo red staining of the biopsy samples of the stomach and duodenum revealed no amyloid deposition. A diagnosis of FAP was confirmed following genetic investigation. Vitrectomy and cataract surgery was performed with intraocular lens implantation in April 1998. Histopathological examination of the vitreous material revealed amyloid fibrils. Intraocular pressure (IOP) gradually elevated and cupping of the optic disc enlarged. Trabeculectomy was performed in February 2000, but postoperative IOP was again elevated and a needling procedure was performed in March 2000. No postoperative recurrence of vitreous opacity has been reported and IOP has remained well controlled. In the present case, ocular manifestations were the only symptoms of FAP and systemic symptoms have not developed, after more than 12 years. FAP should be suspected as the cause in cases of vitreous opacities in patients from areas with endemic foci of FAP.
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PMID:A case of vitreous amyloidosis without systemic symptoms in familial amyloidotic polyneuropathy. 1567 60

We report a patient with lattice corneal dystrophy type II, also known as Meretoja syndrome or familial amyloidosis Finnish type, who developed a corneal melt 15 days after uneventful phacoemulsification. Despite conservative treatment, the corneal melt resulted in perforation. Uneventful penetrating keratoplasty was performed, but delayed graft epithelial healing was noticed postoperatively. Corneal button histopathological evaluation confirmed the initial clinical diagnosis. To our knowledge, this is the first reported case of corneal melt and perforation in a patient with lattice corneal dystrophy type II.
J Cataract Refract Surg 2009 Jan
PMID:Corneal melt in lattice corneal dystrophy type II after cataract surgery. 1910 43


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