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)

Patients for cataract surgery in India routinely undergo preoperative syringing to rule out chronic dacryocystitis. We determined the sensitivity and specificity of the clinical test of regurgitation on pressure over the lacrimal sac (ROPLAS) as a screening test for chronic dacryocystitis and compared it to syringing. 621 consecutive outpatients who needed syringing for various reasons (including 318 who had routine syringing prior to cataract surgery) were examined in a masked manner for regurgitation on pressure over the lacrimal sac. They then underwent syringing by a trained (masked) observer. The sensitivity and specificity of ROPLAS were 93.2% and 99.3%, respectively. Using a 6.6% prevalence of chronic dacryocystitis (the prevalence in our cataract population), the negative predictive value of the test was 99.5%. In the presence of regurgitation of pressure over the sac, the high specificity of ROPLAS confirms chronic dacryocystitis. In view of the opportunity costs, when ROPLAS is negative, preoperative syringing in cataract is perhaps unnecessary, unless the findings are equivocal or the index of suspicion for chronic dacryocystitis is very high.
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PMID:Evaluation of the role of syringing prior to cataract surgery. 956 15

Two siblings (a 24-year-old woman and a 14-year-old girl) with congenital woolly hair, epidermolytic palmoplantar keratoderma, and mitral valve regurgitation are described. An interesting additional feature in one of the patients (the 14-year-old girl) was posterior subcapsular cataract. The association of woolly hair and mitral valve regurgitation has not been reported previously. The occurrence of cataract in one of our patients is also a new ophthalmologic finding in this hair shaft anomaly.
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PMID:A new cardiac manifestation associated with woolly hair: report of two cases of woolly hair, palmoplantar keratoderma, and mitral valve regurgitation. 1782

A 2-month-old male with dysmorphic facies, neonatal thrombocytopenia, left congenital cataract, and long bone abnormalities became hypotensive with ST depression on induction of anesthesia for congenital cataract repair. Echocardiogram demonstrated decreased left ventricular function (ejection fraction 46%), mild mitral valve regurgitation (MR), and regional wall motion abnormalities. The left coronary artery could not be visualized. Subsequent cardiac catheterization confirmed atresia of the left main coronary artery. The patient underwent cardiac surgery with coronary artery bypass grafting of the left internal mammary artery to the left anterior descending coronary artery. His postoperative course was uncomplicated, his ventricular function normalized, and he was discharged home. Over the next few months, he developed progressive, severe MR refractory to medical management. Repeat cardiac catheterization revealed stenosis of the right proximal coronary artery, raising concern for progressive coronary involvement. In addition to dysmorphic features and failure to thrive, there were profound developmental delays and a finding of vacuolated lymphocytes on blood smear, which led to a diagnosis of mucolipidosis II (Inclusion [I]-cell disease). The patient was referred for mitral valve replacement, which was successful; however, ongoing respiratory issues attributed to the progression of I-cell disease led to a prolonged hospitalization with placement of a tracheostomy. Reports of coronary anomalies in patients with I-cell disease are rare. An association between mucopolysaccharidosis syndromes and coronary artery abnormalities has been established and is supported by this case report, highlighting the importance of considering the potential for coronary artery involvement with I-cell disease and other similar storage diseases.
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PMID:Left Main Coronary Artery Atresia in an Infant With Inclusion-Cell Disease. 2761 28