Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P00750 (PLA)
 16,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ligneous conjunctivitis (LC) is a rare disease of unknown etiology characterized by the growth of "woody" plaques on ocular and extraocular mucosa. These lesions are comprised of fibrin and both direct and indirect evidence implicates hypofibrinolysis as the primary defect in LC. To further elucidate the pathophysiology of LC we investigated the biochemical aspects of ligneous lesions with respect to the fibrinolytic system. Ligneous lesions were obtained from the right eye of a 15 year-old female patient with longstanding LC since age 2.5 year. Ligneous conjunctivitis in this patient has exhibited a chronic recurrent coarse and has involved multiple muscosal sites. Samples analyzed included an abundant mucoid thread from the conjunctival fornix and the ligneous plaque attached to the inferior tarsus. Samples were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) to characterize protein profiles and by a variety of zymographic methods to visualize fibrinolytic enzymes. We found that mucoid and ligneous samples were distinct entities. Specifically, ligneous samples contained polypeptides with electrophoretic profiles characteristic of intact fibrin, and were replete in fibrin-bound tissue plasminogen activator (t-PA). Despite the presence of ample t-PA, ligneous samples were essentially devoid of fibrinolytic activity. In contrast, neither proteins nor t-PA could be detected in mucoid samples when fractionated by 7.5-15% SDS-PAGE or analyzed by fibrin zymography, respectively. Despite the absence of t-PA, mucoid samples were replete in fibrinolytic activity. This activity was plasminogen independent, heterogenous and inhibited by PMSF. Degradation profiles suggested that this activity represented in part alpha-chymotrypsin, consistent with this patient's treatment regime, as well as plasmin, elastase and an unidentified neutrophil-derived activity. Interestingly, ligneous samples contained both latent and activated forms of matrix metalloproteinase-9 (MMP-9), whereas mucoid samples contained predominantly activated forms of MMP-9. LC is characterized by defective fibrinolysis, despite the presence of ample t-PA and intact fibrin, and by an abundant mucoid thread which binds both endogenous and exogenous enzymes including serine protease(s) and collagenase(s). The implications of these results with respect to a role for exuberant mucus production or abnormal mucins in the development of a relative mucosal-site specific plasmin(ogen) deficiency is discussed.
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PMID:Ligneous conjunctivitis: biochemical evidence for hypofibrinolysis. 1070 63

Fibromuscular dysplasia, predominantly found in adult women, is a rare disease of small and middle-sized arteries of the kidney and brain. We present a case of a 12-year-old girl with acute ischemic stroke, due to fibromuscular dysplasia of the distal internal carotid artery and the proximal middle cerebral artery, which was successfully treated with t-PA.
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PMID:Thrombolytic stroke treatment of a 12-year-old girl with intracranial fibromuscular dysplasia. 2208

Plastic bronchitis or cast bronchitis is a rare disease of unclear etiology characterized by formation of airway casts that can lead to life-threatening airway obstruction. There is currently limited data regarding optimal treatment of plastic bronchitis. Several therapies have been suggested, but recurrences are common and mortality remains high. We report the case of a 6-year-old boy with refractory eosinophilic bronchial casts, unresponsive to low-dose systemic corticosteroids, inhaled corticosteroids, azithromycin, and dornase alfa, who was treated successfully and safely with direct instillation of tissue-type plasminogen activator (tPA) to the obstructing casts during flexible bronchoscopy and inhaled tPA. Our case illustrates that the current therapy for plastic bronchitis remains inadequate. To our knowledge, this case is the first to show that direct instillation of tPA can be used safely for treatment of this disease. The use of tPA via direct administration into the airways during bronchoscopy and via a nebulizer appeared to be a safe and effective therapy for plastic bronchitis and should be considered early in the course of the disease to prevent complications of severe airway obstruction.
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PMID:Management of plastic bronchitis with topical tissue-type plasminogen activator. 2280 9

Spontaneous spinal epidural hematoma is a rare disease, and the critical form may mimic cerebral infarction in the acute stage. Consequently, misdiagnosis of a cerebral infarction may result in unnecessary antithrombotic therapy. The present study investigated 19 cases of spontaneous spinal epidural hematoma first diagnosed as cerebral infarctions and treated with antithrombotic therapy. Of these, 16 cases(84.2%)presented with pain in the neck, shoulder, and back on admission, 19 cases(100%)with hemiplegia not including the face, 7 cases(36.8%)with limb sensory disturbance, and all 19 cases(100%)underwent MRI findings for definite diagnosis. After diagnosis, 6 of the 19 cases(31.6%)were treated with recombinant tissue-type plasminogen activator(rt-PA)administration, 13 cases(68.4%)with surgical treatment, and 9 cases(47.4%)were without after effect and showed good progress. MRI is effective in detecting spontaneous spinal epidural hematoma, however, CT is also used for this diagnosis. The most common site of spontaneous spinal epidural hematoma is the cervicothoracic spine junction or thoracolumbar spine junction, but occurrence in the upper cervical vertebra may present with hemiplegia similar to cerebral infarction. Pathognomonic symptoms include pain in the neck, shoulder, and back, however, symptoms such as hemiplegia not including the face, cervical spine, and cervical cord lesion may be important indicators. Although, some cases may not present with pain and sensory disturbance symptoms, therefore an initial misdiagnosis of cerebral infarction may be made and subsequent antithrombotic therapy can result in increased bleeding and serious after-effects. In particular, 4 of the 6 cases(66.7%)treated with hyperacute phase thrombolytic therapy(rt-PA treatment)in this study required surgical treatment, and the time until definite diagnosis was shorter compared with other antithrombotic agents, presumably due to the rapid increase in hematoma. Therefore, the possibility of spinal cord epidural hematoma should be considered before beginning rt-PA therapy with careful examination to confirm the presence of lesions in the cervical spine and cervical cord.
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PMID:[Analysis of a Spontaneous Spinal Epidural Hematoma Mimicking Cerebral Infarction:A Case Report and Review of the Literatures]. 3283 Jan 32