Abstract In the present study, the prevalence of thromboembolic events in patients suffering from type I plasminogen deficiency was evaluated. [ncbi.nlm.nih.gov]

Pseudomembraneous lesions are present on affected mucous membranes, such as eye, middle ear, pharynx, duodenum, oral cavity, upper and lower respiratory tracts, and female genital tract. [xpertdox.com]

Regarding biochemistry, there was an increase of 25% in the dosage of the patient's functional plasminogen, that is, at the onset of treatment the patient presented 20% of serum plasminogen, and three years after the onset she presented 25% in the total [scielo.br]

[…] of Oral Medicine, University of Pennsylvania School of Dental Medicine, 240 South 40th Street, Philadelphia, PA 19104, USA., Faizan Alawi University of Pennsylvania, School of Dental Medicine, Philadelphia, United States Full Text A middle-aged woman presented [htct.com.br]

[…] as Day 1 of the present study. [ichgcp.net]

• Surgical Procedure

  Patients may undergo dozens or even more surgical procedures. The frequency of recurrence of fibrous growths in the eye following surgery cannot be predicted, although some patients have required more than 20 surgical procedures. [xpertdox.com]

  Although not well understood, triggers of lesions may include microtrauma (dust, a foreign body), surgical procedures or local inflammation. [orpha.net]

  Since the beginning of the lesions, around 2 years of age, the same underwent surgical procedures several times for the removal of the PL, which were recurrent weeks after removal. [scielo.br]

  The study will evaluate laboratory parameters, information surrounding the management of surgical procedures, obstetric data, and any treatment complications. [theophthalmologist.com]

• Swelling

  Images in Clinical Hematology Gingival swelling associated with hypoplasminogenemia Eric T. [htct.com.br]

  The requested URL /en/gingival-swelling-associated-with-hypoplasminogenemia/articulo/S1516848416300172/ was not found on this server. [rbhh.org]

  These patients all had gingival swellings, and a minority had conjunctival involvement similar to ligneous conjunctivitis, although the etiology was unclear in all. [avesis.istanbul.edu.tr]

  The weakness developed 10 hours after a wasp sting on the right side of face, which was followed by a local reaction in the form of swelling, pain and redness. [bioline.org.br]

  Symptoms develop rapidly after exposure to the allergen and consist of itching, eyelid swelling, conjunctival hyperemia, chemosis, and mucoid discharge. Intense itching is a hallmark symptom. Attacks are usually short-lived and episodic. [entokey.com]

• Intravenous Administration

  Absorption Following 12 weeks of intravenous administration every 2 to 4 days, the mean AUCinf of Ryplazim was 5731.8 hr*% and its Cmax was approximately 125% of the mean physiological level (normal: 70-130%).4 After 12 weeks of therapy, physiological [go.drugbank.com]

  No adverse events considered possibly related to the product were reported following intravenous administration of 2 mg/kg plasminogen. At day 30 no patient exhibited antibodies to plasminogen. [bloodjournal.org]

  Yunos NM, Bellomo R, Hegarty C et al (2012) Association between a chloride-liberal vs chloride-restrictive intravenous fluid administration strategy and kidney injury in critically ill adults. JAMA 308(15):1566–1572 PubMed CrossRef 38. [springermedizin.at]

• Collapse

  Multiple stings can lead to vomiting, diarrhea, generalized edema, dyspnea, hypotension, collapse, renal failure or death. [bioline.org.br]

  […] plasminogen deficiency; patients may experience different types of fibrous lesions (location, size, and various organ system involvement). 1 These fibrous lesions are often quite painful and can compromise organ function, creating emergencies, such as a collapsed [plasminogen.com]

• Dentist

  PLASMINOGEN DEFICIENCY INCIDENCE The incidence of plasminogen deficiency is not well known and may be underestimated because ophthalmologists, dentists, obstetricians, gynecologists, and ENT physicians may treat these patients and either not refer them [ihtc.org]

• Diarrhea

  Multiple stings can lead to vomiting, diarrhea, generalized edema, dyspnea, hypotension, collapse, renal failure or death. [bioline.org.br]

• Thrombosis

  In twenty-four kindreds with hypoplasminogenemia, data concerning the actuarial ages at first thrombotic event were available for construction of thrombosis-free survival curves by the Cutler-Ederer method. [ncbi.nlm.nih.gov]

  Shigekiyo T et al. (1992) Type I congenital plasminogen deficiency is not a risk factor for thrombosis. 2. Mingers AM et al. (1997) Homozygous type I plasminogen deficiency. 3. [moldiag.com]

  1989 72 94 22% SEMINARS IN THROMBOSIS, THROMBOLYSIS, AND VASCULAR BIOLOGY .6. [kth.se]

  Both forms, severe hypoplasminogenemia and dysplasminogenemia, are not causally linked to venous thrombosis. [hero.epa.gov]

• Periodontitis

  Pseudomembranes may develop on the gingivae, and there may be periodontal involvement. Several therapeutic approaches have been developed to treat such patients, but they have had limited effect. [ncbi.nlm.nih.gov]

  Plasminogen Treatment Conjunctiva disease Eye disease Periodontal disease Stomatology Keyword (es) Conjuntivitis Odontología Déficiencia Fracaso Hipoplasminogenemia Parodontitis Plasminógeno Tratamiento Conjuntiva patología Ojo patología Parodontopatía [pascal-francis.inist.fr]

  Pseudomembranes may develop on the gingivae, and there may be periodontal involvement. Methods: Several therapeutic approaches have been developed to treat such patients, but they have had limited effect. [dialnet.unirioja.es]

  Destructive Membranous Periodontal Disease (Ligneous Gingivitis): A Literature Review 2012 4 55 78% Plasminogen deficiency 2007 40 75 53% Ligneous conjunctivitis 2003 56 79 67% Heparin in the long-term management of ligneous conjunctivitis: a case report [kth.se]

  Other sites of involvement include the oral cavity with gingival lesions (ligneous periodontitis), nose, sinuses, ears, throat and tonsils, gastrointestinal tract (duodenal ulcers, lesions in the small and large bowel), the respiratory tract (pseudomembranous [orpha.net]

• Osteoporosis

  A new section on bisphosphonate-associated osteonecrosis thoroughly explores and illustrates this recently identified complication resulting from medications used to prevent bone loss in patients with multiple myeloma, metastatic tumors, and osteoporosis [worldcat.org]

• Ulcer

  Other sites of involvement include the oral cavity with gingival lesions (ligneous periodontitis), nose, sinuses, ears, throat and tonsils, gastrointestinal tract (duodenal ulcers, lesions in the small and large bowel), the respiratory tract (pseudomembranous [orpha.net]

  Hypoplasminogenaemia, gingival swelling and ulceration. Oral Dis, 13 (2007), pp. 515-518 Copyright © 2016. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular [htct.com.br]

  Material and methods. 3 children (5 eyes), in age from 2 to 3 years, who were admitted to the hospital with a diagnosis of traumatic keratitis, corneal ulcer, conjunctivitis of unknown etiology. [eyepress.ru]

  Gingival overgrowth with ulceration has recently been recorded in 4 reports: (1) our report of a British patient with ligneous conjunctivitis in whom the gingival lesions appeared to be related to tranexamic acid-an antifibrinolytic agent; (2) a report [avesis.istanbul.edu.tr]

• Erythema

  When these hypersensitivity disorders involve only the skin, the term erythema multiforme minor is used; when the skin and mucous membranes are involved, the condition is SJS, or erythema multiforme major, which accounts for 20% of all patients with erythema [entokey.com]

  Rarely, membrane formation on the conjunctiva may be associated with bullous mucocutaneous lesions like erythema multiforme (EM) or benign mucous membrane pemphigoid (MMP). [tjmrjournal.org]

  Among these, ligneous conjunctivitis is the most common and is characterized by chronic tearing, erythema of the conjunctiva and white, yellow-white, or red thick masses with a wood-like consistency. [nature.com]

• Stroke

  He is a Fellow of the American Academy of Neurology, American Neurological Association, American College of Physicians, and the Stroke Council of the American Heart Association. [books.google.de]

  Ischemic Stroke 1 Manufacturers Not Available Packagers Not Available Dosage Forms Form Route Strength Injection, powder, lyophilized, for solution Intravenous 68.8 mg/1 Prices Not Available Patents Not Available State Solid Experimental Properties Not [go.drugbank.com]

  Lowering homocysteine in patients with ischemic stroke to prevent recurrent stroke, myocardial infarction, and death. JAMA. 2004 ;291:565-75. 17. Fermo I, D'Angelo SV, Paroni R, Mazzola G, Calori G, D'Angelo A. [degruyter.com]

  Blood clots in the arteries can increase the risk for stroke, heart attack, severe leg pain, difficulty walking, or even the loss of a limb. Hypercoagulable states are usually genetic (inherited) or acquired conditions. [trinityfootandankle.com]

• Irritability

  It may be caused by a variety of noxious influences, the most common being organisms (such as Corynebacterium diphtheriae or streptococci) or chemical irritants such as acids or alkalies. [tjmrjournal.org]

  […] dependent on the area affected and includes polyps, allergies, cholesteatoma, hearing loss, Cowden's Disease, oral fibromas, gingival hypertrophy, reactive airway disease, pneumonia, gastroesophageal reflux disease, ulcers, inflammatory bowel disease, irritable [orpha.net]

  The conjunctiva (the membrane that lines the eyelids and covers the front of the eye) becomes irritated because of a build-up of white, yellow-white, or red thick masses with a wood-like consistency that may replace normal tissue. [ihtc.org]

  A new section on transient lingual papillitis sheds light on this common cause of transitory tongue irritation. A new section on solitary fibrous tumors familiarizes you with this popular designation for certain benign fibrous neoplasms. [worldcat.org]

PLG testing should not be included in routine thrombophilia workup. Mehta R, Shapiro AD. Plasminogen deficiency. Haemophilia. 2008 Nov. 14(6):1261-8. [QxMD MEDLINE Link]. Schuster V, Hügle B, Tefs K. Plasminogen deficiency. [emedicine.medscape.com]

• ST Elevation

  Garg J, Pinnamaneni S, Aronow WS, Ahmad H (2014) ST elevation myocardial infarction after tranexamic acid: First reported case in the United States. Am J Ther 21(6):e221–e224 CrossRef PubMed 25. [springermedizin.de]

Management and treatment Management depends on the site(s) of involvement. Treatment strategies have been targeted mainly towards management of LC. [orpha.net]

In patients with mucosal lesions in the tracheobronchial tree, treatment with Ryplazim may cause respiratory distress due to tissue sloughing. [empr.com]

In connection with the presence of complications, surgical treatment was conducted, a course of conservative treatment was prescribed, including antibacterial, fibrinolytic, epithelializing, general antihistamine, antiedemic drugs, a soft medical contact [eyepress.ru]

Accident 1 2 Completed Treatment Acute Peripheral Arterial Occlusion 1 2 Not Yet Recruiting Treatment Acute Ischemic Stroke 1 2 Not Yet Recruiting Treatment Acute Pulmonary Embolism (PE) 1 2 Recruiting Treatment Ischemic Stroke 1 Manufacturers Not Available [go.drugbank.com]

A Treatment Protocol for Expanded Access Administration of Prometic Plasminogen Due to Closure of Clinical Trial A Treatment Protocol for Extended Administration of Prometic Plasminogen (Human) by Intravenous Infusion in Subjects With Hypoplasminogenemia [ichgcp.net]

Prognosis Where the conjunctivae is involved, the visual prognosis is typically moderate; although chronic lesions can lead to loss of vision. The prognosis for severe cases with multiorgan involvement can be quite poor. [orpha.net]

[…] includes a brief description of each individual lesion or pathologic condition and the kind of pathologic process that it represents, followed by a discussion of its clinical and/or radiographic presentation, histopathologic features, and its treatment and prognosis [books.google.de]

Diagnosis - Congenital Plasminogen Deficiency Prognosis - Congenital Plasminogen Deficiency Not supplied. Treatment - Congenital Plasminogen Deficiency Resources - Congenital Plasminogen Deficiency Not supplied. [checkorphan.org]

The prognosis is variable, depending on the extent, location, size, and site of the lesions. [xpertdox.com]

Material and methods. 3 children (5 eyes), in age from 2 to 3 years, who were admitted to the hospital with a diagnosis of traumatic keratitis, corneal ulcer, conjunctivitis of unknown etiology. [eyepress.ru]

These patients all had gingival swellings, and a minority had conjunctival involvement similar to ligneous conjunctivitis, although the etiology was unclear in all. [avesis.istanbul.edu.tr]

Etiology HPG is caused by homozygous or compound-heterozygous pathogenic variants in the gene PLG (6q26); encoding plasminogen (plg). [orpha.net]

Significantly revised and updated content throughout this edition brings you the latest information on the etiology, clinical features, histopathology, treatment, and prognosis of each disease entity, as well as cutting-edge topics such as bisphosphonate [worldcat.org]

Summary Epidemiology Whilst epidemiological data is limited, the prevalence of hypoplasminogenemia (HPG) is estimated to be in the range of 1/625,000. [orpha.net]

[…] microduplication Silver-Russell syndrome due to imprinting defect of 11p15 Synonym(s): - Plasminogen deficiency type 1 Classification (Orphanet): - Rare genetic disease - Rare systemic or rheumatologic disease Classification (ICD10): (no data available) Epidemiological [csbg.cnb.csic.es]

Genatlas disease for PLG Gene thrombosis,recurrent,dysplasminogenemic type Relevant External Links for PLG Genetic Association Database (GAD) PLG Human Genome Epidemiology (HuGE) Navigator PLG Tumor Gene Database (TGDB): PLG Atlas of Genetics and Cytogenetics [genecards.org]

Linguistic Variant - Portuguese, Brazil) 2015AB LNC-RU-RU (LOINC Linguistic Variant - Russian, Russia) 2015AB LNC-TR-TR (LOINC Linguistic Variant - Turkish, Turkey) 2015AB LNC-ZH-CN (LOINC Linguistic Variant - Chinese, China) 2015AB MCM (Glossary of Clinical Epidemiologic [doctor.am]

Nathan and Oski’s is the only comprehensive product on the market that relates pathophysiology in such depth to hematologic and oncologic diseases affecting children. [books.google.de]

General Pathology The histology shows ulcerated conjunctiva with sub-epithelial, amyloid-like, eosinophilic material containing clotted fibrinogen and immunoglobulins, accompanied by lymphocytes, granulocytes, and plasma cells (Figure 1). [1] Pathophysiology [eyewiki.aao.org]

Pathophysiology of venous thrombosis, thrombophilia, and the diagnosis of deep vein thrombosis-pulmonarycmbolism in the elderly. Clin Geriatr Med. 2006;22:75-92. [degruyter.com]

1:16 – Blood smear with schistocytes 1:40 – Von Willebrand’s protein synthesis 02:09 – ADAMTS-13 and TTP pathophysiology 02:43 – How to interpret ADAMTS-13 activity testing and advantage of doing ADAMTS-13 activity testing with Machaon Dx 03:48 – Use [machaondiagnostics.com]

For the four patients who did not have lesions at the start of the study, Ryplazim appeared to completely prevent lesions from forming. [rxwiki.com]

Two of six patients with hypoplasminogenemia showed thrombotic events, and in one of these symptomatic cases the coexistence of factor V deficiency did not prevent the occurrence of thrombosis. [ncbi.nlm.nih.gov]

Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. ‎ [books.google.ro]

When this therapy is administered every 2 to 4 days, it prevents the build-up of lignous lesions and allows existing lesions to be shed. [go.drugbank.com]

A new section on bisphosphonate-associated osteonecrosis thoroughly explores and illustrates this recently identified complication resulting from medications used to prevent bone loss in patients with multiple myeloma, metastatic tumors, and osteoporosis [worldcat.org]