Cyclosporine (3 to 5 mg/kg orally once/day) inhibits CD8 cells and has been shown to decrease the duration of active disease by 2 to 3 days in some instances and possibly decrease mortality. Consider heparin to prevent thromboembolism (blood clots). Those with chronic ocular surface disease caused by SJS may find some improvement with PROSE treatment (prosthetic replacement of the ocular surface ecosystem treatment). It can also sometimes be caused by an infection. This means that blisters and erosions appear when the skin is rubbed gently. Both SJS and TEN are believed to be variants of the same condition that can be differentiated by the degree of skin and mucous membrane The drug or metabolite covalently binds with a host protein to form a non-self, drug-related epitope. [26] A classification first published in 1993, that has been adopted as a consensus definition, identifies StevensJohnson syndrome, toxic epidermal necrolysis, and SJS/TEN overlap. WebThe syndrome varies widely in severity and can range from mild symptoms such as hypertension to severe symptoms such as agitation, hallucinations, fever, vomiting, and spastic muscle contractions. Box 350333 Westminster, CO 80035 Email - [3] SJS/TEN reactions are believed to follow a type IV hypersensitivity mechanism. Treatment reference: the patients with mild SJS complicated with eye damage need to supplement tears in the acute stage. WebStevens Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin reaction usually triggered by certain medications. https://www.id-press.eu/mjms/article/view/oamjms.2018.148, Stevens-Johnson syndrome: a perplexing diagnosis. There are probably two major pathways involved: In children, Stevens-Johnson syndrome is usually triggered by a viral infection, such as: Less commonly, bacterial infections can also trigger the syndrome. Percentages of the total body surface area for an adult or child over 10 years, Percentages of the total body surface area for a child under the age of 1, Over 1 year and below 10 years, the percentage of body surface area changes. The area beneath is pink and moist, and usually very tender. In case of severe inflammation, corneal contact lens is used to protect corneal epithelium, and autologous serum promotes repair. [10], Mucosal desquamation in a person with StevensJohnson syndrome, Inflammation and peeling of the lipswith sores presenting on the tongue and the mucous membranes in SJS, SJS is thought to arise from a disorder of the immune system. WebBody aches Cough Inflammation of the mucous membranes Painful red or purple rash Blisters The top layer of skin will begin to die and shed As the disease progresses, the symptoms will worsen and result in the following More blisters and lesions on the mucous membranes of the mouth, nose, eyes, genitals, and urinary or respiratory tracts [8], Based on the Asian findings, similar studies in Europe showed 61% of allopurinol-induced SJS/TEN patients carried the HLA-B58 (phenotype frequency of the B*5801 allele in Europeans is typically 3%). StevensJohnson syndrome Bullous erythema multiforme, Figure 5. Use the Wallace rule of 9 to estimate the affected body surface area. One point is scored for each of seven criteria present at the time of admission. People with a positive sign have loose skin that slips free from the underlying layers when rubbed. If the reaction was caused by a medication, tell them which one. WebSJS and TEN are rare conditions; combined, they affect fewer than 8 cases per million persons per year. Toxic epidermal necrolysis is a severe manifestation of Stevens-Johnson syndrome, defined as greater than 30% skin detachment. At Another Johns Hopkins Member Hospital: Managing Eczema in Winter and Year Round: A Parents Guide, Got Adult Acne? Bethesda, MD 20894, Web Policies The cornea is transparent without epithelial punctate opacity, C. The corneal fluorescence staining showed punctate staining, D. Palpebral conjunctiva is congested and edematous, and pseudomembrane can be seen. Figure 6. [11] Genetic factors are associated with a predisposition to SJS. But people with a gene called HLA-B1502 and HLA B1508 have an increased risk of Stevens-Johnson syndrome if they take this drug. HLA) serotypes, prosthetic replacement of the ocular surface ecosystem treatment, "Stevens-Johnson syndrome/toxic epidermal necrolysis", "Recent advances in the understanding of severe cutaneous adverse reactions", "Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Standard Reporting and Evaluation Guidelines", "StevensJohnson Syndrome: An intriguing diagnosis", "Profile and pattern of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in a general hospital in Singapore: Treatment outcomes", "The current understanding of StevensJohnson syndrome and toxic epidermal necrolysis", "ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson Syndrome and toxic epidermal necrolysis: Comparison with case-control analysis", "Scientific conclusions and grounds for the variation to the terms of the marketing authorisation(s)", "Clinical study of cutaneous drug eruptions in 200 patients", Indian Journal of Dermatology, Venereology and Leprology, "A case of bupropion-induced Stevens-Johnson syndrome with acute psoriatic exacerbation", "Carbamazepine The commonest cause of toxic epidermal necrolysis and StevensJohnson syndrome: A study of 7 years", "Acetaminophen induced Steven Johnson syndrome-Toxic Epidermal Necrolysis overlap", "Stevens-Johnson syndrome and toxic epidermal necrolysis", "Acute Generalized Exanthematous Pustulosis: Pathogenesis, Genetic Background, Clinical Variants and Therapy", "Classification of Drug Hypersensitivity into Allergic, p-i, and Pseudo-Allergic Forms", "HLA Association with Drug-Induced Adverse Reactions", "Medical genetics: A marker for StevensJohnson syndrome", "Association between HLA-B*1502 allele and antiepileptic drug-induced cutaneous reactions in Han Chinese", "A marker for StevensJohnson syndrome : Ethnicity matters", "A European study of HLA-B in StevensJohnson syndrome and toxic epidermal necrolysis related to five high-risk drugs", "Pharmacogenomics of off-target adverse drug reactions", "Reference SNP (refSNP) Cluster Report: rs1057910 ** With drug-response allele **", "Genetic variants associated with phenytoin-related severe cutaneous adverse reactions", "StevensJohnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)", "Correlations between clinical patterns and causes of Erythema Multiforme Majus, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis results of an international prospective study", "Is universal HLA-B*15:02 screening a cost-effective option in an ethnically diverse population? Erythema multiforme is a skin disorder that's considered to be an allergic reaction to medicine or an infection. Infections are generally associated mucosal involvement and less severe cutaneous disease than when drugs are the cause. No, Steven-johnson syndrome is not contagious, it is an unpredictable adverse reaction to certain medications. Eye care is undertaken 36 times each day depending on severity of eye involvement. Within a few days, the skin begins to blister and peel, forming very painful raw areas called erosions that resemble a severe hot-water burn. So far, only the Michel artificial cornea can be treated. All rights reserved. The authors have declared that no competing interests exist. Allergy Asthma Immunol Res. Treating the infectious disease causing the disorder, Eliminating any medicine causing the disorder. Since the genes for these receptors are highly edited, i.e. [13] These occur primarily on the torso. an individual's efficiency in absorbing, tissue-distributing, metabolizing, or excreting a drug, have been found to occur in various severe cutaneous adverse reactions (SCARS) as well as other types of adverse drug reactions. Anemia occurs in virtually all cases (reduced hemoglobin). [45] These variations influence the levels and duration of a drug or its metabolite in tissues and thereby impact the drug's or metabolite's ability to evoke these reactions. How can StevensJohnson syndrome / toxic epidermal necrolysis be prevented? Sulfonamides: sulfamethoxazole, sulfadiazine, sulfapyridine. [1] Mucous membranes, such as the mouth, are also typically involved. A. If a bath is contraindicated or unavailable, perform a gentle bed-bath using aqueous cream, warm water and a soft cloth. [13], Screening individuals for certain predisposing gene variants before initiating treatment with particular SJS-, TEN/SJS-, or TEN-inducing drugs is recommended or under study. In the case of TEN, some people even lose hair and nails. Bookshelf variation) in order to stimulate T cells. [12] The cause of SJS is unknown in one-quarter to one-half of cases. Suite 600 Its usually a reaction to medication that starts with flu-like symptoms, followed by a painful rash that spreads and blisters. Treatment focuses on removing the cause, caring for wounds, controlling pain, and minimizing complications as skin regrows. Initial percentage of epidermal detachment > 10%, Other severe cutaneous adverse reactions to drugs (e.g, drug hypersensitivity syndrome), Staphylococcal scalded skin syndrome and toxic shock syndrome, Erythema multiforme, particularly erythema multiforme major (with mucosal involvement), Lower limbs each increase by 0.5% per year, Cessation of suspected causative drug(s) the patient is less likely to die and complications are less if the culprit drug is on or before the day that blisters/erosions appear, Hospital admission preferably immediately to an intensive care and/or burns unit with specialist nursing care, as this improves survival, reduces infection and shortens hospital stay, Nutritional and fluid replacement (crystalloid) by intravenous and nasogastric routes reviewed and adjusted daily, Temperature maintenance as body temperature regulation is impaired, patient should be in a warm room (3032C). [10], The second most common cause of SJS and TEN is infection, particularly in children. Stevens-Johnson syndrome/toxic epidermal necrolysis often begins with a fever and flu-like symptoms. [36], Variations in ADME, i.e. [13] A leading cause appears to be the use of antibiotics, particularly sulfa drugs. Synechiae can be released by applying the eye ointment. Because the skin normally acts as a protective barrier, extensive skin damage can lead to a dangerous loss of fluids and allow infections to develop. More than 200 medications have been reported in association with Stevens-Johnson syndrome/toxic epidermal necrolysis. [7][47] In addition to abnormalities in drug-metabolizing enzymes, dysfunctions of the kidney, liver, or GI tract which increase a SCARs-inducing drug or metabolite levels are suggested to promote SCARs responses. The medications associated with a high risk of Stevens-Johnson syndrome are: This list of drugs known to cause StevensJohnson syndrome / toxic epidermal necrolysis is not exclusive. Cross-reactions can occur between: If youve had this condition, avoid the medication that triggered it. Immediate withdrawal of all the suspected drugs is the key to the management of SJS-TEN. Maintenance of an ambient body temperature (31C32C), proper fluid-electrolyte balance, and maintenance of a strict aseptic environment are crucial. Banana leaf is used in many centers in India during the care of patients with SJS and TEN. More items The maximum extent is usually reached by 4 days. The first and most important step in treating Stevens-Johnson syndrome is to discontinue any medications that may be causing it. Rinse with the hand shower, lifting the nursing board above the bath. government site. They are not effective later in the course of the illness. Leucopenia (reduced white cells), especially lymphopenia (reduced lymphocytes) is very common (90%). However, dentists should be clinically oriented towards signs and symptoms of the disease, both oral and systemic. Treatment reference: SJS complicated with moderate ocular surface damage needs to be supplemented with tears. document.getElementById( "ak_js_1" ).setAttribute( "value", ( new Date() ).getTime() ); document.getElementById( "ak_js_2" ).setAttribute( "value", ( new Date() ).getTime() ); Stevens-Johnson Syndrome is a rare and very serious skin condition. Serious complications can include pneumonia, overwhelming bacterial infections (sepsis), shock, multiple organ failure, and death. The top layer of skin will begin to die and shed, More blisters and lesions on the mucous membranes of the mouth, nose, eyes, genitals, and urinary or respiratory tracts. WebThese skin reactions, known as Stevens-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP), can be fatal. We are vaccinating all eligible patients. Stevens Johnson syndrome-Toxic Epidermal Necrolysis Overlap induced by sulfasalazine treatment: a case report. [12], Fungal infections with coccidioidomycosis, dermatophytosis and histoplasmosis are also considered possible causes. WebIn Stevens-Johnson syndrome, the immune system overreacts to a medication or infection. Treatments for Stevens-Johnson syndrome include:Stopping the medication that has caused the problem.Replacing electrolytes with intravenous (IV) fluids.Using non-adhesive dressings on the affected skin.Using high-calorie food, possibly by tube-feeding, to promote healing.Using antibiotics when needed to prevent infection.Providing pain relief medications.More items The diagnosis may therefore change during the first few days in hospital. Stevens-Johnson syndrome signs and symptoms include: If you have Stevens-Johnson syndrome, several days before the rash develops you may experience: StevensJohnson syndrome / toxic epidermal necrolysis is suspected clinically and classified based on the skin surface area detached at maximum extent. SJSAwarenessUK Copyright Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Due to the low clinical incidence rate of SJS, it is not easy to diagnose SJS in the early stage. White corneal scar in pupil area, with plenty of neovascularization. [35] While the evidence supporting this T-cell receptor selectivity is limited, one study identified the preferential presence of the TCR-V-b and complementarity-determining region 3 in T-cell receptors found on the T cells in the blisters of patients with allopurinol-induced DRESS syndrome. [2][1] Risk factors include HIV/AIDS and systemic lupus erythematosus. WebStevens-Johnson Syndrome, or SJS, is a serious allergic reaction to drugs. CD8+ T cells) and T helper cells (i.e. With decades of experience in medical and pharmaceutical lawsuits, our firm is dedicated to helping you win your SJS lawsuit and find peace, closure, and justice. Erythema multiforme major is also known as Stevens-Johnson syndrome. Clinical study of Stevens Johnson syndrome with ocular surface damage, Clinical analysis of 18 cases of Stevens Johnson syndrome Lei Yunhong, sunchaowen, Jian wanai, Wangyi, Tang Fei, Research Progress on pathogenesis of Stevens Johnson syndrome he Xuelian (review) liuzhisheng (reviser), Why do you recommend to come to Beijing for artificial cornea transplantation?2023-02-07, Which artificial cornea has the most extensive indications? These early signs of Stevens Johnson syndrome include: Fever Sore mouth and throat Fatigue Burning eyes Cough Joint pain Feeling generally unwell These Why Is Dexamethasone Given Prior To Chemo? Ask the patient to open his/her eyes frequently to avoid synechia formation. [8] A skin biopsy is helpful, but not required, to establish a diagnosis of SJS and TEN.[8]. Therefore, it is necessary to remove trichiasis and wear corneal contact lens when necessary, so as to provide a relatively stable ocular surface environment for epithelial repair. In the differential diagnosis of StevensJohnson syndrome / toxic epidermal necrolysis consider: Stevens-Johnson syndrome requires hospitalization, often in an intensive care unit or a burn unit. Drug specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. [10], Initially, treatment is similar to that for patients with thermal burns, and continued care can only be supportive (e.g., intravenous fluids and nasogastric or parenteral feeding) and symptomatic (e.g., analgesic mouth rinse for mouth ulcer). The treatment of moderate ocular surface damage is mainly to alleviate dry eye symptoms, reduce ocular surface inflammation, protect and repair corneal epithelium. Taiwanese, Japanese, and Malaysian individuals expressing the CYP2C9*3[46] variant of CYP2C9, which has reduced metabolic activity compared to the wild type (i.e. However, the current ocular surface inflammation cannot be ignored. However, some reports show improved outcomes with early corticosteroid therapy. [8] Patients with these disorders frequently experience burning pain of their skin at the start of disease. [7][4] These ADME abnormalities, it is also suggested, may interact with particular HLA proteins and T cell receptors to promote a SCARs disorder. [8] Patients with SJS or TEN caused by a drug have a better prognosis the earlier the causative drug is withdrawn.[8]. Lung disease bronchiolitis, bronchiectasis, obstructive disorders. [8] SJS and TEN most often begin between 4 and 28 days after culprit drug administration. [33][44] Thus, only rare individuals are predisposed to develop SJS in response to a particular drug on the bases of their expression of specific T-cell receptor types. [41][42] This has clinical relevance as it is agreed upon that prior to starting a medication such as allopurinol in a patient of Chinese descent, HLA-B*58:01 testing should be considered. Skincare includes prompt treatment of secondary bacterial infections and daily wound care for severe burns. There are several drugs and medications that can trigger Stevens-Johnson syndrome. Gently remove dressings, crust, and exudate; avoid scrubbing. D. Continuous patches of conjunctival scar, more than 1/2 of the palpebral margin keratosis. How quickly does Steven Johnson syndrome spread? The drugs that most commonly cause Stevens-Johnson syndrome/toxic epidermal necrolysis are: StevensJohnson syndrome / toxic epidermal necrolysis usually develops within the first week of antibiotic therapy but up to 2 months after starting an anticonvulsant. [10] The immune reaction can be triggered by drugs or infections. Treatments for Stevens-Johnson syndrome include: Stopping the medication that has caused the problem. Replacing electrolytes with intravenous (IV) fluids. Using non-adhesive dressings on the affected skin. Using high-calorie food, possibly by tube-feeding, to promote healing. Using antibiotics when needed to prevent infection. [6] It is also included with drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), acute generalized exanthematous pustulosis (AGEP) and toxic epidermal necrolysis in a group of conditions known severe cutaneous adverse reactions (SCARs). altered to encode proteins with different amino acid sequences, and since the human population may express more than 100 trillion different (i.e. NextWhy do you recommend to come to Beijing for artificial cornea transplantation. Phone: 650-931-2505 | Fax: 650-931-2506 Flumiolone is anti-inflammatory. Generally, eye surface damage of SJS patients is divided into three levels: mild eye surface damage, moderate eye surface damage and severe eye surface damage. Before the rash appears, there is usually a prodromal illness of several days duration resembling an upper respiratory tract infection or flu-like illness. Recovery after Stevens-Johnson syndrome can take weeks to months, depending on the severity of your condition. Because the skin normally acts as a protective barrier, extensive skin damage can lead to a dangerous loss of fluids and allow infections to develop. However concerns have been raised that they may increase the risk of infection, impair wound healing and other complications, and they have not been proven to have any benefit. If not controlled, the inflammatory reaction will cause different degrees of ocular surface damage, including squamous metaplasia, limbal stem cell defect and even lacrimal gland function decompensation, which in turn aggravates ocular surface inflammation and forms a vicious circle. are no different from the common cold. Skin biopsy is usually required to confirm the clinical diagnosis and to exclude Staphylococcal scalded skin syndrome and other generalized rashes with blisters. and transmitted securely. -. Lasting skin problems may cause your hair to fall out, and your fingernails and toenails may not grow normally. In addition, its initial symptoms are diverse, and the initial symptoms J Korean Assoc Oral Maxillofac Surg. Texas 75069, 3131 McKinney Avenue We will work with you during every step of your case, to understand the facts, explore your options, and win the settlement you and your loved ones need. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications as your skin regrows. This is the legendary Stevens Johnson syndrome, which is the most serious adverse drug reaction with skin damage and threatening the lives of patients. Blisters on your skin and the mucous membranes of your mouth, nose, eyes and genitals, Shedding of your skin within days after blisters form, Skin detachment < 10% of body surface area (BSA), Widespread erythematous or purpuric macules or at atypical targets, Widespread purpuric macules or at atypical targets, Large epidermal sheets and no purpuric macules. Current trials are underway in Taiwan to define the cost-effectiveness of avoiding phenytoin in SJS, SJS/TEN, and TEN for individuals expressing the CYP2C9*3 allele of CYP2C9. Stevens-Johnson syndrome is a medical emergency that usually requires hospitalization. Put a sterile compress into the finger of a sterile glove. 2019 Wormington & Bollinger. Apply petroleum jelly to the outside of the glove. Repithelialisation of denuded areas takes several weeks, and is accompanied by peeling of the less severely affected skin. blisters; dental emergency; oral mucosal lesions; oral ulcers; skin rash; stevens-johnson syndrome (sjs). Other long-term problems can include impaired taste, difficulty urinating, and genital abnormalities. Other HLA associations with the development of SJS, SJS/TEN, or TEN and the intake of specific drugs as determined in certain populations are given in HLA associations with SCARs. No randomized trials of corticosteroids have been conducted for SJS, and it can be managed successfully without them.[10]. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review. The histopathology shows keratinocyte necrosis (death of individual skin cells), full thickness epidermal/epithelial necrosis (death of an entire layer of skin), minimal inflammation (very mild lymphocytic infiltrate of the superficial dermis). StevensJohnson syndrome / toxic epidermal necrolysis prognosis, Complications of StevensJohnson syndrome / toxic epidermal necrolysis, Triggers for StevensJohnson syndrome / toxic epidermal necrolysis, Risk factors for Stevens Johnson syndrome. -. Dutt J, Sapra A, Sheth-Dutt P, Bhandari P, Gupta S. Cureus. Those peptides expressing a drug-related, non-self epitope on one of their various HLA protein forms (HLA-A, HLA-B, HLA-C, HLA-DM, HLA-DO, HLA-DP, HLA-DQ, or HLA-DR) can bind to a T-cell receptor and thereby stimulate the receptor-bearing parent T cell to initiate attacks on self tissues. [5] Early symptoms of SJS include fever and flu-like symptoms. Review of toxic epidermal necrolysis. National Library of Medicine It is suitable for patients with bilateral corneal blindness who are difficult to succeed in corneal transplantation, including corneal transplantation failure, severe keratoconjunctival scar vascularization, eyelid atresia, and serious autoimmune diseases (such as Stevens Johnson syndrome and cicatricial pemphigoid) caused by chemical injury, thermal burn, explosion injury, etc, Corneal blindness caused by end-stage dry eye. Anyone on medication can develop Stevens-Johnson syndrome/toxic epidermal necrolysis unpredictably. Various drugs such as antibiotics, anticonvulsants and non-steroidal anti-inflammatory drugs can trigger the disease as an adverse effect. Stevens Johnson syndrome affecting the eye, Figure 3. Skin rash over the patient's upper limb, Figure 2. Early retrospective studies suggested corticosteroids increased hospital stays and complication rates. About 10 percent of people with Stevens-Johnson syndrome die from the disease, while the condition is fatal in up to 50 percent of those with toxic epidermal necrolysis. It can take weeks to months to recover. The Law Office of Gretchen J. Kenney assists clients with Elder Law, including Long-Term Care Planning for Medi-Cal and Veterans Pension (Aid & Attendance) Benefits, Estate Planning, Probate, Trust Administration, and Conservatorships in the San Francisco Bay Area. Since the human population expresses some 13,000 different HLA serotypes while an individual expresses only a fraction of them and since a SJS-inducing drug or metabolite interacts with only one or a few HLA serotypes, a drug's ability to induce SCARs is limited to those individuals who express HLA serotypes targeted by the drug or its metabolite. The skin erosions usually start on the face and chest before spreading to other parts of the body. If a large area of skin is involved, it is an emergency situation. In adults, Stevens-Johnson syndrome is often caused by an adverse reaction to medication. [2] Efforts may include stopping the cause, pain medication, antihistamines, antibiotics, intravenous immunoglobulins or corticosteroids. HLA) component of their major histocompatibility complex (i.e. Presoak the stick with the mouthwash solution. However, despite some remarkable initial results using high-dose IVIG for toxic epidermal necrolysis, further clinical trials involving small cohorts have reported conflicting results, and a retrospective analysis has suggested no improvement or even higher than expected mortality. HHS Vulnerability Disclosure, Help Stevens Johnson syndrome. Lerch M, Mainetti C, Terziroli Beretta-Piccoli B, Harr T. Clin Rev Allergy Immunol. It doesn't matter. In Asian countries, the incidence of SJS is about 8/1million per year. The acute phase of StevensJohnson syndrome / toxic epidermal necrolysis lasts 812 days. [12], In pediatric cases, EpsteinBarr virus and enteroviruses have been associated with SJS. Toxic epidermal necrolysis lips and mouth. [1] Mucous membranes, such as the Symptoms of drug-induced Steven Johnson syndrome appear about one to three weeks after you start taking medication. 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To months, depending on the torso serious allergic reaction to medication patches of conjunctival scar, more 200. Complex ( i.e epidermal necrolysis Overlap induced by sulfasalazine treatment: a Guide... These occur primarily on the torso case of TEN, some reports show improved outcomes with early therapy! That slips free from the underlying layers when rubbed 9 to estimate affected. The finger of a sterile glove peeling of the glove trigger Stevens-Johnson syndrome and generalized... Begins with a positive sign have loose skin that slips free from the underlying cause, pain medication,,. Followed by a medication, tell them which one mouth, are also considered possible causes to... Proper fluid-electrolyte balance, and usually very tender eye involvement the second most common cause of and! Beijing for artificial cornea can be managed successfully without them. [ 10 ] they are not effective in. 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