Connective Tissue Disorders



Connective Tissue Disorders





OVERVIEW AND ASSESSMENT

Connective tissue is a fibrous tissue that supports and connects internal organs, forms bones and the walls of blood vessels, attaches muscles and bones to bones (tendons and ligaments), and replaces tissue following injury (scar tissue). The long fibers of connective tissue contain a protein called collagen.

Connective tissue disorders are also known as rheumatologic disorders. They affect the integrity of the musculoskeletal system and may also affect blood vessels, the skin, and a variety of organs. They are chronic and may lead to disability; however, they can usually be controlled with medication.


Musculoskeletal and Related Assessment

Assessment for connective tissue disorders focuses on the musculoskeletal system, but also involves remote body systems. Clues to connective tissue disorders may be found in the skin, eyes, lungs, and neurologic system. Functional assessment is also important; many questionnaires and scales have been developed to assess patient function or disability related to connective tissue disorders, such as the Health Assessment Questionnaire first published in 1980, available at www.chcr.brown.edu/pcoc/ehaqdescrscoringhaq372.pdf.


Subjective Data

Obtain history of presenting symptoms, including duration and intensity, course of the illness, and impact of symptoms on the patient’s life.



  • Musculoskeletal pain—characteristics.



    • Joint swelling.


    • Morning stiffness.


  • Constitutional symptoms.



    • Fever.


    • Weight loss, anorexia.


    • Fatigue.


  • Involvement of other body symptoms.



    • Skin.


    • Ocular.


    • Pulmonary.


    • Neurologic.


    • Mucous membranes.


    • Gastrointestinal (GI).


  • Depression or psychosis.


  • Self-care activities and functional ability.


  • Social activities and roles.


  • Family history of rheumatic or autoimmune disorders.


Objective Data



  • Musculoskeletal examination:



    • Pain on palpation or range of motion (ROM).


    • Joint swelling, warmth, or erythema.


    • Joint motion restriction.


    • Pain, swelling, or warmth of soft tissues surrounding joints.


    • Deformities.


  • Skin:



    • Skin rash or other abnormalities such as thickening.


    • Alopecia.


  • Oral mucosa:



    • Ulcerations.


    • Dryness.



  • Ocular:



    • Conjunctival inflammation.


    • Dryness.


  • Pulmonary:



    • Adventitious sounds.


    • Friction rub.


  • Neurologic:



    • Foot drop.


    • Muscle weakness.


    • Neurologic deficits.


Laboratory Studies


Anti-Double-Stranded DNA

Anti-double-stranded DNA (anti-dsDNA) is a highly specific marker for diagnosing systemic lupus erythematosus (SLE). This antibody is also useful for monitoring disease progression because anti-dsDNA levels fluctuate with disease activity.


Nursing and Patient Care Considerations



  • High anti-dsDNA levels are associated with the development of lupus nephritis.


  • There is a low false-positive rate (about 5%) in patients with other connective tissue disorders; in patients taking medications such as minocycline, etanercept, infliximab, and penicillamine; in first-degree relatives of patients with lupus; and in some laboratory workers.


  • There is no special preparation for this blood test.


Anticyclic Citrullinated Peptide

Anticyclic citrullinated peptide (anti-CCP) is a marker that helps diagnose rheumatoid arthritis. These autoantibodies are produced in the joint as a result of the synovial immune response of patients with rheumatoid arthritis.


Nursing and Patient Care Considerations



  • Anti-CCP is about as sensitive, but more specific (95% to 98%), than the rheumatoid factor test. Found in approximately 50% to 55% of patients with RA.


  • It may be valuable in cases of early arthritis when symptoms are mild and nonspecific and aggressive treatment is being contemplated. Anti-CCP antibody is now used with the rheumatoid factor as the gold standard in making the diagnosis of rheumatoid arthritis.


  • Some laboratories are currently doing a second generation of the test—the CCP2 assay.


  • No specific preparation is needed for this blood test, but the specimen may need to be sent out for processing, as not all labs perform this test.


Antinuclear Antibody

Antinuclear antibody (ANA) is a test for antibodies to nucleoprotein (autoantibodies or a heterogeneous group of gamma globulins); it is highly sensitive for detecting SLE, but is nonspecific (high false-positive rate).


Nursing and Patient Care Considerations



  • Be alert for drugs that may cause false-positive results.








    Table 30-1 ANA Staining Patterns and Connective Tissue Disorders




















    ANA PATTERN


    CONNECTIVE TISSUE DISORDER


    Peripheral (rim, ring, membranous)


    Active SLE, usually with renal disease


    Homogenous (diffuse)


    SLE, RA


    Speckled


    SLE, RA, scleroderma, Sjögren’s syndrome, mixed connective tissue disorder


    Nucleolar


    Scleroderma


    ANA, antinuclear antibody; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.



  • Results will be reported in a staining pattern (speckled, homogeneous, peripheral, nucleolar) if positive, which correlates to various types of connective tissue disorders and various subsets of SLE (see Table 30-1).


  • Titer will also be reported with positive ANA but does not reflect disease activity or prognosis.


Complement



  • Complement is a complex cascade system that activates proteins as part of the body’s defense against infection.


  • Specific components include CH50 (total complement), C3, and C4; measurement helps determine immune complex formation or agammaglobulinemia.


  • Complement levels are decreased in certain autoimmune diseases, particularly SLE, because of complement consumption and because of activation of proteolytic enzymes and tissue damage. C3 and C4 are often monitored to evaluate activity in SLE.


Nursing and Patient Care Considerations



  • Obtain venous blood sample and refrigerate; send to laboratory promptly because complement deteriorates at room temperature.


  • Serial measurements may be helpful in monitoring the activity of some rheumatic diseases; decreased levels indicate increased disease activity.


C-Reactive Protein

C-reactive protein (CRP) is produced by the liver and is a nonspecific marker for infection and inflammation. It can be used to support the diagnosis for such inflammatory disorders as autoimmune disease, inflammatory bowel disease, and inflammatory arthritis.


Nursing and Patient Care Considerations



  • CRP reacts quicker to inflammatory changes than erythrocyte sedimentation rate; it rises within a few hours of an infection or inflammatory condition and then decreases quickly when inflammation resolves.


  • CRP can be used to differentiate inflammatory from noninflammatory conditions and to monitor effectiveness of treatment.


  • Normal CRP is usually below 10 mg/L; a level over 100 mg/L usually indicates infection or inflammation; however, some inflammatory conditions can raise CRP a thousandfold.









Table 30-2 Synovial Fluid Analysis








































NORMAL COLOR


WHITE BLOOD CELL COUNT


VISCOSITY


CRYSTALS


Normal


Clear, yellow


200/µm3


Normal


None


Osteoarthritis


Clear, slightly turbid


200-600/µm3


Low


None


Gout


Turbid


2,000-75,000/µm3


Low


Monosodium urate


Inflammatory Arthritis


(Rheumatoid arthritis, systemic lupus erythematosus, Sjögren’s syndrome, psoriatic)


Turbid, yellow


2,000-75,000/µm3


Low


None


Septic Arthritis


Pus, very turbid


Generally 80,000/µm3


Low


None



Erythrocyte Sedimentation Rate



  • Erythrocyte sedimentation rate (ESR) determines the rate at which red blood cells (RBCs) fall out of unclotted blood in 1 hour.


  • The test is based on the premise that inflammatory and other disease processes create changes in blood proteins, thus causing aggregation of RBCs that makes them heavier.


Nursing and Patient Care Considerations



  • ESR is generally elevated in most rheumatic illnesses.


  • The test is sensitive for inflammatory condition, but not specific to connective tissue disorders.



    • Result may be elevated by pregnancy, menstruation, medications (such as heparin and oral contraceptives), infection, malignancy, anemia, and advanced age.


    • Result may be reduced by elevated blood glucose or albumin, high phospholipids, or drugs, such as corticosteroids or high-dose aspirin.


  • Most beneficial in monitoring inflammatory disease.


Rheumatoid Factor

Rheumatoid factor (RF) is a test for macroglobulin found in the blood of patients with rheumatoid arthritis (RA) and other disorders. RF has properties of an antibody and may be directed against immunoglobulin.


Nursing and Patient Care Considerations



  • It is not a highly sensitive or specific test.


  • May be positive in 50% to 75% of patients with RA, SLE, and Sjögren’s syndrome. May be false-positive with endocarditis, tuberculosis, syphilis, sarcoidosis, cancer, viral infections, hepatitis C, patients with skin or renal allographs, and in some liver, lung, or kidney diseases.


  • Negative RF does not exclude the diagnosis of RA.


  • Certain disease manifestations, such as severe joint involvement and extra-articular manifestations, may be more frequent in those with high-titer RF.


Other Tests


Synovial Fluid Analysis



  • A sample of synovial (joint) fluid is analyzed for several components.



    • Color.


    • Clarity/turbidity.


    • Viscosity.


    • White blood cell (WBC) count with differential.


    • Crystals and their identification.


  • Arthrocentesis, a sterile procedure, requires antiseptic cleaning agent, local anesthetic, a 20G needle (an 18G needle if infected fluid is suspected), and a 10- to 20-mL syringe.


Nursing and Patient Care Considerations



  • Patients are generally apprehensive about having a needle inserted into a joint. They require reassurance and explanation of the importance of information derived from test results.


  • Assist the health care provider with the test by collecting supplies, maintaining a sterile field, sending joint fluid samples for testing, and monitoring for bleeding following the procedure.


  • Results help to differentiate infection, inflammation, and crystal deposition in a painful joint (see Table 30-2).


GENERAL PROCEDURES AND TREATMENT MODALITIES


Pharmacologic Agents


Connective tissue disorders may be treated with various drugs to relieve pain and to halt or minimize the disease process. Types of agents include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, immunosuppressive agents, and a group of unrelated drugs known as disease-modifying antirheumatic drugs (DMARDs). Drug therapy may be long term and may require frequent evaluation for adverse effects (see Table 30-3). Patient education information about pharmacologic agents used to treat various disorders can be obtained from the National Institute
of Arthrtitis and Musculoskeletal and Skin Disease (www.niams.nih.gov/Health_Info/default.asp) or from the American College of Rheumatology. (ACR; www.rheumatology.org).








Table 30-3 Drug Therapy for Connective Tissue Disorders

























































































DRUG


ACTION


ADVERSE EFFECTS


Anti-Inflammatory Agents


Salicylates




  • Aspirin (may be buffered or enteric coated)




  • Anti-inflammatory, antipyretic, and analgesic effects




  • Tinnitus, gastric intolerance or GI bleeding and purpuric tendencies


Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)




  • Ibuprofen



  • Fenoprofen



  • Naproxen



  • Tolmetin



  • Sulindac



  • Meclofenamate



  • Ketoprofen



  • Salsalate



  • Diclofenac



  • Nabumetone



  • Ketorolac



  • Oxaprozin



  • Flurbiprofen



  • Diflunisa



  • Piroxicam



  • Etodolac



  • Indomethacin



  • Trisalicylate




  • Anti-inflammatory and analgesic effects



  • Mechanism of action may be related to inhibition of prostaglandin synthesis (prostaglandins have a role in inflammatory process, pain, and fever)



  • Nonsteroidal antirheumatic agents for adjunctive treatment of rheumatoid arthritis



  • Sometimes remarkably effective in control of articular symptoms




  • GI irritation: nausea, vomiting, epigastric distress, precipitation and reactivation of peptic ulcer, hepatitis



  • Hematologic: bone marrow depression, anemia, leukopenia, thrombocytopenia purpura



  • Decrease in renal function can precipitate renal failure



  • Central nervous system: headache, dizziness, drowsiness, aseptic meningitis



  • Cardiovascular: edema, dyspnea palpitations


COX-2 Inhibitors




  • Celecoxib



  • Meloxicam




  • Selective prostaglandin inhibition so that the inflammatory process is reduced without reducing protective prostaglandin effects on gastric mucosa




  • Same adverse effects as other NSAIDs, with less risk for GI bleeding


Disease-Modifying Agents


Antimalarial Agents




  • Hydroxychloroquine sulfate



  • Chloroquine phosphate




  • Remission-induction agents for rheumatoid arthritis



  • Used also in certain forms of lupus




  • Ocular toxicity (retinopathy that can result in permanent loss of vision; blurred vision, night blindness, scotoma)


Sulfonamides




  • Sulfasalazine




  • Salicylate, sulfonamide




  • Anorexia, rash, hemolytic anemia


Immunomodulators




  • Etanercept



  • Adalimumab



  • Golimumab



  • Certolizumab pegol




  • Tumor necrosis factor blocker (subcutaneous)




  • Infection, injection site reaction, dyspepsia, aplastic anemia, demyelinating disorders, activation of latent TB infection




  • Infliximab




  • IV tumor necrosis factor blocker




  • Infusion reaction, headache, fatigue, optic neuritis, seizures, lupus-like syndrome, risk of severe infection




  • Abatacept




  • Selective co-stimulation modulator; available as IV or SC




  • Risk of serious infection




  • Tocilizumab




  • Interleukin-receptor blocker




  • Risk of severe infection, hypersensitivity reaction, gastrointestinal perforation, demyelinating disorder, malignancy


Immunosuppressives




  • Methotrexate




  • Exert anti-inflammatory effect by inhibition of cellular replication




  • Bone marrow suppression, hepatic and pulmonary toxicity, reduced resistance to infection




  • Azathioprine




  • Used in patients with inflammatory synovitis refractory to other therapy




  • Bone marrow depression, GI and hepatic toxicity, carcinogenesis




  • Cyclophosphamide




  • Alkylating agent that interferes with the growth of rapidly dividing cells




  • Possibility of a malignancy occurring many years later after initiating therapy can cause sterility, urinary bladder, fibrosis, cystitis




  • Cyclosporine




  • Inhibition of immunocompetent lymphocytes also inhibits lymphokine production and release




  • Renal dysfunction, tremor, hypertension, gum hyperplasia




  • Leflunomide




  • Pyrimidine synthesis inhibitor




  • Diarrhea, liver function test abnormalities, alopecia, rash, upper respiratory infection, hypertension


Corticosteroids




  • Prednisone



  • Prednisolone



  • Triamcinolone



  • Betamethasone



  • Hydrocortisone



  • Dexamethasone



  • Methylprednisolone




  • Potent anti-inflammatory drugs may also reduce immune response



  • Usually used for short-term management of patients with severe limitations




  • Osteoporosis, fractures, avascular necrosis



  • Gastric ulcers, infection susceptibility



  • Hirsutism, acne, moon facies, abnormal fat deposition, edema, emotional disorders, menstrual disorders



  • Hyperglycemia, hypokalemia



  • Hypertension, cataracts


IV, intravenous; SC, subcutaneous.




Physical and Occupational Therapy

Physical and occupational therapy provide a multimodal program to help reduce pain and to improve joint function. Many other measures can be taught to patients for home practice. Components of the program may include:



  • Joint conservation.


  • Energy conservation.


  • Splinting (in rare instances).


  • ROM exercises.


  • Application of heat and cold.


  • Endurance or aerobic conditioning.


  • Modification of home and work environment.


Joint Conservation

Teach or reinforce the following practices:



  • Perform activities using good body mechanics.


  • Maintain ideal body weight—extra weight places undue stress on weight-bearing joints.


  • Use large joints to perform activities—spread the load over as many joints as possible.


  • Perform activities in smooth movements to avoid trauma induced by abrupt movements.


Energy Conservation

Teach or reinforce the following practices:



  • Organize materials, utensils, and tools.


  • Perform lengthy activities in a seated position.


  • Work at an even pace—avoid rushing.


  • Delegate work to others when possible.


Splinting



  • May be used for wrists and hands.


  • Ensure proper application.


  • Periodically inspect for skin irritation, neurovascular compromise, or improper fit.


  • Usually worn during acute stage of inflammation to protect joint.



Other Measures



  • Reinforce correct use and application of heat and cold.


  • Obtain and teach correct use of assistive devices.


  • Reinforce use of behavior-modification and relaxation techniques as adjuncts to therapy.


  • Suggest discussion with health care provider about complementary and alternative therapies. A wide variety of herbal and nutraceutical products have been used and studied, but data remain inconclusive about efficacy (see Table 30-4).



    • Many herbal and supplemental products are marketed for pain, inflammation, repair of cartilage, and boosting the immune system; however, scientific evidence for clear-cut treatment benefit is lacking. There is preliminary evidence for fish oil, gamma-linolenic acid, and the herb thunder god vine.








      Table 30-4 Complementary and Alternative Drug Therapy for Arthritis

















































      DRUG AND EFFECT


      EFFECT


      COMMENTS


      Fish oil (omega-3 fatty acid)


      May reduce tenderness and stiffness; may reduce the need for NSAIDs.


      May interact with blood thinners, antihypertensives. Avoid high-dose fish liver oil, which may cause vitamin A and D toxicity.


      Gamma-linoleic acid (GLA) (omega-6 fatty acid, evening primrose oil, borage, black currant)


      Converted into substances that reduce inflammation to relieve joint pain, stiffness, tenderness, and possibly NSAID use.


      Appears to be safe but some borage oil preparations contain hepatotoxic chemicals. More research on dose and duration is needed.


      Thunder god vine (Tripterygium wilfordii)


      May fight inflammation and suppress the immune system; may relieve rheumatoid arthritis symptoms.


      May cause serious side effects—diarrhea, stomach upset, hair loss, headache, skin rash, menstrual changes, male infertility; long-term use may reduce bone mineral density.


      Boswellia


      Anti-inflammatory and analgesic effects.


      Generally safe. Lab and animal studies have been done; need clinical studies.


      Ginger


      Anti-inflammatory and analgesic effects.


      Large doses may cause GI adverse effects; may cause bleeding if given with anticoagulant. Lab studies have been done, need clinical studies.


      Green tea


      Substances might be useful in rheumatoid arthritis and osteoarthritis.


      More information is needed.


      Turmeric (curcumin)


      Animal studies show protection of joints from inflammation and damage.


      Generally safe, no adverse effects; however, may cause stomach ulcers in high doses with prolonged use. Animal studies have been done, need clinical studies.


      Cayenne pepper (capsicum)


      Thought to deplete substance P, reducing pain transmission


      Applied topically to skin over joints in concentrations of 0.025%-0.25%


      Takes several days to obtain pain relief; do not use with heat application.


      Glucosamine and chondroitin


      Cartilage repair, pain relief


      Glucosamine: 1,000-2,000 mg daily Chondroitin: 800-1,600 mg daily, based on weight


      Bleeding risk if ASA taken with chondroitin; may be effective either alone or as combination; must be taken for several months to achieve noticeable effect. Studies showed conflicting results.


      GLA, gamma-linoleic acid.




    • Reflexology, tai chi, and acupressure or acupuncture have benefited some patients with arthritis and connective tissue disorders. Assist the patient in finding certified providers in these disciplines, if desired.


    • Use of magnets to relieve pain has not shown effectiveness in numerous studies since the concept was introduced.


    • For more information, refer patients to the National Center for Complimentary and Alternative Medicine at http://nccam.nih.gov/health/RA/getthefacts.htm.


  • Advise patient to modify home and work environments, as needed, to install safety devices, and to maintain a safe environment.


  • Advise patient to seek counseling regarding sexuality if joint pain and inflammation are barriers to performance.


  • Reinforce the chronic waxing-and-waning nature of the illness to lessen susceptibility to quackery.




DISORDERS


Rheumatoid Arthritis

Rheumatoid arthritis (RA) is a chronic inflammatory disease that affects joints and other organ systems. RA affects 0.5% to 1% of the population worldwide. 2010 criteria includes synovitis in any joint where no alternative diagnosis can explain the synovitis, and a score of 6/10 in four domains: number and site of involved joints, serologic abnormality, elevated acute phase response (markers of inflammation), and symptom duration. These criteria permit diagnosis at an earlier stage than 1987 ACR criteria, allowing for earlier treatment and less destruction of joints.






Figure 30-1. Pathophysiology of rheumatoid arthritis. (A) Joint structure with synovial swelling and fluid accumulation in joint. (B) Pannus, eroded articular cartilage with joint space narrowing, muscle atrophy, and ankylosis.



Pathophysiology and Etiology



  • Immunologic processes result in inflammation of synovium, producing antigens and inflammatory by-products that lead to destruction of articular cartilage, edema, and production of a granular tissue called pannus (see Figure 30-1).


  • Granulation tissue forms adhesions that lead to decreased joint mobility.


  • Similar adhesions can occur in supporting structures, such as ligaments and tendons, and cause contractures and ruptures that further affect joint structure and mobility.


  • The etiology is unknown but is probably a combined effect of environmental, epidemiologic, infectious, and genetic factors.


  • An infectious agent has not been identified, but many infectious processes can produce a polyarthritis similar to RA.


  • Women are affected more frequently than men.


Clinical Manifestations



  • Arthritis-2010 criteria includes synovitis in any joint, rather than symmetrical joints in the 1987 ACR criteria (see Figure 30-2, page 1062).


  • Skin manifestations.



    • Rheumatoid nodules—elbows, occiput, sacrum.


    • Vasculitic changes—brown, splinterlike lesions in fingers or nail folds.







    Figure 30-2. Rheumatoid arthritis characteristically involves the joints of the hands, wrist, feet, ankles, knees, elbows, and the glenohumeral and acromioclavicular joints and the hips. The articulations of the cervical spine are also affected.


  • Cardiac manifestations.



    • Acute pericarditis.


    • Conduction defects.


    • Valvular insufficiency.


    • Coronary arteritis.


    • Cardiac tamponade—rare.


    • Myocardial infarction, sudden death—rare.


  • Pulmonary manifestations.



    • Asymptomatic pulmonary disease.


    • Pleural effusion, pleurisy.


    • Interstitial fibrosis.


    • Laryngeal obstruction caused by involvement of the cricoarytenoid joint—rare.


    • Pulmonary nodules.


  • Neurologic manifestations.



    • Mononeuritis multiplex—wrist drop, foot drop.


    • Carpal tunnel syndrome.


    • Compression of spinal nerve roots.


    • Distal sensory neuropathy.


  • Other manifestations.



    • Fever.


    • Fatigue.


    • Weight loss.


    • Episcleritis.


Diagnostic Evaluation



  • Complete blood count (CBC)—normochromic, normocytic anemia of chronic disease; may also have iron-deficiency anemia (hypochromic, microcytic); platelets may be elevated with inflammation.


  • RF—positive in up to 70% to 80% of patients with RA; CCP is more specific for RA than RF testing.


  • ESR and CRP—often elevated due to active inflammation.


  • Synovial fluid analysis—see Table 30-2, page 1057.


  • X-rays—changes develop within 2 years.



    • Hands/wrists—marginal erosions of the proximal interphalangeal (PIP), metacarpophalangeal, and carpal joints; generalized osteopenia.


    • Cervical spine—erosions that produce atlantoaxial subluxation (generally after many years).


  • Magnetic resonance imaging (MRI)—detects spinal cord compression that results from C1 to C2 subluxation and compression of surrounding vascular structures. Also detects erosions earlier then x-ray.


  • Bone scan—increased uptake in the joints involved in RA.


  • Ultrasound—detects synovitis and erosion (very user dependent). Musculoskeletal ultrasound is widely used in Europe, but more sporadic use in United States.


  • Synovial biopsy.



    • Inflammatory cells associated with RA.


    • Excludes other causes of polyarthritis by noting the lack of other pathologic findings, such as crystals.


Management




  • NSAIDs to relieve pain and inflammation.


  • DMARDs to reduce disease activity.



    • Monotherapy or combination of older agent, such as methotrexate or hydroxychloroquine, with newer agent, such as tumor necrosis factor (TNF) inhibitor or other biologic agents.


    • Combination of TNF inhibitor and methotrexate has shown greater benefit in improving signs and symptoms, preventing radiologic deterioration of the joint, and improving physical function in comparison with monotherapy.


    • Goal is to have long-term impact on the joints and to prevent disability.


  • Corticosteroids (by mouth or intra-articular administration) to reduce inflammatory process. Generally used for short periods, due to multiple side effects.



  • Local comfort measures:



    • Application of heat and cold did not show benefit in a meta-analysis, but treatment can be individualized.


    • Use of splints to support painful, swollen joints.


    • Use of transcutaneous electrical nerve stimulation (TENS) unit for 15 minutes three times a week may provide some benefit.


    • Iontophoresis—delivery of medication through the skin using direct electrical current.


    • Parrafin wax baths with exercise may be of some benefit.


  • Nonpharmacologic modalities:



    • Behavior modification.


    • Relaxation techniques.


  • Surgery:



    • Synovectomy.


    • Arthrodesis—joint fusion.


    • Total joint replacement.

Jun 14, 2016 | Posted by in NURSING | Comments Off on Connective Tissue Disorders

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