Abstract
This chapter discusses HIV (human immunodeficiency virus), HIV retinopathy, immune recovery uveitis (IRU), pneumocystis carinii choroidopathy, and mycobacterium avium complex (MAC).
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Keywords
- HIV-associated uveitis
- HIV
- HIV retinopathy
- Immune recovery uveitis
- Pneumocystis carinii choroidopathy
- Mycobacterium avium complex
HIV (Human Immunodeficiency Virus)
Overview
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Retrovirus that infects CD4+ T lymphocyte (cell-mediated immunity)
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Severe immunodeficiency permits the development of opportunistic infections and malignancies
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Transmission
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Sexual contact
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Blood: Intravenous Drug Use (IVDU), blood products
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Mother to child
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Highly active antiretroviral therapy (HAART) has led to dramatic improvement in the prognosis of HIV infection
Ocular Manifestations of HIV Infection
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Most common
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Ocular manifestation of human immunodeficiency virus, acquired immunodeficiency syndrome (HIV-AIDS): HIV retinopathy
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Retinal opportunistic infection: Cytomegalovirus (CMV) retinitis
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Multifocal choroiditis
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Tuberculosis (TB), mycobacterium avium complex, histoplasmosis, cryptococcosis, pneumocystosis, other fungi, lymphoma
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Look for disseminated systemic infection
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HIV Retinopathy
Overview
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Definition
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Retinal microvasculopathy
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Most common ocular manifestation of HIV-AIDS
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Symptoms
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Asymptomatic
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Blurring with macular involvement
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Laterality: Mostly bilateral
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Course: Spontaneously resolved within weeks or months
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Age of onset: any
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Gender/race: no gender or racial predilection
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Systemic association: Usually found in HIV patients with low CD4 count
Exam: Ocular
Anterior Segment
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No inflammation
Posterior Segment
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Cotton-wool spots
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Posterior pole, often adjacent to retinal vessels
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Large or progressive lesions should cause suspicion of early CMV retinitis
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Scattered dot blot or flame-shaped hemorrhages
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Microaneurysms
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Isolated perivascular sheathing without opportunistic infections
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Common in African patients (especially children)
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Exam: Systemic
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Variable depending on opportunistic infections
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Acute systemic infection (HIV prodrome)
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Occurs days to weeks after inoculation
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Fever, lymphadenopathy, pharyngitis, rash, myalgia, malaise, headache, nausea, weight loss, diarrhea, night sweats, neurologic symptoms
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Patients are more infectious during acute primary infection
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Latent stage
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Occurs weeks to years after inoculation
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Lymphadenopathy
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Acquired immune deficiency syndrome (AIDS)
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Less common in the setting of HAART
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Designated as AIDS with the onset of opportunistic infections or malignancies that are uncommon in the general population
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Imaging
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FA: hyperfluorescent microaneurysms; microvasculopathy, occlusive disease; blocking defects with hemorrhages
Laboratory and Radiographic Testing
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HIV 1/2 Ag Ab immunoassay
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CD4 count/%
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Viral load
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Complete Blood Count (CBC) with differential
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Comprehensive Metabolic Panel (CMP)
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βhCG
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Serologic screening for various infectious comorbidities
Differential Diagnosis
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Early CMV retinopathy
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Diabetes/hypertensive retinopathy
Treatment
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No treatment for HIV retinopathy
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HAART for systemic infection
Referral/Co-management
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Infectious Disease
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Primary Care
CMV Retinitis
See Chap. 35 Herpesviruses
Immune Recovery Uveitis (IRU)
Overview
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Definition
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An ocular form of immune reconstitution inflammatory syndrome (IRIS) involving increased ocular inflammation in previously opportunistic infected eyes, after improvement of immune function from HAART therapy
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CMV (most common)
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TB
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Toxoplasma
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Typically manifests 2–16 weeks after CD4 count increases above 100 cells/μL following the initiation of HAART
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Symptoms
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Asymptomatic
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Blurring
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Floaters
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Laterality: unilateral or bilateral
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Course: subacute
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Age of onset: any
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Gender/race: no gender or racial predilection; any exposed individual
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Systemic association
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Diseases associated
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CMV retinitis: most common
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Inactive toxoplasmosis
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Tuberculous retinochoroiditis
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Cryptococcal infection
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Risk factors
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Inactive CMV retinitis involving more than 30% of retinal area
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Patients initially treated with cidofovir
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Low CD4+ cell before starting HAART
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Exam: Ocular
Anterior Segment
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Anterior uveitis
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Cataract
Posterior Segment
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Vitritis
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Cystoid macular edema (CME)
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Epiretinal membrane
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Retinal and optic disc neovascularization
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Glaucoma
Exam: Systemic
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Variable depending on opportunistic infections
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Same as HIV retinopathy, but expect systemic course to be improved with immune recovery
Imaging
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OCT: CME, ERM
Laboratory and Radiographic Testing
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CD4 count elevated above 100 cells/μL
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Serologic testing to evaluate for/rule out other forms of inflammation
Differential Diagnosis
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Relapse of the previous infection
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New opportunistic infection
Treatment
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Can be self-limiting
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Appropriate antimicrobial therapy
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Topical corticosteroid for anterior segment inflammation
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Mild vitritis without CME with good vision can be observed
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Intravitreal, periocular, or short-course systemic corticosteroids for vitritis and CME
Pneumocystis carinii Choroidopathy
Overview
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Definition
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Caused by fungus Pneumocystis jirovecii (former name Pneumocystis carinii)
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Choroidal involvement is a sign of disseminated life-threatening fungal infection
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Symptoms
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Loss of central vision or no visual disturbance
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Laterality: mostly bilateral
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Course: subacute
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Age of onset: any
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Gender/race: no predilection; any exposed individual
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Systemic association
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CD4 count below 50 cells/μL (in contrast to CD4 200 cells/μL in pneumonia patient)
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Pulmonary infection
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Extrapulmonary: lymph nodes, spleen, liver, bone marrow
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Exam: Ocular
Anterior Segment
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No anterior chamber reaction
Posterior Segment
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Plaque-like, deep, yellow-white, round or multilobular foci at the posterior pole
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No vitritis or vasculitis
Exam: Systemic
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Full physical examination may reveal findings, especially in lung, lymph nodes, and GI
Imaging
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FA: early hypofluorescence and late staining
Laboratory and Radiographic Testing
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Diagnosis is based on clinical and FA findings
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Systemic workup for disseminated disease: Chest X-ray, sputum culture, CBC, liver function test
Differential Diagnosis
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Fungus: candidiasis, cryptococcosis, histoplasmosis
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Syphilis
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Tuberculosis, mycobacterium avium complex
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Primary intraocular lymphoma
Treatment
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Systemic trimetroprim (15 mg/kg/day)–sulfamethoxazole (75 mg/kg/day) in 3 divided doses, for 21 days
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Followed by secondary prophylaxis until immune reconstitution (CD4 >200 cells/μL)
Referral/Co-management
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Infectious Disease
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Pulmonologist
Mycobacterium Avium Complex (MAC)
Overview
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Definition
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MAC disease typically is a disseminated, multi-organ infection in AIDS patients who are not on HAART.
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The mode of transmission: inhalation, ingestion, or inoculation via the respiratory or gastrointestinal tract.
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Symptoms
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Blurring
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Floaters
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Laterality: unilateral, but bilateral can occur
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Course: subacute
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Age of onset: any
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Gender/race: no gender or racial predilection; any exposed individual
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Systemic association
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CD4 T cells <50 cells/μL
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Fever, night sweats, weight loss, diarrhea, anemia, and abdominal pain
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Exam: Ocular
Anterior Segment
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Granulomatous anterior uveitis
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Iris nodules
Posterior Segment
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Multifocal choroiditis with panuveitis
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Unifocal choroidal infiltrate
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Endophthalmitis
Exam: Systemic
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Hepatomegaly, splenomegaly, lymphadenopathy, and skin lesion
Laboratory and Radiographic Testing
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Ocular fluid or tissue
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Gram, modified Acid fast bacilli (AFB), AFB staining
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Culture and PCR for mycobacterium
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CBC, liver function test, chest X-ray
Differential Diagnosis
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Fungus: candidiasis, cryptococcosis, histoplasmosis, pneumocystosis
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Syphilis
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TB
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Intraocular lymphoma
Treatment
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At least two antimycobacterial drugs to prevent or delay the emergence of resistance
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Azithromycin (500 mg daily) or clarithromycin (500 mg PO twice daily) and ethambutol (15 mg/kg PO daily)
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Alternative therapy: rifabutin (300 mg PO daily), levofloxacin (500 mg PO daily), moxifloxacin (400 mg PO daily), amikacin, streptomycin
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Followed by secondary prophylaxis until immune reconstitution (CD4 >100 cells/μL) and disease controlled
Referral/Co-management
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Infectious disease
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Hunchangsith, B. (2021). HIV-Associated Uveitis. In: Foster, C.S., Anesi, S.D., Chang, P.Y. (eds) Uveitis. Springer, Cham. https://doi.org/10.1007/978-3-030-52974-1_60
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DOI: https://doi.org/10.1007/978-3-030-52974-1_60
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