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  • 1
    In: Rheumatology International, Springer Science and Business Media LLC, Vol. 38, No. S1 ( 2018-4), p. 267-274
    Type of Medium: Online Resource
    ISSN: 0172-8172 , 1437-160X
    RVK:
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2018
    detail.hit.zdb_id: 1464208-6
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  • 2
    In: The Lancet Child & Adolescent Health, Elsevier BV, Vol. 3, No. 4 ( 2019-04), p. 255-263
    Type of Medium: Online Resource
    ISSN: 2352-4642
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2019
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  • 3
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2022
    In:  Rheumatology Vol. 61, No. Supplement_2 ( 2022-10-06)
    In: Rheumatology, Oxford University Press (OUP), Vol. 61, No. Supplement_2 ( 2022-10-06)
    Abstract: Juvenile inflammatory myositis are systemic autoimmune diseases of unknown aetiology that are characterized by inflammation of skeleton, muscles, skin, and internal organs. Studies from different regions have reported different incidence and age at onset suggesting that the clinical and demographic features may differ by race and geographic regions. This study aims to describe the characteristics of disease among Libyan children who have been treated in the main rheumatology unit covering most of the population in Libya Objectives To describe the demographic and clinical features of patients with juvenile dermatomyositis To determine the outcome of children with juvenile dermatomyositis and factors affecting the outcome. Patients and methods This is a retrospective descriptive study conducted by reviewing patient records diagnosed with juvenile dermatomyositis from 5/2000–2/2022. Results Twenty-one patients were included, female to male ratio was 6:1. Mean age at disease onset was 7.7 ± 2.8 years and their mean follow-up period was 4.36 ± 3.2 years. Most of the patients (14.7%) presented before 1 month of starting symptoms. Most of the patients (13,0.9%), were diagnosed as dermatomyositis, 2 (9.5%) as polymyositis, 2 (9.5%) as amyopathic myositis, and 4 (19%) as overlap syndrome. Family History of dermatomyositis was positive in 4 (19%) of patients and family history of other autoimmune diseases was positive in 5 (23.8%) patients. Around half of the patients had monocyclic disease course (11, 52.4%), 5 patients (23.8%) had polycyclic disease course and other 5 patients (23.8%) had chronic persistent disease course. MRI proximal muscles and EMG were used for diagnosis in 10 patients (48%) and 16 (76.1%) respectively, and muscle biopsy was used in 2 patients to confirm the diagnosis. One overlap patient had severe lung fibrosis on CT scan chest with restrictive lung disease and 4 patients (19%) had restrictive lung disease with normal CT scan chest. Methyleprednisolone pulses were used in 10 patients (50%) with 3 (15%) who required more than one pulse of intravenous prednisolone. Immunoglobulin was used in 12 (60%) of the patients. All patients needed oral prednisolone and methotrexate was used in 15 (75%) of the patients. Other drugs used were Azathioprine in 3 overlap patients, cyclophosphamide and Mycophenolate mofetil in one patient with overlap syndrome. Hydroxychroroquine was used in 6 (30%) of the patients. In the last visit, 12 patients (60%) were in remission, 7 (58%) of them presented before 1 month of disease onset. Fifty-eight percent of the females and 66.7% of the males were in remission. Five patients (25%) had short stature, 3 (15%) had chronic cutaneous changes, 1 (5%) had arrythmia, 2 (10%) had calcinosis, 2 (10%) had osteoporosis, and one patient died. Conclusion A female predominance was noted with age at presentation comparable to other studies. The most frequent manifestations were skin manifestations and musculoskeletal features were the second most frequent symptoms. Patients has low rate of complications with low rate of calcinosis and mortality and none of them had gastrointestinal manifestations. Males were more likely to be in remission.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 1474143-X
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  • 4
    In: Rheumatology, Oxford University Press (OUP), Vol. 60, No. Supplement_5 ( 2021-11-11)
    Abstract: Juvenile Systemic Lupus Erythematous (JSLE) is a systemic autoimmune disorder with speckled manifestations that can emerge overstretched period of time and can affect any organ system, most frequently the skin, joints, kidneys, and the nervous, hematologic, and cardiovascular systems. The Aim is to examine the clinical features, serologic and laboratory characteristics associated with SLE. To probe and outline Clinical and Immunologic features of Incomplete Lupus Erythematous (ILE) patients who progressed from ILE to SLE. Methods The files of patients diagnosed as SLE in pediatric rheumatology clinic from 2001 to 5/2021 were retrospectively reviewed. Result Thirty SLE cases were included; Females were more prevalent with a female: male ratio of 14:1. Mean age at presentation 11 ± 4 years (range of 5 months-13 years), Disease onset was before sixth birthday in (7%), above twelve years in (40%) of the patients, and 53% of the patients was among 6 –12 years age group (31% & 37% of them fulfilled the SLICC & ACR criteria respectively at diagnosis) .The Mean duration between the onset of symptoms and SLE diagnosis was 6 months ±2 years. The Pre-pubertal age group presented early. At diagnosis, 50% of the patient got SLICC score criteria & lt;4, on other hand 70% of the patients had ACR score criteria less than four Variable The most common presenting feature was arthritis (83%) (polyarticular arthritis) followed by dermatological manifestations (46%) photosensitivity, malar rash, and discoid rash in order of most frequent, fatigability (37%), renal manifestation (23%) most commonly as hematuria, one case diagnosed as lupus nephritis by renal biopsy. ANA was positive in 87%, anti-ds-DNA positive in 40% and Anti sm positive in 17%. The most frequently used medications were steroids and hydroxychlorqiune, the most commonly used steroid sparing medications were azathioprine (43%), Mycophenolate mofetil (40%) and ciclosporin (13%), 26% were on antihypertensive, 3% required rituximab to control their disease and one patient received Eltromboag to treat refractory thrombocytopenia. the mean follow-up duration 46.9 ± 43.6m, 20% lost follow up, 13% died due to disease complications (renal system involvement, thrombocytopenia and neurological system involvements). Conclusion jSLE in Libya is very rare before the sixth birthday and presented early among 6–12 years age group with a delay of less than one year between the first presentation and time of diagnosis. SLICC criteria was sufficient to diagnose the disease in  & gt; 50% of patients. High index of suspension should be maintained because in some patient’s years may be passed before fulfilling the diagnostic criteria. The most common cause of death is renal involvement. Glucocorticoids are the backbone of jSLE treatment in the acute phase. Both azathioprine and Mycophenolate mofetil are sufficient to control the disease in most patients. The disease outcome is accepted in our cohort as most patients have mild disease activity with low dose steroid and a steroid-sparing agent.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 1474143-X
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  • 5
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2023
    In:  Rheumatology Vol. 62, No. Supplement_3 ( 2023-08-10)
    In: Rheumatology, Oxford University Press (OUP), Vol. 62, No. Supplement_3 ( 2023-08-10)
    Abstract: Macrophage Activation Syndrome (MAS) in Systemic Juvenile Idiopathic Arthritis (SoJIA) is a potentially life-threatening condition. The prevalence of MAS in SoJIA population is estimated to be 10%. However, reports suggested that the number of SoJIA patients with subclinical MAS may be as high as 40%. Early recognition of MAS is crucial because timely treatment is important for survival. Objective To describe the clinical and laboratory features of patients with MAS associated with SoJIA Methods Data from cohort of 38 SoJIA patients with and without MAS from January 2006 to January 2020 were included and retrospectively reviewed. Data were collected on demography, clinical/laboratory features and treatment. Results There were 38 patients with SoJIA diagnosed according to the International League Against Rheumatism (ILAR) criteria. 47.4% of them were males and 52.6% were females. They were followed up for a period of 6.3 ± 4.4 years. The mean period from appearance of symptoms to first follow up in rheumatology clinic was 5 ± 6 months. The disease course was monocyclic in 39.5%, polycyclic in 28.9% and persistent in 31.6% of the patients. Of the total cohort, 10% of the patients were classified as having MAS, half of them presented with MAS at diagnosis and the rest developed MAS during the disease course. Only One patient experienced recurrent MAS (3 times). Male to female ratio is 3:1. The mean age at diagnosis was 8 ± 6 years. Skin rash was the most presenting feature in the two groups. Hepatosplenomegaly and serositis were most common in sJIA with MAS patients. In comparison to SoJIA patients without MAS, all patients have thrombocytopenia, leukopenias, with mean ferritin level of 1668 in MAS patient and 947 in non-MAS group. The obligate criteria for the diagnosis of MAS according to the 2016 MAS classification criteria are increased ferritin & gt;684 μg/l, elevated lactate dehydrogenase (LDH) and Glutamic Oxaloacetic Transaminase (GOT). All patients have a declining ESR and high CRP and all patients were anaemic. Bone marrow biopsy was done in 50% of the patients. In SoJIA without MAS, ESR mean level was 94 (±38.5) mm/h, CRP mean level of 62 (± 65.8) mg/dl and platelets count mean level of 561 (±215) × 109/l. Treatment all MAS-SoJIA patients were treated with IV methylprednisolone and IVIG compared with 50% of non-MAS-SoJIA patients. In the MAS group, dose of oral prednisolone was (1–2 mg/kg/day) while in non-MAS group was (0.5–1 mg/kg/day). A patient developed MAS twice while on the 7th dose of anakinra and the third MAS on the 6th dose of tocilizumab. Ciclosporin (3 mg per/kg/day) was the treatment of choice for him. Conclusions In our series, prevalence of MAS was 10% which mainly affect the male which is the same as the estimated prevalence of MAS worldwide. Arthritis occurred less frequent in MAS group cases in comparison to hepatosplenomegaly which was more common among MAS group. Rash and serositis were the most common extra-articular manifestations. The main treatment modality was steroid in all of the patients. All the MAS patients were successfully treated with high dose of IVIG, methylprednisolone and ciclosporin, including the patients who developed MAS while on biologics. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 1474143-X
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  • 6
    In: Arthritis Care & Research, Wiley, Vol. 73, No. 12 ( 2021-12), p. 1722-1729
    Abstract: To assess concordance among criteria for inactive disease (ID) and low disease activity (LDA) in juvenile idiopathic arthritis (JIA) and to seek factors driving discordance. Methods The frequency of fulfillment of existing criteria was evaluated in information on 10,186 patients extracted from 3 cross‐sectional data sets. Patients were divided up according to the functional phenotypes of oligoarthritis and polyarthritis. Concordance between criteria was examined using weighted Venn diagrams. The role of each individual component in explaining discordance between criteria was assessed by calculating the absolute number and percentage of instances in which the component was responsible for discrepancy between definitions. Results Criteria for ID were met by 28.6–41.1% of patients with oligoarthritis and by 24.0–33.4% of patients with polyarthritis. Criteria for LDA were met by 44.8–62.4% of patients with oligoarthritis and by 44.6–50.4% of patients with polyarthritis. There was a 57.9–62.3% overlap between criteria for ID and a 67.9–85% overlap between criteria for LDA. Parent and physician global assessments and acute‐phase reactants were responsible for the majority of instances of discordance among criteria for ID (8.7–15.5%, 10.0–12.3%, and 10.8–17.3%, respectively). Conclusion We found fair concordance between criteria for ID and LDA in JIA, with the main drivers of discordance for ID being physician and parent global assessments and acute‐phase reactants. This observation highlights the need for further studies aimed to evaluate the impact of subjective physician and parent perception of disease remission and of laboratory measures of inflammatory activity on the definition of ID.
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
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    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2016713-1
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  • 7
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    Online Resource
    Oxford University Press (OUP) ; 2023
    In:  Rheumatology Vol. 62, No. Supplement_3 ( 2023-08-10)
    In: Rheumatology, Oxford University Press (OUP), Vol. 62, No. Supplement_3 ( 2023-08-10)
    Abstract: Primary vasculitis syndromes are rare in childhood. The clinical manifestations depend on the vessels involved and can lead to significant mortality and morbidity. Aim To study demographic and clinical characteristics of patients with diagnosed with vasculitis other than Kawazaki disease and Henoch-Schonlein purpora (HSP) and to report the outcome of the patients. Materials and methods This is a case series conducted by reviewing the files of patients diagnosed with vasculitis and followed up at the pediatric rheumatology unit of the Tripoli Children Hospital, Libya from year 2000–2022. Data regarding patients’ demography, time of diagnosis, clinical features, laboratory results, treatment and complications were retrieved. Results A total of 37 cases included in the study. 17 (45.9%) were males and 20 (54%) were females followed up for a mean period of 4.48 ± 4.21. Mean age at diagnosis was 8.39 ± 3.84 years. Regarding the diagnosis, there were 3 (8.1%) patients with Takayasu arteritis, 5 (13.5%) patients with polyarteritis nodosa, 2 (5.4%) patients with cutaneous polyarthritis nodosa, 1 (2.7%) patients with granulomatosis with polyahgitis, 1 (2.7%) patient with microscopic polyangiitis, 3 (8.1%) patients with Chrug-Strauss disease, 16 (43.2%) patients with Behcet disease, 1 (2.7%) patients with cogan syndrome, 3(8.1%) patients with urticarial vasculitis, 1 (2.7%) patient with hypocomplementaemic vasculitis, and 1 (2.7%) patient with DADA2. Mean period between the start of symptoms and diagnosis was 1.4 ± 2.8 years. In 6 (16.2%) of the patients there was family history of vasculitis and in 11 (29.7%) the parents are consanguineous. Angiography revealed abnormalities in 5 (13.5%) of the patients. Skin biopsy was done in 4 (10.8%) patients [2 patients with polyarthritis nodosa and 2 patients with urticarial vasculitis] and renal biopsy was done in 1 (2.7%) patient. Steroid was used in 24 (64.9%), Azathioprine in 4 (10.8%) patients. Cellcept was used in 10 (27%) of the patients. Ciclosporin was used 1(2.7%) patient, cyclophosphamide in 3 (8.1%) patients and Immunoglobulin in 6 (16.2%) of patients. Biologics were used in six patients, adalimumab in one patient which was discontinued because of recurrent abscess. In the two patients who used infliximab, treatment was effective. Three patients were treated with tocilizumab, it was ineffective in two, therefore discontinued. However, tocilizumab was effective in one patient with Takayasu arteritis. Complications developed in 23 (62.2%). Muscle atrophy in 1, deforming arthritis in 1, osteoporosis in 1, mouth ulcers in 2, hypertension in 2, hearing loss in 1, chronic sinusitis in 1, cardiovascular accident in 1, cataract in 1, visual impairment in 3, proteinuria in 2, pulmonary fibrosis in 1, chronic asthma in 3, impaired renal function in 1. Conclusion This is the first report of pediatric vasculitis cases in Libya. It showed a heterogeneous spectrum of vasculitis with high rate of consanguinity and low rate of reported family history of vasculitis. Hopefully this report will increase awareness among clinicians of vasculitis and more cases will be diagnosed.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 1474143-X
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  • 8
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2023
    In:  Rheumatology Vol. 62, No. Supplement_3 ( 2023-08-10)
    In: Rheumatology, Oxford University Press (OUP), Vol. 62, No. Supplement_3 ( 2023-08-10)
    Abstract: Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. In comparison to the other (JIA), systemic juvenile idiopathic arthritis has many distinct clinical features that arthritis may be a non-prominent and/or a delayed manifestation. The Pediatric Rheumatology International Trial Organization (PRINTO), currently is developing a new criterion for JIA classification which will allow for better identification of sJIA cases with delayed onset of arthritis and fail to fulfil the currently used International League Against Rheumatism (ILAR) and Yamaguchi criteria. It is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in ‘pre-arthritic’, pure systemic phase of the illness. Objectives The purpose of this study is to compare the performances of the Yamaguchi diagnostic criteria vs PRINTO criteria vs current ILAR criteria for systemic juvenile idiopathic arthritis. We compared the proportions of patients classified and how closely the 3 instruments are related. Methods The study was performed at the Department of Paediatric Rheumatology in Tripoli children hospital. It is a retrospective study, covering the date range 2006–2020. We used clinicobiologic data recorded within 7 months of diagnosis of Patients diagnosed with sJIA. We assigned a diagnosis of sJIA according to the 3 sJIA classification systems (the Yamaguchi diagnostic criteria, PRINTO criteria and according to ILAR classification). Results Three different classification criteria were analysed in 38 assigned sJIA children (13% of total JIA cases). Females were 28 (52.6%), and mean age of JIA onset was 7 ± 3.9 yr. The mean of the time delay between disease onset and diagnosis was 7 ± 9 months. Overall, the ILAR and PRINTO criteria were fulfilled in a higher number of patients in the study (n = 35) (92% of patients), As compared with the Yamaguchi criteria (n = 29).(76% of patients). Among the 7.9% patients who were unclassifiable using the ILAR criteria were diagnosed as sJIA by both the PRINTO and the Yamaguchi diagnostic criteria. 7.89% neither fulfilled the Yamaguchi or the PRINTO Criteria. The table below compares the performance of the three different criteria. Conclusions In this cohort the ILAR criteria appear to be superior in the early recognition and classification of sJIA. Yamaguchi diagnostic criteria can only be used to classify a minority of sJIA patients, leaving a large proportion of sJIA patients unclassified. Compared with the Yamaguchi criteria, ILAR JIA categories aligned better with PRINTO criteria. All the criteria should incorporate strengths of each other. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 1474143-X
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  • 9
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2023
    In:  Rheumatology Vol. 62, No. Supplement_3 ( 2023-08-10)
    In: Rheumatology, Oxford University Press (OUP), Vol. 62, No. Supplement_3 ( 2023-08-10)
    Abstract: Juvenile psoriatic arthritis (JPsA) is a relatively rare condition in childhood as it represents ∼5% of the whole Juvenile Idiopathic Arthritis (JIA). There are fewer reports describing the characteristics and outcome of patients with JPsA. Aim To describe the characteristic features and the treatment modalities of JPsA among Libyan children in pediatric rheumatology clinic at the Tripoli Children Hospital which is the only referral clinic covering the western, northern and southern part of Libya and to compare our results with other populations worldwide. Methods All the medical records of the children who were diagnosed to have JPsA according to ILAR classification criteria or Vancouver criteria (definite or probable) from January 2001 to January 2020 were included and retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment Results The study included a total of 30 cases of JPsA that represents a 12% of total JIA cases over the study period, the mean age at presentation was 5.8 ± 5.3 years with male to female ratio of 1:1, with the mean duration of symptoms prior to diagnosis of JIA of 9 ± 10 months. The foremost common type of arthritis was peripheral polyarthritis (83% & 58.3%), with the Small joints commonly affected, 30% of patients had hip joint involvement. 13% of the patient had psoriasis, half of them before presentation of arthritis. Nail changes secondary to psoriasis was seen in 1 of the cohort, and the percentage of patients with dactylitis among the group was 30%. Family history of psoriasis found in 83% of the cohort, 26% found to be ANA positive. Chronic uveitis occurred in 10%. Patients of the total cohort, ANA positive, Unilateral anterior uveitis being the most common presentation. The following table summarizes the Medications used during the course of the disease At the time of the first visit 100%of total patients fulfilled the Vancouver criteria for juvenile PsA, While 5 of the12 patients didn’t fulfilled the ILAR criteria for JPsA. Conclusions Juvenile psoriatic arthritis constituted 12% of our patients with juvenile idiopathic arthritis. Juvenile psoriatic arthritis constitutes 8% to20% of JIA patients worldwide with equal gender distribution and peak age at presentation of 6–13 years. More than four peripheral joints involvement were the most frequent presentation. Half of the patients had skin involvement before the development of arthritis. Prevalence of chronic anterior uveitis was higher in our cohort than worldwide (10% vs 5% respectively) as an extra articular manifestation. All patients were treated with NSAIDs at presentation and/or during follow-up. Methotrexate was the most commonly used second-line agent Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 1474143-X
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  • 10
    In: Arthritis Care & Research, Wiley, Vol. 73, No. 4 ( 2021-04), p. 586-592
    Abstract: To report the cumulative articular and extraarticular damage in Arab children with juvenile idiopathic arthritis (JIA) and to identify variables that correlate with disease damage. Methods We conducted a multicenter, cross‐sectional study among 14 pediatric rheumatology centers from 7 Arab countries. JIA patients who met the International League of Associations for Rheumatology classification criteria and had a disease duration of 〉 1 year were enrolled. Disease activity status was assessed using the Juvenile Arthritis Multidimensional Assessment Report. Disease damage was assessed by the Juvenile Arthritis Damage Index, articular (JADI‐A) and extraarticular (JADI‐E). Results A total of 702 (471 female) JIA patients with a median age of 11.3 years (interquartile range [IQR] 8.0–14.0 years) were studied. Median age at disease onset was 5 years (IQR 2.0–9.0 years) and the median disease duration was 4 years (IQR 2.0–7.0 years). The most frequent JIA categories were oligoarticular JIA (34.9%), polyarticular JIA (29.5%), and systemic JIA (24.5%). Clinical remission was achieved in 73.9% of patients. At the last clinic visit, 193 patients experienced joint damage, with a mean ± SD JADI‐A score of 1.7 ± 4.5, while 156 patients had extraarticular damage, with a mean ± SD JADI‐E score of 0.5 ± 1.1. Patients with enthesitis‐related arthritis had the highest JADI‐A score. JADI‐A correlated significantly with the presence of a family history of JIA. JADI‐A and JADI‐E had a significant correlation with long disease duration. Conclusion Cumulative damage was common in this Arab JIA cohort, and consanguinity and JIA in a sibling were frequent findings and were associated with a greater cumulative damage.
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    RVK:
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2016713-1
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