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'Background': [1], 'Alpha‐1': [2], 'antitrypsin': [3], 'deficiency': [4], '(': [5], 'AATD': [6, 71, 87, 146, 221, 232], ')': [7], 'is': [8, 51, 61, 236], 'estimated': [9], 'to': [10, 32, 70, 137, 155, 172, 194, 238, 253, 257], 'affect': [11], 'three': [12], 'million': [13], 'people': [14], 'worldwide.': [15], 'It': [16], 'causes': [17], 'liver': [18, 59, 67, 82, 94, 157, 167, 176, 216, 246, 258], 'disease': [19, 68, 83, 138, 217, 247], 'in': [20, 164, 204, 209, 218], 'a': [21], 'proportion': [22], 'of': [23, 25, 37, 43, 81, 93, 145, 151, 161, 215, 229, 244], 'carriers': [24], 'the': [26, 33, 40, 62, 76, 91, 112, 118, 143, 185], 'PiS': [27], 'and': [28, 35, 78, 89, 114, 140, 178, 190, 206, 252], 'PiZ': [29], 'allele': [30], 'due': [31, 69], 'formation': [34], 'retention': [36], 'polymers': [38], 'within': [39], 'endoplasmic': [41], 'reticulum': [42], 'hepatocytes.': [44], 'The': [45, 212], 'reason': [46], 'for': [47, 66, 124, 142], 'this': [48], 'selective': [49], 'penetrance': [50], 'not': [52], 'known.': [53], 'Although': [54], 'clinical': [55, 213], 'trials': [56], 'are': [57], 'underway,': [58], 'transplantation': [60, 95, 183], 'only': [63, 186], 'effective': [64, 187], 'treatment': [65, 144, 188], '.': [72, 147, 233], 'Aims': [73], 'To': [74], 'report': [75], 'prevalence': [77], 'natural': [79], 'history': [80], 'among': [84], 'individuals': [85, 162, 219], 'with': [86, 159, 197, 220, 231], ',': [88], 'assess': [90], 'outcomes': [92, 128, 191], 'through': [96], 'systematic': [97], 'review.': [98], 'Methods': [99], 'A': [100], 'comprehensive': [101], 'search': [102], 'was': [103, 184], 'conducted': [104], 'across': [105], 'multiple': [106], 'databases.': [107], 'Two': [108], 'independent': [109], 'authors': [110], 'selected': [111], 'articles': [113], 'assessed': [115], 'bias': [116], 'using': [117], 'Newcastle‐Ottawa': [119], 'Scale.': [120], 'Data': [121], 'were': [122, 129, 134, 153], 'pooled': [123], 'analysis,': [125], 'where': [126], 'comparable': [127], 'reported.': [130], 'Results': [131], 'Thirty‐five': [132], 'studies': [133], 'identified': [135], 'related': [136], 'progression': [139], '12': [141], 'Seven': [148], 'per': [149], 'cent': [150], 'children': [152, 205], 'reported': [154, 189, 200], 'develop': [156], 'cirrhosis,': [158], '16.5%': [160], 'presenting': [163], 'childhood': [165], 'requiring': [166], 'transplantation.': [168, 181, 259], 'Of': [169], 'those': [170, 230, 240], 'surviving': [171], 'adulthood,': [173], '10.5%': [174], 'had': [175], 'cirrhosis': [177], '14.7%': [179], 'required': [180, 237], 'Liver': [182], 'compare': [192], 'favourably': [193], 'other': [195], 'indications,': [196], '5‐year': [198], 'survival': [199], 'as': [201], 'over': [202, 207], '90%': [203], '80%': [208], 'adults.': [210], 'Discussion': [211], 'course': [214], 'remains': [222], 'poorly': [223], 'understood,': [224], 'but': [225], 'affects': [226], 'about': [227], '10%': [228], 'More': [234], 'research': [235], 'identify': [239], 'patients': [241], 'at': [242, 248], 'risk': [243], 'developing': [245], 'an': [249], 'early': [250], 'stage,': [251], 'provide': [254], 'alternative': [255], 'treatments': [256]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2787643951', 'counts_by_year': [{'year': 2024, 'cited_by_count': 8}, {'year': 2023, 'cited_by_count': 15}, {'year': 2022, 'cited_by_count': 7}, {'year': 2021, 'cited_by_count': 11}, {'year': 2020, 'cited_by_count': 14}, {'year': 2019, 'cited_by_count': 10}, {'year': 2018, 'cited_by_count': 10}], 'updated_date': '2024-09-19T01:33:15.853348', 'created_date': '2018-02-23'}