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Primary localized cutaneous amyloidosis

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Primary localized cutaneous amyloidosis (PLCA) is a condition in which clumps of abnormal proteins called amyloids build up in the skin, specifically in the wave-like projections (dermal papillae) between the top two layers of skin (the dermis and the epidermis). The primary feature of PLCA is patches of skin with abnormal texture or color Amyloidosis comprises a spectrum of diseases, which range from systemic to localized cutaneous types, and is characterized by the extracellular deposition of amyloidosis protein as beta-pleated sheets. The forms of amyloidosis are differentiated by the specific types of protein-derived amyloidosis fibers Primary cutaneous amyloidosis is a form of amyloidosis, a group of conditions in which an abnormal protein (called amyloid) builds up in various organs and tissues throughout the body. In primary cutaneous amyloidosis, specifically, this protein accumulates in the skin. There are three main forms of primary cutaneous amyloidosis

Abstract Fifty-seven patients with primary localized cutaneous amyloidosis (PLCA) were clinically and histopathologically reviewed. Two-thirds of patients had macular amyloidosis (MA). Intermediate cases having macular lesions with micropapules and/or lichens were identified Primary localized cutaneous amyloidosis (PLCA) is the deposition of amyloid in an apparently normal skin without deposits in the internal organs., Amyloid in PLCA is believed to be caused by apoptosis of keratinocytes with a filamentous degeneration A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Primary localized cutaneous nodular amyloidosis Skip to main content U.S. Department of Health & Human Service Primary localized cutaneous amyloidosis is a group of rare conditions where amyloid deposition is limited to the skin without systemic manifestations. Most cases are sporadic; however, mutations in the oncostatin M receptor (OSMR) and interleukin-31 receptor A (IL31RA) genes can cause a familial form of the condition in up to 10% of cases

Primary localized cutaneous amyloidosis (PLCA) is a relatively rare condition characterized by amyloid deposition exclusively in the dermis without involving the internal organs. Clinically, papular, macular and tumefactive forms are presented Primary localized cutaneous amyloidosis is often divided into macular and lichen subtypes although many affected individuals often show both variants coexisting. Lichen amyloidosis characteristically presents as a pruritic eruption of grouped hyperkeratotic papules with a predilection for the shins, calves, ankles and dorsa of feet and thighs In primary localised cutaneous amyloidosis (PLCA) amyloid deposits only occur in the skin. The exception to this is a rare disease called nodular amyloid, which can be associated with amyloid deposits in other body organs and myeloma (a form of bone marrow cancer)

Lichen amyloidosis is one of the two common forms of primary localised cutaneous amyloidosis. It presents as an intensely itchy rash on the shins, thighs, feet, and forearms. Lesions consist of multiple raised spots (papules) that are scaly and red/brown. Papules may coalesce into thickened plaques Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. There are different subtypes of PLCA, namely: lichen amyloidosis (LA), macular amyloidosis (MA), and (primary localized cutaneous) nodular amyloidosis (NA) [ 1 ] Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare form of cutaneous amyloidosis. We report the case of a 65-year-old woman with multiple asymptomatic discrete nodules and atrophic plaques on the thighs of 4 years' duration that had increased in number and size Conjunctival amyloidosis is a unique localized process rarely associated with systemic involvement that usually occurs in middle-aged adults. It generally involves the palpebral conjunctiva but may occur anywhere in the conjunctiva. Patients may present with symptoms of pain or ptosis, or may remain completely asymptomatic

Overview. Primary cutaneous amyloidosis is a form of amyloidosis, a group of conditions in which an abnormal protein (called amyloid) builds up in various organs and tissues throughout the body.In primary cutaneous amyloidosis, specifically, this protein accumulates in the skin. There are three main forms of primary cutaneous amyloidosis:[11065][11066 Primary localized cutaneous amyloidosis is characterized clinically by pruritus and skin scratching and histologically by the finding of deposits of amyloid staining on keratinous debris in the papillary dermis (summary by Tanaka et al., 2009). Genetic Heterogeneity of Primary Localized Cutaneous Amyloidosis Practice Essentials Nodular localized cutaneous amyloidosis (NLCA) is a rare condition characterized by the deposition of amyloid or amyloid-like proteins in the dermis. The cause of NLCA is not.. Nodular amyloidosis may progress to primary systemic disease in up to 50% of cases. Because our patient had no systemic involvement and the lesions did not appear nodular in nature, the patient was given a diagnosis of primary localized AL cutaneous amyloidosis

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  1. Primary localized cutaneous amyloidosis is characterized clinically by pruritus and skin scratching and histologically by the finding of deposits of amyloid staining on keratinous debris in the papillary dermis (summary by Tanaka et al., 2009).. For a general description and a discussion of genetic heterogeneity of PLCA, see 105250. Molecular Genetic
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  3. From Wikipedia, the free encyclopedia Primary cutaneous amyloidosis is a form of amyloidosis associated with oncostatin M receptor. This type of amyloidosis has been divided into the following types: Macular amyloidosis is a cutaneous condition characterized by itchy, brown, rippled macules usually located on the interscapular region of the back
  4. Nodular primary localized cutaneous amyloidosis (NPLCA) is the least reported of the primary localized cutaneous amyloidoses and it is well accepted that a percentage of patients with NPLCA ultimately progress to systemic disease.1, 2, 3,.
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  6. Primary localized cutaneous amyloidosis (PLCA), first described in 1970 [1], is characterized by amyloid deposition in the superficial dermis without systemic manifestations. The precise etiology of PLCA remains poorly understood, although several risk factors have been identified, e.g., South American or Asian heritage, female sex, family.

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Primary localized cutaneous amyloidosis (PLCA) is a condition in which clumps of abnormal proteins called amyloids build up in the skin, specifically in the wave-like projections (dermal papillae) between the top two layers of skin (the dermis and the epidermis). The primary feature of PLCA is patches of skin with abnormal texture or color Primary spotted cutaneous amyloidosis. Primary spotted cutaneous amyloidosis is characterized by the appearance of significantly itchy spots 2-3 cm in diameter, brown or brown, which are localized on the trunk, but most often in the upper part of the back, the interblade area

The manifestations of primary localized cutaneous amyloidosis (PLCA) are usually limited to the skin. The exact etiopathogenesis of PLCA has not been clearly elucidated yet. An increasing number of reports in the literature that associate PLCA with various autoimmune/immune disorders suggest that underlying immune‐mediated factors may be implicated. We report a case of sarcoidosis and a case. Primary localized cutaneous amyloidosis may be classified into three major types: lichen amy- loidosis, macular amyloidosis, and nodular or tu- mefactive amyloidosis. In the rare nodular form of primary localized cutaneous amyloidosis, the amyloid depositffappear as single or multiple pink to yellowish brown waxy nodules on the trunk. Primary cutaneous amyloidosis is a form of amyloidosis associated with oncostatin M receptor. This type of amyloidosis has been divided into the following types:: 520 Macular amyloidosis is a cutaneous condition characterized by itchy, brown, rippled macules usually located on the interscapular region of the back.: 521 Combined cases of lichen and macular amyloidosis are termed biphasic. Cite this entry as: (2004) Primary localized cutaneous amyloidosis. In: Levine N., Levine C.C. (eds) Dermatology Therapy. A to Z Essentials

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  1. or trauma [10]
  2. Primary cutaneous amyloidosis is a condition where amyloid protein is deposited in apparently normal skin without associated deposits in internal organs. Primary systemic amyloid may develop as a consequence of an underlying abnormality of the plasma cells in the bone marrow or plasma cell cancer (multiple myeloma). In systemic amyloidosis, the.
  3. Steciuk A et al (2002) Cutaneous amyloidosis and possible association with systemic amyloidosis. Int J Dermatol 41: 127-132; Weidner et al (2017) Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review. At J Clin Dermatol 18:629-64
Primary cutaneous amyloidosis - wikidoc

related amyloidosis.19 Primary localized cutaneous nodular amyloidosis is characterized by the formation of amyloid fibrils that consist of immunoglobulin light chains, referred to as the amyloid L (AL) type, which is the same type of amyloid fibril protein seen in primary systemic amy-loidosis and myeloma-associated systemic amyloidosis Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare form of cutaneous amyloidosis. We report the case of a 65-year-old woman with multiple asymptomatic discrete nodules and atrophic plaques on the thighs of 4 years' duration that had increased in number and size. Results of extensive clinical, histologic, and laboratory evaluation showed no evidence of systemic amyloidosis or. Primary localized cutaneous amyloidosis is a form limited to the skin without involving any other localization. An amorphous material composed of amyloid is produced and deposited in the dermis, with a varied clinical presentation. Nodular amyloidosis is a rare presentation of primary localized skin amyloidosis

Primary Localized Conjunctival Amyloidosis - EyeWiki

Abstract Familial primary localized cutaneous amyloidosis (FPLCA) is an autosomal dominant disorder associated with chronic itching and skin lichenification. [ncbi.nlm.nih.gov] Tanaka's study [ 16 ] demonstrated that most changes of gene expression revealed alterations in epidermal differentiation and proliferation consistent with lichenification Efficacy of 308-nm Excimer Laser for Primary Localized Cutaneous Amyloidosis Treatment in Asians. The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogeneic amyloid proteins in the skin without systemic involvement. Types of PLCA include the fo.

Primary localized cutaneous amyloidosis: MedlinePlus Genetic

Nodular primary localized cutaneous amyloidosis after trauma: a case report and discussion of the rate of progression to systemic amyloidosis. J Am Acad Dermatol . Aug 2007. 57(2 Suppl):S26-9 PLCNA - Primary Localized Cutaneous Nodular Amyloidosis. Looking for abbreviations of PLCNA? It is Primary Localized Cutaneous Nodular Amyloidosis. Primary Localized Cutaneous Nodular Amyloidosis listed as PLCNA. Primary Localized Cutaneous Nodular Amyloidosis - How is Primary Localized Cutaneous Nodular Amyloidosis abbreviated?. Primary localized cutaneous amyloidosis. At least 13 mutations in the OSMR gene have been found to cause primary localized cutaneous amyloidosis (PLCA) type 1, an itchy skin condition in which clumps of abnormal proteins called amyloids build up in the skin. These mutations change single protein building blocks (amino acids) in OSMRβ Primary cutaneous amyloidosis (PCA) is a form of localized amyloidosis. It is characterized by the deposition of a fibrillar material in the superficial dermis, without affecting other systems or organs. The diagnosis can be made clinically, but usually a skin biopsy is performed in order to exclude other skin diseases with similar appearance

Cutaneous amyloidosis. Cutaneous deposits of amyloid can be discerned in 29-40% of the cases with primary systemic amyloidosis, but they are very rare in the other systemic types. If the cutaneous amyloidosis appears not to be a symptom of a systemic amyloidosis, it is called a primary localized cutaneous amyloidosis Familial primary localized cutaneous amyloidosis (FPLCA) is an autosomal-dominant disorder associated with chronic skin itching and deposition of epidermal keratin filament-associated amyloid material in the dermis. FPLCA has been mapped to 5p13.1-q11.2, and by candidate gene analysis, we identified missense mutations in the OSMR gene, encoding oncostatin M-specific receptor β (OSMRβ), in. An autosomal dominant primary localised cutaneous amyloidosis in Taiwanese families is related to a mutation in the IL-31RA gene which encodes the protein, interleukin-31 receptor A [2]. Women present with macular amyloidosis more often than men; this may be due to women seeking medical attention earlier than men due to cosmetic concerns

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Primary localized cutaneous amyloidosis - PubMe

Primary cutaneous amyloidosis Genetic and Rare Diseases

  1. Rare localized cutaneous amyloidosis, which occurs slowly without itching (in contrast to lichen amyloidosus) and in which system involvement cannot (or initially cannot) be detected.The amyloid is derived from immunoglobulin light chains (AL amyloid). Transition to systemic amyloidosis is possible (transitions are seen in 10-50% of cases), so that patients require constant medical monitoring
  2. Primary localized cutaneous nodular amyloidosis (nodular amyloidosis) is a rare and distinct type of amyloidosis, in which amyloid L deposition is limited to the skin and typically manifested as a tumefactive nodule on the acral sites. However, the definite cause of nodular amyloidosis is still unknown
  3. We have reported that apoE was a component of amyloid deposits of primary localized cutaneous amyloidosis (PLCA) using immunohistochemistry, immunogold electron microscopy, and immunoblotting (Furumoto et al, 1998). PLCA, lichen amyloido-sus, and macular amyloidosis are characterized by the ®nding that the amyloid deposits are limited to the.
  4. Lip-switch flap, is a lower lip flat of mucosa, muscle, skin, and subucutis that is transposed superiorly to the repair an upper lip defect based on inferior labial artery blood supply. What is an Abbe flap. Jessner's is a light-medium chemical peel that contains salicylic acid, lactic acid, ethanol, and resorcinol
  5. NIH GARD Information: Primary localized cutaneous nodular amyloidosis. This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD)

  1. Primary localized nodular cutaneous amyloidosis is the rarest form of primary localized cutaneous amyloidosis, accounting for 1.5% of all cases, and differentiates from the other forms (lichen amyloidosis and macular amyloidosis), Figure 1. Ulcerated and crusted alopecic nodule of the vertex showing diffuse hair (a
  2. tissues.1 In primary localized cutaneous amyloidosis, deposition of amyloid occurs in previously normal skin with no evidence of deposits occurring in internal organs.2 The term primary cutaneous amyloidosis (PLCA) usually includes macular amyloidosis (MA), lichen amyloidosis (LA) and nodular amyloidosis
  3. Primary localized cutaneous amyloidosis (PLCA) is a chronic itchy skin disorder associated with amyloid deposits in the superficial dermis. It is a purely cutaneous disease with no association with systemic forms of amyloidosis. Clinically, skin lesions comprise small, flat-top papules (lichen amyloidosis) or brown-gray macules (macular.
  4. Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren's syndrome, and in a few cases with systemic sclerosis
  5. of primary systemic amyloidosis.3 Localised forms of amyloid deposits may also occur as a primary disease in the eye, or secondary to skin conditions like basal cell carcinoma, Bowen's disease and seborrhoeic keratosis.4 We report a case of primary localised deep cutaneous amyloidosis of the eyelid in the absence of systemic amyloidosis
  6. Primary cutaneous amyloidosis has been reported in different body areas including eyelid 1 and the auricular concha. 2 Cutaneous amyloidosis may be associated with systemic amyloidosis. There are case reports of sarcoidosis and IgA nephropathy in association with extensive macular amyloidosis

Primary localized cutaneous amyloidosis 1 (Concept Id

Primary cutaneous amyloidosis is characterized by amyloid deposition in the skin without systemic involvement. This article reviews the three main variants of primary cutaneous amyloidosis, lichen, macular, and nodular, and briefly discusses rare forms OBSERVATION Familial Primary Localized Cutaneous Amyloidosis in Brazil Thais H. Sakuma, MD; Gunter Hans-Filho, MD, PhD; Ken Arita, MD; Mac¸anori Odashiro, MD cutaneous amyloidosis. Ze!Converter - Download Video From Dailymotion to mp4, mp3, aac, m4a, f4v, or 3gp for free! cutaneous amyloidosis - this is an unpleasant disease. The photos of cutaneous amyloidosis below are not recommended for people with a weak psyche

Video: Primary localized cutaneous amyloidosis - A

Primary localized cutaneous nodular amyloidosis Genetic

Pruritic Patches on the Back and Papules on the Legs

Primary localized cutaneous amyloidosis (PLCA) shows deposition of amyloid in previously apparently normal skin, with no evidence of deposits occurring in internal organs. PLCA is classified into macular and papular forms, and a rare nodular form (NPLCA) Amyloid elastosis is a rare form of cutaneous amyloidosis characterized histologically by the deposition of amyloid-coating elastic fibers of the skin. To date, only 4 cases have been reported, all of them in the setting of systemic amyloidosis. We present the first case of primary cutaneous localized amyloid elastosis Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory. Primary localized cutaneous amyloidosis of eyelid is not a common condition and can be overlooked easily. In this condition we can see Amyloid light chains (AL) deposits in the tissue. As this condition is usually associated with B-cell or Plasma cell proliferation so plasma cell dyscrasia must be excluded.[9,11,12-16

Nodular Amyloidosis | Dermatology | JAMA Dermatology | The

Primary Localized Cutaneous Amyloidosis Affecting Female

Nodular cutaneous amyloidosis (NCA) is the rarest form of primary cutaneous amyloidosis. The amyloid fibrils of NCA are not unique to NCA but are also the prevailing amyloid component in primary systemic amyloidosis (PSA) and myeloma-associated systemic amyloidosis. Age of presentation in NCA has ranged from 20 to 87 years without a clear. Primary localized cutaneous nodular amyloidosis (PLCNA) is the rarest form of PCA and the only one in which the amyloid deposits are of the amyloid light-chain (AL) type, as in the primary and myeloma-associated systemic forms of amyloidosis. AL amyloidosis is due to monoclonal immunoglobulin (Ig) light-chain deposition How is Primary Localized Cutaneous Nodular Amyloidosis abbreviated? PLCNA stands for Primary Localized Cutaneous Nodular Amyloidosis. PLCNA is defined as Primary Localized Cutaneous Nodular Amyloidosis very rarely Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. Int J Dermatol, 49 (2010), pp. 229-23 Primary Cutaneous Lichen Amyloidosis is a rare condition in which there is a deposition of amyloid in the dermis layer of the skin without systemic involvement. The term amyloid means the extracellular proteinaceous deposits that are resistant to the proteolytic digestion and has distinctive physical properties

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Primary Localized Cutaneous Amyloidosis in Patients with

Primary localized cutaneous amyloidosis presents in either a lichen or a macular form. They are best considered as different manifestations of the same disease process. Lichen amyloidosis is characterized by closely set, discrete, brown-red papules that often show some scaling and are most commonly located on the legs, especially the shins One particular pruritic skin disorder often seen by dermatologists is primary localized cutaneous amyloidosis (PLCA [MIM 105250]). This condition usually presents with itching (especially on the lower legs) and visible changes of skin hyperpigmentation and thickening (lichenification) that may be exacerbated by chronic scratching and rubbing. 2. Amyloidosis is a group of conditions that involve the deposition of amyloid proteins in various tissues. It is generally classified as either systemic or cutaneous. Primary localized cutaneous nodular amyloidosis (PLCNA) is the rarest form of cutaneous amyloidosis with the amyloid depositing in the dermis, subcutis, and blood vessel walls

Amyloidosis, primary localized cutaneous,

Abstract The cutaneous amyloidoses represent a heterogeneous group of conditions in which amyloid, a fibrillar material that can result from the degradation of various proteins, is deposited in the skin. In primary cutaneous amyloidosis, the deposits are derived from keratin (macular, lichen, biphasic) or immunoglobulin light chains (nodular) To the Editor: Localized cutaneous amyloidoses can be divided into primary cutaneous amyloidosis and secondary cutaneous amyloidosis. Primary cutaneous amyloidosis is defined as deposition of amyloid in the skin in the absence of systemic involvement. Secondary cutaneous amyloidosis is associated with secondary amyloid deposition and is accompanied by skin neoplasms such as seborrheic. Macular amyloidosis typically presents as gray-brown pruritic macules, which gradually coelesce into patches with a rippled pattern involving the upper back (Figure 1) and less often the arms, chest and thighs. Macular amyloidosis is one of the more common types of primary localized cutaneous amyloidoses Monoclonal anti-cytokeratin antibody reacted with the 45K protein of the amyloid-positive skin extract. These results indicate that apoE is a component of primary localized cutaneous amyloidosis, and that it might play an important role in primary localized cutaneous amyloidosis Abstract. Primary localized cutaneous nodular amyloidosis (nodular amyloidosis) is a rare and distinct type of amyloidosis, in which amyloid L deposition is limited to the skin and typically manifested as a tumefactive nodule on the acral sites

Primary Localised Cutaneous Amyloidosi

Primary localized cutaneous nodular amyloidosis is a rare variant of cutaneous amyloidosis. Approximately 100 cases of nodular cutaneous amyloidosis have been reported in the medical literature. [1] Cutaneous nodular amyloidosis is composed of light-chain immunoglobulins (AL amyloid) derived from a monoclonal expansion of plasma cells Primary localized cutaneous amyloidosis (PLCA) occurs when amyloid is deposited only within the skin and there is no evidence of systemic involvement. Nodular amyloidosis is the rarest subtype of PLCA. It typically involves the acral regions but can sometimes present on the head and neck. The condition usually presents clinically as a single tan or yellow nodule or plaque that may appear waxy Primary localized cutaneous amyloidosis (PLCA) consists of the deposition of amyloid in previously healthy skin with no systemic involvement, and is usually classified into three major forms, lichen amyloidosis, the commonest type, macular, and a rare nodular form.1,2 Macular and papular forms may co-exist in the same patient and is known a A rare case of recurrent primary cutaneous nodular amyloidosis (PCNA) involving both sides of the face is being reported from India where the initial lesion on the right side of face was operated upon and recurred in 1 year. Around the same time the patient developed a similar lesion on the left side. The patient refused any treatment after being informed about the relatively innocuous nature. Background: Primary localized cutaneous amyloidosis is a commonly encountered problem in our scenario.As there is paucity of Indian studies on this subject, a clinico-epidemiological study was carried out. Objectives of the study were to make an insight into the common clinical variants of primary localized cutaneous amyloidosis, to compare the age and sex distribution of various forms of PLCA.

Amyloidosis DermNet N

findings, a diagnosis of primary localized cutaneous nod-ular amyloidosis (PLCNA) was made. The patient under-went shave excision of the lesions under regional anesthesia. A six-month follow-up showed no signs of lo-cal recurrence or systemic progression (Fig. 1E, F). PLCNA is a rarest form of cutaneous amyloidosis that i In primary localized cutaneous amyloidosis (PLCA), which in- cludes lichenoid amyloidosis (LA), macular (MA), bi- phasic amyloidosis (combined type of lichenoid and macular erup- tions), and nodular amyloidosis, amino acid sequence analysis demonstrated that the fibril protein of nodular type has.

Primary Localized Cutaneous Amyloidosis: A Systematic

Primary cutaneous amyloidosis is a form of amyloidosis associated with oncostatin M receptor. This type of amyloidosis has been divided into the following types Alerts and Notices Synopsis Nodular amyloidosis is an uncommonly encountered form of primary cutaneous amyloidosis (a category which also includes lichen and macular amyloidosis, poikiloderma-like cutaneous amyloidosis, primary cutaneous amyloidosis of the auricular concha, and the exceedingly rare entity of amyloidosis cutis dyschromica).In nodular amyloidosis, the material deposited in the.

Journal of Cutaneous and Aesthetic Surgery (JCAS): Table
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