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Article ; Online: Primary immunodeficiency associated with hypopigmentation: A differential diagnosis approach.

Zamani, Raha / Shahkarami, Sepideh / Rezaei, Nima

2021 Volume 49, Issue 2, Page(s) 178–190

Abstract: Primary immunodeficiency diseases (PIDs) are a group of more than 400 disorders representing aberrant functioning or development of immune system. Hypopigmentation syndromes also characterize a distinguished cluster of diseases. However, hypopigmentation ...

Abstract	Primary immunodeficiency diseases (PIDs) are a group of more than 400 disorders representing aberrant functioning or development of immune system. Hypopigmentation syndromes also characterize a distinguished cluster of diseases. However, hypopigmentation may also signify a feature of genetic diseases associated with immunodeficiency, such as Chediak-Higashi syndrome, Griscelli syndrome type 2, Hermansky-Pudlak syndrome type 2 and type 10, Vici syndrome, and P14/LAMTOR2 deficiency, all of which are linked with dysfunction in vesicular/endosomal trafficking. Regarding the highly overlapping features, these disorders need a comprehensive examination for prompt diagnosis and effective management. As an aid to clinician, distinguishing the pathophysiology, clinical phenotype, and diagnosis as well as treatment options of the six mentioned PID disorders associated with hypopigmentation are described and discussed in this review.
MeSH term(s)	Diagnosis, Differential ; Genetic Testing ; Humans ; Hypopigmentation/diagnosis ; Hypopigmentation/genetics ; Hypopigmentation/immunology ; Hypopigmentation/therapy ; Primary Immunodeficiency Diseases/diagnosis ; Primary Immunodeficiency Diseases/genetics ; Primary Immunodeficiency Diseases/immunology ; Primary Immunodeficiency Diseases/therapy ; Skin Pigmentation/genetics ; Skin Pigmentation/immunology
Language	English
Publishing date	2021-03-01
Publishing country	Singapore
Document type	Journal Article ; Review
ZDB-ID	193144-1
ISSN	1578-1267 ; 0301-0546
ISSN (online)	1578-1267
ISSN	0301-0546
DOI	10.15586/aei.v49i2.61
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Article ; Online: Novel Mutations of the TYMP Gene in Mitochondrial Neurogastrointestinal Encephalomyopathy: Case Series and Literature Review.

Mojtabavi, Helia / Fatehi, Farzad / Shahkarami, Sepideh / Rezaei, Nima / Nafissi, Shahriar

Journal of molecular neuroscience : MN

2021 Volume 71, Issue 12, Page(s) 2526–2533

Abstract: Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a multi-system disorder caused by several homozygous or compound heterozygous mutations, mostly in the nuclear gene of TYMP. Our current knowledge on the underlying pathology of the disease ...

Abstract	Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a multi-system disorder caused by several homozygous or compound heterozygous mutations, mostly in the nuclear gene of TYMP. Our current knowledge on the underlying pathology of the disease is derived through the study of about 200 cases of different ethnicities. Clinical presentations include severe cachexia, weakness, ptosis, diplopia, abdominal cramps or digestive tract disorders, hearing impairment, and paresthesia.Herein, we aim to present five novel mutations of the nuclear gene of TYMP in six Iranian patients diagnosed with MNGIE. In our population, age at the time of diagnosis was 18 to 49 years, while the onset of the symptoms varied from 13 to 20 years. We detected two pathogenic non-frameshift nonsense premature stop codon mutations (c.1013C > A, and c.130C > T), one variant of uncertain significance (VUS) non-frameshift missense mutation (c.345G > T), one likely pathogenic frameshift insertion (c.801_802insCGCG), and one likely benign homozygous non-frameshift deletion (c.1176_1187del) from two siblings. Our findings also confirm the autosomal recessive inheritance pattern of MNGIE in the Iranian population. The lack of knowledge in the area of nuclear gene-modifier genes shadows the genotype-phenotype relationships of MNGIE.
MeSH term(s)	Adolescent ; Codon, Nonsense ; Female ; Genes, Recessive ; Humans ; Intestinal Pseudo-Obstruction/genetics ; Intestinal Pseudo-Obstruction/pathology ; Iran ; Male ; Muscular Dystrophy, Oculopharyngeal/genetics ; Muscular Dystrophy, Oculopharyngeal/pathology ; Ophthalmoplegia/congenital ; Ophthalmoplegia/genetics ; Ophthalmoplegia/pathology ; Phenotype ; Thymidine Phosphorylase/genetics ; Thymidine Phosphorylase/metabolism ; Young Adult
Chemical Substances	Codon, Nonsense ; TYMP protein, human (EC 2.4.2.4) ; Thymidine Phosphorylase (EC 2.4.2.4)
Language	English
Publishing date	2021-04-06
Publishing country	United States
Document type	Case Reports ; Journal Article ; Review
ZDB-ID	1043392-2
ISSN	1559-1166 ; 0895-8696
ISSN (online)	1559-1166
ISSN	0895-8696
DOI	10.1007/s12031-021-01822-w
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Article ; Online: Novel CARMIL2 (RLTPR) Mutation Presenting with Hyper-IgE and Eosinophilia: A Case Report.

Zamani, Raha / Zoghi, Samaneh / Shahkarami, Sepideh / Seyedpour, Simin / Jimenez Heredia, Raúl / Boztug, Kaan / Rezaei, Nima

Endocrine, metabolic & immune disorders drug targets

2023

Abstract: Background: Inborn errors of immunity are a growing group of disorders with a wide spectrum of genotypic and phenotypic profiles. CARMIL2 (previously named RLTPR) deficiency is a recently described cause of immune dysregulation, mainly presenting with ... ...

Abstract	Background: Inborn errors of immunity are a growing group of disorders with a wide spectrum of genotypic and phenotypic profiles. CARMIL2 (previously named RLTPR) deficiency is a recently described cause of immune dysregulation, mainly presenting with allergy, mucocutaneous infections, and inflammatory bowel disease. CARMIL2 deficiency is categorized under diseases of immune dysregulation with susceptibility to lymphoproliferative conditions. Case presentation: Here we describe a 29-years-old male from a consanguineous family, with food and sting allergy, allergic rhinitis, facial molluscum contagiosum (viral infection of the skin in the form of umbilicated papules), eosinophilia and highly elevated serum IgE level. Whole exome sequencing revealed numerous homozygous variants, including a CARMIL2 nonsense mutation, a gene regulating actin polymerization, and promoting cell protrusion formation. Conclusion: The selective role of CARMIL2 in T cell activation and maturation through cyto-skeletal organization is proposed to be the cause of immune dysregulation in individuals with CARMIL2 deficiency. CARMIL2 has an important role in immune pathways regulation, through cell maturation and differentiation, giving rise to a balance between Th1, Th2, and Th17 immune response. This case can improve the understanding of the different impacts of CARMIL2 mutations on immune pathways and further guide the diagnosis of patients with similar phenotypes.
Language	English
Publishing date	2023-10-04
Publishing country	United Arab Emirates
Document type	Case Reports
ZDB-ID	2228325-0
ISSN	2212-3873 ; 1871-5303
ISSN (online)	2212-3873
ISSN	1871-5303
DOI	10.2174/0118715303263327230922043929
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Article: Minimal Residual Disease Detection Using Gene Scanning Analysis, Fluorescent Fragment Analysis, and Capillary Electrophoresis for IgH Rearrangement in Adult B-Lineage Acute Lymphoblastic Leukemia: A Cross-Sectional Study.

Shahkarami, Sepideh / Younesian, Samareh / Rostami, Shahrbano / Kompani, Farzad / Bashash, Davood / Mousavi, Seyed Asadollah / Ghaffari, Seyed H

Cell journal

2023 Volume 25, Issue 2, Page(s) 85–91

Abstract: Objective: Minimal residual disease (MRD) is considered the greatest prognostic factor in acute lymphoblastic leukemia (ALL). MRD is a valuable tool for anticipating impending relapse and treatment response assessment. The objective of the present study ...

Abstract	Objective: Minimal residual disease (MRD) is considered the greatest prognostic factor in acute lymphoblastic leukemia (ALL). MRD is a valuable tool for anticipating impending relapse and treatment response assessment. The objective of the present study was to investigate whether the detection of IgH gene rearrangement using polymerase chain reaction (PCR)-based GeneScan analysis could be a complementary method to monitor MRD along with the quantitative realtime PCR (qPCR). Materials and methods: In this cross-sectional study, we valued the MRD levels, based on the GeneScanning analysis (GSA), and then compared the data with quantitative real-time polymerase chain reaction at different time points in peripheral blood (PB) samples of adult B-lineage ALL patients (n=35). The specific polymerase chain reaction (PCR) primers for IGH gene FR-1 and fluorescence-labeled J-primer were used and analyzed by capillary gel electrophoresis on a sequencer. The results of this study were compared with the previously reported MRD results obtained by the IGH rearrangements allele-specific oligonucleotide (ASO) -qPCR methods. Results: The total concordance rate was 86.7%, with a P<0.001. MRD results obtained by GSA and ASO-qPCR methods were concordant in all diagnostic samples and samples on the 14th and 28th days of induction therapy. The results of these 2.5 years' follow-ups demonstrated a significant correlation between the two techniques (r=0.892, P<0.001). Conclusion: It seems that the PCR-based GeneScan analysis of IGH gene rearrangement detection may be a valuable molecular technique to distinguish monoclonality from polyclonality. And also, it may be a precise tool to detect the residual leukemic DNA in the PB follow-up samples of patients.
Language	English
Publishing date	2023-02-01
Publishing country	Iran
Document type	Journal Article
ZDB-ID	2647430-X
ISSN	2228-5814 ; 2228-5806
ISSN (online)	2228-5814
ISSN	2228-5806
DOI	10.22074/cellj.2023.557390.1049
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Article: DNAH11

Sodeifian, Fatemeh / Samieefar, Noosha / Shahkarami, Sepideh / Rayzan, Elham / Seyedpour, Simin / Rohlfs, Meino / Klein, Christoph / Babaie, Delara / Rezaei, Nima

Case reports in medicine

2023 Volume 2023, Page(s) 8436715

Abstract: Background: Primary ciliary dyskinesia (PCD), also known as the immotile-cilia syndrome, is a clinically and genetically heterogeneous syndrome. Improper function of the cilia causes impaired mucociliary clearance. Neonatal respiratory distress, ... ...

Abstract	Background: Primary ciliary dyskinesia (PCD), also known as the immotile-cilia syndrome, is a clinically and genetically heterogeneous syndrome. Improper function of the cilia causes impaired mucociliary clearance. Neonatal respiratory distress, rhinosinusitis, recurrent chest infections, wet cough, and otitis media are respiratory presentations of this disease. It could also manifest as infertility in males as well as laterality defects in both sexes, such as situs abnormalities (Kartagener syndrome). During the past decade, numerous pathogenic variants in 40 genes have been identified as the causatives of primary ciliary dyskinesia. Conclusion: We reported a novel homozygous nonsense variant in
Language	English
Publishing date	2023-04-25
Publishing country	United States
Document type	Case Reports
ZDB-ID	2502642-2
ISSN	1687-9635 ; 1687-9627
ISSN (online)	1687-9635
ISSN	1687-9627
DOI	10.1155/2023/8436715
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Article ; Online: A novel X-linked mutation in IL2RG associated with early-onset inflammatory bowel disease: a case report of twin brothers.

Rayzan, Elham / Sadeghalvad, Mona / Shahkarami, Sepideh / Zoghi, Samaneh / Aryan, Zahra / Mahdaviani, Seyed Alireza / Boztug, Kaan / Rezaei, Nima

Journal of medical case reports

2023 Volume 17, Issue 1, Page(s) 307

Abstract: Background: X-linked severe combined immunodeficiency is caused by IL2RG gene mutation. Several variations have been identified in the IL2RG gene, which potentially can prevent the production of nonfunctional proteins. Herein, a novel X-linked variant ... ...

Abstract	Background: X-linked severe combined immunodeficiency is caused by IL2RG gene mutation. Several variations have been identified in the IL2RG gene, which potentially can prevent the production of nonfunctional proteins. Herein, a novel X-linked variant in the IL2RG gene is reported in twin brothers, associated with inflammatory bowel symptoms. Case presentation: The patients were 26-month-old monozygotic twin middle-eastern males with failure to thrive and several inpatient admissions due to severe chronic nonbloody diarrhea that started at the age of 12 months. Pancolitis was revealed after performing upper and lower gastrointestinal endoscopies on the twin with more severe gastrointestinal symptoms. Flow cytometric evaluation of the peripheral blood cells showed low levels of CD4+ cells in both patients. Next generation sequencing-based gene panel test results of the two patients proved a novel heterozygous missense X-linked IL2RG mutation (70330011 A > G, p.Trp197Arg) in one of the patients, which was predicted to be deleterious (CADD score of 28), which soon after was confirmed by Sanger segregation in his twin brother. Both parents were wild types and had never experienced similar symptoms. The patients received an human leukocyte antigen (HLA)-matched cord blood transplant. The twin with more severe gastrointestinal symptoms died 1 month after transplantation. In his brother, watery diarrhea eventually subsided after transplantation. Conclusion: Intestinal involvement in X-linked severe combined immunodeficiency is a rare presentation that might be neglected. The increasing availability of genetic screening tests worldwide could be helpful for early detection of such lethal primary immunodeficiency diseases and in implementing effective interventions to handle the severe outcomes.
MeSH term(s)	Male ; Humans ; Infant ; Child, Preschool ; X-Linked Combined Immunodeficiency Diseases/genetics ; Siblings ; Mutation ; Inflammatory Bowel Diseases/genetics ; Diarrhea/genetics ; Interleukin Receptor Common gamma Subunit/genetics
Chemical Substances	IL2RG protein, human ; Interleukin Receptor Common gamma Subunit
Language	English
Publishing date	2023-07-18
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	2269805-X
ISSN	1752-1947 ; 1752-1947
ISSN (online)	1752-1947
ISSN	1752-1947
DOI	10.1186/s13256-023-04049-y
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Article ; Online: A 3-Year-Old Boy with an Xp21 Deletion Syndrome: A Case Report.

Sadeghmousavi, Shaghayegh / Shahkarami, Sepideh / Rayzan, Elham / Ahmed, Souran / Gharalari, Farzaneh Hosseini / Rohlfs, Meino / Klein, Christoph / Rezaei, Nima

Endocrine, metabolic & immune disorders drug targets

2022 Volume 22, Issue 8, Page(s) 881–887

Abstract: Background: Chromosome Xp21 deletion syndrome is a rare X-linked recessive defect that occurs as a result of multiple gene deletions, including Glycerol kinase (GK) and its neighboring genes, dystrophin, which causes Duchenne muscular dystrophy (DMD), ... ...

Abstract	Background: Chromosome Xp21 deletion syndrome is a rare X-linked recessive defect that occurs as a result of multiple gene deletions, including Glycerol kinase (GK) and its neighboring genes, dystrophin, which causes Duchenne muscular dystrophy (DMD), and NR0B1, which causes congenital adrenal hypoplasia (CAHhttps://www.omim.org/entry/300200). Patients usually present with glycerol kinase deficiency, congenital adrenal hypoplasia, Duchenne muscular dystrophy, hyperglycerolemia, and glyceroluria, associated with DMD and/or CAH, growth failure, myopathy, osteoporosis, mental retardation, and psychomotor retardation. Case presentation: Herein, we report a 3-year- old boy from Iraq who had bloody diarrhea, food intolerance and abdominal cramp, adrenal insufficiency, recurrent fevers, tuberculosis (TB) infection, cervical abscess, oral thrush, cervical and mediastinal lymphadenopathies, developmental delay, and undescended testis. His parents are non-consanguine and had no family history of diseases. Next generation sequencing demonstrated a hemizygote deletion in chromosome X. Conclusion: Loss of a large part of the X-chromosome most likely can explain the clinical findings of this patient. Contiguous gene deletion syndrome in Xp21 should be considered after diagnosing adrenal insufficiency to treat metabolic complications efficiently.
MeSH term(s)	Adrenal Insufficiency ; Child, Preschool ; Glycerol Kinase ; Humans ; Hypoadrenocorticism, Familial ; Male ; Muscular Dystrophy, Duchenne ; Syndrome ; X Chromosome
Chemical Substances	Glycerol Kinase (EC 2.7.1.30)
Language	English
Publishing date	2022-01-31
Publishing country	United Arab Emirates
Document type	Case Reports
ZDB-ID	2228325-0
ISSN	2212-3873 ; 1871-5303
ISSN (online)	2212-3873
ISSN	1871-5303
DOI	10.2174/1871530322666220201143656
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Article ; Online: A novel VPS13B mutation in Cohen syndrome: a case report and review of literature.

Momtazmanesh, Sara / Rayzan, Elham / Shahkarami, Sepideh / Rohlfs, Meino / Klein, Christoph / Rezaei, Nima

BMC medical genetics

2020 Volume 21, Issue 1, Page(s) 140

Abstract: Background: Cohen syndrome, an autosomal recessive syndrome, is a rare syndrome with diverse clinical manifestations including failure to thrive, hypotonia, hypermobile joints, microcephaly, intellectual disabilities, craniofacial and limb anomalies, ... ...

Abstract	Background: Cohen syndrome, an autosomal recessive syndrome, is a rare syndrome with diverse clinical manifestations including failure to thrive, hypotonia, hypermobile joints, microcephaly, intellectual disabilities, craniofacial and limb anomalies, neutropenia and a friendly character. It is associated with mutations of the vacuolar protein sorting 13 homolog B (VPS13B) gene, which is involved in the development of the ocular, hematological and central nervous systems. This gene encodes a transmembrane protein playing a crucial role in preserving the integrity of the Golgi complex. To date, more than 150 mutations of VPS13B have been reported in over 200 Cohen syndrome patients. Missense or nonsense mutations are the most common mutations. Case presentation: A 4-year-old girl, born to consanguineous parents, was referred to the pediatric clinical immunology outpatient clinic for investigation of recurrent neutropenia with a history of recurrent infections in the past year. On physical examination, she had the characteristic facial features of Cohen syndrome, developmental delay and speech disorder. She had a cheerful disposition, and her mother gave a history of feeding difficulties in her first months of life. She did not present any ophthalmologic or cardiac abnormalities. Her lab results revealed moderate neutropenia. Serum IgG, IgM, IgA and IgE levels were normal. She fulfilled the clinical diagnostic criteria for Cohen syndrome. WES revealed a novel homozygous frameshift variant in VPS13B (LRG_351t1: c.7095del; p.Ser2366AlafsTer49). Currently, she is not experiencing any severe problem, and she undergoes irregular medical treatment once her neutrophil count decreases under the normal limit. Her verbal and motor abilities have improved as a result of speech and occupational therapies. Conclusion: We reported a novel homozygous frameshift variant in VPS13B (LRG_351t1: c.7095del; p.Ser2366AlafsTer49) in a 4-year-old girl with Cohen syndrome. Cohen syndrome should be considered in differential diagnosis of any child with intellectual disability and neutropenia.
MeSH term(s)	Child, Preschool ; Developmental Disabilities/genetics ; Female ; Fingers/abnormalities ; Humans ; Intellectual Disability/genetics ; Microcephaly/genetics ; Muscle Hypotonia/genetics ; Mutation/genetics ; Myopia/genetics ; Obesity/genetics ; Phenotype ; Retinal Degeneration/genetics ; Vesicular Transport Proteins/genetics
Chemical Substances	VPS13B protein, human ; Vesicular Transport Proteins
Language	English
Publishing date	2020-06-30
Publishing country	England
Document type	Case Reports ; Journal Article ; Review
ISSN	1471-2350
ISSN (online)	1471-2350
DOI	10.1186/s12881-020-01075-1
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Article: Enhanced CO2 Adsorption Using MgO-Impregnated Activated Carbon: Impact of Preparation Techniques

Shahkarami, Sepideh / Dalai Ajay K / Soltan Jafar

Industrial & Engineering Chemistry Research. 2016 May 25, v. 55, no. 20

2016

Abstract: The development of a facile and sustainable approach to produce magnesium oxide (MgO) activated carbons impregnated through a single-step activation of biochar is reported. In a single-step activation process, biochar is impregnated with 3 and 10 wt % of ...

Abstract	The development of a facile and sustainable approach to produce magnesium oxide (MgO) activated carbons impregnated through a single-step activation of biochar is reported. In a single-step activation process, biochar is impregnated with 3 and 10 wt % of magnesium salt solutions followed by steam activation. In a two-step method, activated carbon, the product of steam activation of biochar, is impregnated with magnesium salt using the incipient wetness and excess solution impregnation process and calcined. The impacts of activation method, impregnation method, and metal content are evaluated, and the product qualities are compared in terms of porosity and surface chemistry. The sorbents are then used for COâ capture in low partial pressure of COâ at 25 and 100 Â°C from a feed containing 15% COâ in Nâ in a fixed-bed reactor. The incipient wetness of activated carbons results in the highest COâ uptake (49 mg/g) at 25 Â°C, while single-step impregnation of biochar with rinsing step yields the largest surface area (760 mÂ²/g) and the second highest COâ uptake (47 mg/g). The increase in Mg content from 3 to 10 wt % results in the smaller surface area and higher COâ uptake suggesting that the metal content has a greater impact than porosity and surface area. Rinsing the Mg impregnated activated carbon with water results in the larger surface area and higher COâ uptake in all samples. Moreover, the COâ adsorption runs at 100 Â°C shows a 65% increase using MgO impregnated activated carbon as compared to steam activated carbon indicating that MgO impregnation of activated carbon can overcome the limitation of using nontreated activated carbon at moderate operating temperature of 100 Â°C and low partial pressure of COâ of 15 mol %.
Keywords	activated carbon ; adsorption ; biochar ; carbon dioxide ; engineering ; magnesium ; magnesium oxide ; nitrogen ; porosity ; sorbents ; steam ; surface area ; temperature
Language	English
Dates of publication	2016-0525
Size	p. 5955-5964.
Publishing place	American Chemical Society
Document type	Article
ZDB-ID	1484436-9
ISSN	1520-5045 ; 0888-5885
ISSN (online)	1520-5045
ISSN	0888-5885
DOI	10.1021%2Facs.iecr.5b04824
Database	NAL-Catalogue (AGRICOLA)

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Article ; Online: Novel

Shaka, Zoha / Mojtabavi, Helia / Rayzan, Elham / Zoghi, Samaneh / Shahkarami, Sepideh / Raul, Jimenez Heredia / Sedighi, Iraj / Boztug, Kaan / Rezaei, Nima

Allergologia et immunopathologia

2021 Volume 49, Issue 2, Page(s) 80–83

Abstract: Introduction and objectives: X-linked agammaglobulinemia (XLA), the first known primary immunodeficiency, is caused by rare mutations in Bruton's tyrosine kinase (BTK) gene. Mutations in the BTK gene lead to a failure in the development and maturation ... ...

Abstract	Introduction and objectives: X-linked agammaglobulinemia (XLA), the first known primary immunodeficiency, is caused by rare mutations in Bruton's tyrosine kinase (BTK) gene. Mutations in the BTK gene lead to a failure in the development and maturation of B-cell linage. A decreased number of B-cells results in agammaglobulinemia and increased susceptibility to a variety of infections. Therefore, patients with XLA usually manifest with repetitive bacterial infections, such as upper respiratory tract infections, septic arthritis, osteomyelitis, and urinary tract infections, since their infancy. Patients: We report a 17-year-old Iranian boy with XLA, referred to us with a history of severe and recurrent episodes of bacterial infections for a period of six years. Results: Genetic analysis using the whole Exome sequencing revealed a hemizygous missense mutation in the BTK gene (c.428 A > T, p.His143Leu). Conclusion: To our knowledge, c.428 A > T has not been reported in the BTK gene.
MeSH term(s)	Adolescent ; Agammaglobulinaemia Tyrosine Kinase/genetics ; Agammaglobulinemia/blood ; Agammaglobulinemia/diagnosis ; Agammaglobulinemia/genetics ; Agammaglobulinemia/immunology ; DNA Mutational Analysis ; Genetic Diseases, X-Linked/blood ; Genetic Diseases, X-Linked/diagnosis ; Genetic Diseases, X-Linked/genetics ; Genetic Diseases, X-Linked/immunology ; Genetic Testing ; Humans ; Iran ; Male ; Mutation, Missense ; Pedigree
Chemical Substances	Agammaglobulinaemia Tyrosine Kinase (EC 2.7.10.2) ; BTK protein, human (EC 2.7.10.2)
Language	English
Publishing date	2021-03-01
Publishing country	Singapore
Document type	Case Reports ; Journal Article
ZDB-ID	193144-1
ISSN	1578-1267 ; 0301-0546
ISSN (online)	1578-1267
ISSN	0301-0546
DOI	10.15586/aei.v49i2.62
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