{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T00:04:38Z","timestamp":1747181078076,"version":"3.40.5"},"reference-count":13,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","license":[{"start":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T00:00:00Z","timestamp":1725148800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background:<\/jats:title>\n            <jats:p>Hemoglobinopathies and thalassemias are widely prevalent autosomal inherited recessive disorders of the structure and synthesis of hemoglobin, respectively. Given the regional heterogeneity of these disorders, this study was undertaken to elucidate the patterns and prevalence of these disorders from this region.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods:<\/jats:title>\n            <jats:p>This was a tertiary-care hospital-based study in southern India over 4 years. Screening for hemoglobin (Hb) disorders was done using Hb high-performance liquid chromatography in patients based on initial screening of complete blood count parameters and for clinically indicated cases.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results:<\/jats:title>\n            <jats:p>A normal Hb HPLC pattern was observed in 404 (72.1%) and abnormal in 156 (27.9%) of 560 cases studied. The abnormalities seen were heterozygous \u03b2-thalassemia in 73 (46.8%), homozygous \u03b2-thalassemia in 19 (12.2%), heterozygous \u03b1-thalassemia in 7 (4.5%), HbH disease and heterozygous \u03b4\u03b2-thalassemia in 1 (0.6%) each, sickle cell trait in 9 (5.8%), sickle cell anemia in 8 (5.1%), sickle \u03b2-thalassemia in 17 (10.9%), HbS+ Hb D-Punjab in 1 (0.6%), heterozygous HbE in 6 (3.8%), homozygous HbE in 2 (1.3%), HbE \u03b2-thalassemia in 3 (1.9%), Hb J-Meerut in 1 (0.6%), Hb Kirksey in 4 (2.6%), unknown \u03b1-hemoglobinopathy in 2 (1.3%), and Hb Lepore in 2 (1.3%) cases. Most of the patients were from the neighboring districts, and some were referred from other states.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion:<\/jats:title>\n            <jats:p>The most common hemoglobin disorders were heterozygous \u03b2-thalassemia in 73 cases (46.8%) and sickle hemoglobinopathy in 35 cases (22.4%). A heterogeneous group of hemoglobin disorders, including uncommon \u03b1-hemoglobinopathies, was found in the study population, likely due to the referral of patients from various regions.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1097\/j.pbj.0000000000000271","type":"journal-article","created":{"date-parts":[[2024,10,23]],"date-time":"2024-10-23T12:00:33Z","timestamp":1729684833000},"update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Screening for hemoglobin disorders and investigating their hematological and demographic profile among patients attending a tertiary-care hospital in southern India\u2014a descriptive study"],"prefix":"10.1097","volume":"9","author":[{"given":"Dheebika","family":"Kuppusamy","sequence":"first","affiliation":[{"name":"Departments of Pathology,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kolar Vishwanath","family":"Vinod","sequence":"additional","affiliation":[{"name":"Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6041-1512","authenticated-orcid":false,"given":"Rakhee","family":"Kar","sequence":"additional","affiliation":[{"name":"Departments of Pathology,"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","published-online":{"date-parts":[[2024,9]]},"reference":[{"key":"R1-20241024","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1111\/j.1365-2141.2011.08742.x","article-title":"The challenge of haemoglobinopathies in resource-poor countries","volume":"154","author":"Weatherall","year":"2011","journal-title":"Br J Haematol."},{"key":"R2-20241024","doi-asserted-by":"crossref","first-page":"480","DOI":"10.2471\/BLT.06.036673","article-title":"Global epidemiology of haemoglobin disorders and derived service indicators","volume":"86","author":"Modell","year":"2008","journal-title":"Bull World Health Organ"},{"key":"R4-20241024","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/BF02715905","article-title":"Endogamy, consanguinity and community genetics","volume":"81","author":"Bittles","year":"2002","journal-title":"J Genet."},{"key":"R6-20241024","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.4081\/thal.2014.1955","article-title":"Control of thalassemia in India","volume":"4","author":"Colah","year":"2014","journal-title":"Thalassemia Rep."},{"key":"R7-20241024","first-page":"513","article-title":"Spectrum of haemoglobinopathies diagnosed by cation exchange-HPLC & modulating effects of nutritional deficiency anaemias from North India","volume":"132","author":"Rao","year":"2010","journal-title":"Indian J Med Res."},{"key":"R8-20241024","doi-asserted-by":"crossref","first-page":"384","DOI":"10.3109\/03630269.2015.1068799","article-title":"Population screening and prevention strategies for thalassemias and other hemoglobinopathies of Eastern India: experience of 18,166 cases","volume":"39","author":"Chatterjee","year":"2015","journal-title":"Hemoglobin"},{"key":"R9-20241024","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/s12687-012-0114-0","article-title":"Prevalence of \u03b2-thalassemia and other haemoglobinopathies in six cities in India: a multicentre study","volume":"4","author":"Mohanty","year":"2013","journal-title":"J Commun Genet."},{"key":"R10-20241024","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1111\/ijlh.12948","article-title":"The phenotypic and molecular diversity of hemoglobinopathies in India: a review of 15 years at a referral center","volume":"41","author":"Nadkarni","year":"2019","journal-title":"Int J Lab Hematol."},{"key":"R11-20241024","doi-asserted-by":"crossref","DOI":"10.18535\/jmscr\/v6i1.102","article-title":"HPLC-based evaluation of Haemoglobinopathies in a tertiary care setting in North Kerala","volume":"6","author":"Nisha","year":"2018","journal-title":"J Med Sci Clin Res."},{"key":"R12-20241024","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/j.cca.2008.10.025","article-title":"Inherited hemoglobin disorders in Andhra Pradesh, India: a population study","volume":"400","author":"Munshi","year":"2009","journal-title":"Clinica Chim Acta Int J Clin Chem."},{"key":"R13-20241024","doi-asserted-by":"crossref","first-page":"1","DOI":"10.5402\/2011\/748939","article-title":"Hemoglobin disorders in South India","volume":"2011","author":"Chandrashekar","year":"2011","journal-title":"ISRN Hematol."},{"key":"R14-20241024","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1002\/ajh.25425","article-title":"How we diagnose and manage altered oxygen affinity hemoglobin variants","volume":"94","author":"Yudin","year":"2019","journal-title":"Am J Hematol."},{"key":"R15-20241024","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1111\/ped.12808","article-title":"Diagnosis and clinical presentation of hemoglobin Kirksey","volume":"58","author":"Hitaka","year":"2016","journal-title":"Pediatr Int."}],"container-title":["Porto Biomedical 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