{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T18:15:40Z","timestamp":1774548940459,"version":"3.50.1"},"reference-count":29,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2017,6,1]],"date-time":"2017-06-01T00:00:00Z","timestamp":1496275200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematology Reports"],"abstract":"<jats:p>In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years\u2019 retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median age was 10.5 years (range 1\u201324 years). No child was diagnosed by neonatal screening. The median age of diagnosis of sickle cell anemia was 90 months (range: 8\u2013250 months). The median age at the first transfusion was 36 months (range 4\u2013168). In this series, 61 (56.5%) patients were eligible for hydroxyurea. However, this treatment was only performed in 4 (6.6%) of them. Pain episodes, acute anemic crisis and severe infection represent respectively 38.2%, 34.3% and 21.9% of events. Altered sensorium and focal deficit were encountered occasionally and represented 3.4% of acute events. Acute renal manifestations, cholelithiasis and priapism were rarely reported, in this cohort. In Kinshasa, the care of patients suffering from sickle cell anemia is characterized by the delayed diagnosis and low detection of organ complications compared to reports of Western countries. This situation is due to resources deficiencies.<\/jats:p>","DOI":"10.4081\/hr.2017.6952","type":"journal-article","created":{"date-parts":[[2017,6,1]],"date-time":"2017-06-01T09:52:17Z","timestamp":1496310737000},"page":"6952","source":"Crossref","is-referenced-by-count":10,"title":["Acute Crises and Complications of Sickle Cell Anemia among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic of Congo"],"prefix":"10.3390","volume":"9","author":[{"given":"Michel Ntetani","family":"Aloni","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bertin Tshimanga","family":"Kadima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P\u00e9p\u00e9 Mfutu","family":"Ekulu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Al\u00e9ine Nzazi","family":"Budiongo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ren\u00e9 Makuala","family":"Ngiyulu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean Lambert","family":"Gini-Ehungu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2017,6,1]]},"reference":[{"key":"ref1","unstructured":"www.who.ch\/programme\/ncd\/hgm\/haemogl.htm."},{"key":"ref2","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1111\/j.1365-3148.2009.00943.x","article-title":"Prevalence of sickle cell disease in a northeastern region of the Democratic Republic of Congo: what impact on transfusion policy?","volume":"20","author":"Agasa","year":"2010","journal-title":"Transfus Med"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1136\/jcp.2008.058958","article-title":"Neonatal screening for sickle cell anemia in the Democratic Republic of Congo: experience from a pioneer project on 31.204 newborns","volume":"62","author":"Tshilolo","year":"2009","journal-title":"J Clin Pathol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"e1552","DOI":"10.1542\/peds.2010-3686","article-title":"Management of children with sickle cell disease: a comprehensive review of the literature","volume":"128","author":"Kavanagh","year":"2011","journal-title":"Pediatrics"},{"key":"ref5","series-title":"World Malaria Report","year":"2011"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"196","DOI":"10.3109\/03630269.2014.896810","article-title":"Challenge of managing sickle cell disease in a pediatric population living in Kinshasa, Democratic Republic of Congo: a sickle cell center experience","volume":"38","author":"Aloni","year":"2014","journal-title":"Hemoglobin"},{"key":"ref7","first-page":"513","article-title":"Management of children with sickle cell disease in Africa: experience in a cohort of children at the Royal Albert Hospital in Dakar","volume":"63","author":"Diagne","year":"2003","journal-title":"Med Trop (Mars)"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/j.arcped.2009.01.005","article-title":"Hemoglobin sickle cell disease: experience of the Yalgado Ouedraogo University Hospital of Ouagadougou, Burkina Faso","volume":"16","author":"Ay\u00e9rou\u00e9","year":"2009","journal-title":"Arch Pediatr"},{"key":"ref9","first-page":"243","article-title":"Sickle cell anemia: delayed diagnosis in Bahia, Brazil--a largely Afro-descendant population","volume":"21","author":"Zanette","year":"2011","journal-title":"Ethn Dis"},{"key":"ref10","first-page":"221","article-title":"Age at diagnosis of sickle cell disease in a developing country","volume":"39","author":"Brown","year":"2010","journal-title":"Afr J Med Med Sci"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"S398","DOI":"10.1016\/j.amepre.2011.09.013","article-title":"Sickle cell disease in Africa: a neglected cause of early childhood mortality","volume":"41","author":"Grosse","year":"2011","journal-title":"Am J Prev Med"},{"key":"ref12","first-page":"155","article-title":"Vaccination status of children with sickle cell disease in Ouagadougou (Burkina Faso)","volume":"16","author":"Nacoulma","year":"2006","journal-title":"Sante"},{"key":"ref13","first-page":"467","article-title":"Sickle cell disease from Africa to Belgium, from neonatal screening to clinical management","volume":"70","author":"L\u00ea","year":"2010","journal-title":"Med Trop (Mars)"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1111\/j.1365-2141.2009.07771.x","article-title":"An observational study of children with sickle cell disease in Kilifi, Kenya","volume":"146","author":"Sadarangani","year":"2009","journal-title":"Br J Haematol"},{"key":"ref15","first-page":"365","article-title":"Sickle-cell crisis in the child and teenager in Brazzaville, Congo. A retrospective study of 587 cases","volume":"98","author":"Babela","year":"2005","journal-title":"Bull Soc Pathol Exot"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1182\/blood.V86.2.776.bloodjournal862776","article-title":"Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease","volume":"86","author":"Gill","year":"1995","journal-title":"Blood"},{"key":"ref17","first-page":"447","article-title":"Medicine and health in the Democratic Republic of Congo: from Independence to the Third Republic","volume":"67","author":"Wembonyama","year":"2007","journal-title":"Med Trop (Mars)"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1034\/j.1600-0609.2000.90210.x","article-title":"Acute clinical events in 299 homozygous sickle cell patients living in France. French Study Group on Sickle Cell Disease","volume":"65","author":"Neonato","year":"2000","journal-title":"Eur J Haematol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1007\/s12098-007-0129-4","article-title":"Blood transfusion rate in Congolese patients with sickle cell anemia","volume":"74","author":"Tshilolo","year":"2007","journal-title":"Indian J Pediatr"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1016\/0929-693X(96)87087-4","article-title":"Current aspects of sickle cell disease in children in Gabon","volume":"3","author":"Thuilliez","year":"1996","journal-title":"Arch Pediatr"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1111\/j.1600-0609.2005.00590.x","article-title":"Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events","volume":"76","author":"Tarer","year":"2006","journal-title":"Eur J Haematol"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"1364","DOI":"10.1016\/S0140-6736(09)61374-X","article-title":"Bacteraemia in Kenyan children with sickle-cell anaemia: a retrospective cohort and case-control study","volume":"374","author":"Williams","year":"2009","journal-title":"Lancet"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"225","DOI":"10.3109\/03630269.2014.917658","article-title":"Prevalence of sickle cell disease in a pediatric population suffering from severe infections: a Congolese experience","volume":"38","author":"Kondani","year":"2014","journal-title":"Hemoglobin"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1177\/004947550103100109","article-title":"Associated morbidities in children with sickle-cell anaemia presenting with severe anaemia in a malarious area","volume":"31","author":"Ambe","year":"2001","journal-title":"Trop Doct"},{"key":"ref25","first-page":"CD003489","article-title":"Malaria chemoprophylaxis in sickle cell disease","volume":"4","author":"Oniyangi","year":"2006","journal-title":"Cochrane Database Syst Rev"},{"key":"ref26","first-page":"55","article-title":"Infections in Senegalese children and adolescents with sickle cell anemia: epidemiological aspects","volume":"45","author":"Diagne","year":"2000","journal-title":"Dakar Med"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.cell.2011.03.049","article-title":"Sickle hemoglobin confers tolerance to Plasmodium infection","volume":"145","author":"Ferreira","year":"2011","journal-title":"Cell"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1111\/j.1365-2141.1985.tb07390.x","article-title":"Non-benign sickle cell anaemia in western Saudi Arabia","volume":"60","author":"Acquaye","year":"1985","journal-title":"Br J Haematol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1016\/0929-693X(96)85059-7","article-title":"Sickle cell anemia in the south of Zaire. Study of two series of 251 and 340 patients followed-up 1988-1992","volume":"3","author":"Tshilolo","year":"1996","journal-title":"Arch Pediatr"}],"container-title":["Hematology Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.pagepress.org\/journals\/index.php\/hr\/article\/viewFile\/6952\/6878","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.mdpi.com\/2038-8330\/9\/2\/6952\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,23]],"date-time":"2022-02-23T07:14:26Z","timestamp":1645600466000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2038-8330\/9\/2\/6952"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,1]]},"references-count":29,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2017,6]]}},"alternative-id":["hr.2017.6952"],"URL":"https:\/\/doi.org\/10.4081\/hr.2017.6952","relation":{},"ISSN":["2038-8330"],"issn-type":[{"value":"2038-8330","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,1]]}}}