Pediatric Sickle Cell Disease in Sudan: Complications and Management
Background. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic manage...
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| Vydáno v: | Anemia Ročník 2022; s. 1 - 8 |
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Egypt
Hindawi
14.02.2022
John Wiley & Sons, Inc Wiley |
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| ISSN: | 2090-1267, 2090-1275 |
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| Abstract | Background. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management. Objectives. To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan. Method. A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P<0.05 was considered statistically significant. Results. Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3–6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis. Conclusion. The SCD treatment protocol in Gaafar Ibnauf Children’s Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient. |
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| AbstractList | Background. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management. Objectives. To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan. Method. A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P<0.05 was considered statistically significant. Results. Out of 207 pediatric patients, 53.1% were females (mean age of 7.5±3.1 years), with a 1.1:1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3-6months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis. Conclusion. The SCD treatment protocol in Gaafar Ibnauf Children's Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management.BACKGROUNDSickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management.To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan.OBJECTIVESTo assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan.A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P < 0.05 was considered statistically significant.METHODA descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P < 0.05 was considered statistically significant.Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3-6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis.RESULTSOut of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3-6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis.The SCD treatment protocol in Gaafar Ibnauf Children's Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient.CONCLUSIONThe SCD treatment protocol in Gaafar Ibnauf Children's Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient. Background. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management. Objectives. To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan. Method. A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P < 0.05 was considered statistically significant. Results. Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3–6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis. Conclusion. The SCD treatment protocol in Gaafar Ibnauf Children’s Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient. Background. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management. Objectives. To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan. Method. A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P<0.05 was considered statistically significant. Results. Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3–6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis. Conclusion. The SCD treatment protocol in Gaafar Ibnauf Children’s Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient. Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management. To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan. A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. < 0.05 was considered statistically significant. Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3-6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis. The SCD treatment protocol in Gaafar Ibnauf Children's Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient. |
| Audience | Academic |
| Author | Mirghani, Hind Abdalla, Safa Talha, Meysaa Osman, Bashier Abdoon, Iman |
| AuthorAffiliation | 3 Consultant Pediatrician and Hematologist, Gaafar Ibnauf Pediatric Tertiary Hospital, Khartoum, Sudan 1 Clinical Pharmacy Program, Faculty of Pharmacy, University of Khartoum, Khartoum, Sudan 2 Department of Pharmacology, Faculty of Pharmacy, University of Khartoum, Khartoum, Sudan |
| AuthorAffiliation_xml | – name: 2 Department of Pharmacology, Faculty of Pharmacy, University of Khartoum, Khartoum, Sudan – name: 3 Consultant Pediatrician and Hematologist, Gaafar Ibnauf Pediatric Tertiary Hospital, Khartoum, Sudan – name: 1 Clinical Pharmacy Program, Faculty of Pharmacy, University of Khartoum, Khartoum, Sudan |
| Author_xml | – sequence: 1 givenname: Meysaa surname: Talha fullname: Talha, Meysaa organization: Clinical Pharmacy ProgramFaculty of PharmacyUniversity of KhartoumKhartoumSudanuofk.edu – sequence: 2 givenname: Bashier orcidid: 0000-0002-4931-7098 surname: Osman fullname: Osman, Bashier organization: Department of PharmacologyFaculty of PharmacyUniversity of KhartoumKhartoumSudanuofk.edu – sequence: 3 givenname: Safa orcidid: 0000-0002-7043-220X surname: Abdalla fullname: Abdalla, Safa organization: Department of PharmacologyFaculty of PharmacyUniversity of KhartoumKhartoumSudanuofk.edu – sequence: 4 givenname: Hind surname: Mirghani fullname: Mirghani, Hind organization: Consultant Pediatrician and HematologistGaafar Ibnauf Pediatric Tertiary HospitalKhartoumSudan – sequence: 5 givenname: Iman orcidid: 0000-0002-7453-5724 surname: Abdoon fullname: Abdoon, Iman organization: Department of PharmacologyFaculty of PharmacyUniversity of KhartoumKhartoumSudanuofk.edu |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/35198244$$D View this record in MEDLINE/PubMed |
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| CitedBy_id | crossref_primary_10_1093_icvts_ivad033 crossref_primary_10_1016_S2352_3026_24_00007_3 crossref_primary_10_1080_17450128_2023_2253388 crossref_primary_10_7759_cureus_65263 |
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| Copyright | Copyright © 2022 Meysaa Talha et al. COPYRIGHT 2022 John Wiley & Sons, Inc. Copyright © 2022 Meysaa Talha et al. This work is licensed under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. Copyright © 2022 Meysaa Talha et al. 2022 |
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| References_xml | – volume: 64 start-page: 1114 year: 2014 ident: 11 article-title: Management of acute complications in sickle cell disease publication-title: Revue du Praticien – ident: 29 doi: 10.1155/2015/791498 – ident: 1 doi: 10.4081/hr.2010.e2 – ident: 21 doi: 10.1186/s13104-019-4682-5 – ident: 25 doi: 10.1002/14651858.cd003427.pub5 – volume: 59 start-page: 1 year: 2010 ident: 28 article-title: Prevention of pneumococcal disease among infants and children-use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine-recommendations of the Advisory Committee on Immunization Practices (ACIP) publication-title: MMWR. Recommendations and reports: Morbidity and Mortality Weekly Report. Recommendations and Reports – ident: 16 doi: 10.1182/bloodadvances.2020001851 – ident: 12 doi: 10.1016/j.tcm.2020.02.002 – ident: 2 doi: 10.1101/cshperspect.a011783 – ident: 8 doi: 10.3233/ch-189002 – ident: 26 doi: 10.1093/intqhc/mzh033 – ident: 18 doi: 10.7326/aitc202101190 – ident: 7 doi: 10.11604/pamj.2014.18.13.3280 – volume-title: Cureus ident: 10 article-title: Risk factors and complications among pediatric patients with sickle cell anemia: a single tertiary center retrospective study doi: 10.7759/cureus.12440 – ident: 13 doi: 10.1080/17474086.2018.1407240 – volume: 92 start-page: 1069 year: 2015 ident: 15 article-title: Management of sickle cell disease: recommendations from the 2014 expert panel report publication-title: American Family Physician – volume: 14 year: 2014 ident: 20 article-title: Clinical profile of sickle cell anaemia in Sudanese children publication-title: Al Neelain Medical Journal – volume-title: Sickle Cell Crisis year: 2021 ident: 5 – ident: 19 doi: 10.1016/s2352-3026(19)30111-5 – ident: 6 doi: 10.1111/tmi.12689 – ident: 23 doi: 10.1111/bjh.14592 – ident: 24 doi: 10.5001/omj.2012.25 – volume: 100 year: 2014 ident: 4 article-title: Sickle cell disease: a neglected chronic disease of increasing global health importance publication-title: Archives of Disease in Childhood – ident: 30 doi: 10.1056/nejmoa2000146 – ident: 27 doi: 10.1592/phco.20.1.110.34660 – ident: 17 doi: 10.1182/bloodadvances.2019001142 – ident: 9 doi: 10.1016/j.ijpam.2020.12.005 – ident: 22 doi: 10.1212/wnl.0000000000004537 – volume: 33 start-page: 238 year: 2008 ident: 14 article-title: Pharmacological management of sickle cell disease publication-title: P and T: A Peer-Reviewed Journal for Formulary Management – ident: 3 doi: 10.1016/j.amepre.2011.09.013 |
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| Title | Pediatric Sickle Cell Disease in Sudan: Complications and Management |
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