Browsing by Author "Segbefia, C.I."

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Adherence to hydroxyurea therapy and health-related quality of life in children with sickle cell anaemia at Korle Bu Teaching Hospital in Ghana
(Health Science Investigations Journal, 2023) Dwuma-Badu, D.; Segbefia, C.I.; Druye, A.A.; Dei-Adomakoh, Y.A.
Background: Sickle cell anaemia (SCA) causes numerous acute and chronic complications, which can result in significant morbidity and mortality. Hydroxyurea (HU) therapy in individuals with SCA is associated with a reduction in disease severity. Objective: The study aimed to determine the relationship between adherence to HU therapy and health-related quality of life (HRQOL) scores in children with SCA (HbSS) at Korle Bu Teaching Hospital (KBTH) and identify any barriers to HU adherence. Methods: A cross-sectional descriptive study was conducted at the paediatric sickle cell clinic of the KBTH. One hundred and fifteen children aged 2 – 12 years with HbSS receiving HU therapy and their primary caregivers were enrolled on this study. Demographic and HU-related data were obtained from medical records and interviews with caregivers. The 8-item Morisky Medication Adherence Scale (MMAS-8 © ) and haematologic response based on mean corpuscular volume (MCV) were used to assess HU adherence. Caregivers and children aged 8 - 12 years also completed the Paediatric Quality of Life Inventory (PedsQLTM) SCD-module version 3.0. For children aged 5 – 7 years, the PedsQLTM SCD-module version 3.0 was administered by interview. Results: Seventy-nine (68.7%) of children had high adherence to HU therapy using the MMAS-8 © . The mean PedsQLTM scores were 96.9 ± 6.0 and 96.3 ± 7.2 for the child (n = 91) and caregiver (n = 114), respectively. Children with high HU adherence had significantly higher PedsQLTM scores than those with low or moderate adherence. Neither child's current MCV nor mean change in MCV correlated with child or caregiver PedsQLTM scores. The main barrier to HU adherence identified by children aged  8 years was forgetfulness, while the cost of HU was the main barrier to adherence reported by caregivers. Conclusion: Children with SCA with high adherence to HU had the highest HRQOL scores using the PedsQLTM SCD-module version 3.0. Routine assessment of barriers to HU adherence can provide important information to help guide relevant interventions
The changing clinical pattern of endemic Burkitt lymphoma in Western Africa: Experience from a tertiary center in Ghana
(Pediatric Blood and Cancer, 2018-10) Offor, U.T.; Akyea, R.K.; Neequaye, J.E.; Renner, L.A.; Segbefia, C.I.
BACKGROUND: Burkitt lymphoma (BL) is the most common childhood cancer in Ghana, where the endemic variant is the predominant subtype and historically presents as a highly chemo-sensitive jaw tumor. This study aimed to update the current epidemiological characteristics of childhood BL in our institution. PROCEDURE: Patient data for all children diagnosed with BL and seen at Korle Bu Teaching Hospital between January 2007 and December 2012 were retrospectively analyzed. RESULTS: BL was diagnosed in 173 children (<13 years) during the study period, with the abdomen as the most common tumor site (46%) followed by the jaw (31%). Abdominal tumors were associated with advanced/disseminated disease (P = 0.002), and were more likely to occur in females irrespective of tumor stage (relative risk = 1.56 [95% CI; 1.1-12.3]). Twenty-five percent (43/173) of the study cohort died and mortality was influenced by increasing age (P = 0.02) and advanced disease (P = 0.03). Treatment delay was experienced by nine in ten patients primarily due to familial financial constraint (75%). Treatment abandonment was observed as a first event in 94% of patients and two thirds of children in the study were eventually lost to follow-up. CONCLUSION: The predominance of primary abdominal tumors in our study cohort may indicate a changing epidemiological pattern of BL in Ghana. High rates of treatment delay and abandonment were evident and are likely to be contributing factors to the poor childhood cancer survival outcomes seen in resource-limited countries in Africa.
Endomyocardial fibrosis associated with Schistosoma haematobium infection
(Ghana medical journal, 2014-12) Assimeng, J.; Segbefia, C.I.; Neequaye, J.
Endomyocardial fibrosis (EMF) is a form of restrictive cardiomyopathy common in the tropics and subtropics. The aetiology of EMF is unknown but helminth infestations such as schistosomiasis have been implicated. Two boys aged 8 and 10 years with EMF associated with Schistosoma haematobium, are described. The schistosomes in both cases may have been acquired from contact with contaminated water collected and stored in containers and subsequently used for bathing. Both patients were managed conservatively. Overall prognosis of EMF is poor and this report emphasizes the importance of public health interventions in the control of schistosomiasis.
Haemophagocytic lymphohistiocytosis in a Ghanaian child
(Ghana medical journal, 2012-12) Segbefia, C.I.; Osei-Wusu, A.; Obeng, W.; Dei-Adomakoh, Y.
We report a case of a previously well nine-month-old infant who presented with prolonged fever, hepatosplenomegaly and pancytopenia. A diagnosis of haemophagocytic lymphohistiocytosis (HLH) was made during the course of hospital admission. There was good initial response to dexamethasone but the patient died less than two months after diagnosis. This is the first report of HLH from Ghana. The disease has a significant mortality rate if untreated and a high index of suspicion is required in all severely ill children.
Implementing newborn screening for sickle cell disease in Korle Bu Teaching Hospital, Accra: Results and lessons learned
(Wiley, 2021) Segbefia, C.I.; Goka, B.; Welbeck, J.; Amegan-Aho, K.; Dwuma-Badu, D.; Rao, S.; Salifu, N.; Salifu, N.; Oppong, S.A.; Odei, E.; Ohene-Frempong, K.; Odame, I.
Early diagnosis of sickle cell disease (SCD) through newborn screening (NBS) is a cost-effective intervention, which reduces morbidity and mortality. In sub-Saharan Africa (SSA) where disease burden is greatest, there are no universal NBS programs and few institutions have the capacity to conduct NBS. We determined the feasibility and challenges of implementing NBS for SCD in Ghana's largest public hospital. The SCD NBS program at Korle Bu Teaching Hospital (KBTH) is a multiyear partnership between the hospital and the SickKids Center for Global Child Health, Toronto, being implemented in phases. The 13-month demonstration phase (June 2017–July 2018) and phase one (November 2018–December 2019) focused on staff training and the feasibility of universal screening of babies born in KBTH. During the demonstration phase, 115 public health nurses and midwives acquired competency in heel stick for dried blood spot sampling. Out of 9990 newborns, 4427 babies (44.3%) were screened, of which 79 (1.8%) were identified with presumptive SCD (P-SCD). Major challenges identified included inadequate nursing staff to perform screening, shortage of screening supplies, and delays in receiving screening results. Strategies to overcome some of the challenges were incorporated into phase one, resulting in increased screening coverage to 83.7%. Implementing NBS for SCD in KBTH presented challenges with implications on achieving and sustaining universal NBS in KBTH and other settings in SSA. Specific steps addressing these challenges comprehensively will help build on the modest initial gains, moving closer toward a sustainable national NBS program.
Multispectral imaging for MicroChip electrophoresis enables point-of-care newborn hemoglobin variant screening
(Heliyon, 2022) An, R.; Huang, Y.; Rocheleau, A.; Segbefia, C.I.; Mensah, E.; Dei-Adomakoh, Y.; et al.
Hemoglobin (Hb) disorders affect nearly 7% of the world’s population. Globally, around 400,000 babies are born annually with sickle cell disease (SCD), primarily in sub-Saharan Africa where morbidity and mortality rates are high. Screening, early diagnosis, and monitoring are not widely accessible due to technical challenges and cost. We hypothesized that multispectral imaging will allow sensitive hemoglobin variant identification in existing affordable paper-based Hb electrophoresis. To test this hypothesis, we developed the first integrated point-of-care multispectral Hb variant test: Gazelle-Multispectral. Here, we evaluated the accuracy of Gazelle-Multispectral for Hb variant newborn screening in 265 newborns with known hemoglobin variants including hemoglobin A (Hb A), hemoglobin F (Hb F), hemoglobin S (Hb S) and hemoglobin C (Hb C). Gazelle-Multispectral detected levels of Hb A, Hb F, Hb S, and Hb C/E/A2, demonstrated high correlations with the results reported by laboratory gold standard high performance liquid chromatography (HPLC) at Pearson Correlation Coefficient ¼ 0.97, 0.97, 0.93, and 0.95. Gazelle-Multispectral demonstrated accuracy of 96.8% in subjects of 0–3 days, and 96.9% in newborns. The ability to obtain accurate results on newborn samples suggest that Gazelle-Multispectral can be suitable for large-scale newborn screening and for diagnosis of SCD in low resource settings
Severity and Features of Epistaxis in Children with a Mucocutaneous Bleeding Disorder
(Journal of Pediatrics, 2018-02) Stokhuijzen, E.; Segbefia, C.I.; Biss, T.T.; Clark, D.S.; James, P.D.; Riddel, J.; Blanchette, V.S.; Rand, M.L.
Objective To use standardized bleeding questionnaires to compare the severity and patterns of epistaxis in children with a mucocutaneous bleeding disorder and control children. Study design The epistaxis sections of the Pediatric Bleeding Questionnaire (PBQ) administered to pediatric patients with von Willebrand disease or a platelet function disorder and healthy control children were reviewed. Scores and features of epistaxis (frequency, duration, onset, site, seasonal correlation, and need for medical/surgical intervention) were recorded. A PBQ epistaxis score ≥2 was defined as clinically significant. The Katsanis epistaxis scoring system was administered to eligible patients, ie, with ≥5 episodes of epistaxis per year. Results PBQ epistaxis scores were obtained for 66 patients, median age 12 years (range 0.6-18.3 years), and 56 control children. The median PBQ epistaxis score in patients was 2 vs 0 in control children (P <.0001). All of the features of epistaxis, except spontaneous onset, occurred in a significantly greater proportion of patients than control children with epistaxis. A total of 50% of the patients were graded as having severe epistaxis by the Katsanis epistaxis scoring system, and 30 of these (91%) had a clinically significant PBQ epistaxis score. Conclusion Standardized bleeding questionnaires are useful in the assessment of epistaxis severity and pattern and may help to distinguish children with and without a mucocutaneous bleeding disorder.
Sjögren's and plasma cell variant Castleman disease: a case report
(Ghana medical journal, 2018-04) Dei-Adomakoh, Y.A.; Quarcoopome, L.; Abrahams, A.D.; Segbefia, C.I.; Dey, D.I.
Castleman disease is a rare cause of lymphoid hyperplasia and may result in localized symptoms or an aggressive, multisystem disorder. It can mimic other diseases like lymphoma or tuberculosis. It classically presents as a mediastinal mass that involves the lymphatic tissue primarily but can also affect extra lymphatic sites including the lungs, larynx, parotid glands, pancreas, meninges, and muscles. In HIV and HHV8-negative patients with idiopathic multi-centric Castleman disease, pathogenesis may involve autoimmune mechanisms. We highlight and report a case of a 34-year-old Ghanaian female who was successfully diagnosed and managed for Sjögren's as well as plasma cell variant Castleman disease with combination chemotherapy and rituximab followed by eighteen months maintenance therapy with pulse chlorambucil and prednisolone and three monthly rituximab.