Sjögren's and plasma cell variant Castleman disease: a case report

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 multicentric 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.Keywords: lymphadenopathy, chemotherapy, Rituximab, Plasma Cell Variant Castleman Disease, Sjögren's syndrom

Adherence to hydroxyurea therapy and health-related quality of life in children with sickle cell anaemia at Korle Bu Teaching Hospital in Ghana

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

Nutritional status at diagnosis of childhood cancer in Korle Bu Teaching Hospital, Accra, Ghana

Background: Although most childhood cancers are curable, comorbid malnutrition can result in delayed initiation of treatment, increased treatment toxicity, and reduced overall survival. At cancer diagnosis, appropriate classification of nutritional status enhances nutritional surveillance to improve supportive care and cancer treatment outcomes.Objective: The study objective was to assess the prevalence of malnutrition at the diagnosis of childhood cancer, compare weight-based measurements with arm anthropometry in the assessment of acute malnutrition (wasting) and determine the association between malnutrition and selected cancer characteristics.Methods: The study was conducted at the Paediatric Oncology Unit (POU) at the Korle-Bu Teaching Hospital. Using consecutive recruitment, 133 participants of age ≤ 12 years with a new diagnosis of cancer were enrolled from January to December 2019. Stunting was assessed using a height-for-age z-score (HAZ). Wasting was assessed using a weight-for-height z-score (WHZ), body mass index-for-age z-score (BAZ), mid-upper arm circumference (MUAC), and upper arm muscle area (UAMA) percentile. Pearson's Chi-square and Fisher's exact tests were used to determine the association between nutritional status, cancer type and risk group.Results: The median age of participants was 4.5 years, and 64.7% ( n = 86/133) were male. Of the 133 participants, 60.9% ( n = 81) were diagnosed with solid tumours, 23.3% (n = 31) with leukaemia, and 15.8% (n = 21) with lymphoma. At cancer diagnosis, the prevalence of stunting was 16.8% (n = 22/131) while the prevalence of wasting was 21.8% (n = 29/133 ) and 40.5% (n = 53/131 ) using weight-based measurements (WHZ or BAZ) and arm anthropometry (MUAC or UAMA), respectively. No participant was obese. Lymphomas were significantly associated with wasting (p = 0.022). Participants with high-risk cancers were more likely to be stunted and wasted.Conclusion: Mid-upper arm circumference and UAMA detected more children with wasting than WHZ and BAZ at cancer diagnosis. Advanced-stage disease and lymphoma were associated with wasting. Establishing a nutritional rehabilitation programme at the POU, KBTH would ensure early and appropriate nutritional interventions to correct or prevent further nutritional deficits

The changing clinical pattern of endemic Burkitt lymphoma in Western Africa: Experience from a tertiary center in Ghana

Abstract 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

Neonatal Jaundice: awareness, perception and preventive practices in expectant mothers

Background: Neonatal jaundice (NNJ) is a preventable cause of neonatal morbidity and mortality. Improving mothers’ knowledge will help with early recognition of NNJ, prompt and appropriate intervention. This study highlights the knowledge, attitude and practice regarding neonatal jaundice among expectant mothers attending the antenatal clinics of Korle-Bu Teaching Hospital and Mamprobi Polyclinic in Accra.Methods: This was a cross-sectional study involving 175 expectant mothers. Interviewer based questionnaire was used to obtain data on knowledge, attitude and practice concerning NNJ. The study was conducted between 1st and 17th November 2013 at two antenatal clinics in Accra.Results: Out of the 175 respondents, 135 (77.1%) had heard about NNJ but only 37 (27.4%) of them heard it from the hospital. Among those who had heard about NNJ, 98 (72.6%) knew at least one symptom of NNJ; 125 (92.6%) did not know the causes of jaundice or had the wrong information and there was no significant association with theirlevel of education (X2 =6.757, p=0.15). Only 7(5.2%) knew one or more correct forms of treatment of NNJ; 67(49.6%) knew one or more danger signs and 86(63.5%) knew one or more complications.Conclusion: Majority of expectant mothers attending antenatal clinics at a Teaching Hospital and a Polyclinic in Accra, Ghana are aware of NNJ but have poor knowledge about the causes, danger signs and treatment of NNJ, irrespective of their level of education or their parity.Keywords: Jaundice, new born, mothers, knowledge, AccraFunding: None declare

Short-term chemotherapy-related complications and undernutrition in children diagnosed with cancer at Korle Bu Teaching Hospital, Accra, Ghana: A prospective cohort study.

Undernutrition in children with cancer is associated with complications during cancer therapy. The study objective was to determine the association between specific anthropometric parameters and short-term chemotherapy-related complications and mortality. This was a hospital-based, prospective cohort study of children, age ≤12 years, with a new cancer diagnosis at the Paediatric Oncology Unit, Korle Bu Teaching Hospital, Ghana. Socio-demographic information, cancer characteristics and anthropometric measurements were obtained at enrolment. Participants were followed up for twelve weeks from commencement of chemotherapy and selected treatment-related complications such as anaemia and thrombocytopenia requiring transfusions, prolonged neutropenia resulting in treatment delays, febrile neutropenia, mucositis and death were recorded. A total of 133 participants were recruited with a median age of 4.5 years. Eighty-one (60.9%) were diagnosed with solid tumours, 31 (23.3%) had leukaemias and 21 (15.8%) had lymphomas. Of the anthropometric parameters assessed, only arm anthropometry using upper arm muscle area (UAMA) and mid-upper arm circumference (MUAC) were associated with complications. Participants with wasting were more likely to develop anaemia and mucositis. However, the incidence of prolonged neutropenia was significantly higher among participants with average UAMA (p = 0.043) and low average UAMA (p = 0.049) compared to those with low UAMA. Risk of neutropenia was also significantly less among those with wasting by MUAC compared to those well-nourished (p = 0.045). Twenty-three participants (17.3%) died with a greater proportion (11/44; 25%) occurring in those who were wasted using MUAC. These findings underscore the need for nutritional surveillance at diagnosis and during chemotherapy, particularly where co-morbid disease is prevalent

Severity and Features of Epistaxis in Children with a Mucocutaneous Bleeding Disorder

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 disorde

Prospective identification of variables as outcomes for treatment (PIVOT): study protocol for a randomised, placebo-controlled trial of hydroxyurea for Ghanaian children and adults with haemoglobin SC disease

Abstract Background Haemoglobin SC (HbSC) is a common form of sickle cell disease (SCD), especially among individuals of West African ancestry. Persons with HbSC disease suffer from the same clinical complications and reduced quality of life that affect those with sickle cell anaemia (HbSS/Sβ0). Retrospective anecdotal data suggest short-term safety and benefits of hydroxyurea for treating HbSC, yet rigorous prospective data are lacking regarding optimal dosing, clinical and laboratory effects, long-term safety and benefits, and appropriate endpoints to monitor. Prospective Investigation of Variables as Outcomes for Treatment (PIVOT) was designed with three aims: (1) to measure the toxicities of hydroxyurea treatment on laboratory parameters, (2) to assess the effects of hydroxyurea treatment on sickle-related clinical and laboratory parameters, and (3) to identify study endpoints suitable for a future definitive phase III trial of hydroxyurea treatment of HbSC disease. Methods PIVOT is a randomised, placebo-controlled, double blind clinical trial of hydroxyurea. Approximately 120 children and 120 adults ages 5–50 years with HbSC disease will be enrolled, screened for 2 months, and then randomised 1:1 to once-daily oral hydroxyurea or placebo. Study treatment will be prescribed initially at 20 ± 5 mg/kg/day with an opportunity to escalate the dose twice over the first 6 months. After 12 months of blinded study treatment, all participants will be offered open-label hydroxyurea for up to 4 years. Safety outcomes include treatment-related cytopenias, whole blood viscosity, and adverse events. Efficacy outcomes include a variety of laboratory and clinical parameters over the first 12 months of randomised treatment, including changes in haemoglobin and fetal haemoglobin, intracranial arterial velocities measured by transcranial Doppler ultrasound, cerebral oxygenation using near infrared spectrometry, spleen volume and kidney size by ultrasound, proteinuria, and retinal imaging. Exploratory outcomes include functional erythrocyte analyses with ektacytometry for red blood cell deformability and point-of-sickling, patient-reported outcomes using the PROMIS questionnaire, and 6-min walk test. Discussion For children and adults with HbSC disease, PIVOT will determine the safety of hydroxyurea and identify measurable changes in laboratory and clinical parameters, suitable for future prospective testing in a definitive multi-centre phase III clinical trial. Trial registration PACTR, PACTR202108893981080. Registered 24 August 2021, https://pactr.samrc.ac.z

Additional file 1 of Evaluation of treatment patterns, healthcare resource utilization and cost of illness for sickle cell disease in Ghana: a private medical insurance claims database study

Additional file 1: Table S1. Type of Sickle Cell Disease (January 2015-March 2021). Table S2. Hospitalization in All the Patients Having SCD Diagnosis and Inpatient Visit (12-Month Follow-up period). Table S3. Healthcare Resource Utilization and Costs by Visit Type (6-Month Baseline Period). Table S4. Healthcare Resource Utilization and Costs by Activity Type (12-Month Follow-up). Table S5. Specialities Visited by Patients with SCD (January 2015-March 2021). Table S6. Diagnostic Investigations Conducted on Patients With SCD (January 2015-March 2021