Hydroxyurea is widely used in high-income countries for the management of sickle cell disease (SCD) in children. In Kenyan clinical guidelines, hydroxyurea is only recommended for adults with SCD. Yet many deaths from SCD occur in early childhood, deaths that might be prevented by an effective, disease modifying intervention. The aim of this review was to summarise the available evidence on the efficacy, effectiveness and safety of hydroxyurea in the management of SCD in children below 5 years of age to support guideline development in Kenya. We undertook a systematic review and used the Grading of Recommendations Assessment, Development and Evaluation system to appraise the quality of identified evidence. Overall, available evidence from 1 systematic review (n=26 studies), 2 randomised controlled trials (n=354 children), 14 observational studies and 2 National Institute of Health reports suggest that hydroxyurea may be associated with improved fetal haemoglobin levels, reduced rates of hospitalisation, reduced episodes of acute chest syndrome and decreased frequency of pain events in children with SCD. However, it is associated with adverse events (eg, neutropenia) when high to maximum tolerated doses are used. Evidence is lacking on whether hydroxyurea improves survival if given to young children. Majority of the included studies were of low quality and mainly from high-income countries. Overall, available limited evidence suggests that hydroxyurea may improve morbidity and haematological outcomes in SCD in children aged below 5 years and appears safe in settings able to provide consistent haematological monitoring.

Background: Cytomegalovirus (CMV) infection in susceptible patients is associated with serious morbidity and a high mortality. Transmission of cytomegalovirus infection through blood transfusion is markedly reduced by transfusion of CMV seronegative blood products, or by transfusion of leucodepleted blood products. Objective: To determine the prevalence CMV IgG and IgM antibodies among blood donors at the National Blood Transfusion Services (NBTS), Nairobi. Design: Cross-sectional descriptive study. Setting: Four hundred participants were recruited from blood donors at the NBTS and testing was done at the Kenyatta National Hospital (KNH) immunology laboratories and the NBTC. Main outcome measures: Social demographic data and the CMV serologic status for the participants was determined and documented as being positive or negative for immunoglobulin G (IgG) and immunoglobulin M (IgM). The age, gender, marital status, education level and geographical area of residence of the participants were documented. Corresponding results of HIV, hepatitis B antigen, hepatitis C antibody from the patients were obtained from the NBTS. Results: Majority of the blood donors recruited were male at 57.9%. Most blood donors were aged 16-20 years (42.5%) and only 17.2% were above 30 years of age. Unmarried blood donors, those with secondary school education and an income between Kshs 5,000 (US$ 67)and KShs 50,000 (US$ 667) monthly were the majority at 78.5%, 54.8% and 66.1% respectively. Sexually active blood donors constituted 60.5% of the donors recruited. Positivity for transfusion transmissible infections (TTI) tested was 1.3%, 0.3%, 2.3% and 1.0% for human immunodeficiency virus (HIV), syphilis, hepatitis B and hepatitis C respectively. Anti-CMV IgG and IgM positivity was 97.0%,( 95% CI 96.45-97.53%), and 3.6% (95% CI 1.7-5.2%), respectively. There was no statistical difference between different ages, marital status, salary, individual's sexuality in the prevalence of CMV antibodies. However females had a higher prevalence of CMV antibodies. Conclusion: There is a very high prevalence of cytomegalovirus antibodies among blood donors at the NBTS, with virtually all blood donors having been exposed to the virus. Since the CMV remains latent within leucocytes after infection inspite of the prescence of antibodies in seropositive individuals, leucoreduction of blood products is recommended before transfusion to seronegative susceptible patients. In Kenya, susceptible groups of patients include very low birthweight babies, patients with acquired immune deficiency syndrome (AIDS) due to human immunodeficiency virus infections (HIV) patients, patients on myelosuppressive cancer therapy and recipients of kidney transplants. Further studies are recomended to determine the prevalence of CMV antibodies in these patients in order to establish the magnitude of the demand for CMV safe blood.

Genetic testing and counseling have become integral to the timely control of heritable cancers, like the childhood eye cancer retinoblastoma. This study aimed to determine attitudes, knowledge and experiences related to retinoblastoma genetics, among survivors and parents of children with retinoblastoma in Kenya. This qualitative study used focus groups as the primary data collection method, coupled with a brief demographic questionnaire. Study settings were Kenyatta National Hospital and Presbyterian Church of East Africa Kikuyu Hospital. Thematic analysis was used to identify key themes. Thirty-one individuals participated in five focus groups. Two main concepts emerged: (1) the origins of retinoblastoma are unclear, and (2) retinoblastoma is associated with significant challenges. The lack of clarity surrounding the origins of retinoblastoma was linked to limited knowledge of retinoblastoma genetics, and limited genetic counseling delivery and uptake. The challenges associated with retinoblastoma were discussed in terms of the impact of the diagnosis on individuals and families, and unmet healthcare needs related to the diagnosis. Next steps will incorporate these findings to develop evidence-informed and accessible cancer genetic services in Kenya.

Objective: To determine clinical-pathologic characteristics, treatment modalities and treatment outcomes of children diagnosed with neuroblastoma. Design: Cross-sectional descriptive study based on secondary data from patient records. Setting: Records department of Kenyatta National Hospital (KNH), a tertiary teaching and referral hospital based in Nairobi. Subjects: Children aged 15 years and below, admitted with the diagnosis of neuroblastoma, between January 1997 and December 2005. Main outcome measures: Presenting clinical features, diagnostic modalities including laboratory and imaging data, treatment modalities, response to treatment and patient survival. Results: Twenty six patients were eligible for the study; 13 males and 13 females giving a M: F ratio of 1: 1. The age range was 5 days to 12 years, with a median age of five years. Abdominal swelling (53.8%), inability to walk due to bone pains, (50%), and cranial or periorbital swelling, (38.5%) were the commonest presenting features. Diagnosis of neuroblastoma was based on tissue biopsy in 50% (95% CI 40.6-79.8%) of the patients, and on fine needle aspiration cytology of mass or bone marrow in the rest. Bone marrow involvement was present in 16, (75%). Anaemia, was common with 72.7% patients having a haemoglobin (HB) <8g/dl at presentation. Immunohistochemistry and cytological grading were done in two, (8%), patients. Urinary vanilly l mandelic acid (VMA), screening was positive in 50% (95% CI 29.9%-70.1%). The most frequently involved organs were abdomen (88.9%), and skeleton, (84.6%). Majority of patients, (92.3%), presented with advanced stage IV disease. Three patients died before commencement of treatment. All treated patients (100%), received cytotoxic therapy. Only two patients (8.6%) had surgery as part of treatment while one, (4.3%) was treated with radiotherapy. The initial treatment regimen was similar for all the patients. Although most patients had a complete initial response to treatment, early relapse, treatment failure, death or loss to follow up of patients with progressive disease were common. Overall survival (OS) at one year and two years were 19.2% (95% CI 6.6-39.4%) and 7.7% (95% CI 0.9%-25 1%) respectively. Only one patient was alive, (also free of disease), five years after diagnosis. Conclusion: Although other clinical-pathologic findings of the patients were similar to those reported elsewhere, virtually all study patients presented with advanced stage IV disease, which would be associated with poor prognosis irrespective of quality of care. Priority must therefore be on ensuring early diagnosis and referral of patients with neuroblastoma before any other interventions can be expected to positively impact on outcome. The limited role of surgery and radiotherapy observed over the study period may be attributed to late presentation of the patients. Pathologic evaluation of patients was inadequate, to some extent due to unavailability of facilities, but extra S40