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The management of esophageal atresia in a resource-limited environment is plagued with challenges that often lead to poor outcome. The diagnosis and management of babies with long-gap esophageal atresia adds a new dimension to these challenges. We report the success of esophageal replacement surgery for a baby with long-gap esophageal atresia in a resource-limited setting. One year after surgery, she was eating well and her weight gain was appropriate.

Burkitt lymphoma (BL) is responsible for many childhood cancers in sub-Saharan Africa, where it is linked to recurrent or chronic infection by Epstein-Barr virus or Plasmodium falciparum. However, whether human leukocyte antigen (HLA) polymorphisms, which regulate immune response, are associated with BL has not been well investigated, which limits our understanding of BL etiology. Here we investigate this association among 4,645 children aged 0-15 years, 800 with BL, enrolled in Uganda, Tanzania, Kenya, and Malawi. HLA alleles are imputed with accuracy >90% for HLA class I and 85-89% for class II alleles. BL risk is elevated with HLA-DQA1*04:01 (adjusted odds ratio [OR] = 1.61, 95% confidence interval [CI] = 1.32-1.97, P = 3.71 × 10 -6 ), with rs2040406(G) in HLA-DQA1 region (OR = 1.43, 95% CI = 1.26-1.63, P = 4.62 × 10 -8 ), and with amino acid Gln at position 53 versus other variants in HLA-DQA1 (OR = 1.36, P = 2.06 × 10 -6 ). The associations with HLA-DQA1*04:01 (OR = 1.29, P = 0.03) and rs2040406(G) (OR = 1.68, P = 0.019) persist in mutually adjusted models. The higher risk rs2040406(G) variant for BL is associated with decreased HLA-DQB1 expression in eQTLs in EBV transformed lymphocytes. Our results support the role of HLA variation in the etiology of BL and suggest that a promising area of research might be understanding the link between HLA variation and EBV control., (© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Burkitt lymphoma (BL) is an aggressive B-cell lymphoma that significantly contributes to childhood cancer burden in sub-Saharan Africa. Plasmodium falciparum, which causes malaria, is geographically associated with BL, but the evidence remains insufficient for causal inference. Inference could be strengthened by demonstrating that mendelian genes known to protect against malaria-such as the sickle cell trait variant, HBB-rs334(T)-also protect against BL. We investigated this hypothesis among 800 BL cases and 3845 controls in four East African countries using genome-scan data to detect polymorphisms in 22 genes known to affect malaria risk. We fit generalized linear mixed models to estimate odds ratios (OR) and 95% confidence intervals (95% CI), controlling for age, sex, country, and ancestry. The ORs of the loci with BL and P. falciparum infection among controls were correlated (Spearman's ρ = 0.37, p = .039). HBB-rs334(T) was associated with lower P. falciparum infection risk among controls (OR = 0.752, 95% CI 0.628-0.9; p = .00189) and BL risk (OR = 0.687, 95% CI 0.533-0.885; p = .0037). ABO-rs8176703(T) was associated with decreased risk of BL (OR = 0.591, 95% CI 0.379-0.992; p = .00271), but not of P. falciparum infection. Our results increase support for the etiological correlation between P. falciparum and BL risk., (© 2023 Wiley Periodicals LLC. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.)

In high-income countries, mosaic chromosomal alterations in peripheral blood leukocytes are associated with an elevated risk of adverse health outcomes, including hematologic malignancies. We investigate mosaic chromosomal alterations in sub-Saharan Africa among 931 children with Burkitt lymphoma, an aggressive lymphoma commonly characterized by immunoglobulin-MYC chromosomal rearrangements, 3822 Burkitt lymphoma-free children, and 674 cancer-free men from Ghana. We find autosomal and X chromosome mosaic chromosomal alterations in 3.4% and 1.7% of Burkitt lymphoma-free children, and 8.4% and 3.7% of children with Burkitt lymphoma (P-values = 5.7×10 -11 and 3.74×10 -2 , respectively). Autosomal mosaic chromosomal alterations are detected in 14.0% of Ghanaian men and increase with age. Mosaic chromosomal alterations in Burkitt lymphoma cases include gains on chromosomes 1q and 8, the latter spanning MYC, while mosaic chromosomal alterations in Burkitt lymphoma-free children include copy-neutral loss of heterozygosity on chromosomes 10, 14, and 16. Our results highlight mosaic chromosomal alterations in sub-Saharan African populations as a promising area of research., (© 2023. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Interferon lambda 4 (IFN-λ4) is a novel type-III interferon that can be expressed only by carriers of the genetic variant rs368234815-dG within the first exon of the IFNL4 gene. Genetic inability to produce IFN-λ4 (in carriers of the rs368234815-TT/TT genotype) has been associated with improved clearance of hepatitis C virus (HCV) infection. The IFN-λ4-expressing rs368234815-dG allele ( IFNL4 -dG) is most common (up to 78%) in West sub-Saharan Africa (SSA), compared to 35% of Europeans and 5% of individuals from East Asia. The negative selection of IFNL4 -dG outside Africa suggests that its retention in African populations could provide survival benefits, most likely in children. To explore this hypothesis, we conducted a comprehensive association analysis between IFNL4 genotypes and the risk of childhood Burkitt lymphoma (BL), a lethal infection-associated cancer most common in SSA. We used genetic, epidemiologic, and clinical data for 4,038 children from the Epidemiology of Burkitt Lymphoma in East African Children and Minors (EMBLEM) and the Malawi Infections and Childhood Cancer case-control studies. Generalized linear mixed models fit with the logit link controlling for age, sex, country, P. falciparum infection status, population stratification, and relatedness found no significant association between BL risk and 3 coding genetic variants within IFNL4 (rs368234815, rs117648444, and rs142981501) and their combinations. Because BL occurs in children 6-9 years of age who survived early childhood infections, our results suggest that additional studies should explore the associations of IFNL4 -dG allele in younger children. This comprehensive study represents an important baseline in defining the health effects of IFN-λ4 in African populations.

Endemic Burkitt lymphoma (eBL) is a pediatric cancer coendemic with malaria in sub-Saharan Africa, suggesting an etiological link between them. However, previous cross-sectional studies of limited geographic areas have not found a convincing association. We used spatially detailed data from the Epidemiology of Burkitt Lymphoma in East African Children and Minors (EMBLEM) study to assess this relationship. EMBLEM is a case-control study of eBL from 2010 through 2016 in six regions of Kenya, Uganda, and Tanzania. To measure the intensity of exposure to the malaria parasite, Plasmodium falciparum , among children in these regions, we used high-resolution spatial data from the Malaria Atlas Project to estimate the annual number of P. falciparum infections from 2000 through 2016 for each of 49 districts within the study region. Cumulative P. falciparum exposure, calculated as the sum of annual infections by birth cohort, varied widely, with a median of 47 estimated infections per child by age 10, ranging from 4 to 315 infections. eBL incidence increased 39% for each 100 additional lifetime P. falciparum infections (95% CI: 6.10 to 81.04%) with the risk peaking among children aged 5 to 11 and declining thereafter. Alternative models using estimated annual P. falciparum infections 0 to 10 y before eBL onset were inconclusive, suggesting that eBL risk is a function of cumulative rather than recent cross-sectional exposure. Our findings provide population-level evidence that eBL is a phenotype related to heavy lifetime exposure to P. falciparum malaria and support emphasizing the link between malaria and eBL.

Epstein-Barr virus (EBV) is associated with endemic Burkitt lymphoma (eBL), but the contribution of EBV variants is ill-defined. Studies of EBV whole genome sequences (WGS) have identified phylogroups that appear to be distinct for Asian versus non-Asian EBV, but samples from BL or Africa, where EBV was first discovered, are under-represented. We conducted a phylogenetic analysis of EBV WGS and LMP-1 sequences obtained primarily from BL patients in Africa and representative non-African EBV from other conditions or regions using data from GenBank, Sequence Read Archive, or Genomic Data Commons for the Burkitt Lymphoma Genome Sequencing Project (BLGSP) to generate data to support the use of a simpler biomarker of geographic or phenotypic associations. We also investigated LMP-1 patterns in 414 eBL cases and 414 geographically matched controls in the Epidemiology of Burkitt Lymphoma in East African children and minors (EMBLEM) study using LMP-1 PCR and Sanger sequencing. Phylogenetic analysis revealed distinct genetic patterns of African versus Asian EBV sequences. We identified 281 single nucleotide variations (SNVs) in LMP-1 promoter and coding region, which formed 12 unique patterns (A to L). Nine patterns (A, AB, C, D, F, I, J, K and L) predominated in African EBV, of which four were found in 92% of BL samples (A, AB, D, and H). Predominant patterns were B and G in Asia and H in Europe. EBV positivity in peripheral blood was detected in 95.6% of EMBLEM eBL cases versus 79.2% of the healthy controls (odds ratio [OR] =3.83; 95% confidence interval 2.06-7.14). LMP-1 was successfully sequenced in 66.7% of the EBV DNA positive cases but in 29.6% of the controls (ORs ranging 5-11 for different patterns). Four LMP-1 patterns (A, AB, D, and K) were detected in 63.1% of the cases versus 27.1% controls (ORs ranges: 5.58-11.4). Dual strain EBV infections were identified in WGS and PCR-Sanger data. In conclusion, EBV from Africa is phylogenetically separate from EBV in Asia. Genetic diversity in LMP-1 formed 12 patterns, which showed promising geographic and phenotypic associations. Presence of multiple strain infection should be considered in efforts to refine or improve EBV markers of ancestry or phenotype., Lay Summary: Epstein-Barr virus (EBV) infection, a ubiquitous infection, contributes to the etiology of both Burkitt Lymphoma (BL) and nasopharyngeal carcinoma, yet their global distributions vary geographically with no overlap. Genomic variation in EBV is suspected to play a role in the geographical patterns of these EBV-associated cancers, but relatively few EBV samples from BL have been comprehensively studied. We sought to compare phylogenetic patterns of EBV genomes obtained from BL samples in Africa and from tumor and non-tumor samples from elsewhere. We concluded that EBV obtained from BL in Africa is genetically separate from EBV in Asia. Through comprehensive analysis of nucleotide variations in EBV's LMP-1 gene, we describe 12 LMP-1 patterns, two of which (B and G) were found mostly in Asia. Four LMP-1 patterns (A, AB, D, and F) accounted for 92% of EBVs sequenced from BL in Africa. Our results identified extensive diversity of EBV, but BL in Africa was associated with a limited number of variants identified, which were different from those identified in Asia. Further research is needed to optimize the use of PCR and sequencing to study LMP-1 diversity for classification of EBV variants and for use in epidemiologic studies to characterize geographic and/or phenotypic associations of EBV variants with EBV-associated malignancies, including eBL., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Liao, Liu, Chin, Li, Hung, Tsai, Otim, Legason, Ogwang, Reynolds, Kerchan, Tenge, Were, Kuremu, Wekesa, Masalu, Kawira, Ayers, Pfeiffer, Bhatia, Goedert, Lo and Mbulaiteye.)

Background: Falciparum and endemic Burkitt lymphoma (eBL) are co-endemic in Africa, but the malaria experience in eBL patients is unknown. A lower prevalence of falciparum has been reported in eBL patients, but those results are anecdotally attributed to pre-enrollment anti-malaria treatment., Methods: We studied 677 eBL patients and 2920 community controls aged 0-15 years enrolled in six regions in Uganda, Tanzania, and Kenya during 2010-2016. Falciparum was diagnosed using thick blood film microscopy (TFM) and antigen-capture rapid diagnostic tests (RDTs). Guardians of the children answered a 40-item structured questionnaire about their child's pre-enrollment lifetime malaria history and treatment, demographics, socioeconomics, animal exposures, fevers, and hospitalizations. We utilized exploratory factor analysis to reduce the 40 questionnaire variables into six factors, including Inpatient malaria and Outpatient malaria factors that were surrogates of pre-enrollment anti-malaria treatment. The six factors accounted for 83-90% of the variance in the questionnaire data. We calculated odds ratios and 95% confidence intervals (OR 95% CI) of association of eBL with falciparum positivity, defined as positive both on TFM or RDTs, or only RDTs (indicative of recent infection) or TFM (indicative of current falciparum infection) versus no infection, using multivariable logistic regression, controlling for group of age (0-2, 3-5, 6-8, 9-11 and 12-15 years), sex, and study site and the afore-mentioned pre-enrollment factors., Results: The prevalence of falciparum infection was 25.6% in the eBL cases and 45.7% in community controls (aOR = 0.43, 95% CI: 0.40, 0.47; P < 0.0001). The results were similar for recent falciparum infection (6.9% versus 13.5%, aOR = 0.44, 95% CI: 0.38, 0.50; P < 0.0001) and current falciparum infection (18.7% versus 32.1%, aOR = 0.47, 95% CI: 0.43, 0.51; P < 0.0001). These aORs for any, recent and current falciparum infection did not change when we adjusted for pre-enrollment factors (aORs = 0.46, =0.44, and = 0.51, respectively) were significantly lower in stratified analysis for any infection in children < 5 years (aOR = 0.46; 95% CI: 0.29, 0.75) or ≥ 10 years (aOR = 0.47; 95% CI: 0.32, 0.71)., Conclusion: Our study results reduce support for pre-enrollment antimalaria treatment as a sole explanation for the observed lower falciparum prevalence in eBL cases and open a space to consider alternative immunology-based hypotheses.

Platelet counts are decreased in Plasmodium falciparum malaria, which is aetiologically linked with endemic Burkitt lymphoma (eBL). However, the pattern of platelet counts in eBL cases is unknown. We studied platelet counts in 582 eBL cases and 2 248 controls enrolled in a case-control study in Uganda, Tanzania and Kenya (2010-2016). Mean platelet counts in controls or eBL cases with or without malaria-infection in controls versus eBLcases were compared using Student's t-test. Odds ratios (ORs) and two-sided 95% confidence intervals (95% CIs) were estimated using multiple logistic regression, controlling for age, sex, haemoglobin and white blood cell counts. Platelets were decreased with malaria infection in the controls [263 vs. 339 × 10 9 platelets/l, P < 0·0001; adjusted OR (aOR) = 3·42, 95% CI: 2·79-4·18] and eBL cases (314 vs. 367 × 10 9 platelets/l, P-value = 0·002; aOR = 2·36, 95% CI: 1·49-3·73). Unexpectedly, platelets were elevated in eBL cases versus  controls in overall analyses (mean: 353 vs. 307 × 10 9 platelets/l, P < 0·0001; aOR = 1·41; 95% CI: 1·12-1·77), and when restricted to malaria-positive (mean 314 vs. 263 × 10 9 platelets/l, P < 0·0001; OR = 2·26; 95% CI: 1·56-3·27) or malaria-negative (mean 367 vs. 339 × 10 9 platelets/l, P < 0·001; OR = 1·46; 95% CI: 1·17-1·83) subjects. Platelets were decreased with malaria infection in controls and eBL cases but elevated with eBL., (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)

Background: Endemic Burkitt lymphoma (eBL) is an aggressive B cell non-Hodgkin lymphoma associated with antigenic stimulation from Plasmodium falciparum malaria. Whether eBL risk is related to malaria parasite density is unknown. To address this issue, children with eBL, asymptomatic and clinical malaria, as a surrogate of malaria parasite density, were assessed., Methods: Malaria-related laboratory results (parasite density, haemoglobin, platelet count, and white cell count [WBC]) count) were compiled for 4019 eBL cases and 80,532 subjects evaluated for asymptomatic malaria or clinical malaria (severe malaria anaemia, hyperparasitaemia, cerebral malaria, malaria prostration, moderate malaria, and mild malaria) in 21 representative studies published in Africa (mostly East Africa) and 850 eBL cases and 2878 controls with primary data from the Epidemiology of Burkitt Lymphoma in East African Children and Minors (EMBLEM) case-control study in Uganda, Tanzania, and Kenya. The average values of malaria-related laboratory results were computed by condition and trends across single-year age groups were assessed using regression and spline models., Results: Overall, malaria infection or malaria was diagnosed in 37,089 of children compiled from the literature. Children with eBL and asymptomatic parasitaemia/antigenaemia, but not those with clinical malaria, were closest in their mean age (age 7.1-7.2 vs. 7.4-9.8 years), haemoglobin level (10.0-10.4 vs. 11.7-12.3 g/dL), malaria parasite density (2800 vs. 1827-7780 parasites/µL), platelet count (347,000-353,000 vs. 244,000-306,000 platelets/µL), and WBC count (8180-8890 vs. 7100-7410 cells/µL). Parasite density in these two groups peaked between four to five years, then decreased steadily thereafter; conversely, haemoglobin showed a corresponding increase with age. Children with clinical malaria were markedly different: all had an average age below 5 years, had dramatically elevated parasite density (13,905-869,000 parasites/µL) and dramatically decreased platelet count (< 159,000 platelets/µL) and haemoglobin (< 7 g/dL)., Conclusions: eBL and asymptomatic parasitaemia/antigenaemia, but not clinical malaria, were the most similar conditions with respect to mean age and malaria-related laboratory results. These results suggest that children with asymptomatic parasitaemia/antigenaemia may be the population at risk of eBL.

Endemic Burkitt lymphoma (eBL) is the most common childhood cancer in sub-Saharan African countries, however, few epidemiologic studies have been undertaken and none attempted enrolling cases from multiple countries. We therefore conducted a population-based case-control study of eBL in children aged 0-15 years old in six regions in Northern Uganda, Northern Tanzania and Western Kenya, enrolling 862 suspected cases and 2,934 population controls (response rates 98.5-100%), and processing ~40,000 vials of samples using standardized protocols. Risk factor questionnaires were administered, and malaria period prevalence was measured using rapid diagnostic tests (RDTs). A total of 80.9% of the recruited cases were diagnosed as eBL; 61.4% confirmed by histology. Associations with eBL risk were computed using logistic regression models adjusted for relevant confounders. Associations common in at least two countries were emphasized. eBL risk was decreased with higher maternal income and paternal education and elevated with history of inpatient malaria treatment >12 months before enrollment. Reporting malaria-attributed fever up to 6 months before enrollment and malaria-RDT positivity at enrollment were associated with decreased eBL risk. Conversely, reporting exposure to mass malaria suppression programs (e.g., indoor residual insecticide) was associated with elevated risk. HIV seropositivity was associated with elevated eBL risk, but the relative impact was small. The study shows that it is feasible to conduct networked, multisite population-based studies of eBL in Africa. eBL was inversely associated with socioeconomic status, positively associated with inpatient malaria treatment 12 months ago and with living in areas targeted for malaria suppression, which support a role of malaria in eBL., (© 2019 UICC.)

The burden of Plasmodium falciparum ( Pf ) malaria in Kenya is decreasing; however, it is still one of the top 10 causes of morbidity, particularly in regions of western Kenya. Between April 2015 and June 2016, we enrolled 965 apparently healthy children aged 0-15 years in former Nyanza and Western Provinces in Kenya to characterize the demographic, geographic, and household risk factors of asymptomatic malaria as part of an epidemiologic study to investigate the risk factors for endemic Burkitt lymphoma. The children were sampled using a stratified, multistage cluster sampling survey design. Malaria was assessed by rapid diagnostic test (RDT) and thick-film microscopy (TFM). Primary analyses of Pf malaria prevalence (pfPR) are based on RDT. Associations between weighted pfPR and potential risk factors were evaluated using logistic regression, accounting for the survey design. Plasmodium falciparum malaria prevalence was 36.0% (27.5%, 44.5%) by RDT and 22.3% (16.0%, 28.6%) by TFM. Plasmodium falciparum malaria prevalence was positively associated with living in the lake-endemic area (adjusted odds ratio [aOR] 3.46; 95% confidence interval [95% CI] 1.63, 7.37), paternal occupation as peasant farmer (aOR 1.87; 1.08, 3.26) or manual laborer (aOR 1.83; 1.00, 3.37), and keeping dogs (aOR 1.62; 0.98-2.69) or cows (aOR 1.52; 0.96-2.40) inside or near the household. Plasmodium falciparum malaria prevalence was inversely associated with indoor residual insecticide spraying (IRS) (aOR 0.44; 0.19, 1.01), having a household connected to electricity (aOR 0.47; 0.22, 0.98), and a household with two (aOR 0.45; 0.22, 0.93) or ≥ three rooms (aOR 0.41; 0.18, 0.93). We report high but geographically heterogeneous pfPR in children in western Kenya and significant associations with IRS and household-level socioeconomic factors.

Purpose: Wilms tumor is the commonest renal malignancy in childhood. Survival in high-income countries is approximately 90%, whereas in low-income countries, it is less than 50%. This study assessed treatment outcomes of patients with Wilms tumor at a Kenyan academic hospital., Patients and Methods: We conducted a retrospective medical record review of all children diagnosed with Wilms tumor between 2010 and 2012. Data on treatment outcomes and various sociodemographic and clinical characteristics were collected., Results: Of the 39 patients with Wilms tumor, 41% had event-free survival, 31% abandoned treatment, 23% died, and 5% had progressive or relapsed disease. Most patients presented at an advanced stage: stage I (0%), II (7%), III (43%), IV (40%), or V (10%). The most likely treatment outcome in patients with low-stage (I to III) disease was event-free survival (67%), whereas in those with high-stage (IV to V) disease, it was death (40%). No deaths or instances of progressive or relapsed disease were recorded among patients with low-stage disease; their only reason for treatment failure was abandonment of treatment. Stage of disease significantly affected treatment outcomes ( P = .014) and event-free survival estimates ( P < .001). Age at diagnosis, sex, duration of symptoms, distance to hospital, and health insurance status did not statistically significantly influence treatment outcomes or event-free survival estimates., Conclusion: Survival of patients with Wilms tumor in Kenya is lower compared with that in high-income countries. Treatment abandonment is the most common cause of treatment failure. Stage of disease at diagnosis statistically significantly affects treatment outcomes and survival., Competing Interests: The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated. Relationships are self-held unless noted. I = Immediate Family Member, Inst = My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO’s conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/jco/site/ifc. Festus NjugunaNo relationship to discloseHugo A. MartijnNo relationship to discloseRobert Tenge KuremuNo relationship to disclosePeter SaulaNo relationship to disclosePatel KirtikaNo relationship to discloseGilbert OlbaraNo relationship to discloseSandra LangatNo relationship to discloseSteve MartinNo relationship to discloseJodi SkilesNo relationship to discloseTerry VikNo relationship to discloseGertjan J.L. KaspersConsulting or Advisory Role: Jazz Pharmaceuticals, Boehringer Ingelheim Travel, Accommodations, Expenses: Jazz PharmaceuticalsSaskia MostertNo relationship to disclose

Unlabelled: Objectives: To describe the anatomical sub-types of Anorectal malformations, their management and the early outcome at Moi Teaching and Referral Hospital (MTRH) over a 16 month period. Design: A prospective study., Setting: MTRH, in the neonatal Unit and paediatric surgical wards for theinitial capture of patients and initial follow up. The Paediatric Surgical out-patient clinic was used for the subsequent follow ups., Subjects: All infants diagnosed with ARM (Anorectal malformations) at MTRH from November 2011 to April 2013., Main Outcome Measures: Sub-types of the Anorectal malformations, coexisting abnormalities morbidity and mortality rates., Results: There were 42 participants including 24 (57%) males and 18 (43%) females. Neonates presented at an average age of 4 ± 3, three days and older children presented on average age of 152 ± 118, three days. There were 30 (71%) neonates and 12 (29%) older infants. In males, the predominant sub-type was imperforate anus without a fistula found in ten participants (42% of males). In females, the predominant sub-type was recto-vestibular fistula found in 14 participants (78% of females). Mortality occurred in 13 (31%) participants among them ten (24%) had coexisting abnormalities. The main causes of morbidity were: colostomy complications in four (9.5%); wound,infections in one (5%); and wound dehiscence in one (5%)., Conclusions: Patients with Anorectal malformations presented late at MTRH. The diagnosis at birth was missed in babies born at home as well as those delivered in health institutions.

Background: Wilms' tumour is a common malignant neoplasm of the kidney and is ranked among the top six solid tumours in children in Kenya. Despite its rapid growth and therefore debilitating effects on its victims, it is one tumour that has shown good response to combined modality approach to its treatmentwith encouraging possibilities of survival even in resource poor settings., Objective: To evaluate the management and outcome of patients with Wilms'tumour attended to at Moi Teaching and Referral Hospital (MTRH) during the period between January 2000 and December 2007., Design: Retrospective Study., Setting: The Paediatric Oncology Service (Oncology unit in the Paediatric Ward, the Paediatric Surgical Ward and the Outpatient Oncology Clinic) at the Moi Teaching and Referral Hospital, Eldoret, Kenya., Results: Information of 45 patients diagnosed with Wilms' tumour was analysed. Forty two (93%) of the patients were referrals from various health facilities in the region. Twenty three (51%) were male and 34 (76%) were aged less than 48 months. Twenty five (56%) had the left kidney affected, 19 (42%) the right kidney and one (2%) bilateral. All the 45 (100%) had an abdominal ultrasound done but none had exhaustive investigations done to stage the disease. Only eight (18%) of the patients had a medical insurance cover. Fourty one (91%) of the patients received specific cancer treatment with 28 (62%) getting combined modality treatment. Nineteen (42%) were lost to follow up. Thirty (67%), 21 (47%), 15 (33%) and 13 (29%) patients were alive six months, one year, two years and three years respectively from the time of diagnosis. 29% survived beyond three years of diagnosis., Conclusion: Staging of Wilms tumour fell short of the expected. Neo-adjuvant chemotherapy reduced morbidity and mortality of patients managed for Wilms' tumour. Loss to follow up and cost of treatment had a negative impact on the outcome, a situation that requires to be improved.

Background: Cancer regisries worldwide have evolved to provide useful information on the burden and diversity of the patterns of cancer, information that is vital for establishing appropriate programmes for disease management. Population based data on cancer in western Kenya as captured in the Eldoret cancer registry established in 1999 is analysed and reported in this paper., Objective: To determine the burden and pattern of cancer in Western Kenya by use of data from the Eldoret cancer registry., Design: Retrospective study., Setting: The cancer registry located in the Department of Haematology at the Moi University, School of Medicine situated at the Moi Teaching and Referral Hospital, Eldoret, Kenya. The hospital has a catchment population of 13 to 15 million people forming about 40% of the Kenyan population., Results: A total of 5,366 patients were diagnosed to have cancer and attended to at the MTRH and other hospitals in Eldoret during the period between January 1999 and December 2006 giving an average of 671 cases per year. Among those treated 2,699 were males and 2,667 were females giving a M: F ratio of 1:1. About 21% of the patients had haematological malignancies with non-Hodgkins lymphoma being the most common. Another 79% of the patients had solid tumours with cancer of the oesophagus being the commonest. Cancer of the cervix and prostrate were the commonest among the females and males respectively. A general increase in the number of patients with Kaposis sarcoma associated with HIV/AIDS pandemic was observed., Conclusion: The burden of cancer is a significant health problem in western Kenya and there is need for the development of a comprehensive cancer care programme in the region to address the growing problem.

Background: Patients who are critically ill and those requiring emergency care are transported within and between hospitals on a regular basis seeking diagnostic or therapeutic services not available at the bed side or within the referring institution. The emergency of specialty systems often determines the ultimate destination of patients rather than proximity of facility and this has heightened the need for patient transfer. To achieve a favorable outcome, it is necessary to ensure that any transfer is carried out safely and effectively with minimum disruption of the continuum of care., Objectives: To determine the gap between existing knowledge of patient transfer principles and the practice by hospitals in Western Kenya referring patients to Moi Teaching and Referral Hospital (MTRH)., Design: Cross-sectional descriptive study., Setting: Accident and emergency department at MTRH., Subjects: Patients transferred in over a period of six months for critical/emergency care., Results: Evaluation was done for 97 transfers during the six months period. Age ranged from four days old to 70 years with a median of 28 years. A wide spectrum of diseases were seen. However in order of frequency the leading five were; trauma and accidents, vascular disorders, infections; anaemia and malignancies. Of the infections, respiratory infections topped the list with pulmonary tuberculosis as the leading disease entity. Majority of patients 43 (44%) were referred within 24 hours of being seen at the primary hospital. Only 56% were transported by ambulance; appropriate escort(nurse) was provided in 60%; documentation was provided in 85%; monitoring enroute was done in 24%; warmth was provided in 62%, 27% were dehydrated requiring resuscitation; respiratory support was inadequate as only 14% (of those who required) had airway and 32% had oxygen provided; intravenous fluids were provided in 34% of those who required; nasogastric intubation was provided in 30% of those who required; urethral catheterisation was provided in 23% of those who required; 50% of those with long bone fractures were splinted and only 3% of those who required cervical spine stabilisation had cervical collar., Conclusion: There was significant failure by hospitals in Western Kenya in the application of principles of patient transfer while referring patients to MTRH.

Objectives: To determine the outcome of emergency resection of sigmoid volvulus and to determine the factors associated with adverse outcome., Design: Retrospective case series review., Setting: Moi Teaching and Referral Hospital (MTRH), Eldoret, Kenya., Subjects: All cases of sigmoid volvulus operated at MTRH during the six years period between year 2000-2005., Main Outcome Measures: Mortality rate; morbidity rate; and duration of hospital stay., Results: Ninety two case files were analysed. Sigmoid volvulus accounted for 14.1% of all cases of intestinal obstruction and 80% of large gut obstruction. The mean age was 47.3 years and the median was 50 years. The range was 16 to 86 years. The male to female ratio was 29.3:1. Mortality was three (3.3%) cases and the morbidity was 20 (21.7%) cases. The mean duration of hospital stay was 11.8 days. Inadequate intravenous fluid therapy had a statistically significant adverse effect on outcome in this study. The yearly outcome remained unchanged during the six years of the study., Conclusions: Emergency resection in cases with a viable colon had a similar outcome to the traditional standard treatment by emergency endoscopic derotation followed by semi-elective or elective resection. The overall outcome was comparable to global standards. Inadequate postoperative intravenous fluid therapy significantly affected the outcome.

Gastrointestinal tract perforation in neonates is a serious problem associated with high morbidity and mortality. Co-morbid factors, particularly prematurity and low birth weight, impact negatively on the outcome. This article highlights issues related to diagnosis and treatment with a view to improving the poor prognosis found in this condition.

Background: Adhesions after abdominal and pelvic surgery are a major cause of intestinal obstruction in the western world and the pathology is steadily gaining prominence in our practice., Objective: To determine the magnitude of adhesive intestinal obstruction; to determine the types of previous operations in patients who presented with adhesive intestinal obstruction; to determine the outcome of treatment; and to determine the factors affecting the overall management of adhesion-related intestinal obstruction., Design: Retrospective descriptive study., Setting: The Moi Teaching and Referral Hospital (MTRH) - Eldoret, Kenya., Results: Ninety three patients were managed for adhesive intestinal obstruction. Of these, 57 were male and 36 were female. Abdominal distension, bilious vomiting, absolute constipation and abdominal pain were the main symptoms. Forty two (45%) patients were operated on, twenty five (59%) of them being operated on more than 72 hours after the start of the symptoms. Eight (9%) patients had ischaemic gut injury by the time of operation. Fluid therapy was inadequately administered in 86 (92%) patients, and their charts were not completely filled., Conclusion: Adhesion-related intestinal obstruction is a common problem encountered in the surgical service at the Moi Teaching and Referral Hospital-Eldoret. It is the major cause of intestinal obstruction. Fluid therapy and delayed surgical intervention were the major challenges in the management of these patients.

This is a report of the only case of gastroschisis that has survived at the Moi Teaching and Referral Hospital in the past five years. Others, who have been referred to this hospital arriving occasionally without the necessary care for this problem in place, have not even managed to survive through the period of resuscitation. The objective of this report is to show that these children can survive. Immediate post-natal care for protruded abdominal organs, fluid resuscitation and adherence to the principles of neonatal transport are essential.

Objective: To review the management of childhood intussusception at the Moi Teaching and Referral Hospital, Eldoret and identify factors that require attention for improved outcome., Design: A retrospective descriptive study covering the period January 2000 to December 2003., Setting: Moi Teaching and Referral Hospital, Eldoret., Patients: Thirty six children., Results: Of the 36 children, 28 (78%) were males and eight (22%) females giving a ratio of 3.5:1. Median age was six months with a range of 2-72 months. The duration of symptoms was a mean of 5 days with a range of 1-14 days. Sixty one percent were referrals. Initial diagnosis of intussusception based on signs and symptoms was made in 6 out of 36 (17%) patients. The rest were initially treated for other problems. Decision to refer to surgeons, was based on abdominal distension. Plain x-ray was done in four patients and barium enema in one patient. Seventy five percent of the patients required fluid resuscitation before operation. All patients (100%) were managed operatively. Seventy two percent had ileo-colic intussusception, 8% ile-oileal and 22% colo-colic. Perforation was found in 22% and gangrene in 31%. Sixty seven percent were successfully reduced by "milking" while 33% required resection and anastomosis/or stoma creation. Complications included: anastomatic breakdown, bronchopneumonial renal failure, sepsis, recurrent intussusception and death. Mortality was 14% overall., Conclusion: Early diagnosis and presentation coupled with improved peri-operative management are essential in improving outcome.

This is a report of bilateral testicular torsion. The objective of the report is to highlight the serious implications of misdiagnosis of testicular torsion. Other than loss of the testis due to necrosis in unilateral torsion, immunological damage of the opposite testis occurs. Early, accurate diagnosis, and institution of the appropriate treatment are desirable to prevent total loss of reproductive potential in the affected male.

Background: Gastric perforation in neonates is a catastrophe associated with high morbidity. Most are due to underlying primary pathology., Objectives: To review the management of gastric perforation in neonates in Kwa Zulu-Natal, South Africa., Design: Retrospective study of consecutive complete data sets of neonates presenting with gastric perforation., Setting: Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of Natal, Durban, South Africa., Subjects: Eight neonates treated for gastric perforation between January 1998 and April 2003., Main Outcome Measures: Morbidity and mortality., Results: There was an equal number of males and females. Median birth weight was 2.0 kg with a range of 1.4 to 3.2 kg. Five of the eight neonates were premature. Primary pathologies were associated with perforation in seven of the eight neonates. Prematurity, low birth weight and pneumonia were contributing factors to the poor outcome. Sepsis was a complication in seven of the eight neonates leading to their death (88% mortality)., Conclusion: Active perinatal management, early treatment of primary pathologies, and protection of the stomach against distension in neonates at risk are essential in the management of neonatal gastric perforation.

Background: Gastro-intestinal tract (GIT) perforation in neonates is a serious problem associated with high mortality due to resulting sepsis. Co-morbid factors, eg. prematurity, respiratory problems, low birth weight, and nutritional factors, negatively affect the outcome., Objectives: To review the management outcome of gastro-intestinal tract perforation in neonates in KwaZulu-Natal and identify factors that require attention for better survival of neonates with GIT perforation., Design: Retrospective study of consecutive complete data sets of patients presenting with a diagnosis of GIT perforation., Setting: Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of Natal, Durban, South Africa., Subjects: Fifty four neonates treated for gastro-intestinal tract perforation between January 1998 and January 2003., Main Outcome Measures: Morbidity as determined by complications and mortality., Results: More males (69%) were affected than females (31%). The median birth weight was 2.3 kg and median age at presentation was four days. Eighty nine percent were referred from peripheral hospitals. Abdominal distension was the leading symptom and sign (74%). Co-morbid factors were present in 89%, with prematurity as the leading factor (52%). Necrotising enterocolitis (NEC) was the main cause of perforation (33%) and the terminal ileum was the most common site. Most (56%) were treated by excision and primary repair of perforations. Sepsis was the leading complication (44%) and major cause of death (72%). Mortality was highest (56%) in perforations due to other primary pathology followed by NEC (53%). Overall mortality was 46%., Conclusion: It is essential to prevent secondary perforations by early recognition and management of primary pathology. Management of pneumoperitoneum in neonates with respiratory difficulties should be included in resuscitation before transfer. Rectal temperature monitoring and herbal enemas should be strongly discouraged.

Objective: To determine the pattern of surgically managed peptic ulcer disease., Design: A retrospective study., Setting: Department of Surgery, Moi Teaching and Referral Hospital, Eldoret, Kenya., Subjects: Fifty three patients operated on for peptic ulcer disease., Results: The mean age was 47 years with a male/female ratio of 1.7:1. Duodenal ulcer associated complications were the commonest with duodenal ulcer/gastric ulcer ratio of 11.5:1. Most patients had chronic peptic ulcer symptoms with inadequate or no medical treatment. Perforations were the commonest complications (56.6%) followed by gastric outlet obstruction (34.0%). Closure with omental patch was done in 83.3% of perforations while truncal vagotomy and drainage was done in gastric outlet obstruction. Hypostatic pneumonia was the commonest post-operative complication. Seventy one point seven per cent of the patients were free of dyspeptic symptoms during the brief follow-up period., Conclusion: Most patients with chronic peptic ulcers had had inadequate treatment and perforation was the most common complication. Repair of perforations with omental patch, and truncal vagotomy and drainage procedure for gastric outlet obstruction, were satisfactory surgical methods offered to patients at the Moi Teaching and Referral Hospital.

A rare case of spinal hydatid disease presenting with paraparesis and sensory loss is reported. The patient was treated with albendazole resulting in significant improvement within eight weeks. Investigations and treatment modalities are discussed.