Gaucher's Disease at a National Referral Hospital

Objectives: To determine the prevalence and to characterise Gaucher\'s disease in terms of socio-demographic data, clinical presentation, and management as seen at Kenyatta National Hospital. Design: A retrospective record based study. Setting: Kenyatta National Hospital, a referral and teaching hospital. Main outcome measures: Prevalence (number of cases seen a year), of Gaucher\'s disease, sociodemographic data, clinical presentation, mode of diagnosis and treatment modalities of Gauchers disease. Results: Nine patients were studied, four males and five females giving a prevalence of 0.9 cases seen a year and a M:F ratio of about 1:1. The most common presentation was splenomegaly in nine (100%) cases and hepatomegaly in seven (78%) patients, neurological and bone symptoms were rare, in one (11%) cases and in two (22%) cases respectively. Diagnosis was mainly on basis of presence of Gaucher cells in bone marrow and splenic aspirate as enzyme assay was unavailable. Management was mainly supportive and enzyme therapy was only available for two (22%) patients. Anaemia was the most common complication with seven (78%) patients and one death occurred due to osteomyelitis. Only four (44%) patients were followed up for a period of four years. Conclusions: Gaucher\'s disease is a rare condition at the Kenyatta National Hospital (KNH). The presentation of most patients is organomegaly, (hepatosplenomegaly) and best fits the type 1 or non-neuronopathic Gaucher\'s disease. Neurological manifestations are rare. Management of this condition at the KNH is mainly supportive and enzyme therapy still remains out of reach for most patients

Hypo-phosphataemia in children under five years with kwashiorkor and marasmic kwashiorkor

Background: Severe malnutrition contributes up to 50% of childhood mortality in developing countries is frequently characterised by electrolyte depletion, including low total body phosphate. During therapeutic re-feeding, electrolyte shift from extracellular to intra-cellular compartments may induce hypo-phosphataemia (hypo-P) with resultant increased morbidity and mortality. This biochemical imbalance isunder-recognised, and the frequency of this problem among African malnourished children is unclear.Objectives: To determine the magnitude of hypo-phosphataemia in children under five years of age presenting to Kenyatta National Hospital with kwashiorkor and marasmic kwashiorkor and to evaluate the relationship between hypo- phosphataemia and nutritional intervention during the first five days of treatment.Design: Short longitudinal survey.Setting: The General Paediatric wards of the Kenyatta National Hospital (KNH), Nairobi.Subjects: Children under five years of age presenting with kwashiorkor or marasmic kwashiorkor at KNH were recruited into the study. Main outcome measures: Low serum phosphate level

Efficacy of a discharge checklist for neonates in reducing neonatalmorbidity and mortality

Introduction: In 2015, almost 1 million child deaths occurred in the first day of life while about 2 million deaths occurred in the first week. Most of these deaths are readily preventable or treatable with proven, cost-effective interventions like a checklist to reduce neonatal morbidity and mortality.Objective: The primary objective was to determine the efficacy of a standardized neonatal discharge checklist on the rate of  hospitalization during the neonatal period at the Kenyatta National Hospital. The specific objective was to determine the acceptability of a structured postnatal discharge checklist among health care workers at Kenyatta National Hospital.Study design setting and population: A mixed method study that included the Quasi-experimental pre- post intervention design and focus group discussion on nurses was carried out in the post-natal wards at Kenyatta National Hospital. Neonates with no complications were enrolled for the study after consent was obtained.Methodology: Qualitative and quantitave methods were incorporated in this study. Structured questionnaires were administered to mothers in the postnatal ward and trained nurses on the checklist. Qualitative data was obtained using focus group discussions.Results: There was a trend noted for reduced hospitalization following introduction of the neonatal discharge checklist from 7.4% to 3.2% in the pre intervention and post intervention respectively a 56.7% reduction in hospitalization (p=0.06). There was significant improvement in knowledge on cord cleaning after the intervention (p =<0.001) as well as on identifying newborn danger signs  (p=0.005).Conclusion: There was a trend for reduced hospitalization following implementation of the neonatal discharge checklist.&nbsp

Efficacy of Bacillus clausii in reducing duration of illness in acute diarrhoea in children 6-59 months of age admitted with severe dehydration

Background: Acute infectious diarrhoea is the second commonest cause of under-five mortality in the world. It has been associated with an increased morbidity and high rate of admission into hospital due to severe dehydration. Multiple studies document that probiotics are effective in treating infectious diarrhoea in children. This study attempted to determine whether Bacillus.clausii (B.clausii) is effective in shortening acute diarrhoeal illness in under-five population with severe dehydration.Methods: In a randomized, double-blind, placebo-controlled trial, children (age range: 6 months to 5 years) with acute diarrhoea and World Health Organization (WHO) criteria of severe dehydration were administered B.clausii, twice daily, for 5 days. Routine standard care and WHO protocols of managing diarrhea and dehydration were followed in both groups. Primary outcome measure was the duration of diarrhoea.Results: In a per-protocol analysis of 90 children, the mean duration of diarrhoea in the B.clausii group (n=44) was insignificantly shorter (77.59 ± 34.10 hours) than the placebo group (n=46) (86.74 ± 40.16 hours) There was a mean difference between the groups of 9.15 hours (t (88) = 1.163, P = 0.248, 95% C.I -6.88 – 24.79). There was a significant decrease in the mean number of diarrhoeal motions on day 3 [B.clausii group 2.74±1.81 motions vs. Placebo group 3.80±2.70 motions; mean difference=1.05 motions; (t (88) = 2.169, P = 0.033, 95% C.I 0.09 – 2.02)].Conclusion: In children admitted with acute diarrhea and severe dehydration, there was no significant difference in the duration of diarrhoea and duration of hospital stay in the two groups

Seroprevalence of Hepatitis B markers in pregnant women in Kenya

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Seroprevalence of hepatitis B markers in pregnant women in Kenya

Objective: To evaluate hepatitis B serological markers in pregnant women from various geographical sites in Kenya.Design: A cross-sectional observational study of women attending antenatal clinics.Setting: The Kenyatta National Hospital and eight hospitals from five provinces in Kenya.Subjects: All women in their third trimester of pregnancy attending the antenatal clinic over the period June 2001 to June 2002.Main outcome measures: For each pregnant woman age and gestation were documented. Hepatitis serological markers were evaluated.Results: A total of 2,241 pregnant women were enrolled. Among them 205 women (9.3%) were positive for HbsAg and from these 18 (8.8%) were found to have HbeAg. Protective antibodies (anti-HbsAg) were detected in 669 (30.2%) of the women. There were notable significant regionaldifferences for HbsAg rates.Conclusions: These results confirm the presence of high disease carrier rate and the corresponding previously reported low level of HbeAg suggesting questionable low rate of perinatal transmission but high rate of horizontal transmission

Effective training-of-trainers model for the introduction of continuous positive airway pressure for neonatal and paediatric patients in Kenya

Background: Continuous positive airway pressure (CPAP) is a relatively low-cost technology which can improve outcome in neonatal and paediatric patients with respiratory distress. Prior work in a lower middle-income country demonstrated degradation of CPAP skills and knowledge after the initial training. Aims: To determine if a training-of-trainers (ToT) curriculum can decrease gaps in skills and knowledge between first-generation (trained by a United States physician and nurse) and second-generation healthcare providers (trained by local trainers) in Kenya, and to describe the usage pattern, outcome and safety in patients who received CPAP following the trainings. Methods: The first day of training entailed didactic and simulation sessions. On the second day participants were taught how to train others to use CPAP. First- and second-generation healthcare providers were tested on their skills and knowledge. Unpaired t-tests were used to test for equivalence. Prospective data on CPAP usage was collected following the initial trainings. Results: 37 first-generation healthcare providers (16 nurses; 21 physicians, medical/clinical officers) were trained as trainers and 40 second-generation healthcare providers (19 nurses, 21 physicians, medical/clinical officers) trained by first-generation healthcare providers were available for skills and knowledge testing. There were no statistically significant differences between first- and second-generation healthcare providers’ skills (90%, 95% CI 87–93 vs 89%, 95% CI 86–92) or knowledge scores (91%, 95% CI 88–93 vs 90%, 95% CI 88–93). A total of 1111 patients were placed on CPAP mostly by nurses (61%), prematurity/acute respiratory distress syndrome was the most common indication, nasal injury/bleeding (2%) was the most common reported adverse event, and the overall mortality rate was 24%. Conclusion: The ToT model was successful, nurses initiated CPAP most commonly, prematurity with acute respiratory distress syndrome was the most common indication, and adverse events were uncommon.</p