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

Knowledge, Attitude and Perceptions of Village Residents on the Health Risks Posed by Kadhodeki Dumpsite in Nairobi, Kenya.

This study sought to assess the knowledge, attitude and perceptions of the residents of Kadhodeki village on the health risks posed by the Kadhodeki dumpsite. Using households as units of sampling, a descriptive cross sectional survey was carried out in June 2012. Random data were collected using a face-to-face researcher administered structured questionnaire and 323 participants were interviewed. Chi square was used to determine the association between the different variables. Findings indicate that residents possess a significantly low knowledge and a positive attitude (χ2 (1) = 224.03, p < 0.01; χ2 (1) = 8.697, p < 0.01) towards the Kadhodeki dumpsite. They were however no differences in risk perceptions. Participant’s age, duration one had lived in the village and their level of education, were proxy measures of knowledge, attitude and perception. Odds ratio analysis indicates that age and duration did not influence participant’s knowledge, attitude or perceptions. Rudimentary however, education accounted for a non significant 28% variation in respondents’ attitude towards the health risks of the dumpsite (OR= 1.282; CI 0.828- 1.997). Also adequate education significantly accounted for 67% variation in respondents’ health risk perception (OR= 1.671; CI 1.304-2.140). This study would recommend that the ministry of Health come up with health education programmes for the general population on the dangers of uncontrolled waste disposal sites

Factors affecting guidance and counseling programme in primary schools in Nairobi Province, Kenya

The objective of this study was to establish the factors that affect guidance and counseling in primary schools. Guidance and counseling seems not to be adequately helping pupils with physical and psychological problems in Nairobi province of Kenya. Many primary schools are faced with indiscipline and poor performance yet there is a guidance and counseling programme running in these schools. The sample of study was 234 respondents selected from a population of 10,000 primary school teachers in Nairobi city through the stratified and simple random methods. A questionnaire was used to collect data. The results showed that majority of the respondents do not regard guidance and counseling as a critical issue in the development of the pupils. Too, it was found that teachers’ attitudes, training in counselling, availability of time and facilities have a negative impact on the guidance and counselling programme. The Government and the schools’ management do not provide the needed infrastructure for guidance and counseling. It was recommended that training of teachers in guidance and counseling be given priority by the schools and the government.KEY WORDS: Guidance, counselling, primary school and factor

Hereditary haemorrhagic telangiectasia in a black adult male: case report

Hereditary haemorrhagic telangiectasia, (HHT) or Rendu-Osler-Weber disease is a genetic autosomal dominant disorder that is characterised by telangiectasias, (small vascular malformations), in mucocutaneous tissues and arterial venous malformations, (AVMs), in various internal organs. Although HHT is relatively common in whites, the disorder has been reported to be rare in people of black African descent. Majority of HHT patients present with recurrent epistaxis, which in a significant proportion of patients is severe, warranting repeated blood transfusions and iron supplementation. Telangiectasias are most frequent on the tongue, hands, nose, lips and the gastrointestinal tract (GIT). AVMs occur in internal organs, particularly the lungs, brain, and the liver. Early and correct diagnosis of HHT is crucial as patients derive benefit from certain specific treatment modalities. Besides, AVMs which occur in various organs pose serious complications that may lead to death and therefore require early detection. We report a 55 year old black African male with HHT who presented with severe recurrent epistaxis and haematochezia leading to severe anaemia requiring repeated blood transfusions. His son, daughter and a maternal uncle experience milder recurrent epistaxis. The management of this patient and a brief review of the clinical features and management of HHT is presented. Our aim is to raise awareness of the occurrence of HHT in Kenya, in order to enhance early diagnosis and appropriate management.East African Medical Journal Vol. 85 (8) 2008: pp. 412-41