Is renal medullary carcinoma the seventh nephropathy in sickle cell disease? A multi-center Nigerian survey.

Introduction: Previous studies had enlisted renal medullary carcinoma (RMC) as the seventh nephropathy in sickle cell disease (SCD). Clinical experience has contradicted this claim and this study is targeted at refuting or supporting this assumption. Objective: To estimate the prevalence of RMC and describe other renal complications in SCD. Materials and methods: 14 physicians (haematologists and urologists) in 11 tertiary institutions across the country were collated from patients’ case notes and hospital SCD registers. Results: Of the 3,596 registered sickle patients, 2 (0.056%) had been diagnosed with RMC over a ten year period, thereby giving an estimated prevalence rate of 5.6 per 100,000. The most common renal complication reported by the attending physicians was chronic kidney disease (CKD). The frequency of routine renal screening for SCD patients varied widely between centres – most were done at diagnosis, annually or bi-annually. Conclusion: The ten year prevalence of RMC in Nigerian SCD patients was determined to be 5.6 (estimated incidence of 0.56). RMC is not more common in SCD patients and therefore cannot be regarded as a “Seventh Sickle nephropathy”. Most of the managing physicians reported that the commonest nephropathy observed in their SCD patients was chronic kidney disease.

Councellors' Experience During Training and Home Based HIV Councelling and Testing in Zomba District, Malawi

Introduction In 2007, St Luke’s Mission Hospital initiated a district-wide Door to Door HIV counselling and testing (HCT) programme in Zomba district. The intent of the programme was to provide quality HCT services to people in their homes and effectively those found to be HIV positive referred to appropriate services. Methodology This was a cross sectional study using a questionnaire consecutively administered to a sample of 105 counsellors who had resided in the community for a period of over one year. The questionnaire sought to establish, knowledge gained, experiences and recommendations on how the programme has been implemented and assist running of similar future programmes. Data analysis was done manually using both qualitative and quantitative methodologies. Results We report that nearly 23% of the counsellors thought that during their training as a door to door HTC councelor they had benefited in learning to working with communities; an aspect they found to be highly applicable in discharge of their duties. The major setbacks during the training were lack daily allowances, less amount of time spent on understanding child councelling and the manual used was diffucult to follow. Over 32% of the councellors were satisfied with the participation of their clients during pre-test counselling sessions, however, the major challenge they had was the misconception that they were blood suckers, a view reported by nearly 17% of the counsellors. Close to 72% reported not to have met any problems during post-test counselling compared to 24% who reported to have found challenges. Conclusion The study has revealed that there is a need to re-look child children counselling especially in training door to door HCT counsellors. It has also revealed the prevalent allowance culture despite the benefits of training. The common challenges were refusal of test Results and failure to understand discordance. Misconceptions may still exist in the community regarding anything dealing with removing blood. There is still need for more information regarding discordance especially among couples in the community.

Experience of a Single Center in NTBC Use in Management of Hereditary Tyrosinemia Type I in Libya

Background: Hereditary Tyrosinemia type I (HTI) is a metabolic disease caused by deficiency of fumarylacetoacetate hydrolase enzyme. Objectives: This study reports beside its clinical and biochemical presentation, the outcome of NTBC [2- (2-nitro-4-trifloro-methylbenzoyl)-1, 3-cyclohexanedion] treatment of the disease and evaluates its biochemical markers in 16 pediatric Libyan patients. Patients and Methods: The diagnosis was based on presence of high tyrosine levels in blood and succinylacetone in urine. Results: The consanguinity rate was 81.2%, the median age at onset, at diagnosis and at starting treatment were 4.5, 8, and 9.5 months respectively. At presentation hepatomegaly, jaundice, rickets and high gamma glutamyl transferase (GGT) were observed in 87.5% of patients. All patients had extremely high alpha fetoprotein (AFP) and high alkaline phosphatase (ALP) levels. Fifteen patients were treated with NTBC, normalization of PT (Prothrombine time) was achieved in average in 14 days. The other biochemical parameters of liver function (transaminases, GGT, ALP, bilirubin and albumin) took longer to improve and several months to be normalized. Survival rate with NTBC was 86.6%. Patients who started treatment in a median of 3 months post onset observed a fast drop of AFP in 90.6% of patients (P = 0.003). Abnormal liver function and rickets were the common presentations, GGT was an early cholestatic sensitive test. ALP was constantly high even in asymptomatic patients. Conclusions: In HT1 a faster dropping of AFP is a marker of good prognosis.