PRESENTATION OF CASE: We report the first case of Blue Rubber Bleb Nevus Syndrome in Malaysia, a 23 years old Malay girl who suffers from multiple cutaneous venous malformation and gastrointestinal bleeding episodes.
DISCUSSION: The typical morbidity for this syndrome is symptomatic anemia due to secondary iron deficiency due to the gastrointestinal venous malformation bleeding. In managing the gastrointestinal bleeding, it mainly depends on the severity of gastrointestinal bleeding, some may resolve spontaneously, while the others may be needing blood transfusion, and some may require GIT resections. As for cutaneous lesions, normally it is innocuous depending on the region and size. Large or problematic cutaneous venous malformation might benefit from sclerotherapy or excision.
CONCLUSION: Multidisciplinary approach is crucial in managing BRBNS case due to its complexity and the spectrum of multiple organ involvement to ensure the best outcome to the patient.
CASE REPORT: A case of unusual severe HDFN due to anti-D alloimmunisation in undiagnosed RhD negative primigravida Malay woman is reported here. This case illustrates the possibility of an anamnestic response from previous unknown sensitisation event or the development of anti-D in mid trimester. The newborn expired due to hydrops fetalis and severe anaemia. Antenatally, the mother was identified as RhD positive and thus there was no antenatal antibody screening, antepartum anti-D prophylaxis or close fetal monitoring for HDFN.
DISCUSSION: The thorough antenatal ABO and RhD blood grouping with antibody screening is mandatory as part of prevention and early detection of HDFN especially due to anti-D alloimmunisation. Improper management of RhD negative women might lead to severe HDFN including in primigravida.
Methods: The initial part of this study is a descriptive cross-sectional study involving data collection from all requests sent for group, screen, and hold (GSH) and group and cross match (GXM) tests from 2011 to 2017. The association between sociodemographic, workplace, and experience factors with near-miss events amongst HO was analyzed with a case-control study using logistic regression.
Results: We reported 83 near-miss events with a prevalence of 0.034% (95% confidence interval 0.027-0.042). The rate of near-miss events was one in every 2916 requests. The mean reporting rate was 11.9 events per year. Clinical near miss predominated at 89.2% compared to 10.8% laboratory near miss. Mislabeled events (33.7%) were more than miscollected events (10.8%). HO were implicated with most events (83.1%). Most events were predominantly in the medical and obstetrics and gynecology wards amounting to 31.3% each. We found a significant association between the ages of HO with near-miss events.
Conclusions: The prevalence of near-miss events in our hospital was relatively low. Our study has shown areas for improvement include improving sampling practices in clinical areas, adequate training of laboratory technicians, and providing proper transfusion education. Interventions such as encouraging compliance to guidelines and training in clinical and laboratory areas to minimize the risk of mistransfusion should be considered.
METHODS: An analytical cross-sectional study using purposive sampling method was conducted at eight thalassemia societies in Peninsular Malaysia. 260 respondents comprised of patients and caregivers were assessed with two separate sets of questionnaires.
RESULTS: Knowledge on dietary iron among the respondents was unsatisfactory, despite them having good knowledge on thalassemia disorder. Female patients were found to have better dietary knowledge, attitude and practice compared to males. The percentage of caregivers with good attitude and good practice were significantly higher compared to adult patients. Caregivers with children on iron chelators were noted to have better dietary attitude and practice. Thalassemia knowledge and children on vitamins were found to be the predictors of dietary knowledge among the patients and caregivers respectively.
CONCLUSION: The level of knowledge on dietary iron among the patients and caregivers was unsatisfactory in spite of their attitude and practice towards dietary iron were good. Effective delivery of dietary information to the patients and caregivers is essential to enable them to choose a healthy diet for their condition.