ObjectiveThalassaemia is the most common inherited blood disorder in Malaysia. This study aims to report the current status of thalassaemia in Malaysia and provide a comprehensive understanding of the disease through data obtained from the Malaysian Thalassaemia Registry.DesignData were extracted from the Malaysian Thalassaemia Registry, a web-based system accessible to enrolled users through www.mytalasemia.net.my.SettingThe Malaysian Thalassaemia Registry data was recorded from reports obtained from 110 participating government and university hospitals in Malaysia.ParticipantsThe patients were those attending the 110 participating hospitals for thalassaemia treatment.InterventionData were collected from the Malaysian Thalassaemia Registry from 2007 until the fourth quarter of 2018.Primary outcome measure7984 out of 8681 patients with thalassaemia registered in the Malaysian Thalassaemia Registry were reported alive.ResultsMajority of the patients were reported in the state of Sabah (22.72%); the largest age group affected was 5.0–24.9 years old (64.45%); the largest ethnic group involved was Malay (63.95%); and the major diagnosis was haemoglobin E/β-thalassaemia (34.37%). From the 7984 patients, 56.73% were on regular blood transfusions and 61.72% were on chelation therapy. A small fraction (14.23%) has undergone splenectomy, while the percentage of patients with severe iron overload (serum ferritin ≥5000 µg/L) reduced over time. However, cardiac complications are still the main cause of death in patients with thalassaemia.ConclusionData gathered into the registry can be used to understand the progression of the disorder, to monitor iron overload management and to improve the outcomes of treatment, to enhance preventive strategies, reduce healthcare burden and improve the quality of life. Sustainability of the Malaysian Thalassaemia Registry is important for surveillance of thalassaemia management in the country and help the national health authorities to develop more effective policies.

One hundred and twenty patients with confirmed second degree haemorrhoids were randomly allocated to four treatment groups: injection, rubber band ligation, maximal anal dilatation, and haemorrhoidectomy. Each group consisted of 30 patients. All patients were regularly followed up for at least one year. Assessment at one year showed that haemorrhoidectomy “cured” the haemorrhoids in 29 out of 30 patients. Rubber band ligation relieved 25 out of 30 and maximal anal dilatation 24 out of 30. Injection was the least effective treatment, and relieved 18 of the 30 patients, with a cure rate of 60% only. Haemorrhoidectomy caused pain in all cases, anal stenosis in two, postoperative haemorrhage in two, and the patients required an average hospital stay of 11.5 days and an average of a further 15.5 days off work. Rubber band ligation was painless in 26 patients out of 30, and maximal anal dilatation was painless in 25 out of 30. There were no postoperative complications in the latter two treatment groups. Haemorrhoidectomy is good in “curing” the disease, but the higher possibility of postoperative pain and complications and longer hospital stay would not justify its use in the treatment of second degree haemorrhoids. Both rubber band ligation and maximal anal dilatation are effective and relatively free from complications. Rubber band ligation has the additional advantage of not requiring hospital stay or anaesthesia and is therefore considered to be the most appropriate method of treatment for second degree haemorrhoids.

BackgroundTumor prostheses currently give the best short- and medium-term results for limb-salvage reconstruction procedures in the treatment of bone tumors. However, in developing countries, the cost of a tumor prosthesis is beyond the reach of much of the population. We report the use of autoclaved tumor-bearing bone in 10 patients, as an affordable alternative to the use of prostheses.MethodsThis is a case series of 10 patients (mean age 19 years) with osteosarcoma who were treated at our hospital from 1998 to 2008, and followed up for a mean of 35 months (range 14 to 8). The femur was involved in six cases, the humerus in three cases, and the ulna in one case. The mean length of the autoclaved bone was 150 mm (range 60–210).ResultsBone union occurred in seven patients over an mean duration of 12 months (range 8–17). Three patients had non-union. Two of these had associated implant failure, with one of them also developing chronic infection, and the third is still being followed up. Two other patients had local recurrence.ConclusionThe use of autoclaved tumor grafts provides an inexpensive limb-salvage option without sacrificing appropriate oncologic principles. A painless and stable limb is achievable, and the use of this technique can be further refined.

Type of cancer and age of onset in individuals with inherited aberrations in the tumour suppressor gene TP53 are variable, possibly influenced by genetic modifiers and different environmental exposure. Since 2009, the modified Chompret criteria (MCC) have been used to identify individuals for TP53 mutation screening. Using the TP53 mutation database maintained by the International Agency for Research on Cancer (IARC), we investigated if the MCC, mainly developed for a Caucasian population, was also applicable in Asia. We identified several differences in Asian families compared with similar Caucasian cohorts, suggesting that identification and management of Li-Fraumeni syndrome in Asia do not completely mirror that of North America and Western Europe. Early gastric cancer (<40 years) may be considered a new addition to the MCC especially for Asian families.