Iron burden and endocrine complications in transfusion-dependent thalassemia patients In Sarawak, Malaysia: a retrospective study.

INTRODUCTION: Thalassaemia is one of the major health problems in Malaysia. With safe blood transfusion regime, the lifespan of patients with transfusion-dependent thalassaemia (TDT) has improved but at the cost of a higher risk of developing endocrine disorders. It is crucial for us to monitor the iron overload to prevent end organ damage. This study aims to evaluate the iron burden and prevalence of endocrinopathies in patients with TDT in Sarawak. MATERIALS AND METHODS: This retrospective cohort study was conducted between January 2020 to June 2020 in six government hospitals in Sarawak. A total of 89 patients with TDT, aged 10 years and above, were recruited. RESULTS: Out of the 89 patients, there were 54 males (60.7%) and 35 females (39.3%) with a median age of 21 years (range 10.0-65.0). Sixty-seven (75.3%) patients had betathalassaemia major and 15 (16.9%) patients had haemoglobin E beta-thalassaemia (HbE beta-thalassaemia), remaining seven patients had other genotypes. Thirty-one (34.8%) patients had mean serum ferritin 2500ng/ml and above, and 44 (66.6%) had liver iron concentration (LIC) ≥7mg/g. The prevalence of endocrine disorders in our cohort was 69.7%. The most common endocrinopathies were short stature (n=46, 51.7%), followed by hypogonadism (n=24, 26.9%), delayed puberty (n=23, 25.8%), hypothyroidism (n=10, 11.2%), diabetes mellitus (n=9, 10.1%), impaired glucose tolerance (n=6, 6.7%) and hypoparathyroidism (n=3, 3.3%). Endocrinopathies were significantly associated with age (p=0.01), age at initiating regular blood transfusion (p<0.01) and duration of regular blood transfusion (p<0.01). CONCLUSION: Our data shows that the development of endocrinopathies in TDT can be time dependent. Early detection of endocrine-related complications and prompt treatment with iron chelation therapy are important to improve morbidity and mortality. A multidisciplinary approach with good patient-doctor collaboration is the key to improving patient care in our settings.

Demographic characteristics and outcomes of continuous ambulatory peritoneal dialysis related peritonitis in Miri General Hospital, Malaysia.

INTRODUCTION: Sarawak has a population that is geographically and characteristically widely varied. In this study we aimed to determine the demographic characteristics of our patient population who undergo continuous ambulatory peritoneal dialysis (CAPD) and to study the incidence, the microbiology and the outcome of CAPD peritonitis. METHODS: A retrospective record review of all CAPD patients on follow-up at the Miri Hospital, Sarawak, Malaysia from 2014 until 2017 was done. RESULTS AND DISCUSSION: During the 4-year period, the overall peritonitis rate was 0.184 episodes per patient-year. Gram-positive and gram-negative bacteria each constituted one-third of the peritonitis; fungi (2.6%), Mycobacterium tuberculosis (MTB) (5.3%), polymicrobial (2.6%) and sterile culture (26.3%). The most commonly isolated gram-positive bacteria were coagulase-negative Staphylococcus. Our peritonitis rate is comparable to that of other centres i.e., Japan 0.195 and Indonesia 0.25. In comparison, countries like India (0.41), Korea (0.40) and Singapore (0.59) had relatively higher rate of PD-associated peritonitis. Two tuberculosis peritonitis patients died. The rate of catheter removal was approximately 20%. Gram-negative bacteria and MTB have a higher risk of catheter loss. About one-fifth used rainwater to clean their CAPD exit site. Out of this group, 33% did not boil the rainwater prior to usage. CONCLUSION: Patient's characteristics and microbial susceptibility vary in different places of practice. The high rates of culture-negative peritonitis and high mortality risks associated with TB peritonitis warrant special attention. In patients with refractory peritonitis, early catheter removal is warranted in order to reduce mortality and minimize damage to peritoneal membrane.

Thrombotic thrombocytopenic purpura-like syndrome associated with arcanobacterium pyogenes endocarditis in a post-transplant patient: A case report.

Systemic Arcanobacterium pyogenes is a rare bacterial infection in humans.1The diagnosis of thrombotic thrombocytopenic purpura (TTP)-like syndrome and infective endocarditis (IE) is often elusive. We report a case of TTP-like syndrome associated with A. pyogenes endocarditis in a post-allogenic transplant patient.

Cochlear implant in Hong Kong Cantonese.

Cochlear implant surgery was performed in four Cantonese-speaking postlingually deaf Chinese adults, using the House/3M single channel device. This article outlines the methodology, including preoperative assessment and postoperative rehabilitation; and explains the necessary modifications in speech and audiologic work-up in Cantonese-speaking patients. Salient features of Cantonese phonetics, especially its tonal characteristics, are described. The findings of the study are presented. The results of the cochlear implant would suggest a performance superior to that of the hearing aid. Furthermore, the cochlear implant is able to detect tonal cues. This quality of the cochlear implant may prove to be a valuable asset to a tonal language-speaking cochlear implantee.

Leiomyoma of the nasal cavity.

A case of rare leiomyoma of the nasal cavity is reported. The pathological and clinical characteristics of this tumour are discussed.