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Nipah virus outbreak in Malaysia

Chua KB

J Clin Virol, 2003 Apr;26(3):265-75.
PMID: 12637075

Nipah virus, a novel paramyxovirus, closely related to Hendra virus emerged in northern part of Peninsular Malaysia in 1998. The virus caused an outbreak of severe febrile encephalitis in humans with a high mortality rate, whereas, in pigs, encephalitis and respiratory diseases but with a relatively low mortality rate. The outbreak subsequently spread to various regions of the country and Singapore in the south due to the movement of infected pigs. Nipah virus caused systemic infections in humans, pigs and other mammals. Histopathological and radiological findings were characteristic of the disease. Fruitbats of Pteropid species were identified as the natural reservoir hosts. Evidence suggested that climatic and anthropogenic driven ecological changes coupled with the location of piggeries in orchard and the design of pigsties allowed the spill-over of this novel paramyxovirus from its reservoir host into the domestic pigs and ultimately to humans and other animals.

Matched MeSH terms: Hematologic Diseases/epidemiology; Hematologic Diseases/virology
Fulltext Comprehensive screening shows that mutations in the known syndromic genes are rare in infants presenting with hyperinsulinaemic hypoglycaemia

Laver TW, Wakeling MN, Hua JHY, Houghton JAL, Hussain K, Ellard S, et al.

Clin Endocrinol (Oxf), 2018 Nov;89(5):621-627.
PMID: 30238501 DOI: 10.1111/cen.13841

OBJECTIVE: Hyperinsulinaemic hypoglycaemia (HH) can occur in isolation or more rarely feature as part of a syndrome. Screening for mutations in the "syndromic" HH genes is guided by phenotype with genetic testing used to confirm the clinical diagnosis. As HH can be the presenting feature of a syndrome, it is possible that mutations will be missed as these genes are not routinely screened in all newly diagnosed individuals. We investigated the frequency of pathogenic variants in syndromic genes in infants with HH who had not been clinically diagnosed with a syndromic disorder at referral for genetic testing.
DESIGN: We used genome sequencing data to assess the prevalence of mutations in syndromic HH genes in an international cohort of patients with HH of unknown genetic cause.
PATIENTS: We undertook genome sequencing in 82 infants with HH without a clinical diagnosis of a known syndrome at referral for genetic testing.
MEASUREMENTS: Within this cohort, we searched for the genetic aetiologies causing 20 different syndromes where HH had been reported as a feature.
RESULTS: We identified a pathogenic KMT2D variant in a patient with HH diagnosed at birth, confirming a genetic diagnosis of Kabuki syndrome. Clinical data received following the identification of the mutation highlighted additional features consistent with the genetic diagnosis. Pathogenic variants were not identified in the remainder of the cohort.
CONCLUSIONS: Pathogenic variants in the syndromic HH genes are rare; thus, routine testing of these genes by molecular genetics laboratories is unlikely to be justified in patients without syndromic phenotypes.

Matched MeSH terms: Hematologic Diseases/genetics
Fulltext Effects of prolonged confined isolation on status of inflammation, endothelial activation and function: in preparation for possible future manned space expedition to mars

Thuhairah Rahman, Radzi Ahmad, Suhaila Muid, Tengku Saifudin Tengku Ismail, Buravkova, Ludmila B., Hapizah Nawawi

Pertanika Journal of Science & Technology, 2020;28(2):659-672.
MyJurnal

Inflammation and endothelial dysfunction are key components in atherogenesis. Should the status of these pro-atherogenesis factors be enhanced during prolonged confined space travel, specific countermeasures need to be instituted to prevent these processes to ensure safe outcome for astronauts during space expeditions. Six crew members were exposed to prolonged, confined isolation for 520 days. Standard exercise and diet regime were instituted throughout isolation phase. Age and gender-matched healthy, free living controls were recruited in parallel. Serial serum and whole blood were analysed for biomarkers of inflammation (hsCRP and IL-6) and endothelial activation (sICAM-1, sVCAM-1 and E-selectin). Flow-mediated dilatation (FMD) of the artery was performed following the standard protocols set by the International Brachial Artery Reactivity Task Force by trained personnel. There was decreased sVCAM-1 concentration in crew members compared to baseline. However, there was significant decrease in percentage dilatation from baseline in FMD of the brachial artery in the crew members. Percent change increment was observed in hsCRP while percent change reduction was seen in sVCAM-1. The enhanced inflammation and reduced endothelial function could possibly be attributed to the rigorous exercise instituted throughout the confinement period. Furthermore, possible haemoconcentration as a result of psychosocial stress and/ or exercise-induced physiological response could further explain elevations in hsCRP, and unlikely pathological. Furthermore, endothelial activation was attenuated during isolation, suggesting that the diet and exercise program instated throughout the period improved endothelial function.

Matched MeSH terms: Hematologic Diseases
Fulltext Observational study on the current status of thalassaemia in Malaysia: a report from the Malaysian Thalassaemia Registry

Mohd Ibrahim H, Muda Z, Othman IS, Mohamed Unni MN, Teh KH, Thevarajah A, et al.

BMJ Open, 2020 06 29;10(6):e037974.
PMID: 32601117 DOI: 10.1136/bmjopen-2020-037974

OBJECTIVE: Thalassaemia 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.
DESIGN: Data were extracted from the Malaysian Thalassaemia Registry, a web-based system accessible to enrolled users through www.mytalasemia.net.my.
SETTING: The Malaysian Thalassaemia Registry data was recorded from reports obtained from 110 participating government and university hospitals in Malaysia.
PARTICIPANTS: The patients were those attending the 110 participating hospitals for thalassaemia treatment.
INTERVENTION: Data were collected from the Malaysian Thalassaemia Registry from 2007 until the fourth quarter of 2018.
PRIMARY OUTCOME MEASURE: 7984 out of 8681 patients with thalassaemia registered in the Malaysian Thalassaemia Registry were reported alive.
RESULTS: Majority 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.
CONCLUSION: Data 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.

Matched MeSH terms: Hematologic Diseases