Diseases In Southeast Asia Research Articles

Lumpy skin disease: A newly emerging disease in Southeast Asia.

Lumpy skin disease (LSD) is caused by LSD virus (LSDV). This virus has been classified in the genus Capripoxvirus, family Poxviridae which generally affects large ruminants, especially cattle and domestic water buffalo. The first outbreak of LSD was found in 1929 in Zambia, then spreading throughout Africa and with an ongoing expanding distribution to Asia and Europe. In 2020, LSD was found from Southeast Asia in Vietnam and Myanmar before reaching Thailand and Laos in 2021. Therefore, LSD is a newly emerging disease that occurs in Southeast Asia and needs more research about pathology, transmission, diagnosis, distribution, prevention, and control. The results from this review show the nature of LSD, distribution, and epidemic maps which are helpful for further information on the control and prevention of LSD.

Molecular Mechanism and Role of Japanese Encephalitis Virus Infection in Central Nervous System-Mediated Diseases.

The Japanese encephalitis virus (JEV) is the most common cause of neurodegenerative disease in Southeast Asia and the Western Pacific region; approximately 1.15 billion people are at risk, and thousands suffer from permanent neurological disorders across Asian countries, with 10-15 thousand people dying each year. JEV crosses the blood-brain barrier (BBB) and forms a complex with receptors on the surface of neurons. GRP78, Src, TLR7, caveolin-1, and dopamine receptor D2 are involved in JEV binding and entry into the neurons, and these receptors also play a role in carcinogenic activity in cells. JEV binds to GRP78, a member of the HSP70 overexpressed on malignant cells to enter neurons, indicating a higher chance of JEV infection in cancer patients. However, JEV enters human brain microvascular endothelial cells via an endocytic pathway mediated by caveolae and the ezrin protein and also targets dopamine-rich areas for infection of the midbrain via altering dopamine levels. In addition, JEV complexed with CLEC5A receptor of macrophage cells is involved in the breakdown of the BBB and central nervous system (CNS) inflammation. CLEC5A-mediated infection is also responsible for the influx of cytokines into the CNS. In this review, we discuss the neuronal and macrophage surface receptors involved in neuronal death.

S3214 Two Cases of Klebsiella pneumoniae Liver Abscess With Metastatic Infection

Introduction: Klebsiella pneumoniae (K. pneumoniae) is a gram-negative organism that is a known cause of pyogenic liver abscess (PLA). When K. pneumoniae causes PLA in the absence of hepatobiliary disease it is defined as invasive liver abscess syndrome (ILAS), and is commonly seen in individuals from Southeast Asia who have diabetes mellitus, cholelithiasis, or steatosis. ILAS can sometimes include metastatic spread that most commonly results in endophthalmitis or meningitis. Although K. pneumonia PLA is an endemic disease in Southeast Asia, it is now seen throughout the world and should elicit greater awareness as one of the common causes of PLA in the United States. Case Description/Methods: We present two cases of ILAS with metastatic spread causing endophthalmitis in male patients. The first case highlights known epidemiological and medical risk factors of ILAS with a 62-year-old from Southeast Asia who was diagnosed with poorly controlled type 2 diabetes mellitus (DM). The second case highlights an incidence of ILAS without any epidemiological or medical risk factors in a 36-year-old from the United States with no medical history. Both patients required interventional radiology for abscess drainage and intravenous ceftriaxone with subsequent clinical improvement and outpatient follow-ups in primary care and ophthalmology (Figure). Discussion: Liver abscesses most commonly occur with hepatobiliary disease, ILAS from K. pneumoniae is a potentially life-threatening infection and associated with significant morbidity and mortality that can occur without any hepatobiliary disease. ILAS was once commonly thought to occur typically in males and isolated in Southeast Asia, but are now seen in all sexes across the world and becoming increasingly common in the US. DM tends to be associated with K. pneumonia PLA, and the disease process also tends to be more invasive with metastatic spread to other organs, thereby leading to sepsis. Endophthalmitis is the most common infectious sequelae and is specifically associated with more virulent strains of K. pneumonia. ILAS should be part of the differential diagnosis of liver mass in the setting of sepsis, and due to K. pneumoniae metastasizing to other organ systems, early drainage and antibiotic administration is essential to decrease morbidity and mortality. A higher index of suspicion should be held for Southeast Asian diabetic males, the incidence of ILAS in all demographics is increasing, which should elicit greater awareness of risk factors, pathogenesis, and management.Figure 1.: Contrast-enhanced computed tomography scan of the abdomen in axial view demonstrating complex lesion in the left hepatic lobe (Case 1). Figure 2: Inflammation of the episclera indicative of episcleritis (Case 2).

Preparation and Optimization of Peppermint (Mentha pipertia) Essential Oil Nanoemulsion with Effective Herbal Larvicidal, Pupicidal, and Ovicidal Activity against Anopheles stephensi.

The Plasmodium parasite is transmitted directly to humans through the Anopheles mosquito bite and causes vector-borne malaria, leading to the transmission of the disease in Southeast Asia, including India. The problem of persistent toxicity, along with the growing incidence of insect resistance, has led to the use of green pesticides to control the spread of the disease in a cost-effective and environment-friendly manner. Based on this objective, this work investigated the larvicidal, pupicidal, and ovicidal activity of Mentha pipertia using a natural nanoemulsion technique. GC-MS characterized essential oils of Mentha pipertia leaves were formulated as a nanoemulsion for herbal larvicidal, pupicidal, and ovicidal activities. Size of the nanoemulsion was analyzed by photon correlation spectroscopy. The herbal activities against Anopheles Stephensi of nanoemulsion were evaluated in terms of the lethal concentration for 50% (LC50) and 90% (LC90) to prove low cost, pollution free active effective formulation. Chiral, keto, and alcohol groups are obtained from Mentha pipertia leaves' essential oil, and the nanoemulsions have demonstrated good results in the larvicidal probit analysis, with values of LC50=09.67 ppm and LC90=20.60 ppm. Activity results of the most stable nano formulation with 9.89 nm size showed a significant increase when compared to the bulk. The nanoemulsion of Mentha pipertia leaves can be a promising eco-friendly widely available, low-cost herbicide against the Anopheles mosquito.

Imaging manifestations of pulmonary melioidosis: A case series

Melioidosis is an endemic disease in Southeast Asia and Oceania caused by the gram-negative bacillus Burkholderia pseudomallei. We studied 15 adult patients from Colombia with microbiologically diagnosed pulmonary melioidosis. We reviewed 15 chest X-rays and 10 chest computed tomography (CT) studies. Of the 15 patients, 87% met the criteria for acute infection and 13% met the criteria for chronic infection. The most common findings on chest X-rays were consolidation (86%), nodules (26%), and cavitation (20%). On CT studies, consolidation and nodules were observed in 90% of cases; the areas of consolidation were predominantly located in the basal and central zones in 60%. Areas of cavitation were observed in 50%, pleural effusion in 60%, and mediastinal lymph nodes in 30%. In patients with acute pulmonary melioidosis (n=8), the findings observed were nodules (100%), mixed pattern with nodules and consolidation (87%), pleural effusion (88%), and mediastinal lymph nodes (25%). The two patients with chronic pulmonary melioidosis both had cavitation. Acute lung infection with B. Pseudomallei has radiologic manifestations similar to those of pneumonia due to other causes. In areas where the disease is endemic, it is essential to include acute melioidosis in the differential diagnosis of pulmonary nodules and chronic melioidosis in the differential diagnosis of cavitated chronic lung lesions.

Red blood cell alloimmunization and other transfusion-related complications in patients with transfusion-dependent thalassemia: A multi-center study in Thailand.

Thalassemia is a common genetic disease in Southeast Asia. Red blood cell (RBC) transfusion is an essential treatment for severe forms of thalassemia. We performed a study to demonstrate RBC alloimmunization and other transfusion-related complications in patients with transfusion-dependent thalassemia (TDT). A multi-center web-based registry of TDT was conducted in eight medical centers across Thailand. Thalassemia information, transfusion therapy, and transfusion-related complications were collected. Factors associated with each complication were demonstrated using the logistic regression analysis. Of 1000 patients recruited for the study, 449 were males (44.9%). The mean age was 23.9 ± 15.4 years. The majority of patients, 738 (73.8%) had hemoglobin E/beta-thalassemia. In the study, 421 transfusion-related complications were reported from 357 patients (35.7%). Alloimmunization was the most common complication which was found in 156 patients (15.6%) with 284 positive antibody tests. The most frequent antibodies against RBC were anti-E (80/284, 28.2%) followed by anti-Mia (45/284, 15.8%) and anti-c (32/284, 11.3%). Age ≥3 years at initial blood transfusion, splenomegaly, higher frequencies, and volumes of transfusion were significant factors associated with alloimmunization. None of the patients had to terminate blood transfusion due to multiple alloantibodies. Other commonly seen complications were allergic reactions (130, 13.0%), autoimmune hemolytic anemia (70, 7.0%) and febrile non-hemolytic transfusion reaction (54, 5.4%). Transfusion-related complications, especially alloimmunization, were common among Thai patients with TDT. Extended RBC antigen-matching for the Rh system and Mia should be implemented to prevent the development of alloantibodies in multi-transfused patients.

Study on HPLC Fingerprint, Network Pharmacology, and Antifungal Activity of Rumex japonicus Houtt.

Rumex japonicus Houtt (R. japonicus) is used mainly to treat various skin diseases in Southeast Asia. However, there are few studies on its quality evaluation methods and antifungal activity. To establish the quality control criteria for the effective parts from R. japonicus against psoriasis. High-performance liquid chromatography (HPLC) was established for its fingerprint, and the similarity evaluation, cluster analysis (CA) and principal component analysis (PCA) were used to reveal the differences of those fingerprints among the tested R. japonicus. Network pharmacology analyzed the relationship between the components and psoriasis, revealing the potential targets of R. japonicus. Oxford cup anti-C. albicans experiment was used to verify the antifungal activity of R. japonicus. HPLC was developed for the R. japonicus fingerprint by optimizing for 10 batches of quinquennial R. japonicus from different habitats; the 18 common peaks were identified with 10 characteristic peaks such as rutin, quercetin, aloe-emodin, nepodin, emodin, musizin-8-O-β-D-glucoside, chrysophanol, emodin-8-O-β-D-glucopyranoside, chrysophanol-8-O-β-D-glucopyranoside, and aloin, respectively. The network pharmacology-based analysis showed a high correlation between R. japonicus and psoriasis, revealing the potential targets of R. japonicus. The oxford cup anti-Candida albicans experiment displayed a significant activity response to emodin-8-O-β-D-glucopyranoside and the ethyl acetate fraction of R. japonicus acidic aqueous extract. A new and optimized HPLC method was created, and the research provides an experimental basis for the development of effective drugs related to C. albicans. The fingerprint of R. japonicus was organically combined with network pharmacology to further clarify its criteria for quality control.

Frequency of consanguineous marriage among the thalassaemia major patients in Bangabandhu Sheikh Mujib Medical University

Thalassaemia is a commonly occurring hereditary disorder. There is a high prevalence of thalassaemia disease in South-East Asia as well as Bangladesh. It is an autosomal recessive disorder, so consanguineous marriage is a very important factor for this disease. Mutated beta globin gene of haemoglobin from both parents is responsible for this disease to occur. But when the number of thalassaemia carriers is miserably high, then only avoidance of consanguineous marriage can’t prevent the birth of children with thalassaemia major. So, this cross sectional study was conducted among 120 diagnosed (by haemoglobin electrophoresis or high performance liquid chromatography) thalassaemia major patients in the Department of Haematology, Bangabandhu Sheikh Mujib Medical University, Dhaka from July 2019 to May 2020. History from each patient was taken and blood samples were collected from their parents to confirm carrier state by haemoglobin electrophoresis. Blood samples were also collected from patients and their parents toperform complete blood count and peripheral Blood Film. This study showed the mean age of the participants was 15±9.34 and 73 (60.8%) patients were male and 47 (39.2%) were female. About, 71% of the study population lived in urban area, 81 (67%) patients were transfusion dependent thalassaemia patients. 15% thalas- saemia major patients had the history of consanguinity of their parents. Among the parents of the thalassaemia major patients, 62.5% were Beta thalassaemia trait (heterozygous state), 25.83% parents were Hb E trait ( heterozygous state), 7.08% parents were suffering from Beta Thalassaemia Major(homozygous state) and 11 (4.58%) parents were suffering from Hb E/Beta Thalassaemia which is a compound heterozygous state. Though consanguinity is a very influential factor, but the most important causative factor is the presence of high number of thalassaemia carrier in the population. So, it’s a burning issue for the nation to perform a routine carrier screening for all rather than focusing on consanguinity only, to lessen the burden of thalassaemia disease in Bangladesh. BSMMU J 2022; 15(1): 25-28

A rare case of melioidosis presenting as myositis in Sri Lanka

BackgroundMelioidosis caused by Burkholderia pseudomallei is an emerging infection in Sri Lanka with a high case fatality rate. The disease usually manifests as pneumonia, however multisystem involvement is common. Myositis is an extremely rare occurrence and this is the only documented case where the initial presentation of melioidosis has been myositis and later complicated to myonecrosis.Case presentationA 45-year-old gentleman with pre-existing diabetes presented with a tender, right thigh lump for 1 week duration without any history of trauma or infection. Investigations revealed neutrophil leukocytosis, high erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels whilst ultrasonography showed focal myositis of right quadriceps. The patient went into sepsis amidst antibacterial treatment which warranted urgent surgery. At surgery, a large intramuscular abscess with myonecrosis was observed within vastus medialis which was completely drained and pus was taken for culture which eventually isolated Burkholderia pseudomallei. Melioidosis was diagnosed and intravenous meropenem was prescribed for 3 weeks. Following complete recovery, the patient was discharged on doxycycline and trimethoprim sulfamethoxazole for 3 months.ConclusionsMelioidosis, an endemic disease in south east Asia and northern Australia, is an emerging infection in Sri Lanka. Myositis is a rare presentation of the disease that can lead to myonecrosis and abscess formation which can cause rapid disease escalation and sepsis. Early surgical intervention may be life-saving in such cases where antibiotic therapy alone may not suffice.

Draft Genome Sequence of Ralstonia syzygii subsp. celebesensis from Indonesia, the Causal Agent of Blood Disease of Banana.

Draft Genome Sequence of Ralstonia syzygii subsp. celebesensis from Indonesia, the Causal Agent of Blood Disease of Banana.

Sociocultural Factors Influencing Human Streptococcus suis Disease in Southeast Asia.

The public health systems of Southeast Asian countries are financially challenged by a comparatively higher incidence of human S. suis infections than other geographical areas. Efforts to improve practices in production settings, including improved meat inspection regulations, prevention of the slaughtering of non-healthy pigs, and enhanced hygiene practices at processing facilities, along with improvements in the pork supply chain, all appear promising for reducing food cross-contamination with S. suis. However, opportunities for intervention at the societal level are also needed to effect changes, as population behaviors such as the consumption of raw pork, blood, and offal products are important contributors to the increased incidence of human S. suis disease in Southeast Asia. A plethora of factors are associated with the consumption of these high-risk dishes, including traditional culture and knowledge, shared beliefs, socio-economic level, and personal attitudes associated with gender and/or marital status. Education and intervention in behavioral attitudes that are sensible to cultural practices and traditions may provide additional means to reduce the burden of S. suis human disease in Southeast Asia.

First report of Rickettsia asembonensis in small ruminants.

Rickettsioses is a group of emerging infectious diseases in Southeast Asia caused by Gram-negative obligate intracellular bacteria in the Rickettsiae tribe. However, there is limited information regarding the vertebrate hosts of Rickettsia spp. in this region. This study aims to detect and identify Rickettsia agents present in wildlife and domesticated animals in Malaysia using Polymerase Chain Reaction (PCR) and sequencing of citrate synthase gene (gltA), followed by genotyping and phylogenetic analysis. Rickettsia sp. was detected in 2 (0.67%) of 300 wildlife and domesticated animal blood samples. The positive samples were derived from a goat (5.56% of 18) and a sheep (2.22% of 45). Both sequences demonstrated 99.64% sequence similarity to Rickettsia asembonensis, a species that is known to infect humans and macaques. This study reported for the first time the detection of R. asembonensis in sheep and goats in Malaysian farms, suggesting this species may be adapting to a wider range of animals, specifically farm animals. Therefore, this bacterium may pose a zoonotic threat to the local community particularly to the farmworkers or animal handlers. The low infection rate of this pathogen across different animals highlighted the need of continuous surveillance of emerging and reemerging pathogens among animal populations.

Young Psychiatrist Award

Background and Objective:Thalassemia is one of the most prevalent hereditary diseases in south east Asia. Thalassemia is a concern not only for the children’s physical health but also mental health of themselves and their caregiver. This study assessed the presence of psychiatric morbidities and global functioning among thalassaemic children and the presence of psychiatric problems and caregiver burden in their caretakers.Materials and Methods:In this observational cross-sectional study, a total of 46 children with thalassemia dependent transfusion (28 boys and 18 girls) with mean age of 8.83 yrs ±2.70 years were included and they were assessed for psychiatric morbidity and global functioning. 46 parents ( 12 fathers and 34 mothers ) were assessed for psychiatric morbidity and care giver burden faced by them. All the parents completed 3 different questionnaires to assess their knowledge about psycho-social functioning(PSC-35) of their children and level of burden (CBS) All participants consented to undergo screening and voluntarily participated in the screening tests.Results.More than 32 children had some psychosocial problem on screening by PSC-35 56.52 %, 23.91% and 21.73% of parents reported high, moderate and low level of burden, as assessed by Caregiver Burden Scale. 65.3 % children and 62.7 % parents were diagnosed with psychiatric problems.Conclusion:Thalassemia affects not only the persons with the disorder but also their caregivers in several aspects, including their psychosocial well-being.

How to dampen the surge of non-communicable diseases in Southeast Asia: insights from a systematic review and meta-analysis.

Non-communicable diseases (NCDs), such as diabetes, cancer, cardiovascular diseases and chronic respiratory diseases, have overtaken infectious diseases as the number one cause of death worldwide. The rise of these diseases is especially grave in Southeast Asia, where existing research however falls short on offering guidance on how policy can best prevent and control NCDs in the region. Additionally, low- and middle-income countries in Southeast Asia cannot directly incorporate lessons drawn from interventions in richer countries, since health system capacities and human and financial resources are thoroughly different. Preventive interventions, thus, need to correspond to local capacities and require contextual solutions. In this article, we provide a systematic review of a wide scope of NCD interventions conducted in Southeast Asia to inform about existing intervention designs and to derive sound evidence of their effectiveness. Our literature search results in 51 studies from five Southeast Asian countries from which we can extract 204 estimates. We sort the studies into six intervention categories and analyse them with respect to 23 different health and behavioural outcomes. While we find positive and significant average effects across all six types of interventions, we also document evidence of substantial publication bias. Using a meta-regression approach in which we correct for the publication bias, we instead fail to confirm positive average effects for some interventions. Especially dietary and physical activity interventions fail to achieve improvements in analysed health outcomes, while programs focusing on smoking cessation, on the take-up of preventive screening activities or educating patients on how to cope with NCDs achieve sizeable effects. We also present evidence that the size of the effect differs with the participants’ characteristics as well as with design features of the intervention. For local policymakers, the results provide important knowledge on how to address the increasing NCD burden in the coming years.

Geographic Expansion of Banana Blood Disease in Southeast Asia.

Blood disease in bananas caused by Ralstonia syzygii subsp. celebesensis is a bacterial wilt causing significant crop losses in Indonesia and Malaysia. Disease symptoms include wilting of the plant and red-brown vascular staining, internal rot, and discoloration of green banana fruit. There is no known varietal resistance to this disease in the Musa genus, although variation in susceptibility has been observed, with the popular Indonesian cooking banana variety Kepok being highly susceptible. This study established the current geographic distribution of Blood disease in Indonesia and confirmed the pathogenicity of isolates by Koch's postulates. The long-distance distribution of the disease followed an arbitrary pattern indicative of human-assisted movement of infected banana materials. In contrast, local or short-distance spread radiated from a single infection source, indicative of dispersal by insects and possibly contaminated tools, water, or soil. The rapid expansion of its geographical range makes Blood disease an emerging threat to banana production in Southeast Asia and beyond.

Microbial community occupying cocoa branch and petiole with vascular streak dieback disease symptoms

Vascular Streak Dieback (VSD) disease caused by Ceratobasidium theobromae is the most severe emerging cocoa disease in Southeast Asia. Once the tree is infected, tree defoliation will occur as leaf senescence followed by leaf falls. VSD to have associated with endophytes. However, a fundamental question is whether a similar microbe inhabiting vascular tissue of branch and petiole is entire or patchy? The aim of research was to track and quantify microbial diversity associated with VSD symptoms occupying branches and petiole throughout the stem in different clones. The study focused on VSD symptoms expressing in local (Sulawesi 02), susceptible (RCC 70), and resistant (MCC 02) clones to VSD disease. Sample collection was conducted in Lili Riaja, Soppeng District. The 30 cm-branch with the attached petiole of interest was cut into small pieces 1 cm and transferred into water agar medium before submersion into 2.5% NaOCl for 3 minutes, 70% alcohol for 2 minutes and sterilized water for 1 minute. Morphological characterization followed by microscope check was determined to microbial genera. Eight different fungal communities occupying branch and/or petiole consisted of Cladosporium sp., Fusarium sp., Colletotrichum sp., Penicillium sp., Aspergillus sp., Moniliophthora sp., Lasiodiplodia sp., Curvularia sp. and one Oomycete Phytophthora palmivora. Among occupants in branch and petiole, Fusarium sp. and Colletotrichum sp. were the most frequent. Only one Oomycete Phytophthora palmivora occupying clone samples was found. The most diverse microbes were found to associate with Sulawesi 02 and RCC 70 clones, while fewer microbes were detected with MCC 02 clone.

Safety and immunogenicity of inactivated Japanese encephalitis vaccine derived from Vero cell. Phase III, double-blind, randomized, control trial (JECEVAX, VABIOTECH, Vietnam)

Japanese encephalitis (JE) is one of the most important infectious disease in Southeast Asia and the Pacifc region and more than 3 billion people are living in endemic areas. The annual incidence is estimated to be 60,000 cases/year of which 15,000 deaths, 50% of the people survive with neurological sequelae. Since 1997, Vietnam has produced and used mouse brain - derived (MB) inactivated Japanese encephalitis vaccine (JEVAX®) for children 1 - 5 years of age under the Expanded programme on immunization (EPI). In 2006, VABIOTECH started the research and development of vero cell-derived inactivated JE vaccine (JECEVAX). The clinical trial phases I, II & IIB were conducted successfully and the phase III was completed in January 2019. The study participants included 655 healthy infants aged 9 - 24 months were immunized with JECEVAX testing vaccine, and JEVAX® control vaccine. The results showed that both vaccines were safe with good tolerability in children. The seroconversion rates of JECEVAX (n = 547) were 99.6 - 100%, compared with JEVAX® (n =108) were 99 - 100%. The GMT titer of JECEVAX and JEVAX® after the second dose was 2.07log and 2.02log after the third dose, the neutralizing antibody (PRNT50) increased to 3.04log and 3.19log respectively. The registration dossier with the MOH of Vietnam is being fnalized before scaling up the production to prevent JE disease with JECEVAX high qualifed second generation vaccine.

Manifestaciones radiológicas de la melioidosis pulmonar. Serie de casos

Melioidosis is an endemic disease in Southeast Asia and Oceania caused by the gram-negative bacillus Burkholderia pseudomallei. We studied 15 adult patients from Colombia with microbiologically diagnosed pulmonary melioidosis. We reviewed 15 chest X-rays and 10 chest computed tomography (CT) studies. Of the 15 patients, 87% met the criteria for acute infection and 13% met the criteria for chronic infection. The most common findings on chest X-rays were consolidation (86%), nodules (26%), and cavitation (20%). On CT studies, consolidation and nodules were observed in 90% of cases; the areas of consolidation were predominantly located in the basal and central zones in 60%. Areas of cavitation were observed in 50%, pleural effusion in 60%, and mediastinal lymph nodes in 30%. In patients with acute pulmonary melioidosis (n=8), the findings observed were nodules (100%), mixed pattern with nodules and consolidation (87%), pleural effusion (88%), and mediastinal lymph nodes (25%). The two patients with chronic pulmonary melioidosis both had cavitation. Acute lung infection with B. Pseudomallei has radiologic manifestations similar to those of pneumonia due to other causes. In areas where the disease is endemic, it is essential to include acute melioidosis in the differential diagnosis of pulmonary nodules and chronic melioidosis in the differential diagnosis of cavitated chronic lung lesions.

Clinical and demographic pattern of chronic granulomatous disease (CGD) from a multicenter perspective: Malaysia\u2019s experience over 26 years

BackgroundA retrospective review of clinical manifestations and demographic pattern of patients diagnosed as chronic granulomatous disease (CGD) from 7 hospitals in Malaysia. An analysis of the available database would establish clinical characteristics, diagnoses and outcome including microbiologic pattern. Studying the demography allows us to document the occurrence of CGD amongst multiethnic groups and its geographical distribution for Malaysia.MethodsData from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.ResultsTwenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGD patients with C. violaceum infection displayed CD4 + (T helper cells) lymphopenia.ConclusionThis study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGD patients with C. violaceum infection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.

Behcet’s Disease in Southeast Asia. Clinical Features, Genetic Study, and Review of Recent Treatment

Behçet’s disease (BD) is a chronic systemic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and ocular inflammations. It also can affect other organs such as blood vessels, nervous, gastrointestinal, and articular systems. The disease has a worldwide distribution, but is more prevalent in countries along the Silk Road. Genetic study has shown that the disease is associated with the presence of human leukocyte antigen (HLA)-B*51/B%51:01. BD is considered an uncommon disease in Southeast Asia, and only a few case series have been reported. This article reviews the clinical manifestations and genetic association of BD in Southeast Asian countries and compares them with those in East Asia, South Asia, and the Middle East. It also updated the treatment of BD, particularly the use of biologic agents.