OBJECTIVES: To elucidate the clinico-laboratory characteristics associated with pediatric systemic lupus erythematosus (pSLE) patients with higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score in a retrospective cohort of pSLE patients.
METHODS: A retrospective study involving 32 pSLE patients was conducted at Hospital Universiti Sains Malaysia, Kelantan, Malaysia between 2006 and 2017.
RESULTS: Within the group of 32 pSLE patients, 23 were girls and 9 were boys (3:1 ratio). The most common symptom was renal disorder (n=21; 65.6%) followed by malar rash (n=9; 28.1%), oral ulcers (n=7; 21.9%), prolonged fever (n=5; 15.6%) and arthritis (n=4; 12.5%). Antinuclear antibodies (ANA) were detected in all patients and 25 patients (78.1%) were positive for anti-double stranded DNA (anti-dsDNA) antibodies. Eighteen (56.3%) patients had active SLE (SLEDAI ≥6), and these patients were significantly associated with heavy pyuria (p=0.004), a high ANA concentration (1:160; p=0.040, 1:320; p=0.006), elevated ESR (p=0.006), low C3 levels (p=0.008), oral ulcers (p=0.010), heavy hematuria (p=0.017) and heavy proteinuria (p=0.017), lupus erythematosus (LE)-nonspecific lesion manifestations (p=0.019) and malar rash (p=0.044).
CONCLUSION: Pediatric systemic lupus erythematosus patients with higher SLEDAI score were most significantly associated with pyuria, high ANA titers, and elevated ESR.
Introduction: Ascariasis is a parasitic infection, which commonly affects immunocompromised patients. Most pa-tients remained asymptomatic during the early larval migration stage and respond well with conventional anti-hel-minthic drugs. Previous literature had reported symptomatic Ascaris infection mimicking bacterial pneumonia and the typical eosinophilia found in Loeffler syndrome was absent in patients on corticosteroids. Thus, a high index of suspicion for ascariasis is needed for immunosuppressed patients presented with infection. We present here a case of severe ascariasis infection in a systemic lupus erythematosus patient. Case description: A 16-year-old boy presented with fever, generalized maculopapular rash associated with neutropenia and thrombocytopenia. He was treated initially as Dengue Fever initially. However his symptoms did not resolve at even day 14 of admission. On further assessment, we were convinced he has SLE based upon presence of malar rash, oral ulcers, urinary protein-uria, persistent leucopenia, thrombocytopenia with low complements and ANA positive. He was promptly started on IV hydrocortisone. He showed a good progress in the first few days. On day 5 of admission, he coughed out a round worm which later identified as Ascarisis lumbricoides. He was started on Albendazole. Unfortunately he developed hemoptysis and respiratory compromisation where he required intubation. Post intubation he went into cardiac arrest, which required CPR. Following that event, his condition further deteriorated with multi organ failure. He succumbed to his illness three days later. Conclusion: Immunocompromised patients are prone to opportunistic infections including parasitic infections. we present here a case of ascariasis in an SLE patient who unfortunately succumbed to the illness. Due to the variable clinical symptoms that mimic other infections, screening for parasitic infections needs to be considered especially if the patients do not respond to antibiotics and routine treatments.
Natural products have demonstrated various activities beneficial to general health. Flaxseed (Linum usitatissimum) has been reported in many studies for its antimicrobial, antioxidant, and anti-inflammatory effects. Additionally, flaxseed extracts have skin wound healing activity and potential for treating oral ulcers.L. usitatissimum was extracted using 70% ethanol via soxhlet method and gas chromatography mass spectrum (GC-MS) was used to analyze the components of L. usitatissimum extract. The crude flaxseed oil were applied to human oral fibroblasts (HOrF), and 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide (MTT) assay was used to assess the cell viability after 24, 48 and 72 hours. Scratched HOrF cells were treated with crude flaxseed oil and healing was monitored per wound healing assay. GC-MS indicate that the major components present in L. usitatissimum oil extract are linolic, palmitic and oleic acid. L. usitatissimum crude oil extract showed high proliferation effect on HOrF cells at 24 and 48 hours, while the highest proliferation effect was recorded at 72 hours post-treatment. The wound healing assay results showed that healing activity of HOrF cells occurred as soon as 18 hours post-treatment when treated with L. usitatissimum crude oil extract. L. usitatissimum crude oil extract has proliferating and healing effects on HOrF cell line. Therefore, it can be considered as a potential promising oral wound healing agent.
The objective of the study was to attempt to verify the cause of self-reported oro-facial pain among 12-yearold children, objectively via a clinical examination. This is a descriptive, cross-sectional survey using a combination of self-reported questionnaire, face-to-face interview and clinical oral examination. The children were first asked to answer a self-filled questionnaire about their oro-facial pain experience in the past 4- weeks. In order to verify its cause, a clinical examination and an interview followed. Normative oral health status data was also collected. The sample was 1492 Malay schoolchildren with diverse socioeconomic background from the states of Johore, Kelantan and Sabah. The sample size for each state was calculated to give a sampling error of not more than 5 %. In each state, quota sampling was done to achieve a balanced distribution between gender and location. The data collected were normative status for caries, periodontal disease and traumatized teeth. Orofacial pain experience represented the subjective status for oral well-being. The cause of pain was confirmed through a clinical examination. The normative oral health status data implies a very low untreated disease and good oral health among the schoolchildren. However the subjective health status, as reflected by the prevalence of pain suggested that oro-facial pain and suffering was high (27.3%) with about 49% "of moderate and severe" intensity. The two main causes were caries and mouth ulcers. However in about onequarter of pain cases, diagnosis cannot be confirmed in the field survey setting. More than one-half of those with pain experienced disturbed sleep and study. It was concluded that overall oral health status and well-being can be better described if normative data is complemented with subjective data such as pain prevalence. The study shows that the majority (more than 75 %) of cases of subjective pain can be objectively verified in a field epidemiology survey setting. The reliability of the subjective data can be improved by a clinical examination as compared to unverified self-report. The study also confirms that the major source of oro-facial pain among the 12 year-olds were caries and mouth ulcers.
The hand, foot, and mouth disease (HFMD) outbreak occurred in the state of Johor, Malaysia in the months 0f October and November 2000 aber the HFMD outbreak started in Singapore in September 2000. The HFMD notification and investigation forms sent to the Epidemiology Unit of the Johor Health Department from district health offices and hospitals during the outbreak were analysed. Patients’ specimens were sent to the Institute for Medical Research, Kuala Lumpur and the Universiti Malaya Medical Centre, Kuala Lumpur for viral isolation. A total of 1,122 HFMD cases were notified during the outbreak period with 2 deaths giving 0.2% casefatality rate. From all the cases, 544 (48.5%) patients were admitted to the hospital while the rest were treated as outpatients. Majority (81.7%) of children with HFMD were aged below 5 years old with the commonest age group (46.9%) being between 1 to 2 years old. Sixty-eight patients had positive specimens for viral isolation belonging to enteroviruses namely Enterovirus 71 (43 cases), Coxsackievirus Type A16 (11), Echovirus 7 (3), and Coxsackievirus Type B (2); and non-enteroviruses namely Herpes Simplex Virus Type 1
(5), and Adenovirus (4). Fever and mouth ulcer were the two most common symptoms among HFMD children but vesicles on palms and soles were more common than rashes in children with positive enteroviral isolation.
Study site: University Malaya Medical Centre (UMMC)
A 3-year-old male child was presented with worsening abdominal pain, abdominal distension, lethargy, pallor and hepatosplenomegaly. The patient had multiple outpatient visits in the past and was treated with oral antibiotics, oral anthelmintic agents, albeit with minimal benefit. The patient also had non-neutropenic pyrexia spikes and oral ulcers. The patient was an adopted child; hence details about his biological parents' previous history were unclear. Differential diagnosis of Chronic Myelomonocytic Leukemia (CMML), Juvenile Myelomonocytic Leukemia (JMML), Gaucher's disease, Thalassemia and discrete pancreatic pathology was considered. Hemoglobin electrophoresis was indicative of thalassemia. Also, molecular detection method by polymerase chain reaction confirms a concurrent infection with Plasmodium knowlesi malaria. The BCR-ABL fusion gene was found to be negative. Correlating with peripheral monocytosis, bone marrow aspiration and trephine biopsy with blasts only 3-4% and hepatosplenomegaly, a diagnosis of JMML was established. We present a rare phenomenon with an overlap of signs and symptoms between JMML, underlying thalassemia, and Plasmodium knowlesi, posing a diagnostic challenge to physicians.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease afflicting multiple organs. Lupus nephritis (LN) is a serious complication of SLE and remains a major cause of mortality and morbidity. Curative therapy remains unavailable as etiology from genetic and environmental factors is still unclear. The present study was conducted to elucidate the link between HLA-DRB1 gene polymorphisms with SLE and LN through clinical and laboratory/biological presentations in a population of Malaysian Malay females with SLE. A total of 100 Malay female SLE patients inclusive of 70 SLE patients without LN and 30 patients with LN were included in this study. HLA-DRB1 allele examination in SLE patients was performed using PCR-SSO, and the alleles' frequencies were compared with 951 publicly available datasets representing Malay healthy controls in Malaysia. Cytokines and free radical levels were detected by ELISA and bead-based multiplexed Luminex assays. The association between HLA-DRB1 alleles with clinical and serological manifestations and immune mediators was analyzed using different statistical approaches whenever applicable. Our study showed that HLA-DRB1*0405, HLA-DRB1*1502, and HLA-DRB1*1602 were associated with the increased risk of SLE while HLA-DRB1*1201 and HLADRB1*1202 alleles were associated with a lower risk of SLE development. Furthermore, HLA-DRB1*04 showed significant association to LN and arthritis while HLA-DRB1*15 was significantly associated with oral ulcer in Malay SLE patients. Association analysis of HLA-DRB1*04 with clinical and biological factors revealed that HLA-DRB1*04 was significantly associated with Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores, anti-nuclear antibody (ANA), C-reactive protein (CRP) in the blood, and total protein in the urine. SLE carriers with the HLA-DRB1*04 allele were significantly correlated to the increased levels of cytokines (IFN-y, GM-CSF, IL-17F, IL-18, IL-21, and VEGF) and were significantly showing negative correlation to IL-5 and free radicals (LPO and catalase enzyme) levels compared to SLE carriers without HLA-DRB1*04 allele. The results suggested that disease severity in SLE may be determined by HLA-DRB1 alleles. The risk of HLA-DRB1*04 allele with LN was supported by the demonstration of an intense inflammatory response in Malay SLE patients in Malaysia. More studies inclusive of a larger and multiple SLE cohorts in the future are warranted to validate these findings.