Displaying publications 1 - 20 of 25 in total

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  1. Suhaini SA, Azidin AH, Cheah CS, Lee Wei Li W, Khoo MS, Ismail NAS, et al.
    Medicina (Kaunas), 2022 Dec 12;58(12).
    PMID: 36557034 DOI: 10.3390/medicina58121833
    Background and Objectives: Kawasaki Disease (KD) incidence has been on the rise globally throughout the years, particularly in the Asia Pacific region. KD can be diagnosed based on several clinical criteria. Due to its systemic inflammatory nature, multi-organ involvement has been observed, making the diagnosis of KD more challenging. Notably, several studies have reported KD patients presenting with hepatobiliary abnormalities. Nonetheless, comprehensive data regarding the hepatobiliary manifestations of KD are limited in Malaysia, justifying a more in-depth study of the disease in this country. Thus, in this article, we aim to discuss KD patients in Malaysia with hepatobiliary manifestations. Materials and Methods: A total of six KD patients with hepatobiliary findings who presented at Hospital Canselor Tuanku Muhriz (HCTM) from 2004 to 2021 were selected and included. Variables including the initial presenting signs and symptoms, clinical progress, laboratory investigations such as liver function test (LFT), and ultrasound findings of hepatobiliary system were reviewed and analyzed. Results: Out of these six KD patients, there were two patients complicated with hepatitis and one patient with gallbladder hydrops. Different clinical features including jaundice (n = 3) and hepatomegaly (n = 4) were also observed. All patients received both aspirin and intravenous immunoglobulin (IVIG) as their first-line treatment and all of them responded well to IVIG. The majority of them (n = 5) had a complete recovery and did not have any cardiovascular and hepatobiliary sequelae. Conclusions: Despite KD mostly being diagnosed with the classical clinical criteria, patients with atypical presentations should always alert physicians of KD as one of the possible differential diagnoses. This study discovered that hepatobiliary manifestations in KD patients were not uncommon. More awareness on the epidemiology, diagnosis, and management of KD patients with hepatobiliary manifestations are required to allow for the initiation of prompt treatment, thus preventing further complications.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  2. Lee VWM, Khoo TB, Teh CM, Heng HS, Li L, Yusof YLM, et al.
    Dev Med Child Neurol, 2023 Sep;65(9):1256-1263.
    PMID: 36748407 DOI: 10.1111/dmcn.15536
    This case series compared clinical variables and various combinations of immunotherapy received with outcomes of patients with severe acute necrotizing encephalopathy (ANE). We performed a retrospective review of clinical variables, immunotherapy received, and outcomes (based on the modified Rankin Scale) in Malaysia between February 2019 and January 2020. Twenty-seven children (12 male), aged 7 months to 14 years (mean 4 years) at diagnosis were included. Of these, 23 had an ANE severity score of 5 to 9 out of 9 (high risk). Eleven patients received tocilizumab (four in combination with methylprednisolone [MTP], seven with MTP + intravenous immunoglobulin [IVIG]) and 16 did not (two received MTP alone, 14 received MTP + IVIG). Nine died. Among the survivors, six had good outcomes (modified Rankin Score 0-2) at 6 months follow-up. All patients who received tocilizumab in combination with MTP + IVIG survived. Twenty children received first immunotherapy within 48 hours of admission. No significant association was found between the timing of first immunotherapy with outcomes. Those with brainstem dysfunction (p = 0.016) were observed to have poorer outcomes. This study showed a trend towards better survival when those with severe ANE were treated with tocilizumab in combination with MTP + IVIG. However, larger studies will be needed to determine the effect of this regime on the long-term outcomes.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  3. Mat Bah MN, Alias EY, Razak H, Sapian MH, Foo FH, Abdullah N
    Eur J Pediatr, 2021 Aug;180(8):2599-2606.
    PMID: 34086103 DOI: 10.1007/s00431-021-04135-7
    Data on Kawasaki disease from tropical countries are scarce. Hence, this population-based study aims to determine the epidemiology, clinical characteristics, and outcome of Kawasaki disease in children enrolled in the Kawasaki disease registry between 2006 and 2019 in Southern Malaysia. Diagnosis of Kawasaki disease was made using standard criteria. Primary outcome measure was a coronary artery aneurysm. Multivariable logistic regression was used to analyze the associated risk factors for coronary artery aneurysm. There were 661 Kawasaki disease, with 27% incomplete and 11% atypical presentations. Male-to-female ratio was 2:1, and median age of diagnosis was 1.4 years (interquartile range 9 to 32 months). Incidence in children of less than 5 years was 14.8 (95% confidence interval [CI]: 13.6 to 16.0) per 100,000 population, higher in males (19/100,000) and Chinese (22/100,000), with a gradual increase from 5.7/100,000 in 2006 to 19.6/100,000 in 2019, p < 0.001. Incidence in children between 5 and 9 years old was 1.3 (95% CI: 0.9 to 1.6) per 100,000 population and remained stable over time. There was a seasonal pattern with peak incidence during the rainy season. Out of 625 intravenous immunoglobulins (IVIG)-treated Kawasaki disease, 7.4% were resistant, and 9% had coronary artery aneurysms. Atypical presentation, male sex, late diagnosis, and IVIG resistance were independent risk factors for coronary artery aneurysms.Conclusions: Despite the tropical climate, Kawasaki disease epidemiology is similar to non-tropic regions with seasonal patterns and a rising incidence. Atypical presentation, male sex, late diagnosis, and IVIG resistance were significantly associated with coronary artery aneurysms. What is Known: • Kawasaki disease predominantly occurs in males, children less than 5 years old, and the Asian population. • Male sex, late diagnosis, incomplete Kawasaki disease, and IVIG resistance were associated with coronary artery aneurysms. What is New: • In multi-ethnic Asian countries such as Malaysia, ethnic Chinese have a higher prevalence of Kawasaki disease compared to other ethnicities. • Kawasaki disease with atypical presentation can occur in both complete and incomplete Kawasaki disease and is significantly associated with a coronary artery aneurysm.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  4. Lee JL, Mohamed Shah N, Makmor-Bakry M, Islahudin FH, Alias H, Noh LM, et al.
    J Clin Immunol, 2020 07;40(5):682-698.
    PMID: 32417999 DOI: 10.1007/s10875-020-00788-5
    PURPOSE: We conducted a systematic review and meta-regression analysis to evaluate the impact of increasing immunoglobulin G (IgG) trough levels on the clinical outcomes in patients with PID receiving intravenous immunoglobulin G (IVIG) treatment.

    METHODS: Systematic search was conducted in PubMed and Cochrane. Other relevant articles were searched by reviewing the references of the reviewed article. All clinical trials with documented IgG trough levels and clinical outcome of interest in patients receiving IVIG treatment were eligible to be included in this review. Meta-regression analysis was conducted using Comprehensive Meta-analysis Software. Additional sensitivity analyses were undertaken to evaluate the robustness of the overall results.

    RESULTS: Twenty-eight clinical studies with 1218 patients reported from year 2001 to 2018 were included. The mean IVIG dose used ranges from 387 to 560 mg/kg every 3 to 4 weekly, and mean IgG trough obtained ranges from 660 to 1280 mg/dL. Random-effects meta-regression slope shows that IgG trough level increases significantly by 73 mg/dL with every increase of 100 mg/kg dose of IVIG (p 

    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  5. Shahrizaila N, Yuki N
    Expert Opin Pharmacother, 2011 Jul;12(10):1551-60.
    PMID: 21473704 DOI: 10.1517/14656566.2011.564160
    Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paralysis and, despite treatment, there continues to be an associated mortality and severe disability ranging from 9 to 17%. This article reviews the rationale behind the existing immunotherapy in GBS and discusses the future direction that work in this area should follow.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  6. Lim KS, Cheong KL, Tan CT
    Lupus, 2010 May;19(6):748-52.
    PMID: 20133346 DOI: 10.1177/0961203309351539
    A 13-year-old girl with a known diagnosis of systemic lupus erythematosus presented with seizures and psychosis. An electroencephalogram (EEG) revealed continuous, non-evolving periodic lateralized epileptiform discharges (PLEDs) in the left temporal region, which did not resolve with benzodiazepine. A magnetic resonance imaging (MRI) brain scan demonstrated a focal hyperintensity in the left medial temporal and left occipital lobes, left thalamus and bilateral cerebellar white matter, with evidence of vasculitis in the magnetic resonance angiography. Intravenous immunoglobulin was given because of failed steroid therapy, which resulted in a full resolution of clinical, EEG and MRI abnormalities. Lupus cerebritis should be considered as a possible aetiology in PLEDs, and immunoglobulin can be effective in neuropsychiatric lupus.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  7. Kanaheswari Y, Baizura J, Zulfiqar A
    Med J Malaysia, 2004 Mar;59(1):103-7.
    PMID: 15535344
    We describe a case of acute disseminated encephalomyelitis in a child. This case is unusual in that the illness was characterised by recurrent episodes rather than a monophasic course and that the choice of treatment was intravenous immunoglobulin over corticosteroids. The rapid and remarkable recovery is highlighted and a review of the treatment for this rare condition is discussed.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  8. Sakthiswary R, D'Cruz D
    Medicine (Baltimore), 2014 Oct;93(16):e86.
    PMID: 25310743 DOI: 10.1097/MD.0000000000000086
    Prepared from the plasma of thousands of blood donors, therapeutic intravenous immunoglobulin (IVIg) mostly consists of human polyspecific immunoglobulin G (IgG). The use of IVIg in systemic lupus erythematosus (SLE) is still considered experimental without any clear indications. The purpose of this systematic review is, therefore, to evaluate the available evidence to determine the therapeutic role of IVIg in SLE. A comprehensive, computerised search was performed in the MEDLINE (Pubmed), Scopus, EMBASE, and Cochrane controlled trials. The study eligibility criteria were randomized controlled trials, and prospective and retrospective observational studies that examined the efficacy of IVIg in adult patients with SLE who were considered the participants.IVIg therapy was the mode of intervention in these patients. Data abstracted included the study design, study population, changes in the disease activity scores (Systemic Lupus Erythematosus Disease Activity Index, Systemic Lupus Activity Measure, and Lupus Activity Index-Pregnancy), steroid dose, complement levels, autoantibodies, and renal function. Thereafter, data analysis established statistical procedures for meta-analysis. Thirteen studies (including 3 controlled and 10 observational) were eligible for inclusion. There was significant reduction in the SLE disease activity scores with IVIg therapy with a standard mean difference of 0.584 (P = 0.002, 95% confidence interval [CI] 0.221-0.947). In terms of rise in complement levels, the response rate was 30.9% (P = 0.001, 95 CI 22.1-41.3). The effects of IVIg on other clinical outcome measures including anti-double-stranded DNA, antinuclear antibody, average steroid dose, and renal function could not be determined because of the limited numbers of trials. The limitations of this review were lack of well-designed controlled trials with adequate sample size on the use of IVIg in SLE. In conclusion, the use of IVIg is associated with significant reduction in SLE disease activity and improvement in complement levels.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  9. Chia PK, Hung SKY, Hiew FL
    J Clin Neurosci, 2019 Nov;69:114-119.
    PMID: 31416736 DOI: 10.1016/j.jocn.2019.08.016
    We determined the clinical progression, disability and outcome of 11 Multifocal Motor Neuropathy (MMN) patients from Malaysia. Mean patient age was 46.8 (SD 13.3), with mean disease duration of 108.0 months (SD 80.2). All reported unilateral limb weakness at onset. At diagnosis, after mean 49.9 months (SD 73.5) delay, 7 (63.6%) had more than 2 limbs involvement. Nine (90%) of 10 patients received induction IVIg dose of 2.0 gm/kg responded, demonstrated improvement in MRCSS of > 2 points or mRS score of > 1 point. We observed 38.5% drop in IVIg dose to mean 1.12 gm/kg/month after 12 months of treatment, and a further 34.8% drop upon 24th month treatment to mean dose of 0.73 gm/kg/month. This was in parallel with initial improvement in MRCSS and mRS, observed among 88.9% and 77.8% of the patients, and later further improvement (33.3%) or stabilization (66.7%) of mRS score toward 2nd year. During the same period, 50% of patients reported deterioration in ONLS, 33.3% in grip strength and 16.7% in MMN-RODS. Beyond 36th month, average annual IVIg dose increased at 0.12 gm/kg/year (SD 0.09) or 11.2%, up to the 84th months. Despite that, progressive deterioration was observed in term of number of limbs involvement, definite motor conduction blocks on electrophysiology study, and both clinical as well as functional scores. Although IVIg dose reduction for maintenance treatment in MMN is recommended, careful clinical assessment is required to prevent under-treatment. Use of reliable and responsive modern outcome measures is important to quantify clinically relevant change to guide therapy.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  10. Ng SH, Roshan S
    Med J Malaysia, 2018 12;73(6):410-412.
    PMID: 30647216
    Kawasaki disease is an autoimmune disease that commonly affects children below the age of 5 years. It is a vasculitic disease of unknown aetiology effecting the skin, eyes, lymph nodes and mucosal layer. Intravenous Immunoglobulin (IVIG) and aspirin therapy are the mainstay treatment however a number of cases have been shown to be refractory to this treatment. Evidence regarding approach and treatment for such cases is limited. This case report is to share our experience in the management of Refractive Kawasaki disease at a district level.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  11. Viswanathan S, Wong AH, Quek AM, Yuki N
    J Neuroimmunol, 2015 May 15;282:92-6.
    PMID: 25903734 DOI: 10.1016/j.jneuroim.2015.03.021
    To evaluate the use of intravenous immunoglobulin (IVIG) in preventing relapses in patients with neuromyelitis optica (NMO) and its spectrum disorders (NMOSDs).
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  12. Payus AO, Leow Wen Hsiang J, Leong JQ, Ibrahim A, Raymond AA
    Am J Case Rep, 2021 Jan 20;22:e928419.
    PMID: 33468985 DOI: 10.12659/AJCR.928419
    BACKGROUND Myasthenic crisis is a condition characterized by the sudden onset of myasthenic weakness involving the respiratory muscles and requires ventilatory support to prevent death. This is a case report of respiratory failure in a 43-year-old man as the first presentation of myasthenia gravis. CASE REPORT A 43-year-old man with underlying hypertension and a lacunar stroke with good muscle-power recovery presented with severe community-acquired pneumonia, complicated with respiratory failure requiring invasive ventilatory support. He responded well to the intravenous antibiotic therapy and after 1 week of treatment, he was hemodynamically stable and his septic parameters improved. However, he persistently failed to maintain adequate spontaneous respiratory effort after the removal of the ventilatory support and had to be reintubated multiple times. There was no other identifiable cause for the worsening respiratory failure. He had no clinical features or muscle weakness suggestive of myasthenia gravis. However, his blood test was positive for serum anti-acetylcholine receptor antibodies and repetitive nerve stimulation tests showed the characteristic decremental response of compound muscle action potential amplitude, in keeping with the diagnosis of myasthenia gravis. He responded well to intravenous immunoglobulin and was discharged with anticholinesterase inhibitors and long-term immunosuppression therapy. CONCLUSIONS This report demonstrates that when patients are admitted to the hospital with acute respiratory failure without any underlying pulmonary disease and with weakness of the respiratory muscles, the diagnosis of myasthenia gravis presenting with a myasthenic crisis should be considered.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  13. Chong JL, Sapari S, Kuan YC
    J Microbiol Immunol Infect, 2011 Aug;44(4):319-22.
    PMID: 21524955 DOI: 10.1016/j.jmii.2010.07.001
    Influenza A "novel H1N1" with severe acute respiratory distress syndrome (ARDS) is a serious illness that poses a challenge to clinicians managing such cases. This case report reveals a patient with ARDS secondary to influenza A with deteriorating clinical status, who improved tremendously after intravenous immunoglobulin G (IV IgG). Patients with H1N1 associated with ARDS may be given a trial of IV IgG. More case reports and trials are required to ascertain the efficacy of IV IgG and the best dosage and timing of starting IV IgG in relation to antiviral therapy.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  14. Hamidah A, Thambidorai CR, Jamal R
    PMID: 16124452
    We describe a patient with Evans syndrome (autoimmune hemolytic anemia and autoimmune thrombocytopenia) who was refractory to steroids and intravenous immunoglobulin. She responded to splenectomy and has remained in clinical remission for 3 years. In the majority of cases, splenectomy rarely induces a durable remission but it may be beneficial in a small group of patients, hence should be considered as alternative therapy in the management of these patients.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  15. Wan Jamaludin WF, Periyasamy P, Wan Mat WR, Abdul Wahid SF
    J Clin Virol, 2015 Aug;69:91-5.
    PMID: 26209387 DOI: 10.1016/j.jcv.2015.06.004
    Infection associated hemophagocytic syndrome is increasingly recognized as a potentially fatal complication of dengue fever. It should be suspected with prolonged fever beyond seven days associated with hepatosplenomegaly, hyperferritinemia, worsening cytopenias and development of multiorgan dysfunction. Surge of similar pro-inflammatory cytokines observed in dengue associated hemophagocytic syndrome and multiorgan dysfunction may indicate they are part of related inflammatory spectrum. A proportion of patients recovered with supportive therapy, however most required interventions with corticosteroids, intravenous immunoglobulin or chemotherapy. We report three cases of dengue associated IAHS with good outcome following early recognition and treatment with dexamethasone and intravenous immunoglobulin.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  16. Hung SKY, Hiew FL, Viswanathan S, Puvanarajah S
    J Peripher Nerv Syst, 2018 Sep;23(3):183-189.
    PMID: 30027593 DOI: 10.1111/jns.12282
    Intravenous immunoglobulin (IVIG), corticosteroids and therapeutic plasma exchange (TPE) are evidence-based conventional treatments for chronic inflammatory demyelinating polyneuropathy (CIDP). In many centres, unconventional treatments are frequently used as alternatives. We evaluated the outcome of conventional and unconventional therapies in 31 CIDP patients. Overall response rate with conventional first-line immunotherapies was 77% (20/26), comparable between IVIG and corticosteroids (80% vs 70%). Use of TPE was limited. Treatment response among typical and atypical CIDP were comparable (76 vs 80%). Non-responders were patients with progressive form of typical CIDP and DADS. Majority (21/26, 81%) of patients with persistent neurological deficits received maintenance therapy. Two subgroups of patients frequently treated with maintenance immunosuppressants were those with improving or stable disease following first-line treatment (12, 57%) and those with progressive form of CIDP (2, 10%). Primary indications for immunosuppressant use were corticosteroids-sparing and additional immunosuppression effects. Nine (64%) patients with improving or stable disease given azathioprine were taken off corticosteroids after a median duration of 14 months (range 12-108). Two (14%) eventually achieved cure or clinical remission without treatment. Maintenance IVIg was given to 6 (29%) relapsing CIDP patients; none of achieved cure or remission after similar median duration of treatment. Less potent immunosuppressant drugs (azathioprine, mycophenolate mofetil, and methotrexate) were frequently used, with moderate adverse effect profiles. In resource limited setting, unconventional treatments were commonly used among CIDP patients with different clinical course of progression. In most cases, careful risk-benefit re-assessment is required to justify its further use.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  17. Hiew FL, Ong JJ, Viswanathan S, Puvanarajah S
    J Clin Neurosci, 2018 Apr;50:203-207.
    PMID: 29398193 DOI: 10.1016/j.jocn.2018.01.018
    Long-term outcome in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is very limited, especially from Asian countries. We aimed to determine the outcome of our cohort of CIDP patients and to define the relevant clinical, electrophysiological and laboratory determinants of disease activity, progression and treatment response. We retrospectively reviewed records of 23 CIDP patients attending our Neurology service at Kuala Lumpur Hospital, Malaysia between January 2000 and December 2016. We analysed data on neurological deficits, electrophysiological and laboratory parameters to determine diagnostic characteristics, correlation with disease activity and clinical outcomes following treatment. Included were 15 (65%) males and 8 (35%) females with a mean age of 42.7 years (SD 14.4). Mean duration of follow-up visit was 66 months (range 6-134 months). The cohort consists of 19 classical (sensory-motor) CIDP and 4 MADSAM. Large majority of patients (66%) had either stable active disease (CDAS 3, 44%) or were in remission (CDAS class 2, 22%) following treatment with standard immunotherapies (Intravenous Immunoglobulins, steroids or immunosuppressants). The proportion of CIDP patients in each CDAS class was comparable to published cohorts from North America and Europe. Medical Research Council (MRC) sum score was the only clinical score that differed across CDAS classes (p = .010) with significant inverse correlation (Spearman's rho -0.664, p = .001). In conclusion, treatment outcomes of our CIDP cohort was comparable to those of published series. Further studies with larger cohort of patients from other parts of Asia are important to determine the long-term outcome of this heterogenous disease in this region.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use*
  18. Jasmin R, Sockalingam S, Shahrizaila N, Cheah TE, Zain AA, Goh KJ
    Lupus, 2012 Sep;21(10):1119-23.
    PMID: 22433918 DOI: 10.1177/0961203312440346
    Peripheral neuropathy is a known manifestation of systemic lupus erythematosus. However, the association of primary autoimmune inflammatory neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) with SLE is uncommon. We report a 26-year-old man who simultaneously presented with severe CIDP and photosensitive rash, but was unresponsive to intravenous immunoglobulin infusion and continued to progress. He was found to have underlying SLE and improved with combined corticosteroid and immunosuppressive therapy with oral cyclophosphamide. CIDP with underlying SLE may be more resistant to conventional therapy with IVIG, requiring the addition of other immunosuppressive agents.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  19. Ibrahim SB, Yashodhara BM, Umakanth S, Kanagasabai S
    BMJ Case Rep, 2012;2012.
    PMID: 22744241 DOI: 10.1136/bcr.02.2012.5850
    A 23-year-old pregnant woman in her second trimester of pregnancy presented with blisters on the face, abdomen and the leg. Based on the clinical presentation and skin biopsy (histopathology and direct immunofluorescence) the diagnosis of pemphigus vulgaris was established. The child born to this patient also had similar skin lesions. The lesions in the mother and the child improved after treatment. The authors report a rare case of pemphigus vulgaris in a pregnant lady and neonatal pemphigus in her child, both of whom were treated successfully.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
  20. Shahrizaila N, Goh KJ, Abdullah S, Kuppusamy R, Yuki N
    Clin Neurophysiol, 2013 Jul;124(7):1456-9.
    PMID: 23395599 DOI: 10.1016/j.clinph.2012.12.047
    Recent studies have advocated the use of serial nerve conduction studies (NCS) in the electrodiagnosis of Guillain-Barré syndrome (GBS). The current study aims to elucidate when and how frequent NCS can be performed to reflect the disease pathophysiology.
    Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
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