Displaying publications 1 - 20 of 85 in total

  1. Jasmin R, Sockalingam S, Ramanaidu LP, Goh KJ
    Lupus, 2015 Mar;24(3):248-55.
    PMID: 25253567 DOI: 10.1177/0961203314552115
    OBJECTIVE: Peripheral neuropathy in systemic lupus erythematosus (SLE) is heterogeneous and its commonest pattern is symmetrical polyneuropathy. The aim of this study was to describe the prevalence, clinical and electrophysiological features, disease associations and effects on function and quality of life of polyneuropathy in SLE patients, defined using combined clinical and electrophysiological diagnostic criteria.
    METHODS: Consecutive SLE patients seen at the University of Malaya Medical Centre were included. Patients with medication and other disorders known to cause neuropathy were excluded. Demographic, clinical and laboratory data were obtained using a pre-defined questionnaire. Function and health-related quality of life was assessed using the modified Rankin scale and the SF-36 scores. Nerve conduction studies (NCS) were carried out in both upper and lower limbs. Polyneuropathy was defined as the presence of bilateral clinical symptoms and/or signs and bilateral abnormal NCS parameters.
    RESULTS: Of 150 patients, 23 (15.3%) had polyneuropathy. SLE-related polyneuropathy was mainly characterized by sensory symptoms of numbness/tingling and pain with mild signs of absent ankle reflexes and reduced pain sensation. Function was minimally affected and there were no differences in quality of life scores. NCS abnormalities suggested mild length-dependent axonal neuropathy, primarily in the distal lower limbs. Compared to those without polyneuropathy, SLE-related polyneuropathy patients were significantly older but had no other significant demographic or disease associations.
    CONCLUSIONS: SLE-related polyneuropathy is a chronic, axonal and predominantly sensory neuropathy, associated with older age. Its underlying pathogenetic mechanisms are unknown, although a possibility could be an increased susceptibility of peripheral nerves in SLE patients to effects of aging.
  2. Jasmin R, Sockalingam S, Cheah TE, Goh KJ
    Lupus, 2013 Aug;22(9):967-71.
    PMID: 23846232 DOI: 10.1177/0961203313496299
    OBJECTIVES: Ethnic differences in systemic lupus erythematosus (SLE) have been previously described in the multiethnic Malaysian population. However, there have since been many demographic and socioeconomic changes in the country. The aim of this study is to re-examine the clinical and immunological profiles of Malaysian SLE patients of different ethnic backgrounds.
    METHODS: Consecutive follow-up patients at the University Malaya Medical Centre (UMMC) from July 2010 until March 2011 were included in the study.
    RESULTS: The most common clinical manifestations were malar rash (61.3%), arthritis (52.3%), haematological disease (51.6%), oral ulcers (51%) and renal disease (40.6%). Ethnic Indians had fewer malar and discoid rashes but were at higher risk of arthritis, serositis, renal and neuropsychiatric disease compared to Malays and Chinese Malaysians. Antiphospholipid syndrome (APS) was less common in Chinese. A longer duration of SLE correlated with a lower SLEDAI score.
    CONCLUSION: Overall, the spectrum disease expression was similar to the earlier Malaysian study but the frequency of the more severe disease manifestations, viz. renal, haematological, neuropsychiatric involvements and serositis, were lower. This study further emphasises differences primarily between ethnic Indians and the other races in Malaysia.
    KEYWORDS: Indians; Malaysia; Systemic lupus erythematosus; clinical manifestations; ethnicity
  3. Chew NK, Tan CT, Goh KJ
    J Clin Neurosci, 2002 Sep;9(5):604-5.
    PMID: 12383430
    A 24-year-old woman presented with a 3.5-year history of paroxysmal dystonia that was precipitated by sudden movement, especially when she started to walk. It was characterised by shrugging of shoulders, flexion of the neck and thoracic spine, and stiffness of the right leg followed by falls. Each attack lasted for less than 5min. Inadequate sleep and stress were exacerbating factors. There was no similar family history. Physical examination and investigations were normal. The following manoeuvres that caused vestibular stimulation precipitated attacks: turning her head from side to side while standing still, sitting still on a rotating chair and an ice-water caloric test. She had partial responses to phenytoin and levodopa, and a good response to haloperidol. Vestibular stimulation as a precipitating factor in paroxysmal kinesigenic choreoathetosis has not been reported previously.
  4. Goh KJ, Wong KT, Tan CT
    J Clin Neurosci, 2000 Jul;7(4):334-6.
    PMID: 10938615
    We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.
  5. Ng S, Wong, KT, Goh KJ
    Neurology Asia, 2013;18(2):177-181.
    Myopathies, although presenting more commonly in the younger age group, can occur and contribute significantly to disability in the elderly. To describe the spectrum of elderly myopathies, we reviewed 52 elderly patients (> 65 years) from the University of Malaya Medical Centre muscle biopsy databank, constituting 6.8% of 759 adult patients (> 18 years) who underwent muscle biopsy between 1992 and 2012. Commonest were the inflammatory myopathies (41/52, 78.8%), of which 43.9% had dermatomyositis; 23.9% polymyositis; 14.6% sporadic inclusion body myositis; 9.8% undifferentiated myositis and 2.4% overlap myositis. Seven patients (13.4%) had genetic myopathy; 2 muscular dystrophy and 5 chronic progressive external ophthalmoplegia, while 4 patients (7.7%) had drug-associated myopathy, 3 with statins. Malignancies were seen in 9.8% of inflammatory myopathies at diagnosis. Both acquired and genetic myopathies are seen in elderly Malaysians of all ethnicities and should not be misdiagnosed as some are potentially treatable and/or associated with malignancy.
  6. Tan CY, Shahrizaila N, Goh KJ
    J Oral Facial Pain Headache, 2017 10 27;31(4):e15-e20.
    PMID: 29073667 DOI: 10.11607/ofph.1793
    AIMS: To describe the clinical characteristics of trigeminal neuralgia (TN) in a multi-ethnic Malaysian population and to relate them to standardized measures of pain severity, anxiety, depression, and quality of life (QoL).

    METHODS: Patients fulfilling the International Headache Society (IHS) criteria for TN were prospectively interviewed for their demographic and clinical data. Pain intensity was rated with a visual analog scale (VAS), anxiety and depression were determined by the Hospital Anxiety and Depression Scale (HADS), and QoL was assessed by the Short-Form 36 (SF-36) questionnaire. Chi-square, Mann-Whitney U, and Spearman correlation tests were used to test for differences considering a significance level of P < .05.

    RESULTS: Of the 75 included patients, 52 (69.3%) were women with a mean ± standard deviation (SD) onset age of 52.0 ± 12.7 years, and 57.3% were Chinese, 24.0% Malay, and 18.7% Indian. Pain was more common on the right side (69.3%) and in the maxillary and mandibular divisions. VAS scores for pain at its worst were higher in anxious/borderline anxious patients compared to non-anxious patients (89.5 ± 15.9 vs 80.9 ± 17.2, respectively; P < .05), and VAS scores for pain at its least were higher in depressed/borderline depressed subjects compared to non-depressed subjects (38.4 ± 25.8 vs 23.0 ± 19.2, respectively; P < .05). Chinese patients had lower VAS scores for pain at its least compared to Indian patients (19.7 ± 16.1 vs 39.9 ± 24.7; P < .01). TN patients scored lower in all eight domains of the SF-36 compared to the general population. Indian patients had lower scores in role limitations due to physical health (8.9 ± 23.2 vs 49.4 ± 43.8; P < .01) and social function (56.3 ± 13.6 vs 76.5 ± 23.6; P < .01) than Chinese patients, and Malay patients had lower mental health scores compared to Chinese patients (59.1 ± 19.5 vs 73.0 ± 21.0; P < .01).

    CONCLUSION: Clinical characteristics of TN patients were similar to those of other populations. There were differences in pain ratings and QoL between TN patients of different ethnicities, as well as between those with anxiety and depression.

  7. Chai CH, Yuki N, Nor HM, Goh KJ, Shahrizaila N
    Pract Neurol, 2012 Oct;12(5):328-31.
    PMID: 22976064 DOI: 10.1136/practneurol-2011-000205
  8. 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.
  9. Shahrizaila N, Goh KJ, Kokubun N, Abdullah S, Yuki N
    J Neurol Sci, 2011 Oct 15;309(1-2):26-30.
    PMID: 21849173 DOI: 10.1016/j.jns.2011.07.042
    The electrodiagnosis of Guillain-Barré syndrome (GBS) can be broadly divided into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Fisher syndrome (FS) is a variant of GBS, although the underlying neuropathy of FS has yet to be established. Serial nerve conduction studies (NCS) can provide further insight into the likely pathophysiology by further subtyping of GBS and FS. We present a patient with an initial diagnosis of AIDP in whom repeated NCS revealed the AMAN variant. This led us to investigate serial NCS in five patients with GBS, FS and FS/GBS overlap presenting over a period of a year. Three patients with AIDP showed a gradual increase in distal motor latencies during the acute phase of illness. NCS of two patients with FS and FS/GBS overlap showed no demyelinating features suggesting underlying axonal neuropathy in this group of patients. The importance of serial NCS in establishing the underlying pattern of neuropathy in GBS and FS is further emphasized in this study. Larger studies incorporating serial NCS are required to confirm the observations seen in our case series especially when pathological studies are often not justified in this group of patients.
  10. 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.
  11. Tan CY, Razali SNO, Goh KJ, Shahrizaila N
    J Peripher Nerv Syst, 2019 06;24(2):168-173.
    PMID: 31001904 DOI: 10.1111/jns.12320
    Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that has variable disease course and outcome. The Erasmus GBS outcome score (EGOS), modified EGOS (mEGOS), and Erasmus GBS respiratory insufficiency score (EGRIS) are prognostic models designed to predict the functional outcome of GBS patients at 6 months (EGOS and mEGOS) and the need for mechanical ventilation within a week of admission (EGRIS). The models were primarily developed in the Dutch GBS population, and thus the usefulness of these models in other GBS cohorts is less clear. In the current study, we aimed to validate mEGOS, EGOS, and EGRIS in Malaysian GBS patients. A total of 107 patients with GBS and its variants were consecutively recruited. Patients with GBS and Miller Fisher syndrome (MFS) were analysed separately. In the GBS cohort, high mEGOS and EGOS scores were significantly correlated with poor outcome at 6 months (mEGOS on admission: r = .381, P = .005; mEGOS at day 7 of admission: r = .507, P 
  12. Tan CY, Razali SNO, Goh KJ, Shahrizaila N
    J Clin Neurol, 2021 Apr;17(2):273-282.
    PMID: 33835749 DOI: 10.3988/jcn.2021.17.2.273
    BACKGROUND AND PURPOSE: Several variants of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) exist, but their frequencies vary in different populations and do not always meet the inclusion criteria of the existing diagnostic criteria. However, the GBS classification criteria by Wakerley and colleagues recognize and define the clinical characteristics of each variant. We applied these criteria to a GBS and MFS cohort with the aim of determining their utility.

    METHODS: Consecutive GBS and MFS patients presenting to our center between 2010 and 2020 were analyzed. The clinical characteristics, electrophysiological data, and antiganglioside antibody profiles of the patients were utilized in determining the clinical classification.

    RESULTS: This study classified 132 patients with GBS and its related disorders according to the new classification criteria as follows: 64 (48.5%) as classic GBS, 2 (1.5%) as pharyngeal-cervical-brachial (PCB) variant, 7 (5.3%) as paraparetic GBS, 29 (22%) as classic MFS, 3 (2.3%) as acute ophthalmoparesis, 2 (1.5%) as acute ataxic neuropathy, 2 (1.5%) as Bickerstaff brainstem encephalitis (BBE), 17 (12.9%) as GBS/MFS overlap, 4 (3%) as GBS/BBE overlap, 1 (0.8%) as MFS/PCB overlap, and 1 (0.8%) as polyneuritis cranialis. The electrodiagnosis was demyelinating in 55% of classic GBS patients but unclassified in 79% of classic MFS patients. Anti-GM1, anti-GD1a, anti-GalNAc-GD1a, and anti-GD1b IgG ganglioside antibodies were more commonly detected in the axonal GBS subtype, whereas the anti-GQ1b and anti-GT1a IgG ganglioside antibodies were more common in classic MFS and its subtypes.

    CONCLUSIONS: Most of the patients in the present cohort met the criteria of either classic GBS or MFS, but variants were seen in one-third of patients. These findings support the need to recognize variants of both syndromes in order to achieve a more-complete case ascertainment in GBS.

  13. Tang SY, Hara S, Melling L, Goh KJ, Hashidoko Y
    Biosci Biotechnol Biochem, 2010;74(9):1972-5.
    PMID: 20834139
    Root-associating bacteria of the nipa palm (Nypa fruticans), preferring brackish-water affected mud in Sarawak, Malaysia, were investigated. In a comparison of rhizobacterial microbiota between the nipa and the sago (Metroxylon sagu) palm, it was found that the nipa palm possessed a group of Burkholderia vietnamiensis as its main active nitrogen-fixing endophytic bacterium. Acetylene reduction by the various isolates of B. vietnamiensis was constant (44 to 68 nmol h(-1) in ethylene production rate) in soft gel medium containing 0.2% sucrose as sole carbon source, and the bacterium also showed motility and biofilm-forming capacity. This is the first report of endophytic nitrogen-fixing bacteria from nipa palm.
  14. Goh KJ, Wong KT, Nishino I, Minami N, Nonaka I
    Neuromuscul Disord, 2005 Mar;15(3):262-4.
    PMID: 15725589
    Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disorder of middle age presenting as progressive dysphagia and eyelid ptosis, due to short expansions of the GCG trinucleotide repeat (from GCG6 to GCG8-13) in the polyadenylate binding-protein nuclear 1 (PABPN1) gene. OPMD is rarely seen in Asians and morphologically and/or genetically confirmed cases have been reported in Japanese kindreds only. We report a 64 year old Chinese-Malaysian woman who presented with progressive dysphagia and bilateral ptosis for about 6 years. Her mother and elder brother (both deceased) were believed to be affected. Muscle histopathology revealed angulated fibres with rimmed vacuoles. Genetic analysis showed repeat expansion in one allele to (GCG)9 while normal in the other (GCG)6. This is the first non-Japanese Asian family with genetically confirmed OPMD.
  15. Tan CY, Razali SNO, Goh KJ, Shahrizaila N
    J Peripher Nerv Syst, 2020 09;25(3):256-264.
    PMID: 32511817 DOI: 10.1111/jns.12398
    We aimed to evaluate the key diagnostic features of Guillain-Barré syndrome (GBS) in Malaysian patients and validate the Brighton criteria. This was a retrospective study of patients presenting with GBS and Miller Fisher syndrome (MFS) between 2010 and 2019. The sensitivity of the Brighton criteria was evaluated. A total of 128 patients (95 GBS, 33 MFS) were included. In the GBS cohort, 92 (97%) patients presented with symmetrical limb weakness. Reflexes were depressed or absent in 90 (95%) patients. Almost all patients (94, 99%) followed a monophasic disease course, with 5 (5%) patients experiencing treatment-related fluctuations. Cerebrospinal fluid (CSF) albuminocytological dissociation was seen in 62/84 (73%) patients. Nerve conduction study (NCS) revealed neuropathy in 90/94 (96%) patients. In GBS patients with complete dataset (84), 56 (67%) patients reached level 1 of the Brighton criteria, 21 (25%) reached level 2, 3 (4%) reached level 3, and 4 (5%) reached level 4. In MFS, the clinical triad was present in 25 (76%) patients. All patients had a monophasic course. CSF albuminocytological dissociation was present in 10/25 (40%) patients. NCS was normal or showed sensory neuropathy in 25/33 (76%) patients. In MFS patients with complete dataset (25), 5 (20%) patients reached level 1 of the Brighton criteria, 14 (56%) reached level 2, 2 (8%) reached level 3, and 4 (16%) reached level 4. Inclusion of antiganglioside antibodies improved the sensitivity of the Brighton criteria in both cohorts. In the Malaysian cohort, the Brighton criteria showed a moderate to high sensitivity in reaching the highest diagnostic certainty of GBS, but the sensitivity was lower in MFS.
  16. Tan CY, Sekiguchi Y, Goh KJ, Kuwabara S, Shahrizaila N
    Clin Neurophysiol, 2020 01;131(1):63-69.
    PMID: 31751842 DOI: 10.1016/j.clinph.2019.09.025
    OBJECTIVE: We aimed to develop a model that can predict the probabilities of acute inflammatory demyelinating polyneuropathy (AIDP) based on nerve conduction studies (NCS) done within eight weeks.

    METHODS: The derivation cohort included 90 Malaysian GBS patients with two sets of NCS performed early (1-20days) and late (3-8 weeks). Potential predictors of AIDP were considered in univariate and multivariate logistic regression models to develop a predictive model. The model was externally validated in 102 Japanese GBS patients.

    RESULTS: Median motor conduction velocity (MCV), ulnar distal motor latency (DML) and abnormal ulnar/normal sural pattern were independently associated with AIDP at both timepoints (median MCV: p = 0.038, p = 0.014; ulnar DML: p = 0.002, p = 0.003; sural sparing: p = 0.033, p = 0.009). There was good discrimination of AIDP (area under the curve (AUC) 0.86-0.89) and this was valid in the validation cohort (AUC 0.74-0.94). Scores ranged from 0 to 6, and corresponded to AIDP probabilities of 15-98% at early NCS and 6-100% at late NCS.

    CONCLUSION: The probabilities of AIDP could be reliably predicted based on median MCV, ulnar DML and ulnar/sural sparing pattern that were determined at early and late stages of GBS.

    SIGNIFICANCE: A simple and valid model was developed which can accurately predict the probability of AIDP.

  17. Tan CY, Razali SNO, Goh KJ, Shahrizaila N
    J Med Ultrasound, 2021 04 03;29(3):181-186.
    PMID: 34729327 DOI: 10.4103/JMU.JMU_105_20
    Background: High-resolution nerve ultrasound provides morphological information of peripheral nerves. We aimed to determine the normal ultrasonographic reference values of nerve cross-sectional area (CSA) in multiethnic Malaysian healthy participants.

    Methods: Nerve ultrasound of the median, ulnar, radial, tibial, fibular, and sural nerves was performed in 84 healthy participants at anatomical-defined locations. The CSA at each scanned site was measured by tracing circumferentially inside the hyperechoic rim of each nerve. Comparisons were made between genders and ethnic groups. Correlations with age, ethnicity, gender, height, weight, and body mass index (BMI) were evaluated.

    Results: CSA values and reference ranges in healthy participants were generated. Nerve CSA was significantly different in different gender (P = 0.002-0.032) and ethnic groups (P = 0.006-0.038). Men had larger nerve CSA than women, and Malay participants had larger nerve CSA compared to other ethnic groups. Nerve CSA had significant correlations to age, height, weight, and BMI (r = 0.220-0.349, P = 0.001-0.045).

    Conclusion: This study provides normative values for CSA of peripheral nerves in a multiethnic Malaysian population, which serves as reference values in the evaluation of peripheral nerve disorders. The ethnic differences in nerve CSA values should be considered during nerve ultrasound.

  18. Raja J, Balaikerisnan T, Ramanaidu LP, Goh KJ
    Int J Rheum Dis, 2021 Mar;24(3):347-354.
    PMID: 33432774 DOI: 10.1111/1756-185X.14042
    AIM: The reported prevalence of peripheral neuropathy in systemic sclerosis (SSc) is variable between 0.01% to 28%, probably due to differences in sample size, study design and population. Our aim is to determine the prevalence of large fiber peripheral neuropathy in SSc and to identify any contributing factors.

    METHOD: A prospective cross-sectional study of 60 SSc patients were evaluated for large fiber neuropathy using the modified clinical Total Neuropathy Score (cTNS) and nerve conduction study (NCS) of the upper and lower limbs. A combination of clinical (cTNS score ≥ 2) and NCS criteria (≥2 abnormal nerves including 1 sural [symmetrical polyneuropathy] and NCS abnormalities consistent with individual nerves/nerve roots [focal neuropathy]) was used to diagnose peripheral neuropathy.

    RESULTS: The majority had limited cutaneous subset (75%). Mean age was 55.73 (SD ± 13.04) years and mean disease duration was 8.61 (SD ± 8.09) years. Twenty-two (36.7%) had combined clinical and NCS criteria for peripheral neuropathy, 14 (23.3%) with symmetrical polyneuropathy and 8 (13.3%) with focal neuropathy. Symmetrical polyneuropathy patients had significantly lower hemoglobin levels (11.2 vs. 12.35 g/L; P = .047). Serum vitamin B12 levels were normal, therefore excluding vitamin B12 deficiency. No other associations were found for both polyneuropathy and focal neuropathy with demography, co-morbid diseases and SSc disease factors such as Raynaud's phenomenon and modified Rodnan skin score.

    CONCLUSION: Large fiber neuropathy is common in SSc patients, which could contribute to non-lethal burden in SSc with sensory loss and muscle weakness. Apart from lower hemoglobin in polyneuropathy, there were no associations with disease-specific features or co-morbid diseases.

  19. Fong SY, Raja J, Wong KT, Goh KJ
    Rheumatol Int, 2021 02;41(2):355-360.
    PMID: 32488429 DOI: 10.1007/s00296-020-04610-8
    Asymptomatic electrophysiological peripheral neuropathy is described in systemic lupus erythematosus (SLE) patients. To determine if SLE could have an even earlier effect on peripheral nerve function even before the development of electrophysiological abnormalities, we compared nerve conduction studies (NCS) of SLE patients without electrophysiological or clinical peripheral neuropathy with healthy controls. Consecutive SLE patients without clinical neuropathy (or other known causes of neuropathy) underwent sensory and motor NCS of all four limbs. Results of 61 patients without electrophysiological criteria of neuropathy were compared with age- and gender-matched controls. Although still within the laboratory's range of normal values, significant differences were found in several NCS parameters between patients and controls. SLE patients had lower amplitudes for ulnar, fibular, and tibial compound muscle action potentials (CMAP) and sural sensory nerve action potentials (SNAP); slower conduction velocities for median, ulnar, and fibular motor nerves, and median, ulnar and sural sensory nerves. SLE patients also had longer minimum F-wave latencies for median, ulnar, fibular, and tibial nerves. H reflexes were more often absent in patients. Correlations were found between the number of disease relapses and motor conduction velocities of the fibular and tibial nerves. SLE may have early effect on peripheral nerve function in patients even before they develop electrophysiological or clinical neuropathy.
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