MyMedR

Fulltext Comparison of three different methods in the assessment of neutrophil function

Noah RM, Jais MR, Noh LM

Med J Malaysia, 1995 Jun;50(2):136-40.

Three different methods to measure the oxidative respiratory burst of neutrophils were performed. Of the three, the chemiluminescence technique was observed to be the most sensitive among them. The strong statistical correlation and an acceptable agreement between chemiluminescence with that of the killing assay provides evidence for using the chemiluminescence assay as an alternative method of detecting gross defects of neutrophil respiratory burst killing assays.

Evaluation of variable parameters in neutrophil function test using chemiluminescence assay

Noah RM, Jais MR, Noh LM

Malays J Pathol, 1994 Dec;16(2):157-60.

PMID: 9053565

Variable parameters in chemiluminescence assay, one of the methods used to assess the functional capacity of neutrophils, were evaluated for suitable adaptation locally. The use of pooled normal human serum as compared to single normal human serum in opsonizing particles for phagocytosis was found to exhibit lower chemiluminescence activity (reduction range of 30%-50%). A similar degree of depression was observed when the particles were opsonized using normal human serum in comparison to that using autologous serum. Different intensity of chemiluminescence was also noted when the opsonized particle used was the Oxford strain of Staphylococcus aureus (NCTC 6571) in contrast to a strain of Staphylococcus aureus isolated from a patient. The results obtained warrant clinicians to deliver appropriate samples as best they can when the chemiluminescence assay is requested.

Antibody deficiency with hyper IgM--a case report

Noh LM, Low SM, Lajin I, Abdullah N

Malays J Pathol, 1992 Dec;14(2):121-3.

PMID: 1304625

A 20-month-old Indian boy presented with recurrent pyogenic infections and failure to thrive. His IgG and IgA levels were low, but his IgM was elevated. He also had undetectable isohaemagglutinin titre and neutropenia, both parameters being poor prognostic indicators in this very rare primary immunodeficiency state--antibody deficiency with hyper IgM. Our patient subsequently succumbed to Pseudomonas aeruginosa septicaemia and meningitis inspite of aggressive antibiotic and intravenous gammaglobulin therapy. To the best of our knowledge, this is the first such case to be documented in Malaysia.

Fulltext Immunology of tuberculosis: clinical and research persepctives

Mohd Noh L, Wan Omar A, Abdul Latif A

Malaysian Journal of Medicine and Health Sciences, 2005;1(2):71-85.

Disseminated tuberculosis in acute leukemia

Menon BS, Maziah W, Aiyar S, Zainul F, Shuaib I, Noh L

Pediatr Int, 2001 Apr;43(2):161-3.

PMID: 11285069

Fulltext Severe combined immunodeficiency in a Malaysian child

Noh LM, Amir HL, Hung LC, Zulkifli I, Nasuruddin BA

Med J Malaysia, 1997 Mar;52(1):88-91.

PMID: 10968061

A 3-month-old Malay male infant presented with multiple infections (candidiasis, Pseudomonas aeruginosa, Cytomegalovirus), persistent pneumonia, intractable diarrhoea and failure to thrive. There was lymphopaenia affecting both T and B subsets. He developed Graft versus Host disease weeks following transfusion with non irradiated blood. In spite of aggressive microbicidal and supportive therapy including regular immunoglobulin infusions, the child succumbed to infection before a bone marrow transplant could be instituted.

Chronic mucocutaneous candidiasis with deficient CD2 (E receptor) but normal CD3 mononuclear cells

Noh LM, Hussein SH, Sukumaran KD, Rose I, Abdullah N

J Clin Lab Immunol, 1991 Jun;35(2):89-93.

PMID: 1688166

A case of chronic mucocutaneous candidiasis in a Malaysian child who subsequently developed disseminated tuberculosis and toxoplasmosis is described. The phenotype of her peripheral blood mononuclear cells showed discordance for her T cell markers. The presence of a subpopulation of CD2-/CD3+ mononuclear cells leading to an immunodeficiency state is consistent with failure of activation of CD2-mediated alternative pathway resulting in immunodeficiency. Such abnormal CD2-/CD3+ subpopulations have been described in lepromatous leprosy and foetal abortuses.

Near miss diagnosis of childhood tuberculosis

Norlijah O, Intan HI, Feizel AM, Kasim MS, Noh LM

Southeast Asian J. Trop. Med. Public Health, 2006 Sep;37(5):953-7.

PMID: 17333739

Tuberculosis (TB) remains a public health problem in Malaysia. We describe three atypical cases of serious tuberculosis in children. The potential diagnostic pitfall in these cases is highlighted by its unusual presentation in a setting of culture-negative infection. A positive polymerase chain reaction (PCR) in each case assists in gauging the diagnosis in concordance with appropriate clinical findings.

Clinical patterns of X linked agammaglobulinemia in Malaysian children

Noh LM, Ismail Z, Zainudin BM, Low SM, Azizi BH, Noah RM, et al.

Acta Paediatr Jpn, 1995 Jun;37(3):331-5.

PMID: 7645382

X linked agammaglobulinemia (XLA) is rarely reported from developing countries especially from South East Asia. It appears that X linked agammaglobulinemia is less common in certain ethnic groups. It is very uncommon in black people in USA and South Africa. In multiracial Malaysia we have documented five XLA in Malays and Indians but not in the Chinese that constitute about 31% of the population. First degree relatives afflicted with XLA or other primary immunodeficiencies occurred more often in our study. All showed lung involvement although the etiologic organisms involved were atypical, being Gram negative.

Idiopathic CD4+ T-lymphocytopenia in a child with disseminated cryptococcosis

Menon BS, Shuaib IL, Zamari M, Haq JA, Aiyar S, Noh LM

Ann Trop Paediatr, 1998 Mar;18(1):45-8.

PMID: 9692001

We describe a Malay girl with disseminated cryptococcosis affecting the lungs, liver, lymph nodes and bones. The diagnosis was made by culture of the bone marrow. Tests of immune function showed that she was HIV-negative but the CD4 percentage was persistently low. Idiopathic CD4+ T-lymphocytopenia was diagnosed. The child died despite two courses of anti-fungal therapy.

Clinical and laboratory observation on immunoglobulin replacement therapy switching from an intravenous to a subcutaneous route in a Malaysian X-linked agammaglobulinemia patient

Adli A, Wahab AA, Abdul Latiff AH, Ismail IH, Faizah MZ, Boekhren KB, et al.

Med J Malaysia, 2022 Jan;77(1):95-97.

PMID: 35087003

We report a clinical and laboratory observation in a boy with X-linked agammaglobulinemia (XLA) who underwent an immunoglobulin replacement therapy (IRT) via the subcutaneous route (IGSC) seven years after his IRT via intravenous route (IGIV). He was free of invasive infections when on IGIV but not the troublesome coughs a week before the next infusion. A switch to a subcutaneous route resulted in significant improvement of symptoms with good weight gain. When on 2-weekly IGSC cycle, adjusting dose for weight resulted in an IgG trough level of > 600 mg/dl.

Fulltext Chronic granulomatous disease--a report in two Malay families

Mohd Noh L, Noah RM, Wu LL, Nasuruddin BA, Junaidah E, Ooi CP, et al.

Singapore Med J, 1994 Oct;35(5):505-8.

PMID: 7701372

Chronic granulomatous disease (CGD) is a very rare disease whose defect lies in an abnormal intracellular killing resulting in recurrent abscesses, lymphadenitis and granuloma formation. We describe 2 Malay male infants with CGD whom we believe to be the first report of this disorder in Malays. Both children presented with recurrent abscesses, pneumoniae and hepatosplenomegaly; lymphadenopathy was also present in one of the patients. The organisms isolated were catalase positive bacteria. Both neutrophil chemiluminescence (against fungal and bacterial antigens, phorbol myristate acetate) and intracellular killing assays were severely depressed. Recognition of CGD is important as great strides have been made in the treatment of this disease which include gamma interferon therapy besides the conventional prophylactic antibacterial therapy.

Fulltext Health-Related Quality of Life of Patients and Families with Primary Immunodeficiency in Malaysia: a Cross-Sectional Study

Ahmed Meelad R, Abd Hamid IJ, Zainudeen ZT, Hashim IF, Azizuddin MNA, Mangantig E, et al.

J Clin Immunol, 2023 Jul;43(5):999-1006.

PMID: 36882668 DOI: 10.1007/s10875-023-01463-1

PURPOSE: Primary immunodeficiency disease (PID) affects various aspects of a patient's life. However, the health-related quality of life (HRQOL) of PID among Malaysian patients is poorly described. This study aimed to determine the quality of life of PID patients and their respective parents.

METHOD: This cross-sectional study was performed from August 2020 to November 2020. Patients with PID and their families were invited to answer the PedsQL Malay version (4.0) questionnaire, the tool used to assess the HRQOL. A total of 41 families and 33 patients with PID answered the questionnaire. A comparison was performed with the previously published value of healthy Malaysian children.

RESULT: Parents of respondents recorded a lower mean of total score than the parents of healthy children (67.26 ± 16.73 vs. 79.51 ± 11.90, p-value = 0.001, respectively). PID patients reported lower mean total score to healthy children (73.68 ± 16.38 vs. 79.51 ± 11.90, p-value = 0.04), including the psychosocial domain (71.67 ± 16.82 vs. 77.58 ± 12.63, p-value = 0.05) and school functioning (63.94 ± 20.87 vs. 80.00 ± 14.40, p-value = 0.007). No significant difference of reported HRQOL when comparing between subgroup of PID on immunoglobulin replacement therapy and those without immunoglobulin replacement (56.96 ± 23.58 vs. 65.83 ± 23.82, p-value 0.28). Socioeconomic status was found to be predictive of the lower total score of PedsQL in both parent and children reports.

CONCLUSION: Parents and children with PID, especially those from middle socioeconomic status, have lower HRQOL and school function impairment than healthy children.

Fulltext Impact of Primary Immunodeficiency Diseases on the Life Experiences of Patients in Malaysia From the Caregivers' Perspective: A Qualitative Study

Ahmed Meelad R, Abd Hamid IJ, Hashim IF, Zainudeen ZT, Abu Bakar FF, Taib F, et al.

Front Pediatr, 2022;10:846393.

PMID: 35433547 DOI: 10.3389/fped.2022.846393

INTRODUCTION: Primary immunodeficiency diseases (PIDs) are chronic diseases that affect the various aspects of a patient's life. However, the impact of living with PIDs is poorly described.

OBJECTIVE: This study aimed to explore the living experience challenges among the Malaysian caregivers of the patients with PID who underwent a follow-up in the Universiti Sains Malaysia or those registered members of the Malaysian PIDs Society.

METHODOLOGY: The study was conducted from March 1 to May 30, 2021. The parents of children with PIDs were invited to participate for a semi-structured in-depth interview at the PID clinics in the USM. The estimated time of each interview was 30 min. The semi-structured interview was performed via a telephone call because of COVID-19 pandemic restrictions. The audio recording of each interview was transcribed and translated from Malay to English. Subsequently, a thematic analysis utilizing the ATLAS.ti software was performed.

RESULTS: The thematic analysis revealed five main themes, which are living with fear and anxiety with four sub-themes (sickness, psychological issues, fear of infections and hereditary issues), PID healthcare support struggles with four sub-themes (PID health system, treatment, diagnosis and financial issues), knowledge with two sub-themes (educational issues and disease understanding), social constraint with two sub-themes (relationships and social isolations) and coping with three sub-themes (acceptance, child health improvement and emotional hygiene).

CONCLUSION: Living with fear and anxiety is a major theme impacting the living experiences of Malaysian patients with PIDs. Improvements in healthcare delivery and disease education are needed to ensure optimal quality of life.

Fulltext Clinical-Epidemiological Pattern of Primary Immunodeficiencies in Malaysia 1987-2006: A 20 year experience in Four Malaysian Hospitals

Noh LM, Nasuruddin BA, Abdul Latiff AH, Noah RM, Kamarul Azahar MR, Norzila MZ, et al.

Med J Malaysia, 2013;68(1):13-7.

PMID: 23466760

To determine the clinical and epidemiological characteristics of patients seen with primary immunodeficiencies referred at four Malaysian Hospitals between 1987 to 2007.

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