MyMedR

First study of the F508del mutation in Malaysian children diagnosed with cystic fibrosis

Nathan AM, Thong MK, deBruyne J, Ariffin H

J Paediatr Child Health, 2011 Aug;47(8):573-5.

PMID: 21843195 DOI: 10.1111/j.1440-1754.2011.02149.x

Development of an algorithm of satellite markers for monitoring chimerism status in post-allogeneic haematopoietic stem cell transplantation patients

Choong SS, Rosmanizam S, Ibrahim K, Gan GG, Ariffin H

Int J Lab Hematol, 2011 Apr;33(2):182-6.

PMID: 20868447 DOI: 10.1111/j.1751-553X.2010.01264.x

Analysis of variable number tandem repeats (VNTRs) by polymerase chain reaction (PCR) is a common method used to predict engraftment status in post-allogeneic haematopoeitic stem cell transplantation (HSCT) patients. Different populations have different copies of repeated DNA sequence and hence, different percentage of informativeness between patient and donor.

Chemical recycling of polyhydroxyalkanoates as a method towards sustainable development

Ariffin H, Nishida H, Hassan MA, Shirai Y

Biotechnol J, 2010 May;5(5):484-92.

PMID: 20408140 DOI: 10.1002/biot.200900293

Chemical recycling of bio-based polymers polyhydroxyalkanoates (PHAs) by thermal degradation was investigated from the viewpoint of biorefinery. The thermal degradation resulted in successful transformation of PHAs into vinyl monomers using alkali earth compound (AEC) catalysts. Poly(3-hydroxybutyrate-co-3-hydroxyvalerate)s (PHBVs) were smoothly and selectively depolymerized into crotonic (CA) and 2-pentenoic (2-PA) acids at lower degradation temperatures in the presence of CaO and Mg(OH)(2) as catalysts. Obtained CA from 3-hydroxybutyrate sequences in PHBV was copolymerized with acrylic acid to produce useful water-soluble copolymers, poly(crotonic acid-co-acrylic acid) that have high glass-transition temperatures. The copolymerization of CA derived from PHA pyrolysis is an example of cascade utilization of PHAs, which meets the idea of sustainable development.

Li-Fraumeni syndrome in a Malaysian kindred

Ariffin H, Martel-Planche G, Daud SS, Ibrahim K, Hainaut P

Cancer Genet. Cytogenet., 2008 Oct;186(1):49-53.

PMID: 18786442 DOI: 10.1016/j.cancergencyto.2008.06.004

We report on a Malaysian kindred with Li-Fraumeni syndrome. The proband was an 8-year-old girl who presented with embryonal rhabdomyosarcoma of the trunk at the age of 8 months and developed a brain recurrence at the age of 7 years, which was 5 years after remission. A younger sister later developed adrenocortical carcinoma at the age of 6 months. Their mother and maternal grandmother were diagnosed with breast cancer at the ages of 26 and 38 years, respectively. TP53 mutation detection in this family revealed a duplication of a GGCGTG motif starting at nucleotide 17579 in exon 10, resulting in an in-frame insertion of two amino acids between residues 334 and 336 in the tetramerization domain of the p53 protein. This mutation was found in the proband and her affected sister as well as her mother. In addition, the mutation was detected in two other siblings (a brother aged 3 years and a sister aged 18 months) who have not yet developed any malignancy. Sequencing of TP53 in the father and two other asymptomatic siblings revealed wild-type TP53. To our knowledge, this is a first report of a Li-Fraumeni syndrome family in Southeast Asia.

Cefepime monotherapy for treatment of febrile neutropenia in children

Ariffin H, Ai CL, Lee CL, Abdullah WA

J Paediatr Child Health, 2006 Dec;42(12):781-4.

PMID: 17096713

Empirical therapy for children with febrile neutropenia has traditionally consisted of combination antibiotics, usually a beta-lactam and an aminoglycoside. However, recent trends and international guidelines have now made monotherapy a feasible option in the management of this group of patients. We prospectively evaluated the efficacy and safety of cefepime monotherapy in our population of paediatric cancer patients with febrile neutropenia.

Pediatric Vaginal Yolk Sac Tumor: Reappraisal of Treatment Strategy in a Rare Tumor at a Unique Location

Rajagopal R, Ariffin H, Krishnan S, Abdullah WA, Lin HP

J Pediatr Hematol Oncol, 2015 Jul;37(5):391-5.

PMID: 25929614 DOI: 10.1097/MPH.0000000000000344

Review of the management of 6 young girls with vaginal yolk sac tumor over 25 years showed that the α-fetoprotein levels normalized in 5/6 within 4 cycles of primary cisplatin, bleomycin, etoposide (PEB)/carboplatin, etoposide, bleomycin (JEB)/cisplatin, vinblastine, bleomycin (PVB) chemotherapy. Radioimaging revealed residual tissue but viable tumor was found in only 1 of 2 biopsied. Resection/biopsy is necessary to avoid giving additional primary chemotherapy or to identify patients who need different treatment. If markers do not decay appropriately, PEB/JEB/PVB chemotherapy should not be continued. Taxol-containing salvage chemotherapy regimens, adjuvant modern radiotherapeutic treatment, and fertility-saving curative surgery should then be considered. Despite having mostly advanced disease, 5/6 patients were cured, 2 with chemotherapy alone.

Single-daily ceftriaxone plus amikacin versus thrice-daily ceftazidime plus amikacin as empirical treatment of febrile neutropenia in children with cancer

Ariffin H, Arasu A, Mahfuzah M, Ariffin WA, Chan LL, Lin HP

J Paediatr Child Health, 2001 Feb;37(1):38-43.

PMID: 11168867

OBJECTIVE: Empirical antibiotic treatment for febrile neutropenic patients has been the mainstay of treatment for many years. Beta-lactam antibiotics and aminoglycosides have been the most frequently used drug combination. The purpose of this study was to evaluate the efficacy, safety, tolerance and costs of single-daily ceftriaxone plus amikacin versus thrice-daily dose of ceftazidime plus amikacin.

METHODOLOGY: One hundred and ninety-one episodes of fever and neutropenia in 128 patients from October 1997 to December 1998 were included in a prospective, open-label, single-centre study. Patients were randomly assigned to either treatment group and evaluated as successes or failures according to defined criteria. Daily assessments were made on all patients and all adverse events recorded. Univariate and multivariate analysis of outcomes and a cost analysis were carried out.

RESULTS: There were 176 evaluable patient-episodes with 51.1% in the single-daily ceftriaxone-amikacin group and 48.9% in the ceftazidime-amikacin group. There were 50 positive blood cultures: 12 Gram-positive bacteria, 33 Gram-negative bacteria and five fungi. Pseudomonas aeruginosa (P. aeruginosa) accounted for 14% of total isolates. The overall success rate was 55.5% in the ceftriaxone group compared to 51.2% in the ceftazidime group (P = 0.56). Mean time to defervescence was 4.2 days in the single-daily group and 4.3 days in the thrice-daily group. There were nine infection-related deaths; five in the single-daily ceftriaxone group. The daily cost of the once-daily regime was 42 Malaysian Ringgit less than the thrice-daily regime. There was a low incidence of adverse effects in both groups, although ototoxicity was not evaluable.

CONCLUSIONS: The once-daily regime of ceftriaxone plus amikacin was as effective as the 'standard' combination of thrice-daily ceftazidime and amikacin with no significant adverse effects in either group. The convenience and substantial cost benefit of the once-daily regime will be particularly useful in developing countries with limited health resources and in centres with a low prevalence of P. aeruginosa.

Fulltext Providing a cure for beta thalassaemia major

Chan LL, Lin HP, Ariffin WA, Ariffin H

Med J Malaysia, 2001 Dec;56(4):435-40.

PMID: 12014762

The current treatment options for beta thalassaemia major patients include conservative treatment with blood cell transfusions and iron chelation or stem cell transplantation. Regular blood transfusions inevitably lead to multi-organ haemosiderosis and are attended by risks of blood-borne infections. Results from stem cell transplantation are good and suggest that this should be offered as first line therapy when a matched sibling donor is available because the patient is often cured and able to live a normal life. Of 38 Malaysian children who underwent bone marrow or cord blood transplantations using matched sibling donors, 29 (76%) are now cured.

Fulltext Infantile myofibromatosis

Ariffin H, Teh KH, Looi LM, Ariffin WA, Lin HP

Med J Malaysia, 2001 Dec;56(4):497-9.

PMID: 12014771

Infantile myofibromatosis (IMF) is a rare tumour with a wide spectrum of disease activity ranging from a solitary cutaneous nodule through to a multicentric form with widespread visceral involvement. It is characterised by its unique ability to spontaneously regress and has a typical histological appearance of actin-positive fibroblasts arranged in whorls or fascicles and vessels in a pericytomatous pattern. A male infant with multiple lesions involving the subcutaneous tissue and bone from birth is described and followed-up for two years. Treatment of IMF is dependent on the location of the tumour/s with surgery or chemotherapy reserved for rapidly progressive or symptomatic disease. However, due to the low rate of recurrence and the possibility of spontaneous tumoral regression, therapeutic abstention, as practised in our patient, is justified.

Fulltext Treating high risk childhood solid tumours with autologous peripheral blood stem cell transplantation--early experience in University Hospital, Kuala Lumpur

Chan LL, Lin HP, Ariffin WA, Ariffin H, Saw MH

Med J Malaysia, 1999 Jun;54(2):175-9.

PMID: 10972026

Although survival rates for childhood cancers have improved steadily over the past two decades, the outcome for advanced stage solid tumours remains poor. Many of these tumours are chemosensitive but most chemotherapeutic regimens are limited by their haematological toxicities. Much attention is now focused on mega-dose chemotherapy followed by stem cell rescue in the treatment of disseminated neuroblastoma, rhabdomyosarcoma, germ cell tumour and brain tumours. There is a preferential shift towards peripheral blood stem cell transplantation instead of bone marrow transplantation because of its advantages of faster engraftment, decreased transfusion and antibiotic usage and shortened hospitalisation. This mode of therapy is dependent on technologies including peripheral blood stem cell harvesting, cell cryopreservation and thawing. These technologies were recently made available in Malaysia and we report our early experience.

Streptokinase infusion for asparaginase-induced arterial thrombosis

Omar KZ, Ariffin H, Abdullah WA, Chan LL, Lin HP

Med. Pediatr. Oncol., 2000 May;34(5):377-8.

PMID: 10797367

Fulltext Second malignant neoplasms: an increasingly recognized complication of childhood cancer

Ariffin H, Ariffin WA, Chan LL, Lam SK, Lin HP

Med J Malaysia, 1997 Jun;52(2):174-7.

PMID: 10968078

Second malignant neoplasms (SMN) are an increasingly recognized late complication seen in childhood cancer survivors. A total of 3 cases of SMN have been found in the Department of Paediatrics, University Hospital Kuala Lumpur after a 15-year experience of treating childhood malignancies. Two cases are described here. The first developed abdominal non-Hodgkin's lymphoma 3 years after undergoing an allogeneic bone marrow transplant for second relapse of acute lymphoblastic leukaemia, while the second child developed myeloid leukaemia two years after completing treatment for acute lymphoblastic leukaemia. Progress in the management of childhood cancer in Malaysia and the availability of bone marrow transplantation facilities have increased the number of childhood cancer survivors; leading to increased incidence of SMN.

Belief in traditional healers amongst Malaysian parents of children with cancer

Ariffin H, Abdullah WA, de Bruyne J, Lee CL, Peng LH

J Trop Pediatr, 1997 12;43(6):375-6.

PMID: 9476465 DOI: 10.1093/tropej/43.6.375

Antibiotic resistance patterns in nosocomial gram-negative bacterial infections in units with heavy antibiotic usage

Ariffin H, Navaratnam P, Kee TK, Balan G

J Trop Pediatr, 2004 Feb;50(1):26-31.

PMID: 14984166

The pattern of antibiotic resistance amongst gram-negative bacteria (GNB) in paediatric units, which have heavy empirical usage of broad-spectrum antibiotics, was studied prospectively over a 6-month period. A total of 200 consecutive, non-duplicate gram-negative isolates were obtained from 109 patients admitted to intensive care and oncology units in two hospitals. The commonest isolates were Klebsiella spp (36.5 per cent) and Pseudomonas (20.0 per cent). The isolates showed lower susceptibility rates to the third-generation cephalosporins (47-62 per cent) compared with cefepime (91 per cent), imipenem (90 per cent) and ciprofloxacin (99 per cent). Fifty-four (52.8 per cent) Klebsiella and Escherichia coli isolates were determined to be extended-spectrum beta-lactamase (ESBL) producing strains. Antibiotics found to be effective against ESBL-producers were imipenem and ciprofloxacin. The high resistance rate amongst GNB to third-generation cephalosporins is a likely consequence of heavy empirical usage of this group of antibiotics. The carbapenems and quinolones remain useful agents in the management of patients admitted to these units.

Secondary B-cell acute lymphoblastic leukemia following Wilms' tumor: clinical and in vitro chemosensitivity studies

Ariffin H, Muthukkumaran T, Stanslas J, Sabariah AR, Veerasekaran N, Lin HP

Leuk Lymphoma, 2005 Aug;46(8):1233-7.

PMID: 16085568

We report the clinical features and in vitro chemosensitivity assay findings of a 13-year-old girl who developed secondary B-cell acute lymphoblastic leukemia (ALL) 7 years after a diagnosis of Wilms' tumor. The patient was treated using the Berlin - Frankfurt - Muenster (BFM) ALL chemotherapy protocol with poor response to initial therapy before succumbing to sepsis. An in vitro chemosensitivity assay on her peripheral blood lymphoblasts was performed while she was undergoing induction therapy and showed a high level of resistance to drugs commonly used for ALL therapy, e.g. steroids, anthracyclines, vincristine and L-asparaginase. The mechanism of chemoresistance was not elicited, but was probably not related to P-glycoprotein (P-gp) over-expression. We believe that the in vitro chemosensitivity assay is a good indicator of cellular response to chemotherapy and may provide reliable information for the basis of the selection of drugs to be used for the treatment of similarly rare patients rather than relying on "standard" protocols.

Focal Segmental Membranoproliferative Glomerulonephritis: A Histological Variant of Denys-Drash Syndrome

Karmila AB, Yap YC, Appadurai M, Oh L, Fazarina M, Abd Ghani F, et al.

Fetal Pediatr Pathol, 2021 Apr;40(2):113-120.

PMID: 31707902 DOI: 10.1080/15513815.2019.1686788

Introduction: Denys-Drash Syndrome (DDS) consists of a triad of pseudohermaphroditism, Wilms'tumor and nephropathy. This condition may manifest as a complete triad or in an incomplete form; with either one or a combination of the above features. The characteristic glomerular abnormality in DDS is diffuse mesangial sclerosis (DMS).Case report: We report two cases of DDS with focal membranoproliferative glomerulonephritis (MPGN). Both of our cases were males with ambiguous genitalia. They had a similar heterozygous germline mutation in exon 9 of WT1, c.1180C>T, p.R394W; a known mutation hotspot for DDS. Case 1 had nephropathy at the age of 4 years and Case 2 at 2.5 years with different rates of progression to end-stage renal failure. Conclusion: Our findings, in combination with other reports, illustrate the clinicopathological heterogeneity of DDS. There are no universal recommendations for optimal management of patients with DDS due to the inability to accurately predict affected individuals' progress.

Mature B-cell acute lymphoblastic leukaemia associated with a rare MLL-FOXO4 fusion gene

Lim L, Chen KS, Krishnan S, Gole L, Ariffin H

Br J Haematol, 2012 Jun;157(6):651.

PMID: 22429121 DOI: 10.1111/j.1365-2141.2012.09091.x

Isolation and characterization of microcrystalline cellulose from roselle fibers

Kian LK, Jawaid M, Ariffin H, Alothman OY

Int J Biol Macromol, 2017 Oct;103:931-940.

PMID: 28549863 DOI: 10.1016/j.ijbiomac.2017.05.135

In this study, microcrystalline cellulose (MCC) was extracted from roselle fiber through acid hydrolysis treatment and its properties were compared with those of commercially available MCC. The physicochemical and morphological characteristics, elemental composition, size distribution, crystallinity and thermal properties of the obtained MCC were analyzed in this work. Fourier transform infrared spectroscopy (FTIR) analysis provided clear evidence that the characteristic peak of lignin was absent in the spectrum of the MCC prepared from roselle fiber. Rough surface and slight aggregation of MCC were observed by scanning electron microscopy (SEM). Energy dispersive X-ray (EDX) analysis showed that pure MCC with small quantities of residues and impurities was obtained, with a similar elemental composition to that of commercial MCC. A mean diameter of approximately 44.28μm was measured for MCC by using a particle size analyzer (PSA). X-ray diffraction (XRD) showed the crystallinity increased from 63% in roselle pulp to 78% in roselle MCC, the latter having a slightly higher crystallinity than that of commercial MCC (74%). TGA and DSC results indicated that the roselle MCC had better thermal stability than the roselle pulp, whereas it had poorer thermal stability in comparison with commercial MCC. Thus, the isolated MCC from roselle fibers will be going to use as reinforcing element in green composites and may be a precursor for future roselle derived nanocellulose, and thus a promising subject in nanocomposite research.

Doing less, accomplishing more for childhood acute lymphoblastic leukaemia (ALL)

Yeoh AEJ, Oh BL, Ariffin H

Br J Haematol, 2021 08;194(4):661-662.

PMID: 34231199 DOI: 10.1111/bjh.17556

Fulltext HLA-Haploidentical Family Donors: The New Promise for Childhood Acute Lymphoblastic Leukaemia?

Ab Rahman S, Matic T, Yordanova M, Ariffin H

Front Pediatr, 2021;9:758680.

PMID: 35127585 DOI: 10.3389/fped.2021.758680

Allogeneic haematopoietic stem cell transplantation (HSCT) is indicated in children with high-risk, relapsed or refractory acute lymphoblastic leukaemia (ALL). HLA-matched grafts from cord blood and stem cell repositories have allowed patients without suitable sibling donors to undergo HSCT. However, challenges in procuring matched unrelated donor (MUD) grafts due to high cost, ethnic disparity and time constraints have led to the exponential rise in the use of stem cells from human leukocyte antigen (HLA)-haploidentical family donors. Whilst HLA-haploidentical HSCT (hHSCT) performed in adult patients with acute leukaemia has produced outcomes similar to MUD transplants, experience in children is limited. Over the last 5 years, more data have emerged on hHSCT in the childhood ALL setting, allowing comparisons with matched donor transplants. The feasibility of hHSCT using adult family donors in childhood ALL may also address the ethical issues related to selection of minor siblings in matched sibling donor transplants. Here, we review hHSCT in paediatric recipients with ALL and highlight the emergence of hHSCT as a promising therapeutic option for patients lacking a suitable matched donor. Recent issues related to conditioning regimens, donor selection and graft-vs.-host disease prophylaxis are discussed. We also identify areas for future research to address transplant-related complications and improve post-transplant disease-free survival.

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