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Fulltext Interstitial lung disease in children--a report of four cases

Norzila MZ, Azizi BH, Deng CT, Zulfiqar A

Med J Malaysia, 1997 Dec;52(4):429-32.
PMID: 10968122

Interstitial lung disease (ILD) is very rare in children. In the majority of cases the aetiology is unknown. Very little is known about the clinical course of this condition in children. Prognosis may be influenced by sex, age of onset of symptoms, radiographic features, presence of right ventricular hypertrophy and histopathology. We report our experience in managing four children with interstitial lung disease. All these children presented in early infancy with cough, respiratory distress, cyanosis and failure to thrive. Three of these children had finger clubbing and right ventricular hypertrophy. All patients received oral steroids. Chloroquine was added in two patients who showed no response. A trial of oral cyclophosphamide was started in one patient who failed with both drugs. One child is oxygen independent while another is on home oxygen therapy. The other two patients eventually died.
Fulltext Iatrogenic ureter injuries: eleven years experience in a tertiary hospital

Mahendran HA, Singam P, Ho C, Goh EH, Tan GH, Zuklifli MZ

Med J Malaysia, 2012 Apr;67(2):169-72.
PMID: 22822637 MyJurnal

Iatrogenic ureteric injuries are rare complications of abdomino-pelvic surgery but associated with high morbidity from infection and possible loss of renal function. A successful repair is related to the timing of diagnosis, site of injury and method of repair. This study was a retrospective review of outcomes of iatrogenic ureteric injury and factors contributing to successful operative repair. Twenty consecutive cases referred to the Urology Unit of the UKM Medical Center during an 11-year period from 1998 to 2009 were reviewed. Thirteen patients were diagnosed intraoperatively and underwent immediate repair. Seven patients had delayed diagnosis but also underwent immediate repair. In our series, there was no significant difference in outcome between injuries diagnosed intraoperatively versus injuries with delayed diagnosis. There was significant difference in the outcomes between methods of ureteric repair where ureter reimplantation via psoas hitch or Boari flap yielded better results than primary end-to-end anastomosis Three patients suffered loss of renal function from unsuccessful ureter repair. We conclude that all iatrogenic ureteric injury should be repaired immediately in the absence of overt sepsis. Ureter reimplantation using a Boari flap or psoas hitch is preferred to the end-to-end anastomosis especially when there is delayed diagnosis
The Cobblestone Heart

Tan WJ, Suz CS, Azza O, Zuki M

Med J Malaysia, 2021 03;76(2):241-244.
PMID: 33742636

Sarcoidosis is a chronic, multisystem disorder. A 38 years old lady presented at Hospital Raja Perempuan Zainab II, Kota Bharu ,Malaysia with cough and breathless for 2 months and constitutional symptoms of weight loss and loss of appetite. She was initially treated as smear negative pulmonary tuberculosis for 5 months. However, her clinical condition deteriorated with worsening New York Heart Association (NYHA) class 1 to class 3. Subsequently, workout of computed tomography( CT) thorax showed multiple perilymphatic distribution of nodules and multiple mediastinal lymphadenopathy coupled with pleura biopsy showed non caseating granuloma and cardiac magnetic resonance imaging (MRI) with positive late gadolinium enhancement revised the diagnosis of pulmonary sarcoidosis with cardiac involvement. Patient's functional status and cough improved with immunosuppresant was given in tapering dose fashion.
Fulltext Early outcome of reamed interlocking nail for non-union of tibia

Faisham WI, Sulaiman AR, Sallehuddin AY, Zuhmi W

Med J Malaysia, 2006 Aug;61(3):339-42.
PMID: 17240586 MyJurnal

Aseptic non-union is a major problem following complicated fracture tibia, which carries significant morbidity and prolonged course of treatment. Plate fixation and autogenous bone grafting has been established as a method of treatment. However the risk of infection, implant failure and donor site morbidity are high. We reviewed twelve consecutive cases of established non-union tibia treated by closed reamed interlocking nail in our centre. All patients had clinical and radiological union at three months. Three patients were complicated with infection and one required removal of implant and re-reaming to eradicate infection. Reamed interlocking nailing is an alternative treatment for selected non-union of fracture tibia with promising results.
Fulltext Colon cancer recurrence mimicking renal cell carcinoma

Yusoff AR, Aripin YM, Zuhanis AH

Med J Malaysia, 2014 Dec;69(6):286-7.
PMID: 25934963 MyJurnal

No abstract available.
Fulltext Haemoglobin Sickle D Punjab: - a case report

Rahimah A, Syahira Lazira O, Siti Hida HM, Faidatul Syazlin AH, Nur Aisyah A, Nik Hafidzah NM, et al.

Med J Malaysia, 2014 Feb;69(1):42-3.
PMID: 24814631 MyJurnal

Haemoglobin S D-Punjab is a rare compound heterozygous haemoglobinopathy characterised by the presence of two β globin gene variants: Β6(GAG→GTG) and Β121(GAA→CAA). These patients' clinical and haematological features mimic haemoglobin S disease. We describe the first case of doubly heterozygous HbSD-Punjab from Malaysia managed with regular blood transfusion at the age of one. This case highlights the propensity for occurrence of rare phenotypes within our multi-ethnic population and emphasises the importance of accurate genotyping to avoid erroneous counselling, and to plan an effective patient management strategy before complication evolves.
Fulltext Distribution of alpha thalassaemia in 16 year old Malaysian Students in Penang, Melaka and Sabah

Rahimah AN, Nisha S, Safiah B, Roshida H, Punithawathy Y, Nurul H, et al.

Med J Malaysia, 2012 Dec;67(6):565-70.
PMID: 23770946 MyJurnal

OBJECTIVES: Alpha thalassaemia is wide spread in Malaysia and is a public health problem. This study aimed to describe the carrier frequencies of α‒thalassaemia and its distribution among major ethnic groups in three states of Malaysia.

METHODS: Educational forums were organised and study was explained to students from three schools. Students were invited to take part in the screening with parent consent. A total of 8420 adolescent students aged 16 years volunteered to participate in the study. Peripheral blood samples were analysed for complete blood counts, haemoglobin quantification and typing, and serum ferritin levels. Genomic DNA was used for screening alpha thalassaemia alleles by PCR based molecular methods.

RESULTS: We identified seven α‒globin gene defects in 341 (4.08%) students: amongst them α(+)‒ and α(0)‒thalassaemias were detected in 232 (2.77%) and 107 (1.28%) students respectively. Genotype ‒α(3.7)/αα was the most prevalent among sub-populations of Malay, indigenous communities of Sahab and Indian, while ‒‒(SEA)/αα deletion is more prevalent in Malaysian Chinese. It is estimated that 63 pregnancies annually are at risk of Hb Bart's hydrops fetalis.

CONCLUSIONS: We have demonstrated the prevalence and mutation patterns of α‒thalassaemia in the 16 year olds in three states of Malaysia. High α(0)‒thalassaemia deletions amongst the study subjects place these carriers at an increased risk of conceiving fetuses with HbH disease and Hb Bart's hydrops fetalis should they choose another heterozygous partner. It is therefore highly recommended to institute community screening programmes and provide prospective carriers with genetic counselling to help them make informed choices.
Fulltext Characterization and safety assessment of bioengineered limbal epithelium

Lim MN, Umapathy T, Baharuddin PJ, Zubaidah Z

Med J Malaysia, 2011 Oct;66(4):335-41.
PMID: 22299553 MyJurnal

Transplantation of cultivated limbal epithelium on substrates such as amniotic membrane is an established treatment for severe ocular surface disease with limbal stem cell deficiency. In this study, we adapted an established method to generate sheets of limbal epithelium on amniotic membrane and characterized the cells contained in these sheets and tested them for safety with regard to microbial contamination. Human limbal biopsies were cultivated on denuded amniotic membranes. After three weeks of culture, the phenotypes of cultivated cells were analyzed by immunohistochemistry and real-time RT-PCR for the expression of a panel of specific markers. Cultivated limbal epithelial cell sheets were also analyzed by scanning (SEM) and transmission (TEM) electron microscopy. Sterility tests and mycoplasma assays were conducted for the safety of product. A confluent layer of polygonal cells was formed in 2 weeks and 1-3 stratified layer of cells were observed after three weeks of culture. Cultivated cells were positive for p63, K3, K19, and involucrin but negative for K14, integrin alpha9 and ABCG2 when analyzed by immunohistochemistry. Expression of molecular markers was detectable with real-time RT-PCR. SEM showed multilayer of flat squamous polygonal epithelial cells. Desmosomal and hemidesmosomal attachments were evident. Our study showed that cultivated limbal epithelium consists of limbal progenitors as well as differentiated corneal epithelial cells. SEM and TEM analysis showed cultivated cells demonstrated typical features of corneal epithelium. The risk of contamination is low and can be prevented by culturing the cells in a clean room facility complying to Good Manufacturing Practice standard.
Fulltext A rare case of alpha-thalassaemia intermedia in a Malay patient double heterozygous for alpha(+)-thalassaemia and a mutation in alpha1 globin gene CD59 (GGC --> GAC)

George E, Jama T, Azian AS, Rahimah A, Zubaidah Z

Med J Malaysia, 2009 Dec;64(4):321-2.
PMID: 20954559

A rare case of thalassaemia-intermedia involving a non-deletion alpha thalassemia point mutation in the alpha1-globin gene CD59 (GGC --> GAC) and a deletion alpha+ (-alpha(3.7)) thalassaemia in which use of high performance liquid chromatography (HPLC) C-gram Hb subtype profile and DNA molecular analysis helped establish the diagnosis.
Fulltext Serum high-sensitivity C-reactive protein and lipoprotein(a) levels: a comparison between diabetic and non-diabetic patients with coronary artery disease

Shahid HS, Kurdi MI, Zohair AA

Med J Malaysia, 2011 Jun;66(2):113-6.
PMID: 22106689

OBJECTIVE: The aim of this study was to compare high-sensitivity C-reactive protein (hsCRP) and Lipoprotein(a) levels [Lp(a)] levels between diabetic and non-diabetic patients with coronary artery disease (CAD).
STUDY DESIGN: Cross sectional Study.
PLACE AND DURATION OF STUDY: This study was conducted in the department of Physiology of College of Medicine & King Khalid University Hospital, King Saud University, Riyadh between August 2006 and December 2007.
METHODS: One hundred and three individuals with CAD and 30 healthy individuals matched for age and BMI were studied. CAD patients were divided into two groups based on presence (n=62) and absence (n=41) of type 2 diabetes mellitus. Overnight fasting blood samples were collected, and analyzed for total cholesterol (TC), Triglycerides (TG), Low density Lipoprotein (LDL) and High density lipoprotein (HDL), Lp(a) and hsCRP. Data about CAD severity was obtained from medical records.
RESULTS: Both groups of CAD without and with DM had significantly higher levels of Lp(a) [mg/dl] (25.58 +/- 25.99, 25.90 +/- 24.67 respectively) and hsCRP [mg/dl] (0.52 +/- 0.71, 0.82 +/- 0.78 respectively) when compared with healthy control subjects (Lp(a) =16.93 +/- 15.34 & hsCRP=0.27 +/- 0.21) [p<0.05]. Lp(a) levels between the two CAD groups were non significant. While, hsCRP levels were significantly high in CAD with DM compared to those without DM [p<0.05]. Gensini Score of CAD severity was also higher in CAD with DM [67.60 +/- 45.94] than those without DM [52.05 +/- 42.27, p<0.05].
CONCLUSION: Elevated Lp(a) and hsCRP levels are associated specifically with angiographically defined CAD. However, hsCRP elevation but not Lp(a) is also associated with CAD in type 2 diabetes mellitus. Measurement of hsCRP and Lp(a) may be considered optional markers for better prediction of cardiovascular risk.
Sturge-Weber syndrome and variability of clinical presentation

Zeka N, Zeka B, Gerguri A, Bejiqi R, Retkoceri R, Maloku A, et al.

Med J Malaysia, 2023 Mar;78(2):145-148.
PMID: 36988522

INTRODUCTION: Sturge-Weber syndrome (SWS) is a congenital syndrome characterised by intellectual disability, glaucoma, a characteristic port-wine stain on the skin around the route of the ophthalmic branch of the trigeminal nerve and the affection of the leptomeninges in the brain in the form of abnormal capillary venous vessels. The aim of this study is to look at the clinical features as well as the correlation of SWS with other comorbidities in hospitalised children.
MATERIALS AND METHODS: Records of admitted children over the period 2000-2019 were retrospectively studied. Epidemiological variables, gender and age at the time of diagnosis, changes in the skin, central nervous system affection and ophthalmological changes were analysed and recorded.
RESULTS: Eleven cases of SWS were identified and included in the study. Age at the time of diagnosis ranged from 1 to 36 months. EEG showed specific grapho-elements, with partial seizures presenting in five cases out eight total cases with epilepsy. Ophthalmological complications were common, with glaucoma and choroidal haemangioma being the most common. Cognitive problems were found in seven cases, headache in eight cases and hemiparesis in four.
CONCLUSION: SWS is associated with other medical conditions. The study has described some of the features of SWS and found its correlation with epilepsy and other neurological problems, glaucoma, headache, hemiparesis and cognitive problems.
Fulltext Screening of dystrophin gene deletions in Malaysian patients with Duchenne muscular dystrophy

Marini M, Salmi AA, Watihayati MS, SMardziah MD, Zahri MK, Hoh BP, et al.

Med J Malaysia, 2008 Mar;63(1):31-4.
PMID: 18935728 MyJurnal

Duchenne Muscular Dystrophy (DMD) is an X-linked recessive genetic disorder characterized by rapidly progressive muscle weakness. The disease is caused by deletion, duplication or point mutation of the dystrophin gene, located on the X chromosome (Xp21). Deletion accounts for 60% of the mutations within the 79 exons of the dystrophin gene. Seven exons (43, 44, 45, 46, 49, 50, and 51) were found to be most commonly deleted among the Asian patients. To detect the frequency of deletion of these 7 exons in Malaysian DMD patients, we carried out a molecular genetic analysis in 20 Malaysian DMD patients. The mean age of initial presentation was 60 months (SD 32 months, range 5-120 months). Fourteen patients were found to have deletion of at least one of the seven exons. The remaining six patients did not show any deletion on the tested exons. Deletions of exons 49, 50 and 51 were the most frequent (71.43%) and appear to be the hot spots in our cohort of patients.
Cimetidine for chronic duodenal ulceration: short term clinical trial

Chelvam P, Zhmad Z

Med J Malaysia, 1979 Mar;33(3):274-6.
PMID: 392272
Fulltext Update on the management of peripheral arterial disease (PAD)

Liew NC, Moissinac K, Lee L, Gee T, Zezeman RB

Med J Malaysia, 2011 Oct;66(4):386-8; quiz 389.
PMID: 22299571 MyJurnal

There has been much progress made in the management of peripheral arterial disease (PAD) in the past two decades. Progress in the understanding of the endothelial-platelet interaction during health and disease state have resulted in better antiplatelet drugs that can prevent platelet aggregation, activation and thrombosis during angioplasty and stenting. Collaborative effort by different international societies has resulted in a consensus guideline that recommends the modality of intervention in certain disease states. Progress in perioperative care has reduced the morbidity and mortality associated with peripheral vascular reconstruction surgery. Nevertheless, the advances in percutaneous peripheral intervention (PPI) have made a paradigm shift in the current management of patients. The procedure is safe and effective and is emerging as the first choice revascularization procedure.
Biomimetic structures for implants and tissue engineering scaffolds

Ambrosio L, Battista S, Borzacchiello A, Borselli C, Causa F, De Santis R, et al.

Med J Malaysia, 2004 May;59 Suppl B:71-2.
PMID: 15468824
Fulltext Posterior intraorbital metallic foreign body: a case discussion

Vinodh VP, Sellamuthu P, Harun RH, Zenian MS

Med J Malaysia, 2014 Apr;69(2):89-91.
PMID: 25241819 MyJurnal

Intraorbital foreign body (IOFB) has been a rare phenomenon in the cases of gunshot wounds and always represents a dilemma in medical management. In Sabah, this scenario is becoming common as there is still certain population in the interiors who owns self made guns for hunting. They either present with self inflicted gunshot injuries or after being mistakenly shot while hunting. There are very few articles on this topic especially when it is located posteriorly in the orbit and occurs without visual impairment. This case reports the challenges faced in the management of the patient with a posteriorly located metallic IOFB. The appropriate management of the patient is discussed based on several international literatures. Author describes a 37 years old male farmer, who was referred from a nearby district hospital after he sustained gunshot injuries to his face and scalp. Urgent computed tomography (CT) scan showed that bullet pellets were at the left orbital floor, the left mandible and the left frontal bone. All bullet pellets were located extracranially only without intracranial involvement. Patient clinically does not have any neurologic deficit and without any visual impairment, thus he refused any surgical intervention. As there is no proper guideline in managing such cases, decision was made based on evidences from international literatures. It was concluded that metallic IOFB located in the posterior orbit may be conservatively managed with observation and regular follow-ups as they are well-tolerated and does not cause much impact on visual deterioration unless inflammation, infection, optic neuropathy or functional deficit occurs. This avoids unnecessary surgery and prevents risk of iatrogenic injury to the eye.
Group A Streptococcus puerperal sepsis with invasive neonatal infection: A fatal case

Siti Hafsyah MH, Ibrahim NR, Noraida R, Ismail AA, Anani Aila MZ, Hajissa K, et al.

Med J Malaysia, 2021 Sep;76(5):731-733.
PMID: 34508383

Neonatal invasive Group A Streptococcus (GAS) infection is a rare occurrence nowadays. Prior maternal vaginal colonization is an important factor in early neonatal disease. We report a case of invasive and fatal infection in a neonate. At Day 1 of life, a term baby was found to be lethargic, with poor feeding, and later became unresponsive. Consequently, the baby was immediately brought to the Emergency Department of Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan via ambulance. Despite the active resuscitation efforts in the hospital, the baby died. An autopsy was carried out to determine the cause of death. The mother was noted to have puerperal pyrexia secondary to vaginal discharge. Her high vaginal swab culture was positive for GAS. GAS was also isolated from the intracardiac blood, pleural fluid, peritoneal fluid, and umbilical swab of the baby, giving evidence to the aetiology of the mortality. Vaginal colonization of GAS is an important factor for high morbidity and mortality for both mother and infant due to its invasiveness and virulence.
Fulltext Cholinergic deregulation in traumatic brain injury could be a pathophysiology-related biphasic epiphenomenon

Bimpis A, Baillie GS, Zarros A

Med J Malaysia, 2014 Jun;69(3):148.
PMID: 25326361

No abstract available.
Fulltext The Prevalence of Sensorineural Hearing Loss in β-thalassaemia patient treated with Desferrioxamine

Kong MH, Goh BS, Hamidah A, Zarina AL

Med J Malaysia, 2014 Feb;69(1):9-12.
PMID: 24814621 MyJurnal

OBJECTIVE: This study aimed to evaluate the prevalence of sensorineural hearing loss (SNHL) in β-thalassaemia patients treated with Desferrioxamine (DFO) and determine the correlation of SNHL with average daily DFO dosage, serum ferritin level and Therapeutic index (T.I).

METHODS: This is a cross sectional descriptive study carried out for a period of 14 months and 54 patients were recruited. The recruited patients are transfusion dependant β- thalassaemia patient aged 3 years and above treated with DFO. An interview, clinical examination and hearing assessment, which included tympanogram, and Pure Tone Audiometry (PTA) or behaviour alaudiometry were performed. The data on age started on DFO, average daily DFO, duration of DFO intake, serum ferritin past 1 year and Therapeutic Index (T.I) were obtained from patients' case notes.

RESULTS: The prevalence of SNHL was 57.4% and majority has mild hearing loss (93.6%). Fourteen patients (25.9%) have bilateral ear involvement and as many as 17 patients (31.5%) have SNHL in either ear. A total of 23 patients (42.6%) have normal hearing level. Although the prevalence of SNHL was 57.4%, only a small percentage of the patient noticed and complained of hearing loss (11.1%). There is no association between age started on DFO, average daily DFO and duration of DFO intake with normal hearing group and those patients with SNHL. Positive correlation was seen between average daily DFO with 2000 and 4000Hz on PTA in the left ear and between serum ferritin level past 1 year with 4000 and 8000Hz in the right ear and 8000Hz in the left ear. No significant correlation was seen between T.I on PTA.

CONCLUSION: The prevalence of SNHL from hearing assessment is high in β-thalassaemia patients in this study. However, it is manifested clinically in a smaller percentage. We suggest a baseline hearing assessment should be carried on all β-thalassaemia patients prior to DFO chelation therapy.
Fulltext A review of cervical cancer research in Malaysia

Zaridah S

Med J Malaysia, 2014 Aug;69 Suppl A:33-41.
PMID: 25417949 MyJurnal

Despite cervical cancer being potentially preventable, it is the second most common cancer among women in Malaysia. One hundred and five articles related to Cervical Cancer were found in a search through a database dedicated to indexing all original data relevant to medicine published in Malaysia between the years 2000-2013. Fifty seven articles were selected and reviewed for the articles' clinical relevance and future research implications. This article reviews the various aspects of cervical cancer in Malaysia, mainly persistent infection of high risk human papillomavirus (HPV), primary prevention (HPV vaccination), screening method (Pap smear issues), and the attitude and knowledge of various groups of Malaysian women that contributed to the failure to reduce the incidence and mortality of cervical cancer. Most of the studies focused on prevention, Pap smear issues, HPV DNA testing, HPV vaccination and various recommendations for prevention of cervical cancer. Secondary prevention by screening is still an important aspect because even with HPV vaccination, screening still plays an important role as vaccination does not cover all high risk HPVs. There is a need to seriously consider a properly organised screening programme, taking into consideration what we already know about the attitude and knowledge of Malaysian women, economic factors and psychosocial issues of the screening method. There is also a large gap in clinical studies on the outcome, management and survival of cervical cancer patients in Malaysia.

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