Skoliosis kongenital adalah perkembangan tulang belakang yang tidak normal yang merangkumi pembentukan sebahagian tulang sahaja, kurangnya pemisahan di antara tulang belakang atau kehilangan bahagian tertentu tulang belakang. Etiologi sebenar skoliosis kongenital masih tidak jelas. Walau bagaimanapun, ia dipengaruhi oleh kecenderungan genetik dan faktor persekitaran. Kami melaporkan siri kes skoliosis kongenital dengan ciri-ciri dismorfik dalam empat orang adik-beradik dan membincangkan mengenai sindrom spondylocostal dysostosis yang mempunyai kaitan dengan skoliosis kongenital. Ciri-ciri dismorfik termasuk hipertelorisme, ‘ptosis’ kedua-dua mata, 'high arch palate', langit-langit yang tinggi dan leher ‘webbed’. Pembedahan instrumentasi tulang belakang dilakukan dalam tiga adik beradik. Semua pesakit pulih dengan baik selepas pembedahan tanpa komplikasi kecederaan saraf. Rawatan susulan pada tahun pertama dan kedua selepas pembedahan menunjukkan tiada perubahan pada kadar lengkung dan tulang belakang telah bercantum.
A cross sectional study was done to determine the prevalence and distribution of human papillomavirus (HPV) genotypes in pre-invasive (cervical intraepithelial neoplasia, grade 3 or CIN 3) and invasive cervical cancer (ICC), in Universiti Kebangsaan Malaysia Medical Centre (UKMMC). A total of 80 paraffin-embedded tumour tissue blocks (20 CIN 3, 60 invasive cancers) between 1999 to 2007 were retrieved from the archives of the Department of Pathology. Patient’s medical records were obtained from the Medical Records Office. Among invasive cancers (n=60), squamous cell carcinoma (SCC) account for 75% and adenocarcinoma 25%. The mean age of cases studied was 52.0 ± 12.2 years and Chinese was the predominant ethnicity (66.3%). Twelve HPV genotypes were identified, namely, HPV 16, 33, 18, 39,52, 45, 58, 59, 31, 35, 6 and 11. The prevalence of HPV was 92.5% with types 16 being the most common (73.8%), followed by types 33 (30%) and 18 (22.5%). A total of 31 cases (38.8%) showed single HPV genotype, while 43 (53.8%) had multiple HPV (two genotypes or more) genotypes. In ICC, HPV 16, followed by types 33, 18, 52 and 39 were the top five common HPV genotypes detected. High prevalence of HPV and multiple HPV infections were major findings among patients with pre-invasive and invasive cervical cancer.
Pengurusan kendiri asma menggunakan pelan tindakan asma secara bertulis (WAAP) penting dalam memastikan pengurusan asma yang berkesan kerana ia mengurangkan kadar kehadiran ke hospital. Kajian ini menilai peningkatan pengetahuan dan kawalan asma menggunakan WAAP di kalangan pesakit asma di Jabatan Kecemasan. Kajian cubaan kawalan rawak ini dijalankan selama 19 bulan di Jabatan Perubatan Kecemasan sebuah hospital universiti. Kajian ini melibatkan pesakit dewasa serangan asma ringan dan sederhana. Pesakit dibahagikan secara rawak kepada dua kumpulan iaitu kumpulan intervensi dan kawalan. Soal selidik pengetahuan penyakit asma diberikan pada pesakit. Pesakit dari kedua kumpulan menerima kaunseling tentang penyakit asma berdasarkan risalah asma yang telah disediakan. Kumpulan intervensi, kemudiannya menerima kaunseling WAAP mengikut keperluan individu tersebut. Kumpulan kawalan menerima kaunseling lisan berdasarkan perawatan. Susulan kajian dijalankan pada 1 dan 3 bulan. Pengetahuan penyakit asma dan kawalan penyakit asma dinilai melalui tinjauan telefon. Lima puluh pesakit telah dibahagikan secara rawak kepada kumpulan intervensi dan kawalan. Setiap kumpulan menerima 25 pesakit. Skor pengetahuan kumpulan intervensi jauh lebih tinggi daripada kawalan (16.88+0.44 vs 13.36+3.72; p19) pada 1 bulan berbanding kumpulan kawalan (20.64+3.26 vs 17.72+4.17). Kedua-dua kumpulan mencapai kawalan yang baik pada 3 bulan. Pengurusan kendiri asma dengan menggunakan WAAP di Jabatan Kecemasan meningkatkan pengetahuan dan kawalan asma di kalangan pesakit yang mendapat serangan asma ringan dan sederhana.
Electrocardiographic abnormalities can be associated with acute pancreatitis. However, data regarding the actual causative factor still remains elusive. Many previous cases were reported on non-specific ST and T wave abnormalities concurrent with acute pancreatitis but rarely with an increasing trend of cardiac markers. We describe the case of a 70-year-old female who presented with one such conundrum. Our patient had typical presentation of acute pancreatitis but had dynamic ECG changes with markedly increased cardiac markers. Subsequently after initiation of treatment for acute pancreatitis and observation for the course of several days, the ECG returned to the baseline as pre admission. This substantiates the fact that acute pancreatitis can mimic both biochemical and electrical manifestation of an acute coronary syndrome. Thus, Emergency Physicians should consider acute pancreatitis as a possible diagnosis in patients who present with abnormal electrocardiograms.
Trauma is thought to complicate 1 in 12 pregnancies. The management of trauma during pregnancy requires special consideration because pregnancy alters maternal physiology and the foetus is a potential collateral victim. The approach of these cases in the setting of the Emergency Department should not only be diagnostic for any foetal injuries but also prognostic for any future undue outcome. Antenatal traumatic brain injury is a rare but real complication of maternal blunt force trauma. Our case involves a 22-year-old primigravida who suffered a motor vehicle accident and on initial assessment revealed normal foetal assessment but subsequently after premature labour revealed a new born with traumatic brain injury. Early ultrasonographic evaluation and observational period with continuous electronic foetal monitoring may improve the detection and emergent treatment in these cases.
Revascularisation and replant surgery occasionally produces sub-optimal results. We present two cases of near total amputation of the digits. The first patient was an 11-year-old boy with a middle finger avulsion injury sustained after the finger was caught in a washing machine with the flexor digitorum profundus still attached. The second patient was a 42-year-old male who sustained a machete attack and had near total amputation of his small and ring fingers with a 2 cm skin bridge still attached. All patients underwent revascularization with repair of only one digital artery for each finger as the opposite artery was non-viable. No digital veins were repaired. Relief of venous congestion was done by pin-prick six times a day up till one week. At one week post-surgery, the fingers were discoloured black but had good pulp turgor and full contour with arterial bleed on pin-prick. Inexperienced surgeons may view this as gangrene and amputate the fingers. However, we continued observation and at one month post-surgery, the black skin had desloughed and the underlying skin was pink.
The presence of thrombus within the inferior vena cava (IVC) is often a sign of advance hepatocellular carcinoma (HCC). Various treatment methods have been described with variable and inconclusive results. Now, the advancement of endovascular approach offers new possibility as a potential treatment modality. We discuss the removal of tumour thrombus with catheter directed mechanical thrombectomy. IVC tumour secondary HCC was removed by AngioJet® rheolytic system (Possis Medical, Minneapolis, MN, USA) with good result. Further work should be encouraged to explore the prospect of this technique with other treatment modalities.
Discontinuing antihistamines for patch testing (PT) in allergic contact dermatitis (ACD) is more conventional than evidence based. Data suggests that non-sedating antihistamines do not interfere with PT. Investigating the effects of sedating antihistamines are more relevant as these are recommended for eczema. We aimed to evaluate the effect of chlorpheniramine on PT, to determine the prevalence of nickel sensitization and common sensitizing allergens. An open labeled cohort study was conducted at two dermatology clinics. Patients indicated for PT underwent standard protocol where antihistamines were discontinued. Patients sensitised to nickel were subjected to a second nickel PT while taking chlorpheniramine. Results were evaluated using the North American Contact Dermatitis Research Group (NACDRG) score, a Mexameter measured erythema and pruritus was assessed using a visual analogue score. A total 82 patients were recruited, 28 (34.1%) were sensitised to nickel. The mean age was 40 ± 17.7 years with 22(26.8%) males and 60 (73.2%) females. Indications for PT included suspected ACD (57.3%), hand and feet eczema (34.1%) and severe eczema with suspected superimposed ACD (6.1%). The commonest sensitizing allergens were methyldibromoglutaronitrile (40.2%) nickel sulphate (34.1%), potassium dichromate (29.3%) and formaldehyde (24.4%). A second PT was performed on 23 patients. There was no difference in the NACDRG score with chlorpheniramine or without chlorpheniramine (p=0.968). Pruritus score was reduced by 1.39 ± 2.9, p=0.031 with chlorpheniramine. The degree of erythema was 611.46 ± 21.59 with chlorpheniramine versus 613.87 ± 27.5 without chlorpheniramine, p=0.671. Chlorpheniramine did not affect PT based on clinical and objective scorings. It has the additional benefit of reducing test-induced itch.
Study site: Dermatology clinics, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur and Hospital Pulau Pinang, Malaysia
Pengetahuan yang baik tentang teknik perahan, penyimpanan dan penggunaan susu perahan ibu adalah sangat penting kepada ibu-ibu yang ingin meneruskan penyusuan ibu selepas kembali bekerja. Objektif kajian ini adalah untuk menilai pengetahuan dan sikap ibu bekerja terhadap perahan, penyimpanan dan penggunaan susu ibu. Kajian hirisan lintang ini disertai oleh 300 ibu bekerja sepenuh masa yang melahirkan bayi di sebuah hospital tertiari di Kuala Lumpur, Malaysia. Kajian ini menggunakan soal selidik yang mengandungi 28 soalan menguji pengetahuan dan 9 soalan menilai sikap yang telah disahkan kandungannya. Skor tertinggi adalah 28 untuk pengetahuan dan 45 untuk sikap. Skor purata untuk pengetahuan adalah 20.47 (SD 4.06). Ibu yang mendapat skor ≥21 (≥75% skor maksimum) dikategorikan sebagai mempunyai “pengetahuan yang baik” manakala mereka yang mendapat skor
Employment among patients with schizophrenia is known to positively influence patients’ recovery. In their crucial supporting role for patients, caregivers’ burden of care and quality of life may also be influenced by the employment status of patients. This study aimed to investigate the association between employment among patients with schizophrenia and burden and quality of life (QOL) of their caregivers. It was a cross-sectional survey among the primary caregivers of patients meeting DSM-IV-TR’s diagnostic criteria for schizophrenia who were under outpatient or community care at Hospital Bahagia Ulu Kinta. Socio-demographic data, including patients’ employment status, were collected. The Burden on Family Interview Schedule (BFS) and the 36-Item Short Form (SF-36) questionnaires were used to evaluate caregiver burden and quality of life, respectively. Altogether, 201 primary caregivers of patients with schizophrenia participated. Most caregiver burden was experienced in the aspects of finance and routine family activities. There was significant association between patient employment status (p
Langerhans cell histiocytosis (LCH) is a clonal histiocytic disorder. The variable clinical manifestations from isolated bone lesion to multisystem disease can cause difficulties and delay in diagnosis. We report a 2 years and 8 months-old girl who presented with a 2 weeks history of persistent fever and weight loss associated with progressive abdominal distension. Physical examination revealed pallor, bilateral proptosis, seaborrheic dermatitis over the scalp and hepatosplenomegaly. Skull X-ray demonstrated multiple lytic lesions at the base and the skull vault. Bone marrow morphology showed numerous abnormal Langerhans cells (LCs) and foamy macrophages. The trephine immunohistochemistry (IHC) stains for CD1a, S-100 and CD68 were inconclusive. The diagnosis of multisystem Langerhans cell histiocytosis (MS-LCH) in this patient was based on the clinical presentation, radiological and morphological analysis. She subsequently received chemotherapy and currently she is on maintenance therapy with a good clinical response. LCH is a rare disease and although the IHC was inconclusive, the correlation of clinical, radiological and morphological data are essential for the diagnosis.
Worldwide, there is an increasing number of people living with HIV and AIDS especially in the reproductive age group. This has a huge impact when they wish to start a family. Managing reproductive issues among serodiscordant HIV couples is complex and often presents as a challenge especially in minimizing the risk of transmission. We report a case of a HIV positive man and his seronegative wife who successfully achieve conception and deliver a healthy baby without disease transmission of HIV to mother or child. Both partners were managed with treatment where the HIV positive male partner was treated with HAART while the unaffected female partner was on PrEP and conception was achieved through intrauterine insemination (IUI). Planning of conception, the use of medications and options for conception methods will be discussed in detail.
Sharps injury among health care workers in HUKM has received serious attention lately due to the possible transmission of diseases like HIV, Hepatits B and Hepatitis C, which can cause serious consequences for the victim. The aim of the study was to gain a deeper understanding of the emotional problems experienced by health care workers who had incurred a sharps injury and the coping strategies adopted by them to overcome the problem. A 28 item semi-structured questionnaire was designed by the supervisors and the researcher as there was no existing questionnaire to be adopted, as no research had been carried out in this area prior to this. The target group consisted of 64 health care workers of different categories working in HUKM. Data was collected over a period of five months from November 2000 to March 2001. The results of the study revealed that the most frequently injured were the staff nurses, nearly 90% of whom were emotionally upset and were worried that they might contract a disease. There was no significant difference in the emotional stress experienced by the different categories of the health care workers, all were equally upset about the injury but each category of staff adopted different methods to overcome the tragedy. Open comments and opinions about prevention and the management of the sharps injury in HUKM as given by the respondents here are taken into account. Several views given by the participants were explored and discussed. Finally, several implications for nursing and recommendations have been presented.
Study site: Wards and the general operating theatres in Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
Home Care Nursing Program (HCNP) at HUKM was started in 1998 and evaluation of the program was required. The objective of this study is to evaluate the effectiveness of HCNP on stroke rehabilitation at HUKM. A retrospective study using a matched pair design involving 69 stroke patients admitted to HUKM from August 1998 to December 2000 was carried out. The participants of this study were the HCNP group (n=35), and Non- HCNP (n=34). The matching was based on five criteria; age, sex, race, data of admission and severity of disability. The HCNP group was identified through data at the HCNP unit and their medical reports were traced. For the Non-HCNP group, the participants were identified based on criteria which were similar to that of the HCNP group. Based on the criteria identified, there was no significant difference between the two groups. The Activities of Daily Living was measured using the Modified Barthel Index twice; once on admission (based on record) and one post stroke. The t-test was applied to parametric data and non-parametric data; Chi-square was used to detect any significant relationships between the scores of the two groups. Results showed no significant difference (p=0.1990) in ADL on admission and ADL post stroke patients between the two groups. However, there were significant difference (HCNP p=0.000 and Non-HCNP p=0.000) in ADL on admission and post stroke for stroke patients who sought alternative treatment compared to those who had not.
Individuals with double aneuploidy of Down-Turner syndrome are very rare and to date, fewer than 50 cases have been reported, worlwide. We report a case of a male infant who presented with dysmorphic features of upslanting eyes, flat nasal bridge, wide spaced nipples and macroglossia. Based on the clinical features, he was diagnosed with Down syndrome. His peripheral blood sample was taken and sent for cytogenetic analysis for confirmation. Chromosome analysis of his lymphocyte cell culture revealed a mosaic pattern of double aneuploidy with monosomy X identified in 31 metaphases and trisomy 21 in 14 metaphases: (45,X[31]/47,XY,+21[14]). Further analysis with fluorescence in situ hybridization (FISH) using Vysis LSI SRY Spectrum Orange/CEP X Spectrum Green Probe and Vysis CEP Y Spectrum Aqua Probe and Vysis LSI 21 Spectrum Orange Probe performed on the cells (nuclei and metaphases) has confirmed the presence of the abnormal two cell lines (81% monosomy X and 19% trisomy 21) in the patient. Ultrasound investigations of his pelvic region showed normal testes and no evidence of uterus, ovary or vagina. To the best of our knowledge, this is the first Down-Turner syndrome reported in Malaysia. In conclusion, this case demonstrates the importance of Giemsa-banded karyotype and FISH analyses as diagnostic tools in identifying the chromosomal abnormality and determining the ratio of the normal:abnormal cells present in the patient. An annotated bibliography of earlier reported cases of Down-Turner with documented karyotyping is also included in this report.
In this report we demonstrate the role of fluorescence in situ hybridisation (FISH) and conventional cytogenetic methods in clinically and cytogenetically confirmed cases of microdeletion syndromes. A total of nine cases were referred to the Cytopathology and Cytogenetic Unit, Hospital Universiti Kebangsaan Malaysia (HUKM) from 2002 to 2004. They include three Prader-Willi syndrome, three DiGeorge syndrome, one Williams syndrome, one Miller-Dieker syndrome and one Kallmann syndrome. Blood samples from the patients were cultured and harvested following standard procedures. Twenty metaphases were analysed for each of the cases. FISH analysis was carried out for all the cases using commercial probes (Vysis, USA): SNRPN and D15S10 for Prader-Willi syndrome, LIS1 for Miller Dieker syndrome, ELN for Williams syndrome, KAL for Kallmann syndrome, TUPLE 1 and D22S75 for DiGeorge syndrome. Conventional cytogenetic analysis revealed normal karyotypes in all but one case with structural abnormality involving chromosomes 9 and 22. FISH analysis showed microdeletions in all of the nine cases studied. This study has accomplished two important findings ie. while the FISH method is mandatory in ruling out microdeletion syndromes, conventional cytogenetics acts as a screening tool in revealing other chromosomal abnormalities that may be involved with the disease.
The most common extra-articular manifestation of rheumatoid arthritis (RA) is interstitial lung disease (ILD). RA related ILD (RA-ILD) is associated with significant morbidity and mortality. The main objective of this study was to determine the correlation between the rheumatoid factor (RF) serotypes and the severity of RA-ILD based on computed tomography (CT) findings. We recruited a total of 100 RA patients who were tested for IgA RF, IgG RF and IgM RF and had high resolution CT chest performed. Seventy-two patients had ILD changes on HRCT of the chest and were included in this study. We found that the the CT scores for ground glass showed significant positive correlation with disease duration and IgA RF levels whereas the fibrosis scores had significant relationship with multiple clinical covariates i.e age, disease duration, IgA RF levels, IgG RF levels and anti-CCP levels. On multivariate analysis, only IgA levels remained significantly (p
Rheumatoid arthritis (RA) is the most common chronic systemic autoimmune disease worldwide. Although incurable, there are available therapies to effectively control the disease activity and minimize the joint damage. Numerous cytokines, enzymes and other forms of proteins have been implicated in the disease process of RA. In general, pharmacological therapies in RA target cytokine pathways. Despite a wide variety of disease modifying antirheumatic drugs (DMARD), a significant proportion of patients remain refractory to the available therapies. Hence, the search for newer drugs with different modes of actions is an ongoing process. The present review aimed to explore novel therapeutic targets in RA based on data from the literature. Inhibitors of spleen tyrosine kinase, choline kinase, galectin 3 and hypoxia-inducible factor may have a promising role in the
The search for novel biomarkers has taken centre stage in the past decades of research in Rheumatoid Arthritis (RA). The purpose of the present study was to determine the correlation of serum matrix metalloproteinase-3 (MMP-3) with disease activity, joint damage and functional disability in patients with RA. We consecutively recruited RA patients who were under follow-up at the Universiti Kebangsaan Malaysia Medical Centre (UKMMC). Information on the RA disease characteristics were obtained from the medical records and all RA patients were
assessed for DAS28 (disease activity score based on 28 joints) and Stanford Health Assessment Questionnaire (HAQ) 8-item Disability Index (HAQ-DI). The hand radiographs of the RA patients were assessed for joint damage using the Modified Sharp Score (MSS). Serum MMP-3 levels from RA patients and healthy controls were measured using the ELISA method. We recruited a total of 77 RA patients and 18 healthy controls. The serum MMP-3 levels were significantly higher among the RA patients (p<0.05). There were significant correlations between the serum MMP3 levels and MSS (r =0.327) and HAQ-DI (r=0.256), both p<0.05. The mean serum MMP levels in RA patients with radiographic joint erosions was significantly higher than in patients without erosions (p<0.05). Likewise, the subjects with significant functional impairment i.e HAQ-DI ≥1; had significantly higher mean MMP-3 levels compared to RA patients without significant disability (p<0.05). Using multivariate analysis, HAQ-DI remained the independent predictor of serum MMP-3 in RA patients. Serum MMP-3 is a potential biomarker and predictor of radiographic joint damage and functional disability in RA.
Keywords: acquired joint deformity, matrix metalloproteinases, rheumatoid arthritis
Open tracheal injury is rare but can lead to disastrous consequences such as massive bleeding, airway obstruction or failure and aspiration of blood. We present a case of open tracheal injury of a man who tried to attempt suicide using a knife. In this case, the initial management was securing the airway by attempting orotracheal intubation. However, it was unsuccessful when the tube came out from the laceration wound. Intubation was then re-attempted through the distal cut-end of the trachea in the face of airway failure. A quick initial assessment and anticipation of a failed airway should always be the top priority in any emergency physician managing these cases. Direct intubation through the laceration wound might be the only option when all else fail and your patient is crashing.
Keywords: intubation, laceration, trachea