Introduction: Sarawak has a population that is geographically and characteristically widely varied. This study aimed to determine the demographic profile of patients in Sarawak, Malaysia. Materials and Methods – A cross-sectional study was conducted in 2019 at four major haemophilia treatment centres in Kuching, Sibu, Bintulu and Miri Hospitals, Sarawak. Demographic and clinical data were collected with consents from patients. Results and Discussion: Ninety-six haemophilia patients were identified - 79(82.3%) haemophilia A(HA) and 17(17.7%) haemophilia B(HB). Severe haemophilia patients were noted in 45.6% (36/79) of HA and 64.7% (11/17) of HB. In all 44.3% of the HA and 52.9% of the HB population had no identifiable family history of haemophilia. Two-thirds of the patients with severe HA were on prophylaxis [24/36 (66.7%)] and only onethird [4/11 (36.4%)] in severe HB. Inhibitors developed in 9/79 (11.4%) of the HA population [3/79 (3.8%) high responders]. The median inhibitor titre was not significantly different between the different treatment groups – on demand versus prophylaxis (1.0BU versus 2.0BU; z statistic -1.043, p-value 0.297, Mann-Whitney test). None of the patients developed inhibitory alloantibodies to factor IX. Four HA patients (5.1%) underwent immune tolerance induction where one case had a successful outcome. Three severe HA patients received emicizumab prophylaxis and showed remarkable reduction in bleeding events with no thromboembolic events being reported. One female moderate HA patient received PEGylated recombinant anti-haemophilic factor. Eleven patients underwent radiosynovectomy. One mild HB patient succumbed to traumatic intracranial bleeding. Our data reported a prevalence (per 100,000 males) of 5.40 cases for all severities of HA, 2.46 cases for severe HA; 1.16 cases for all severities of HB, and 0.75 cases for severe HB. The overall incidence of HA and HB was 1 in 11,500 and 1 in 46,000, respectively. Conclusion: This study outlines the Sarawakian haemophilia landscape and offers objective standards for forward planning. Shared responsibilities among all parties are of utmost importance to improve the care of our haemophilia population.

Introduction: Awareness for paediatric palliative care has resulted in the impetus for paediatrician-led palliative care services across Malaysia. However, there is paucity of local data on patients receiving hospital-based paediatric palliative care. We aim to review the clinical spectrum of patients referred to these services. Methods: An observational study of children aged between 0-18 years receiving palliative care at 13 hospitals between 1st January and 31st December 2014 was carried out. Results: There were 315 patients analysed, 90 (28.6%) and 46 (14.6%) were neonates and adolescents respectively. The main ICD-10 diagnostic categories for all patients were identified to be ‘Congenital malformations, deformations and chromosomal abnormalities’ 117 (37.1%), ‘Diseases of nervous system’ 76 (24.1%) and ‘Neoplasms’ 60 (19.0%). At referral 156 (50%) patients had holistic needs assessments. Patients with ‘Diseases of nervous system’ were assessed to have significantly more physical needs than the other two diagnostic categories. Majority of patients who knew of their diagnosis and prognosis were those with malignancy. Over a fifth of referrals were at their terminal admission. Of 144 who died, 111 (77.1%) had advanced care plans. There was bereavement follow-up in 98 (68.1%) patients. Conclusion: Patients referred for palliative care have varied diagnoses and needs. To ensure all paediatricians are competent to deliver quality care to all children, further education and training initiatives is imperative.
Palliative Care

INTRODUCTIONThe objective of this study was to examine the clinical outcome of single-level lumbar artificial disc replacement (ADR) compared to that of transforaminal lumbar interbody fusion (TLIF) for the treatment of symptomatic degenerative disc disease (DDD) in an Asian population.

METHODSThis was a retrospective review of 74 patients who had surgery performed for discogenic lower backs that involved only the L4/5 and L5/S1 levels. All the patients had lumbar DDD without radiculopathy or spondylolithesis, and concordant pain with discogram at the pathological level. The patients were divided into two groups--those who underwent ADR and those who underwent TLIF.

RESULTSA trend suggesting that the ADR group had better perioperative outcomes (less blood loss, shorter operating time, shorter hospital stay and shorter time to ambulation) than the TLIF group was observed. However, a trend indicating that surgical-approach-related complications occurred more frequently in the ADR group than the TLIF group was also observed. The rate of revision surgery was comparable between the two groups.

CONCLUSIONOur findings suggest that for the treatment of discogenic lower back pain, lumbar ADR has better perioperative outcomes and a similar revision rate when compared with TLIF. However, the use of ADR was associated with a higher incidence of surgical-approach-related complications. More studies with bigger cohort sizes and longer follow-up periods are needed to determine the long-term efficacy and safety of ADR in lumbar DDD.

Adult ; Female ; Follow-Up Studies ; Humans ; Intervertebral Disc Degeneration ; surgery ; Lumbar Vertebrae ; Male ; Middle Aged ; Minimally Invasive Surgical Procedures ; methods ; Retrospective Studies ; Singapore ; epidemiology ; Spinal Fusion ; methods ; Total Disc Replacement ; methods ; Treatment Outcome

The Academy of Medicine (AMS) and the Ministry of Health (MOH) have developed the clinical practice guidelines on Attention Deficit Hyperactivity Disorder (ADHD) to provide doctors and patients in Singapore with evidence-based treatment for ADHD. This article reproduces the introduction and executive summary (with recommendations from the guidelines) from the MOH clinical practice guidelines on ADHD, for the information of SMJ readers. Chapters and page numbers mentioned in the reproduced extract refer to the full text of the guidelines, which are available from the Ministry of Health website: http://www.moh.gov.sg/content/moh_web/healthprofessionalsportal/doctors/guidelines/cpg_medical.html.The recommendations should be used with reference to the full text of the guidelines. Following this article are multiple choice questions based on the full text of the guidelines.
Attention Deficit Disorder with Hyperactivity ; diagnosis ; drug therapy ; therapy ; Caregivers ; Child ; Evidence-Based Medicine ; Humans ; Methylphenidate ; therapeutic use ; Parents ; Psychiatry ; methods ; standards ; Singapore ; Societies, Medical

Carbon monoxide (CO) is a well-known chemical asphyxiant, which causes tissue hypoxia with prominent neurological injury. Therapeutic hypothermia (TH) has been shown to be an effective neuroprotective method in post-cardiac arrest patients. A 26-year-old man presented to the emergency department with severe CO poisoning. On arrival, the patient was comatose. His vital signs were blood pressure, 130/80 mm Hg; heart rate, 126/min; respiratory rate, 26/min; body temperature, 36degrees C; and O2 saturation, 94%. Initial carboxyhemoglobin was 45.2%. Because there was no available hyperbaric chamber in our local area, he was intubated and treated with TH. The target temperature was 33 +/- 1degrees C for 24 hours using an external cooling device. The patient was then allowed to reach normothermia by 0.15-0.25degrees C/hr. The patient was discharged after normal neurological exams on day 11 at the hospital. TH initiated after exposure to CO may be an effective prophylactic method for preventing neurological sequelae.
Anoxia ; Blood Pressure ; Body Temperature ; Carbon ; Carbon Monoxide ; Carbon Monoxide Poisoning ; Carboxyhemoglobin ; Coma ; Emergencies ; Heart Rate ; Humans ; Hypothermia ; Hypothermia, Induced ; Respiratory Rate ; Vital Signs

PURPOSE: Despite recent advances in laparoscopic pancreatic surgery, few studies have compared laparoscopic central pancreatectomy (LCP) with open central pancreatectomy (OCP). The aim of this study was to compare clinical outcomes between LCP and OCP as a single institutional study. METHODS: During the study period (From January, 1998 to December, 2010), we performed central pancreatectomy in 95 cases. Among them, 26 cases of totally LCP and 55 cases of OCP were compared retrospectively. RESULTS: Benign pancreatic neoplasm was the main indication. The mean operation time for the LCP group (350.2 min) was longer than that for the OCP group (283.4 min). And there was no significant difference in mean actual blood loss (477 ml versus 714 ml, p=0.083) between the LCP and OCP groups. Return to a normal bowel movement and resumption of a liquid diet were achieved 5.5+/-2.6 days after the operation in the LCP group and 6.6+/-2.0 days after the operation in the OCP group (p=0.039). The mean duration of postoperative hospital stay was 13.8 days for the LCP group, which was significantly shorter than the 22.5 days for the OCP group (p=0.015). The overall complication rate was 42.3% (11 cases) in the LCP group and 45.5% (25 cases) in the OCP group (p=0.790). CONCLUSION: Use of LCP for benign or low grade malignant lesions of the pancreatic neck portion is feasible and safe. Compared to the open method, the laparoscopic approach to central pancreatectomy appears to provide advantages of early resumption of a normal diet and reduction of postoperative hospital stay without further complications.
Diet ; Laparoscopy ; Length of Stay ; Neck ; Pancreatectomy ; Pancreatic Fistula ; Pancreatic Neoplasms

BACKGROUND: Smoking, upper respiratory tract infection, genetic factors and hydrocarbons are known as risk factors of Goodpasture's syndrome. We studied a patient with Goodpasture's syndrome who had worked for 27 years in a foundry company. Based on a study on the work-relatedness of the syndrome, we describe and discuss our study results. CASE: A 46-year-old man, who had worked as a foundry worker for 27 years and had a 12 1/2 packyear history of smoking cigarettes, was admitted into a hospital on 15th February 2006 with coughing, chest pain and dyspnea. On admission, he had hematuria, proteinuria, severe restrictive pulmonary function disorder and rapid elevation of blood urea nitrogen/creatinine. Immunological examination showed ANA (+), ANCA (-) and Anti-GBM Ab (+). Kidney biopsy showed pauci-immune crescentic glomerulonephritis. Mild bleeding was revealed through bronchoscopy and no vasculitis and granuloma were present on at lung biopsy. Finally, we diagnosed the worker's illness as Goodpasture's syndrome and carried out hemodialysis and plasmapheresis. In the workplace survey, the exposure level of respirable crystalline silica exceeded the TLV-TWA (0.0106 mg/m3), which was calibrated for overtime. CONCLUSION: Based on both the clinical test and industrial hygiene examination, we concluded that the Goodpasture's syndrome in this case was caused by long-term silica exposure.
Anti-Glomerular Basement Membrane Disease ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Biopsy ; Bronchoscopy ; Chest Pain ; Cough ; Crystallins ; Dyspnea ; Glomerulonephritis ; Granuloma ; Hematuria ; Hemorrhage ; Humans ; Hydrocarbons ; Kidney ; Lung ; Middle Aged ; Occupational Health ; Plasmapheresis ; Proteinuria ; Renal Dialysis ; Respiratory Tract Infections ; Risk Factors ; Silicon Dioxide ; Smoke ; Smoking ; Threshold Limit Values ; Tobacco Products ; Urea ; Vasculitis

BACKGROUND: Smoking, upper respiratory tract infection, genetic factors and hydrocarbons are known as risk factors of Goodpasture's syndrome. We studied a patient with Goodpasture's syndrome who had worked for 27 years in a foundry company. Based on a study on the work-relatedness of the syndrome, we describe and discuss our study results. CASE: A 46-year-old man, who had worked as a foundry worker for 27 years and had a 12 1/2 packyear history of smoking cigarettes, was admitted into a hospital on 15th February 2006 with coughing, chest pain and dyspnea. On admission, he had hematuria, proteinuria, severe restrictive pulmonary function disorder and rapid elevation of blood urea nitrogen/creatinine. Immunological examination showed ANA (+), ANCA (-) and Anti-GBM Ab (+). Kidney biopsy showed pauci-immune crescentic glomerulonephritis. Mild bleeding was revealed through bronchoscopy and no vasculitis and granuloma were present on at lung biopsy. Finally, we diagnosed the worker's illness as Goodpasture's syndrome and carried out hemodialysis and plasmapheresis. In the workplace survey, the exposure level of respirable crystalline silica exceeded the TLV-TWA (0.0106 mg/m3), which was calibrated for overtime. CONCLUSION: Based on both the clinical test and industrial hygiene examination, we concluded that the Goodpasture's syndrome in this case was caused by long-term silica exposure.
Anti-Glomerular Basement Membrane Disease ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Biopsy ; Bronchoscopy ; Chest Pain ; Cough ; Crystallins ; Dyspnea ; Glomerulonephritis ; Granuloma ; Hematuria ; Hemorrhage ; Humans ; Hydrocarbons ; Kidney ; Lung ; Middle Aged ; Occupational Health ; Plasmapheresis ; Proteinuria ; Renal Dialysis ; Respiratory Tract Infections ; Risk Factors ; Silicon Dioxide ; Smoke ; Smoking ; Threshold Limit Values ; Tobacco Products ; Urea ; Vasculitis

Tsutsugamushi disease (Scrub typhus) is an acute, febrile illness caused by Orientia tsutsugamushi, which is transmitted to humans through chigger bites. Leptospirosis, a febrile disease caused by various pathogenic Leptospira, and is acquired by exposure to contaminated water and soil. Both diseases have been the most common acute febrile diseases in the autumn in Korea for many years. Concomitant leptospirosis and scrub typhus is quite rare. We report a case of a coinfection with leptospirosis and scrub typhus in a 51-year-old male who presented with fever, abdominal pain and acute dyspnea. The patient was diagnosed with as acalculous cholecystitis, acute respiratory distress syndrome, and septic shock caused by the infection. This is the first case report of a coinfection with leptospirosis and scrub typhus in Korea.
Abdominal Pain ; Acalculous Cholecystitis ; Coinfection ; Dyspnea ; Fever ; Humans ; Korea ; Leptospira ; Leptospirosis* ; Male ; Middle Aged ; Orientia tsutsugamushi ; Respiratory Distress Syndrome, Adult ; Scrub Typhus* ; Shock, Septic ; Soil ; Trombiculidae ; Water Pollution

PURPOSE: The aim of this study was to assess the safety of TME with pelvic autonomic nerve preservation in male rectal cancer patients in terms of voiding and sexual function. METHODS: We performed uroflowmetry using Urodyn (Dantec, Denmark) and a standard questionnaire employing the IIEF (International Index of Erectile Function) and the IPSS (International Prostate Symptom Score) pre- and postoperatively in 68 male rectal cancer patients. RESULTS: There were significant differences of mean maximal flow rate and voided volume before and after surgery (18.9+/-5.7 vs 13.7+/-7.0, 240+/-91.9 vs 143+/-78, P<0.05, P<0.05), respectively. But, there was no difference of residual volume before and after surgery (4.4 2.6 vs 8.1 4.4, P>0.05). The total IPSS (International Prostate Symptom Score) was increased after surgery from 6.2+/-5.8 to 9.8+/-5.9 (P<0.05). There were no changes of score for one of each seven IPSS items in from 49 patients (73.5 percent) to 61 patients (89.7 percent). Five IIEF (International Index of Erectile Function) domain score (erectile function, intercourse satisfaction, orgasmic function, sexual desire and overall satisfaction) was statistically decreased after surgery (18.2+/-9.3 vs 13.5+/-9.0, 8.4+/-4.2 vs. 4.4+/-2.9, 5.8+/-2.9, vs. 4.4+/-2.9, 6.1+/-2.4 vs. 4.8+/-2.0, 6.1+/-2.2 vs. 4.5+/-2.3, P<0.05, respectively. Erection was possible in 55 patients (80.9 percent), but penetration ability was possible in 51 patients (75 percent). Complete inability for erection and intercourse was observed in 3 patients (5.5 percent). Retrograde ejaculation was noted in 9 patients (13.2 percent). IIEF domains such as sexual desire and overall satisfaction were markedly decreased in 39 patients (57.4 percent), 43 patients (63.2 percent), respectively. Multiple regression analysis of factors affecting postoperative sexual dysfunction showed that over 60 years (sexual desire: P=0.019), within 6 months (erectile function: P=0.04, intercourse satisfaction: P=0.011, orgasmic function: P=0.03), lower rectal cancer (erectile function: P=0.02, intercourse satisfaction: P=0.036, orgasmic function: P=0.027) were significant factors. CONCLUSIONS: TME with pelvic autonomic nerve preservation technique showed a safety and comparable data in preserving sexual and voiding function. The IPSS and IIEF questionnaire were useful and more investigative in assessing urinary and sexual function.
Autonomic Pathways* ; Ejaculation ; Humans ; Male* ; Orgasm ; Prostate ; Surveys and Questionnaires ; Rectal Neoplasms* ; Residual Volume