KMT2B-linked dystonia (DYT-KMT2B) is a childhood-onset dystonia syndrome typically beginning in the lower limbs and progressing caudocranially to affect the upper limbs with eventual prominent craniocervical involvement. Despite its recent recognition, it now appears to be one of the more common monogenic causes of dystonia syndromes. Here, we present an atypical case of DYT-KMT2B with oromandibular dystonia as the presenting feature, which remained restricted to this region three decades after symptom onset. This appears to be the first reported case of DYT-KMT2B from Southeast Asia and provides further supporting evidence for the pathogenic impact of the KMT2B c.6210_6213delTGAG variant.

INTRODUCTION: Our aim was to study the prevalence of frailty and its associated factors in a subacute geriatric ward. METHODS: This was a cross-sectional study of 167 participants between June 2018 and June 2019. Baseline demographics and participants' Mini Nutritional Assessment, Geriatric Depression Scale, Mini Mental State Examination, Charlson's Comorbidity Index and LACE index scores were obtained. Functional measurements such as modified Barthel's Index scores and hand grip strength (HGS) were taken. Frailty was assessed using the Clinical Frailty Scale (CFS) and the FRAIL scale. Data on history of healthcare utilisation, medications, length of stay, selected blood investigations and presence of geriatric syndromes were also collected. RESULTS: The prevalence of pre-frailty (CFS 4) and frailty (CFS ≥ 5) was 16.2% and 63.4%, respectively. There were significant associations between CFS and age (pre-frail vs. non-frail: odds ratio [OR] 1.14, 95% confidence interval [CI] 1.04-1.25, P = 0.006; frail vs. non-frail: OR 1.08, 95% CI 1.01-1.15, P = 0.021), HGS at discharge (frail vs. non-frail: OR 0.90, 95% CI 0.82-0.99, P = 0.025), serum albumin (frail vs. non-frail: OR 0.90, 95% CI 0.82-0.99, P = 0.035) and the presence of urinary incontinence (frail vs. non-frail: OR 3.03, 95% CI 1.19-7.77, P = 0.021). CONCLUSION Frailty is highly prevalent in the subacute geriatric setting and has many associated factors. In this study, independent factors associated with frailty were age, HGS at discharge, serum albumin and urinary incontinence. This has implications for future resource allocation for frail older inpatients and may help direct further research to study the effectiveness of frailty-targeted interventions.
Humans ; Aged ; Frailty/epidemiology* ; Frail Elderly ; Hand Strength ; Prevalence ; Singapore/epidemiology* ; Cross-Sectional Studies ; Fatigue Syndrome, Chronic ; Geriatric Assessment ; Urinary Incontinence ; Serum Albumin

Cranial nerve (CN) III palsy is rarely caused by intraorbital compression, let alone from a subgaleal abscess. We present a case of a hypertensive 55-year-old man with an acute isolated pupillary-sparing left CN III palsy from a left subgaleal abscess with associated pterional osteomyelitis and frontotemporal mass. This is the first reported such case and the third reported case of a chronic spontaneous subgaleal abscess. A seemingly routine case of an acute, pupillary-sparing, isolated CN III palsy from hypertension turned out to be a rare case in terms of etiology of the palsy and of the source of the abscess.
Cranial Nerve III ; palsy ; ptosis ; ophthalmoplegia

Purpose: Sperm DNA fragmentation (SDF) has been associated with male infertility and poor outcomes of assisted reproductive technology (ART). The purpose of this study was to investigate global practices related to the management of elevated SDF in infertile men, summarize the relevant professional society recommendations, and provide expert recommendations for managing this condition. Materials and Methods: An online global survey on clinical practices related to SDF was disseminated to reproductive clinicians, according to the CHERRIES checklist criteria. Management protocols for various conditions associated with SDF were captured and compared to the relevant recommendations in professional society guidelines and the appropriate available evidence. Expert recommendations and consensus on the management of infertile men with elevated SDF were then formulated and adapted using the Delphi method. Results: A total of 436 experts from 55 different countries submitted responses. As an initial approach, 79.1% of reproductive experts recommend lifestyle modifications for infertile men with elevated SDF, and 76.9% prescribe empiric antioxidants. Regarding antioxidant duration, 39.3% recommend 4–6 months and 38.1% recommend 3 months. For men with unexplained or idiopathic infertility, and couples experiencing recurrent miscarriages associated with elevated SDF, most respondents refer to ART 6 months after failure of conservative and empiric medical management. Infertile men with clinical varicocele, normal conventional semen parameters, and elevated SDF are offered varicocele repair immediately after diagnosis by 31.4%, and after failure of antioxidants and conservative measures by 40.9%. Sperm selection techniques and testicular sperm extraction are also management options for couples undergoing ART. For most questions, heterogenous practices were demonstrated. Conclusions This paper presents the results of a large global survey on the management of infertile men with elevated SDF and reveals a lack of consensus among clinicians. Furthermore, it demonstrates the scarcity of professional society guidelines in this regard and attempts to highlight the relevant evidence. Expert recommendations are proposed to help guide clinicians.

This clinical pathway for the diagnosis and risk stratification of patients presenting with acute chest pain, including acute coronary syndromes, provides recommendations and algorithms for clinicians to diagnose, risk stratify, and manage acute chest pain in adult patients. The writing committee reviewed existing international and local guidelines. Modifications to the algorithm following face-to-face and virtual meetings resulted in expert decisions written as recommendations and presented in a flow diagram format. The USTH Chest Pain Pathway provides guidance based on current guidelines and recommendations on assessing and evaluating acute chest pain, tailored to local needs and institution-specific facilities. We recommend its use to ensure quality patient care in the hospital.
Acute Coronary Syndrome|critical Pathways

Background. A 2017 situational analysis assessing Clinical Practice Guidelines (CPG) development in the Philippines revealed CPGs of inconsistent quality. In response, the Department of Health (DOH)-Philippine Health Insurance Corporation Manual for CPG Development was developed to outline the standardized steps of the CPG development process. To implement this, technically qualified institutions and individuals should be commissioned.

Objective. To identify qualified institutions and individuals and map out their technical skills and potential for capacit building in CPG development

Methods. Mixed methods were used in this cross-sectional study. A snowballing method identified specific institutions and individuals. Self-administered surveys and key informant interviews were conducted to determine competence, strengths, and gaps in the development of CPGs.

Results. A total of 74 individuals from 45 institutions with competencies in CPG development were identified. Of the 45 institutions, 72% were non-clinical, with roughly half working on formal research. Of the 74 individuals, 96% possessed relevant knowledge and skills and 85% already provided training on CPG development topics. Around half of the respondents have been part of a CPG development task force. Only about half were able to incorporate social concepts of equity, and only one-third had experience in managing conflicts of interest.

Conclusion. Qualified institutions and individuals identified in this capacity mapping can be tapped in future CPG development in the country. Incorporation of social concepts and management of conflicts of interest still need to be ensured.

Objective: This study aims to identify the epidemiological characteristics and co-morbidities of eyelid injuries among Filipino patients seen at the Emergency and Out-patient Clinics of a single, tertiary, government, urbanbased hospital from June 2014 to June 2015. Methods: This is a retrospective, cross-sectional, descriptive single-center study. Medical records of 85 patients who consulted at the Emergency or Out-Patient Clinics of the Department of Ophthalmology and Visual Sciences of the Philippine General Hospital, Manila due to eyelid injuries that resulted to skin breaks with or without tissue loss or other ocular co-morbidities were reviewed. Information collected included: demographic data of the patient; the offending object; mechanism, place, date and time of injury; date and time of consult; possible risk factors and predisposing conditions such as alcohol intoxication and lack of use of protective gears. Complications associated with eyelid injuries were also noted. Descriptive statistics was used for analysis of patient demographics, epidemiologic characteristics of eyelid injuries and its associated complications. Results: Of the 85 cases, 78% were males. Most of the patients were below 50 years of age with a range of 1-79 and a mean age of 25 years old. There is male preponderance across all age groups except in the pediatric group below 10 years of age. The right eye was involved in 58% of cases. The most common area affected was the right lower lid as seen in 36 cases. Most of the eyelid injuries were severe: 21 (25%) were margin-involving, 17 (20%) had canalicular transection and 34 (40%) had both. Other co-morbidities included some degree of tissue loss or avulsion (16%) and globe involvement (29%). Majority were accidental (36%). Public places (33%) and the home (33%) were the most common places of injury. Most of the injuries that happened at home were accidents (82%) while those that occurred in public places were mostly assault cases (71%). The most common offending objects that caused the eyelid injuries included metal (33%), pavement/cement (18%), and wood (16%). The most common mechanism of injury was blunt trauma (67%). There were 18 cases of vehicular crash, 89% of the patients were not wearing helmets. All work-related injuries reported absence of protective gear. Alcohol intoxication was reported in 30% of assault cases and 72% of vehicular crash. Conclusion Eyelid injuries continue to pose as a public health concern. Campaigns organized toward educating the public can stress on the importance of increased vigilance regarding safety especially in children and young adults. Use of protective gear and eyewear while driving or working in hazardous environments should also be emphasized. Monitoring of public places for any disturbance can prevent violent acts from escalating. The AntiDrunk and Drugged Driving Act of 2013 should also be strictly enforced.
Accidents ; Driving Under the Influence

A risk management plan (RMP) contains an overview of important safety concerns associated with the use of a therapeutic product (TP) and proposed pharmacovigilance activities to monitor and manage these safety concerns in the post-approval stage. The Health Sciences Authority (HSA) of Singapore has legislated its requirements for RMPs as part of its product life cycle approach in the management of safety concerns associated with TPs. Under the Health Product (Therapeutic Products) Regulations enacted in November 2016, companies may be required to implement RMPs under the direction of HSA to help ensure that the benefits of TPs outweigh their risks. In this commentary, we present a case study on the local RMP for Dengvaxia®, the world's first dengue vaccine, which has been approved for use in Singapore. We hope that this article raises awareness among healthcare professionals about the concept of RMPs and how RMPs are implemented locally.

BACKGROUND: Lupus nephritis is very common complications in SLE, with clinical symptoms of renal involvement occurring in 30%-70% of patients. Outcomes in children with proliferative lupus nephritis (PLN) show 9-15% progress to end-stage renal disease (ESRD) at 5 years.

OBJECTIVES: This study compared the outcome of children and adolescent patients with lupus nephritis treated with 9 month versus 6 month induction of cyclophosphamide therapy. Renal frequency and adverse effects of IV cyclophosphamide during and after induction therapy were described and determined.

DESIGN: Retrospective Cohort Study

SETTING: Tertiary Hospital

METHODS: Retrospective cohort study comparing 6 and 9 month protocol of IV cyclophosphamide for lupus nephritis were conducted in a government tertiary pediatric hospital in the Philippines. A total of 39 patients with lupus nephritis were gathered, 23 patients underwent 6 months and 16 patients underwent 9 months protocol.

RESULTS: The comparison of two protocols in the administration of intravenous cyclophosphamide (IVCY) did not show significant difference between the two in terms of changes in GFR levels, but some evidence of a greater percent increase from baseline with the 6 months protocol post treatment were observed. Among 39 subjects, creatinine, albumin and urinalysis profile did not also differ between the two groups and levels within each group changed insignificantly over time up to 24 months. Proportion of subjects with renal flare ups, adverse effects and who expired during the study period were also essentially similar between the two groups.

CONCLUSION: IV Cyclophosphamide seems efficacious if given at the very beginning of the flare and at the start after patient was diagnosed with lupus nephritis. No statistically difference between the duration of the protocol. Renal flare ups and adverse effects of cyclophosphamide such as nausea, vomiting and headache were observed similarly between two protocols. Diligent follow up is needed for further studies and specificity of the results.

Adult ; Aged ; Aged, 80 and over ; Anti-Bacterial Agents ; therapeutic use ; Bacteremia ; drug therapy ; Cefazolin ; therapeutic use ; Drug Resistance, Bacterial ; Female ; Humans ; Klebsiella Infections ; drug therapy ; Klebsiella pneumoniae ; Male ; Microbial Sensitivity Tests ; Middle Aged ; Retrospective Studies ; Young Adult