The Senior–Loken syndrome was first described in 1961 as an oculo-renal disease consisting of familial juvenile nephronophthisis and Leber congenital amaurosis. It is a rare autosomal recessive disorder with a prevalence of 1:1,000,000 caused by mutations in nine genes (NPHP 1-8 and NPHP 10). Ocular manifestations (e.g., photophobia, nystagmus, and extreme hyperopia) occur within the first few years of life while renal manifestations (e.g., formation of multiple cysts impairing kidney function and end-stage renal disease) appear in late childhood to adolescence. Here, we report a case of a Filipino male presenting with rotatory nystagmus and progressive deterioration of vision since childhood. He had congenital amaurosis and juvenile nephronophthisis that progressed to end stage renal disease by age 19. All laboratory and imaging findings were consistent with chronic kidney disease. Molecular genetic testing of ciliopathy-related genes was performed revealing a homozygous mutation in exon 11 of the IQCB1/NPHP5 gene, c.1090C>T (p.Arg364*). This sequence change created a premature translational stop signal resulting in a truncated protein product, nephrocystin-5 and its consequent loss of function. His symptoms eventually improved with initiation dialysis. The prognosis of Senior–Loken syndrome remains dismal and a high index of suspicion, early diagnosis and timely intervention of renal complications are warranted.

Allergic diseases, such as asthma, allergic rhinitis, eczema, and food allergy, are preventable diseases. Primary prevention strategies of allergic diseases have been in scrutiny. Effective prevention strategies maybe started prenatally, postnatally, during infancy, and even during childhood. These guidelines have been prepared by the Philippine Society of Allergy, Asthma and Immunology and the Philippine Society for Pediatric Gastroenterology, Hepatology and Nutrition. They aim to provide evidence-based recommendations for the dietary primary prevention of allergic diseases in children. The primary audience of these guidelines is all healthcare practitioners who manage patients with potential allergic conditions. These guidelines are based on an exhaustive review of evidences, mostly systematic reviews, randomized controlled trials, and cohort studies. However, there are still many gaps in the evidence of dietary primary prevention of allergic diseases.
Allergy and Immunology ; Asthma ; Child ; Cohort Studies ; Delivery of Health Care ; Eczema ; Food Hypersensitivity ; Gastroenterology ; Humans ; Hypersensitivity ; Primary Prevention ; Rhinitis, Allergic

Background: In 2008, the Department of Health (DOH) issued Administrative Order 2008-0023 that called for an “effective and efficient monitoring system that will link all patient safety initiatives”. However, there are still no explicit and harmonized targets to measure effectiveness and to provide benchmarks that assess whether previous efforts were helpful. Objective: The study aimed to describe the status of patient safety performance measures and indicators on the international patient safety goals (IPSGs) in select hospitals in the Philippines. Methods: Descriptive, cross-sectional design was used to investigate currently used performance measures and indicators. Data collection included administration of a Hospital Patient Safety Indicators Questionnaire (HPSIQ) that summarized the currently used patient safety measures and indicators in the sampled Level 2 and level 3 hospitals and triangulation by review of documents such as hospital databases, protocols on reporting, and manuals for information gathering regarding patient safety. Performance measures were categorized using the Donabedian framework. Core indicators were identified through review of standards that cut across the six IPSGs and evaluation of overarching processes and concepts in patient safety. Results: Forty-one level 2 and 3 hospitals participated in the study. Most performance indicators were process measures (52%), while structure (31%) and outcome measures (17%) accounted for the rest. There is an obvious lack of structural requirements for patient safety in the hospitals included in this study. Less than half the hospitals surveyed implement risk assessment and management consistently. Reporting of events, near- misses, and patient safety data are widely varied among hospitals. Data utilization for quality improvement is not fully established in many of the hospitals. Patient engagement is not integrated in service delivery and performance measurement but is crucial in promoting patient safety. Conclusion Mechanisms to improve hospitals’ capacity to monitor, anticipate, and reduce risk of patient harm during the provision of healthcare should be provided. Having a unified set of definitions and protocols for measurement will facilitate reliable monitoring and improvement. Leadership and governance, both internal (e.g., hospital administrators) and external (e.g., DOH) that recognize a data-driven approach to policymaking and improvement of service delivery are crucial in promoting patient safety
Patient Safety ; Outcome and Process Assessment, Health Care

Background and Objectives: The immediate post-discharge period of COVID-19 patients is a vulnerable state due to several complications that may arise during this time. Some patients get readmitted shortly after being discharged while others report persistence of symptoms, develop specialized needs, or observe a decline from their baseline functional capacity. Information on the outcomes of post-COVID discharge patients in our institution is currently lacking. This study described the outcomes of patients with COVID-19 after their discharge from the service areas of Philippine General Hospital. Methods: This study is a retrospective chart review involving charts of all adult patients discharged from the PGH COVID service areas last August 2021 to October 2021. Data from their follow up consults at 1 week, 1 month, and 3 months post-discharge were reviewed. Baseline characteristics and post-discharge outcomes including post-COVID symptoms, special care needs, mortality, rehospitalization, emergency consult, level of dependence, and ability to return to work were assessed. Results: A total of 171 patient charts were included. The mean age of patients was 53.7 years. Most were male (60.2%), unemployed (59.7%), non-smoker (55%), hypertensive (57.9%), diabetic (50.2%), and obese (50.2%). Most of them were oxygen requiring (80%) and with severe to critical COVID infection (72.5%) during admission. At 3 months post-discharge, 113 (66%) were stable and able to complete the follow up, 8 (4.6%) died, 9 (5.2%) got readmitted, and 41 (23.9%) were lost to follow up. Among those who were able to follow up after 3 months, 84 (74%) were asymptomatic. Among those who remained symptomatic, the most common symptoms were dyspnea, fatigue, and cough. After 3 months, 100 (88%) did not require special care needs, 100 (88%) were fully independent, and 45 (39.8%) were able to return to baseline work. Conclusions Despite the majority of patients having severe to critical COVID infection during admission, most were asymptomatic within 3 months post-discharge. In those who developed persistent symptoms, dyspnea, cough, and fatigue were the most common symptoms identified regardless of COVID severity. Majority did not require special care needs.
COVID-19

Background and Objectives: Heart Failure (HF) remains a major health concern worldwide. In the Philippine General Hospital (PGH), HF is consistently a top cause of mortality and readmissions among adults. The American College of Cardiology (ACC) and European Society of Cardiology (ESC) published guidelines for interventions that improve quality of life and survival, but they are underused and untested for local acceptability. Hospitals overseas used order sets created from these guidelines, which resulted in a considerable decrease in in-hospital mortality and healthcare costs. We aimed to develop an order set for adult patients with acute heart failure (AHF) admitted to the PGH Emergency Department (ED) to improve care outcomes. Methods: This study utilized a mixed methods approach to create the AHF order set. ESC and ACC HF guidelines were appraised using the AGREE II tool. Class I interventions for AHF were included in the initial order set. Through focused group discussions (FGD), clinicians and other care team members involved in the management of AHF patients at PGH ED modified and validated the order set. Stakeholders were asked to use online Delphi and FGD to get a consensus on how to amend, approve, and carry out the order given. Results: Upon review of HF guidelines, 29 recommendations on patient monitoring, initial diagnostic, and therapeutic interventions were adopted in the order set. Orders on subspecialty referrals and ED disposition were introduced. The AHF patient was operationally defined in the setting of PGH ED. The clinical orders fit the PGH context, ensuring evidence-based, cost-effective, and accessible care responsiveness to patients’ needs and suitable for local practice. Workflow changes due to COVID-19 were considered. Potential barriers to implementation were identified and addressed. The final order set was adopted for implementation through stakeholder consensus. Conclusion The PGH developed and adopted its own AHF order set that is locally applicable and can potentially optimize outcomes of care.
Quality Improvement ; Critical Pathways