Polycystic kidney disease (PKD) is a group of inherited disorders characterized by cystic lesions in the kidneys and multiple organs, primarily including autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD is mainly caused by variations in the PKD1 and PKD2 genes. Its clinical manifestations include progressive renal cyst growth, hypertension, and multi-system complications. ARPKD, on the other hand, is primarily caused by mutations in the PKHD1 gene. It commonly occurs in infants and young children, with hepatorenal cystic fibrosis being a key feature. Although there is currently no cure for PKD, the integration of multi-omics and precision medicine strategies holds promise for optimizing patient management and improving outcomes in the future. This review summarizes the genetic basis, pathogenic mechanisms, diagnostic techniques, and therapeutic advances in PKD, providing a reference for clinical practice and research.
Humans ; Polycystic Kidney Diseases/genetics* ; TRPP Cation Channels/genetics* ; Mutation ; Polycystic Kidney, Autosomal Dominant/therapy* ; Receptors, Cell Surface

INTRODUCTION

Acute coronary syndrome (ACS) and end-stage renal disease (ESRD) are both prevalent globally. The diagnosis and management of ACS in ESRD is difficult because the interplay of cardiovascular and renal disease is complicated. The guidelines for ACS may not be applicable to the ESRD population because the trials from which these are drawn mostly excluded ESRD patients.

To determine the clinical profile and outcomes of CKD patients on dialysis admitted for ACS in the Philippine General Hospital (PGH).

We did a retrospective cohort study and employed a retrospective review of electronic medical records among ESRD patients presenting with ACS in PGH from May 2021 to November 2023. The collected data was analyzed using univariate and bivariate statistics using PRISM software.

A total of 48 patients with ESRD were admitted for ACS in this study – 8 with STEMI and 40 with NSTEMI. The mean age was 61 years old and 33 (68.8%) were male. Among those with STEMI, six (75%) presented with Kilip II or more. While among those with NSTEMI, 17 (42.5%) had a GRACE score >140 and 27 (67.5%) had an NSTEMI TIMI risk score >2. On average, the patients were on hemodialysis for 31 months prior to admission. The most common comorbidities were hypertension (91.7%) and heart failure (83.3%). On admission, 18 (37.5%) presented with SBP >160, 7 (14.6%) patients presented with shock, and 4 (8.3%) patients presented with cardiac arrest. 38 (79.2%) patients had anemia on admission. 21 (43.8%) patients had left ventricular hypertrophy on electrocardiogram while 34 (70.8%) patients had cardiomegaly on chest radiography. The average left ventricular ejection fraction on echocardiogram was 46% and 27 (90%) patients had segmental wall motion abnormalities. The most common angiographic finding was 3-vessel coronary artery disease seen in 50% of patients. Almost all patients received dualantiplatelet therapy, high dose statin, and beta-blocker. The mortality rate was high at 43.8% with cardiovascular causes being the most common cause of death.

This study demonstrates the high mortality rate among patients with ESRD presenting with ACS. Our study portrays that patients with ESRD present with higher risk features including abnormalities in vital signs, laboratories, imaging, high prognostications score, and high in-hospital morbidity.

Valacyclovir-associated neurotoxicity (VAN) is a recognized adverse effect in elderly patients with renal impairment but remains underdiagnosed due to its nonspecific presentation and overlap with acute neurologic emergencies. We report a 78-year-old Filipino female with end-stage renal disease on maintenance hemodialysis who developed acute disorientation, agitation, vivid visual hallucinations and generalized weakness shortly after initiation of valacyclovir for herpes zoster. Given the abrupt onset of neuropsychiatric symptoms, viral encephalitis was initially considered. Magnetic resonance imaging of the brain showed no evidence of acute infarction or encephalitis, while electroencephalography demonstrated diffuse generalized slowing consistent with an encephalopathic process. Review of the medication history revealed valacyclovir dosing that exceeded recommendations for patients with end-stage renal disease. Valacyclovir was discontinued and emergent hemodialysis was initiated resulting in marked improvement in sensorium after the second session and complete resolution of symptoms after the third. This case shows VAN as an important diagnostic mimic of acute encephalopathy in elderly patients with renal failure and emphasizes the critical role of early medication review in preventing unnecessary investigations and enabling prompt, reversible management.

OBJECTIVES

In the past decade, the prevalence of diabetes in the Philippines has been increasing. The prevalence of diabetes is 7.1% and it is the sixth leading cause of death among adults. Preventive measures to address the incidence of diabetes, as well as diabetes-related complications, have been in place. However, knowledge of the risk factors for developing cardiovascular or renal-related Type 2 Diabetes Mellitus (T2DM) complications has numerous benefits for T2DM patients. This study aimed to describe common risk factors among Filipino T2D patients which increase their risk of developing cardiorenal complications including Heart Failure and CKD. Furthermore, the study aimed to assess the
awareness of T2DM patients regarding these risk factors

A clinic-based cross-sectional survey among Filipinos with T2DM Philippines was conducted in the different regions in the Philippines from April 2021 to January 2022. The study participants were 16,268 patients of various ages and sex, however, only 11,578 of them answered the form completely. The patients and their corresponding physicians answered a validated self-assessment form on whether they had 8 identified risk factors for T2DM.

This survey identified Hypertension as the most common risk factor present in 96.49% of T2DM patients followed by Dyslipidemia (82.56%), Family History of CVD (77.11%), Obesity (59.66%), Kidney Disease (38.08%), Personal History of CVD (36.22%), Smoking (35.49%) and Family History of Kidney Disease (33.05%). Most patients were aware of the presence of these risk factors. However, there were some identified by the physician but unknown to the patient. In decreasing order of frequency, these were personal history of heart and blood vessel diseases (7.19%), followed by family history of kidney disease (5.44%) and smoking (5.1%).

This survey was able to identify crucial risk factors in developing T2DM complications that are most common among T2DM patients in the Philippines. Furthermore, for the first time, the study was able to show the level of awareness of T2DM patients regarding the risk factors that they have in developing these complications. These data show the importance of determining and addressing the common risk factors in preventing T2DM complications. Moreover, identification of these risk factors is an important step in prevention of cardiorenal complications.

BACKGROUND AND OBJECTIVE

Gastrointestinal (GI) manifestations among COVID-19 patients are common; however, their relation with patient outcomes remains unclear. The study, therefore, aims to determine the association of GI manifestation with in-hospital mortality among end-stage renal disease patients (ESRD).

This is a retrospective cohort design. All 501 ESRD patients with COVID-19 and admitted to the National Kidney and Transplant Institute from June 2020 to 2021 were included. GI manifestation was defined as the presence of any of the following signs and symptoms on admission: dysgeusia, anorexia, abdominal pain, nausea, vomiting, diarrhea, and jaundice. The outcome of interest was in-hospital mortality, defined as death due to any cause during hospital stay. Stata17 was used for data analysis.

The overall prevalence of GI manifestation was 58.08% (95% CI: 53.63-62.45). The most common symptoms were abdominal pain (27.15%), loss of appetite (24.35%), and nausea (19.76%). Patients with GI manifestation had a higher proportion of stroke, lower median systolic blood pressure, and a lower proportion of abnormal oxygen saturation and diastolic blood pressure than those without GI manifestation. Across all clinical outcomes, only hospital stay significantly differ between those with and without GI manifestation. In-hospital mortality was 31.14% (95% CI: 27.10-35.39%) and showed no significant association with GI manifestation (OR= 0.94, p=0.749).

GI manifestation was common among ESRD patients with moderate-to-severe COVID-19. The inhospital mortality rate is also high; however, GI manifestation was not associated with this outcome. Meanwhile, GI manifestation leads to longer hospital stay.

BACKGROUND

Non-alcoholic fatty liver disease (NAFLD) is prevalent in patients with Type 2 Diabetes Mellitus (T2DM) and is associated with chronic kidney disease (CKD). The aim of this cross-sectional study was to determine the association of Fibrosis-4 (FIB-4) index with CKD among T2DM patients with concomitant NAFLD.

A single center, analytical cross-sectional study was conducted among 216 T2DM patients with concomitant NAFLD. Clinical data were obtained via retrospective review of medical charts. The outcome of interest was CKD which was based on self-report obtained from medical charts or estimated Glomerular Filtration Rate (eGFR)RESULTS

Higher FIB-4 index was found to be significantly associated with CKD. Patients with FIB-4 index of 1.45-3.25 (moderate risk) and >3.25 (high risk) have about 3 times higher odds of CKD. However, after controlling for the significant confounders, only those who belong to high-risk group was found to be associated with CKD.

This study has demonstrated that FIB4 index > 3.25, an index of liver fibrosis, is significantly associated with development of CKD in T2DM patients with concomitant NAFLD.

INTRODUCTION

Chronic Kidney Disease (CKD) is a leading cause of morbidity and mortality in the Philippines. Most Filipino CKD patients prefer hemodialysis due to barriers such as cost and availability of Kidney Transplant. End-stage kidney disease (ESKD) patients face high symptom burden and unmet palliative care needs. Even with advancement in dialysis technology, the annual mortality rate of dialysis patients remains between 20% and 25%. While Advance Care Planning (ACP) can help align care with patient preferences by facilitating discussions about values and future decisions, its utilization in dialysis population remains low due to barriers in implementation. There is limited research specifically addressing the preferences and influencing factors of Advance Care Planning among CKD patients on hemodialysis in the Philippines.

This study aimed to determine the ACP preferences of CKD patients undergoing hemodialysis and to identify the clinicodemographic factors associated with these preferences.

An analytic cross-sectional study was conducted involving 96 chronic kidney disease (CKD) patients undergoing hemodialysis at Baguio General Hospital and Medical Center (BGHMC) from October to November 2024. Data were collected using validated questionnaires administered either through face-to-face interviews or self-administration, depending on patients’ preferences and capabilities. Descriptive and inferential statistical methods were employed for data analysis.

The study revealed limited awareness of ACP among participants (86.5%), underscoring the need for education. Family-centered decision-making was prominent, with most participants preferring family members as surrogate decision-makers and confidants. Quality of life was prioritized over life extension, and preferences for “Do Not Resuscitate” (DNR) orders were notable. Educational attainment and ethnicity significantly influenced preferences, with higher education linked to greater awareness; and Ethnicity shaping preferences for decision-makers, confidants, timing of discussions, and resuscitation choices. Additionally, duration of dialysis was linked to care setting preferences, while social support systems influenced the preferred place for discussions.

The findings highlight critical associations between clinicodemographic factors and ACP preferences among hemodialysis patients. Addressing these associations through targeted education and culturally sensitive approach can promote high-quality end-of-life care, aligned with diverse patient needs, values, and preferences.

BACKGROUND

Chronic Kidney Disease (CKD) patients often face critical treatment decisions that significantly affect their quality of life, making Shared Decision-Making (SDM), a collaborative approach between patients and healthcare providers, an essential component of patient-centered care.

This study aimed to investigate the impact of SDM on decisional readiness and patient satisfaction among CKD patients undergoing hemodialysis (HD) at Southern Isabela Medical Center (SIMC).

An analytical cross-sectional design was utilized. The Tagalog Version of SDM Q-9 questionnaire, Decision survey and CSAT survey tool were given to HD patients of SIMC from September – October 2024. Descriptive statistics, Pearson product-moment correlation and chi square test were used to report and analyze data.

One hundred eight (108) CKD patients on HD were included in the study. Shared decision-making score was high and overall satisfaction on health services was outstanding. Higher decisional readiness was associated with greater odds of reporting outstanding satisfaction (OR = 3.47, 95% CI: 2.563–4.688, p = 0.009). There was no significant association between patient satisfaction and SDM (r= 0.111, p =0.253.). Shared decision-making had a significant but weak positive correlation with decisional readiness (r =0.2043, p 0.035).

This study showed that fostering SDM positively enhances patients’ preparedness to make healthcare decisions. Decisional readiness is strongly associated with patient satisfaction, as confident patients are more likely to be satisfied with their care. The findings underscore the need for improved patient education to boost decisional readiness and support ongoing SDM practices. Integrating SDM into clinical workflows is essential to advancing patient-centered care and improving health outcomes for CKD patients undergoing hemodialysis.

BACKGROUND

Diabetes mellitus (DM) is a significant and growing global health concern. Worldwide, 537 million adults have diabetes and 206 million of them are from the Western Pacific Region1. Local prevalence continues to remain high at 7.5%, with 4,303,899 adult Filipinos suffering from diabetes in 2021. DM significantly contributes to the growing burden of chronic kidney disease (CKD) worldwide with about 50% of end-stage renal disease (ESRD) being due to diabetic nephropathy alone. Likewise, 60% of Filipinos on maintenance dialysis have ESRD due to DM and hypertension. The primary care setting is the initial point of contact between healthcare providers and patients with type 2 diabetes, hence, the development of clinical practice guidelines that will provide guidance in caring for patients with stable complications of diabetes. The guideline is the first of 3 that are being developed by the Philippine Academy of Family Physicians for the diagnosis and management of adult patients with type 2 diabetes and stable microvascular complications – nephropathy, retinopathy and neuropathy.

This guideline aims to provide evidence-based recommendations on the diagnosis and management of adults with type 2 diabetes mellitus (T2DM) and early stage CKD and is divided into 5 main sections – Clinical Assessment, Diagnostic Tests, Pharmacologic Treatment, Non-pharmacologic Treatment and Patient Outcomes.

The method of guideline development followed the ADAPTE process. The Technical Working Group identified 19 key questions after consultation with colleagues and patients. Recommendations were adopted from high-quality clinical practice guidelines whenever applicable for most of the key clinical questions. On the other hand, the De Novo method of evidence review was used to answer key clinical questions for which recommendations from reviewed guidelines were not available. A modified GRADEPro was used in assessing the quality of evidence – high, moderate, low or very low. Following external review by a nephrologist, the draft recommendations were sent to the members of the consensus panel. Voting on whether to include or not by the consensus panel was facilitated to determine the strength of each recommendation – strong, moderate or weak.

After reviewing 3 high-quality clinical practice guidelines and the current evidence, the technical working group was able to develop 40 recommendations for the 19 key clinical questions.

Peripheral nerve block (PNB) has been successfully used as the sole anesthetic for Peritoneal dialysis (PD) catheter insertion, and has been shown to provide satisfactory anesthesia and analgesia perioperatively, especially among critically – ill patients.

This report describes the anesthetic management of an 18 – year old underweight patient with End-stage renal disease (ESRD) and decompensated heart failure who was scheduled for PD catheter insertion. He was given a left lateral Transversus abdominis plane (TAP) block and a right Rectus sheath (RS) block as the main anesthetic. Fifteen mL of Isobaric Bupivacaine 0.375% with Epinephrine 1:400,000 dilution was injected for the TAP block, and 10mL for the RS block, for a total volume of 25mL (93.7mg). Sedation was given via a Remifentanil infusion at 0.1mcg/kg/min. Intraoperatively, the patient was awake, conversant, and comfortable, no pressors were used, and no conversion to general anesthesia was done. Post-operatively, he had good pain control, with a pain score of 1/10, and successfully underwent dialysis via the PD catheter on the 2ndhospital day.

This pediatric patient who is critically ill is not a good candidate for general or neuraxial anesthesia due to the risk of hemodynamic instability and perioperative decompensation. PNB was done to provide anesthesia, and ensure good pain control post-operatively, and a right TAP and left RS were done instead of a bilateral TAP to lower the LA volume and decrease the risk of LA toxicity.

Unilateral TAP with contralateral RS is a safe anesthetic technique among critically-ill pediatric patients who will undergo PD catheter insertion without the risk of hemodynamic instability with general or neuraxial anesthesia.