BACKGROUND

Observational studies have increasingly reported transthyretin amyloid cardiomyopathy (ATTR-CM) as an under-recognized cause of heart failure. We report the first ATTR-CM diagnosed via multi-modality imaging in the Philippines signifying an important milestone in recognition and management of this formerly believed rare disease, locally. Utilization of non-invasive imaging such as echocardiography, cardiac MRI and technetium-99m pyrophosphate scintigraphy (PYP) demonstrates the potential for accurate diagnosis as well as timely and appropriate treatment strategies.

An 81/M Filipino with a history of carpal tunnel surgery, post-percutaneous coronary intervention (PCI), had three months’ history of refractory heart failure symptoms despite optimized medical treatment. His 2D-echo showed an ejection fraction (EF): 45%-50%, increased left ventricular (LV) posterior wall thickness with mild basal inferior wall hypokinesia and ECG: atrial fibrillation with low voltage. Speckle tracking imaging showed average global longitudinal strain: - 6.5% with cherry-on-top pattern on polar strain map. Cardiac MRI demonstrated diffuse late gadolinium enhancement from endocardial to transmural layers of biventricular and biatrial walls, highly suggestive of cardiac amyloidosis (CA). Light-chain amyloidosis was excluded by negative serum/urine protein electrophoresis/immunofixation. Tc-99m PYP scan revealed greater myocardial-than-bone uptake with a Perugini score 3 and calculated heart-to-contralateral ratio of 1.7. Congestion was controlled with intravenous loop diuretics and he was discharged stable with metoprolol succinate, dapagliflozin and apixaban. At the time of paper submission, he is currently being evaluated for tafamidis treatment.

The case highlighted the advantage of multi-modality imaging for noninvasive yet accurate identification of the disease. A tailored approach is required in slowing the disease progression and improving outcomes.

Pathological vascular remodeling is a hallmark of various vascular diseases. Previous research has established the significance of andrographolide in maintaining gastric vascular homeostasis and its pivotal role in modulating endothelial barrier dysfunction, which leads to pathological vascular remodeling. Potassium dehydroandrographolide succinate (PDA), a derivative of andrographolide, has been clinically utilized in the treatment of inflammatory diseases precipitated by viral infections. This study investigates the potential of PDA in regulating pathological vascular remodeling. The effect of PDA on vascular remodeling was assessed through the complete ligation of the carotid artery in C57BL/6 mice. Experimental approaches, including rat aortic primary smooth muscle cell culture, flow cytometry, bromodeoxyuridine (BrdU) incorporation assay, Boyden chamber cell migration assay, spheroid sprouting assay, and Matrigel-based tube formation assay, were employed to evaluate the influence of PDA on the proliferation and motility of smooth muscle cells (SMCs). Molecular docking simulations and co-immunoprecipitation assays were conducted to examine protein interactions. The results revealed that PDA exacerbates vascular injury-induced pathological remodeling, as evidenced by enhanced neointima formation. PDA treatment significantly increased the proliferation and migration of SMCs. Further mechanistic studies disclosed that PDA upregulated myeloid differentiation factor 88 (MyD88) expression in SMCs and interacted with T-cadherin (CDH13). This interaction augmented proliferation, migration, and extracellular matrix deposition, culminating in pathological vascular remodeling. Our findings underscore the critical role of PDA in the regulation of pathological vascular remodeling, mediated through the MyD88/CDH13 signaling pathway.
Mice ; Rats ; Animals ; Myeloid Differentiation Factor 88/metabolism* ; Vascular Remodeling ; Cell Proliferation ; Vascular System Injuries/pathology* ; Carotid Artery Injuries/pathology* ; Molecular Docking Simulation ; Muscle, Smooth, Vascular ; Cell Movement ; Mice, Inbred C57BL ; Signal Transduction ; Succinates/pharmacology* ; Potassium/pharmacology* ; Cells, Cultured ; Diterpenes ; Cadherins

CAPOS syndrome is an autosomal dominant neurological disorder caused by mutations in the ATP1A3 gene. Initial symptoms, often fever-induced, include recurrent acute ataxic encephalopathy in childhood, featuring cerebellar ataxia, optic atrophy, areflflexia, sensorineural hearing loss, and in some cases, pes cavus. This report details a case of CAPOS syndrome resulting from a maternal ATP1A3 gene mutation. Both the child and her mother exhibited symptoms post-febrile induction,including severe sensorineural hearing loss in both ears, ataxia, areflexia, and decreased vision. Additionally, the patient's mother presented with pes cavus. Genetic testing revealed a c. 2452G>A（Glu818Lys） heterozygous mutation in theATP1A3 gene in the patient . This article aims to enhance clinicians' understanding of CAPOS syndrome, emphasizing the case's clinical characteristics, diagnostic process, treatment, and its correlation with genotypeic findings.
Humans ; Child ; Female ; Cerebellar Ataxia/diagnosis* ; Talipes Cavus ; Hearing Loss, Sensorineural/diagnosis* ; Optic Atrophy/diagnosis* ; Mutation ; Phenotype ; Sodium-Potassium-Exchanging ATPase/genetics* ; Foot Deformities, Congenital ; Reflex, Abnormal

Sodium ; Wastewater ; Potassium ; Diet ; Blood Pressure ; China ; Sodium, Dietary ; Sodium Chloride, Dietary

BACKGROUND: Sacubitril/valsartan is currently a standard medication in the treatment of reduced ejection fraction heart failure (HFrEF), and studies have also shown its efficacy for controlling hypertension. However, its efficacy varies between populations, and current recommendations are predominantly based on non Asian data. Hence, this study synthesizes the available evidence to determine its overall efficacy and safety among Asians. METHODS: A systematic search through PubMed, ScienceDirect, Cochrane, HERDIN PLUS, and ClinicalTrials.gov was done to include randomized controlled trials with Asian data comparing sacubitril/valsartan against an active control. The Cochrane Risk of Bias 2.0 was used to assess each article for bias. Forest plots in fixed-effects model for major adverse cardiovascular events (MACEs), hypertension control, and safety were created using RevMan 5.4. RESULTS AND DISCUSSION: Ten articles with an overall low risk of bias were included involving 6120 Asians. Sacubitril/valsartan showed better hypertension control against conventional angiotensin blocker (odds ratio [OR], 1.63; confidence interval [CI], 1.38–1.92; I2 = 7%). However, MACE reduction was not significant in HFrEF (hazard ratio, 0.89; CI, 0.73–1.08; I2 = 0%) or acute myocardial infarction (hazard ratio, 0.90; CI, 0.65–1.24; I2 = 0%). Safety was comparable to conventional angiotensin-converting enzyme inhibitors angiotensin receptor blocker (ARB) with a severe adverse event OR of 0.81 (CI, 0.44–1.50; I2 = 38%) and nonsevere adverse event OR of 1.09 (CI, 0.88–1.35; I2 = 44%). These results implicate the nee for efficacy studies focused on Asians, reassessment of the strength of recommendations in the treatment of heart failure, and consideration of sacubitril/valsartan as a treatment option for hypertension. CONCLUSION Among Asians, better hypertension control is seen with LCZ696 than conventional ARB. However, MACE reduction in HFrEF or acute myocardial infarction is insignificant, although there is a trend toward benefit. Finally, safety is comparable to conventional angiotensin-converting enzyme inhibitors/ARBs.\.
Asian ; Heart Failure ; Hypertension ; LCZ696 ; sacubitril and valsartan sodium hydrate drug combination

Sodium nitrite (SN, NaNO2) is a water-soluble, white-yellow crystalline powder with broad applications in food preservation, automotive maintenance, and animal control. It is a strong oxidizing agent that can oxidize hemoglobin iron (Fe) to its oxidized state, leading to methemoglobin formation. An increasing trend of suicide cases by SN ingestion has been reported globally following its popularization in online suicide forums providing detailed instructions of its use solely or as part of a “suicide kit.” We report a case of a 21-year-old male who was found continuously vomiting, with blood per orem and cyanosis of the mouth and digits. Within minutes of the onset of symptoms, the patient lost consciousness and was pronounced dead on arrival at the nearest emergency room. Autopsy findings showed lip erosions, hemorrhage, and perioral and peripheral cyanosis. Internal examination showed characteristic bright red muscle discoloration, dark brown arterial blood, red-brown congested visceral organs, and hyperemic esophageal and gastric mucosa. Methemoglobin studies from sampled arterial blood showed elevated levels (17.5%). Further investigation of the decedent’s belongings, social media posts, and recent online purchases reinforced the intentional sodium nitrite ingestion. While there are plenty of reported SN poisoning in suicide cases internationally, limited reports have been published locally. Death by SN poisoning is preventable with Methylene blue. The role of forensic pathologists through autopsy may be the last chance to detect such cases. The lack of systemic death investigation, experts, and local laboratories to reliably detect the signs of SN poisoning may have affected the low detection rate of cases locally. Further reporting of cases can raise the awareness of medical professionals that is fundamental to the ultimate saving of lives.

BACKGROUND

With the utility of nail dermoscopy to supplement the diagnosis of onychomycosis, studies have been done in describing common findings. However, most of these were based in Europe and the Americas. Given the geographical variation of onychomycosis, a local study is needed.

The general objective of this cross-sectional study is to determine the different patterns among the clinical subtypes of laboratory diagnosed onychomycosis patients seen at the Philippine General Hospital (PGH).

Clinically suspected Filipino onychomycosis patients seen at the PGH - Department of Dermatology, and at the PGH inpatient wards were recruited. All patients underwent the following: direct KOH microscopy, fungal culture studies, and nail clipping with PAS staining.

A total of 75 samples were included; 93.33% of the samples were categorized as DLSO subtype, 5.33% as TDO, and 1.33% as PSO. The three most common onychoscopic patterns were subungual hyperkeratosis (93.33%), chromonychia (85.33%), and longitudinal striae in 81.33%. KOH was positive in 57.33% of the cases, cultures in 38.67% and PAS stain in 80%. Onychoscopic pattern was not associated with clinical type of onychomycosis and causative organism. Negative KOH result was associated with linear edges (p=0.02).

Among patients with clinical and laboratory diagnosed onychomycosis, linear edges and jagged proximal edges were associated with negative KOH microscopy and fungal CS, respectively. Hence, in situations where such examinations are negative despite a strong clinical suspicion of onychomycosis, the above onychoscopic findings can be used for empirical diagnosis.

BACKGROUND

Accurate diagnosis of onychomycosis is important because its treatment is long-term, costly, and sometimes with complications. KOH test is quick and cheap but has low sensitivity. Hence, the need for additional tests such as culture and/or PAS. However, these two tests are not readily available and expensive. Dermoscopy is an emerging tool for the diagnosis of nail conditions.

This study aims to evaluate the accuracy of dermoscopy as a point-of-care tool in diagnosing distal subungual onychomycosis (DSO).

This is a prospective, cross-sectional study of 22 clinically diagnosed DSO patients selected via convenience sampling in a tertiary hospital from November 2019 to March 2021. Participants had gross nail examination, dermoscopy, KOH and PAS tests. Measures of diagnostic accuracy for the different dermoscopic patterns were obtained, with KOH and PAS results as reference standard.

Fifty-one nail samples were submitted for processing with only 49 nails accepted for analysis. The most common pattern was jagged edge with spikes (65.3%). Combining all 5 dermoscopic patterns increased the sensitivity to 91.2% (95% CI: 76.3, 98.1).

Dermoscopy may be used as a first-step, point-of-care tool in the diagnosis of DSO. Addition of mycological and histopathological tests is still warranted for confirmation.

BACKGROUND

Caustic ingestion is a major public health problem worldwide [1]. It imposes huge economic burden in terms of medical cost to poorly resourced health system of developing countries [2]. Household cleaning products, such as sodium hypochlorite (NaOCI), are the most common cause of caustic exposure [3]. NaOCI ingestion consistently ranks second in caustic ingestion cases in Northern Mindanao Medical Center (NMMC) based on toxicology census from 2015 to 2018. The aim of this study is to determine the epidemiologic profile, clinical findings and outcome of admitted patients who ingested NaOCI.

This a single-center descriptive, cross-sectional study of 40 cases of sodium hypochlorite ingestion admitted at NMMC from January 2015 to December 2018.

There were a total of 17 males and 23 females. Majority of patients were 0-5 years of age (46.5%; mean age of 2), followed by age range of 18-64 years old (40.0%; mean age of 35). Twenty-nine cases (72. 5%) cases of NaOCI ingestion were accidental and the remaining 11 (27.5%) were non-accidental. The non-accidental cases belong to the 12-17 (mean age 15.3) and 18-64 (mean age 31) years old age groups at 33.3% and 66.7%, respectively, with prevalence in females at 81.8%. Non-accidental cases increase with age, and among females. Majority of all ingested less than 50 ml of NaOCI. Two non-accidental cases (18.2%) ingested more than 100 ml of NaOCI were observed to have more than 2 signs and symptoms. Majority of patients presented with vomiting. Greater portion of non- accidental cases complained of abdominal or epigastric pain (54.5% vs 10%), oropharyngeal erythema (9.1 % vs 6.9), and odynophagia (45.5% vs 0). None of the patients had serious signs and symptoms. All patients were discharged improved and without sequelae.

Most NaOCI cases were accidental especially in children. Non-accidental cases increase significantly with age, with most cases found among adolescents and young adults, with higher prevalence among females. Majority of patients only ingested less than 50 ml, and that ingestion of 100 ml or more of NaOCI is associated with more than 2 signs and symptoms. The most predominant symptom is vomiting. Greater portion of non-accidental cases complained of abdominal or epigastric pain and odynophagia. None of the patients exhibit severe signs and symptoms. This study showed that all 40 cases of sodium hypochlorite ingestion did not have sequelae upon discharge.

BACKGROUND: The clinical importance of hypokalemia is likely underrecognized in Chinese dialysis patients, and whether its clinical effect was mediated by serum albumin is not fully elucidated. This study aimed to explore the association between serum potassium and mortality in dialysis patients of a Chinese nationwide multicenter cohort, taking albumin as a consideration. METHODS: This was a prospective nation-wide multicenter cohort study. Restricted cubic splines were used to test the linearity of serum potassium and relationships with all-cause (AC) and cardiovascular (CV) mortality and a subsequent two-line piecewise linear model was fitted to approach the nadir. A mediation analysis was performed to examine relations of albumin to potassium and mortalities. RESULTS: A total of 10,027 patients were included, of whom 6605 were peritoneal dialysis and 3422 were hemodialysis patients. In the overall population, the mean age was 51.7 ± 14.8 years, 55.3%(5546/10,027) were male, and the median dialysis vintage was 13.60 (4.70, 39.70) months. Baseline serum potassium was 4.30 ± 0.88 mmol/L. After a median follow-up period of 26.87 (14.77, 41.50) months, a U-shape was found between potassium and mortality, and a marked increase in risk at lower potassium but a moderate elevation in risk at higher potassium were observed. The nadir for AC mortality risk was estimated from piecewise linear models to be a potassium concentration of 4.0 mmol/L. Interestingly, the significance of the association between potassium and mortality was attenuated when albumin was introduced into the extended adjusted model. A subsequent significant mediation by albumin for potassium and AC and CV mortalities were found ( P < 0.001 for both), indicating that hypokalemia led to higher mortality mediated by low serum albumin, which was a surrogate of poor nutritional status and inflammation. CONCLUSIONS Associations between potassium and mortalities were U-shaped in the overall population. The nadir for AC mortality risk was at a potassium of 4.0 mmol/L. Serum albumin mediated the association between potassium and AC and CV mortalities.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; East Asian People ; Hypokalemia/etiology* ; Kidney Failure, Chronic/mortality* ; Potassium/blood* ; Prospective Studies ; Renal Dialysis ; Serum Albumin/analysis*