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.

Human ; Metals, Heavy ; Tobacco ; Tobacco Products

Humans ; Aluminum/adverse effects* ; Pruritus/etiology* ; Skin Neoplasms ; Vaccination ; Hypersensitivity

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.

Cadmium/metabolism* ; Kidney ; Cells, Cultured ; Mitochondria ; Metallothionein/metabolism* ; Liver

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

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

INTRODUCTION: 68Ga-PSMA PET is an effective imaging modality in the evaluation of prostate cancer. However, there is limited data on its use in the evaluation of therapeutic response, particularly in radioligand therapy. OBJECTIVE: Our aim is to investigate the diagnostic accuracy of 68Ga-PSMA PET hybrid imaging in evaluating response to 177Lu-PSMA therapy in patients with mCRPC compared with the standard use of serum PSA. METHODOLOGY: A systematic review was done according to the Cochrane diagnostic accuracy reviews guidelines and the PRISMA checklist of literature from January 2015 to August 2020. Literature search, study selection, and data extraction were conducted by 2 reviewers. Statistical analysis of data was done using Meta-DiSc v1.4 RESULTS: A total of 5 studies were included following screening. A total of 128 patients were included in the review. Using PSA response as the reference standard, the pooled sensitivity and specificity of 68Ga-PSMA PET hybrid imaging to evaluate treatment response to 177Lu-PSMA therapy was 85% (Cl: 74 to 92%) and 74% (Cl: 62 to 84%), respectively. The computed diagnostic accuracy was 79.7%. CONCLUSION 68Ga-PSMA PET hybrid imaging is an effective diagnostic procedure in evaluating treatment response to 177Lu-PSMA therapy ligand therapy with good sensitivity, specificity, and diagnostic accuracy.
Gallium ; lutetium ; prostatic neoplasms