Introduction: Early identification of patients at risk for severe multisystem inflammatory syndrome in children (MIS-C) is essential for favourable clinical outcomes. This study aims to identify the clinical characteristics, factors and outcomes associated with severe MIS-C. Materials and methods: In this retrospective cohort study involving 14 major hospitals in Malaysia, children <15 years who met the United States Centres for Disease Control and Prevention case definition for MIS-C were included. Severe MIS-C was defined as children who required inotropic support, ventilatory support (invasive or non-invasive ventilation), or left ventricular ejection fraction of <55%. The factors investigated for severe MIS-C were demographic characteristics, the presence of comorbidities, clinical characteristics, and laboratory measures. Multivariable logistic regression was used to compute the adjusted odds ratio (aORs) of factors associated with severe MIS-C. Results: Among the 155 patients, 91 (58.7%) presented with severe MIS-C. Severe MIS-C was more likely in patients aged ≥5 years old (aOR 2.13, 95% confidence interval [CI] 1.08-4.21), with dehydration (aOR 3.80, 95% CI 1.53-9.45), lethargy (aOR 2.02, 95% CI 0.97-4.18), tachycardia (aOR 8.33, 95% CI 3.27-21.22), albumin <30g/L (aOR 3.36, 95% CI 1.58-7.13), creatine kinase >200U/L (aOR 3.68, 95% CI 1.57-8.64), D-dimer >3.0µg/mL (aOR 2.11, 95% CI 1.08-4.13), ferritin >500ng/mL (aOR 3.77, 95% CI 1.88-7.55), prothrombin time >12.7 seconds (aOR 3.22, 95% CI 1.61-6.43), and urea >6mmol/L (aOR 5.09, 95% CI 2.04-12.71). Conclusion: Identification of these associated factors of severity in MIS-C could aid in early recognition and prompt escalation of care, leading to better outcomes.

Background: Intrinsic capacity (IC) is defined as "all the physical and mental attributes possessed by the older person." This concept has gained momentum in recent years because it provides insights into the changes in the functional capacity of individuals during their life. This study examined common factors associated with IC decline among older adults in Mexico and Colombia. Methods: This cross-sectional, correlational study included 348 community-dwelling older adults. Sociodemographic, clinical, and family conditions were assessed as possible associated factors, and IC was analyzed across five domains: cognitive, locomotor, psychological, vitality (malnutrition through deficiency and excess), and sensory (visual and auditory). Parametric and non-parametric statistical analyses were performed. Results: The common factors associated with impairment according to domain were family dysfunctionality (cognitive domain); myocardial infarction, family dysfunctionality, age >80 years, home occupation, and not having a partner (locomotor domain); dysfunctional family and risk of falls (psychological domain); age >80 years and not having a partner (malnutrition by deficiency domain); age 60–79 years, walking <7,500 steps/day, and peripheral vascular disease (malnutrition by excess domain); risk of falling and being female (visual sensory domain); risk of falling (auditory sensory domain); and dysfunctional family and risk of falling (total intrinsic capacity). Conclusion Both populations had common sociodemographic, clinical, and familial factors that directly affected total IC stocks and their domains.

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder caused by uncontrolled terminal complement activation, which leads to intravascular hemolysis (IVH), thromboembolism (TE), renal failure, and premature mortality. Methods: We performed a secondary analysis of data collected from patients enrolled in the Korean National PNH Registry to assess the relative importance of risk factors, specifically lactate dehydrogenase (LDH) and hemoglobin (Hb), in predicting the incidence of TE, impaired renal function, and death in complement inhibitor-naïve patients with PNH. Results: Multivariate regression modeling indicated that LDH ≥ 1.5 × upper limit of normal (ULN), male sex, and pain were associated with increased risk of TE (P = 0.016, 0.045, and 0.033, respectively), hemoglobinuria and pain were associated with an increased risk of impaired renal function (P = 0.034 and 0.022, respectively), and TE was associated with an increased incidence of death (P < 0.001). Hb < 8 g/dL was not a predictor of TE, impaired renal function, or death in multivariate regression analyses. Standardized mortality ratio analysis indicated that LDH ≥ 1.5 × ULN (P < 0.001), Hb < 8 g/dL (P < 0.001), and Hb ≥ 8 g/dL (P = 0.004) were all risk factors for death; in contrast, patients with LDH < 1.5 × ULN had similar mortality to the general population. Conclusion In complement inhibitor-naïve patients with PNH, LDH ≥ 1.5 × ULN was a significant predictor of TE, and TE was a significant predictor of death. Hb was not a significant predictor of TE, impaired renal function, or death. Therefore, controlling IVH will improve clinical outcomes for patients with PNH.

This paper documents the first reported cases of patients in the country who underwent transvaginal ultrasound‑guided Tru‑cut biopsy of pelvic tumors in a tertiary level health institution in 2019. Different indications for Tru‑cut biopsy were demonstrated highlighting its clinical utility in the diagnosis and management of malignant pelvic tumors. All patients warranted histologic diagnosis for further planning of the most appropriate management. Adequate tissue samples were obtained from all three patients with no procedure‑related complications.

Context: Endometrial cancer is the third most common malignancy of the female genital tract in the Philippines, following cervical and ovarian cancer. Ultrasound as the first line in imaging has a major role in preoperative treatment and planning. Aims: To compare the diagnostic accuracy of subjective versus objective ultrasound measurement techniques in detecting cervical stromal invasion (CSI) and deep myometrial invasion (MI). Materials and Methods: Fifty‑seven patients were enrolled in this cross‑sectional study. Deep MI and CSI were evaluated both subjectively and objectively by measuring tumor‑free distance (TFD), distance from the outer cervical os to lowest edge of the tumor border (Dist‑OCO), and distance from the internal cervical os to caudal tumor border (Dist‑ICO). Histopathological result used as the gold standard. Results: Subjective assessment for deep (MI) had 79.3% sensitivity, 82.1% specificity, 82.1% positive predictive value (PPV), 82.1% negative predictive value (NPV), and 80.7%. Subjective assessment for CSI had a sensitivity, specificity, PPV, NPV, and overall accuracy of 80%, 90.4%, 44.4%, 97.9%, and 89.5%. Objective measurement (TFD ≤0.8 cm) to detect deep MI had 86.2% sensitivity, 57.1% specificity, 67.4% PPV, 80% NPV, and 71.9% overall accuracy. Adjusting TFD cutoff to 0.65 increased to 71.4% specificity, making it comparable with subjective assessment. Dist‑OCO (≤2.1 cm) yielded 100% sensitivity, 86.3% specificity, 30% PPV, 100% NPV, and 87% overall accuracy. Dist‑ICO was first used in this study, hence no cutoff yet. By using receiver operating characteristics, cutoff was 0.45 cm, which yielded a 60% sensitivity and 92% specificity (area under the curve 0.731, P = 0.09). Conclusions Subjective assessment of CSI and deep MI performs better than objective measurement techniques. TFD and Dist‑OCO as the objective measurements showed clinically comparable accuracy to subjective assessment by an expert. Dist‑ICO needs to be validated to a larger population to determine its clinical value in predicting CSI.
Endometrial Neoplasms

Pregnancy ; Female ; Humans ; Mosaicism ; Prenatal Diagnosis ; Prenatal Care ; Aneuploidy

Purpose: Targeted next-generation sequencing (NGS) is widely used for simultaneously detecting clinically informative genetic alterations in a single assay. Its application in clinical settings requires the validation of NGS gene panels. In this study, we aimed to validate a targeted hybridization capture-based DNA panel (ONCOaccuPanel) using the Illumina MiSeq sequencing platform. The panel allows the simultaneous detection of single-nucleotide variants (SNVs), insertions, deletions, and copy number changes of 323 genes and fusions of 17 genes in solid tumors. Materials and Methods: We used 16 formalin-fixed paraffin-embedded (FFPE) tumor samples with previously known genetic mutations and one reference material (HD827) for validation. Moreover, we sequenced an additional 117 FFPE tumor samples to demonstrate the clinical utility of this panel. Results: Validation revealed a 100% positive percentage agreement and positive predictive value for the detection of SNVs, insertions, deletions, copy number changes, fusion genes, and microsatellite instability–high types. We observed high levels of reproducibility and repeatability (R2 correlation coefficients=0.96-0.98). In the limit of detection assessment, we identified all clinically relevant genes with allele frequencies > 3%. Furthermore, the clinical application of ONCOaccuPanel using 117 FFPE samples demonstrated robust detection of oncogenic alterations. Oncogenic alterations and targetable genetic alterations were detected in 98.2% and 27.4% cases, respectively. Conclusion ONCOaccuPanel demonstrated high analytical sensitivity, reproducibility, and repeatability and is feasible for the detection of clinically relevant mutations in clinical settings.

We aimed to perform a systematic review and meta-analysis of all randomized placebo-controlled trials (RCTs) that examined the analgesic benefits of preemptive pregabalin among patients undergoing minimally invasive hysterectomy. Five major databases were systematically screened from inception until August 29, 2021 Relevant studies were evaluated for risk of bias. Endpoints were analyzed using the random-effects model and pooled as the mean difference or risk ratio with a 95% confidence interval. Four studies with seven treatment arms met the inclusion criteria. The total sample size was 304 patients: 193 and 111 patients were allocated to the pregabalin and placebo groups, respectively. Overall, the included studies revealed a low risk of bias. The summary results revealed that the mean postoperative pain scores at rest were significantly lower in the pregabalin group than in the control group at 0, 2, 4, 6, 12, and 24 hours. Moreover, the mean postoperative pain scores on movement/coughing were significantly lower in the pregabalin group than in the control group at 12 and 24 hours. The rate of patients who were opioid-free postoperatively was significantly higher in the pregabalin group than in the control group. There was no significant difference between the groups in terms of the mean postoperative time to first rescue analgesic and the rates of adverse events. Compared with placebo, preemptive pregabalin was largely safe, and was correlated with superior analgesic effects in terms of lower postoperative pain scores and higher opioid-sparing effects. Additional RCTs are needed to confirm these findings.

Among the three subtypes of neurofibromatosis are type 1 and 2 neurofibromatosis and schwannomatosis, von Recklinghausen disease also known as type 1 neurofibromatosis has an autosomal dominant inheritance. It is the commonest form as and presents with numerous café-au-lait macules and neurofibromas. Giant congenital melanocytic nevus (CGMN) on the other hand is characterized by a melanocytic proliferation that present at birth. CGMN develops due to a defective embryonic pigment cell (melanocyte) precursors development and are often present at birth. Giant congenital melanocytic nevus (CGMN) and type 1 neurofibromatosis may occur together rarely. Clinicians should be aware of the rare presentation of both CGMN and type 1 neurofibromatosis in a patient.

Gout is a commonly treated inflammatory arthritis that is often managed in the primary care setting. This disease is prevalent among the multi-ethnic Malaysian population. Unfortunately, gout is still frequently managed sub-optimally, even in the hospital and primary care settings. Gout should be considered a major disease since it can potentially lead to multiple disabilities from joint destruction, nephropathy and increased cardiovascular morbidity and mortality. The objectives of this review are to summarise the latest updated information and management of gout in the primary care setting.