Background and Objectives: Clinical characteristics and outcomes in acute heart failure (AHF) vary by phenotype. We assessed phenotype-specific features, treatment patterns, and outcomes in a nationwide Korean cohort. Methods: The Korean Heart Failure III registry prospectively enrolled 7,351 AHF admissions at 47 hospitals. Among 6,777 patients with available left ventricular ejection fraction (EF), phenotypes were defined as heart failure with reduced EF (HFrEF, ≤40%), mildly reduced EF (HFmrEF,41–49%), or preserved EF (HFpEF, ≥50%). The primary endpoint was a 12-month composite of all-cause death or heart transplantation, evaluated from index admission and, among hospital survivors, from discharge. We used inverse probability weighting (multinomial generalized boosted models with stabilized, trimmed weights) and weighted Cox proportional-hazards models to estimate hazard ratios (HRs). Results: Phenotype distribution was 58.9% HFrEF, 13.6% HFmrEF, and 27.5% HFpEF. Crude 12-month composite rates from index admission were 13.4% (HFrEF), 12.7% (HFmrEF), and 16.8% (HFpEF). After weighting, from index admission, HFmrEF (HR, 0.892; 95% confidence interval [CI], 0.731–1.088) and HFpEF (HR, 1.101; 95% CI, 0.939–1.291) did not differ from HFrEF; from discharge, HFpEF had modestly higher risk (HR, 1.207; 95% CI, 1.008–1.445) whereas HFmrEF did not (HR, 1.039; 95% CI, 0.844–1.279). Hyponatremia and chronic kidney disease were consistent adverse markers, while angiotensin-converting enzyme inhibitor/ angiotensin II receptor blocker use at discharge was protective. Conclusions Across the EF spectrum, phenotypes showed distinct profiles and risk. Postdischarge risk was modestly higher in HFpEF, supporting phenotype-tailored care and systematic discharge optimization in Korean patients with AHF.

Purpose: In this study, subjects with femoral anterior glide type were examined to investigate the effects of femoral head anterior glide fixation during active straight leg raise on the strength of the hip flexor in a supine position. Methods: Fifteen subjects participated in this study. All subjects were classified through an evaluation form for femoral anterior glide type (FAGT). The strength of the hip flexor was measured during active straight leg raise test (ASLR test), and compared with and without femoral anterior glide fixation in a supine position. The fixation of the femoral head was achieved as per the therapist’s manual guidelines. Paired t-test was applied to compare changes in the strength of the hip flexor according to fixation conditions. The level of statistical significance was set at α= 0.05. Results: The strength of the hip flexor was lesser during the ASLR test with fixation compared to without fixation (p= 0.007). Conclusion The strength of the hip flexor decreases with fixation. Results of this study revealed a difference between hip flexor strength, with and without femoral anterior glide fixation during ASLR, in subjects with femoral anterior glide type.

Background: Gaucher disease (GD) is an autosomal recessive disorder characterized by excessive accumulation of glucosylceramide in multiple organs. This study was performed to determine the detection rate of GD in a selected patient population with unexplained splenomegaly in Korea. Methods: This was a multicenter, observational study conducted at 18 sites in Korea between December 2016 and February 2020. Adult patients with unexplained splenomegaly were enrolled and tested for β-glucosidase enzyme activity on dried blood spots (DBS) and in peripheral blood leukocytes. Mutation analysis was performed if the test was positive or indeterminate for the enzyme assay. The primary endpoint was the percentage of patients with GD in patients with unexplained splenomegaly. Results: A total of 352 patients were enrolled in this study (male patients, 199; mean age, 48.42 yr). Amongst them, 14.77% of patients had concomitant hepatomegaly. The most common sign related to GD was splenomegaly (100%), followed by thrombocytopenia (44.32%) and, anemia (40.91%). The β-glucosidase activity assay on DBS and peripheral leukocytes showed abnormal results in sixteen and six patients, respectively. Eight patients were tested for the mutation, seven of whom were negative and one patient showed a positive mutation analysis result. One female patient who presented with splenomegaly and thrombocytopenia was diagnosed with type 1 GD. The detection rate of GD was 0.2841% (exact 95% CI, 0.0072‒). Conclusion The detection rate of GD in probable high-risk patients in Korea was lower than expected.However, the role of hemato-oncologists is still important in the diagnosis of GD.

The Carney complex (CNC) is an autosomal dominant disorder characterized by endocrine and nonendocrine tumors. Loss-of-function variants of protein kinase A regulatory subunit 1 alpha (PRKAR1A) are common causes of CNC. Here, we present the case of a patient with CNC with a novel PRKAR1A missense variant. A 21-year-old woman was diagnosed with CNC secondary to acromegaly and adrenal Cushing syndrome. Genetic analysis revealed a novel missense heterozygous variant of PRKAR1A (c.176A>T). Her relatives, suspected of having CNC, also carried the same variant. RNA analysis revealed that this variant led to nonsense-mediated mRNA decay. In vitro functional analysis of the variant confirmed its role in increasing protein kinase A activity and cyclic adenosine monophosphate levels. This study broadens our understanding of the genetic spectrum of CNC. We suggest that PRKAR1A genetic testing and counseling be recommended for patients with CNC and their families.

PURPOSE: To compare changes in anterior segment parameters after Nd:YAG laser capsulotomy in eyes that underwent either combined phacovitrectomy or cataract surgery. METHODS: This retrospective study enrolled 37 eyes of 35 patients with posterior capsular opacification treated with combined phacovitrectomy (group A), and 35 eyes of 32 patients with posterior capsular opacification treated with cataract surgery (group B). Anterior segment parameters, including anterior chamber depth (ACD), anterior chamber angle, and anterior chamber volume, were measured by a Pentacam before Nd:YAG laser capsulotomy and 1 hour, 1 day, 1 week, 1 month, and 3 months after this treatment. RESULTS: In the cataract surgery group, the ACD was significantly lower 1 day (3.75 ± 0.74 mm), 1 week (3.73 ± 0.24 mm), and 3 months (3.74 ± 0.33 mm) after Nd:YAG laser capsulotomy compared with the pretreatment value (4.20 ± 0.62 mm, p = 0.002). By contrast, the ACD did not change significantly over time in the combined phacovitrectomy group. The ACD differed significantly between the two groups at 1 week, 1 month, and 3 months after capsulotomy. There were no significant changes in the anterior chamber volume, anterior chamber angle, central corneal thickness, or pupil size from before to after capsulotomy in either group. A non-significant trend toward myopic shift was observed in group A (p = 0.072) and B (p = 0.055). CONCLUSIONS: The results of the present study may help determine the power of the intraocular lens in patients who underwent combined surgery or cataract surgery and who will receive Nd:YAG laser capsulotomy.
Anterior Chamber ; Cataract ; Humans ; Lenses, Intraocular ; Pupil ; Retrospective Studies