An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.

An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.

An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.

An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.

An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.

Purpose: Spinal muscular atrophy (SMA) is an autosomal recessive genetic disease characterized by the loss of motor neurons in the spinal cord and brainstem, leading to muscle atrophy and weakness. To understand the diagnostic process of Korean patients with SMA, we analyzed their clinical characteristics and challenges. Materials and Methods: We conducted a retrospective study of 38 patients with SMA (9 type II and 29 type III) between January 2000 and September 2023. Clinical, laboratory, and genetic data were reviewed. Results: The median ages at symptom onset and diagnosis were 3.0 years [interquartile range (IQR): 1.0–7.3 years] and 25.0 years (IQR: 10.5–37.3 years), respectively. The median diagnostic delay was 19.6 years (IQR: 6.4–31.0 years). A significantly longer delay was observed in SMA type III patients (median: 21.0 years, IQR: 11.0–31.0 years) compared to SMA type II patients (median: 3.0 years, IQR: 0.9–21.0 years) (p=0.021). No significant difference was observed in the number of clinic visits before diagnosis between patients with SMA type II (median: 2.0, IQR: 1.0–4.5) and those with type III (median: 2.0, IQR: 2.0–6.0, p=0.282). The number of clinic visits before diagnosis showed no significant association with the age at symptom onset and diagnosis (p=0.998 and 0.291, respectively). Conclusion Our investigation is the first examination of the diagnostic journey of Korean patients with SMA. As treatments for SMA progress, the significance of an accurate diagnosis has increased, highlighting the importance of reviewing the diagnostic advancements made thus far.

Background/Aims: Daratumumab has shown an encouraging antitumor effect in patients with multiple myeloma (MM), and was known to alter the immune properties by off-targeting immunosuppressive cells. Here, we aimed to evaluate the change in absolute lymphocyte count (ALC) as a surrogate marker for predicting survival outcomes of patients treated with daratumumab. Methods: Between 2018 and 2021, the medical records of patients with relapsed/refractory MM (RRMM) treated with daratumumab monotherapy at 10 centers in South Korea were reviewed. We collected the ALC data at pre-infusion (D0), day 2 after the first infusion (D2), and prior to the third cycle of daratumumab therapy (D56). Results: Fifty patients who were administered at least two cycles of daratumumab were included. Overall response rate was 54.0% after two cycles of daratumumab treatment. On D2, almost all patients experienced a marked reduction in ALC. However, an increase in ALC on D56 (ALCD56) was observed in patients with non-progressive disease, whereas failure of ALC recovery was noted in those with progressive disease. Patients with ALCD56 > 700/μL (n = 39, 78.0%) had prolonged progression- free survival (PFS) and overall survival (OS) than those with ALCD56 ≤ 700/μL (median PFS: 5.8 months vs. 2.6 months, p = 0.025; median OS: 24.1 months vs. 6.1 months, p = 0.004). In addition, ALCD56 >700/μL was a significant favorable prognostic factor for PFS (hazard ratio [HR], 0.22; p = 0.003) and OS (HR, 0.23; p = 0.012). Conclusions Increase in ALC during daratumumab treatment was significantly associated with prolonged survival outcomes in patients with RRMM. The ALC value can predict clinical outcomes in patients treated with daratumumab.

Purpose: In patients with glufosinate poisoning, severe neurological symptoms may be closely related to a poor prognosis, but their appearance may be delayed. Therefore, this study aimed to determine whether the Acute Physiology and Chronic Health Evaluation II (APACHE II) score could predict the neurological prognosis in patients with glufosinate poisoning who present to the emergency room with alert mental status. Methods: This study was conducted retrospectively through a chart review for patients over 18 years who presented to a single emergency medical center from January 2018 to December 2022 due to glufosinate poisoning. Patients were divided into groups with a good neurological prognosis (Cerebral Performance Category [CPC] Scale 1 or 2) and a poor prognosis (CPC Scale 3, 4, or 5) to identify whether any variables showed significant differences between the two groups. Results: There were 66 patients (67.3%) with good neurological prognoses and 32 (32.8%) with poor prognoses. In the multivariate logistic analysis, the APACHE II score, serum amylase, and co-ingestion of alcohol showed significant results, with odds ratios of 1.387 (95% confidence interval [CI], 1.027–1.844), 1.017 (95% CI, 1.002–1.032), and 0.196 (95% CI, 0.040–0.948), respectively. With an APACHE II score cutoff of 6.5, the AUC was 0.826 (95% CI, 0.746–0.912). The cutoff of serum amylase was 75.5 U/L, with an AUC was 0.761 (95% CI, 0.652–0.844), and the AUC of no co-ingestion with alcohol was 0.629 (95% CI, 0.527–0.722). Conclusion The APACHE II score could be a useful indicator for predicting the neurological prognosis of patients with glufosinate poisoning who have alert mental status.

Background: Normocalcemic primary hyperparathyroidism (NPHPT) was first described in 2008. It is defined as consistently elevated serum parathyroid hormone (PTH) levels with normal serum calcium (sCa) concentration, after excluding secondary causes of PTH elevation. However, the exact definition and management strategy for NPHPT remain controversial. We retrospectively investigated the clinicopathological features and short-term outcomes of NPHPT patients. Methods: A total of 280 patients who were surgically indicated for primary hyperparathyroidism (PHPT) at the Yonsei Severance Medical Center between 2015 and 2019 were included. Patients were classified according to preoperative PTH, corrected sCa, and ionized calcium (iCa) levels as follows: typical primary hyperparathyroidism (TPHPT, elevated PTH, sCa, and iCa, n = 158) and NPHPT (elevated PTH, normal sCa, n = 122). Results: NPHPT was commonly seen in younger individuals (aged < 50 years, P = 0.025);nephrolithiasis and bone fractures were common. Preoperative PTH level was higher in the TPHPT group (P < 0.001). The NPHPT group had higher numbers of multiple parathyroid lesions (P = 0.004) that were smaller (P = 0.011). NPHPT patients were further divided into two subgroups according to iCa levels: the elevated (n = 95) and normal iCa (n = 27) groups. There was no significant difference between the two subgroups regarding symptoms and multiplicity of lesions. Conclusion We found that NPHPT may be a heterogeneous disease entity of PHPT with high rates of multi-gland disease, which appears to be biochemically milder but symptomatic.Intraoperative PTH monitoring might help increase the surgery success rate. Moreover, the short-term outcomes of NPHPT after surgery did not differ from that of TPHPT.