Background/Aims: A clinical unmet need persists for medications capable of modulating the progression of primary sclerosing cholangitis (PSC). This study aimed to assess the clinical feasibility of HK-660S (beta-lapachone) in PSC. Methods: In this multicenter, randomized, double-blind, placebo-controlled, parallel-group phase 2 trial, participants were assigned in a 2:1 ratio to receive either 100 mg of HK-660S or a placebo twice daily for 12 weeks. The primary outcomes were the reduction in serum alkaline phosphatase (ALP) levels and the percentage of participants showing improvements in PSC severity, as determined by magnetic resonance cholangiopancreatography with the Anali score. Secondary endpoints included changes in liver stiffness and adverse events. Results: The analysis included 21 patients, 15 receiving HK-660S, and six receiving a placebo. Improvements in the Anali score were observed in 13.3% of the HK-660S group, with no improvements in the placebo group. HK-660S treatment resulted in a 15.2% reduction in mean ALP levels, compared to a 6.6% reduction in the placebo group. A stratified ad-hoc analysis based on baseline ALP levels showed a statistically significant response in the HK-660S group among those with ALP levels greater than twice the upper limit of normal, with a 50% responder rate (p=0.05). Additionally, 26.7% of the HK-660S group showed improvements in the enhanced liver fibrosis score, with no improvements in the placebo group. HK-660S was generally well tolerated. Conclusions HK-660S is well tolerated among patients with PSC and may improve bile duct strictures, decrease serum ALP levels, and reduce liver fibrosis (cris.nih.go.kr, Number KCT0006590).

Colonoscopy, a widely used procedure for diagnosing and treating colonic diseases, induces transient gastrointestinal symptoms and alterations in the gut microbiota. This review comprehensively examines the evidence on alterations in the gut microbiota following colonoscopy and their possible mechanisms. Factors such as rapid colonic evacuation, increased osmolality, and mucus thinning caused by bowel preparation and exposure to oxygen during the procedure contribute to these alterations. Typically, the alterations revert to the baseline within a short time. However, their long-term implications remain unclear, necessitating further investigation. Split-dose bowel preparation and CO2 insufflation during the procedure result in fewer alterations in the gut microbiota. Probiotic administration immediately after colonoscopy shows promise in reducing alterations and gastrointestinal symptoms. However, the widespread use of probiotics remains controversial due to the transient nature of both the symptoms and gut microbial alterations following a colonoscopy. Probiotics may offer greater benefits to individuals with preexisting gastrointestinal symptoms. Thus, probiotic administration may be a viable option for selected patients.

Ectopic thyroid is thyroid tissue found in places other than the anterolateral aspect of the second to fourth tracheal ring. Mediastinal ectopic thyroid is rare and only few cases have been reported. The authors experienced a case of 41-year-old female patient with an anterior neck mass. The patient had mild chest discomfort when breathing with no other symptoms. Imaging studies suggested tumor of thymic tissue origin and surgical excision was done. The mass was successfully removed and histopathologically determined to be thyroid tissue. We hereby report with a review of literature a case of ectopic thyroid found in the mediastinum, which was successfully removed by transcervical incision.

Objective: : Palliative care is a specialized approach designed to enhance the quality of life for both patients and their families, offering patient-centered care through comprehensive assessment and care planning. However, the integration of palliative care within neurocritical care settings has been relatively understudied. This descriptive study aims to identify the characteristics, palliative care needs, and outcomes of patients referred to palliative care services during admission to the neurosurgical intensive care unit (NS-ICU). Methods: : A retrospective analysis of adults admitted to the NS-ICU at a referral hospital between December 2019 and December 2021 was conducted. The study focused on those referred to the inpatient palliative care team with diagnoses of non-traumatic brain hemorrhage, traumatic brain injury, or brain neoplasm. Excluded were patients who died before palliative care consultation or lacked sufficient information. The investigation assessed demographic and clinical characteristics at consultation, along with post-consultation hospital outcomes derived from medical records and interview notes. Results: : In this study involving 38 enrolled patients, the median age was 65, with 42.1% females. The most prevalent diagnosis was nontraumatic brain hemorrhage (47.4%). Reasons for palliative care consultation included psychosocial support (95%), goal-of-care discussions (68%), decision-making support (50%), and communication facilitation (39%). The median time from NS-ICU admission to consultation was 3.5 days (interquartile range, 1–8 days), and all interviews involved family members. Key decision topics encompassed mechanical ventilation (23.7%) and tracheostomy (21.1%). Patient preferences for life-sustaining treatment could be estimated in only 47.4% of cases, often resulting in treatment disagreement. Among the 38 patients, 26 (68.4%) died during admission. Before the consultation, full code status, partial code status, and comfort care alone were reported as 32%, 66%, and 2%, respectively; post-consultation, these figures shifted to 11%, 42%, and 47%, respectively. Conclusion : Palliative care was predominantly sought for psychosocial support and discussions concerning goals of care. Despite challenges in ascertaining patient treatment preferences, palliative care consultations proved invaluable in aiding family members and facilitating treatment decision-making. Our study suggests the potential integration of palliative care within neuro-critical care, contributing to a heightened utilization of comfort care at the end-of-life.

Managing giant partially thrombosed intracranial aneurysms presents significant challenges due to their unfavorable natural history and the lack of standardized treatment approaches. Conventional treatments, whether open surgical or endovascular, often struggle to manage these aneurysms effectively, resulting in high recurrence rates or significant morbidity. The patient was a 62-year-old male with a symptomatic giant partially thrombosed aneurysm at the tip of the basilar artery, presenting with left-sided hemiparesis and dysarthria. Diagnostic imaging revealed a giant aneurysm with a wide-necked, canalized portion. A two-stage endovascular treatment was conducted, involving a balloon occlusion test and intraoperative monitoring for maximum patient safety. The treatment utilized stent-assisted Woven EndoBridge (WEB) embolization and serial bilateral vertebral artery trapping. The procedure successfully isolated the aneurysm and postoperative imaging confirmed the absence of recanalization, preserving the intact posterior circulation. The patient showed stable recovery and no neurological deficits during the 12-month follow-up period. This technical note demonstrates the feasibility and efficacy of strategically integrating intrasaccular flow diversion using a WEB device and flow reversal through bilateral vertebral artery trapping for treating giant partially thrombosed aneurysms.

Plexiform neurofibromas (PNFs), a critical clinical feature of neurofibromatosis type 1, mainly involve several peripheral nerve branches and extend widely, including the skin and bones and the internal organs. Determining the appropriate treatment is difficult. Additionally, they possess the potential to develop into malignant peripheral nerve sheath tumors (MPNSTs), which are linked to an extremely poor prognosis. Active treatment is critical in patients with symptoms or progressive tumor growth, especially in pediatric cases. Surgery remains the standard treatment for managing PNFs and MPNSTs; however, it has often demonstrated insufficient results because of its wide distribution and the frequent involvement of major organs. Selumetinib, a recently approved mitogen-activated protein kinase kinase inhibitor, is gaining traction in treating inoperable PNFs in children.

Background: Improving β-cell function can lead to remission in some patients with type 2 diabetes mellitus (T2DM). However, research on pharmacotherapy-induced remission in youth-onset T2DM remains scarce. Our study aimed to identify the clinical characteristics of pediatric patients who experience remission. Methods: We retrospectively reviewed 88 pediatric patients with T2DM followed for at least 1 year at Seoul National University Bundang Hospital between 2013 and 2023. Remission was defined as a glycosylated hemoglobin (HbA1c) level less than 6.5% for at least 3 months after ceasing glucose-lowering pharmacotherapy. Results: Among 88 patients (60 males, 68.2%) diagnosed at an average age of 14.4±2.1 years, 19 patients (21.6%) achieved remission after a median duration of 1.4 years. The remission group had a larger proportion of males (89.5% vs. 62.3%, P=0.024) and a lower urinary albumin-to-creatinine ratio (ACR) at diagnosis (P=0.011). They also showed lower HbA1c levels at 1 year and more significant changes in HbA1c and body mass index (all P<0.05). Higher urinary ACR levels correlated with a longer duration to achieve remission (hazard ratio, 0.928; P= 0.013). In three of the 19 remission patients (15.8%), recurrence occurred after a median of 1.5 years. Conclusion Among Korean youth with T2DM, 21.6% achieved remission after a median duration of 1.4 years.Those who experienced remission were predominantly male, had lower ACR at diagnosis, and had significant weight loss within the first year. Further investigation into the factors influencing remission and long-term outcomes is essential.

Background: Primary Merkel cell carcinoma of the salivary gland is currently not listed in the World Health Organization classification. However, cases of Merkel cell type neuroendocrine carcinomas of the salivary gland with perinuclear cytokeratin 20 positivity have been intermittently reported. We here investigated the clinicopathologic features of additional cases. Methods: Data of four cases of Merkel cell type small cell neuroendocrine carcinoma of the salivary gland were retrieved. To confirm the tumors’ primary nature, clinical records and pathologic materials were reviewed. Optimal immunohistochemical staining was performed to support the diagnosis. Results: All tumors were located in the parotid gland. Possibilities of metastasis were excluded in all cases through a meticulous clinicopathological review. Tumor histology was consistent with the diagnosis of small cell neuroendocrine carcinoma. Tumors’ immunohistochemical phenotypes were consistent with Merkel cell carcinoma, including Merkel cell polyomavirus large T antigen positivity in two of the four cases. Conclusions Merkel cell carcinomas can originate in salivary glands and are partly associated with Merkel cell polyomavirus infection as in cutaneous Merkel cell carcinomas.

Chronic active Epstein-Barr virus (CAEBV) can induce complications in various organs, including the brain and gastrointestinal tract. A 3-year-old boy was referred to the hospital with a history of fever and seizures for 15 days. A diagnosis of encephalitis based on computed tomography (CT) and magnetic resonance imaging findings and clinical correlation was made. Laboratory tests showed positive serology for Epstein-Barr virus (EBV) and negative for Rotavirus antigen and IgG and IgM antibodies for cytomegalovirus, herpes simplex virus, and varicella zoster virus, respectively. Abdominal CT showed diffuse wall thickening with fluid distension of small bowel loops, lower abdomen wall thickening, and a small amount of ascites. The biopsy demonstrated positive Epstein-Barr encoding region in situ hybridization in cells within the crypts and lamina propria. The patient was managed with steroids and hematopoietic stem cell transplantation (HSCT). This case showed histopathological characteristics of concurrent EBV-associated encephalitis and colitis in CAEBV infection. The three-step strategy of immunosuppressive therapy, chemotherapy, and allogeneic HSCT should be always be considered for prevention of disease progression.