Familial male-limited precocious puberty (FMPP) is a rare form of gonadotropin-independent precocious puberty that is caused by an activating mutation of the LHCGR gene. Herein, we report a case of FMPP with a mutation of the LHCGR gene in a Korean boy with familial history of precocious puberty through 3 generations. A 16-month-old boy presented with signs of precocious puberty, including pubic hair, acne, and increased growth velocity. The patient's grandfather and father had a history of precocious puberty and profound short stature. On physical examination, the patient had prepubertal testes with pubic hair development appropriate for Tanner stage II. The stretched penile length was 7 cm (>2 standard deviation score), and observed bone age was that of a 4-year-old boy. Laboratory findings showed high serum testosterone (5.74 ng/mL [appropriate for Tanner IV–V]; normal range, <0.05 ng/mL) with suppressed luteinizing hormone (<0.07 mIU/mL) and normal serum level of follicular stimulating hormone (0.56 mIU/mL; normal range, 0.38–1.11 mIU/mL). Genetic testing revealed a pathogenic variant of LHCGR (c.1730 C>T (p.Thr577Ileu)), confirming FMPP. Bicalutamide and anastrozole were administered, and pubertal progression was sufficiently suppressed without any specific side effects. To our knowledge, this is the first case of genetically confirmed FMPP in Korea.

Pregabalin is a gamma-aminobutyric acid analogue; it has been used clinically as an anticonvulsant and analgesic agent. Few documented reports exist of deaths resulting from pregabalin overdose. This report discusses a case of pregabalin intoxication in a 27-year-old male, who was found unconscious in a prison and later pronounced dead at a local hospital. An autopsy and toxicological analysis revealed the presence of pregabalin, alprazolam, diazepam, escitalopram, fluoxetine, lorazepam, bromazepam, flunitrazepam, zolpidem, and piroxicam. The concentrations of pregabalin and alprazolam were 10.3 mg/L and 0.10 mg/L in heart blood, and 11.4 mg/L and 0.08 mg/L in femoral blood, respectively. The other detected drugs were within therapeutic concentrations. Ethyl alcohol was not detected in the blood. Although the pregabalin concentration was within the therapeutic or toxic range, the concomitant use of other drugs, particularly benzodiazepines and zolpidem, likely enhanced its toxicity. Based on the autopsy findings and toxicological results, the cause of death was determined to be multidrug intoxication, including pregabalin. Although pregabalin is generally considered safe and deaths from its use alone are very rare, it can be fatal at relatively low blood concentrations when combined with opioids or other medications. The rising use of pregabalin in Korea increases the risk of overdose deaths, similar to this case. Therefore, in forensic practice, the possibility of such fatalities should be considered when pregabalin is detected.

Background/Aims: There is no clear consensus on the relative ranking of interventional and radiation techniques with indications similar to those of radiofrequency ablation (RFA) for the treatment of early hepatocellular carcinoma (HCC). We used a network meta-analysis to compare the efficacy of non-surgical treatments for early HCC. Methods: We searched databases for randomized trials assessing the efficacy of loco-regional treatments for HCCs ≤5 cm with no extrahepatic spread or portal invasion. The primary outcome was the pooled hazard ratio (HR) for overall survival (OS), and secondary outcomes included overall and local progression-free survival (PFS). A frequentist network meta-analysis was performed, and the relative ranking of therapies was assessed with P-scores. Results: Nineteen studies comparing 11 different strategies in 2,793 patients were included. Chemoembolization plus RFA improved OS better than RFA alone (HR 0.52, 95% confidence interval [CI] 0.33–0.82; P-score=0.951). Cryoablation, microwave ablation, laser ablation, and proton beam therapy had similar effects on OS compared with RFA. For overall PFS, but not local PFS, only chemoembolization plus RFA performed significantly better than RFA (HR 0.61, 95% CI 0.42–0.88; P-score=0.964). Injection of percutaneous ethanol or acetic acid was significantly less effective than RFA for all measured outcomes, while no differences in progression outcomes were identified for other therapies included in the network. Conclusions Our results suggest that chemoembolization combined with RFA is the best option for local treatment of early HCC. Cases with potential contraindications for RFA may benefit from a tailored approach using thermal or radiation modalities.

Rhabdomyolysis associated with psychogenic polydipsia and hyponatremia is a rare condition that can cause substantial morbidity and mortality. We report a 14-year-old boy with psychogenic polydipsia who experienced recurrent hyponatremia and subsequent rhabdomyolysis. Treatment involved intravenous fluids and restriction of oral water intake. This case emphasizes the importance of early recognition and management of this condition. The possibility of rhabdomyolysis should be considered in patients with hyponatremia who have myalgia.

OBJECTIVES: As few mpox cases have been reported in Korea, we aimed to identify the characteristics of mpox infection by describing our epidemiologic investigation of a woman patient (index patient, the third case in Korea) and a physician who was infected by a needlestick injury (the fourth case). METHODS: We conducted contact tracing and exposure risk evaluation through interviews with these 2 patients and their physicians and contacts, as well as field investigations at each facility visited by the patients during their symptomatic periods. We then classified contacts into 3 levels according to their exposure risk and managed them to minimize further transmission by recommending quarantine and vaccination for post-exposure prophylaxis and monitoring their symptoms. RESULTS: The index patient had sexual contact with a man foreigner during a trip to Dubai, which was considered the probable route of transmission. In total, 27 healthcare-associated contacts across 7 healthcare facilities and 9 community contacts were identified. These contacts were classified into high (7 contacts), medium (9 contacts), and low (20 contacts) exposure risk groups. One high-risk contact was identified as a secondary patient: a physician who was injured while collecting specimens from the index patient. CONCLUSIONS The index patient visited several medical facilities due to progressive symptoms prior to isolation. Although the 2022 mpox epidemic mainly affected young men, especially men who have sex with men, physicians should also consider mpox transmission in the general population for the timely detection of mpox-infected patients.

Background/Aims: We aimed to investigate the silent atherosclerotic burden of cervicocephalic vessels in cirrhotic patients compared with the general population, as well as the relevant risk factors including coronary parameters. Methods: This study included 993 stroke-free patients with liver cirrhosis (LC) who underwent magnetic resonance angiography (MRA) of the head and neck as a pre-liver transplant assessment and 6,099 health checkup participants who underwent MRA examination. The two cohorts were matched for cerebrovascular risk factors, and the prevalence of atherosclerosis in major intracranial and extracranial arteries was compared in 755 matched pairs. Moreover, traditional, hepatic, and coronary variables related to cerebral atherosclerosis were assessed in cirrhotic patients. Results: Overall, intracranial atherosclerosis was significantly less prevalent in the LC group than in the matched control group (2.3% vs. 5.4%, P=0.002), whereas the prevalence of extracranial atherosclerosis was similar (4.4% vs. 5.8%, P=0.242). These results were maintained in multivariate analyses of the pooled samples, with corresponding adjusted odds ratios [ORs] of LC of 0.56 and 0.77 (95% confidence intervals [CIs], 0.36–0.88 and 0.55–1.09). In the LC group, lower platelet count was inversely correlated with intracranial atherosclerosis (adjusted OR, 0.31; 95% CI, 0.13–0.76). Coronary artery calcium (CAC) score ≥100 was the only predictive factor for both intracranial and extracranial atherosclerosis (adjusted ORs, 4.06 and 5.43, respectively). Conclusions LC confers protection against intracranial atherosclerosis, and thrombocytopenia may be involved in this protective effect. High CAC score could serve as a potential surrogate for cervicocerebral vascular screening in asymptomatic cirrhotic patients.

Purpose: We examined the effects of the 8-week moderate-intensity Pilates exercise program on body composition, balance ability, metabolic parameters, arterial condition, and eating habits among overweight and obese women. Methods: From the general sample of overweight or obese Korean women (body mass index ≥ 23 kg/m2 ), those who had not been diagnosed with any chronic degenerative diseases were enrolled in the study (n = 39). After 8 weeks of the Pilates exercise program, the participants were subdivided into adherence and non-adherence groups. Among the study participants, 24 women were matched for age and menopausal status to reduce the bias, and then finally included for the comparison (Pilates-adherence, n = 12; Pilates-non-adherence, n = 12). Results: The body balance measured by the Y-balance test, body mass index, and subcutaneous fat areas were significantly improved in both groups. However, the Pilatesadherence group showed more positive changes in body balance and had significant improvement in body composition parameters such as waist size, visceral fat area, systolic blood pressure, arterial aging index, fasting blood glucose, and glycated hemoglobin than the Pilates-non-adherence group. In addition, the nutrition quotient for Korean adults (balance,moderation, and behavior except diversity) were significantly improved in both groups after dietary education. However, the participants did not show dramatic improvement in the metabolic parameters, because all the study subjects were in relatively good health and did not have any diagnosed diseases. Conclusion This study demonstrated that higher adherence to the Pilates exercise program together with a modification of eating habits may effectively improve body balance, body composition, and obesity-related parameters among overweight and obese women.

Purpose: A safe and effective hemostatic care is necessary after bone marrow examination to minimize bleeding, pain, and discomfort. However, a standardized hemostatic care protocol following bone marrow examination has not been established. The purpose of this study was to investigate the differences in bleeding, hematoma, pain, and discomfort by the hemostatic method used following bone marrow examination. Methods: This study was carried out with a pre-test/post-test nonequivalent control group design. Sixty-four patients undergoing bone marrow examination at the hemato-oncology ward in a tertiary hospital in South Korea were assigned to an intervention (n = 30) and comparison group (n = 34). The intervention group was treated using a compression dressing alone, while the comparison group received a compression dressing followed by sandbag compression. Both groups received two hours of bedrest. Bleeding, hematoma, pain, and discomfort were measured at one and two hours after the biopsy. Results: No significant differences in the occurrence of bleeding between the groups at one and two hours after bone marrow examination were observed, and no participant developed hematoma. The intervention group had significantly lower pain than the comparison group two hours after the bone marrow examination as well as lower discomfort one hour and two hours after the bone marrow examination (p < .05). Conclusion Applying only compression dressing after a bone marrow examination is effective in reducing pain and discomfort without measurable differences in bleeding and hematoma, suggesting that compression dressings alone could be effective in lowering pain and discomfort following bone marrow examination.

Chemokines are key factors that influence the migration and maintenance of relevant immune cells into an infected tissue or a tumor microenvironment. Therefore, it is believed that the controlled administration of chemokines in the tumor microenvironment may be an effective immunotherapy against cancer. Previous studies have shown that CCL3, also known as macrophage inflammatory protein 1-alpha, facilitates the recruitment of dendritic cells (DCs) for the presentation of tumor Ags and promotes T cell activation. Here, we investigated the role of CCL3 in regulating the tumor microenvironment using a syngeneic mouse tumor model. We observed that MC38 tumors overexpressing CCL3 (CCL3-OE) showed rapid regression compared with the wild type MC38 tumors. Additionally, these CCL3-OE tumors showed an increase in the proliferative and functional tumor-infiltrating T cells. Furthermore, PD-1 immune checkpoint blockade accelerated tumor regression in the CCL3-OE tumor microenvironment. Next, we generated a modified CCL3 protein for pre-clinical use by fusing recombinant CCL3 (rCCL3) with a non-cytolytic hybrid Fc (HyFc).Administering a controlled dose of rCCL3-HyFc via subcutaneous injections near tumors was effective in tumor regression and improved survival along with activated myeloid cells and augmented T cell responses. Furthermore, combination therapy of rCCL3-HyFc with PD-1 blockade exhibited prominent effect to tumor regression. Collectively, our findings demonstrate that appropriate concentrations of CCL3 in the tumor microenvironment would be an effective adjuvant to promote anti-tumor immune responses, and suggest that administering a long-lasting form of CCL3 in combination with PD-1 blockers can have clinical applications in cancer immunotherapy.

Purpose: Despite curative resection, the 5-year survival for patients with resectable pancreatic cancer is less than 20%. Recurrence occurs both locally and at distant sites and effective multimodality adjuvant treatment is needed. Materials and Methods: Patients with curatively resected stage IB-IIB pancreatic adenocarcinoma were eligible. Treatment consisted of chemotherapy with gemcitabine 1,000 mg/m2 on days 1 and 8 and cisplatin 60 mg/m2 on day 1 every 3 weeks for two cycles, followed by chemoradiotherapy (50.4 Gy/28 fx) with weekly gemcitabine (300 mg/m2/wk), and then gemcitabine 1,000 mg/m2 on days 1 and 8 every 3 weeks for four cycles. The primary endpoint was 1-year disease-free survival rate. The secondary endpoints were disease-free survival, overall survival, and safety. Results: Seventy-four patients were enrolled. One-year disease-free survival rate was 57.9%. Median disease-free and overall survival were 15.0 months (95% confidence interval [CI], 11.6 to 18.4) and 33.0 months (95% CI, 21.8 to 44.2), respectively. At the median follow-up of 32 months, 57 patients (77.0%) had recurrence including 11 patients whose recurrence was during the adjuvant treatment. Most of the recurrences were systemic (52 patients). Stage at the time of diagnosis (70.0% in IIA, 51.2% in IIB, p=0.006) were significantly related with 1-year disease-free survival rate. Toxicities were generally tolerable, with 53 events of grade 3 or 4 hematologic toxicity and four patients with febrile neutropenia. Conclusion Adjuvant gemcitabine and cisplatin chemotherapy followed by chemoradiotherapy with gemcitabine and maintenance gemcitabine showed efficacy and good tolerability in curatively resected pancreatic cancer.