Leiomyomas of vagina and vulva are rare solid tumors, but most common mesenchymal tumors in adult woman. Most of the reported cases have been benign. The tumors were firm, well-defined submucosal masses in a majority of patients. We experienced two cases of vulvar and vaginal leiomyoma, thus it is presented with a brief review of the cases and its literatures.
Adult ; Female ; Humans ; Leiomyoma* ; Vagina* ; Vulva*

Purpose: : Intensive care unit (ICU) diaries have been implemented across the international ICU community. This study aimed to comprehend the meaning and nature of the lived experience of patients’ families using the ICU diary in Korea. Methods: : This qualitative study adopted van Manen’s hermeneutic phenomenology. The participants comprised eight women and two men who were the family members of patients in the ICU for more than three days. Data were collected using in-depth interviews and observation from July 2018 to January 2019. Results: : Patients’ families who experienced the ICU diary recognized it with six beings according to time: a good idea, forgotten stuff, burdensome work, touching service, my stuff, and a thing in the memory. The ICU diary had three essential meanings for the families: communication, solace and hope, and a record of life. These findings were rearranged according to van Manen’s fundamental existential, and the lived things and lived others were remarkably confirmed. Conclusion : Patients’ families experienced various ICU diary forms over time and recognized an ICU diary as a means of communication. Therefore, the ICU diary is expected to be used as an intervention between families and healthcare providers in the ICU to support mutual communication.

Interferon regulatory factor 3 (IRF3) integrates both immunological and non-immunological inputs to control cell survival and death. Small GTPases are versatile functional switches that lie on the very upstream in signal transduction pathways, of which duration of activation is very transient. The large number of homologous proteins and the requirement for site-directed mutagenesis have hindered attempts to investigate the link between small GTPases and IRF3. Here, we constructed a constitutively active mutant expression library for small GTPase expression using Gibson assembly cloning. Small-scale screening identified multiple GTPases capable of promoting IRF3 phosphorylation. Intriguingly, 27 of 152 GTPases, including ARF1, RHEB, RHEBL1, and RAN, were found to increase IRF3 phosphorylation. Unbiased screening enabled us to investigate the sequence-activity relationship between the GTPases and IRF3. We found that the regulation of IRF3 by small GTPases was dependent on TBK1. Our work reveals the significant contribution of GTPases in IRF3 signaling and the potential role of IRF3 in GTPase function, providing a novel therapeutic approach against diseases with GTPase overexpression or active mutations, such as cancer.

Background: Diabetes is recognized as a risk factor for various inflammatory conditions, including periodontitis. There exists a bidirectional relationship between glycemic control and oral health in individuals with diabetes. This study aimed to analyze the link between glycemic control and oral health status among Korean patients with diabetes. Methods: Using data from a population-based nationwide survey conducted between 2007 and 2019, we identified 70,554 adults with diabetes-related information. The study population included 9,090 individuals diagnosed with diabetes and 61,164 healthy controls. The association between glycemic control, defined by mean glycated hemoglobin (HbA1c) values, and various oral health measures, such as tooth brushing frequency, periodontitis, denture wearing, Decayed, Missing, and Filled Teeth (DMFT) index, number of remaining teeth, and past-year dental clinic visits, was evaluated using multivariate logistic regression analyses. Results: Compared to the control group, patients with diabetes exhibited a higher prevalence of periodontitis (88.6% vs. 73.3%), complete dentures (5.0% vs. 1.5%), and elevated DMFT index (33.2% vs. 26.7%) (all P < 0.001). Multivariate analyses revealed significant associations between diabetes and several oral health factors: denture status (No denture: adjusted odds ratio [aOR], 0.784; 95% confidence interval [CI], 0.627–0.979), and having fewer permanent teeth (0–19) (aOR, 1.474; 95% CI, 1.085–2.003). Additionally, a positive correlation was found between higher HbA1c levels and the risk of having fewer remaining teeth (0–19) (HbA1c < 6.5%: aOR, 1.129; 95% CI, 0.766–1.663; 6.5% ≤ HbA1c < 8.0%: aOR, 1.590; 95% CI, 1.117– 2.262; HbA1c ≥ 8%: aOR, 1.910; 95% CI, 1.145–3.186) (P for trends = 0.041). Conclusion We found a positive association between diabetes and poor oral health, as well as a noteworthy relationship between reduced permanent teeth (≤ 19) and glycemic control.These insights emphasize the critical role of oral health management in diabetic care and underscore the importance of maintaining effective glycemic control strategies for overall health and well-being in patients with diabetes.

Acardiac fetus in triplet pregnancy is a very rare, fatal congenital anomaly that had not been reported in Korea. It only occurs in multiple gestations associated with placental vascular anastomoses between the affected fetus and its co-twin. The major complications associated with acardiac fetus in triplet pregnancy are congestive heart failure in normal pumping fetus, maternal polyhydramnios, preterm labor, intrauterine fetal death, etc, and perinatal diagnosis can be made with the perinatal ultrasonographic examination. We report a case of acardiac fetus in a spontaneous triplet pregnancy at 23 weeks of gestational age with a brief review of the literature.
Diagnosis ; Female ; Fetal Death ; Fetus* ; Gestational Age ; Heart Failure ; Humans ; Korea ; Obstetric Labor, Premature ; Polyhydramnios ; Pregnancy ; Pregnancy, Triplet* ; Triplets*

OBJECTIVE: Incidence of chronic subdural hematoma (CSDH) is gradually increasing in young adults for several reasons. In this study, we aimed to identify features of CSDH noted in young adults that distinguish the disease from CSDH diagnosed in the elderly. METHODS: One hundred eighty-two patients with CSDH who underwent a total of 218 surgical procedures between January 2003 and February 2010 were retrospectively reviewed with regard to clinical presentation, radiographic results and prognosis including recurrence. To compare younger patients with the elderly, patients were divided into three groups on the basis of age (Group A: < or =40, Group B: 41-64, Group C: > or =65 years). RESULTS: Group A showed a male predominance (p=0.0001), lower rate of recurrence (p=0.0012), shorter symptom duration (p=0.035), and fewer leading signs such as hemispheric symptoms (p=0.005) compared to Groups B and C. Radiologic findings such as maximal hematoma thickness (p<0.0001) and degree of midline shift (p=0.028) were less severe in Group A than Groups B and C. Alcoholism was the most prevalent illness in all three groups. When exempting infants with hematologic malignancy, non-recurrence, previous trauma history, headache as leading symptom, and no mortality were all common in younger adults (all p<0.05). CONCLUSION: Young adults with CSDH show less severe clinical and radiologic features as well as fewer recurrences than noted in the elderly population. Even if a clinician's index of suspicion of CSDH in young adults complaining of headache is not high, meticulous radiologic surveillance could find CSDH, leading to satisfactory results including less frequent recurrence.
Adult ; Aged ; Alcoholism ; Headache ; Hematologic Neoplasms ; Hematoma ; Hematoma, Subdural, Chronic ; Humans ; Incidence ; Infant ; Male ; Prognosis ; Recurrence ; Retrospective Studies ; Young Adult

OBJECTIVE: Because of the complex anatomical association among vascular, dural, and bony structures, paraclinoid internal carotid artery (ICA) aneurysms remain a major challenge for vascular neurosurgeons. We studied the clinical outcomes of 61 paraclinoid ICA aneurysms after microsurgical clipping in comparison with endovascular coiling. MATERIALS AND METHODS: Between January 2008 and December 2012, we treated 61 paraclinoid ICA aneurysms created by surgical clipping or endovascular coiling. Preoperative neurologic status and postoperative outcome were evaluated using the Glasgow coma scale (GCS) and the modified Rankin scale (mRS). Postoperative hydrocephalus and vasospasm were reviewed using the patients' medical charts. RESULTS: Most patients were in good clinical condition before the operations and had good treatment outcomes. Clinical vasospasm was observed after the operation in five patients, and hydrocephalus occurred in six patients. No statistically significant difference regarding aneurysm size, sex, GCS score, H-H grade, and mRS was observed between the surgical clipping group and the endovascular coiling group. In addition, the treatment results and complications did not show statistically significant difference in either group. CONCLUSION: Surgical occlusion of paraclinoid ICA aneurysms is difficult; however, no significant differences were observed in the treatment results or complications when compared with coil embolization. In particular, use of an adequate surgical technique may lead to better outcomes than those for coil embolization in the treatment of large and/or wide-neck paraclinoid ICA aneurysms.
Aneurysm* ; Carotid Artery, Internal* ; Embolization, Therapeutic ; Glasgow Coma Scale ; Humans ; Hydrocephalus ; Neck* ; Surgical Instruments

Nephrogenic systemic fibrosis (NSF) is a progressive systemic fibrosing disease that may occur after gadolinium contrast exposure. It can lead to severe complications and even death.NSF is highly prevalent among patients with advanced chronic kidney disease (CKD). In this report, however, we describe the case of a patient with NSF that occurred during early CKD. A 65-year-old man with stage 3a CKD was transferred to our hospital because of lower extremity edema. The medical history revealed that he was exposed to gadolinium 185 days earlier, and the result of his tibial skin biopsy was consistent with NSF. The patient underwent a combined therapy with ultraviolet-A1 phototherapy and methotrexate and steroid therapy for 6 months. The combined therapy stopped the systemic progression of NSF.

Neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant inherited disorders affecting the nervous system. NF1 is associated with mutations in the NF1 gene, which is located on chromosome sub-band 17q11.2 and contains 57 exons spanning approximately 300 kb of genomic DNA. NF1 is caused by a loss of function mutation of the NF1 gene, a tumor suppressor gene, which encodes for neurofibromin, a GTPase-activating protein (GAP) involved in the negative regulation of Ras activity. The GAP-related domain, which is encoded for by exons 20-27a, is one of the most important functional domains in neurofibromin. The cysteine-serine-rich domain has been recognized as an important functional domain in NF1-related pheochromocytomas. As the result of many genetic analyses of NF1-related pheochromocytomas, pheochromocytoma has generally been recognized as a true component of NF1. We recently experienced two families with NF1 accompanied by pheochromocytoma. The proband of family 1 is a 31-year-old female diagnosed with NF1 and pheochromocytoma. Gene analysis of the proband and her sister showed that the mutation of the NF1 gene (c.7907+1G>A) led to the skipping of exon 53 during NF1 mRNA splicing. The proband of family 2 is a 48-year-old male who was diagnosed with the same condition. Gene analysis demonstrated the mutation of the NF1 gene (c.5206-8C>G) with missplicing of exon 37. These novel germline mutations did not fall into the GAP-related nor the cysteine-serine-rich domains, but into the C-terminal area of the NF1 gene. This suggests that the correlation between the genotype and phenotype of NF1-related pheochromocytoma is somewhat difficult to characterize. Further studies will be necessary to confirm the function of the C-terminal area of the NF1 gene and its contribution to the development of NF1 and pheochromocytoma.
Adult ; DNA ; Exons ; Female ; Genes, Neurofibromatosis 1 ; Genes, Tumor Suppressor ; Genotype ; Germ-Line Mutation ; GTPase-Activating Proteins ; Humans ; Male ; Middle Aged ; Nervous System ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibromin 1 ; Phenotype ; Pheochromocytoma ; RNA, Messenger ; Siblings