Noncirrhotic hyperammonemia as a cause of acute confusion remains diagnostic challenge. Deficiency of ornithine transcarbamylase (OTC) is the urea cycle disorder, inborn errors caused by a defect of the enzymes in the urea cycle, leading to an accumulation of ammonia mainly in newborn. There were very few cases, in which OTC deficiency result in hyperammonemia in adulthood. Herein, we report a young adult woman of hyperammonemic encephalopathy with OTC deficiency, diagnosed by high blood ammonia, glutamine and low plasma levels of citrulline. Next generation sequencing showed the c.386+5G>A mutation of the OTC gene.

Recurrence of Rathke’s cleft cysts (RCC) following surgery is not uncommon. We present a 33-yearold male patient with chronic headache and visual disturbances whose MRI showed mostly cystic, suprasellar mass with peripheral enhancement. Endoscopic extended transsphenoidal approach and tumor resection was performed and RCC was pathologically confirmed postoperatively. Early recurrence was first suspected at 3 months following surgery, and his serial MRIs showed a recurred mass without associated clinical symptoms. Upon further histopathological study, extensive squamous metaplasia and high Ki-67 were seen. Also, in this study, we discuss important factors associated with cyst recurrence following surgery.

Purpose: The benefit of adjuvant chemotherapy following curative-intent surgery in pancreatic ductal adenocarcinoma (PDAC) patients who had received neoadjuvant FOLFIRINOX is unclear. This study aimed to assess the survival benefit of adjuvant chemotherapy in this patient population. Materials and Methods: This retrospective study included 218 patients with localized non-metastatic PDAC who received neoadjuvant FOLFIRINOX and underwent curative-intent surgery (R0 or R1) between January 2017 and December 2020. The association of adjuvant chemotherapy with disease-free survival (DFS) and overall survival (OS) was evaluated in overall patients and in the propensity score matched (PSM) cohort. Subgroup analysis was conducted according to the pathology-proven lymph node status. Results: Adjuvant chemotherapy was administered to 149 patients (68.3%). In the overall cohort, the adjuvant chemotherapy group had significantly improved DFS and OS compared to the observation group (DFS: median, 13.8 months [95% confidence interval (CI), 11.0 to 19.1] vs. 8.2 months [95% CI, 6.5 to 12.0]; p < 0.001; and OS: median, 38.0 months [95% CI, 32.2 to not assessable] vs. 25.7 months [95% CI, 18.3 to not assessable]; p=0.005). In the PSM cohort of 57 matched pairs of patients, DFS and OS were better in the adjuvant chemotherapy group than in the observation group (p < 0.001 and p=0.038, respectively). In the multivariate analysis, adjuvant chemotherapy was a significant favorable prognostic factor (vs. observation; DFS: hazard ratio [HR], 0.51 [95% CI, 0.36 to 0.71; p < 0.001]; OS: HR, 0.45 [95% CI, 0.29 to 0.71; p < 0.001]). Conclusion Among PDAC patients who underwent surgery following neoadjuvant FOLFIRINOX, adjuvant chemotherapy may be associated with improved survival. Randomized studies should be conducted to validate this finding.

Purpose: Although most colorectal malignancies are adenocarcinomas from mucosa, various types of malignant and benign tumors can develop. Due to extremely low incidence, little research has been conducted. The purpose was to assess incidence and compare it according to demographic factors. Methods: Data from the Korea National Cancer Registry from 2007 to 2016 were used. The crude incidence, age-standard incidence rate (ASR) of colorectal nonadenocarcinomas were calculated. Results: Over 11 years, there were 267,142 patients with colorectal malignancies. The patients of 14,495 (5.43%) were diagnosed with nonadenocarcinoma. The ASR was 2.52 per 100,000 in men and 1.56 in women. Lesions were classified according to histologic categories; neuroendocrine tumor (NET) was the most common malignancy (10,919 [75.33%]). Nonadenocarcinoma was the most common in 40s and 50s (40 to 49 years, 3,530 [24.35%]; 50 to 59 years, 3,991 [27.53%]). Lymphoma was high (54.46%) in patients in teenagers. Proportion of NET decreased with age and that of carcinoma increased with age. Carcinoma, sarcoma, and lymphoma were more common among men and melanoma was more common among women. The most common site was the rectum (11,066 [76.34%]). Lymphoma occurred more frequently in proximal colon. Melanoma, gastrointestinal stromal tumor, and NET occurred mostly in rectum. A total of 10,155 patients (70.06%) were classified as having localized disease. Conclusion This study is meaningful as it is the first study to examine incidence of colorectal nonadenocarcinoma. Differences in incidence of different lesions based on demographic factors were identified. This study will play a role in cancer prevention and diagnosis projects.

Mounier-Kuhn syndrome (MKS) is a disease characterized by dilation of the trachea and mainstem bronchi. Due to the risk of airway leakage, pulmonary aspiration, and tracheal damage, MKS can be fatal in patients undergoing tracheal intubation. Moreover, MKS may not be diagnosed preoperatively due to its rarity. In this case, a patient undergoing neurosurgery was incidentally diagnosed with MKS during general anesthesia. During anesthesia induction, difficulties in airway management led the anesthesiologist to suspect MKS. Airway leakage was resolved in this case using oropharyngeal gauze packing. Anesthesiologists should be aware of the possibility of MKS and appropriate management of the airways.
Airway Management ; Anesthesia ; Anesthesia, General ; Bronchi ; Humans ; Intubation ; Intubation, Intratracheal ; Neurosurgery ; Trachea ; Tracheobronchomegaly

BACKGROUND: Numerous studies have shown that healthcare professionals are exposed to psychological distress. However, since most of these studies assessed psychological distress using self-reporting questionnaires, the magnitude of the problem is largely unknown. We evaluated the risks of mood disorders, anxiety disorders, sleep disorders, and any psychiatric disorders in workers in healthcare industry using Korea National Health Insurance (NHI) claims data from 2014, which are based on actual diagnoses instead of self-evaluation. METHODS: We used Korea 2014 NHI claims data and classified employees as workers in the healthcare industry, based on companies in the NHI database that were registered with hospitals, clinics, public healthcare, and other medical services. To estimate the standardized prevalence of the selected mental health disorders, we calculated the prevalence of diseases in each age group and sex using the age distribution of the Korea population. To compare the risk of selected mental disorders among workers in the healthcare industry with those in other industries, we considered age, sex, and income quartile characteristics and conducted propensity scored matching. RESULTS: In the matching study, workers in healthcare industry had higher odds ratios for mood disorders (1.13, 95% CI: 1.11–1.15), anxiety disorders (1.15, 95% CI: 1.13–1.17), sleep disorders (2.21, 95% CI: 2.18–2.24), and any psychiatric disorders (1.44, 95% CI: 1.43–1.46) than the reference group did. Among workers in healthcare industry, females had higher prevalence of psychiatric disorders than males, but the odds ratios for psychiatric disorders, compared to the reference group, were higher in male workers in healthcare industry than in females. CONCLUSIONS: The prevalence of mood disorders, anxiety disorders, sleep disorders, and all psychiatric disorders for workers in the healthcare industry was higher than that of other Korean workers. The strikingly high prevalence of sleep disorders could be related to the frequent night-shifts in these professions. The high prevalence of mental health problems among workers in healthcare industry is alarming and requires prompt action to protect the health of the “protectors.”
Age Distribution ; Anxiety Disorders ; Delivery of Health Care ; Diagnosis ; Diagnostic Self Evaluation ; Female ; Health Care Sector ; Humans ; Korea ; Male ; Mental Disorders ; Mental Health ; Mood Disorders ; National Health Programs ; Odds Ratio ; Prevalence ; Sleep Wake Disorders

Eosinophilic myenteric ganglionitis is a disorder characterized by infiltration of the Auerbach myenteric plexus by eosinophils. As a cause of chronic intestinal pseudo-obstruction (CIPO), eosinophilic myenteric ganglionitis has been rarely reported and the majority of the reported cases in the literature were children. We experienced a case of eosinophilic myenteric ganglionitis associated with CIPO in a 53-year-old female patient. Histologic examination of the resected descending colon showed moderate eosinophilic infiltrates with hypogangliosis in the myenteric plexus. Immunohistochemical study revealed increased number of CD4-positive lymphocytes and stronger but scantier glial fibillary acid protein expression in the inflamed myenteric plexus.
CD4-Positive T-Lymphocytes ; Child ; Colon, Descending ; Eosinophils* ; Female ; Ganglion Cysts* ; Humans ; Intestinal Pseudo-Obstruction ; Middle Aged ; Myenteric Plexus

BACKGROUND: With the development of technology, extensive use of computers in the workplace is prevalent and increases efficiency. However, computer users are facing new harmful working conditions with high workloads and longer hours. This study aimed to investigate the association between computer use at work and self-reported depressive and anxiety disorder (DAD) in a nationally representative sample of South Korean workers. METHODS: This cross-sectional study was based on the third Korean Working Conditions Survey (2011), and 48,850 workers were analyzed. Information about computer use and DAD was obtained from a self-administered questionnaire. We investigated the relation between computer use at work and DAD using logistic regression. RESULTS: The 12-month prevalence of DAD in computer-using workers was 1.46 %. After adjustment for socio-demographic factors, the odds ratio for DAD was higher in workers using computers more than 75 % of their workday (OR 1.69, 95 % CI 1.30−2.20) than in workers using computers less than 50 % of their shift. After stratifying by working hours, computer use for over 75 % of the work time was significantly associated with increased odds of DAD in 20–39, 41–50, 51–60, and over 60 working hours per week. After stratifying by occupation, education, and job status, computer use for more than 75 % of the work time was related with higher odds of DAD in sales and service workers, those with high school and college education, and those who were self-employed and employers. CONCLUSIONS: A high proportion of computer use at work may be associated with depressive and anxiety disorder. This finding suggests the necessity of a work guideline to help the workers suffering from high computer use at work. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s40557-016-0146-8) contains supplementary material, which is available to authorized users.
Anxiety Disorders* ; Anxiety* ; Commerce ; Cross-Sectional Studies ; Depressive Disorder ; Education ; Humans ; Logistic Models ; Occupations ; Odds Ratio ; Prevalence

Actinomycosis is a chronic, slowly progressive, and suppurative disease caused by filamentous anaerobic bacteria Actinomyces, which results in characteristic sulfur granules. Clinically, actinomycosis can present with a mass-like lesion, and this bacterial nidus has been frequently mistaken for a malignancy. For that reason many patients undergo surgical resection before the correct diagnosis is established. We report a case of a 63-year-old man with a solitary, asymptomatic pancreatic actinomycosis that masqueraded as pancreatic cancer. He did not have any other concurrently infected organs and did not have any signs or symptoms of infection. All radiologic images of the patient favored a malignancy to a great extent rather than an inflammatory mass. He was finally diagnosed with actinomycosis by endoscopic ultrasound (EUS)-guided fine needle aspiration biopsy without surgery. After one month of treatment with antibiotics, the pancreatic head mass was completely resolved on the follow-up computed tomography (CT).
Actinomyces ; Actinomycosis* ; Anti-Bacterial Agents ; Bacteria, Anaerobic ; Biopsy ; Biopsy, Fine-Needle ; Diagnosis ; Follow-Up Studies ; Head ; Humans ; Middle Aged ; Pancreas ; Pancreatic Neoplasms* ; Sulfur ; Ultrasonography

BACKGROUND: There is a subjective disagreement about nuclear chromatin in the field of pathology. Objective values of red, green, and blue (RGB) light intensities for nuclear chromatin can be obtained through a quantitative analysis using digital images. METHODS: We examined 10 cases of well differentiated neuroendocrine tumors of the rectum, small cell lung carcinomas, and moderately differentiated squamous cell lung carcinomas respectively. For each case, we selected 30 representative cells and captured typical microscopic findings. Using an image analyzer, we determined the longest nuclear line profiles and obtained graph files and Excel data on RGB light intensities. We assessed the meaningful differences in graph files and Excel data among the three different tumors. RESULTS: The nucleus of hematoxylin and eosin-stained tumor cells was expressed as a combination of RGB light sources. The highest intensity was from blue, whereas the lowest intensity was from green. According to the graph files, green showed the most noticeable change in the light intensity, which is consistent with the difference in standard deviations. CONCLUSIONS: The change in the light intensity for green has an important implication for differentiating between tumors. Specific features of the nucleus can be expressed in specific values of RGB light intensities.
Chromatin ; Hematoxylin ; Image Processing, Computer-Assisted ; Light ; Lung ; Neuroendocrine Tumors ; Rectum ; Small Cell Lung Carcinoma