A case of esophagitis and mediastinitis developed in an infant with an esophageal duplication cyst

An esophageal duplication cyst (EDC) is a congenital anomaly of the foregut. EDCs are often asymptomatic, so they are found incidentally on chest radiographs. In symptomatic patients, EDCs present with cough, dyspnea, vomiting, dysphagia, and chest pain. Potential complications of EDCs include perforation, obstruction, bleeding, and infection. The rarity of this condition may hinder timely diagnosis, which may lead to life-threatening complications. This article describes a case of esophagitis and mediastinitis developed in a 12-month-old infant with an EDC. We emphasize that although rare, EDCs should be considered in the differential diagnosis of non-specific respiratory signs such as cough, dyspnea, and stridor.

A case of ROHHAD (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) syndrome in an 11-year-old girl

Rapid-onset obesity with hypoventilation, hypothalamic, and autonomic dysregulation (ROHHAD) syndrome is a rare disease characterized by rapid progression of obesity and central hypoventilation with autonomic and endocrine dysregulation. There is no gold-standard diagnostic method for ROHHAD syndrome; it is diagnosed based on a years-long clinical course. For this reason, diagnosis of ROHHAD syndrome is often delayed. In particular, ROHHAD has a high mortality rate due to cardiopulmonary arrest when quick diagnosis and appropriate intervention of central sleep apnea are not timely. We report a case in which an 11-year-old girl with central sleep apnea was diagnosed with ROHHAD syndrome: the clinical course with early breathing intervention using noninvasive positive pressure ventilation. We emphasize the importance of respiratory interventions in the clinical course of ROHHAD syndrome.

Association between Promoter Polymorphisms of TFF1, TFF2, and TFF3 and the Risk of Gastric and Diffuse Gastric Cancers in a Korean Population

Gastric cancer is one of the most common cancers in the world. The aims of this study were to evaluate the association between polymorphisms in TFF gene family, TFF1, TFF2, and TFF3 and the risk of gastric cancer (GC) and GC subgroups in a Korean population via a case-control study. The eight polymorphisms in TFF gene family were identified by sequencing and genotyped with 377 GC patients and 396 controls by using TaqMan genotyping assay. The rs184432 TT genotype of TFF1 was significantly associated with a reduced risk of GC (odds ratio, [OR) = 0.45; 95% confidence interval, [CI] = 0.25-0.82; P = 0.009), more protective against diffuse-type GC (OR = 0.20; 95% CI = 0.05-0.89; P = 0.035) than GC (OR = 0.34; 95% CI = 0.14-0.82; P = 0.017) in subjects aged < 60 yr, and correlated with lymph node metastasis negative GC and diffuse-type GC (OR = 0.44; 95% CI = 0.23-0.86; P = 0.016 and OR = 0.20; 95% CI = 0.05-0.87; P = 0.031, respectively). In addition, a decreased risk of lymph node metastasis negative GC and diffuse-type GC was observed for rs225359 TT genotype of TFF1 (OR = 0.46, 95% CI = 0.24-0.88; P = 0.020 and OR = 0.21, 95% CI = 0.05-0.88; P = 0.033, respectively). These findings suggest that the rs184432 and rs225359 polymorphisms in TFF1 have protective effects for GC and contribute to the development of GC in Korean individuals.

Validation Study of a Dietary Questionnaire for Assessing Exposure to Food-Borne Hazards

Assessing human exposure to food-borne hazards requires standardized assessment tools. The objective of this study was to validate a newly developed dietary assessment questionnaire to assess human exposure to food-borne hazards, which include dietary behavior and food consumption patterns such as eating frequency, types of food containers and cooking methods. A total of 216 adults were recruited for two questionnaire surveys (questionnaire 1 and 2) about 1 week apart with a 3 day diet record. Reproducibility was evaluated by comparing responses from questionnaires 1 and 2, and validity was checked by comparing responses from questionnaire 2 and the 3 day diet record. Comparisons were based on the percent agreement and Spearman's rank correlation coefficient. The mean exact agreement of food containers at purchase between questionnaires 1 and 2 was 73.5%, for storing containers it was 71.9%, and for cooking methods it was 83.0%. The mean correlation coefficient for food intake frequency between questionnaires 1 and 2 was 0.71 (range, 0.50?0.83). The mean correlation coefficient of the food intake frequency between questionnaire 2 and the 3 day diet record was 0.21 (range, 0.04-0.48). The exact and adjacent agreement of food intake frequency quartile assessed by questionnaire 2 and the 3 day diet record was 65.4% (range, 51.0-82.1%). Although the correlation coefficient for food intake frequency between questionnaire 2 and the 3 day diet record was low, the exact and adjacent food intake frequency agreement was higher than 50% and reproducibility of the dietary behaviors exceeded 70%. Therefore, the questionnaire developed in this study could be applied to assess diets for the human exposure to food-borne hazards as a qualitative assessment in a large population.

Developing a Questionnaire to Assess Exposure to Food-Borne Hazards

The aim of this study was to develop a reliable dietary questionnaire to assess human exposure to food-borne hazards. Eleven food-borne hazards were chosen as a priority control list through a literature review and advisory committees. The 11 food-borne hazards were phthalate, aflatoxin, bisphenol A, polycyclic aromatic hydrocarbons, dioxin, polychlorinated biphenyls, mercury, lead, cadmium, arsenic, and acrylamide. The characteristics, exposure level, and paths of these hazards were reviewed, and questionnaire items were identified to assess human exposure from the literature. A questionnaire was developed for each selected food based on its characteristics. Based on the items in the individual questionnaires, a comprehensive questionnaire, which contained demographic characteristics, job information, socioeconomic factors, health related lifestyles, and dietary behaviors, was developed. A 99-item food frequency questionnaire (FFQ) to assess food-borne hazard exposure was also developed. The FFQ included frequency of food intake during the previous year, container type for purchasing and storing food, and cooking method. The questionnaire developed in this study could be applied to assess dietary factors during an exposure assessment of food-borne hazards in a large population. A validation study for the questionnaire is needed before applying it to surveys.

Disruption of Microtubules Sensitizes the DNA Damage-induced Apoptosis Through Inhibiting Nuclear Factor kappaB (NF-kappaB) DNA-binding Activity

The massive reorganization of microtubule network involves in transcriptional regulation of several genes by controlling transcriptional factor, nuclear factor-kappa B (NF-kappaB) activity. The exact molecular mechanism by which microtubule rearrangement leads to NF-kappaB activation largely remains to be identified. However microtubule disrupting agents may possibly act in synergy or antagonism against apoptotic cell death in response to conventional chemotherapy targeting DNA damage such as adriamycin or comptothecin in cancer cells. Interestingly pretreatment of microtubule disrupting agents (colchicine, vinblastine and nocodazole) was observed to lead to paradoxical suppression of DNA damage-induced NF-kappaB binding activity, even though these could enhance NF-kappaB signaling in the absence of other stimuli. Moreover this suppressed NF-kappaB binding activity subsequently resulted in synergic apoptotic response, as evident by the combination with Adr and low doses of microtubule disrupting agents was able to potentiate the cytotoxic action through caspase-dependent pathway. Taken together, these results suggested that inhibition of microtubule network chemosensitizes the cancer cells to die by apoptosis through suppressing NF-kappaB DNA binding activity. Therefore, our study provided a possible anti-cancer mechanism of microtubule disrupting agent to overcome resistance against to chemotherapy such as DNA damaging agent.

Long-term Activation of c-Jun N-terminal Kinase through Receptor Interacting Protein is Associated with DNA Damage-induced Cell Death

Activation of c-Jun N-terminal kinase (JNK), a member of the mitogen-activated protein kinase family, is an important cellular response that modulates the outcome of the cells which are exposed to the tumor necrosis factor (TNF) or the genotoxic stress including DNA damaging agents. Although it is known that JNK is activated in response to genotoxic stress, neither the pathways to transduce signals to activate JNK nor the primary sensors of the cells that trigger the stress response have been identified. Here, we report that the receptor interacting protein (RIP), a key adaptor protein of TNF signaling, was required to activate JNK in the cells treated with certain DNA damaging agents such as adriamycin (Adr) and 1-beta-D-arabinofuranosylcytosine (Ara-C) that cause slow and sustained activation, but it was not required when treated with N-methyl-N-nitro-N-nitrosoguanidine (MNNG) and short wavelength UV, which causes quick and transient activation. Our findings revealed that this sustained JNK activation was not mediated by the TNF (tumor necrosis factor) receptor signaling, but it required a functional ATM (ataxia telangiectasia) activity. In addition, JNK inhibitor SP-600125 significantly blocked the Adr-induced cell death, but it did not affect the cell death induced by MNNG. These findings suggest that the sustained activation of JNK mediated by RIP plays an important role in the DNA damage-induced cell death, and that the duration of JNK activation relays a different stress response to determine the cell fate.