Colonic interposition is the main procedure used in esophageal reconstruction. We report a rare case of simultaneous treatment of an anastomotic site stricture and a neoplasm in the interpositioned colon. A 69-year-old female visited our outpatient clinic with symptoms of progressive dysphagia for 1 year. At the age of 30 years, the patient underwent esophagectomy with retrosternal colonic interposition because of severe esophageal burns after chemical ingestion. Upper gastrointestinal endoscopy revealed stricture at the anastomosis site and a 10-mm flat elevated high-grade dysplasia in the interpositioned colon. First, through-the-scope balloon dilatation was performed for strictures. However, stenosis was observed during the second upper gastrointestinal endoscopy session.Therefore, a second session of through-the-scope balloon dilatation was performed, and simultaneously, endoscopic submucosal dissection was also successfully performed. After 2 months of follow-up, stenosis persisted; consequently, balloon dilatation was performed. No recurrence of neoplasm was confirmed endoscopically. Through-the-scope balloon dilatation of the stricture site and simultaneous endoscopic submucosal dissection of the neoplasm in the interpositioned colon were successfully performed.

Purpose: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital anomaly that can cause frequent digestive and nutritional problems, even after repair. The most common complication is anastomotic stricture, for which reoperation or balloon dilatation is performed. This study aimed to evaluate the postoperative complications of EA and the role of endoscopic balloon dilatation (EBD) in cases of anastomotic stricture. Methods: We retrospectively analyzed patients diagnosed with EA with or without TEF between January 2000 and February 2021. Patients’ baseline characteristics, associated anomalies, and postoperative complications were reviewed. Results: Among 26 patients, 14 (53.8%) were male, 12 (46.2%) had coexisting anomalies, and the median follow-up was 6.1 years (range, 1.2–15.7 years). In univariate analysis, prematurity, low birth weight, and long-gap EA were associated with postoperative complications in 12 (46.2%) patients. Among the 10 (38.5%) patients with anastomotic stricture, nine (90.0%) required EBD. Regarding the first EBD, it was performed at a median of 3.3 months (range, 1.2–7.6 months) post-repair, while the average patient weight was 4.6 kg. The mean diameter ranged from 3.3 to 9.1 mm without major complications. In univariate analysis, long-gap EA alone was significantly associated with EBD. Conclusion Approximately half of the patients experienced complications after EA repair.In particular, patients with a long-gap EA had a significantly increased risk of complications, such as anastomotic strictures. EBD can be safely used, even in infants.

Diagnostic techniques for spinal pathologies have been developed in accordance with advances in technology. Accurate diagnosis of spinal pathology is essential for appropriate management of spinal diseases. Since the development of X-rays in 1895 and computed tomography (CT) in 1967, several diagnostic imaging modalities have been utilized for detecting spinal pathologies, including radiography, CT, magnetic resonance imaging, and radionuclide imaging. In addition to diagnostic imaging technologies, electrodiagnostic tests, including electromyography and nerve conduction studies, play a significant role as diagnostic tools, as spinal diseases are mostly profoundly associated with pathologies of the neural structures, such as the spinal cord and nerve root, and extent of injury at the structure cannot be adequately detected by conventional imaging techniques. In patient-specific treatment strategies, usage of diagnostic modalities is of great importance; thus, we should be aware of the basic details and approaches of the different diagnostic modalities. In this review, the authors discuss the details of the technologies that aid in the diagnosis of spinal pathologies.

Purpose: Identification of type I protein arginine methyltransferase (PRMT) substrates and their functional significance during tumorigenesis is becoming more important. The present study aimed to identify target substrates for type I PRMT using 2-dimensional (2D) gel electrophoresis (GE) and 2D Western blotting (WB). Methods: Using immunoblot analysis, we compared the expression of type I PRMTs and endogenous levels of arginine methylation between the primary colorectal cancer (CRC) and adjacent noncancerous tissues paired from the same patient. To identify arginine-methylated proteins in HCT116 cells, we carried out 2D-GE and 2D-WB with a type I PRMT product-specific antibody (anti-dimethyl-arginine antibody, asymmetric [ASYM24]). Arginine-methylated protein spots were identified by mass spectrometry, and messenger RNA (mRNA) levels corresponding to the identified proteins were analyzed using National Center for Biotechnology Information (NCBI) microarray datasets between the primary CRC and noncancerous tissues. Results: Type I PRMTs and methylarginine-containing proteins were highly maintained in CRC tissues compared to noncancerous tissues. We matched 142 spots using spot analysis software between a Coomassie blue (CBB)-stained 2D gel and 2D-WB, and we successfully identified 7 proteins that reacted with the ASYM24 antibody: CACYBP, GLOD4, MAPRE1, CCT7, TKT, CK8, and HSPA8. Among these proteins, the levels of 4 mRNAs including MAPRE1, CCT7, TKT, and HSPA8 in CRC tissues showed a statistically significant increase compared to noncancerous tissues from patients using the NCBI microarray datasets. Conclusion Our results indicate that the method shown here is useful in identifying arginine-methylated proteins, and significance of arginine modification in the proteins identified here should be further identified during CRC development.

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive urea cycle disorder. HHH is caused by a deficiency of the mitochondrial ornithine transporter protein, which is encoded by the solute carrier family 25, member 15 (SLC25A15) gene. Recently, government supported Korean newborn screening has been expanded to include a tandem mass spectrometry (MS/MS) measurement of ornithine level. We report a case of a neonate with HHH syndrome showing a normal MS/MS measurement of ornithine level. A female newborn was admitted to neonatal intensive unit due to familial history of HHH syndrome. Her parents were consanguineous Parkistani couple. The subject's older sister was diagnosed with HHH syndrome at age of 30 months based on altered mental status and liver dysfunction. Even though the subject displayed normal ammonia and ornithine levels based on MS/MS analysis, a molecular test confirmed the diagnosis of HHH syndrome. At 1 month of age, amino acid analysis of blood and urine showed high levels of ornithine and homocitrulline. After 11 months of follow up, she showed normal growth and development, whereas affected sister showed progressive cognitive impairment despite no further hyperammonemia after protein restriction and standard therapy. Our report is in agreement with a previous Canadian study, which showed that neonatal samples from HHH syndrome patients demonstrate normal ornithine levels despite having known mutations. Considering the delayed rise of ornithine in affected patients, genetic testing, and repetitive metabolic testing is needed to prevent patient loss in high risk patients.

PURPOSE: The aim of this study was to investigate the clinical features and extraintestinal manifestations (EIMs) of Crohn disease (CD) in Korean pediatric patients. METHODS: The medical records of 73 children diagnosed with CD were retrospectively reviewed. Data regarding baseline demographic and clinical characteristics, including CD phenotype at diagnosis based on the Montreal classification, and clinical features and course of EIMs were investigated. RESULTS: Fifty-two (71.2%) of the patients were males. The mean age of the patients was 12.5 years. The mean follow-up period was 3.4 years. The disease location was ileal in 3 (4.1%) of the patients, colonic in 13 (17.8%), ileocolonic in 56 (76.7%). The clinical behavior was inflammatory in 62 (84.9%) of the patients, stricturing in 8 (11.0%), and penetrating in 3 (4.1%). Perianal abscesses or fistulas were found in 37 (50.7%) of the patients. EIMs observed during the study period were anal skin tag in 25 patients (34.2%), hypertransaminasemia in 20 (27.4%), peripheral arthritis in 2 (2.7%), erythema nodosum in 2 (2.7%), vulvitis in 1 (1.4%), uveitis in 1 (1.4%), and pulmonary thromboembolism in 1 (1.4%). CONCLUSION: Perianal diseases and manifestations were present in more than half of Korean pediatric CD patients at diagnosis. Inspection of the anus should be mandatory in Korean children with suspicious CD, as perianal fistulas, abscesses, and anal skin tags may be the first clue to the diagnosis of CD.
Abscess ; Anal Canal ; Arthritis ; Child* ; Classification ; Colon ; Crohn Disease* ; Diagnosis ; Erythema Nodosum ; Fistula ; Follow-Up Studies ; Humans ; Male ; Medical Records ; Phenotype ; Pulmonary Embolism ; Retrospective Studies ; Skin ; Uveitis ; Vulvitis

A Middle East respiratory syndrome (MERS) - coronavirus (CoV) cluster that attacked Korea in May 2015 revealed several weaknesses in Korea's health care system in the face of the crisis of an emerging infectious disease and its public health implications. This experience has shown that is necessary to prepare comprehensive countermeasures through the cooperation of civil and public agencies to prevent a second or even third MERS outbreak and to control future crises of infectious disease and public health. The MERS Policy Committee of the Korean Medical Association has thus proposed a master plan for reform of the national infectious disease prevention and management system to prepare a new framework for national infectious disease prevention and control. The specific targets of the master plan are improvement of the system of usage of medical services and of the medical culture among national health insurance subscribers, improvement of the emergency room management system to prevent the spread of infectious disease, the establishment of a support system to promote effective voluntary infectious disease prevention activities among medical institutions, the building of a public health crisis communication system in collaboration with medical organizations, the establishment of an independent Ministry of Health and capacity building of the Korea Centers for Disease Control (KCDC), the securing of an advanced research and development system in the field of prevention and control of infectious disease, and the capacity building of professional epidemiologists and personnel needed to prevent and control infectious disease. The five core strategies of the master plan have been planned by medical experts in this order of priority: the reform of the healthcare delivery system, the independence of the Ministry of Health and capacity building of the KCDC, fundraising for a public infection management fund, enforcement of medical organization infection control, and improvement of the emergency room management system.
Capacity Building ; Centers for Disease Control and Prevention (U.S.) ; Communicable Diseases* ; Communicable Diseases, Emerging ; Cooperative Behavior ; Coronavirus ; Coronavirus Infections ; Delivery of Health Care ; Emergency Service, Hospital ; Financial Management ; Infection Control ; Korea* ; Middle East ; National Health Programs ; Public Health

Sodium nitrite is commercially used as a coloring agent, food preservative, and corrosion inhibitor. Accidental poisoning with sodium nitrite from contaminated food and water causes gastrointestinal irritation, vasodilatation, and methemoglobinemia with subsequent tissue hypoxia. We describe an outbreak case of sodium nitrite-induced methemoglobinemia following the ingestion of noodles contaminated with industrial antifreeze. The eEight patients involved initially complained that their noodles tasted 'unpleasant' and soon afterwards experienced nausea, vomiting, dizziness, and fatigue. Some of them noted cyanosis on their lips and fingers. Subsequent investigations demonstrated a high methemoglobin concentration which was corrected by the intravenous administration of methylene blue three hours after the onset of symptoms. The patients made a prompt, uncomplicated recovery and were discharged home 4 four days later. Industrial antifreeze contains sodium nitrite and calcium nitrite. Because an accidental poisoning of industrial antifreeze causes fatal methemoglobinemia, emergency physicians should promptly identify its symptoms and institute treatment with methylene blue promptly. In addition, industrial agencies must caution construction businesses against such contamination events.
Administration, Intravenous ; Anoxia ; Calcium ; Commerce ; Corrosion ; Cyanosis ; Dizziness ; Eating ; Emergencies ; Fatigue ; Fingers ; Humans ; Lip ; Methemoglobin ; Methemoglobinemia ; Methylene Blue ; Nausea ; Sodium ; Sodium Nitrite ; Vasodilation ; Vomiting

Vibrio vulnificus causes rapid progressing fulminant infections in susceptible individuals, especially those with elevated serum iron levels. This ferrophilic bacterium can directly acquire iron from heme-containing proteins, such as, hemoglobin, via its heme receptor protein HupA. This study was undertaken to determine the roles of cyclic AMP-receptor protein (Crp) as an activator and of ferric uptake regulator (Fur) as a repressor in regulating hupA expression at various iron and glucose concentrations. Under severely iron-deficient conditions, hupA expression in the absence of Crp was induced albeit at low levels and repressed by the addition of iron. In contrast, hupA expression in the presence of Crp was increased by the addition of iron. Under moderately iron-deficient and iron-sufficient conditions, iron addition repressed hupA expression in the presence of Fur, but not in the absence of Fur. Glucose addition repressed hupA expression in the presence of Fur but not in the absence of Fur. Furthermore, a mutation in cyaA encoding adenylate cyclase required for cAMP synthesis hupA expression, and this repression was prevented by the exogenous addition of cAMP. These results indicate that hupA expression is under the coordinate control of cAMP or Crp, which responds to glucose availability, and of Fur, which responds to iron availability, and that Crp is not essential for the constitutional expression of hupA, but is required for the optimal expression of hupA, whereas Fur is essential for the prevention of hupA over-expression.
Adenylyl Cyclases ; Glucose ; Heme ; Hemoglobins ; Iron ; Proteins ; Receptors, Cell Surface ; Repression, Psychology ; Vibrio ; Vibrio vulnificus