Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.
Hand ; Heart Septal Defects, Ventricular ; Hemoptysis ; Humans ; Korea ; Lung ; Male ; Pulmonary Artery ; Sputum ; Stress, Psychological ; Tetralogy of Fallot ; Tolnaftate

BACKGROUND: To determine the molecular structure of type II collagen-specific T-cell receptors associated with rheumatoid arthritis (RA). METHODS: We generated CII-specific T-cell lines of 8 RA patient s by prolonged in vitro culture with bovine CII (bCII) and the immunogenic peptide (256-270) of human CII. The proliferation response towards CII stimulation was measured from the uptake of 3 H-thymidine. Changes in the secretion of Th 1 and Th2 cytokines in the culture supernatent were measured by ELISA. The TCR clonotypes of these T-cells were examined by RT-PCR/ SSCP analyses of all 22 V beta chains. RESULTS: T-cells from patients' tissue exhibited strong proliferation index upon CII stimulation, which was maintained up to 6 months in the culture. The secretion of INF-gamma from these T-cells increased along with the duration of culture time, while the amount of IL-4 production did not show significant changes. The SSCP band patterns of patients' T-cells appear as discrete bands unlike the smeary streak produced from normal samples. Some SSCP bands, each representing selected expansion of a TCR containing certain subtype of V beta peptides, appeared to be identical in more than one patients. Among these, the expansion of SSCP band representing the V beta 14 CDR3 region persisted after switching the antigen to the immunogenic human peptide (256-270). CONCLUSION: CII-reactive T-cells expressing distinct CDR3 motifs are selectively expanded in the peripheral blood and synovial fluid of RA patients, and their persistent proliferation upon CII stimulation, as well as the production Th 1-type cytokines, may play pivotal roles in RA pathogenesis.
Arthritis, Rheumatoid* ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Humans ; Interleukin-4 ; Molecular Structure ; Peptides ; Polymorphism, Single-Stranded Conformational ; Receptors, Antigen, T-Cell ; Synovial Fluid ; T-Lymphocytes*

Endoscopic resection is currently accepted as a standard therapy for colon polyp because of its safety and efficiency. The indications for endoscopic resection have been expanded to treat mucosal colon cancer and submucosal tumor. The major complications of endoscopic resection are hemorrhage, perforation and post- polypectomy coagulation syndrome. Hemorrhage is the most common complication, and this can occur immediately following colonoscopic polypectomy or it can be delayed after completion of the procedure. Delayed hemorrhage usually occurs within 7 days and this can stop by itself or the hemorrhage can be controlled endoscopically in the majority of patients, with only the unusual and serious cases requiring transfusion, angiography and surgery. We experienced a case of delayed massive hemorrhage with hypotension that required transfusion 12 days after performing endoscopic resection for rectal carcinoid tumor. We report here on this case to provide a good example and to place emphasis on delayed massive hemorrhage after endoscopic resection.
Angiography ; Carcinoid Tumor ; Colon ; Colonic Neoplasms ; Hemorrhage ; Humans ; Hypogonadism ; Hypotension ; Mitochondrial Diseases ; Ophthalmoplegia ; Polyps

BACKGROUND: Central venous cannulation is one of the important parts of anesthetic management. Because general anesthesia causes external-landmarks (e.g. sternocleidomastoid muscle, carotid artery pulse) indistinct, we developed a new external landmark for internal jugular vein cannulation that can be easily detected under general anesthesia. METHODS: We cannulated the right internal jugular vein of 110 patients under general anesthesia. We used the external jugular vein and cricoid cartilage as a landmark. RESULTS: Cannulation of the right internal jugular vein was successful in 108 out of 110 patients (98.2%). Patients required an average 1.48+/-0.63 attempts. There were 2 carotid punctures (1.8%), 3 discomforts (2.7%) and no brachial plexus irritation. CONCLUSIONS: This new landmark technique has a high success rate and few complications.
Anesthesia, General ; Brachial Plexus ; Carotid Arteries ; Catheterization* ; Central Venous Catheters ; Cricoid Cartilage ; Humans ; Jugular Veins* ; Punctures

A 13-year-old girl presented with multiple skin abscesses. She was diagnosed as having juvenile dermatomyositis (DM) at the age of 7 years. She had suffered from recurrent skin infections, atypical pruritic dermatitis and pneumonia since the age of 8 years. Bacteriologic and fungal cultures for skin abscesses and oral mucosa were positive S. aureus and C. albicans, respectively. Chemotactic defect in peripheral blood neutrophils was observed. The level of serum IgE was markedly elevated, and anti-S.aureus specific IgE was found. A diagnosis of hyperimmunoglobulin E-recurrent infection syndrome (HIE) was made and she was successfully treated with surgical drainage and antibiotics. To our knowledge, this is the first case report of HIE in a patient with juvenile dermatomyositis.
Adolescence ; Case Report ; Dermatomyositis/complications* ; Female ; Human ; IgE/blood ; Job's Syndrome/immunology ; Job's Syndrome/diagnosis ; Job's Syndrome/complications* ; Staphylococcal Infections/immunology ; Staphylococcal Infections/complications ; Staphylococcus aureus/immunology

Bronchopulmonary infections caused by trichomonads have been reported principally in patients with pre-existing pulmonary diseases, such as bronchial carcinoma, lung abscess, or bronchiectasis. Pulmonary trichomoniasis is most often caused by Trichomonas tenax, which is usually regarded as a harmless commensal organism of the human mouth. However, pulmonary infection may rarely be caused by other trichomonas species, including Trichomonas vaginalis from the genitourinary tract and Trichomonas hominis from the intestines. Because of the rarity of trichomonas pulmonary infection, and because of its close association with underlying lung and systemic disease, pulmonary trichomoniasis is considered an opportunistic infection. We recently treated a case of pulmonary trichomoniasis occurring in a young, healthy male without obvious underlying pulmonary or systemic illness. To our knowledge, there has been only one reported case of pulmonary trichomoniasis in Korea, and there have been only two reported cases of pulmonary trichomoniasis occurring in normal lung worldwide.
Bronchiectasis ; Carcinoma, Bronchogenic ; Humans ; Intestines ; Korea ; Lung ; Lung Abscess ; Lung Diseases ; Male ; Mouth ; Opportunistic Infections ; Polymers ; Trichomonas ; Trichomonas vaginalis

Purpose: The purpose of this study was to investigate the concordance rate of PIK3CA mutations between primary and matched distant metastatic sites in patients with breast cancer and to verify whether there are differences in the frequency of PIK3CA hotspot mutations depending on the metastatic sites involved. Materials and Methods: Archived formalin-fixed paraffin-embedded (FFPE) specimens of primary breast and matched distant metastatic tumors were retrospectively obtained for 49 patients. Additionally, 40 archived FFPE specimens were independently collected from different breast cancer metastatic sites, which were limited to three common sites: the liver, brain, and lung. PIK3CA mutations were analyzed using droplet digital PCR, including hotspots involving exons 9 and 20. Results: After analysis of 49 breast tumors with matched metastasis sites, 87.8% showed concordance in PIK3CA mutation status. According to PIK3CA hotspot mutation testing in 89 cases of breast cancer metastatic sites, the proportion of PIK3CA mutations at sites of metastasis involving the liver, brain, and lung was 37.5%, 28.6%, and 42.9%, respectively, which did not result in statistical significance. Conclusion The high concordance of PIK3CA mutation status between primary and matched metastasis sites suggests that metastatic sites, regardless of the metastatic organ, could be considered sample sources for PIK3CA mutation testing for improved therapeutic strategies in patients with metastatic breast cancer.

The central nervous system (CNS) has important roles in the regulation of sodium and water homeostasis, so the risk of hyponatremia is increased for patients with neurological disorders. Cerebral salt wasting (CSW) is a syndrome that is characterized by hyponatremia and extracellular volume contraction, and these problems are caused by natriuresis and the concomitant polyuria in patient with intracranial disease. It is critical to differentiate CSW from syndrome of inappropriate secretion of antidiuretic hormone (SIADH) for patients with hyponatremia and after they experience cerebral insults. This is because fluid restriction is the treatment of choice for SIADH and administration of isotonic or hypertonic saline is important for treating CSW. We report here on a case of CSW following trassphenoidal surgery for pituitary tumor in a 67-year-old man with nonfunctioning pituitary macroadenoma. Hyposmotic hyponatremia, increased urine output and signs of dehydration developed postoperatively, and the patient was managed by water and salt supplementation.
Aged ; Central Nervous System ; Dehydration ; Homeostasis ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Natriuresis ; Nervous System Diseases ; Pituitary Neoplasms ; Polyuria ; Sodium

Mucosal bridge, endoscopically observed, is a cord-like mucosal connection across the lumen. The bridge is very elastic and stretches easily, unlike granulation tissue. Mucosal bridges of the esophagus have been occasionally described in various circumstances, particularly in congenital or acquired origin as the inflammatory diseases. The occurrence of mucosal bridges due to inflammatory process may arise anywhere from the esophagus to the colon. It has been more frequently reported in the colon than in the esophagus, stomach, and duodenum. We experienced four cases of esophageal mucosal bridges and three of them were accompanied by esophageal diverticulum. We report these cases with a review of relevant literatures.
Colon ; Diverticulum ; Diverticulum, Esophageal ; Duodenum ; Esophagus* ; Granulation Tissue ; Stomach

Mucosal bridge, endoscopically observed, is a cord-like mucosal connection across the lumen. The bridge is very elastic and stretches easily, unlike granulation tissue. Mucosal bridges of the esophagus have been occasionally described in various circumstances, particularly in congenital or acquired origin as the inflammatory diseases. The occurrence of mucosal bridges due to inflammatory process may arise anywhere from the esophagus to the colon. It has been more frequently reported in the colon than in the esophagus, stomach, and duodenum. We experienced four cases of esophageal mucosal bridges and three of them were accompanied by esophageal diverticulum. We report these cases with a review of relevant literatures.
Colon ; Diverticulum ; Diverticulum, Esophageal ; Duodenum ; Esophagus* ; Granulation Tissue ; Stomach