The First Case Series of Cryopyrin-Associated Periodic Syndrome in Korea

Cryopyrin-associated periodic syndrome (CAPS) is a hereditary autoinflammatory syndrome caused by mutations in NLRP3 (encoding cryopyrin), which presents with fever, fatigue and arthralgia. Thus far, however there have been no reports of CAPS in Korea. Herein, we report 3 cases of CAPS for the first time in Korea. The first case, a 28-year-old man with recurrent urticaria, arthralgia and fever induced by cold, all of which were observed in his father, showed elevated erythrocyte sedimentation rate and C-reactive protein. He exhibited a p.Gly303Asp variant of the NLPR3 gene. The second case, a 2-year-old girl who had recurrent urticaria, arthritis and oral and genital ulcers, was positive for HLA B51 and a p.Glu569Lys mutation in exon 3 of the NLRP3 gene. Administration of anakinra greatly improved her symptoms. The third case, a 4-year-old boy who presented with recurrent urticaria, arthralgia, and fever, exhibited a p.Val72Met mutation in exon 1 of the NLRP3 gene. Administration of tocilizumab improved all of his symptoms. This small case series suggests that clinicians consider CAPS and conduct genetic studies when arthralgia and fever are accompanied by urticaria in Korea.

Surgical Treatment for Rupture of the Right Common Iliac Artery Caused byTranscatheter Balloon Aortic Valvuloplasty in a Baby with Congenital Aortic Stenosis: A case report / 대한흉부외과학회지

Transcatheter interventions are widely used for diagnosis and treatment of congenital heart disease. Complications associated with transcatheter interventions are uncommon. However, when they occur they are most often self- limited. Rarely, however, serious catheter related complications occur that may require emergent surgical intervention. In this case, the right common iliac artery was disrupted during transcatheter balloon valvuloplasty during the treatment of congenital aortic stenosis in a 2-week-old baby. After immediate surgical intervention with bleeding control using two balloon catheters the baby did well. Here we report this case and review the medical literature.

Is Preventive Bilateral Surgery Needed in Case of Bilateral Bullae on HRCT at Unilateral Primary Spontaneous Pneumothorax / 대한흉부외과학회지

BACKGROUND: Due to the advancement of video assisted thoracoscopic techniques, an operation for primary spontaneous pneumothorax is now considered a common procedure. However, whether a preventive operation is necessary when a contralateral bulla is found on High Resolution Computed Tomography (HRCT) at the time of the first primary spontaneous pneumothorax attack is still unknown. In this retrospective study, it was our intension to find whether contralateral bullae are related to the occurrence of pneumothorax. MATERIAL AND METHOD: Between January 1999 and April 2006, 550 patients were admitted to the Chungnam University hospital with primary spontaneous pneumothorax, which was confirmed by the HRCT scans in 190 patents. In these 190 patients, 159 had not received a bilateral operation after their first primary spontaneous pneumothorax attack. In these 159 patients, the relationship between the presence of contralateral bullae and the occurrence of pneumothorax was measured. RESULT: In these 159 patients, 67 had contralateral bullae confirmed inform the HRCT scan, and 92 had no visible contralateral bullae. During the follow up period, 6 patients (8.9%) with contralateral bullae had an occurrence of contralateral pneumothorax, and 5 patients (5.4%) without contralateral bullae had an occurrence of contralateral pneumothorax. (p=0.529 [Fisher's exact test]) CONCLUSION: In patients with unilateral primary pneumothorax, an HRCT scan is a useful way of confirming contralateral pulmonary bullae. However, the presence of bullae is not a significant predictive sign of an occurrence of contralateral pneumothorax. Also, surgery for pneumothorax is not completely uncomplicated, and bilateral surgery is still doubtful. A further prospective study will be required to find the relationship between the bullae found on HRCT and the occurrence of pneumothorax.

Right Atrial Thrombus Mimicking Right Atrial Myxoma / 대한흉부외과학회지

A right atrial thrombus in structurally normal heart is very rare. A 66-year-old woman was admitted with chest discomfort and dyspnea. She was diagnosed of right atrial myxoma on echocardiography and chest computed tomography. We performed an excision of the mass attached to atrial septum, which was found to be an organized mural thrombus by pathologic examination. We report this rare case with a review of literature.

Changes of Brain Natriuretic Peptide Levels according to Right Ventricular HemodynaMics after a Pulmonary Resection / 대한흉부외과학회지

BACKGROUND: The correlation between levels of brain natriuretic peptide (BNP) and the effect of pulmonary resection on the right ventricle of the heart is not yet widely known. This study aims to assess the relationship between the change in hemodynamic values of the right ventricle and increased BNP levels as a compensatory mechanism for right heart failure following pulmonary resection and to evaluate the role of the BNP level as an index of right heart failure after pulmonary resection. MATERIAL AND METHOD: In 12 non small cell lung cancer patients that had received a lobectomy or pnemonectomy, the level of NT-proBNP was measured using the immunochemical method (Elecsys 1010(R), Roche, Germany) which was compared with hemodynamic variables determined through the use of a Swan-Ganz catheter prior to and following the surgery. Echocardiography was performed prior to and following the surgery, to measure changes in right ventricular and left ventricular pressures. For statistical analysis, the Wilcoxon rank sum test and linear regression analysis were conducted using SPSSWIN (version 11.5). RESULT: The level of postoperative NT-proBNP (pg/mL) significantly increased for 6 hours, then for 1 day, 2 days, 3 days and 7 days after the surgery (p=0.003, 0.002, 0.002, 0.006, 0.004). Of the hemodynamic variables measured using the Swan-Ganz catheter, the mean pulmonary artery pressure after the surgery when compared with the pressure prior to surgery significantly increased at 0 hours, 6 hours, then 1 day, 2 days, and 3 days after the surgery (p=0.002, 0.002, 0.006, 0.007, 0.008). The right ventricular pressure significantly increased at 0 hours, 6 hours, then 1 day, and 3 days after the surgery (p=0.006, 0.009, 0.044, 0.032). The pulmonary vascular resistance index [pulmonary vascular resistance index=(mean pulmonary artery pressure-mean pulmonary capillary wedge pressure)/cardiac output index] significantly increased at 6 hours, then 2 days after the surgery (p=0.008, 0.028). When a regression analysis was conducted for changes in the mean pulmonary artery pressure and NT-proBNP levels after the surgery, significance was evident after 6 hours (r=0.602, p=0.038) and there was no significance thereafter. Echocardiography displayed no significant changes after the surgery. CONCLUSION: There was a significant correlation between changes in the mean pulmonary artery pressure and the NT-proBNP level 6 hours after a pulmonary resection. Therefore, it can be concluded that changes in NT-proBNP level after a pulmonary resection can serve as an index that reflects early hemodynamic changes in the right ventricle after a pulmonary resection.

Alteration of Apurinic/Apyrimidinic Endonuclease-1/Redox Factor-1 in Human Non-small Cell Lung Cancer / 대한흉부외과학회지

BACKGROUND: An imbalance between oxidants and antioxidants leads to oxidative stress, and this has been proposed to play an important role in the pathogenesis of lung neoplasm. Apurinic/apyrimidinic endonuclease-1/redox factor-1 (APE/ref-1) is a multifunctional protein involved in DNA base excision repair and the redox regulation of many transcription factors. However, the alteration of the expressed levels of APE/ref-1 in non-small cell lung cancer is unknown. MATERIAL AND METHOD: Forty-nine patients with surgically resected non-small cell lung cancer (NSCLC) were included in this study. Immunohistochemical staining with APE/ref-1 antibodies was performed, and their expressions were analyzed via Western blotting for specific antibodies. RESULT: APE/ref-1 was localized at the nucleus and mainly in the non-tumor region of the NSCLC tissue specimens; it was expressed in the cytoplasm and nucleus of the NSCLC. The nuclear and cytoplasmic expressions of APE/ref-1 in lung cancers were markedly up-regulated in the NSCLC, and this was correlated with the clinical stage. Catalase, as first-line antioxidant defense, was dramatically decreased in the NSCLC. CONCLUSION: Taken together, our results suggest that APE/ref-1, and especially cytoplasmic APE/ref-1, was upregulated in the lung cancer regions, and this may contribute to the compensatory defense system against oxidative stress. A low expression of catalase might have fundamental effects on the extracellular redox state of lung tumors, along with the potential consequences for the tumors.

Carotid-jugular Arteriovenous Fistula Caused by Gunshot Injury / 대한흉부외과학회지

Carotid artery-internal jugular vein arteriovenous fistula is very rare, but it should be suspected in case of vascular injury by neck trauma because the diagnosis may be missed due to anatomical complexity of neck. We report a 57-year old male who had the carotid artery-jugular vein arteriovenous fistula caused by gunshot injury in the neck 44 years ago.

Benign Symmetrical Lipomatosis (Madelung's Disease): A case report / 대한흉부외과학회지

Benign symmetric lipomatosis (BSL), also called Madelung's disease, is a rare disease in middle-aged chronic alcohol user. The cause of BSL is unknown. A 63 year-old man with rapid growing lesions in both shoulders for 2 months visited our hospital. Except for cosmetic problem, no abnormal finding was found in blood cell analysis and chemistry; however, excessive fat deposition was found on radiographic findings. Lipoma was revealed in pathologic examination and BSL was diagnosed clinically. Patient is being followed up without any specific problem.

Comparison of Actigraphic Performance between ActiWatch (r) and SleepWatch (r) : Focused on Sleep Parameters Utilizing Nocturnal Polysomnography as the Standard / 수면정신생리

OBJECTIVES: We attempted to compare the performance of 2 commercially available actigraphies with focus on sleep parameters, using polysomnography as standard comparison tool. METHODS: Fourteen normal volunteers (5 males and 9 females, mean age of 28+/-4.6 years) participated in this study. All the participants went through one night of polysomnography, simultaneously wearing 2 different kinds of actigraphies on each wrist. Polysomnographic and actigraphic data were stored, downloaded, and processed according to standard protocols and then statistically compared. RESULTS: Both ActiWatch (r) and SleepWatch (r) tended to overestimate the total sleep time, compared to the polysomnography. Sleep-Watch (r) tended to underestimate the sleep latency. The two actigraphs and the polysomnograph did not show significant difference of sleep efficiency, when compared with one another. In addition, all of the sleep parameters from the instruments showed linear correlations except in SleepWatch (r) 's sleep latency. The sleep parameters from the two actigraphs did not show much noteworthy difference, and linear relationships were found between the sleep parameters from the two actigraphs. There was no significant distinction in the results of the two different actigraphs. CONCLUSION: The results of two actigraphies can be used interchangeably since the sleep parameters of the two different actigraphies do not show significant differences statistically. Overall, it is not legitimate to use actigraphy as a substitute for polysomnography. However, since sleep parameters except sleep latency show linear correlations, actigraphy might possibly be used to follow up patients after polysomnography.

Surgical Treatment of Traumatic Ventricular Septal Defect by Penetrating Chest Injury / 대한흉부외과학회지

Thirteen year old boy who had been stabbed in his left chest by the knife was transferred to our department from a general hospital, because of the massive bleeding from the intercostal tube drainage. Chest X-ray showed homogeneous density in the left lung field. He was confused and his vital signs were unstable. He was moved into a operating room as soon as possible. After resuscitation, his lacerated left ventricle wound was sutured through median sternotomy. The interventricular shunt was detected with intraoperative transesophageal echocardiography. The traumatic ventricular septal defect was closed via left ventricle using Dacron patch. His postoperative course was uneventful, and he was discharged with small residual shunt.