Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab / 대한내과학회지

Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury, which results from uncontrolled complement activation. Delayed diagnosis and treatment of aHUS may result in end-stage renal disease (ESRD) and an associated dependence on dialysis. In extreme cases, it may cause death due to multi-organ failure. Eculizumab, a humanized monoclonal antibody against C5, inhibits the formation of the terminal membrane attack complex and is used to treat aHUS. Here, we report a 46-year-old male patient who suffered from aHUS relapse, despite prior treatment with repeated plasma exchange and hemodialysis. Eculizumab therapy improved his hematologic findings without use of hemodialysis.

Glomangiomyoma of the Trachea

A glomus tumor is an uncommon soft tissue tumor that is most commonly found in the subungual area and a glomus originating in the trachea is extremely rare. Histologically and ultrastructurally, these tumors have been divided into three subtypes: classic glomus tumors, glomangiomas, and glomangiomyomas. Glomangiomyomas account for less than 10% of all glomus tumors and are the least common type. We report a case of a 54-year-old man with glomangiomyoma of the trachea who presented with stridor. We treated the tumor by segmental resection and primary repair via a transcervical approach.

Clinical Analysis of the Surgical Treatments for Large Primary Spontaneous Pneumothorax / 대한흉부외과학회지

BACKGROUND: The clinical history and physical findings of the patients with spontaneous pneumothorax depend largely on the extent of the collapse of the lung and the presence of pre-existing pulmonary disease. Large primary spontaneous pneumothorax is a possible serious condition and so more active treatment will be necessary for these patients. The therapeutic guideline for large pneumothorax remains controversial. Therefore, by assessing the clinical results of surgical treatment for large primary pneumothorax, we aim to determine the indicators of treatment. MATERIAL AND METHOD: Among 348 patients with primary spontaneous pneumothorax and who underwent surgical treatment from August 2004 through December 2007, 58 patients who responded to treatment for a large primary pneumothorax were included in the current study. We then retrospectively evaluated the operative findings and the surgical results. The patients with a pneumothorax of 80% or more, including those patients with tension pneumothorax, were considered to have a "large pneumothorax". Most of these patients should be treated with a 12F chest tube. Thoracoscopic wedge resection was considered for treating recurrent pneumothorax, continuous air leakage, controlateral pneumothorax and first episode pneumothorax with visible blebs (> 1 cm) seen on the computed tomography. RESULT: There were 50 men and 8 women with a mean age of 28.2 years (range: 14~54 years). The mean length of hospitalization was 5.3 days (range: 2~10 days). Nine patients underwent chest tube drainage only. Forty-nine patients underwent thoracoscopic wedge resection. The mean follow up time was 27.8 months (range: 10~58 months). The actual site of air leakage could be located in 35 patients (71.4%) and this was correlated with pleural adhesion (p=0.005). The initial air leakage tended to be more correlated with intraoperative air leakage, although this was not statistically significant (p=0.066). The recurrence rate was 11.1% for the patients with chest tube drainage and 2.0% for the patients with thoracoscopic wedge resection. CONCLUSION: Large primary pneumothorax requires an early diagnosis and early treatment. Thoracoscopic wedge resection may help to prevent recurrence of large primary pneumothorax.

Surgical Correction of Total Anomalous Pulmonary Venous Connection without Total Circulatory Arrest / 대한흉부외과학회지

BACKGROUND: Circulatory arrest under deep hypothermia is an important auxiliary means for surgical correction of total anomalous pulmonary venous connection (TAPVC). However, cardiac operations under deep hypothermic circulatory arrest are associated with the risk of post-arrest neurologic abnormalities. The purpose of this study is to evaluate the results of the surgical correction of total anomalous pulmonary venous connection without the total circulatory arrest. MATERIAL AND METHOD: Between April 2000 and October 2004, hospital records of 10 patients were reviewed retrospectively. RESULT: The locations for abnormal anatomical connections were supracardiac in 7 cases, cardiac in 1 case, and infracardiac in 2 cases. The mean cardiopulmonary bypass time and aorta cross clamp time were 116.8+/-40.7 and 69.5+/-24.1 minutes. There was no surgical mortality. Postoperative complications were post-repair pulmonary venous stenosis in 1 case, pneumonia in 1, pneumothorax in 1, wound infection in 1, and diaphragmatic paralysis in 1. All patients without pulmonary venous stenosis were in NYHA class I at mean follow-up of 16.6 months (3~49 months) CONCLUSION: We could obtain excellent results by repair without the total circulatory arrest for total anomalous pulmonary venous connection.

Acute Abdominal Aortic Occlusion after Open Heart Surgery: A case report / 대한흉부외과학회지

Acute abdominal Aortic occlusion is rare but it is a vascular emergency with high mortality and morbidity. Therefore, delay in diagnosis can have severe impact on the prognosis. A 60-year-old women complained of paresthesia, paralysis, and severe pain in bilateral lower extremities on 13th day after open heart surgery for mitral stenosis, atrial fibrillation, coronary arterial stenosis, tricuspid regurgitation, and atrial septal defect. Her skin was mottled and cool from the umbilicus to the feet, and there were no palpable pulses in the lower exteremities. We diagnosed an acute abdominal aortic occlusion using the Multi-Detector Row Spiral Computed Tomography and successfully treated the problem with emergent thrombo-embolectomy and Aortobifemoral bypass.

Surgical Treatment of Pulmonary Mucormycosis: 1 case report / 대한흉부외과학회지

Pulmonary mucormycosis is very rare but has a devastating opportunistic fungal infection in immunocompromised hosts. The infection usually occurs in patients with hematologic malignancy, chronic renal failure, diabetes mellitus, or in solid organ transplant recipients. We experienced a case of pulmonary mucormycosis associated with renal cadeveric allograft recipient who had uncontrolled diabetes mellitus. The patient was successfully treated by surgical resection with Amphotericin B therapy.