The majority of cardiac myxoma occur sporadically as isolated lesions in the left atrium of middle-aged women. However, a familial form and a syndrome form of this lesion have also been identified. The syndrome myxoma can present itself with pigmented skin lesions and peripheral or endocrine neoplasms. The familial and syndrome forms of cardiac myxomas can usually be distinguished from the sporadic form by their occurrences at younger ages, their unusual locations, the multicentricity of the lesions, and the presence of rare pathological conditions. In addition, a higher rate of recurrent lesions is usually associated with the familial and syndrome forms of this disease. We present a case of complex cardiac myxoma with pigmented skin lesions and breast myxoma and report it with a review of literature.
Breast ; Female ; Heart Atria ; Humans ; Myxoma* ; Nipples* ; Skin

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system (CNS) caused by a persistent, aberrant measles virus infection. The outcome is usually fatal. After a latent period of 6 to 7 years, there is subtle and slow cognitive decline and visuospatial disorientation develops followed by myoclonic jerks, extrapyramidal symptoms, ataxia, and seizures, progressing to coma or vegetative state. The diagnosis is based on at least three of the following criteria 1) clinical manifestations 2) abnormal EEG 3) hyperglobulinorrachia, elevated serum or spinal fluid measles antibody 4) histologic features. No therapeutic maneuver has been proven conclusively to be of value. We have diagnosed and experienced a case of subacute sclerosing panencephalitis (SSPE) in a 5-year-old child with the chief complaint of myoclonic seizure and mental deterioration. We report a case and the brief review of related literature.
Ataxia ; Central Nervous System ; Child ; Child, Preschool ; Coma ; Diagnosis ; Electroencephalography ; Humans ; Measles ; Measles virus ; Myoclonus ; Persistent Vegetative State ; Seizures ; Subacute Sclerosing Panencephalitis*

PURPOSE: Asthma exacerbation from human rhinovirus (HRV) infection is associated with deficient antiviral interferon (IFN) secretion. Although chronic rhinosinusitis (CRS), an inflammatory upper airway disease, is closely linked to asthma, IFN-beta responses to HRV infections in human nasal epithelial cells (HNECs) from CRS patients remain to be studied. We evaluated inflammatory and antiviral responses to HRV infection in HNECs from CRS patients. METHODS: HNECs, isolated from turbinate tissue of 13 patients with CRS and 14 non-CRS controls, were infected with HRV16 for 4 hours. The HRV titer, LDH activity, production of proinflammatory cytokines and IFN-beta proteins, and expression levels of RIG-I and MDA5 mRNA were assessed at 8, 24, and 48 hours after HRV16 infection. RESULTS: The reduction in viral titer was slightly delayed in the CRS group compared to the non-CRS control group. IL-6 and IL-8 were significantly increased to a similar extent in both groups after HRV infection. In the control group, IFN-beta production and MDA5 mRNA expression were significantly increased at 8 and 24 hours after HRV16 infection, respectively. By contrast, in the CRS group, IFN-beta was not induced by HRV infection; however, HRV-induced MDA5 mRNA expression was increased, but the increase was slightly delayed compared to the non-CRS control group. The RIG-I mRNA level was not significantly increased by HRV16 infection in either group. CONCLUSIONS: HRV-induced secretion of proinflammatory cytokines in CRS patients was not different from that in the non-CRS controls. However, reductions in viral titer, IFN-beta secretion, and MDA5 mRNA expression in response to HRV infection in CRS patients were slightly impaired compared to those in the controls, suggesting that HRV clearance in CRS patients might be slightly deficient.
Asthma ; Cytokines ; Epithelial Cells* ; Humans ; Interferon-beta* ; Interferons ; Interleukin-6 ; Interleukin-8 ; Rhinovirus ; RNA Helicases ; RNA, Messenger ; Turbinates

Pierre Robin syndrome presents in the neonatal period with upper airway obstruction and feeding difficulties. Infants with pronounced micrognathia may fail to thrive because of chronic airway obstruction, or experience severe respiratory distress and feeding difficulties. This is potentially fatal and surgical intervention in these cases is necessary. We experienced two newborns with pronounced micrognathia who were managed with tongue-lip adhesion and tracheostomy. Herein, we report these two cases with literature reviews.
Airway Obstruction ; Humans ; Infant ; Infant, Newborn ; Pierre Robin Syndrome* ; Tracheostomy*

BACKGROUND AND OBJECTIVES: Cervical lymph node (LN) metastases are common in papillary thyroid carcinoma (PTC) and cervical neck dissection (ND) is frequently performed to improve regional control of the disease. However, there is a controversy as to the extent of lateral cervical ND. In the present study, we examined the diagnostic accuracy of ultrasonography (US) or CT and the predictive factors of level II LN metastases in PTC patients with clinically positive lateral neck nodes. SUBJECTS AND METHOD: We retrospectively analyzed 78 patients who underwent thyroidectomy and lateral ND including level II LN between August 1998 to June 2008. To identify predictive factors of cervical node metastases to level II, diverse factors were analyzed. RESULTS: The most common site of metastasis was level IV (83.3% of cases). The accuracy of diagnostic imaging in the detection of level II metastasis revealed sensitivity of 89.4%, and specificity of 93.5%. The results of the univariate analysis showed that the presence of level II metastases was significantly associated with the location of primary tumor (p<0.001), number of metastatic LN (p=0.001), and great size of metastatic LN (p=0.010). In addition, the multivariate analysis showed that the location of primary tumor and number of metastatic LN were an independent risk factor for the presence of level II metastasis. CONCLUSION: Preoperative imaging was accurate for detecting the presence of level II LN metastasis. Patients with upper lobe tumor and multiple metastatic LN have a higher risk of harboring metastatic disease at the level II. We suggest that routine dissection of the level II LN may not be necessary for patients with no evidence of diagnostic imaging and predictive factors of level II LN metastasis.
Carcinoma ; Diagnostic Imaging ; Factor IX ; Humans ; Lymph Nodes ; Multivariate Analysis ; Neck ; Neck Dissection ; Neoplasm Metastasis ; Retrospective Studies ; Risk Factors ; Sensitivity and Specificity ; Thyroid Gland ; Thyroid Neoplasms ; Thyroidectomy

BACKGROUND AND OBJECTIVES: Squamous cell carcinoma of the buccal mucosa (BMSCC) has been known to have poor prognosis characterized by a high locoregional failure rate. The purpose of this study is to assess the current treatment outcome and to estimate the prognostic factors in patients with BMSCC. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 20 patients who were diagnosed and treated for BMSCC between August 1996 and May 2008. Potential prognostic factors including age, gender, stage, histologic grade, treatment modality were evaluated. RESULTS: The 5-year overall survival and failure-free survival rate were 47% and 42%, respectively. The 3-year overall survival rate in the early staged carcinoma (stage I and II) was 76%, and in the advanced staged carcinoma (stage III and IV), 38% (p=0.022). Patients who had surgery as initial treatment or well differentiated cancer achieved better overall survival rates (p=0.003, p=0.018). Forty-five percent of patients had treatment failure during follow-up. The univariate analysis showed that clinical stages and histologic grades are the two most important prognostic factors responsible for treatment failure. CONCLUSION: The 5-year survival rate of BMSCC was 47%. The strong influence of disease stage on prognosis emphasizes the importance of early diagnosis of BMSCC and aggressive treatment for patients with poorly/moderated differentiated cancer.
Carcinoma, Squamous Cell ; Early Diagnosis ; Follow-Up Studies ; Humans ; Medical Records ; Mouth Mucosa ; Mouth Neoplasms ; Prognosis ; Retrospective Studies ; Survival Rate ; Treatment Failure ; Treatment Outcome

Four trials of external quality assessment in diagnostic hematology were performed in 2008 with average 822 participating laboratories in Korea. We performed quality assessment for white blood cell count, hemoglobin, hematocrit, red blood cell count, platelet count, blood cell morphology, prothrombin time and activated partial thromboplastin time. The response rate was more than 96.5%. The coefficients of variation in hemoglobin, hematocrit and RBC was stable but variable in platelet count and WBC count according to measuring cell count. Test results of blood cell morphology showed variation among various cell morphologies.
Blood Cells ; Cell Count ; Erythrocyte Count ; Hematocrit ; Hematology ; Hemoglobins ; Korea ; Leukocyte Count ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time

Totally implanted central venous access devices for chemotherapy (chemoport) are being used increasingly in lung cancer patients. Vascular catheters are associated with various complications including infection, thrombosis as well as spontaneous fractures and embolization of the catheter, which is known as 'pinch-off syndrome'. 'Pinch-off syndrome' refers to the compression of a subclavian central venous catheter between the clavicle and first rib resulting in an intermittent or permanent obstruction, which can lead to tears, transection, or embolization. We report two cases of fractured and embolized implanted subclavian venous catheters in which the fragments were removed percutaneously. A 62-year-old man presented with back pain with a duration of a few weeks. The chest radiograph revealed complete transsection and embolization of the catheter into the right atrium. In addition, a 47-year-old woman with a chemoport had a grade 3 pinch-off sign in a chest radiograph demonstrating complete transsection and embolization of the catheter into the pulmonary artery. Both cases were managed by retrieving the embolized distal fragment percutaneously and removing the proximal section of the catheter.
Back Pain ; Catheters ; Central Venous Catheters ; Clavicle ; Drug Therapy ; Female ; Fractures, Spontaneous ; Heart Atria ; Humans ; Lung Neoplasms* ; Lung* ; Middle Aged ; Pulmonary Artery ; Radiography, Thoracic ; Ribs ; Thrombosis ; Vascular Access Devices

Four trials of external quality assessment in diagnostic hematology were performed in 2003 with about 430 participating laboratories in Korea. We performed quality assessment for white blood cell count, hemoglobin, red blood cell count, platelet count, white cell differential count, red blood cell morphology and coagulation test. The response rate was more than 95%. The performance of quality assessment appeared to be gradually improved year by year.
Equidae* ; Erythrocyte Count ; Erythrocytes ; Hematology* ; Korea* ; Leukocyte Count ; Platelet Count

Four trials of external quality assessment in diagnostic hematology were performed in 2004 with about 440 participating laboratories in Korea. We performed quality assessment for white blood cell count, hemoglobin, red blood cell count, platelet count, white cell differential count, red blood cell morphology and coagulation test. The response rate was more than 96%. The coefficients of variation in hemoglobin and RBC number was stable but variable in platelet number and WBC number according to measuring cell counts. Blood coagulation study was performed twice. Test results show wide variation according to measuring machine and reagents.
Blood Coagulation ; Cell Count ; Equidae* ; Erythrocyte Count ; Erythrocytes ; Hematology* ; Indicators and Reagents ; Korea* ; Leukocyte Count ; Platelet Count