BACKGROUND: A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagnosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a short nose with bulbous tip. METHODS: Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. RESULTS: The Apert syndrome patient had a shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. CONCLUSION: Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
Acrocephalosyndactylia ; Adult ; Child ; Exophthalmos ; Forehead ; Hand ; Humans ; Hypertelorism ; Male ; Maxilla ; Nasal Septum ; Nose ; Osteotomy ; Palate ; Prognathism ; Prosencephalon ; Skull ; Skull Base ; Sutures ; Syndactyly

Peripheral T-cell lymphomas (PTCLs) are aggressive neoplasms which may involve the liver. The imaging manifestations of hepatic lymphoma are highly variable and show overlapping appearances of numerous other hepatic diseases. As the management and prognosis of lymphoma differ markedly from those of other malignant diseases, prompt diagnosis and early effective treatment are very important. Here, we report an atypical case of primary PTCL not otherwise specified involving the liver that exhibited a solitary hepatic mass mimicking hepatocellular carcinoma (HCC) on CT. Liver biopsy is not commonly recommended in highly suspicious cases of HCC. However, in a patient without risk factors for HCC, consideration of other diagnostic possibilities is required and needle biopsy may be a more rational choice. An imaging approach, based on a careful review of clinical and laboratory findings is essential to prevent false-positive diagnosis of HCC and subsequent invasive treatment.
Biopsy ; Biopsy, Needle ; Carcinoma, Hepatocellular* ; Diagnosis ; Hepatectomy ; Humans ; Liver ; Lymphoma ; Lymphoma, T-Cell, Peripheral* ; Prognosis ; Risk Factors

Calcium channel blockers (CCBs) are very popular drugs to lower blood pressure (BP) without significant side effects. A 72-year-old man admitted for uncontrolled hypertension. He had history of hypertension, atrial fibrillation with slow ventricular response, angina, abdominal aortic aneurysm, and stage 3 chronic kidney disease. He had taken several anti-hypertensives, such as amlodipine 5 mg, perindopril 8 mg, and indepamide 1.5 mg. To control BP, nifedipine 120 mg was added. Then pulmonary edema and pleural effusion was developed. Echocardiography showed preserved left ventricular ejection fraction and mild mitral regurgitation. Fluid restriction and high dose furosemide did not cease pleural fluid accumulation. Thus a total of 4 times of thoracentesis were done and all fluid analyses revealed transudate. We thought that pleural effusion and pulmonary edema was induced by CCBs and discontinued the drugs. He recovered quickly and finally discharged in a stable condition.
Aged ; Amlodipine ; Antihypertensive Agents ; Aortic Aneurysm, Abdominal ; Atrial Fibrillation ; Blood Pressure ; Calcium Channel Blockers* ; Calcium Channels* ; Calcium* ; Echocardiography ; Exudates and Transudates ; Furosemide ; Humans ; Hypertension* ; Mitral Valve Insufficiency ; Nifedipine ; Perindopril ; Pleural Effusion* ; Pulmonary Edema* ; Renal Insufficiency, Chronic ; Stroke Volume

A 74-year-old woman who had paroxysmal atrial fibrillation without mitral stenosis was hospitalized for syncope and right-sided weakness. Echocardiography revealed a large free-floating thrombus in the left atrium, sometimes prolapsing partially into the left ventricle in diastole. Because of her poor neurological status, she was managed with anticoagulation. On the 12th day, the thrombus had disappeared on the follow-up echocardiography, and aortoiliac embolization was later detected on computed tomography. Unfortunately, she developed various complications of stroke and limb infarction, and died after 4 months of hospital care. In addition to this case report, we reviewed a total 70 cases of left atrial free-floating thrombus. Atrial fibrillation and mitral pathology were two major causative factors. All the cases, except 1, were confirmed on echocardiography. The most common presentation that led to echocardiography was systemic embolization, followed by heart failure. Others were acute hemodynamic decompensation from mitral obstruction, chest pain, palpitation, and bacteremia. Cardiac thrombectomy was the preferred treatment modality with favorable outcomes.
Aged ; Atrial Fibrillation ; Bacteremia ; Chest Pain ; Diastole ; Echocardiography ; Extremities ; Female ; Follow-Up Studies ; Heart Atria ; Heart Failure ; Heart Ventricles ; Hemodynamics ; Humans ; Infarction ; Mitral Valve Stenosis ; Pathology ; Stroke ; Syncope ; Thrombectomy ; Thrombosis*

BACKGROUND: Reduced heart rate varaibility (HRV) after acute myocardial infarction (AMI) is an important risk factor for mortality and life-threatening arrhythmias. The correlation between the left ventricular (LV) diastolic function and autonomic balance expressed by HRV in patients with AMI was evaluated in this study. METHODS: A 2-dimensional and Doppler echocardiography and a 24-hour Holter monitoring were performed at 5th to 7th day after attack in 50 patients with AMI. The restrictive filling pattern of the LV diastolic function was defined by E/A ratio >2 or deceleration time (DT) of the mitral inflow Arrhythmias, Cardiac ; Deceleration ; Echocardiography, Doppler ; Electrocardiography, Ambulatory ; Heart Rate* ; Heart* ; Humans ; Mortality ; Myocardial Infarction* ; Risk Factors ; Ventricular Function

Sclerosing lymphangitis of the penis is a rare condition involving the distal lymphatics that is characterized by cord-like lesions on the shaft or coronal sulcus. Although the etiology is unknown, its association with mechanical trauma, anatomic variants, or infection has been shown. Clinically, the patient notices a painless, firm, cord-like lesion just proximal to the sulcus. Histologic study reveals hypertrophy and sclerosis of lymphatic vessel walls and, in some cases, thrombus formation within the dilated vessels. Most cases are self-limited, and conservative management is indicated, but surgical excision is warranted for persistently symptomatic lesions. We report three cases of sclerosing lymphangitis of the penis that required treatment by surgical excision.
Humans ; Hypertrophy ; Lymphangitis* ; Lymphatic Vessels ; Male ; Penis* ; Sclerosis ; Thrombosis

BACKGROUND: To evaluate the efficacy and safety of gemcitabine and cisplatin chemotherapy in advanced non-small cell lung cancer (NSCLC). MATERIALS AND METHODS: Forty patients (21 men, 19 women ; age range, 37 to 73 years; median, 63 years) with unresectable stage IIIB to IV NSCLC were evaluated. Patients received cisplatin 60mg/m2 (Day 1), gemcitabine 1200mg/m2 (Day 1 and 8) every 21 days. Eighteen patients had stage IIIB disease and 22 had stage IV. There were 28 patients of adenocarcinoma (70.0%), 11 of squamous cell carcinoma (27.5%), and one of large cell carcinoma (2.5%). RESULTS: Of 40 patients, no patients showed complete response while 15(37.5%) showed partial response, 7(17.5%) had stable diseases, 18(45%) had progressive diseases. During a total of 195 courses of chemotherapy, grade 3 or more granulocytopenia and thrombocytopenia occured in 12.5% and 2.5% of patients respectively. Non-hematologic toxicity was mild and easily controlled. There was one case of treatment-related death by pneumomia. The median survival was 55 weeks (95% CI, 34~75weeks), and the time to progression was 19 weeks (95% CI, 16~23weeks). One year survival rate was 55% and 2 year survival rate was 10%. CONCLUSION: The efficacy of cisplatin and gemcitabine combination chemotherapy was acceptable in the treatment of advanced NSCLC.
Adenocarcinoma ; Agranulocytosis ; Carcinoma, Large Cell ; Carcinoma, Non-Small-Cell Lung* ; Carcinoma, Squamous Cell ; Cisplatin ; Drug Therapy ; Drug Therapy, Combination* ; Female ; Humans ; Male ; Survival Rate ; Thrombocytopenia

Mitomycin (MMC) is a naturally ocurring alkylating agent, introduced for clinical use as early as 1958. This drug is useful in the therapy of gastrointestinal carcinomas when used in combination with 5-fluorouracil. Nephrotoxicity among toxicities from MMC is unusual with cumulative doses less than 30 mg/m2. In large studies in which the incidence of MMC nephrotoxicity were assessed, 3-15% of patients developed total dose related renal dysfunction. Three patients in our clinical practice have developed thrombotic microangiopathy clearly related to MMC. We report the clinical and pathologic features of our cases. In view of the probable dose-related and delayed toxicity of MMC, it seems necessary to monitor regularly after initiation of chemotherapy. Early detection of the renal impairment and withdrawal of MMC might halt further progression of renal failure.
Drug Therapy ; Fluorouracil ; Humans ; Incidence ; Mitomycin* ; Renal Insufficiency ; Thrombotic Microangiopathies*

BACKGROUN: Talc sclerotherapy is widely used for symptomatic malignant pleural effusion. The object of this study was to evaluate the outcome of talc slurry sclerotherapy, and to compare the efficacy of the small-bore catheter with that of chest tube in sclerotherapy of malignant pleural effusion. METHODS: From January 2000 to May 2002, 37 patients with malignant pleural effusion were enrolled and randomized to the chest tube (28F, n=17) or the small-bore catheter (14F, n=20) groups. The majority of patients had lung cancer (n=33, 89%) and two had breast cancer. The median age was 55 years. After verification of reexpansion of lung on chest radiogram. five grams of purified asbestos-free talc in 50 mL of normal saline were used for talc slurry sclerosis. The success of the procedure was defined as daily drainage below 50 mL within 1 week after talc slurry instillation. Side effects of the sclerotherapy and complications were compared by the drainage method and the recurrence rates in 3, 6 and 9 months were evaluated. RESULTS: Initial success rates of sclerotherapy by small-bore catheter was 80% and that of chest tube was 70.5% (p=0.07). The most common early complication after talc slurry instillation was pain followed by fever. But procedure related mortality or respiratory failure was not developed. The mean duration of drainage by small-bore catheter was 8.2days and that of chest tube was 8.8days (p=0.60). But the catheter-related complications of pain, subcutaneous infection and, emphysema were significantly less in the small-bore catheter group than the chest tube groups (15% vs 88%, 5% vs 23.5%, 0% vs 17.5% respectively) There was no statistically significant difference between the two groups in the recurrence rate at 3 months (37.5% for the small-bore catheter vs. 33.3% for the chest tube, p=0.45), 6 months (56.3% vs. 58.3%, p=0.75), and 9 months (87.5% vs. 83.3%, p=0.65). CONCLUSION: Talc slurry sclerotherapy via chest tube or small-bore catheter was a safe and effective method for the treatment of symptomatic malignant pleural effusion. But small-bore catheters are preferred to the chest tube in the sense of catheter-related complications.
Breast Neoplasms ; Catheters* ; Chest Tubes* ; Drainage ; Emphysema ; Fever ; Humans ; Lung ; Lung Neoplasms ; Mortality ; Pleural Effusion, Malignant* ; Recurrence ; Respiratory Insufficiency ; Sclerosis ; Sclerotherapy* ; Talc* ; Thorax*

BACKGROUND: Small cell lung cancer represents approximately 20% of all carcinomas of the lung, and is recognized as having a poor long term outcome compared to non-small cell lung cancers. Therefore, this study investigated the prognostic factors in small cell lung cancer patients in order to improve the survival rate by using the proper therapeutic methods. MATERIAL AND METHOD: The clinical data from 394 patients, who diagnosed with small cell lung cancer and treated from 1993 to 2001 at the Kosin University Gospel Hospital, were analyzed. RESULT: There were 314 male patients (79.7%), and 80 female patients (20.3%). The number of those with limited disease was 177 (44.9%), and the number of those with extensive disease was 217 (55.1%). Overall, 366 out of 394 enrolled patients had died. The median survival time was 215 days (95% CI : 192-237days). The disease stage, Karnofsky performance state, 5% body weight loss for the recent 3 months, chemotherapy regimens, and the additive chest radiotherapy were identified as being statistically significant factors for the survival time. The median survival times of the supportive care group, one anticancer therapy, and two or more treatment groups were 71 days, 211 days, and 419 days, respectively (p<0.001). The data emphasizes the importance of anticancer treatment for improving the survival time for patients. The group of concurrent chemoradiotherapy regimens (30 patients) showed a significantly longer survival time than the group given sequential chemoradiotherapy (55 patients) (528 days versus 373 days, p=0.0237). The favorable prognostic factors of the laboratory study were groups of leukocytes =8,000/mm3, ALP=200 U/L, LDH=450 IU/L, NSE=15 ng/mL, s-GOT=40 IU/L. In extensive disease, there was no difference according to the number of metastatic sites. However, the median survival time of the patients with an ipsilateral pleural effusion was longer than the patients with other metastatic sites. According to the survey periods, three groups were divided into 1993-1995, 1996-1998, and 1999-2001. The median survival time was significantly prolonged after 1999 in comparison to previous groups (177 days, 194 days, 289 days, p=0.001, 0.002, respectively). CONCLUSION: Disease stage and 5% body weight loss for the recent 3 months at the diagnostic state were significant prognostic factors. In addition, the performance status, serum ALP, LDH, NSE, CEA levels also appear to be prognostic factors. The survival time of those patients with small cell lung cancer has been prolonged in recent years. It was suggested that the use of the EP (etoposide and cisplatin) chemotherapy method and concurrent chemoradiotherapy for patients with a limited stage contributed to the improved survival time.
Body Weight ; Chemoradiotherapy ; Drug Therapy ; Female ; Humans ; Leukocytes ; Lung ; Lung Neoplasms ; Male ; Pleural Effusion ; Radiotherapy ; Small Cell Lung Carcinoma* ; Survival Rate ; Thorax