Classic pulmonary blastoma is a variant of carcinosarcoma which is seen almost exclusively in adults. By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, which was proposed as pleuropulmonary blastoma. Recently we experienced a case of pleuropulmonary blastoma, type 1. This 27-month-old male baby was transferred to our hospital due to the left tension pneumothorax. Chest CT revealed a subpleural pulmonary cystic lesion on the left upper lung and an open cystectomy was performed. Histologically the lesion was composed of variable-sized cystic structures lined with a single layer of respiratory-type epithelium. the underlying stroma was composed of sheets of small, round to oval, primitive tumor cells. Some of them had eccentric, eosinophilic cytoplasm, suggestive of rhabdomyoblastic differentiation. These rhabdomyoblastic cells were fuchsinophilic and positive with desmin and vimentin on immunohistochemistry.
Child ; Adult ; Male ; Female ; Humans

No abstract available.
Nortriptyline* ; Plasma*

Aneurysms of the pulmonary artery are uncommon in general but have a potentially fatal prognosis. This is derived from the potential for rupture of the aneurysm from the fact that there is commonly underlying severe pulmonary hypertension. Most cases are documented in the large postmortem series. Aneurysms of the pulmonary artery can be classified as congenital or acquired. Possible symptoms include dyspnea on exertion, cough, hemoptysis, and thoracic pain. When a large aneurysm formation of the pulmonary artery is diagnosed, surgical intervention is indicated because conservative treatment of the aneurysm will undoubtedly result in rupture with fatal outcome. We report a case of 46-year old patient with a giant left pulmonary artery aneurysm associated with severe pulmonary hypertension. A Doppler echocardiogram and a computed tomographic scan showed a giant saccular aneurysm of the left pulmonary artery (12 cm in diameter) and estimated systolic right ventricular pressure of 80 mmHg.
Aneurysm* ; Cough ; Dyspnea ; Fatal Outcome ; Hemoptysis ; Humans ; Hypertension, Pulmonary* ; Middle Aged ; Prognosis ; Pulmonary Artery* ; Rupture ; Ventricular Pressure

Ischemic heart disease is a common complication of thyrotoxicosis, although the exact mechanism has not been defined. In patient with dextrocardia as a part of situs inversus, coronary heart disease may occur with similar frequency and manifestation as in the general population. A case is reported of angina and thyrotoxicosis in patient with situs inversus totalis in which the angina was manifested by ST-segment elevation during graded exercise, relieved by antithyroid treatment. This unusual case establishes an association between hyperthy-roidism and ischemic heart disease.
Coronary Disease ; Dextrocardia ; Humans ; Hyperthyroidism* ; Myocardial Ischemia ; Situs Inversus* ; Thyrotoxicosis

No abstract available.

Edwards syndrome is first introduced by Edwards and characterized by facial anomalies, multiple cardiovascular, gastrointestinal, urogenital, and skeletal malformations. It results from triplication of part or all of chromosome 18 in some or all of the patient's cells. It has an incidence of 1 in 4,500 live births or less and short life expectancy. Recently we experienced a case of 3-day-old female new born infant with this syndrome. Post mortem examination showed progeric face with prominent occiput, large flabby ears, microphthalmia, and micrognathia, bilateral clenched hands with flexion contraction of middle fingers, and bilateral rockerbottom feet. Internal examination revealed horseshoe kidney, esophageal atresia with tracheoesophageal fistula, two accessory spleens, and multiple cardiac anomalies. A trisomy 18 was confirmed by the cytogenetic study.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Incidence

No abstract available.
Acute Kidney Injury*

No abstract available.
Adolescent* ; Humans ; Korea*

OBJECTIVES: To investigate the common causes of persistent septal deviation in revision septoplasty and to report the surgical techniques and results to correct them. METHODS: A total of 100 consecutive patients (86 males) who had revision septoplasty due to persistent septal deviation from 2008 and 2014 were included in the study. Their mean age was 35.6 years and the mean follow-up duration was 9.1 months. Presenting symptoms, sites of persistent septal deviation, techniques used to correct the deviation, and surgical results were reviewed. RESULTS: The mean interval between primary and revision surgery was 6.2 years. Forty-eight patients received revision septoplasty and 52 received revision septoplasty combined with rhinoplasty. Nasal obstruction was the most presenting symptom in almost all patients. The most common site of persistent septal deviation was middle septum (58%) followed by caudal septum (31%). Correcting techniques included further chondrotomy and excision of deviated portion in 76% and caudal batten graft in 39%. Rhinoscopic and endoscopic exams showed straight septum in 97% and 92 patients had subjective symptom improvement postoperatively. CONCLUSION: Middle septum and caudal septum were common sites of persistent deviation. Proper chondrotomy with excision of deviated middle septum and correction of the caudal deviation with batten graft are key maneuvers to treat persistent deviation.
Follow-Up Studies ; Humans ; Nasal Obstruction ; Rhinoplasty ; Transplants

BACKGROUND: There has been reports which suggest that non-specific symptom of patients with mitral valve prolapse is associated with autonomic dysfunction. METHODS: To assess autonomic dysfunction of patients, we examined five cardiovascular reflex tests in 25 asymptomatic MVP patients(identified as MVP group), 25 symptomatic MVP patients(identified as MVP syndrome group) and 25 control group. RESULTS: In the five cardiovascular autonomic function tests, abnormalities of Valsalva ratio were detected in 1(4%) control group, 7(28%) MVP group, 9(36%) MVP syndrome group, heart rate response to deep breathing in 0(0%), 2(8%), 4(16%) respectively, immediate heart rate response to standing in 0(0%), 2(7.4%), 2(8%) respectively and in postural hypotension, there were no abnormal group. Abnormalities of blood pressure response to sustained handgrip were only detected in 2(8%) MVP syndrom group. According to the five categories of cardiovascular autonomic functon tests, normal in 24(96%) and early damage in 1(4%) were detected in control group. In the MVP group, normal 17(68%), early damage 6(24%) and definite damage 2(8%) were noted. In the MVP syndrome group, normal 9(36%), early damage 13(52%), definite damage 1(4%) and combined damage 2(8%) were detected. In case of heart rate response to deep breathing, we found significant differences between control and MVP syndrome group(p=0.043), and between MVP and MVP syndrome group(p=0.0043). In case of heart rate response to standing, between control and MVP syndrome group(p=0.0009), between MVP and MVP syndrome group(p=0.001), the differences were noted. In case of blood pressure response to standing, between control group and MVP group(p=0.0019), between MVP and MVP syndrome group(p=0.0075), we found significant differences. Resulting from our study, heart rate response to deep breathing and standing, blood pressure response to standing were of considerable value in assessing the autonomic dysfunction of patients with mitral valve proapse. CONCLUSION: We found autonomic dysfunction in addition to increased autonomic tone and responsiveness which have been already known previously in mitral valve prolapse. And autonomic dysfunction was more severe in symptomatic patients with mitral valve prolapse than asymptomatic ones.
Blood Pressure ; Heart Rate ; Humans ; Hypotension, Orthostatic ; Mitral Valve Prolapse* ; Mitral Valve* ; Reflex ; Respiration