Pfeiffer Syndrome is as rare as Apert syndrome in the Western population. This condition is very rare in the Asian population and has not been previously reported in Korea. The authors report with a review of literature the case of a newborn baby with Pfeiffer syndrome, manifested by bicoronal craniosynostosis, broad thumbs, and big toes. The infant also had bilateral syndactyly of the fingers and toes, mild proptosis, choanal hypoplasia and maxillary hypoplasia.
Korea ; Infant, Newborn ; Humans ; Female ; Acrocephalosyndactylia/*diagnosis/genetics/radiography/surgery

We experienced a complication of brachial plexus palsy secondary to operative position during thoracoscopic thoracic sympathectomies. His general health was excellent and no previous histories vulnerable to peripheral nerve systems were observed. The thoracic sympathectomies were done under general anesthesia. The patient was placed left lateral position with his right arm abduced 150o on padded arm board. An operation was lasted 2 hours and 30 minutes at this position because of severe right apical lung adhesion. The controlateral side was performed same procedure and lasted 20 minutes. After the patient recovered from the anesthesia, the patient had a complete paralysis of right arm. There was also slightly diminished sensation to pinprick on the arm and hand. Neurologic examination and EMG study revealed brachial plexus palsy. Nerve blocks and physiotherapy were performed to treat brachial plexus injuries. His motor functions were improved day by day and he was discharged with a complete range of motion against gravity on 14th. postoperation day. However, there were loss of muscle powers against some resistances and tingling sensations of fingertips. Two months later, he was recovered completely and there was no residual disabilities.
Anesthesia ; Anesthesia, General ; Arm ; Brachial Plexus* ; Gravitation ; Hand ; Humans ; Lung ; Nerve Block ; Neurologic Examination ; Paralysis* ; Peripheral Nerves ; Range of Motion, Articular ; Sensation ; Sympathectomy*

BACKGROUND: During coronary angiography, some electrocardiographic changes occured due to contrast media, which do life threatening influences. METHODS: We compared the electrocardiographic changes which were induced by injection of three radiopaque contrast media during selective coronary angiography in 49 patients with chest pain. One of the contrast media was high osmolar ionic(Urografin_76) and the another was low osmolar ionic(Hexabrix) and the last was non-ionic(Ioversol). Electrocardiograms were obtained before, during and after selective coronary angiography. RESULTS: The changes of S-T segment or T were decreased in non-ionic group rather than high osmolar or ionic group. And there was significant Q-Tc interval prolongation among all three groups except comparision of low osmolar ionic contrast dye and non-ionic contrast dye in left coronary angiography. CONCLUSION: Non-ionic low osmolar contrast media was safer than high osmolar or ionic contrast medial because of lesser change of Q-Tc interval during selective coronary angiography.
Chest Pain ; Contrast Media* ; Coronary Angiography* ; Diatrizoate Meglumine ; Electrocardiography* ; Humans ; Ioxaglic Acid ; Osmolar Concentration

A 44-year-old woman who comlpained of dizziness and generalized weakness was admitted. The hemoglobin was 6.6g/dL, hematocrit 25.5%, and serum ferritin 2.14 ng/mL Stool occult blood was positive and microcytic hypochromic anemia was found on periyheral blood smear. Gastroscopic examination showed about 2 x 1 cm sized hemispherical sebmucosal tumor on antrum. The patient underwent operatioh for confirmatory diagnosis and treatment. The final pathologic diagnosis of the resected lesion was hemangioma of stomach. Cavernous hemangioma of stomach is a rare disease.Mostly, it has a benign course clinically, but early diagnosis is important because massive hemorrhage and anemia by chronic blood loss can occur. We report a case of hemangioma of stomach with review of literature.
Adult ; Anemia ; Anemia, Hypochromic ; Diagnosis ; Dizziness ; Early Diagnosis ; Female ; Ferritins ; Hemangioma ; Hemangioma, Cavernous* ; Hematocrit ; Hemorrhage ; Humans ; Occult Blood ; Stomach*

Several predetermined concentrations of beta-amyloid peptide, (betaA) were administered to the rat cardiac myocyte cultures for three days to determine the effects of betaA. Stainings with congo red and crystal violet were used to evaluate the deposition of betaA in the cardiac myocytes and MTT assay was used to elucidate the cytotoxic effects of betaA by anlaysis of cell viability. Beating rates and morphological changes were investigated with inverted microscope and TEM was used to study the fine structures. Administration of 0.5 microgram/ml of betaA to cardiac myocytes induced the reduction of beating rate, however, it did neither affect the viability nor fine structures. No significant differences in cell viability or fine structures were noted in the experimental groups which were exposed to 5 microgram/ml or higher concentration of betaA. Deposition of betaA was confirmed in the cytoplasm of betaA treated cardiac myocytes with congo red and crystal violet amyloid stains. The viability of cardiac myocytes exposed to betaA was found to be reduced significantly (19%) compared to control cultures with the MTT assay. Cardiac myocytes treated with betaA presented a reduced cytoplasmic area that appeared very condensed under inverted microscope. Mitochondrial abnormalities in betaA treated cardiac myocytes included their significant enlargement, vacuolization, disorganization or paucity of cristae, paracrystalline inclusion, and accumulation of amorphous material in mitochondrial space. Mitochondrial abnormalities were present sometimes in betaA treated cardiac myocytes without disorganization of myofibils or degeneration of other cell organelles. To understand the mechanism involved in amyloid deposit and its role in pathogenesis of the diseases such as Alzheimer and inclusion body myositis (IBM), a need for in vitro model is imperative. This model of betaA treated cultured cardiac myocytes represent a amyloidosis model, and it offers several advantages for future studies of betaA to help elucidate the pathogenesis of amyloid diseases. For example, cardiac myocytes can be easily accessible, and since cardiac myocytes can be cultured for quite a long time, it is possible to study morphological and physiological changes consequent to amyloid deposits.
Amyloid ; Amyloidosis ; Animals ; Cell Survival ; Coloring Agents ; Congo Red ; Cytoplasm ; Gentian Violet ; Myocytes, Cardiac* ; Myositis, Inclusion Body ; Organelles ; Plaque, Amyloid ; Rats

Pituicytoma is a rare solid benign tumor of the sellar and/or suprasellar region originating from the pituicytes of the neurohypophysis or infundibulum, which is not differentiated from a pituitary adenoma that is diagnosed mostly in the sellar and/or suprasellar region. In addition, cystic tumors are very rare and have not been reported due to their solid and hypervascular natures. A 33-year-old man presented with a chronic headache which exacerbated recently. MRI was performed and revealed a cystic tumor in the sellar and suprasellar regions with a small parenchymal island in the cyst compressing the optic chiasm. The endoscopic endonasal transsphenoidal approach was used to remove the tumor. Immunohistochemical staining was positive for thyroid transcription factor 1, S-100 protein, and glial fibrillary acidic protein. The pituicytoma was diagnosed based on histologic findings. The authors review herein the literature on clinical presentation, diagnosis, surgical management, and outcome.

Acute thrombosis of the portal and superior mesenteric vein (SMV) due to inflammation of abdominal organs is a rare condition, but delayed diagnosis causes severe problems and serious long term complications. Therefore the early diagnosis and adequate management of the underlying disease and thrombus is very important. Here a case of an 84-year-old man with portal vein and SMV thrombosis on Doppler ultrasonography and computed tomography (CT) after cholecystectomy and choledochostomy by the 10th day is reported. The patient's condition improved without complication after the treatments with an anticoagulant regimen and antimicrobials. In the follow up, there was no thrombus on the CT or sign of a recurrent disease.
Aged, 80 and over ; Cholangitis ; Cholecystectomy* ; Choledochostomy* ; Delayed Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Humans ; Inflammation ; Mesenteric Veins* ; Portal Vein* ; Thrombosis* ; Ultrasonography, Doppler ; Venous Thrombosis

PURPOSE: Although it is well recognized that SCLC is a chemo and radiosensitive tumor, only fraction of treated patients have a complete remission, fewer still have durable remissions. This study was performed to evaluate the clinical effects of IEP chemotherapy in patients with SCLC. MATERIALS AND METHODS: Patients with histologically proven SCLC who has measurable disease and previously untreated, were eligible. Treatment consisted of ifosfamide 1000 mg/m2 iv infusion for 1 hour on days 1~5 with mesna uroprotection; epirubicin 60 mg/m2 iv on day 1; and cisplatin 20 mg/m2 iv infusion on days 1~5 with hydration; repeated treatment every 4 weeks RESULTS: Twenty four patients(20 males, 4 females) were eligible for response to IEP chemotherapy. The two patients were excluded because one died before evaluating response to chemotherapy and the other had brain metastasis. The median age was 61(range 34-74). Fifteen patients had a limited disease(LD), nine patients had a extensive disease(ED). The overall response rate was 86.4%(CR 36.4%, PR 50%). In LD, response rate was 86.7%(CR 46.7%) and in ED, response rate was 85.7%(CR 14.3%). The median overall survival time was 43.5 weeks. The median survival time of LD and ED was 46.5 weeks and 43.5 weeks respectively. The median time to progression was 20 weeks in responders. The toxicity was moderate. One toxic death was observed. Grade 1 or 2 non-hematologic toxicities consisted of alopecia, nausea and vomiting in all cases, peripheral neuropathy in 3, hematuria in 2, mucositis in 11, and fever/infection in 6. Hematologic toxic effects included leukopenia(> or =grade.3, 16.5%), anemia(> or =grade 3, 1%), and thrombocytopenia(> or =grade 3, 6.8%). CONCLUSIONS: These results suggest that IEP chemotherapy may be useful as a treatment strategy in small cell lung cancer, but its efficacy is equivalent. The phase III study should be needed.
Alopecia ; Brain ; Cisplatin ; Drug Therapy ; Epirubicin* ; Hematuria ; Humans ; Ifosfamide* ; Male ; Mesna ; Mucositis ; Nausea ; Neoplasm Metastasis ; Peripheral Nervous System Diseases ; Small Cell Lung Carcinoma* ; Vomiting

No abstract available.
Female ; Humans ; Ovarian Cysts* ; Ovary*

PURPOSE: Endovascular therapy (ET) for peripheral arterial occlusive disease has increased dramatically in the past decade. ET is currently being performed by different specialists with available resources, with some of these resources being far superior to others, yet there have been no studies comparing the interventional outcomes according to the varying resources. The aim of this study was to analyze the outcomes of ET for superficial femoral artery (SFA) atherosclerosis using a mobile C-arm, in comparison to a historical control group. METHODS: Between March 2009 and December 2010, ET for SFA atherosclerosis was performed in 54 limbs from 47 patients using a mobile C-arm in the operation theater (mobile group). In contrast, a historical group for comparison consisted of 60 patients, for whom ET for SFA atherosclerosis was performed in 76 limbs using a fixed C-arm in the angiographic suite, between July 2003 and May 2008 (fixed group). The outcomes of ET for both groups were retrospectively analyzed by a medical chart review. RESULTS: There was no statistically significant difference in gender, age, risk factors, Trans-Atlantic Inter-Society Consensus (TASC) classification, intervention type, and postoperative blood creatinine levels between the two groups. However, procedural time was statistically higher in the mobile group. Patency rates at 1 year were 68.3% and 68.1% in the fixed and mobile group, respectively, which was not statistically significant. Subgroup analysis of 1 year patency rates for TASC A, B lesions and TASC C, D lesions were also similar. CONCLUSION: ET using the mobile C-arm in the operating theater is as effective as using the fixed C-arm, in the treatment of SFA atherosclerosis in terms of the technical success, patency, and early postoperative outcomes.
Arterial Occlusive Diseases ; Atherosclerosis ; Consensus ; Creatinine ; Endovascular Procedures ; Extremities ; Femoral Artery ; Humans ; Peripheral Arterial Disease ; Retrospective Studies ; Risk Factors ; Specialization