Acquired hemophilia A (AHA) is a rare disease where typically coagulation factor VIII is inhibited by autoantibodies. It occurs in patients with no personal or familial history of bleeding. In this case study a 61-year-old male presented with a huge psoas hematoma. He had no history of bleeding disorders. He was initially diagnosed with delayed traumatic hematoma. Despite conservative and surgical treatments, coagulopathy was not resolved and postoperative bleeding continued. Consequently, coagulation factor tests were performed and showed reduced activity of factor VIII (2.7%). In addition, factor VIII inhibitor was detected. The patient was diagnosed with AHA and administered recombinant factor VIII for 3 days which resulted in the cessation of bleeding. AHA can lead to a life-threatening hemorrhage, and needs to be considered in differential diagnoses in any patients presenting with unexplained and repeated bleeding, where there is no personal or familial history of bleeding disorders.
Autoantibodies ; Blood Coagulation Disorders ; Blood Coagulation Factors ; Diagnosis, Differential ; Factor VIII ; Hematoma ; Hemophilia A ; Hemorrhage ; Humans ; Male ; Middle Aged ; Rare Diseases

BACKGROUND: Minimally invasive surgery is currently popular, but this has been applied very sparingly to cardiac surgery because of some limitations. Our study evaluated the safety and efficacy of atrial septal defect (ASD) closure through a video-assisted mini-thoracotomy. MATERIAL AND METHOD: Fifteen patients were analyzed. Their mean age was 31+/-6 years. The mean ASD size was 24+/-5 mm and there were 3 cases of significant tricuspid regurgitation. The working window was made through the right 4th intercostal space via a 4~5 cm inframammary skin incision. CPB was conducted with performing peripheral cannulation. After cardioplegic arrest, the ASDs were closed with a patch (n=11) or direct sutures (n=4), and the procedures were assisted by using a thoracoscope. There were 3 cases of tricuspid repair and 1 case of mitral valve repair. The mean CPB time and aortic occlusion time were 160+/-47 and 70+/-26 minutes, respectively. RESULT: There was no mortality, but there were 3 minor complications (one pneumothorax, one wound dehiscence and one arrhythmia). The mean hospital stay was 5.9+/-1.8 days. The mean follow-up duration was 10.7+/-6.4 months. The follow-up echocardiogram noted no residual ASD or significant tricuspid regurgitation. Three patients suffered from pain or numbness. CONCLUSION: This study showed satisfactory clinical and cosmetic results. Although the operative time is still too long, more experience and specialized equipment would make this technique a good option for treating ASD.
Catheterization ; Cosmetics ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Humans ; Hypesthesia ; Length of Stay ; Mitral Valve ; Operative Time ; Pneumothorax ; Skin ; Sutures ; Thoracic Surgery ; Thoracoscopes ; Thoracoscopy ; Tricuspid Valve Insufficiency

Moyamoya disease is an occlusive cerebrovascular disease characterized by stenosis or occlusion of main cerebral arteries. The etiology of moyamoya disease is still unclear, but the frequent familial occurrence suggests that some genetic factors may contribute to its etiology. In Korea, though the moyamoya disease is not infrequent, its familial occurrence has not been reported yet. We report four female patients(in two families) of moyamoya disease with its familial occurrence. Two patients are siblings and the other two are mother and daughter. The ages of symptom onset were thirties in three patients and sixties in one patient. Three patients had intracerebral hemorrhage including intraventricular hemorrhage(recurrent in two). We got cerebral angiography in three patients and magnetic resonance angiography (MRA) in one patient. Full laboratory evaluations were done in three patients, including HLA typing in two patients(mother and daughter). All the patients did not have any hereditary disease or any other disease which are known to cause moyamoya-like changes. Considering the reported familial incidence in Japan, careful search for the family members would reveal much more familial cases in Korea also.
Cerebral Angiography ; Cerebral Arteries ; Cerebral Hemorrhage ; Constriction, Pathologic ; Female ; Genetic Diseases, Inborn ; Histocompatibility Testing ; Humans ; Incidence ; Japan ; Korea ; Magnetic Resonance Angiography ; Mothers ; Moyamoya Disease* ; Nuclear Family ; Siblings

PURPOSE: To describe clinical and MRI findings of growth hormone-secreting pituitary adenoma, to determine ifthere are any characteristic MRI findings different from those of other pituitary adenomas, to evaluate the relationship between tumor size and serum growth hormone level, and to assess the results of immunohistochemical study. MATERIALS AND METHODS: We retrospectively analysed clinical and MRI findings of 29 patients with growth hormone-secreting pituitary adenoma confirmed by serum growth hormone level and surgery. We also evaluated the relationship between the tumor volume and serum growth hormone level, and the results of immunohistochemical study. Coronal and sagittal T1-weighted MR images in all patients and gadolinium enhanced T1-weighted MR images in28 patients were obtained with 2.0 T(24 cases) and 0.5 T(5 cases) MR imagers. The images were analyzed in terms oftumor size, signal intensity, degree of contrast enhancement, extent of tumor growth and the presence or absenceof cystic change, hemorrhage and calcification. RESULTS: Clinical manifestations included facial feature changeand soft tissue swelling of hands and feet(n=29), headache(n=12), impaired visual acuity(n=9), symptoms of hyperprolactinemia(n=8), visual field defect(n=5), and others(n=6). On MR images, all of the 29 cases were seen tobe macroadenomas and the size of the tumors averaged 2.2cm(1-5.2 cm). Supra- and infrasellar extensions were seenin 21 and 22 patients, respectively. Cavernous sinus invasion was noted in seven, and in one this was bilateral.Signal intensity was isointense with cortical grey matter in 26 cases(90%). Cystic change or necrosis was seen ineight cases(28%), hemorrhage in four(14%), and calcification in two(7%). After enhancement, most(25/28) of the tumors enhaned less than mormal pituitary in degree. There was no correlation between serum growth hormone level and tumor size. Immunohistochemical study showed positive growth hormone-secreting cells in only 69%(11/16). CONCLUSION: Clinical findings in patients with growth hormone-secreting pituitary adenomas were various and included acromegaly, headache, visual impairment, and symptoms of hyperprolactinemia, for example. On T1-weightedMR images, all tumor were macroadenoma, and there were no characteristic findings different from those of other pituitary tumors. The volume of a tumor did not correlate with serum hormone level. Immunohistochemical study showed positive growth hormone-secreting cells in a limited number of cases.
Acromegaly ; Cavernous Sinus ; Growth Hormone ; Growth Hormone-Secreting Pituitary Adenoma* ; Hand ; Headache ; Hemorrhage ; Humans ; Hyperprolactinemia ; Magnetic Resonance Imaging* ; Necrosis ; Pituitary Neoplasms ; Retrospective Studies ; Tumor Burden ; Vision Disorders ; Visual Fields

PURPOSE: To assess the utility of spin-echo magnetic resonance(MR) imaging in the evaluation of thoracic and abdominal aortic aneurysm. MATERIALS AND METHODS: The spin-echo MR images of 27 aortic aneurysms in 22 patients were analyzed and correlated with angiography and/or operative findings retrospectively. Evaluations included location, type, and maximum diameter of the aneuyusm, mural thrombus, major branch involvement, and relationship with adjacent organ. RESULTS: The location of aneurysms was ascending thoracic aorta in seven cases, ascending thoracic aorta and aortic arch in one, descending thoracic aorta in six, thoracoabdominal aorta in three, and abdominal aorta in eight. Nineteen were fusiform, and eight were saccular. The mean of maximum diameters of the aneurysms was 7.9cm (4-10cm) on MR and 7.3cm (3-10cm) on angiography. Mural thrombus were noted in 13 cases on MR imaging and seven cases on angiograhpy. Angiography also underestimated the amount of mural thrombus. Eight cases involved major aortic branches. Although MR imaging and angiography were equal in the assessment of major abdominal aortic branches, MR imaging could not clearly demonstrate arch vessels, especially left subclavian artery, in aortic arch aneurysms. Among seven ascending thoracic aneurysms, six had aortic regurgitation. MR imaging showed left ventricular enlargement in all six cases. There was pericardial effusion in four cases which were noted only on MR imaging. MR imaging demonstrated hydronephrosis and renal atrophy in two cases of abdominal aortic aneurysms respectively. CONCLUSION: In the assessment of size of the aneurysm, mural thrombus, and relation with adjacent organs, MR imaging was better than angiography. MR and anglographic findings were equal in the assessment of the location and type of the aneurysm. Angiography was better than MR imaging in the assessment of major branch involvement, especially left subclavian artery.
Aneurysm ; Angiography ; Aorta ; Aorta, Abdominal ; Aorta, Thoracic ; Aortic Aneurysm ; Aortic Aneurysm, Abdominal* ; Aortic Valve Insufficiency ; Atrophy ; Humans ; Hydronephrosis ; Magnetic Resonance Imaging ; Pericardial Effusion ; Retrospective Studies ; Subclavian Artery ; Thrombosis

The most important factor in the effective chemotherapy of the bone and joint tuberculosls is based on the use of sensitive antituberculous drugs. Failure of chemotherapy to control the bone and joint tuberculosis may be caused by drug resistance. For twenty years from 1960 to 1979, we observed and analysed for bacteriological study in terms of primary and acquired resistances in 120 bone and joint tuberculosis patients who yielded positive on culture for AFB. The results were obtained as follows: 1. Cultures for AFB with 412 surgical specimens taken from the tuberculous focl of bone and joints have been carrled out, Out of the 412 patients, 120 cultures for AFB were positive (29.1%) and no significant difference in all years. The cases were then grouped in 5 years for convenience. 2. Of the 120 culture-positive patients, 40.8% had resistance to 1 or more drugs. 3. 23.3% had drug resistance to 1 drug only, 6.7% to 2 drugs and 10.8% to all 3 drugs respectively. 4. Resistant cases to INH, PAS, SM were 25 (20.8%), 32 (26.7%) and 26 (21.7%) respectively. 5. Drug resistances to 1 or more drugs by involved site were 38.8% In spine, 45.8% in hip and 23.3% in knee. 6. Of the 61 patients with no history of previous chemotherapy, 16 (26.2%) had resistance to 1 or more drugs, while ot the 59 patients with history of prevlous chemotherapy, 33 (55.9%) had resistance. 7. Drug resistance was found to be proportionally Increased to the duration of previous chemotherapy, 8. Drug resistance was mainly related to previous history of chemotherapy rather than duration of illness. 9. Obtalning drug resistance to all 3 drugs tends to be increased every-year regardless of prevlous chemotherapy.
Drug Resistance ; Drug Therapy ; Hip ; Humans ; Joints ; Knee ; Spine ; Tuberculosis ; Tuberculosis, Osteoarticular

The osteochondroma is the most common benign bone tumor, of which the typical form is a cartilage-capped bony projection, far often found at the metaphysis of the long tubular bones, and may arise from any bone preformed in cartilage. To our knowledge, however, osteochondroma of accessory bones has not been reported, but we experienced a rare case of osteochondroma located in the position normally occupied by a tarsal accessory bone(Os Tibiale Externum). The mass was treated by excisioa and it discloeed typical microscopic findings of ostechondroma as shown in the pictures.
Cartilage ; Osteochondroma