Congenital pseudarthrosis is frequently associated with various stigmata of neurofibromatosis, particularly Cafe-Au-Lait spots and,subcutaneous nodules and in these patients, it is noteworthy that the treatment is very difficult even with extensive operative approach. Also it is interesting to find many controversies on etiological relation between neurofibroma and pseudarthrosis, per se and even the existance of nerve element in and around the tissue of pseudarthrosis. A thirteen years old female patient with definite neurofibromatosis associated with congenital tibial pseudarthrosis treated by intramedullary nailing and massive bone graft is reported.
Cafe-au-Lait Spots ; Christianity ; Female ; Fracture Fixation, Intramedullary ; Humans ; Neurofibroma ; Neurofibromatoses ; Pseudarthrosis ; Transplants

Osteopoikilosis is a familial, rare condition only discovered on radiological examination for a coincident disease. It is characterized by numerous nodules of dense bone in the spongiosa of the metaphysis or in the epiphysial nucleus. They are particularly seen in the small bones of the hands and feet and at the ends of the large bones of the extremities. Most of the bones have been reported as being affected but the skull is rarely involved. This condition is usually symptomless and no specific treatment is necessary. A case of osteopoikilosis is presented with a brief review of literatures.
Extremities ; Foot ; Hand ; Osteopoikilosis ; Skull

Hemimasticatory spasm is a rare neurologic disorder characterized by unilateral, paroxysmal involuntary contraction of the masticatory muscles. It can be confused with other unilateral facial spasms. In this report, we present two patients with hemimasticatory spasm who were initially misdiagnosed with other disorders. The first patient was a 54-year-old man with a 9-year history of right facial spasm. He underwent microvascular decompression, without benefit, at another hospital with the diagnosis of hemifacial spasm two years prior to presenting at our hospital.On examination, right masseter and temporalis muscles had irregular contractions with twitches and prolonged spasms. His brain magnetic resonance imaging was normal.He showed a good response to carbamazepine. The second case was a 60-year-old man presented with a 10-year history of jaw-closing movement. Previous treatment for temporomandibular joint disorder was ineffective. He was previously diagnosed as oromandibular dystonia at another hospital, and biperiden and diazepam treatment resulted in slight improvement. There was tonic contraction and hypertrophy of the left masseter. He improved with carbamazepine. These cases illustrate the importance of hemimasticatory spasm as differential diagnosis, and the good response to carbamazepine.
Nervous System Diseases

Foreatiers disease is a peculiar type of ankylosing hyperostosis of the spine characterized by ossification of the anterior and right lateral aspects of the vertebral column, particularly in the thoracic region. The clinical, pathological and reontgenographic features of the disorder allowed its differentiation from other spinal diseases including ankylosing spordylitis and osteoarthritis. A case of Forestiers disease is presented with brief review of literatures.
Hyperostosis ; Hyperostosis, Diffuse Idiopathic Skeletal ; Osteoarthritis ; Spinal Diseases ; Spine

BACKGROUND: Aprotinin, which is a nonspecific serine protease inhibitor, has an antiinflammatory and thrombogenic effect. However, it has an antithrombogenic effect during the cardiopulmonary bypass. This study was performed to evaluated the effects of aprotinin on the activated clotting time(ACT) and the total amount of the heparin used during the cardiopulmonary bypass. MATERIAL AND METHOD: From December 1998 to November 1999, 82 consecutive patients electively underwent open heart surgery at Gachon medical school. The patients were older than 18 years. Eighty two patients were classified into a control group(group C, n=36) and a aprotinin-treated group(group A, n=46). Body weight, height, body surface area(BSA), pump time(PT), aortic cross clamping time(ACCT), and body temperature(BT) were determined. Total amount of heparin and protamine during the CPB were also measured. ACT was determined before heparin administration, at 20, 40 and 60 minutes after heparin administration, and after protamine administration. RESULT: No significant differences were noted in either group in body weight, height, BSA, BT, and the total amoun of heparin and protamine. Group A demonstrated a significant(p<0.05) increase in age, PT, ACCT, and ACT at 20, 40, and 60 minutes after heparin administration. CONCLUSION: In summary, the use of aprotinin prime resulted in an increase in ACT. The total amount of heparin in aproinin-treated patient was similar to that of the control group in spite of having the prolonged pump time. Therefore aprotinin may reduce the requirement of heparin.
Aprotinin* ; Body Height ; Body Weight ; Cardiopulmonary Bypass ; Constriction ; Heart* ; Heparin* ; Humans ; Schools, Medical ; Serine Proteases ; Thoracic Surgery*

Renal blood flow was measured by single injection technique of HippuranI13 in 13 patients with chronic glomerulonephritis and 11 control subjects. There was a significant decrease in renal blood flow in chronic glomerulonephritis particulary in those with elevated blood urea nitrogen. Renal blood flow was inversely proportionate to renal vascular resistance but no correlation was noted between renal blood flow and creatinine clearance.
Blood Urea Nitrogen ; Creatinine ; Glomerulonephritis* ; Humans ; Renal Circulation* ; Vascular Resistance

In clinical experience, patients complaining of knee joint pain which is not belong to the category of well documented causative diseases, such as rupture of menisci, collateral ligaments and so forth, have been encountered, Since the report of Mayedas chorda cavi articularis genu in 1918, internal derangement of knee due to rupture of plica synovialis has been presented from time to time, Numerous nomenclatures have been offered and various histologic and clinicopathologic features have been observed. Majority of uncomplicated plica synovialis are asymptomatic and respond well to conservative treatment, whereas in complicated cases operation is often inevitable. Here, a symptomatic case, due to rupture of plica synovialis mediopatellaris which was hard to differentiate from rupture of meniscus in clinical aspect and on operation field is reported with available references.
Collateral Ligaments ; Humans ; Knee Joint ; Knee ; Rupture

PURPOSE: The CD5 molecules are pan-T cell antigens and are found on a minor subpopulation of B cells. CD5 antigens are involed in an intracellular signal transduction as well as in an intercellular signal transduction between CDS+ T cell/CD72+ B cell by CD5/CD72 interaction. CD5 antigens are known to be participated in classic immune reactions and in this study CDS mRNA expressions by lymphocytes were examined in allergic patients controls, acute febrile infectious disease controls and normal controls to elucidate the possibility of CDS involvement in allergic immune reactions. METHODS: Fifteen allergic patients, ten patients of acute febrile infectious disease patients and ten normal controls were studied. Venous blood was drawn and mononuclear cells were separated. T cells and B cells were separated using immunomagnetic beads. Total RNA was extracted and RT-PCR (reverse transcriptase - polymerase chain reaction) was done to detect CDS antigen mRNA expression. RESULTS: 1) CDS mRNA overexpressions were detected in allergic patient controls as compared to that in acute febrile infectious controls. CDS mRNA was not detected in normal controls. Semiquantitative CD5 mRNA expressions were measured as relative expressions of CD5 to GAPDH. Relative quantities of CD5 mRNA expressions were 90.656.24% in allergic patient controls and 23.76+3.58% in acute febrile infectious patients. CONCLUSIONS: CDS mRNA overexpression is a characteristic phenomenon in allergic immune reactions. From these result, CD5/CD72 pathway might be the preference immune mechanism in allergic immune reaction and the further study for the exact mechanism of CDS involvement in allergic immune reactions may be necessary
Antigens, CD5 ; B-Lymphocytes ; Communicable Diseases ; DNA-Directed RNA Polymerases ; Humans ; Hypersensitivity ; Lymphocytes* ; RNA ; RNA, Messenger* ; Signal Transduction ; T-Lymphocytes

No abstract available.
Child* ; Humans ; Infant, Newborn* ; Plasma* ; Renin*

BACKGROUND: To evaluate the clinical and functional outcomes of arthroscopic-assisted reduction and percutaneous screw fixation for glenoid fractures with scapular extension, and investigate the radiologic and clinical benefits from the results. METHODS: We evaluated patients treated with arthroscopic-assisted reduction and percutaneous screw fixation for glenoid fractures with scapular extension from November 2008 to September 2015. Fractures with displacement exceeding one-fourth of the anterior-articular surface or more than one-third of the posterior-articular surface in radiographic images were treated by surgery. Clinical assessment was conducted based on range of motion, Rowe score, and Constant score of injured arm and uninjured arm at last follow-up. RESULTS: Fifteen patients with Ideberg classification grade III, IV, and V glenoid fracture who underwent arthroscopic-assisted reduction using percutaneous screw fixation were retrospectively enrolled. There were no differences in clinical outcomes at final follow-up compared to uninjured arm. Bone union was seen in all cases within five months, and the average time to bone union was 15.2 weeks. Ankylosis in one case was observed as a postoperative complication, but the symptoms improved in response to physical therapy for six months. There was no failure of fixation and neurovascular complication. CONCLUSIONS: We identified acceptable results upon radiological and clinical assessment for the arthroscopic-assisted reduction and percutaneous fixation. For this reason, we believe the method is favorable for the treatment of Ideberg type III, IV, and V glenoid fractures. Restoration of the articular surface is considered to be more important than reduction of fractures reduction of the scapula body.
Ankylosis ; Arm ; Arthroscopy ; Classification ; Follow-Up Studies ; Fracture Fixation ; Glenoid Cavity ; Humans ; Methods ; Postoperative Complications ; Range of Motion, Articular ; Retrospective Studies ; Scapula