No abstract available.
Iron*

Rheumatoid arthritis is a chronic inflammatory systemic disease of young or middle aged adults, characterized by destructive and proliferative changes in the synovial membrane, periarticular structures, skeletal muscle and perineural sheath. Eventually, the joints are destroyed, ankylosed and deformed. Therfore, the aim of treatment is to keep the inflammatory process at a minimum, thereby preserving the joint motion, maintaining the health of muscle supplying motor power about the joint and preventing secondary joint stiffness and deformity. Surgical treatment in rheumatoid arthritis has progressed and there have been advances in the relief of pain and increase in the range of motion. Among them the results of total knee arthroplasty (TKA) have improved steadily during the past decade due to refinements in design, fixation, and surgical technique. At orthopedic department of seoul national university hospital, we performed 31 total knee replacement in 18 patients who had suffered from rheumatoid arthritis during the period from Aug. 1982 to Dec. 1988. Following results were obtained. 1. Knee score increased from 37.8 to 76.9. 2. Tibio-femoral angle was corrected from 0.9° valgus to 5.3° valgus. 3. Conplications were peroneal nerve palsy in 3 knees, instability in 1 knee, tuberculous arthritis in 1 knee. 4. In 25 out of 31 knees, good functional results were obtained.
Adult ; Arthritis ; Arthritis, Rheumatoid ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Congenital Abnormalities ; Humans ; Joints ; Knee ; Middle Aged ; Muscle, Skeletal ; Orthopedics ; Paralysis ; Peroneal Nerve ; Range of Motion, Articular ; Seoul ; Synovial Membrane

Isolated ACTH deficiency is a uncommon disorder causing secondary adrenocortical insufficiency. Less than 200 cases have been reported in the world. The major clinical manifestations are hypoglycemia, weight loss, hypotension, anemia, weakness, nausea, inability to excrete water load, and hyponatremia. A 56-year-old male was admitted because of weakness, fatigue, nausea and vomiting. He was pallor and not associated with hyperpigmentation. The basal plasma ACTH and cortisol levels were 8.30 pg/ml and 0.6 ug/dl. The serum cortisol did not response to rapid ACTH stimulation test. On combined pituitary stimulation test, the cortisol did not response to insulin-induced hypoglycemia. Other anterior pituitary hormones showed normal responses except elevated prolactin level. Six months after glucocorticoid replacement therapy, the elevated basal prolactin level returned to normal. Brain MRI did not show any anatomic abnormalities of the sellar and suprasella area. We report a case of isolated ACTH deficiency accompanied by hyperprolactineia, which respond to glucocorticoid replacement therapy.
Adrenal Insufficiency ; Adrenocorticotropic Hormone* ; Anemia ; Brain ; Fatigue ; Humans ; Hydrocortisone ; Hyperpigmentation ; Hyperprolactinemia* ; Hypoglycemia ; Hyponatremia ; Hypotension ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Nausea ; Pallor ; Pituitary Hormones, Anterior ; Plasma ; Prolactin ; Vomiting ; Water ; Weight Loss

PURPOSE: To evaluate the efficacy of modified Anderson procedure in infantile nystagmus with face turn less than 30 degrees. METHODS: This study was a retrospective review of the medical records of 13 consecutive patients who underwent a modified Anderson procedure for abnormal head position in infantile nystagmus at Pusan National University hospital from February 2002 to March 2017. We compared best-corrected visual acuity (logarithm of minimal angle of resolution, logMAR), refraction, degree of face turn, ocular motility, and angle of deviation preoperatively and at 1 week postoperatively, 6 months postoperatively, and the final visit. Recession of the yoke rectus muscles responsible for the slow phase of nystagmus was performed, depending on the direction of face turn; the medial rectus muscle of the eye to which the head was turned was recessed 10 mm, whereas the lateral rectus muscle of the contralateral eye was recessed 12 mm. RESULTS: The mean age of the patients was 8.9 (2–25) years, and the mean follow-up period after surgery was 51.3 (6–183) months. Twelve patients (92.3%) had no residual head posture or <10° at 6 months postoperatively, and three patients (23.1%) achieved this outcome with an additional operation. The mean degrees of face turn were 22.30° before surgery and 3.85° post-operatively (p = 0.001). Best-corrected visual acuity (logMAR) was 0.41 in the better eye and 0.50 in the worse eye before surgery; this improved to 0.34 and 0.45 at 6 months postoperatively (p = 0.068 and p = 0.228, respectively). Despite the large recessions involved, only one patient showed mild limitation of abduction after surgery. CONCLUSIONS: The modified Anderson procedure may be effective for correcting abnormal head position in infantile nystagmus with face turn less than 30 degrees; patients were not overcorrected.
Busan ; Follow-Up Studies ; Head ; Humans ; Medical Records ; Muscles ; Posture ; Retrospective Studies ; Visual Acuity

PURPOSE: To report two cases of orbital apex syndrome caused by blunt orbital trauma without structural damage of the orbit. CASE SUMMARY: (Case 1) A 50-year-old male came to our clinic complaining of visual loss after blunt orbital trauma by a metal bar. The best-corrected visual acuity was no light perception and light reflex was not observed in the affected eye. He also presented with complete ptosis and ophthalmoplegia with relative sparing of adduction and depression. High signal intensity of the orbital soft tissue including the optic nerve sheath was revealed using a T2-weighted image in magnetic resonance imaging. After starting steroid pulse therapy, his visual acuity improved to counting fingers on the third day. Ocular movement and levator function recovered to the normal range while visual acuity remained counting fingers. (Case 2) A 64-year-old female presented with complete ptosis after trauma to her right eyeball. The best-corrected visual acuity was 20/25 in the right eye. Complete ptosis and ophthalmoplegia with relative sparing of abduction and depression in the right eye were observed at the initial presentation. Magnetic resonance images showed enhancement of the right periphery optic nerve and distal rectus muscle. Two months after undergoing steroid pulse therapy, levator function and ocular movement recovered completely, and visual acuity improved to 20/20. CONCLUSIONS: The orbital apex syndrome caused by blunt orbital trauma showed good response to steroid pulse therapy. Steroid treatments may therefore be considered for the treatment of traumatic orbital apex syndrome.
Depression ; Female ; Fingers ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Ophthalmoplegia ; Optic Nerve ; Orbit ; Reference Values ; Reflex ; Visual Acuity

BACKGROUND: Lung cancer is one of the leading causes of cancer-related deaths throughout the world. The gene tumor protein 53 (TP53) is frequently mutated in cases of lung cancer. This study was performed to investigate the frequencies and types of mutations in the TP53 gene in Korean patients with lung cancer. METHODS: We obtained tissue samples from 80 lung cancer patients and synthesized TP53 cDNA by using RNA isolated from these tissues by performing reverse transcriptase polymerase chain reaction. Hybridization and denaturing high-performance liquid chromatography were performed to identify the TP53 gene mutations, and then, the mutations were validated by direct sequencing. RESULTS: Forty mutations out of the 80 patients (50.0%) were noted in the TP53 gene. The frequencies of TP53 gene mutation for different cancer types, namely, squamous cell carcinoma, adenocarcinoma, and small cell carcinoma were 61.1%, 27.3%, and 26.7%, respectively. The mutation frequencies in the different regions of the gene were 10.0% for exon 4, 35.0% for exon 5, 12.5% for exon 6, 22.5% for exon 7, 17.5% for exon 8, and 2.5% for exon 9. The frequently mutated positions were codon 179 in exon 5, codons 202 and 220 in exon 6, and codons 266 and 273 in exon 8. CONCLUSIONS: Exon 5 was the most frequently mutated region in the TP53 gene. Compared to the patients with the other types of cancers, patients with squamous cell carcinoma showed a higher frequency of TP53 mutation. Codon 179 was the most frequently mutated codon in the TP53 gene.
Adenocarcinoma ; Carcinoma, Small Cell ; Carcinoma, Squamous Cell ; Chimera ; Chromatography, Liquid ; Codon ; DNA, Complementary ; Exons ; Genes, p53 ; Humans ; Lung ; Lung Neoplasms ; Mutation Rate ; Reverse Transcriptase Polymerase Chain Reaction ; RNA

PURPOSE: This study was performed in order to evaluate the clinical characteristics, effect of treatment and prognosis in patients with testicular tumors. MATERIALS AND METHODS: We retrospectively reviewed 42 patients with testicular tumor treated at our hospital. We analyzed the pathologic classification, clinical stage, preoperative tumor markers, treatment methods, clinical characteristics, and prognosis. RESULTS: The histologic type was seminoma in 11, teratoma in 10, yolk sac tumor in 5, embryonal cell carcinoma in 4, mixed type in 8 and secondary neoplasm in 4. Clinically, 31 patients were stage I, 2 stage IIa, 2 stage IIb, 1 stage IIc, 6 stage IV. Following orchiectomy, 22 patients underwent surveillance, 12 chemotherapy, 4 radiation therapy, 1 retroperitoneal lymph node dissection (RPLND), 2 radiation plus chemotherapy, and 1 radiation plus chemotherapy and RPLND. The 10-year survival rate was 90.9% in the cases of seminoma. Similarly, the 10-year survival rate was 96.0% in the cases of non-seminomatous germ cell tumors (NSGCT). CONCLUSION: In our study, patients with testicular tumor revealed an excellent survival rate. Testicular tumors are solid tumors that have a possibility of complete remission by additional chemotherapy or radiation following radical surgery. Therefore, early detection and aggressive treatment were mandatory.
Classification ; Drug Therapy ; Endodermal Sinus Tumor ; Humans ; Lymph Node Excision ; Neoplasms, Germ Cell and Embryonal ; Orchiectomy ; Prognosis ; Retrospective Studies ; Seminoma ; Survival Rate ; Teratoma ; Testicular Neoplasms ; Testis* ; Biomarkers, Tumor

No abstract available.
Holoprosencephaly*

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.
Tomography, X-Ray Computed ; Sarcoma/*pathology ; S100 Proteins/biosynthesis ; Radiography, Thoracic ; Pancreatic Neoplasms/*pathology ; Male ; Langerhans Cells/*pathology ; Immunohistochemistry ; Humans ; Histiocytosis, Langerhans-Cell/diagnosis/*pathology ; Gene Expression Regulation, Neoplastic ; Antigens, Differentiation, Myelomonocytic/biosynthesis ; Antigens, CD1/biosynthesis ; Antigens, CD/biosynthesis ; Adult

Aspirin intolerance is defined as acute urticaria-angioedema, bronchospasm, severe rhinitis, or shock occuring within three hours of aspirin ingestion. Aspirin intolerance occurs most frequently in individuals with chronic urticaria, asthma, chronic rhinitis, and normal individual in order of decreasing frequency. NSAIDs frequently cross-react with aspirin in intolerant individuals. We report a case of aspirin intolerance, which cross-reacts with naproxen, in a 42-year-old male with chronic urticaria. He experienced angioedema within 20 minutes after ingesting aspirin (700mg) and within 2 hours after ingesting naproxen (125mg). He has also experienced itching, wheal, chest tightness, and nausea during oral challenge test with aspirin.
Adult ; Angioedema ; Anti-Inflammatory Agents, Non-Steroidal ; Aspirin* ; Asthma ; Bronchial Spasm ; Eating ; Humans ; Male ; Naproxen ; Nausea ; Pruritus ; Rhinitis ; Shock ; Thorax ; Urticaria*