Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.
Angioplasty ; Antihypertensive Agents ; Cardiovascular System ; Diagnosis ; Humans ; Hypertension* ; Kidney Failure, Chronic ; Moyamoya Disease* ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1* ; Renal Artery Obstruction* ; Renal Artery*

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.
Angioplasty ; Antihypertensive Agents ; Cardiovascular System ; Diagnosis ; Humans ; Hypertension* ; Kidney Failure, Chronic ; Moyamoya Disease* ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1* ; Renal Artery Obstruction* ; Renal Artery*

The use of pure titanium and titanium alloys have been increased recently in fixed, removable prosthodontics and implant fields as a framework. But when they were used for superstructures of implant of metal framework of removable prosthesis, welding is necessary to reconnect the fracture site to control the casting distortions. To overcome the difficulties in soldering the titanium due to high oxidation property, much effort have been devoted. In this study, some of mechanical properties were compared between pure titanium and Ti-6Al-4V alloy by usingl aser welding, electron beam welding technique and tungsten arc welding. Mechanical properties such as tensile strength, yield strength, elongation and microhardness were measured. And, in order to compare the effect of welding site and surrounding metal tissue according to the welding condition, SEM photographs were taken an element distribution was observed by Wave Dispersion Spectroscopy. Through analyses of the data, following results were obtained; 1. In items such as tensile strength, yield strength and elongation according to the welding techniques of pure titanium, only tungsten arc welded group showed significant lower value than other groups(P<0.05). 2. In items such as tensile strength and yield strength according to the welding techniques of Ti-6Al-4V alloy, control group and tungsten arc welded group showed significant difference among all the groups(P<0.05). 3. Ti-6Al-4V alloy exhibited significantly greater elongation than control group when the laser welding method and electron beam welding method were used, and elongation showed increasing tendency. 4. Pure titanium specimens exhibited increasing tendency of microhardness regardless of the welding technique applied, and especially tungsten arc welded group demonstrated a great increase of microhardness than parent metal. 5. There was no hardness change in laser welded group and electron bean welded group of Ti-6Al-4V ally, but in tungsten arc welded group, hardness changed greatly from parent metal to weld seam. 6. Through the metallographic examination and scanning electron microscopy, laser welding caused central fusion and recristallizations were formed and tungsten arc welding caused localized fusion to 0.3-0.7mm from the surface.
Alloys ; Hardness ; Humans ; Microscopy, Electron, Scanning ; Parents ; Prostheses and Implants ; Prosthodontics ; Spectrum Analysis ; Tensile Strength ; Titanium* ; Tungsten ; Welding*

Pneumoconisosis was the first reported occupational disease in korea and was the most common occupational disease until 1990. Nowadays pneumoconiosis is the second most common occupational disease which accounts for more than at least 40% of all the occupational diseases in Korea. Many studies have been carried out in the prevalence rate, incidence rate, and risk factors assessment of pneumoconiosis. Workers exposed to dust used to take health examination once annually by the Industrial Safety and Health Act(1981). The number of coal workers has steadily decreased due to coal rationalization projects which have been strongly driven by the government since 1988 and the occupational environment has been improving. So, the incidence rate of pneumoconiosis will probably be lower in the future. But a disease control system administed for patient control and compensation, which is not involved in epidemiologic studies may not figure out the prevalence rate, incidence rate, mortality rate and the extent of severity of pneumoconiosis. Several problems and expected solutions are mentioned here as follows: 1. workers exposed to dust and pneumoconiosis patients are under government control, but the retired workers are not. Since we evaluate only visiting retired workers, we don't know exactly the current status and whole scale of the retired workers. If possible, the construction of cohort in all the retired workers is needed. 2. Since most of pneumoconiosis patients retired from the work and had changed their job, it is difficult to figure out the prevalence rate of pneumoconiosis just depending on the annual health exam for those workers at risk. So, systemic control for all pneumoconiosis patients is needed. 3. It is difficult to diagnose the onset of pneumoconiosis. We make a decision the time when the patient is diagnosed with pneumoconiosis on its onset. It is difficult to estimate it, especially in the case of retired workers because we can evaluate only persons with respiratory symptoms. The solution of such a problem is construction of cohort in all of the retired workers. 4. Because the patients who died outside of hospital don't seem to be reported, the mortality rate of pneumoconiosis is underestimated. So, systemic control and follow-up observation for all pneumoconiosis patients is needed. 5. A definite severity classification criteria for pneumoconiosis hasn't been established in Korea. We should try to make one. 6. Since workers who had exposed to dust in various mines at least 1 yr are subject to pneumoconiosis laws, workers easily don't report their full dust exposure history. Therefore we can't obtain the exact lifetime dust exposure from administrative data. We should try to make basic raw data of whole dust exposure in workers. It's concluded that the construction of cohort in workers who are or were exposed to dust in various mines is required and epidemiologic study of pneumoconiosis should be carried out with the administrative control of pneumoconiosis side by side. With the database of these materials, we can speculate and devise the measure for the further affecting subjects who are presumed to be most common in manufacturing industries.
Classification ; Coal* ; Cohort Studies ; Compensation and Redress ; Dust ; Epidemiologic Studies* ; Follow-Up Studies ; Humans ; Incidence ; Jurisprudence ; Korea* ; Mortality ; Occupational Diseases ; Pneumoconiosis* ; Prevalence ; Rationalization ; Risk Factors

Acetabular fractures assume great clinical importance because acetabular is weight bearing joint in the lower extremity. The undisplaced fractures of the acetabulum gave satisfactory results with closed method, but the treatment of displaced fractures have been controversial. A clinical analysis was performed on the 72 patients with displaced acetabular fractures, who had been treated at Soonchunhyang University Hospital from January 1980 to December 1989. The results obtained were as follows. 1. The prevalent age ranged from 21 to 50 years, which comprised 86.1% of all, and the ratio between male and female was 3:l. 2. The most common cause of injury was traffic accident, which comprised 76.4%. 3. According to Letournel's classification, simple fracture was 38 cases (52.8%), associated fracture was 34 cases (47.2%) and hip dislocation was associated in 28 cases (38.9%) and most common was posterior (20.8%). 4. Closed treatment was done in 47 cases and open treatment in 25 cases. 5. Results of open treatment group were better than closed treatment group in both clinical and roentgenographic study. 6. The complication were occured in 14 (29.8%) out of 47 cases of closed treatment group, and 10 (40.0%) out of 25 cases of open treatment group. 7. Not only choice of treatment but also determination of surgical approach require accurate assessment of the fracture pattern in this region of complex bony architecture by careful radilogic analysis.
Accidents, Traffic ; Acetabulum ; Classification ; Female ; Hip Dislocation ; Humans ; Joints ; Lower Extremity ; Male ; Methods ; Weight-Bearing

Postpubertal mumps may result in ochitis and permanent testicular atrophy may develop following infection. This present study was initiated to evaluate the preventive effect of interferon-alpha2B on infertilty after mumps orchitis. There were 21 patients with mumps orchitis between May 1990 and June 1997. Patients were randomly distributed into 2 groups: group 1 patients (n=13) maintained therapy with interferon-alpha2B (3x10(6) IU per day) and group 2 were managed by conservatively. All of the patients were evaluated with testis size measurement, mumps virus titer, hormone level, and if possible semen analysis. For group 1 patients symptoms disappeared within 2 to 3 days and the volume of testis returned to normal within 11 days and testis atrophy was not observed in all patients in follow up. But asthenospermia was continued in 4 patients (unilateral 2, bilateral 2). For group 2 patients symptoms disappeared within 5 to 6 days and the volume of testis returned to normal within 10 days and testis atrophy was observed in 3 patients (unilateral 2, bilateral 1) in floow up. Asthenospermia was continued in 4 patients (unilateral 2, bilateral 2). Sperm count and morphology were recovered all the recover in group 1, 4 patients had persistent reduced sperm count and morphology in group 2, respectively. These observations suggest that systemic interferon-alpha2B treatment is highly effective in preventing infertility as well as testicular atrophy after mumps orchitis.
Atrophy ; Follow-Up Studies ; Humans ; Infertility* ; Male ; Mumps virus ; Mumps* ; Orchitis* ; Semen Analysis ; Sperm Count ; Testis

PURPOSE: To report MALT (mucosa associated lymphoid tissue) type lymphoma in ocular adnexa. METHODS: This retrospective study included 11 patients (13 cases) of MALT type lymphoma between August 1995 and July 2000. We identified 11 lymphoma cases with MALT characteristics by conventional examination and immunohistochemical staining. Twelve cases were treated with partial excision and radiotherapy, one case with partial excision and chemotherapy. RESULTS: The patients consisted of 4 females and 7 males with an age range of 29~88 (average 48) years old. Mean follow up period was 24.8 (6~60) months following treatment. Eleven patients had ocular adnexal involvement at presentation; 7 eyes in conjunctiva, 5 eyes in orbit and one in eyelid. All cases represented extranodal marginal zone B-cell lymphoma by REAL classification. Control of lymphomas was achieved in all but 4 cases which developed recurrence after irradiation and chemotherapy, and were salvaged with further radiotherapy. Complications such as cataract, radiation retinopathy, ptosis and alopecia developed later in 4 eyes. CONCLUSIONS: Surgical excision and radiotherapy seemed to be effective for the treatment of primary ocular adnexal MALT lymphoma. Long-term follow up should be warranted.
Alopecia ; Cataract ; Classification ; Conjunctiva ; Drug Therapy ; Eyelids ; Female ; Follow-Up Studies ; Humans ; Lymphoma* ; Lymphoma, B-Cell, Marginal Zone ; Male ; Orbit ; Radiotherapy ; Recurrence ; Retrospective Studies

No abstract available.

Acute scrotal swelling is unusual in neonates and it should be treated as an emergency, especially in case with a clear urine. Only a few cases of an infected hydrocele have been previously described in the neonateal period. We report a 29-days-old newborn who presented with acute scrotal swelling that proved to be an idiopathic infected hydrocele on surgical exploration.
Emergencies ; Humans ; Infant, Newborn* ; Scrotum

Malignant schwannoma is a relatively rare malignant neoplasm arising from schwann cell of neuroectodermal origin that infiltrate locally and widely, and metastases. Inreaosseous bone involvement or retroperitoneal location of this tumor is extremely rare. A rare case of solitary malignant schwannoma which originated in the sacrum and extended to the retroperitoneal space in a 34 year-old male without neurofibromatosis reported. Simple X-ray showed not sclerotic, smooth marginated lytic defect in the right sacral bone. Computed tomography(CT) of sacrum revealed homogenous soft mass with intraosseous and dumbel shaped retroperitoneal extension without enhancement after were appeared in serial iliac artery angiogram. We could remove the tumor mass without specific technical difficulty with the help of CT and angiographic findings.
Adult ; Humans ; Iliac Artery ; Male ; Neoplasm Metastasis ; Neural Plate ; Neurilemmoma* ; Neurofibromatoses ; Retroperitoneal Space ; Sacrum*