Lymphangioleiomyomatosis(LAM) is a rare disease of women of child-bearing age in which there is progressive hyperplasia of atypical smooth mucle along lymphatics in the lung, and/or axial lymphatics in the thorax and abdomen, resulting in honeycombing of lung. Interestingly there has been a speculation that it represents a forme furste or incomplete expression of tuberous sclerosis complex. This is based on the observation that patients with tuberous sclerosis can manifest pulmonary lesions indistinguishable from LAM. We report a case of LAM occuring in a 39-year-old female, who complained of recurrent pneumothorax, chest pain and shortness of breath. Three years ago, the patient had right nephrectomy under the diagnosis of ruptured angiomyolipoma. A X-ray film of the chest showed honeycombing with a diffusely reticulonodular pattern and cyst-like spaces. She had a characteristic facial appearance of adenoma sebaceum, which her father and uncle had. Microscopically, the lung showed a marked smooth muscle proliferation around the slit-like lymphatic spaces and also some respiratory bronchioles.
Female ; Humans ; Adenoma

To estimate the manganese (Mn, below) exposure of workers in the ferromanganese manufacture factory and to evaluate its health effects, airborne, blood and urine Mn concentration measurements, questionnaire and other neurologic examinations were performed on 80 Mn-handing productive male workers (exposed group), 47 non-Mn-handling productive male workers (internal control group) and 144 productive male workers in other factory (external control group). The results obtained were as follows; The highest airborne Mn fume concentration among the work process was found at charging (0.42 mg/m3), and ferromanganese crushing process (1.14 mg/m3) was the highest in Mn dust. However all of them were below threshold limit value. Mean Mn concentrations in blood and urine of crushing workers were higher than those of other part workers. Among all of them, workers whose urine Mn concentration were exceed normal reference level (10 microgram/l) were 31 (18.5%). There was statistically significant correlation between airborne and urine Mn concentration (r=0.60), and so between airborne and blood Mn concentration (r=0.49), while there was no statistically significant correlation between blood and urine Mn concentration. Mean Mn concentration in airborne (0.60 mg/m3), urine (6.92 microgram/l) and blood (3.16 microgram/dl) in exposed group were significantly higher than those of control groups (p<0.01). Clinical symptoms such as excessive sweating, hypoesthesia, libido change, anosmia, decreased visual acuity and difficulty in writing showed higher positive rate in exposed group. Positive rate of clinical signs such as eye blinking and masked face in exposed group was higher than external control group. However clinical and laboratory findings such as blood pressure, blood chemistry, grip strength in exposed group were not statistically different from those of control groups. The results suggested that further studies were followed to evaluate the workers whose blood Mn concentration were below normal reference level but urine Mn concentrations above normal reference level, and to establish the questionnaire and the diagnostic tools to detect the Mn poisoning workers early.
Blinking ; Blood Pressure ; Chemistry ; Dust ; Hand Strength ; Humans ; Hypesthesia ; Libido ; Male ; Manganese* ; Masks ; Neurologic Examination ; Olfaction Disorders ; Poisoning ; Questionnaires ; Sweat ; Sweating ; Visual Acuity ; Writing

Catastrophic failure of a zirconia head with Hylamer acetabular component and early failure due to the phase transformation of zirconia ceramic head after total hip arthroplasty have been indebted issues. We report two cases who had failure of the zirconia ceramic head with polyethylene articulations shortly after total hip arthroplasty. Revision retrieval studies were performed subsequently.
Acetabulum ; Arthroplasty, Replacement, Hip* ; Ceramics* ; Head* ; Polyethylene*

PURPOSE: We analyzed the difference in values between noncycloplegic and cycloplegic autorefraction and refraction, as measured by ophthalmologist, in children according to age. METHODS: We classified the 84 children (153 eyes) whose best corrected visual acuity was better than 0.6 into four groups according to age. The first age group ranged from 29 to 57 months (36 eyes); the second age group ranged from 58 to 75 months (47 eyes); the third age group ranged from 76 to 95 months (36 eyes); and the fourth age group ranged from 96 to 121 months (34 eyes). The children were examined with an autorefractometer (Canon Auto Ref RK-2) and a retinoscope before and after cycloplegia. RESULTS: The children in all age groups tend to show more myopia in autorefraction than refraction regardless of cycloplegia. Also, differences in spherical component and spherical equivalents in noncycloplegic autorefraction and cycloplegic refraction were regarded as significant statistically (P<0.05) in all age groups. However, in all groups, none of the refractive values in cycloplegic autorefraction and refraction were statistically significant. CONCLUSION: Refraction measurement should be recommended for cycloplegic refraction in children. It is suggested that cycloplegic autorefraction could be useful for the refraction partially.
Child* ; Humans ; Myopia ; Retinoscopes ; Visual Acuity

Choroid plexus carcinoma is a rare malignant brain tumor that occurs predominantly in childhood. A 203-day-old infant was admitted to our hospital with macrocephaly and right hemiparesis. The skull protruded diffusely in the left parietal area. Brain magnetic resonance imaging) revealed a huge mass in the left ventricle. We performed an open biopsy and discovered a choroid plexus carcinoma. The tumor bled very easily and hemostasis was difficult. After three cycles of chemotherapy, we resected the entire mass in a staged operation. Chemotherapy enabled us to resect the entire tumor by reducing its size. We report this case to stress the benefits of preoperative chemo-therapy and review the relevant literature.
Biopsy ; Brain ; Brain Neoplasms ; Choroid Plexus* ; Choroid* ; Drug Therapy ; Heart Ventricles ; Hemostasis ; Humans ; Infant* ; Macrocephaly ; Paresis ; Rabeprazole ; Skull

The pathogenesis of IgA nephropathy and acute poststreptococcal glomerulonephritis is not fully understood. In the past, acute poststreptococcal glomerulonephritis was the most common cause of gross hematuria in children, but now IgA nephropathy is the most common one. We experienced two cases of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy in boys. Case 1 had upper respiratory infection before elevation of anti-streptolysin O, generalized edema, gross hematuria and proteinuria. The complement levels were normal. Electron microscopic findings of renal biopsy at ten days after onset showed a few big subepithelial 'humps' and localized heavy subendothelial and mesangial deposits. Immunofluoroscopic findings revealed predominant IgA deposition in the mesangium. The electron microscopic findings were diagnostic of acute poststreptococcal glomerulonephritis. On the other hand, immunoflorescence microscopic findings were compatible to IgA nephropathy. In case 2, the renal biopsy which was done 2 years after onset showed only finding of IgA nephropathy. To our knowledges, there has been few reports of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy which was confirmed by renal biopsy. We report two cases of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy with a brief review of the literatures.
Biopsy ; Child ; Complement System Proteins ; Edema ; Glomerulonephritis* ; Glomerulonephritis, IGA* ; Hand ; Hematuria ; Humans ; Immunoglobulin A* ; Proteinuria

Glass Fiber is manufactured from typical glass-making raw materials, silicon dioxide with various metals and other elements. There are three distinct types of commercial glass fiber products: glass wool, an entangled mass of interlocking fibers; continuous glass filament, a product with a more ordered arrangement of fibers; and special-purpose glass fiber, a small-diameter fiber (less than 3 um) . We report a case of glass fiber-induced health hazard due to continuous glass filament from fiber reinforced plastics (FRF) as car interior material. A 40 years old man complained a sudden onset of severe itching on whole body, especially neck and forearms while driving a new car. He also complained burning of eyes, sore throat and productive cough. We diagnosed the glass fiver-induced skin lesion by placing skin scraping on a slide glass and examining the specimen under a microscope. We found same fibers from the air samples in the car and from the material of the interior surface of the car by the microscopic examination. Therefore we confirmed that the patient's symptoms and signs were induced by glass fiber.
Adult ; Burns ; Cough ; Forearm ; Glass* ; Humans ; Metals ; Neck ; Nerve Fibers, Myelinated ; Pharyngitis ; Plastics ; Pruritus ; Silicon Dioxide ; Skin ; Wool

Oral prostaglandin E1 (PGE1) is a medicine that is clinically applied during a treatment of patients suffering with vascular disease with chronic arterial obstruction because it has vasodilation and anti-platelet effects. The mechanisms of lumbosacral symptoms associated with spinal stenosis probably include vascular insufficiency with hypoxic injury to the cauda equina and the nerve roots. Thus, increasing the blood supply would be beneficial to improve the pathophysiologic condition. Several studies on the improvement of clinical symptoms of spinal stenosis by PGE1 treatment have been reported on. In this case, 47-year old female underwent posterior compression and posterolateral fusion with a cage at L2-4 due to L3 compression fracture, and she did not show improvement of the radiating pain of her right leg after the operation. Therefore, she received repetitive epidural catheterization and adhesiolysis, epidural block and physical therapy, but her symptoms deteriorated after temporary improvement. Finally, she was given PGE1 and the radiculopathy was completely improved, although some muscle weakness still remained.
Alprostadil* ; Catheterization ; Catheters ; Cauda Equina ; Failed Back Surgery Syndrome* ; Female ; Fractures, Compression ; Humans ; Leg ; Middle Aged ; Muscle Weakness ; Radiculopathy ; Spinal Stenosis ; Vascular Diseases ; Vasodilation

Fiberglass, as a substitute of asbestos, is used for more than 60 years as a insulator material. Health hazards including irritation of skin, mucosa and respiratory system associated with use of fiberglass is reported. Many studies on the fibrogenicity and carcinogenicity of fiberglass was conducted but evidence is not sufficient to confirm the carcinogenicity or fibrogenicity. Authors studied the health hazards among 152 inhabitants(71 men and 81 women) from 32 households living around the fiberglass factory which produced the fiberglass insulators and glasswool panels for 20 years. Questionnaire survey on household and persons, examination of underground water, pathologic examination of subcutaneous tumors and examination of fiberglass in tumor tissues were done. The results are as follows; 1. Fiberglass concentration of underground water sampled from 33 households in the study area was 13. 7-95. 9 fiber/cc with the diameter to length ratio more than 1:20. 2. Prevalence of dermatosis among study subjects was 23.0 % (35 cases). Prevalence was not associated with the distance from the factory nor duration of exposure. 3. There were 15 cases of subcutaneous tumor with prevalence of 9. 9 %. Age of subcutaneous tumor cases was all above 30 year-old except one cases, who was 5 year old child, who lived in the surveyed area since he was born. Prevalence of subcutaneous tumor was significantly high in area A(42.9 %) than area B(4.6 %, p<0.01). Prevalence of subcutaneous tumor was higher in long-term exposed inhabitants, but was not statistically significant. 4. Pathologic examination exhibits partly encapsulated fat tissue masses and cut surfaces were pale yellow with gritty sensation. The masses consist of mature fat cells showing variation in size and shape. On polarizing microscope, the peripheries of tumors include small irregular threads of doubly refractile material probably represent glass fibers in fibrocollagenous tissue. Concentration of fiberglass in tissue was 5.1-10.2 fiber/rag wet tissue in case 1, 25.8-184.9 fiber/mg wet tissue in case 2 and 40.8-126.5 fiber/mg wet tissue in case 3. Length of fiberglass was shorter than that in underground water. 5. Cases of malignant tumor among inhabitants since last 10 years were 4, 3 of whom was developed in a same household just near the factory. Diagnoses of malignancy cases were stomach cancer, stomach and esophageal cancer, oral cavity cancer, and stomach cancer with liver metastasis. On review examination of tissues of endoscopic biopsy specimen from a case of stomach cancer, there was adenocarcinoma with no evidence of fiberglass materials. Authors concluded there is evidences that fiberglass was strongly associated with the development of the health hazards including dermatosis and benign subcutaneous tumor. However, the association of fiberglass exposure with the development of malignant tumor was not clear, although strongly suggested. For the prevention of development of further health hazards, it is recommended that under ground water source should be closed and further experimental study to confirm the mechanism of the tumorigenesis and follow up survey on the inhabitants should be conducted.
Adenocarcinoma ; Adipocytes ; Adult ; Asbestos ; Biopsy ; Carcinogenesis ; Child ; Child, Preschool ; Diagnosis ; Epidemiologic Studies* ; Esophageal Neoplasms ; Family Characteristics ; Glass* ; Groundwater ; Humans ; Liver ; Male ; Mouth ; Mucous Membrane ; Neoplasm Metastasis ; Prevalence ; Respiratory System ; Sensation ; Skin ; Skin Diseases ; Stomach ; Stomach Neoplasms ; Surveys and Questionnaires

PURPOSE: To confirm the effect of the frog leg lateral radiographs for determining the clinical indication of total hip arthroplasty for high riding dislocation and to discuss its theoretical background. MATERIALS AND METHODS: A consequent series of reconstructive THA were studied in 28 recent tuberculous hips. Twenty-three hips (17patients) with developmental dysplastic hip were compared. Preoperatively, frog-leg lateral radiographs were performed in all the patients, which were enhanced using a 2-dimensional computer model to predict the reattachment of the greater trochanter. RESULTS: The modified scores of Merle d'aubigne and Postel improved from a mean of 2.6 points preoperatively to a mean of 5.1 points. Sciatic nerve palsies occurred in three cases. In two cases, they fully recovered, but in one case, it did not. We confirmed that old high dislocation of the hip could be adopted in type 1 and 2 categories of frog leg lateral position. The reconstruction of hip was relatively simple in type 3, however, further study is necessary in type 5 due to unsuitable abduction contracture. CONCLUSION: We confirm that frog leg lateral radiography is effective for determining the operative indication of high riding dislocation. However irreducible frog leg lateral position is absolutely contraindirated for total hip arthroplasty.
Arthroplasty* ; Arthroplasty, Replacement, Hip ; Computer Simulation ; Contracture ; Dislocations* ; Femur ; Hip* ; Humans ; Leg ; Radiography ; Sciatic Neuropathy