The occurence of pseudarthroses associated with neurofibromatosis is a well documented phenomenon, with the majority of reported cases in the tibia but quite rare in forearm,Only ten cases in the English-literature and one case in Korean-literature of this lesion revealed appearing in the bones of the forearm till now. Of these, four in solitary pseudarthroses of the ulna(4.5.15), five in a solitary lesion of the radius(7.8.10.16.17), and only two cases was on the both bones of the forearm(9.14). We are presenting the case report of a Korean adult male with the clinical stigmata and a positive familial history of neurofibrormotosis who had congenital pseudarthroses of both the radius and ulna of the right forearm. Therefore, the present case is the third to be reported. We treated him with by production of an one-bone forearm using a dual a onlay bone graft positioned in neutral of supination and pronation. A stable, functional extremity with good union of pseudarthroses were encountered.
Adult ; Christianity ; Extremities ; Forearm ; Humans ; Inlays ; Male ; Neurofibromatoses ; Neurofibromatosis 1 ; Pronation ; Pseudarthrosis ; Radius ; Supination ; Tibia ; Transplants ; Ulna

For the measurement of the bone quantity, various methods have been used. Recently, the methods such as a microdensiometry, single photon absorptiometry, quantitative computed tomography were introduced. However, these methods are expensive modalities to be used in daily clinical practice. For the evaluation of the osteoporosis, author's observed Saville's grades, spine contral score and spine fracture on the simple spine roentgenograms of 346 patients over 40 years of age who were exsmined due to their backache at the Department of Orthopedadic Surgery, Kang Nam St. Mary's Hospital, Seoul, and following results were obtained: 1. The percentile incidence of osteoporosis with regard to age and sex : (1) The percentile incidence of osteoporosis over Saville's Grade I was 59.2%(205 of 346 persons); in males 47.4%(55 of 116 persons) and in females 65.2%(150 of 230 persons). (2) The percentile incidence of osteoporosis over Grade 1 in 5th decade was 27.2%(17.1% in males and 33.9% in females), 55.8% in 6th decade(46.6% in males, and 60.4% in females), 78.1% in 7th decade(64.7% in males, and 82.9% in females), 93.7% in 8th decade(87.5% in males and 97% in females), and 100% in both sexes of 9th decade. (3) The percentile incidence of Grade 1 osteoporosis in 5th decade was 23.9%, and in 6th decade it was 46.7%. Thus, Grade I osteoporosis was most common in these age groups. In 7th and 8th decades, Grade I osteoporosis was more common in males, while in the females ot these age groups Grade II was more common. There was no sex difference in the incidence of osteoporosis after the age of 80. Grade IV osteoporosis was found only in a woman who had hysterectomy 7 years ago, and has suffered from Parkinsonism for 3 years. (4) The overall incidence of Grade I osteoporosis was 34.6%, and Grade II was 21.3 %. The incidence of osteoporosis over Grade III was 1.7% in males, and 6.5% in females. 2. Incidence of osteoporosis assessed by Barnett's central score and its correlation with Saville's assessment: (1) The central score obtained at the 3rd lumbar spine ranged from 71.4 to 106.4. The number of cases were 16 in the score range of 70 to 80, 93 in the score range of 101 to 110, and 5 in the score range of 111 to 120. In consequence the most high incidence was in the score range of 91 to 100. (2) Grade O Oxteoporosis corresponded with the central score of 96.4 on an average (range : 86.8 to 106.4), Grade I corresponded with 91.6 (range : 85.7–103.1), Grade II corrosponded with 86.4 (range : 78.3–96.9), Grade III corresponded with 79.4 (range : 71.4–91), and Grade IV corresponeded with 75.8. (3) Barnett defined the spine is osteoporotic, when the central score is below 80. However, in this study only 16 patients(4.6%) had the score below 80. No bodies having Grade 0 and I osteoporosis had the score below 80; of those 16 patients having the score below 81, 5 were Grade II, 10 Grade III, and one Grade IV. Through this study, it is clesr that there is no correlation in these two assessing methods. The central score below 80 was found in severely porotic patients such as Grade III and IV, but was not found in most cases of Grade I and II osteoporotic patients. (4) The vertebral defromity was found only in 38 cases (10.9%), and it was found only in 18.9% of osteoporosis patients. The percentile incidence of deformity was 5.3% of Grade I, 31% of Grade II and 52.9% of Grade Ill. (5) Of these deformities, wedge-shaped one was found in 6 cases of Grade I porosis, 20 of Grade II, and 3 of Grade III. Cod-fish vertebrae (biconcave) were found in 2 of Grade II and 4 of Grade III. The flattened body was found in 1 of Grade II and 2 of Grade III. Thus, biconcave and flattened vertebrae were found only in the porotics over Grade II severity. (6) In 24 cases, one body was deformed, and in 14 cases multilevel vertebral deformities were found. In these 20 cases had deformity in the Ll, 10 cases each in T12 and L2, 7 cases in L3, 6 cases in L4, and 4 cases in Tll. In summary, the most deformities were in the dorsolumbar region. (7) Fracture was induced by trauma in the 11 cases(29%), among which 10 were wedgeshaped, and the rest was flattened. The biconcave vertebra was found not to be related with injury.
Absorptiometry, Photon ; Back Pain ; Congenital Abnormalities ; Female ; Humans ; Hysterectomy ; Incidence ; Male ; Osteoporosis ; Parkinsonian Disorders ; Seoul ; Sex Characteristics ; Spine

No abstract available.
Lung*

The gene for tyrosinase has been mapped to the long arm of chromosome 11 at 11q14-21. The gene is at least 50Kb in length and its coding region is divided into five exons. Until now several mutations of the tyrosinase gene have been identifed in patient with typical oculocutaneous albinism (OCA) who are responsible for tyrosinase negative OCA. It may be possible to determine the types of OCA by measuring the hairbulb tyrosinase activity. Hairbulb tyrosinase activity was examined in a Korean albino to determine the type of OCA. And also tyrosinase assay was carried out in normally pigmented individuals and all members of a Korean albino's family to examine the tyrosinase activities. Five exons of tyrosinase gene from a Korean albino were amplified by polymerase chain reaction. Each amplified exon segments were independently subcloned and DNA sequences of clones were determined. The results obtained were as follows : 1. A Korean albino had no measurable hairbulb tyrosinase activity and was identified as type IA (tyrosinase negative) oculocutaneous albinism. 2. Normally pigmented individuals had different ranges of hairbulb tyrosinase activity. 3. A Korean albino had two single base insertions within exon V (between 337bp and 338bp, 353bp and 354bp) of tyrosinase gene. These insertional mutations might disrupt tyrosinase function and were associated with a total lack of melanin biosynthesis.
Albinism* ; Albinism, Oculocutaneous ; Arm ; Base Sequence ; Chromosomes, Human, Pair 11 ; Clinical Coding ; Clone Cells ; Exons ; Humans ; Melanins ; Monophenol Monooxygenase* ; Polymerase Chain Reaction

BACKGROUND: A mummy is a dead body of a human being or an animal that has been preserved artificially or naturally from decaying. Because the natural environment of Korea isn't appropriate for mummification and Korean people haven't artificially made mummies, mummies were rarely studied in Korea. METHODS: On September 6, 2002, a well-preserved female mummy was found in the grave of a family in Kyunggi-do. She was submitted to a thorough autopsy examination along with the review of genealogical documents. RESULTS: The mummy died in winter. She was pregnant and the fetal head was observed at the vaginal orifice. The uterine wall was ruptured, and the peritoneum was discolored, probably by hemorrhage. Histologically, the gastric mucosa was well preserved. On the smear cytology of gastrointestinal material and the fluid from the coffin, pollens and parasitic eggs were observed. The woman seemed to be death from hypovolemic shock due to uterine rupture during the 2nd phase of labor. CONCLUSION: From this case, we concluded the causes of the woman's mummification included the cold and dry circumstance at the time of her death, and the thick mortared wall of the grave that completely isolated the body from the outside.
Animals ; Autopsy ; Eggs ; Female ; Gastric Mucosa ; Gyeonggi-do ; Head ; Hemorrhage ; Humans ; Korea ; Mummies ; Ovum ; Paleopathology ; Peritoneum ; Pollen ; Pregnant Women* ; Shock ; Uterine Rupture

STUDY DESIGN: This is a retrospective study determining the surgical result of lumbar HIVD associated with spondylolysis. OBJECTIVES: To analyze the incidence of lumbar HIVD associated with spondylolysis and to compare the results of open discectomy for lumbar HIVD associated with spondylolysis to simple lumbar HIVD. SUMMARY OF LITERATURE REVIEW: Lumbar HIVD associated with spondylolysis need be treated by spinal fusion. MATERIALS AND METHODS: Nine patients(5 males and 4 females) who had lumbar HIVD with spondylolysis, no instability, fol-low-up period of 1yr were identified out of 273 patients with lumbar HIVD, treated by open discectomy from March 1989 to Feb. 1999. The type of HIVD and level of spondylolysis were evaluated, the clinical symptoms and signs including SLR, motor deficit, sensory deficit, change of DTR and severity of radiating pain were periodically followed up on the predesigned protocol. RESULTS: The incidence of lumbar HIVD associated with spondylolysis is 3.7%. The recovery of back pain was 2.1 to 2.1 by visu-al analogue scale, radiating pain was 7.6 to 0.8. The recovery rate of SLR was 100%, motor deficit; 100%, sensory deficit; 85%, change of DTR; 40%. The clinical evaluation was excellent(2), good(6), fair(1). CONCLUSIONS: According to the recovery rate of the clinical symptoms, the results of open discectomy for lumbar HIVD associ-ated with spondylolysis without spinal instability and simple HIVD was not different. Therefore, we conclude that lumbar HIVD associated with spondylolysis need not be treated by spinal fusion.
Back Pain ; Diskectomy ; Humans ; Incidence ; Male ; Retrospective Studies ; Spinal Fusion ; Spondylolysis*

Since 1976, several cases of acute renal failure and acute hepatitis after ingestion of raw carp bile have been reported. We experienced 1 case of acute renal failure and hepatitis after ingestion of the raw fish(Cyprinus carpio nudus) gall bladder. The patient ate raw fish gall bladder to improve the health. The size of the fish he ate was over 1 m and that of the gall bladder was about an egg size. The clinical manifestations were characterized by dizziness, headache, diffuse abdominal pain, both flank pain, nausea, vomiting and diarrhea. These symptoms were followed by gross hematuria, oliguria and jaundice. Elevation of blood urea nitrogen, creatinine, transaminases lasted for about 2 weeks. Biopsy of the kidney was performed 13 days after ingestion of raw fish gall bladder. The kidney pathology showed recovery phase of acute tubular necrosis. The patient recovered with conservative treatment.
Abdominal Pain ; Acute Kidney Injury* ; Bile ; Biopsy ; Blood Urea Nitrogen ; Carps ; Creatinine ; Diarrhea ; Dizziness ; Eating* ; Flank Pain ; Headache ; Hematuria ; Hepatitis* ; Humans ; Jaundice ; Kidney ; Nausea ; Necrosis ; Oliguria ; Ovum ; Pathology ; Transaminases ; Urinary Bladder* ; Vomiting

Thin glomerular basement membrane nephropathy, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and by hematuria. The present study was based on a retrospective review of 366 native kidney biopsies performed at Chungnam National University Hospital from January 1993 to November 1996. Fifteen of these cases satisfied the criteria for definition of thin glomerular basement membrane nephropathy, placing the incidence of the disease at 4.0%. The criteria for definition of the disease are 1) normal findings by light microscopy, 2) no deposits by immunofluorescent microscopy, and 3) diffuse thinning of the glomerular basement membrane by electronmicroscopy. The studied patients had a mean age of 35.8 years (range from 14 to 59) and included 12 females and 3 males. A positive family history was present in one case. All pateints were normotensive and had hematuria (four cases showed gross hematuria). Seven patients had mild proteinuria, but two patients showed proteinuria of nephrotic range. The serum creatinine level was within normal limits except one. Abnormalities were not observed in immunoglobulins, complement component, serologic tests (RA, ASO) and HBsAg. Renal biopsy findings by light and immunofluoroscent microscopy did not reveal any abnormalities but diffuse thinning of the glomerular basement membrane (range from 154 to 279nm) was observed by electronmicroscopy. With the above results, patients with idiopathic renal hematuria with normal renal function and normal blood pressure, thin glomerular basement membrane nephropathy should be considered.
Biopsy ; Blood Pressure ; Chungcheongnam-do ; Complement System Proteins ; Creatinine ; Female ; Glomerular Basement Membrane* ; Hematuria ; Hepatitis B Surface Antigens ; Humans ; Immunoglobulins ; Incidence ; Kidney ; Male ; Membranes ; Microscopy ; Proteinuria ; Retrospective Studies ; Serologic Tests

Congenital arteriovenous malformation (AVM) usually involves the head, extremities and internal organs, but isuncommon in the chest wall. It is, moreover, rarely combined with aneurysms. It can be cured by surgery, but isoften difficult to treat. The purpose of this study is to report a case of extensive AVM involving the rightlateral chest wall combined with multiple aneurysms, which was successfully treated by transcatheter embolizationusing several embolic materials.
Aneurysm ; Arteriovenous Malformations* ; Extremities ; Head ; Thoracic Wall* ; Thorax*

We evaluated retrospectively the clinical characteristics of 26 adult nephrotic syndrome patients with minimal-change disease who were followed up for more than six months. Median follow up period was 23months. They consisted of 16 men and 10 women, and the mean age was 34.3 years (range 18-68 years). We administered prednisolone 1.0mg/Kg body weight to the patients for eight weeks and tapered it gradually. Complete remission was obtained in 22 (85%) patients treated with prednisolone and 54% of them reached complete remission in four weeks after beginning prednisolone treatment. Nine patients who initially responded to prednisolone therapy relapsed. Two of these nine patients showed single relapse and seven patients had frequent relapses during the study period. Cyclophosphamide or cyclosporin A was added when the patients did not respond to prednisolone and showed frequent relapse. Of the six patients treated with cyclophosphamide, four patients who were frequent relapsors and one patient who was a non-reponder to prednisolone went into complete remission. The other prednisolone non-responder did not remit. Microscopic hematuria was found in three patients and these patients showed elevated serum creatinine level at the time of diagnosis. Seven patients showed renal impairment at the time of renal biopsy. All of them recovered from renal failure after treatment. Six patients showed complete remission of nephrotic syndrome after prednisolone and/or cyclophosphamide treatment. There was no patient who showed a deterioration of renal function during the study period. In conclusion, complete remission was obtained in 85% of adult minimal change nephrotic syndrome patients and 41% of patients relapsed within one year after attaining complete remission. The serum level of creatinine and microscopic hematuria at the time of diagnosis were not associated with poor prognosis during the study period.
Adult* ; Biopsy ; Body Weight ; Creatinine ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Female ; Follow-Up Studies ; Hematuria ; Humans ; Male ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Prednisolone ; Prognosis ; Recurrence ; Renal Insufficiency ; Retrospective Studies