Maroteaux and Lamy coined the term Pycnodysostosis derived from the Greek, Pycnos (thick or dense), Dys (defective), and Ostosis (bone). The principal characters of this syndrome are short stature, dysplasis of the skull, obtuse manibular angle, dysplastic clavicles,partial or total aplasia of the terminal phalanges, and generalized increased roentgenographic density of the skeleton. Patient so affected are predisposed to fracture. Pycnodysostosis seems to be a genetic disorder, inherited as an autosomal recessive trait. Here, it is reported a case of incidental finding of pycondysostosis with chronic renal failure in 12 years old girl.
Female ; Humans ; Incidental Findings ; Kidney Failure, Chronic ; Numismatics ; Pycnodysostosis ; Skeleton ; Skull

Neuroblastoma is a malignant tumor of sympathetic nerve origin. They are derived from sympathetic formative cells, the primitive neuroblasts that have failed to mature. They may be located wherever sympathetic nerve tissue is found, The majority of cases occured in the suprarenal medulla, posterior mediastinum, and orbit. The tumor metastasized early by the hematogenous or lympathic route, or both to skeletal system, liver, and skin, etc. Since the vast majority of children with neuroblastoma have local or distant metastases at the time of original diagnosis, the clinical manifestation and the prognosis are variable. The age distribution is from birth to 19 years old. Many methods of treatment of neuroblastoma have been advocated such as surgical excision,roentgen thrapy, and its combipation therapy but the effect is questionable. Here, it is reported a case of neuroblastoma with systemic skeletal metastasis originated from posterior mediastinum in 8 years old girl.
Age Distribution ; Child ; Diagnosis ; Female ; Humans ; Liver ; Mediastinum ; Neoplasm Metastasis ; Nerve Tissue ; Neuroblastoma ; Orbit ; Parturition ; Prognosis ; Skin

The metallic failure is one of the annoying problems after fracture surgery. The seventeen cases of metallic failures after compression plate fixation were treated at the Department of Orthopaedic Surgery. Yonsei University Wonju College of Medicine between January, 1980 and December, 1988. The results were summerized as follows: l. Among 17 cases, 12(70.5%) were under 40 years of age and 14(80%) were male patients. 2. The sites of fracture of the seventeen cases; 12 femurs, 3 tibiae, and 2 radii. Of the cases, there were breakage of plates in 13 cases, bending 1 case and failure of screw in 3 cases. 3. The pattern of fracture: communited fracture
Femur ; Fractures, Bone ; Fractures, Open ; Gangwon-do ; Humans ; Incidence ; Male ; Osteoporosis ; Radius ; Tibia ; Transplants ; Weight-Bearing

Osteoporosis causes a decrease in bone mineral density. To overcome the decrease in fixation strength, a variety of techniques and devices have been developed, including cement augmentation of pedicle screws. Polymethyl methacrylate (PMMA), is commonly known as bone cement, and is widely used for implant fixation in various orthopaedic and spine surgery. In general, PMMA augmentation of pedicle screws is simple and safe if performed with technical precautions. PMMA is a safe agent, but it may rarely lead to significant foreign body reactions. In this report, we present a patient who developed bone cement-related epidural space foreign body granuloma
Osteoporosis

No abstract available.
Pregnancy*

In a series of 20 young rats, three cortical lesions were made in each hemisphere. Two of these lesions were filled with avitene and gelfoam, while the third was left empty as a control. The animals were killed successively on weeks 1, 2, 4 and 8 after the operation. The results were as follows : 1) Although there was no difference in the type of tissue reactions, avitene was more rapid and profound than gelfoam in the process and degree of inflammatory reaction at the same periods. 2) Avitene biodegradaded more rapidly than gelfoam. 3) The extent of fibrosis and adhesion to the surrounding brain tissues were not grossly different between these two agents but more profound histopathologically in avitene at the same periods. With these results, we could conclude that the avitene was superior to the gelfoam as the hemostatic agent in neurosurgical area.
Animals ; Brain* ; Collagen ; Fibrosis ; Gelatin Sponge, Absorbable ; Rats

Kaposi's sarcoma is a rare tumor comprising 0.1 per cent of all malignancies worldwide. There is, however, an increased ineidence following renal transplantation, immunosupression and in the acquried immunodeficency syndrome(AIDS) Kaposi's sarcoma has been shown to involve every organ of the body except the brain. Gastrointesinal involvement is the most common extracutaneous site of involvement. Gaatrointesinal Kaposis sarcoma is preaent in approximately half of patients with cutaneous Kaposis sarcoma and the acquired immune deficiency syndrome(AIDS). Although usually asymptomatic, gastrointestinal Kaposi's sarcoma may cause pain, bleeding, diarrhea, obstruction, intussusception, perforation. malabsorption, and protein-losing enteropathy. Three distinct gastroscopic appearances of Kaposi's sarcoma have been described: maculopapular, polypoid, and umbilicated nodular lesions. We report a case of Kaposis sarcoma of the stomach.
Brain ; Diarrhea ; Hemorrhage ; Humans ; Intussusception ; Kidney Transplantation ; Protein-Losing Enteropathies ; Sarcoma, Kaposi* ; Stomach*

The potential of malignant melanoma to metastasize to all parts of the body is well known. Metastatic melanoma of the stomach may present with vague gastrointestinal symptoms, abdominai pain, or gastrointestinal bleeding. When gastrointestinal symptoms occur in a patient with known melanoma, gastric metastases should be considered. Melanoma metastatic to the stomach develops multiple small ulcerating masses. These sharply delineated submucosal lesions have been described as having a "bull's eye" or "target" configuration. Barium X-ray study, endoscopy, cytology study, and biopsy may yield the diagnosis. The prognosis is poor. We report a case of metastatic melanoma of the stomach with the review of the literature.
Barium ; Biopsy ; Diagnosis ; Endoscopy ; Hemorrhage ; Humans ; Melanoma* ; Neoplasm Metastasis ; Prognosis ; Stomach* ; Ulcer

PURPOSE: Nitric oxide(NO) is a highly reactive messenger molecule that is synthesized by nitric oxide synthase(NOS) in a number of tissues including the brain. Our aim was to examine the possible role of NO on pentylenetetrazole(PTZ)-induced seizure in rats as a proconvulsant or an anticonvulsant. METHODS: Male Sprague-Dawley rats weighing 200-300g were used. PTZ(40mg/kg) was used to induce seizure. In order to examine the role of NO in seizures, the neuronal nitric oxide synthase (nNOS) was blocked by a specific inhibitor, 50mg/kg 7-nitroindazole(7-NI), and a non-specific inhibitor, 50mg/kg L-nitroarginine(L-NA). Both 7-NI and L-NA were administered 30 min before the administration of PTZ. We evaluated the onset time of the first myoclonic jerk and the nature of seizures, such as the types and severity of the seizures in each group. Western blot and RT-PCR were used to examine the expression of nNOS mRNA and proteins in the hippocampus of each group. RESULTS: The onset time of the first rnyoclonic jerk was markedly delayed in the 7-NI and L-NA pretreated groups in comparison to the PTZ group. In addition, 7-NI and L-NA markedly sup- pressed the severity of PTZ-induced seizure. The expression of nNOS mRNA was higher in the PTZ and 7-NI pretreated groups than in the control group. The expression of nNOS protein was more suppressed in the 7-NI and L-NA pretreated groups than in the PTZ group. CONCLUSION: The expression of nNOS protein appears to increase in PTZ-induced seizures. Both specific and non-specific NOS inhibitors produce a reduction in seizure activity and nNOS protein expression. These results suggest that nNOS may be in a close relationship with seizures, and NO may play an important role as a proconvulsant in the PTZ-induced seizure in rats.
Animals ; Blotting, Western ; Brain ; Hippocampus ; Humans ; Male ; Myoclonus ; Nitric Oxide Synthase Type I ; Nitric Oxide* ; Pentylenetetrazole* ; Rats ; Rats, Sprague-Dawley ; RNA, Messenger ; Seizures*

BACKGROUND: Chemokines are molecules with chemotatic activities on selective leukocyte populations and are sub-grouped into alpha-chemokine acting primarily on PMNL (polymorphonuclear leukocyte) and beta-chemokines attracting mainly lymphocytes and monocytes. We conducted a prospective study to investigate the serum levels of interleukin (IL)-8, monocyte chemoattractant protein (MCP)-1, and macrophage inflammatory protein (MIP)-1 alpha in patients with acute ischemic stroke and carotid atherosclerosis. METHODS: Serum was sampled from patients with acute ischemic stroke (<24hrs), with persistent ischemic neurological deficits associated with atherosclerosis (>1 month), and from normal subjects without a history of vascular disease. Concentrations of chemokines were measured by enzyme linked immunosorbent assay ( ELISA ). RESULTS: Compared with carotid atherosclerotic patients and control subjects, the serum levels of IL-8 were significantly elevated in those with acute ischemic stroke. The serum levels of MCP-1 in patients with large artery disease were higher than those in patients with small vessel disease and cardioembolism. CONCLUSIONS: This study suggested that IL-8 can be involved in acute ischemic stroke and MCP-1 plays a role in the pathogenesis of atherosclerosis.
Arteries ; Atherosclerosis* ; Carotid Artery Diseases ; Chemokine CCL2* ; Chemokines ; Chemokines, CC ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Humans ; Interleukin-8* ; Interleukins ; Leukocytes ; Lymphocytes ; Macrophage Inflammatory Proteins* ; Macrophages* ; Monocytes* ; Prospective Studies ; Stroke* ; Vascular Diseases