Congenital pseudarthrosis is frequently associated with various stigmata of neurofibromatosis, particularly Cafe-Au-Lait spots and,subcutaneous nodules and in these patients, it is noteworthy that the treatment is very difficult even with extensive operative approach. Also it is interesting to find many controversies on etiological relation between neurofibroma and pseudarthrosis, per se and even the existance of nerve element in and around the tissue of pseudarthrosis. A thirteen years old female patient with definite neurofibromatosis associated with congenital tibial pseudarthrosis treated by intramedullary nailing and massive bone graft is reported.
Cafe-au-Lait Spots ; Christianity ; Female ; Fracture Fixation, Intramedullary ; Humans ; Neurofibroma ; Neurofibromatoses ; Pseudarthrosis ; Transplants

Foreatiers disease is a peculiar type of ankylosing hyperostosis of the spine characterized by ossification of the anterior and right lateral aspects of the vertebral column, particularly in the thoracic region. The clinical, pathological and reontgenographic features of the disorder allowed its differentiation from other spinal diseases including ankylosing spordylitis and osteoarthritis. A case of Forestiers disease is presented with brief review of literatures.
Hyperostosis ; Hyperostosis, Diffuse Idiopathic Skeletal ; Osteoarthritis ; Spinal Diseases ; Spine

OBJECTIVE: To evaluate the efficacy of prenatal multiplex PCR with chorionic villi, in the case of family history of DMD due to exon deletion. METHODS: DNA was extracted when cells' size are 1x 106 on T- flask surface area reach to 25 cm2 after 2 weeks from sex confirmation. Average DNA concentration was 50-100 ng and multiplex PCR test was performed from DNA extraction. RESULT: PCR was done for 17 exons devided into 4 groups. Seventeen exons were all amplified with their right size. CONCLUSIONS: This method is DNA analysis for prenatal diagnosis of DMD with chorionic villi in the family of DMD. This is useful when preimplantation genetic diagnosis is not available.
Chorion* ; Chorionic Villi* ; DNA ; Exons ; Humans ; Multiplex Polymerase Chain Reaction* ; Muscular Dystrophy, Duchenne* ; Polymerase Chain Reaction ; Preimplantation Diagnosis ; Prenatal Diagnosis*

BACKGROUND: Propofol has a high incidence of pain with intravenous injection, and different methods have been used to minimize the incidence and severity of this pain. In this study, we have compared the effect of lidocaine pretreatment with that of ketorolac pretreatment on propofol injection pain. METHODS: Sixty children, aged 7 - 12 years, scheduled for general anesthesia were randomly divided into three groups; saline group (n = 20), lidocaine group (n = 20), and ketorolac group (n = 20). All patients were inserted with a 20 G or 22 G angiocatheter into the veins on the dorsal hand or antecubital fossa. After applying an arm tourniquet at an inflation pressure of 30 - 40 mmHg, the saline group received 2 ml of 0.9% saline, the lidocaine group received lidocaine 0.75 mg/kg mixed with 2 ml of 0.9% saline, and the ketorolac group received ketorolac 0.75 mg/kg mixed with 2 ml of 0.9% saline. The tourniquet was released 1 minute later, followed by an intravenous injection of 2.5 mg/kg of propofol. Pain and involuntary movement at propofol injection and changes of blood pressure and heart rate were compared. RESULTS: 1. The incidence of pain diminished significantly in the lidocaine group (15%) and the ketorolac group (25%) compared with the saline group (80%) at the time of injection of propofol and there was no significant difference between the lidocaine group and the ketorolac group. 2. The incidence of involuntary movement diminished significantly in the lidocaine group (grade 0: 80%, grade 1: 20%, grade 2: 0%) and the ketorolac group (grade 0: 65%, grade 1: 30%, grade 2: 5%) compared with the saline group (grade 0: 10%, grade 1: 25%, grade 2: 65%) at the injection of propofol and there was no significant difference between the lidocaine group and the ketorolac group. 3. Blood pressure and heart rate significantly increased after intubation in all groups. CONCLUSIONS: From the above results, both intravenous lidocaine and ketorolac pretreatment are effective in alleviating propofol injection pain and involuntary movement, but there is no significant difference between the lidocaine group and the ketorolac group.
Analgesics ; Anesthesia, General ; Arm ; Blood Pressure ; Child ; Dyskinesias ; Hand ; Heart Rate ; Humans ; Incidence ; Inflation, Economic ; Injections, Intravenous ; Intubation ; Ketorolac* ; Lidocaine* ; Propofol* ; Tourniquets ; Veins

BACKGROUND: The minimal effect of etomidate on cardiovascular function sets it apart from other rapid fast-acting induction agents. Clinically, etomidate has been reported to cause minimal effects on systemic hemodynamics and PVR. There are few reports of direct effects of etomidate in pulmonary vessels or other vascular beds. METHODS: We studied the effects of etomidate on the tension of the aortic smooth muscle using an isolated rat thoracic aortic preparation. We studied the cumulative effect of etomidate in a rat thoracic aorta after phenylephrine (PE) pretreating, the cumulative effect of phenylephrine (PE) in a rat thoracic aorta with or without endothelium after etomidate pretreating, the effect of L-NAME and indomethacin and metylene blue in a rat thoracic aorta contractile response for phenylephrine after etomidate pretreating, and the effects of etomidate on a phenylephrine and ECF Ca2 induced contraction in a rat thoracic aorta. RESULTS: Etomidate produced dose-dependent relaxation and these relaxation responses were significantly less in a thoracic aorta with denuded endothelium than in a thoracic aorta with intact endothelium. Response of PE contraction with etomidate was increased by pretreatment with L-NAME and methylene blue, but was decreased by pretreatment with indomethacin in intact endothelium. Response of PE contraction had no significant change in Ca2 free, but Etomidate significantly attenuated the response of PE contraction to Ca2 entry. CONCLUSIONS: We have found that vasodilation produced by etomidate is endothelium-dependent and this effect is related with cyclooxygenase inhibition and also guanylate cyclase activation. In addition, a relaxation effect is caused by an extracellular Ca2 influx blockade through receptor-operated calcium channels.
Animals ; Aorta, Thoracic* ; Arteries ; Calcium Channels ; Endothelium ; Etomidate* ; Guanylate Cyclase ; Hemodynamics ; Indomethacin ; Methylene Blue ; Muscle, Smooth ; NG-Nitroarginine Methyl Ester ; Phenylephrine ; Prostaglandin-Endoperoxide Synthases ; Rats* ; Relaxation ; Vasodilation

Diabetic neuropathy (DN) is the most common and disabling complication of diabetes that may lead to foot ulcers and limb amputations. Despite widespread awareness of DN, the only effective treatments are glucose control and pain management. A growing body of evidence suggests that DN is characterized by reduction of vascularity in peripheral nerves and deficiency in neurotrophic and angiogenic factors. Previous studies have tried to introduce neurotrophic or angiogenic factors in the form of protein or gene for therapy, but the effect was not significant. Recent studies have shown that bone marrow (BM)-derived stem or progenitor cells have favorable effects on the repair of cardiovascular diseases. Since these BM-derived stem or progenitor cells contain various angiogenic and neurotrophic factors, these cells have been attempted for treating experimental DN, and turned out to be effective for reversing various manifestations of experimental DN. These evidences suggest that cell therapy, affecting both vascular and neural components, can represent a novel therapeutic option for treatment of clinical DN.
Adult ; Amputation ; Angiogenesis Inducing Agents ; Bone Marrow ; Cardiovascular Diseases ; Diabetic Neuropathies ; Extremities ; Foot Ulcer ; Glucose ; Humans ; Nerve Growth Factors ; Pain Management ; Peripheral Nerves ; Stem Cells ; Tissue Therapy

BACKGROUND: This study aimed to assess the recent changes of radiation therapy (RT) modalities in Korea. In particular, we focused on intensity-modulated radiation therapy (IMRT) utilization as the main index, presenting the application status of advanced RT. METHODS: We collected information from the Korean Health and Insurance Review and Assessment Service data based on the National Health Insurance Service claims and reimbursements records by using treatment codes from 2010 to 2016. We classified locating region of each institution as capital vs. non-capital areas and metropolitan vs. non-metropolitan areas to assess the regional difference in IMRT utilization in Korea. RESULTS: IMRT use has been steadily increased in Korea, with an annual increase estimate (AIE) of 37.9% from 2011 to 2016 (P < 0.001) resulting in IMRT being the second most common RT modality following three-dimensional conformal radiotherapy. In general, an increasing trend of IMRT utilization was observed, regardless of the region. The rate of AIE in the capital areas or metropolitan areas was higher than that in non-capital areas or non-metropolitan areas (40.7% vs. 31.9%; P < 0.001 and 39.7% vs. 29.4%; P < 0.001, respectively). DISCUSSION: The result of our survey showed that IMRT has become one of the most common RT modalities. IMRT is becoming popular in both metropolitan and non-metropolitan areas, while metropolitan area has faster AIE possibly due to concentration of medical resources and movement of advanced patients.
Humans ; Insurance ; Korea ; National Health Programs ; Radiotherapy ; Radiotherapy, Conformal

Liver involvement in multiple myeloma has been known to be common in autopsied series. However, since its clinical significance is uncertain yet, invasive procedure confirming plasma cell infiltration of the liver has been rarely performed. We report a case with multiple myeloma which had plasma cell infiltration of a liver. A peritoneoscopic biopsy of the liver for the purpose of disclosing the nature of the aggravating liver function in a carrier of the hepatitis B virus showed infiltration of lymphoreticular cell which were identified later as lambda-light chain producing primitive plasma cells by immunohistochemical stain.
Adult ; Case Report ; Female ; Human ; *Laparoscopy ; Liver/*pathology ; Multiple Myeloma/*pathology/radiography/radionuclide imaging ; Neoplasm Invasiveness ; Tomography, X-Ray Computed

BACKGROUND: The analgesic mechanisms of cyclooxygenase (COX)-2 inhibitors have been explained mainly on the basis of the inhibition of prostaglandin biosynthesis. However, several lines of evidence suggest that their analgesic effects are mediated through serotonergic or adrenergic transmissions. We investigated the roles of these neurotransmitters in the antinociception of a selective COX-2 inhibitor at the spinal level. METHODS: DUP-697, a selective COX-2 inhibitor, was delivered through an intrathecal catheter to male Sprague-Dawley rats to examine its effect on the flinching responses evoked by formalin injection into the hindpaw. Subsequently, the effects of intrathecal pretreatment with dihydroergocristine, prazosin, and yohimbine, which are serotonergic, alpha1 adrenergic and alpha2 adrenergic receptor antagonists, respectively, on the analgesia induced by DUP-697 were assessed. RESULTS: Intrathecal DUP-697 reduced the flinching response evoked by formalin injection during phase 1 and 2. But, intrathecal dihydroergocristine, prazosin, and yohimbine had little effect on the antinociception of intrathecal DUP-697 during both phases of the formalin test. CONCLUSIONS: Intrathecal DUP-697, a selective COX-2 inhibitor, effectively relieved inflammatory pain in rats. Either the serotonergic or adrenergic transmissions might not be involved in the analgesic activity of COX-2 inhibitors at the spinal level.
Adrenergic Antagonists ; Analgesia ; Animals ; Catheters ; Cyclooxygenase 2 ; Cyclooxygenase 2 Inhibitors ; Dihydroergocristine ; Formaldehyde ; Humans ; Male ; Neurotransmitter Agents ; Prazosin ; Prostaglandin-Endoperoxide Synthases ; Rats ; Rats, Sprague-Dawley ; Receptors, Adrenergic ; Spinal Cord ; Thiophenes ; Yohimbine

The morphologic spectrum of papillary thyroid carcinoma (PTC) is wide, and many different variants have been recognized. Papillary thyroid carcinoma with nodular fasciitis-like stroma (PTC-NFS) is one of the rare variants of a papillary thyroid carcinoma. Because of the rarity of PTC-NFS, the pathogenesis and clinicopathological significance are still unknown. We recently experienced a case of papillary thyroid carcinoma with extensive nodular fasciitis-like stroma. A 45-year-old female was admitted due to thyroid incidentaloma. An sonographic examination revealed well-demarcated nodules in the right lobe of the thyroid. Fine-needle aspiration cytology showed a finding suspicious for PTC, but was not diagnostic. The patient underwent a right hemithyroidectomy with central compartment node dissection. On gross examination, a well-demarcated white solid ovoid mass measuring 1.0 x 1.0 cm was seen at the upper pole of the right thyroid gland. An intraoperative frozen section also was unable to differentiate between a benign and malignant lesion. An histopathological test showed prominent stromal cell proliferation, exuberant myofibroblastic proliferation associated with deposition of dense bands of collagen, and an area of papillary carcinoma merging with the desmoplastic stroma. Finally, the patent was diagnosed as having a papillary thyroid carcinoma with nodular fasciitis-like stroma.
Biopsy, Fine-Needle ; Carcinoma, Papillary ; Collagen ; Female ; Frozen Sections ; Humans ; Middle Aged ; Myofibroblasts ; Stromal Cells ; Thyroid Gland* ; Thyroid Neoplasms* ; Ultrasonography