Acrodysostosis is a rare multiple congenital malformation syndrome with peripheral dysostosis, nasal hypoplasia, mental retardation and growth failure. In 1968, Maroteaux and Malamut first described this disorder in three patients, and thus far now over 30 published cases have been reported worldwide. Radiographic changes include severe shortening of metacarpals, metatarsals, and phalages, cone-shaped epiphyses of the hands and feet, premature fusion of the epiphyses, and advanced bone age. Most patients do relatively well except for the problems of mental deficiency and arthritic complaints. Progressive restriction of movement of the hands, elbows, and spine may occur. Recently, we experienced a case of acrodysostosis in and 11-year-old boy who presented with short stature, short hands and feet, nasal hypoplasia, mental retardation, and typical radiographic findings including severe shortening of metacarpals, metatarsals and phalanges, cone-shaped epiphyses of the hands and feet.
Child ; Dysostoses ; Elbow ; Epiphyses ; Foot ; Hand ; Humans ; Intellectual Disability ; Male ; Metacarpal Bones ; Metatarsal Bones ; Spine

OBJECTIVE: To evaluate the prevalence of drooling in children with cerebral palsy and to determine their parents' needs for adequate treatment. METHOD: 107 children with cerebral palsy who attended to the pediatric rehabilitational treatment center were investigated whether they had the problems related with drooling. For the children with drooling, the severity of drooling was measured with subjective and objective measurement tools, and the types of cerebral palsy were classified. The parents answered to the questionnaire for several problems caused by drooling and whether they wanted treatment for drooling or not. RESULTS: 25 (23%) out of 107 children with cerebral palsy had drooling problems. 16 (64%) of them were spastic quadriplegic, which was the most common type. Parents of 17 (68%) drooling children had much uncomfortable feeling to drooling, and of 21 (84%) parents wanted treatment. In 21 (84%) children, their therapists felt difficulties during their treatment sessions due to drooling. CONCLUSION: 23% of children with cerebral palsy in rehabilitation clinic had drooling and most of their parents wanted treatment for drooling.
Cerebral Palsy* ; Child* ; Humans ; Muscle Spasticity ; Parents ; Prevalence ; Surveys and Questionnaires ; Rehabilitation ; Sialorrhea*

PURPOSE: To describe the MR imaging findings of post-operative change and residual/recurrent tumor following resection of the glioma and to determine whether there are any specific MR imaging findings useful for differentiation of post-operative change from a residual/recurrent tumor. MATERIALS AND METHODS: We retrospectively analysed 71 post-operative follow-up brain MR images of 26 patients who had undergone surgical resection of intracranial glioma. They consisted of 49 MRI studies of 12 patients with post-operative change and22 MRI studies of 14 patients with residual/recurrent tumors. The follow-up MRI examinations were performed fromone to 75 months after tumor resection. The lesion was defined as post-operative change when any enhancing lesion disappeared or diminished during follow-up MRI studies of at least 20 months. The diagnosis of residual/recurrent tumor was established when on MR images, lesion size increased definitively during the follow up period of between four and 66 months ; residual/recurrent tumors were surgically proven in five patients. The shape, degree of contrast enhancement and time of appearance and disappearance of the lesions were analysed. RESULTS: Post-operative change consisted of hemorrhage(n=3), marginal(n=7) and nodular(n=1) enhancement of sugical bed, adjacent dural enhancement(n=9), extracerebral fluid collection(n=4) and only tissue defect(n=1). Hemorrhage was obseved at between two and eight months ; marginal and nodular enhancement of surgical bed were seen at between three and 30 months (usually less than one year) ; dural enhancement and fluid collection were seen at between three and 75 months. Residual/recurrent tumor appeared most frequently as enhancing solid nodules(n=7) or solidand cystic masses(n=2) followed by non-enhancing solid nodules(n=3). In five of seven cases, marginal enhancement of a residual/recurrent tumor appeared after more than one year. CONCLUSION: Marginal and dural enhancement around surgical tissue defects are the most common finding of post-operative change, whereas nodular enhancementis the most frequent finding of the residual/recurrent tumor. Marginal enhancement lasting longer than one year may, however, be an early finding of residual/recurrent tumor.
Brain ; Diagnosis ; Follow-Up Studies ; Glioma* ; Hemorrhage ; Humans ; Magnetic Resonance Imaging*

We evaluated the conditions required for sutures tied to a post for tibial fixation during anterior cruciate ligament (ACL) reconstruction. Harvested porcine tendon was used as a graft material and nonabsorbable suture was used for sutures. Samples were tested for ultimate tensile load and elongation according to thread numbers, knot numbers, suture techniques and stitch numbers. As thread numbers were increased, ultimate tensile load was increased and elongation was decreased. However, more than 4 strands of threads provided the sufficient ultimate tensile load more than 454 N of normal ACL for daily activities. As knot numbers were increased, ultimate tensile load was increased, but elongation was decreased. In terms of failure mode, unraveling occurred 100% in 3 and 4 knots, 81.2% in 5 knots, 54.5% in 6 knots, and 0% in 7 knots. Suture techniques and stitch numbers didn't significantly affect the ultimate tensile load and the elongation. For sutures tied to a post for tibial fixation in ACL reconstruction, more than 4 threads, more than 7 knots, and more than 4 stitches provide adequate ultimate tensile load and elongation.
Anterior Cruciate Ligament ; Anterior Cruciate Ligament Reconstruction* ; Knee ; Suture Techniques* ; Sutures* ; Tendons* ; Transplants

BACKGROUND: Recent studies have demonstrated the benefits of off-pump coronary bypass grafting over the on-pump technique in patients with chronic kidney disease (CKD). To further reduce the risk of acute kidney injury and the need for renal replacement therapy, even in patients undergoing off-pump coronary artery bypass grafting, we adopted protocol-based perioperative management for patients with CKD. METHODS: From December 2012 to March 2015, 265 patients underwent isolated off-pump coronary artery bypass grafting. To analyze renal function in a stable condition, we excluded 12 dialysis-dependent end stage renal failure and 10 emergency or urgent cases. Among the remaining 243 patients, 208 patients had normal kidney function (normal group), and 35 patients had CKD (CKD group). Minimizing contrast exposure, ensuring adequate hydration, using strict drug dosage adjustment, and optimizing hemodynamic status were key elements of the protocol for the CKD group. RESULTS: The risk of acute kidney injury was about ×3 higher in the CKD group than in the normal group (p=0.01). Estimated glomerular filtration rates and serum creatinine levels deteriorated until the third postoperative day in the CKD group. However, by adopting protocol-based perioperative management, this transient renal dysfunction recovered to preoperative levels by the fifth postoperative day without requiring renal replacement therapy in all cases. CONCLUSION: Off-pump coronary bypass surgery combined with this protocol-based perioperative management strategy in patients with non-dialysis-dependent CKD could mostly be performed without renal replacement therapy.
Acute Kidney Injury ; Coronary Artery Bypass ; Coronary Artery Bypass, Off-Pump* ; Creatinine ; Emergencies ; Glomerular Filtration Rate ; Hemodynamics ; Humans ; Kidney ; Perioperative Care ; Renal Insufficiency ; Renal Insufficiency, Chronic* ; Renal Replacement Therapy ; Transplants*

We have used mivacurium in two myasthenic patients, a generalized myasthenia gravis (MG) patient presenting for thymectomy and a Lambert-Eaton myasthenic (LEM) patient for mediastinoscopic lymph node biopsy. Both of them received nitrous oxide/oxygen (1:1)-narcotic-enflurane anesthesia with mivacurium as a muscle relaxant and the neuromuscular blocking effect of mivacurium was monitored continuously through the operation as well as before the induction of anesthesia. The dose of mivacurium for MG patient was 5.5 mg and LEM patient was 12 mg, because MG patient showed more severe clinical symptoms. The response to train-of-four (TOF) ulnar nerve stimulation was recorded using accelography. The onset times to maximal block in MG and LEM patients were 30 and 120 sec, respectively after injection and the recovery times to 25% from maximal block were 117 and 76 min, respectively. Mivacrium would be safe and appropriate for use in myasthenic patients, with relatively small dose under the neuromuscular monitoring.
Anesthesia ; Biopsy ; Humans ; Kinetocardiography ; Lymph Nodes ; Myasthenia Gravis ; Neuromuscular Blockade ; Neuromuscular Monitoring ; Thymectomy ; Ulnar Nerve

A case of gonadoblastoma with dysgerminoma and choriocarcinoma in the right ovary of a 23-year-old woman is reported. A case of gonadoblastoma without a Y chromosome is very rare. She had a 46XX chromosomes, normal genitalia, no history of menstrual irregularities, thereby differing from the other reproted case. The patient had a normal term pregnancy 2 years after surgery and chemotherapy. It is suggested that gonadoblastoma may occur in functionally and morphologically normal gonads. There have been no signs of recurrence or metastasis for 3 years after the first operation.
Choriocarcinoma* ; Drug Therapy ; Dysgerminoma* ; Female ; Genitalia ; Gonadoblastoma* ; Gonads ; Humans ; Karyotype* ; Neoplasm Metastasis ; Ovary* ; Pregnancy ; Recurrence ; Y Chromosome ; Young Adult

Coronary arterial involvement in Takayasu arteritis (TA) is not uncommon. Herein, we describe a case of TA with celiac trunk and superior mesenteric artery occlusion combined with coronary artery disease. Bilateral huge internal thoracic arteries (ITAs) and the inferior mesenteric artery provided the major visceral collateral circulation. After percutaneous intervention to the right coronary artery, off-pump coronary artery bypass grafting for the left coronary territory was done using a right ITA graft and its large side branch because of its relatively minor contribution to the visceral collateral circulation.
Collateral Circulation* ; Coronary Artery Bypass ; Coronary Artery Bypass, Off-Pump ; Coronary Artery Disease ; Coronary Vessels* ; Mammary Arteries* ; Mesenteric Artery, Inferior ; Mesenteric Artery, Superior ; Takayasu Arteritis* ; Transplants

BACKGROUND: The proper use of disinfectants has been a major issue in infection control programs. We performed a survey to evaluate the current usage of disinfectants in Korea. METHODS: A questionnaire was e-mailed to 95 hospitals that had infection control programs and data were collected between 10 and 28 October 2005. The questionnaire included questions about whether the hospital had official procedures for selection and use of disinfectants and an education program for proper use; also included were questions about the general characteristics of the hospitals. The types of disinfectants consisted of antiseptics and disinfectants for medical devices, endoscopes, and environments. The questionnaire was analyzed using SPSS 12.0 program. RESULTS: Thirty-nine (41.1%) of the 95 hospitals responded to the questionnaire. Thirty-six hospitals (84.6%) had an official decision making process in selecting disinfectants in their hospitals. Ten percent povidone iodine (PVI) (94.9% for central line) and alcohol (92.3% for peripheral line) were commonly used for skin preparation for intravenous catheterization. Most hospitals used 10% PVI for clean wound (97.4%), dirty/infected wound (94.8%) and sore wound (61.5%); 3% H2O2 (43.6%) for dirty/infected wound; and saline (25.6%) for sore wound. Waterless alcohol handrubs were used in general ward (92.3%), ICU (97.4%), and OR (43.6%). Over the 60% of the hospitals used sodium hypochlorites for cleansing the floors and bathrooms of patients' rooms. As a disinfectant for endoscopes, peracetic acid (40-60%) was used the most frequently, followed by orth-pthalaldehyde (25-36%), and superoxidized water (8-23%). Most hospital followed the manufactures' guidelines on the exposure time of gastroscopes to disinfectants such as orth-pthalaldehyde (100%) and peracetic acid (96%). But, some hospitals re-used peracetic acid (12.0%) and superoxidized water (28.6%) for a longer duration than was recommended by the manufacturers. Fifty to eighty percents of the hospitals used tap water for rinsing endoscopes after disinfection, and only about 80% of these dried with alcohol after rinsing. Most hospitals (84.6%) had teaching sessions for the proper use of disinfectants more than once a year. CONCLUSION: The current use of disinfectants has been improved since 1999 in its suitability and appropriateness Proper use of disinfectants ensures the safety of invasive and non-invasive medical processes; therefore, the current guidelines for disinfection should be strictly followed by the hospital. And continuous staff education will facilitate the correct use of disinfectants.
Anti-Infective Agents, Local ; Catheterization ; Catheters ; Decision Making ; Disinfectants* ; Disinfection ; Education ; Electronic Mail ; Endoscopes ; Gastroscopes ; Infection Control ; Korea* ; Patients' Rooms ; Peracetic Acid ; Povidone-Iodine ; Skin ; Sodium ; Water ; Wounds and Injuries ; Surveys and Questionnaires

PURPOSE: The purpose of this study was to assess the usefulness of multiplanar reconstruction (MPR) images in the preoperative evaluation of advanced gastric cancer. METHODS: Multidetector-row CT (MDCT) was performed on 61 patients with advanced gastric cancer, and the coronal and sagittal multiplanar images reconstructed from the transaxial data. The combined axial and MPR images were compared to the axial images alone to determine if the image quality and diagnostic accuracy had been improved. RESULTS: The observed image quality of the combined axial and MPR images, graded relative to the axial image alone, was fair in 22 (36.1%), good in 27 (44.2%), and excellent in 12 cases (19.7%). For the T staging, the diagnostic accuracy of combining the axial and MPR images (75.4%) was higher than that of the axial image alone (70.7%). However, there was no significant difference in the accuracies between the two methods (McNeamar test, P>0.05). For specific regions, the diagnostic accuracies of combining the axial and MPR images and the axial image alone were as follows: 90.2, and 73.8% with antral lesser curvature involvement; 93.4, and 75.4% with antral greater curvature involvement; 83.6, and 73.8% with gastric angle involvement; 96.7, and 88.5% with liver left lobe invasion; 90.2, and 83.6% with pancreas head invasion and 96.7, and 85.2% with colon or mesocolon invasion, respectively. CONCLUSION: Combining the axial and MPR images does not improve the depiction of the T staging compared to the axial image alone in advanced gastric cancer. However, combining the axial and MPR images improved the imaging quality and diagnostic accuracy of specific regions where the delineation of the conventional axial image was insufficient. Therefore, combining the axial and MPR images may be very useful in the preoperative evaluation of advanced gastric cancers.
Colon ; Head ; Humans ; Liver ; Mesocolon ; Pancreas ; Stomach Neoplasms*