In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP) with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.
Autoantibodies ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia* ; Dermatomyositis* ; Dyspnea ; Fibrosis ; Humans ; Immunoglobulins* ; Immunosuppressive Agents ; Inflammation ; Lung Diseases, Interstitial ; Middle Aged ; Pneumonia ; Prognosis ; Respiratory Insufficiency ; Steroids

OBJECTIVE: The purpose of this study was to evaluate the physicochemical properties and cytocompatibility of microporous, spherical biphasic calcium phosphate(BCP) ceramics with a 60/40 hydroxyapatite/beta-tricalcium phosphate weight ratio for application as a bone graft substitute. MATERIALS AND METHODS: Microporous, spherical BCP granules(MGSB) were prepared and their basic characteristics were compared with commercially available BCP(MBCP; Biomatlante, France) and deproteinized bovine bone mineral(Bio-Oss; Geistlich-Pharma, Switzerland, BBP; Oscotec, Korea). Their physicochemical properties were evaluated by scanning electron microscopy, X-ray diffractometry, Fourier-transform infrared spectroscopy, inductively coupled plasma atomic emission spectrometer, and Brunauer-Emmett-Teller method. Cell viability and proliferation of MC3T3-E1 cells on different graft materials were evaluated. RESULTS: MGSB granules showed a chemical composition and crystallinity similar with those in MBCP, they showed surface structure characteristic of three dimensionally, well-interconnected micropores. The results of MTT assay showed increases in cell viablity with increasing incubation times. At 4d of incubation, MGSB, MBCP and BBP showed similar values in optical density, but Bio-Oss exhibited significantly lower optical density compared to other bone substitutes(p < 0.05). MGSB showed significantly greater cell number compared to other bone substitutes at 3, 5, and 7d of incubation(p < 0.05), which were similar with those in polystyrene culture plates. CONCLUSION: These results indicated the suitable physicochemical properties of MGSB granules for application as an effective bone graft substitute, which provided compatible environment for osteoblast cell growth. However, further detailed studies are needed to confirm its biological effects on bone formation in vivo.
Bone Substitutes ; Calcium* ; Cell Count ; Cell Survival ; Ceramics* ; Crystallins ; Microscopy, Electron, Scanning ; Osteoblasts ; Osteogenesis ; Plasma ; Polystyrenes ; Spectrum Analysis ; Switzerland ; Transplants*

In 1965 Gillespie reported a new syndrome of bilateral aniridia, cerebellar ataxia, and oligophrenia (mental retardation). This new syndrome was named Gillespie syndrome. Since then only 17 cases of Gillespie syndrome have been reported in UK, Brazil, Ireland, Belgium, Australia, and US. A case of Gillespie syndrome was not reported in Korea. A 4 year-old girl has triad of Gillespie syndrome, which are partial aniridia, cerebellar ataxia and mental retardation. We confirmed this with ophthalmologic examination, brain MRI, and developmental delay. We report the typical manifestation of Gillespie syndrome in a 4 year-old girl with the brief review of literature.
Aniridia* ; Australia ; Belgium ; Brain ; Brazil ; Cerebellar Ataxia* ; Child, Preschool ; Female ; Humans ; Intellectual Disability ; Ireland ; Korea ; Magnetic Resonance Imaging

In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP) with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.
Autoantibodies ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia ; Dermatomyositis ; Dyspnea ; Fibrosis ; Humans ; Immunoglobulins ; Immunosuppressive Agents ; Inflammation ; Lung Diseases, Interstitial ; Middle Aged ; Pneumonia ; Prognosis ; Respiratory Insufficiency ; Steroids

Open heart surgery under cardiopulmonary bypass (CPB) in patients with liver cirrhosis is prone to the development of various complications associated with high mortality rates. According to recent studies, patients with advanced cirrhosis (Child-Pugh class B or C cirrhosis) have a significantly higher mortality rate (50-100%) after open heart surgery under CPB. We report the anesthetic management of cardiac surgery using CPB of 61-year-old man with aortic valve regurgitation, tricuspid regurgitation and ventricular septal defect (VSD) who had complicated by liver cirrhosis of Child-Pugh class B. The patient underwent successfully aortic valve replacement, tricuspid valvuloplasty and VSD closure. The use of tranexamic acid and transfusion of fresh whole blood appears to produce beneficial effects for outcome.
Aortic Valve ; Cardiopulmonary Bypass ; Fibrosis ; Heart Septal Defects, Ventricular ; Humans ; Liver ; Liver Cirrhosis ; Middle Aged ; Thoracic Surgery ; Tranexamic Acid ; Tricuspid Valve Insufficiency

OBJECTIVE: The use of segmental instrumentation technique using pedicle screw has been increasingly popular in recent years owing to its biomechanical stability. Recently, intralaminar screws have been used as a potentially safer alternative to traditional fusion constructs involving fixation of C2 and the cervicothoracic junction including C7. However, to date, there have been few clinical series of C7 laminar screw fixation in the literature. Thus, the purpose of this study is to report our clinical experiences using C7 laminar screw and the early clinical outcome of this rather new fixation technique. METHODS: Thirteen patients underwent C7 intralaminar fixation to treat lesions from trauma or degenerative disease. Seventeen intralaminar screws were placed at C7. The patients were assessed both clinically and radiographically with postoperative computed tomographic scans. RESULTS: There was no violation of the screw into the spinal canal during the procedure and no neurological worsening or vascular injury from screw placement. The mean clinical and radiographic follow up was about 19 months, at which time there were no cases of screw pull-out, screw fracture or non-union. Complications included two cases of dorsal breech of intralaminar screw and one case of postoperative infection. CONCLUSION: Intralaminar screws can be potentially safe alternative technique for C7 fixation. Even though this technique cannot be used in the cases of C7 laminar fracture, large margin of safety and the ease of screw placement create a niche for this technique in the armamentarium of spine surgeons.
Follow-Up Studies ; Humans ; Spinal Canal ; Spine ; Vascular System Injuries

We report a healthy patient with postpartum headache and neck stiffness which were diagnosed as symptoms of pseudoaneurysm of vertebral artery. She had received a Cesarean section under the spinal anesthesia, and complaint of headache and neck stiffness. Epidural blood patches were done twice, but symptoms persisted. Eight days later, she experienced sensory disturbance and emergent laminectomy was done. When persistent postpartum headache occurs after epidural blood patch, more precise differential diagnosis should be made and considering other possible pathologies.
Anesthesia, Spinal ; Aneurysm, False ; Blood Patch, Epidural* ; Cesarean Section ; Diagnosis, Differential ; Female ; Headache ; Hematoma, Subdural, Spinal* ; Humans ; Laminectomy ; Neck ; Pathology ; Post-Dural Puncture Headache ; Postpartum Period* ; Pregnancy ; Vertebral Artery

Heat-moisture exchanger (HME) is an inexpensive and effective device used to prevent respiratory complications that can be caused by endotracheal tube insertion during general anesthesia. But, HME can increase airway resistance and be occluded by the patient's secretions. Whether a HME could be occluded by clear fluids such as condensate in the airway circuit is not certain yet. In vitro, a case of HME occlusion by normal saline was reported. We report a case of HME obstruction by distilled water came from the heated wire circuit which was unintentionally connected to the HME.
Airway Resistance ; Anesthesia, General ; Hot Temperature ; Porphyrins ; Water

Objective: Geriatric patients requiring gynecological surgery is increasing worldwide. However, older patients are at higher risk of postoperative morbidity and mortality, particularly cardiopulmonary complications. Laparoscopic surgery is widely used as a minimally invasive method for reducing postoperative morbidities. We compared the outcomes of open and laparoscopic gynecologic surgeries in patients older than 55 years. Methods: We included patients aged >55 years who underwent gynecological surgery at a single tertiary center between 2010 and 2020, excluding vaginal or ovarian cancer surgeries were excluded. Surgical outcomes were compared between the open surgery and laparoscopic groups, with age cutoff was set at 65 years for optimal discriminative power. We performed linear or logistic regression analyses to compare the surgical outcomes according to age and operation type. Results: Among 2,983 patients, 28.6% underwent open surgery and 71.4% underwent laparoscopic surgery. Perioperative outcomes of laparoscopic surgery were better than those of open surgery in all groups. In both the open and laparoscopic surgery groups, the older patients showed worse overall surgical outcomes. However, age-related differences in perioperative outcomes were less severe in the laparoscopic group. In the linear regression analysis, the differences in estimated blood loss, transfusion, and hospital stay between the age groups were smaller in the laparoscopy group. Similar results were observed in cancer-only and benign-only cohorts. Conclusion Although the surgical outcomes were worse in the older patients, the difference between age groups was smaller for laparoscopic surgery. Laparoscopic surgery offers more advantages and safety in patients aged >65 years.

Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis has been reported in Graves' disease patients treated with propylthiouracil (PTU). In most cases, it's renal involvements has been known as crescentic glomerulonephritis. A 41-year-old female patient with hyperthyroidism has been treated with PTU for 3 years. The patient had developed isolated hematuria and polyarthralgia with p-ANCA positivity, 6 months and 10 months after PTU treatment, respectively. She had been continuously treated with PTU until she was admitted at our hospital. Three months before admission, polyarthralgia was aggravated and purpura in both lower legs and hands was developed. Urinalysis revealed hematuria, proteinuria. Serologic evaluation showed p-ANCA positive. Skin biopy showed leukocytoclastic vasculitis and renal biopsy showed focal segmental glomerulosclerosis (FSGS). She was diagnosed as PTU-associated vasculitis with FSGS. Polyarthralgia and purpura were improved after discontinuing the PTU with prednisolone treatment but hematuria, proteinuria were not changed. We suggest that progression of PTU-associated focal segmental necrotizing glomerulonephritis to FSGS over two years might be due to continued PTU medication.
Adult ; Antibodies, Antineutrophil Cytoplasmic* ; Arthralgia ; Biopsy ; Cytoplasm ; Female ; Glomerulonephritis ; Glomerulosclerosis, Focal Segmental* ; Graves Disease ; Hand ; Hematuria ; Humans ; Hyperthyroidism ; Leg ; Prednisolone ; Propylthiouracil ; Proteinuria ; Purpura ; Skin ; Urinalysis ; Vasculitis*