We developed a new blood management protocol that allows patients to not undergo transfusion during major orthopaedic surgery. Here, we report the safety of or our protocol. The preoperative pharmacological protocol consisted of the administration of 40 µg of recombinant erythropoietin subcutaneously and 100 mg of iron supplements intravenously. During the operation, reinfusion of drainage blood using a cell saver and plasma expander was used. The cell saver device passed the collected blood through a filter, which washed the blood, removing the hemolyzed cells and other impurities. Intravenous tranexamic acid 1 g is given just before the operation, except high-risk patients for venous thromboembolism. Postoperatively, recombinant erythropoietin and iron supplements were administered in the same manner with the preoperative protocol and continued until a hemoglobin level reached 10 g/dL.
Drainage ; Erythropoietin ; Humans ; Iron ; Orthopedics ; Plasma ; Tranexamic Acid ; Venous Thromboembolism

Esophageal perforation is a rare but potentially fatal complication of robot-assisted thyroidectomy (RAT). Herein, we report the long-term outcome of an esophageal reconstruction with a jejunal free flap for esophageal rupture after RAT. A 33-year-old woman developed subcutaneous emphysema and hoarseness on postoperative day1 following RAT. Esophageal rupture was diagnosed by computed tomography and endoscopy, and immediate surgical exploration confirmed esophageal rupture, as well as recurrent laryngeal nerve injury. We performed a jejunal free flap repair of the 8-cm defect in the esophagus. End-to-side microvascular anastomoses were created between the right external carotid artery and the jejunal branches of the superior mesenteric artery, and end-to-end anastomosis was performed between the external jugular vein and the jejunal vein. The right recurrent laryngeal nerve injury was repaired with a 4-cm nerve graft from the right ansa cervicalis. Esophagography at 1 year after surgery confirmed that there were no leaks or structures, endoscopy at 1 year confirmed the resolution of vocal cord paralysis, and there were no residual problems with swallowing or speech at a 5-year follow-up examination. RAT requires experienced surgeons with a thorough knowledge of anatomy, as well as adequate resources to quickly and competently address potentially severe complications such as esophageal rupture.
Adult ; Animals ; Carotid Artery, External ; Deglutition ; Endoscopy ; Esophageal Perforation ; Esophagus ; Female ; Follow-Up Studies ; Free Tissue Flaps* ; Hoarseness ; Humans ; Jejunum ; Jugular Veins ; Mesenteric Artery, Superior ; Microsurgery ; Rats ; Recurrent Laryngeal Nerve Injuries ; Rupture* ; Subcutaneous Emphysema ; Surgeons ; Thyroidectomy* ; Transplants ; Veins ; Vocal Cord Paralysis

BACKGROUND AND PURPOSE: Upper respiratory infection (URI), including influenza, may exacerbate the symptoms of myasthenia gravis (MG), which is an autoimmune disease that causes muscle weakness. There is also concern that the influenza vaccine may trigger or worsen autoimmune diseases. The objective of this study was to determine the impacts of influenza infection and vaccination on symptom severity in MG patients. METHODS: Patients diagnosed with MG were enrolled from 10 university-affiliated hospitals between March and August 2015. Subjects completed a questionnaire at the first routine follow-up visit after enrolling in the study. The patient history was obtained to determine whether a URI had been experienced during the previous winter, if an influenza vaccination had been administered before the previous winter, and whether their MG symptoms were exacerbated during or following either a URI or vaccination. Influenza-like illness (ILI) was defined and differentiated from the common cold as a fever of ≥38℃ accompanied by a cough and/or a sore throat. RESULTS: Of the 258 enrolled patients [aged 54.1±15.2 years (mean±SD), 112 men, and 185 with generalized MG], 133 (51.6%) had received an influenza vaccination and 121 (46.9%) had experienced a common cold (96 patients) or ILI (25 patients) during the analysis period. MG symptoms were aggravated in 10 (40%) patients after ILI, whereas only 2 (1.5%) experienced aggravation following influenza vaccination. The rate of symptom aggravation was significantly higher in patients experiencing an ILI (10/25, 40%) than in those with the common cold (15/96, 15.6%, p=0.006). CONCLUSIONS: The results of this study suggest that the potential risk of aggravating autoimmune disease is higher for ILI than for influenza vaccination, which further suggests that influenza vaccination can be offered to patients with MG.
Autoimmune Diseases ; Common Cold ; Cough ; Fever ; Follow-Up Studies ; Humans ; Influenza Vaccines ; Influenza, Human* ; Male ; Muscle Weakness ; Myasthenia Gravis* ; Pharyngitis ; Vaccination*

BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty. RESULTS: The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS. CONCLUSIONS: Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.
Action Potentials ; Antibodies ; Axons* ; Classification ; Diagnosis ; Early Diagnosis ; Electrodiagnosis ; Guillain-Barre Syndrome* ; Humans ; Medical Records ; Neural Conduction ; Retrospective Studies ; Ulnar Nerve ; Upper Extremity*

PURPOSE: Mitochondrial diseases are clinically and genetically heterogeneous disorders, which make their exact diagnosis and classification difficult. The purpose of this study was to identify pathogenic mitochondrial DNA (mtDNA) mutations in 2 Korean families with myoclonic epilepsy with ragged-red fibers (MERRF) and Leigh syndrome, respectively. MATERIALS AND METHODS: Whole mtDNAs were sequenced by the method of mtDNA-targeted next-generation sequencing (NGS). RESULTS: Two causative mtDNA mutations were identified from the NGS data. An m.8344A>G mutation in the tRNA-Lys gene (MT-TK ) was detected in a MERRF patient (family ID: MT132), and an m.9176T>C (p.Leu217Pro) mutation in the mitochondrial ATP6 gene (MT-ATP6) was detected in a Leigh syndrome patient (family ID: MT130). Both mutations, which have been reported several times before in affected individuals, were not found in the control samples. CONCLUSION: This study suggests that mtDNA-targeted NGS will be helpful for the molecular diagnosis of genetically heterogeneous mitochondrial diseases with complex phenotypes.
Classification ; Diagnosis ; DNA, Mitochondrial ; Humans ; Leigh Disease* ; MERRF Syndrome* ; Mitochondrial Diseases ; Phenotype

BACKGROUND/AIMS: Adefovir dipivoxil (ADV) is a nucleotide analogue that is effective against lamivudine-resistant hepatitis B virus (HBV). The aim of this study was to determine the long-term clinical outcomes after ADV rescue therapy in decompensated patients infected with lamivudine-resistant HBV. METHODS: In total, 128 patients with a decompensated state and lamivudine-resistant HBV were treated with ADV at a dosage of 10 mg/day for a median of 33 months in this multicenter cohort study. RESULTS: Following ADV treatment, 86 (72.3%) of 119 patients experienced a decrease in Child-Pugh score of at least 2 points, and the overall end-stage liver disease score decreased from 16+/-5 to 14+/-10 (mean +/- SD, P<0.001) during the follow-up period. With ADV treatment, 67 patients (56.3%) had undetectable serum HBV DNA (detection limit, 0.5 pg/mL). Virologic breakthrough occurred in 38 patients (36.1%) and 9 patients had a suboptimal ADV response. The overall survival rate was 89.9% (107/119), and a suboptimal response to ADV treatment was associated with both no improvement in Child-Pugh score (> or =2 points; P=0.001) and high mortality following ADV rescue therapy (P=0.012). CONCLUSIONS: Three years of ADV treatment was effective and safe in decompensated patients with lamivudine-resistant HBV.
Adenine/*analogs & derivatives/therapeutic use ; Adult ; Aged ; Antiviral Agents/*therapeutic use ; Cohort Studies ; DNA, Viral/blood ; Drug Resistance, Viral ; Female ; Hepatitis B/complications/*drug therapy ; Hepatitis B e Antigens/blood ; Hepatitis B virus/genetics ; Humans ; Lamivudine/*therapeutic use ; Liver Cirrhosis/*diagnosis/etiology/mortality ; Male ; Middle Aged ; Odds Ratio ; Organophosphonates/*therapeutic use ; Retrospective Studies ; Severity of Illness Index ; Survival Rate

The term tumoral calcinosis in used to describe the deposition of nodular calcareous masses in the soft tissue around large joints, such as the hips, shoulders, and elbows. Although the cause has not yet been clearly determined, according to the hypothesis, failure of phosphorus metabolism in the proximal tubule in kidney, chronic renal disease and hyperparathyroidism may cause tumoral calcinosis. No cases of tumoral calcinosis treated with surgical resection in chronic renal failure patients on hemodialysis, so called uremic tumoral calcinosis, have been reported in Korea. The authors experienced the case of a 57-year-old woman with chronic kidney disease on hemodialysis who presented with a mass around the hip. We made a diagnosis using plain radiographs, magnetic resonance imaging, and computed tomography of tumoral calcinosis, and treated the patient successfully with surgical resection. We report on a case of uremic tumoral calcinosis with a review of the literature.
Calcinosis* ; Diagnosis ; Elbow ; Female ; Hip ; Hip Joint* ; Humans ; Hyperparathyroidism ; Joints ; Kidney ; Kidney Failure, Chronic ; Korea ; Magnetic Resonance Imaging ; Metabolism ; Middle Aged ; Phosphorus ; Renal Dialysis* ; Renal Insufficiency, Chronic ; Shoulder

PURPOSE: This study was designed to investigate the characteristics of Korean adrenomyeloneuropathy (AMN) patients. MATERIALS AND METHODS: We retrospectively selected 12 Korean AMN patients diagnosed by clinical analysis and increased plasma content of very long chain fatty acids. RESULTS: All 12 patients were men. Patient ages at symptom onset ranged from 18 to 55 years. Family history was positive in two patients. The phenotype distributions consisted of AMN without cerebral involvement in seven patients, AMN with cerebral involvement in two patients, and the spinocerebellar phenotype in three patients. Nerve conduction studies revealed abnormalities in four patients and visual evoked tests revealed abnormalities in three patients. Somatosensory evoked potential tests revealed central conduction defects in all of the tested patients. Spinal MRI showed diffuse cord atrophy or subtle signal changes in all 12 patients. Brain MRI findings were abnormal in six of the nine tested patients. These brain abnormalities reflected the clinical phenotypes. Mutational analysis identified nine different ABCD1 mutations in 10 of 11 tested patients. Among them, nine have been previously reported and shown to be associated with various phenotypes; one was a novel mutation. CONCLUSION: In conclusion, the present study is the first to report on the clinical and mutational spectrum of Korean AMN patients, and confirms various clinical presentations and the usefulness of brain MRI scan.
ATP-Binding Cassette Transporters/genetics ; Adolescent ; Adrenoleukodystrophy/*diagnosis/*genetics ; Adult ; Brain/pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Republic of Korea ; Young Adult

Tinea incognito (TI) is a dermatophytic infection which has lost its typical clinical appearance because of improper use of steroids or calcineurin inhibitors. The incidence of TI is increasing nowadays. We conducted retrospective review on 283 patients with TI from 25 dermatology training hospitals in Korea from 2002-2010 to investigate the demographical, clinical, and mycological characteristics of TI, and to determine the associated risk factors. More than half (59.3%) patients were previously treated by non-dermatologists or self-treated. The mean duration of TI was 15.0 +/- 25.3 months. The most common clinical manifestations were eczema-like lesion, psoriasis-like, and lupus erythematosus-like lesion. The trunk and face were frequently involved, and 91 patients (32.2%) also had coexisting fungal infections. Among 67 isolated strains, Trichophyton rubrum was the most frequently detected (73.1%). This is the largest study of TI reported to date and the first investigational report concerning TI in Korea. We suggest that doctors should consider TI when a patient has intractable eczema-like lesions accompanied by tinea pedis/unguium. Furthermore, there should be a policy change, which would make over-the-counter high-potency topical steroids less accessible in some countries, including Korea.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Demography ; Eczema/pathology ; Face/pathology ; Female ; Humans ; Lupus Erythematosus, Cutaneous/pathology ; Male ; Middle Aged ; Psoriasis/pathology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Tinea/*diagnosis/microbiology ; Trichophyton/isolation & purification ; Young Adult

No abstract available.
Brain ; Brain Abscess ; Lateral Sinus Thrombosis ; Otitis ; Otitis Media ; Petrositis ; Transverse Sinuses