No abstract available.
Adenoma* ; Humans ; Infant, Newborn*

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption and characterized histopathologically by the presence of granulomatous inflammation with or without leukocytoclastic vasculitis. PNGD is known to be associated with various immune-mediated connective tissue diseases such as rheumatoid arthritis and lupus erythematosus. However, to our knowledge, a case of PNGD in a patient with Behçet’s disease is extremely rare and only one case has been reported in foreign literature to date. Herein, we report an unusual case of a 60-year-old female with Behçet’s disease who presented multiple erythematous to flesh-colored papules on the extremities, buttocks, and ear lobes and was diagnosed with PNGD. After the treatment of systemic corticosteroids, colchicine and azathioprine, the skin lesions and oral ulcers improved. The patient is under observation without recurrence of skin lesions for 6 months.

OBJECTIVE: To analyze the clinical data and surgical results from symptomatic chronic subdural hematoma (CSDH) patients who underwent burr-hole drainage (BHD) at the maximal thickness area and twist-drill craniostomy (TDC) at the precoronal point. METHODS: We analyzed data from 65 symptomatic CSDH patients who underwent TDC at the pre-coronal point or BHD at the maximal thickness area. For TDC, we defined the pre-coronal point to be 1 cm anterior to the coronal suture at the level of the superior temporal line. TDC was performed in patients with CSDH that extended beyond the coronal suture, as confirmed by preoperative CT scans. Medical records, radiological findings, and clinical performance were reviewed and analyzed. RESULTS: Of the 65 CSDH patients, 13/17 (76.4%) with BHD and 42/48 (87.5%) with TDC showed improved clinical performance and radiological findings after surgery. Catheter failure was seen in 1/48 (2.4%) cases of TDC. Five patients (29.4%) in the BHD group and four patients (8.33%) in the TDC group underwent reoperations due to remaining hematomas, and they improved with a second operation, BHD or TDC. CONCLUSION: Both BHD at the maximal thickness area and TDC at the pre-coronal point are safe and effective drainage methods for symptomatic CSDHs with reasonable indications.
Catheters ; Drainage* ; Hematoma ; Hematoma, Subdural, Chronic* ; Humans ; Medical Records ; Sutures ; Tomography, X-Ray Computed

OBJECTIVE: The intracranial pathologies after head trauma should be usually progressed. It is clearly visualized in the non-invasive brain CT. The invasive monitor such as intracranial pressure (ICP) monitoring may be accompanied with the complications. This study aims whether the patients with severe head injury could be managed with serial CT scans. METHODS: The medical records of 113 patients with severe head injury in the prospectively enrolled trauma bank were retrospectively analyzed. After the emergency care, all the patients were admitted to the intensive care unit for the aggressive medical managements. Repeat brain CT scans were routinely taken at 6 hours and 48 hours after the trauma. ICP monitoring was restrictively applied for the uncertain intracranial pressure based on the CT. The surgical intervention and the mortality rate were analyzed. RESULTS: Immediate surgical intervention after the initial CT scan was done in 47 patients. Among the initially non-surgical patients, 59 patients were managed with the serial CT scans and 7 with the ICP monitoring. Surgical interventions underwent eventually for 10 patients in the initially non-surgical patients; 1 in the ICP monitoring and 9 in the serial CT. The mortality rate was 23.7% in the serial brain CT and 28.6% in the ICP monitoring. There was no statistical difference between two groups in the aspect of mortality (p=0.33). CONCLUSION: Serial CT scans in time could be a good way to monitor the intracranial progression in the severe head injury and reduce the implantation of an invasive ICP probe.
Brain* ; Craniocerebral Trauma* ; Emergency Medical Services ; Humans ; Intensive Care Units ; Intracranial Pressure* ; Medical Records ; Mortality ; Pathology ; Prospective Studies ; Retrospective Studies ; Tomography, X-Ray Computed*

PURPOSE: There has been no exact criteria established for when to discontinue antiepileptic drugs (AEDs) in epileptic children who had been well controlled for a long period. This study was undertaken to evaluate the recurrence rate and predictive risk factors of relapse after discontinuation of AEDs in epileptic children who had been seizure-free. METHODS: We retrospectively studied 294 children whose discontinued AEDs therapy was after a long seizure-free period in Asan Medical Center. RESULTS: Seizure recurred in 59 patients(21.0%) after discontinuation of AEDs. The mean duration of follow-up after discontinuation was 4.0 years for the patients, the mean duration of the medication period was 3.5 years and the mean seizure-free period was 2.8 years. The factors associated with an increasing recurrence risk were old age at onset, long duration of seizure, old age at discontinuation of AEDs and abnormal radiologic findings in univariate analysis. From multivariate analysis, the factors related to higher recurrence rate were old age at onset, long duration of seizure and abnormal radiologic findings. CONCLUSION: The majority of epileptic children who are older than 10 years, who have developed seizures for longer than six years and who show abnormal radiologic findings will easily relapse. The patients who do not have the additional risk factors noted above may have an excellent chance to remain seizure-free after the discontinuation of AEDs, so patient in the latter group can consider the discontinuation of AEDs.
Anticonvulsants* ; Child* ; Chungcheongnam-do ; Epilepsy ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Recurrence* ; Retrospective Studies ; Risk Factors* ; Seizures

Sternocostoclavicular hyperostosis is an uncommon disease, characterized by an inflammatory arthrosteitis of the sternocostoclavicular region. Clinically, it manifests as a painful swelling of the upper anterior chest wall, which is associated with occasional pustulosis palmaris and plantaris. A 48-year-old man had suffered from pain in both shoulders and the upper anterior part of the chest for 6 months. On examination, a venous engorgement in the neck with dilated collateral veins in the upper chest and shoulders was observed. Swelling was noticed in his face, neck and both arms. Radiologically, the clavicles, the sternum and the first ribs were enlarged with complete fusion between them. 99Tc scintigraphy showed increased uptake in the clavicles and the sternum. Selective venography resulted in a bilateral subclavian and brachiocephalic vein occulation, which resulted from a subclavian vein thrombosis. All the above suggested a sternocostoclavicular hyperostosis. He underwent a vascular graft interposition between the right jugular vein and the left innomianate vein (using 8mm ringed Gore-Tex graft) and a resection of the bilateral medial half of clavicle and 1st rib. Here, we present a case on sternoclavicular hyperostosis with subclavian and brachiocephalic vein thrombosis, and report this case study with a review of the appropriate literature.
Arm ; Brachiocephalic Veins ; Clavicle ; Humans ; Hyperemia ; Hyperostosis ; Hyperostosis, Sternocostoclavicular* ; Jugular Veins ; Middle Aged ; Neck ; Phlebography ; Polytetrafluoroethylene ; Radionuclide Imaging ; Ribs ; Shoulder ; Sternum ; Subclavian Vein ; Thoracic Wall ; Thorax ; Thrombosis ; Transplants ; Veins ; Venous Thrombosis*

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), which is caused by mutations in SACS gene, is a very rare neurodegenerative disorder characterized by the clinical triad of early onset cerebellar ataxia, pyramidal tract features, and sensorimotor polyneuropathy. Herein, we report a 35-year-old Korean male who presented with gait disturbance and lower extremity weakness. Neuroimaging and ophthalmologic evaluation revealed features consistent with ARSACS. Mutation in SACS gene was demonstrated in clinical exome sequence analysis and the patient was finally diagnosed as ARSACS.
Adult ; Ataxia ; Cerebellar Ataxia ; Exome ; Gait ; Humans ; Lower Extremity ; Male ; Muscle Spasticity ; Neurodegenerative Diseases ; Neuroimaging ; Polyneuropathies ; Pyramidal Tracts ; Sequence Analysis ; Spinocerebellar Degenerations

PURPOSE: This study evaluated the usefulness of a new scoring system in diagnosing acute appendicitis which expresses the patient's symptoms, physical examination, and laboratory findings more clearly and objectively. METHODS: A prospective study was conducted with 314 patients who were hospitalized with suspicion of acute appendicitis. After analyzing the symptoms, physical examination, and laboratory findings, 10 meaningful variables were selected, each of which were scored separately. The diagnostic value of the new scoring system was evaluated, and analyzed in comparison to the preexisting Alvarado score. RESULTS: Ten variables including vomiting, migration pain, fever, Dunphy's sign, Rovsing's sign, tenderness, rebound tenderness, increased white blood cell counts, increased neutrophil proportion, and increased CRP levels were associated with acute appendicitis. The new scoring system is developed by applying 1 point for each variable, with a total score of 10 points. In the new scoring system, a score above 5 points had sensitivity of 0.75, specificity of 0.73, positive predictive value of 0.92, and diagnostic accuracy of 0.71. The area under the receiver operating characteristic curve was 0.80, which is larger than 0.72 of the preexisting Alvarado score, and thus has a higher diagnostic accuracy. As acute appendicitis progresses, the average score tends to become significantly higher (P=0.001). CONCLUSION: The new scoring system, which objectively reflects the clinical variables of the patient's symptoms, physical examination and laboratory findings, will be useful in accurately diagnosing acute appendicitis and in quickly deciding a therapeutic policy in patients with right lower abdominal pain.
Abdominal Pain ; Appendicitis ; Fever ; Humans ; Leukocyte Count ; Neutrophils ; Physical Examination ; Prospective Studies ; ROC Curve ; Sensitivity and Specificity ; Vomiting

BACKGROUND/AIMS: Mutation of Cationic trypsinogen gene is clearly associated with hereditary pancreatitis and plays an important role in the pathogenesis of pancreatitis. According to literature, this mutation is occasionally occurred in patients with pancreatitis in Western countries and Japan. The aim of this study was to find out whether the mutation was observed in Korean patients with chronic idiopathic pancreatitis. METHODS: Peripheral blood samples of 11 patients with chronic idiopathic pancreatitis were collected consecutively, and DNA was extracted from the samples. Polymerase chain reaction was performed in exon 2 and 3 of cationic trypsinogen gene. Then, DNA products were purified and sequenced. RESULTS: The mutation was not found in exon 2 and 3 of cationic trypsinogen gene in these patients. CONCLUSIONS: There was no cationic trypsinogen mutation in Korean patients with chronic idiopathic pancreatitis. Further large sampled cohort study is needed.
Adolescent ; Adult ; Chronic Disease ; English Abstract ; Female ; Humans ; Male ; Middle Aged ; *Mutation ; Pancreatitis/*genetics ; Polymerase Chain Reaction ; Trypsin/*genetics ; Trypsinogen/*genetics

1alpha,25-dihydroxyvitamin D3 (1,25(OH)2D3), which is the biologically active form of vitamin D, has anti-inflammatory effects and can prevent experimental Parkinson's disease (PD). 1,25(OH)2D3 exerts most of its actions only after it binds to its specific nuclear receptors. Eighty-five Korean patients with PD and 231 unrelated healthy individuals were evaluated to determine if vitamin D receptor gene (VDRG) BsmI polymorphisms were markers for the susceptibility to PD in Korean patients. Each polymorphism was detected using polymerase chain reaction (PCR)-based restriction analysis. In addition, the relationship between the BsmI polymorphisms and the clinical manifestations of PD was evaluated. Overexpression of the b allele (91.2 vs. 85.7%; p=0.069) and homozygote bb (84.7 vs. 72.7%; p=0.043) was found in the PD patients compared with the controls. These results show for the first time an association between PD and a VDRG polymorphism, which might be involved in the pathogenesis of PD, or in the linkage disequilibrium of the VDRG to another pathogenic gene locus.
Aged ; Alleles ; DNA/genetics/metabolism ; Deoxyribonucleases, Type II Site-Specific/metabolism ; Female ; Gene Frequency ; Genotype ; Humans ; Korea ; Linkage Disequilibrium ; Male ; Middle Aged ; Parkinson Disease/*genetics ; *Polymorphism, Genetic ; Receptors, Calcitriol/*genetics