The cleidocranial dysostosis is characterized by deficient formation of the clavicle, delayed and imperfect ossification of the cranium and less often involvement of other bones. The cleidocranial dysostosis is relatively rare congenital and familial disorder with autosomal dominant inheritance. We report one case of cleidocranial dysostosis with a brief review of the literatures
Clavicle ; Cleidocranial Dysplasia ; Skull ; Wills

No abstract available.
Femur* ; Fracture Fixation, Intramedullary* ; Tibia*

A 51-year-old male patient was referred for a sudden out-of-hospital cardiac arrest. Upon arrival, he was conscious and had no chest pain complaints. There was no abnormality in initial electrocardiographic and echocardiographic examinations. However, episodes of recurrent ventricular fibrillation (VF) were documented on rhythm monitoring. Each VF episode was triggered by an isolated monomorphic ventricular premature complex (VPC). Suspecting idiopathic VF, emergency radiofrequency catheter ablation was planned for the VPCs. However, when coronary angiography was performed to exclude silent ischemia, the results showed a total occlusion of the right coronary artery posterolateral branch, which is thought to supply the left ventricular inferior and septal wall. After successful reperfusion, VF episodes and the triggering VPCs disappeared. We are documenting this case to emphasize the potential for silent myocardial infarction to cause out-of-hospital sudden cardiac arrest even in a patient without any symptom or sign of acute coronary syndrome.
Acute Coronary Syndrome ; Catheter Ablation ; Chest Pain ; Coronary Angiography ; Coronary Vessels ; Death, Sudden, Cardiac ; Echocardiography ; Electrocardiography ; Emergencies ; Heart Arrest* ; Humans ; Ischemia ; Male ; Middle Aged ; Myocardial Infarction* ; Out-of-Hospital Cardiac Arrest ; Reperfusion ; Ventricular Fibrillation* ; Ventricular Premature Complexes*

OBJECTIVES: Rapidly progres s ive glomerulonephritis (RPGN) is a clinico- pathologic entity characterized by extens ive crescent formation(usually involving 50% or more of glomeruli) as the principal his tologic finding and a rapid deterioration of kidney function, which can lead to end s tage renal disease within a few weeks. T he etiology and incidence of RPGN has been well defined in Europe and North America, however, there has been no report of a large series in Korea. T he aim of the present s tudy was to analyze the etiology and clinico- pathologic features of 26 patients with RPGN, seen during 1983-1997. METHODS: T wenty-six patients with RPGN(crescents in > 50% of glomeruli) were obs erved during a period of las t 14 years. Male to female ratio was 1:1.4, and the mean age was 30(6-75) years. Mean time from the initial symptoms to the ESRD was 3.1 months . RESULTS: The incidence of RPGN in our series was 2.1% of primary glomerulonephritis. Immunecomplex mediated disease was presented in 14 cases (54%), including 6 sys temic lupus erythematos us, 3 post- streptococcal glomerulonephritis, 3 Henoch- Schonlein purpura, and 2 IgA nephropathy. Pauci- immune disease was presented in 12 cases (46%), including 3 Wegener' s granulomatos is, one necrotizing crescentic glomerulonephritis, and 8 idiopathic crescentic glomerulonephritis. However, there was none of anti-GBM- mediated disease in our s tudy. ANCA were found in 6 patients. All 3 patients with WG were C- ANCA pos itive, whereas one patient with PSGN, necrotizing cres centic GN, and idiopathic crescentic GN were P- ANCA pos itive, respectively. Initial clinical and laboratory features included edema(80%), hypertens ion(72%), oliguria(68%), a decreased renal function(serum creatinine > 5mg/dL, 35%), and gros s hematuria(36%). Renal biopsy showed large crescents more than 80% of the glomeruli in 14 cases (54%) which were predominantly fibrocellular. Fifteen patients (58%) were treated with prednis olone alone, and 12 of them received puls e doses of corticosteroids. Five patients were treated with prednisolone and cyclophos phamide IV pulse. Two cases received plasma exchange. During the mean follow-up of 31+/-37 months, 18 patients (69%) developed inexorable progression of renal failure, three(12%) showed recovery of renal function, and two(8%) showed partial improvement, which is followed by varying degrees of renal insufficiency. During follow-up, three patients died : two from res piratory failure with severe pulmonary hemorrhage and one from opportunistic pulmonary infection during immunosuppressive therapy. Poor prognos is is as sociated with hypertension, increased serum creatinine level at the time of diagnosis, large crescents more than 85% of glomeruli, and glomerular scleros is . CONCLUSION: We conclude that an earlier diagnos is including kidney biopsy and the more aggressive treatment are essential in the management of RPGN.
Adrenal Cortex Hormones ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Creatinine ; Diagnosis ; Europe ; Female ; Follow-Up Studies ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Hemorrhage ; Humans ; Hypertension ; Immune System Diseases ; Incidence ; Kidney ; Kidney Failure, Chronic ; Korea ; Male ; North America ; Plasma Exchange ; Prednisolone ; Purpura ; Renal Insufficiency ; Systemic Vasculitis

Purpose: Height at 3 years of age, when catch-up growth based on birth history is completed, is considered a major prognostic factor for predicting short stature, underweight, and growth faltering. However, too few large-scale studies have followed and analyzed height, weight, and body mass index (BMI) changes in children whose stature was short at 3 years of age. This study followed and compared the growth parameters (height, weight, and BMI) of children with short stature at 3 years of age and children with nonshort stature at 3 years of age for 6 years after birth using nationwide, population-based data. Methods: We retrospectively analyzed physical measurement data from the National Health Screening Program for Infants and Children for people born in 2011–2014 in Korea and followed to 2020. The data were provided by the National Health Insurance Service’s customized data service. Growth parameters were compared using chi-square tests, Student t-tests, analyses of variance, and linear regressions. Results: Among 210,902 enrolled participants, 759 (0.4%) and 210,143 (99.6%) were in the short stature at 3 years group and the nonshort stature at 3 years group, respectively. In both sexes, height, weight, and BMI for 6 years after birth were significantly higher in the nonshort stature at 3 years group than in the short stature at 3 years group (P<0.0001). The BMI rebound was observed later than the standard period in the short stature at 3 years group. Conclusion Early intervention and close follow-up are necessary to prevent persistent short stature and growth faltering in children with short stature at 3 years of age.

Schwannoma is a benign tumor rarely found in the head and neck and much less commonly found in the intraparotid facial nerve. It is a slow-growing encapsulated tumor originating from the Schwann cells or axonal nerve sheath. It can occur anywhere along the course of the facial nerve. Patients may present with symptoms of facial palsy, but the most common presenting symptom is an asymptomatic swelling. Diagnosis is usually difficult before surgical removal and histopathological examination. We report a rare case of intraparotid facial nerve schwannoma in a 57-year-old female who had sustained a mass of the right preauricular area for 3 years. She reported no pain or facial muscle weakness. Enhanced computed tomography findings revealed the impression of pleomorphic adenoma. However, intraoperative gross findings were not characteristic of pleomorphic adenoma, and a frozen biopsy was performed resulting in the impression of a nerve sheath tumor. We performed an extracapsular surgical excision without parotidectomy. Permanent histopathology and immunohistochemistry reports diagnosed the mass as schwannoma. There were no complications including facial palsy after surgery. No recurrence was found at 6 months after surgery
Adenoma, Pleomorphic ; Axons ; Biopsy ; Diagnosis ; Facial Muscles ; Facial Nerve ; Facial Paralysis ; Female ; Head ; Humans ; Immunohistochemistry ; Middle Aged ; Neck ; Neurilemmoma ; Parotid Gland ; Recurrence ; Schwann Cells

PURPOSE: The purpose of this study was to retrospectively analyze the etiologic transition of the predisposing factors and organisms for septic arthritis of the knee. MATERIALS AND METHODS: Between January 1995 and December 2006, 122 cases of septic arthritis of the knee were retrospectively evaluated for the etiologic transition and causative organism with using the patients' medical records. We researched the incidence and causative factor of septic arthritis of the knee, which was diagnosed via the patients' symptoms, physical examinations, hematologic exams, culture studies & cytology of the joint fluid. We assessed the correlation of disease with age, the year the disease happened, the causative factors and the incidence. RESULTS: Septic arthritis of the knee was increased after 40 years old, and it also increased after 1998. The most common predisposing factor was intra-articular injection of the knee joint. Even though there were no detected organisms in 79 cases (64.8%), the most common causative organism was S. aureus (20.5%). CONCLUSION: The most common etiologic factor of septic arthritis of the knee was an intra-articular injection. We should be prudent for using good aseptic technique and the correct procedure to reduce the secondary infection that's recently due to increased invasive treatment of the knee joint.
Arthritis, Infectious ; Coinfection ; Incidence ; Injections, Intra-Articular ; Joints ; Knee ; Knee Joint ; Medical Records ; Physical Examination ; Retrospective Studies

OBJECTIVE: Post-traumatic stress disorder (PTSD) is a group of diseases that are observed in patients who had experienced a serious trauma or accident. However, some experienced it even after only a mild traumatic brain injury (TBI), and they are easily ignored due to the relatively favorable course of mild TBI. Herein, the authors investigated the incidence of PTSD in mild TBI using brief neuropsychological screening test (PTSD checklist, PCL). METHODS: This study was conducted on patients with mild TBI (Glasgow coma scale > or =13) who were admitted from January 2012 to December 2012. As for PCL, it was done on patients who showed no difficulties in communication upon admission and agreed to participate in this study. By using sum of PCL, the patients were divided into high-risk group and low-risk group. PTSD was diagnosed as the three major symptoms of PTSD according to the Diagnostic and Statistical Manual of Mental Disorders, fourth-edifion. RESULTS: A total of 314 TBI patients were admitted and 71 of them met the criteria and were included in this study. The mean age was 52.9 years-old (range: 15-94). The mean PCL score was 28.8 (range: 17-68), and 10 patients were classified as high-risk group. During follow-up, 2 patients (2.7%) of high risk group, were confirmed as PTSD and there was no patient who was suspected of PTSD in the low-risk group (p=0.017). CONCLUSION: PTSD is observed 2.8% in mild TBI. Although PTSD after mild TBI is rare, PCL could be considered as a useful tool for screening of PTSD after mild TBI.
Brain Injuries* ; Checklist ; Coma ; Diagnostic and Statistical Manual of Mental Disorders ; Follow-Up Studies ; Humans ; Incidence* ; Mass Screening* ; Stress Disorders, Post-Traumatic*

OBJECTIVE: Helicopter ambulance transport (HAT) is a highly resource-intensive facility that is a well-established part of the trauma transport system in many developed countries. Here, we review the benefit of HAT for neurosurgical patients in Korea. METHODS: This retrospective study followed neurotrauma patients who were transferred by HAT to a single emergency trauma center over a period of 2 years. The clinical benefits of HAT were measured according to the necessity of emergency surgical intervention and the differences in the time taken to transport patients by ground ambulance transport (GAT) and HAT. RESULTS: Ninety-nine patients were transferred to a single university hospital using HAT, of whom 32 were taken to the neurosurgery department. Of these 32 patients, 10 (31.3%) needed neurosurgical intervention, 14 (43.8%) needed non-neurosurgical intervention, 3 (9.4%) required both, and 11 (34.4%) did not require any intervention. The transfer time was faster using HAT than the estimated time needed for GAT, although for a relatively close distance (<50 km) without ground obstacles (mountain or sea) HAT did not improve transfer time. The cost comparison showed that HAT was more expensive than GAT (3,292,000 vs. 84,000 KRW, p<0.001). CONCLUSION: In this Korean-based study, we found that HAT has a clinical benefit for neurotrauma cases involving a transfer from a distant site or an isolated area. A more precise triage for using HAT should be considered to prevent overuse of this expensive transport method.
Air Ambulances* ; Ambulances ; Developed Countries ; Emergencies ; Humans ; Korea ; Neurosurgery ; Retrospective Studies ; Trauma Centers ; Triage

Simultaneous or subsequent bilateral thalamic hemorrhagic events have ranged from 12 to 19 in prior reports, with a time lag between bilateral thalamic hemorrhage of up to two days. Herein, we report the first case of delayed (17 days) consecutive contralateral thalamic hemorrhage after spontaneous first thalamic hemorrhage. A 65-year-old female initially presented with a drowsy mentality with a left-side motor weakness (grade II/III). Brain computed tomography (CT) demonstrated right side thalamic and intraventricular hemorrhage. She regained alertness with mild residual motor weakness (grade III/IV) under medical management. Seventeen days later, a sudden and generalized tonic-clonic seizure developed. Brain CT scans revealed a new contralateral thalamic hemorrhage coincident with microbleeds. Neurologic status remained unchanged, consisting of a stuporous mentality with quadriparesis of grade II/II. We report the first case of delayed consecutive contralateral thalamic hemorrhage up to 17 days after first thalamic hemorrhage. The case highlights the need for close monitoring of patients with thalamic hemorrhage who experience microbleeds on the contralateral side, due to the possibility of delayed hemorrhage.
Aged ; Brain ; Female ; Hemorrhage* ; Humans ; Hypertension ; Intracranial Hemorrhages ; Quadriplegia ; Seizures ; Stupor ; Thalamus ; Tomography, X-Ray Computed