Apert described acrocephalosyndactyly as a clinical entity in 1906. At first he included a patient with craniofacial deformities combined with syndactyly of both the upper and lower extremities. This is a rare congenital disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. More than 200 cases have been reported in the world literature, and three cases in Korea. Authors experienced one case of the classic pattern of Apert's syndrome and treated surgically for syndsctyly.
Acrocephalosyndactylia ; Congenital Abnormalities ; Foot ; Hand ; Head ; Humans ; Korea ; Lower Extremity ; Syndactyly

BACKGROUND: The goal of successful resuscitation is not only to stop the process of ischemia as soon as possible but also to overcome the secondary injury process after resuscitation, which involves a complex interplay of mechanisms. Brain damage accompanying cardiac arrest and resuscitation is frequent and devastating. Cells die by one of two mechanisms: necrosis or delayed neuronal death. Delayed neuronal death may require protein synthesis. Neurons in the CA1 subfield of the hippocampus are selectively vulnerable to death after injury by ischemia and reperfusion. Death of these neurons occurs after an interval of 1 or 2 days. We assessed the effects of a protein synthesis inhibitor, cycloheximide(CHX), on hippocampal neuronal death of rats by using the ventricular fibrillation cardiac arrest(VFCA) model. METHODS: The effect of CHX(3mg/kg, s.c.) on hippocampal neuronal death was studied in two groups of 18 rats each, one group being subjected to a 2-min VFCA and the other to a 3-min VFCA. Each group was divided into three subgroups: control(group I,II) without subcutaneous injection of CHX, 'exp-12' of group I/II treated with CHX 12 hours after return of spontaneous circulation (ROSC), and 'exp-24' of group I/II treated with CHX 24 hours after ROSC. The coronal sections of the hippocampus levels were stained with hematoxylin-eosin after 72 hours of survival. The histologic damage score(HDS) was used to assign a score to the total number of damaged neurons counted in each of the hippocampal CA1 subfields. RESULTS: 1. There were not significan differences in heart rates, blood pressures, blood sugar, and blood gas in group I & II during the pre-arrest steady state or at 5 min and 30 min after ROSC. 2. In group I & II, the HDS, were significantly reduced in rats(I exp-12, 1.1+/-0.6; I exp-24, 1.3+/-0.5; II exp-12, 1.4+/-0.7; and II exp-24, 1.8+/-0.8) treated with CHX 12 hours or 24 hours after ROSC than control rats(I, 2.5+/-0.9, II, 2.9+/-0.8)(p<0.05). CONCLUSION: These results suggest that delayed hippocampal neuronal death from ischemic insult after ventricular fibrillation cardiac arrest followed by resuscitation can be prevented by a protein synthesis inhibitor, CHX. Further experimental studies of the action mechanism of protein synthesis inhibitors to delayed neuronal death and clinical applications are required.
Animals ; Blood Glucose ; Brain ; Heart Arrest* ; Heart Rate ; Hippocampus ; Injections, Subcutaneous ; Ischemia ; Necrosis ; Neurons* ; Protein Synthesis Inhibitors ; Rats* ; Reperfusion ; Resuscitation ; Ventricular Fibrillation*

No abstract available.
Zygoma*

No abstract available.
Femur Neck* ; Fractures, Stress*

No abstract available.

The interstitial lung diseases(ILD) comprises a large, heterogeneous group of disorders characterized by derangement of alveolar walls and alveolar capillary unit causing restrictive pulmonary dysfunction and disordered gas exchange. It is rare, especially in children, and the opinions on investigation and treatments are controversial. The classification of adult ILD is applied to that of children. But considering occurrence during the developing state of lung and immune systems, we should be more cautious because the clinical manifestations and prognosis will be different from those of adults. In 1995, Katzenstein named a unique type of ILD, chronic pneumonitis of infancy (CPI) for the first time. CPI is characterized by marked alveolar septal thickening, striking type II pneumocyte hyperplasia, and eosinophilic alveolar exudate containing numerous macrophages. We report a 14 month-old girl, who had continuing respiratory difficulty and cyanosis after upper respiratory infection, could not sustain oxygen saturation and was finally diagnosed as CPI by histologic examination.
Adult ; Capillaries ; Child ; Classification ; Cyanosis ; Eosinophils ; Exudates and Transudates ; Female ; Humans ; Hyperplasia ; Immune System ; Infant ; Lung ; Lung Diseases, Interstitial* ; Macrophages ; Oxygen ; Pneumocytes ; Pneumonia* ; Prognosis ; Strikes, Employee

Hemorrhagic metastatic brain tumors of hepatocellular carcinoma (HCC) are rare and have been mostly presented as intracranial hemorrhage (ICH). A 51-year-old male patient presented with sudden altered level of consciousness. He suffered from HCC since 2010 and transarterial chemoembolization was performed three times for HCC. The brain computed tomography (CT) scans revealed subdural hematoma (SDH) in the right fronto-temporal area and 6.0x3.5 cm sized ICH in the right parieto-occipital lobe. Brain angiographic CT scans demonstrated that the hemorrhagic lesions did not include any enhancing lesions and vascular abnormalities. We undertook a decompressive craniectomy and evacuation of the acute SDH and ICH. During evacuation of ICH, the yellowish mass was observed in the cortical surface of the right occipital lobe. Pathological examination displayed the findings of metastatic brain tumor from HCC. Metastatic brain tumors should be considered in the differential diagnosis as a cause of spontaneous SDH with ICH.
Brain Neoplasms ; Brain* ; Carcinoma, Hepatocellular* ; Cerebral Hemorrhage* ; Consciousness Disorders ; Decompressive Craniectomy ; Diagnosis, Differential ; Hematoma, Subdural* ; Humans ; Intracranial Hemorrhages ; Male ; Middle Aged ; Neoplasm Metastasis* ; Occipital Lobe ; Tomography, X-Ray Computed

PURPOSE: To report the relative frequency and clinical characteristics of patients with benign eyelid tumors. METHODS: A retrospective study of 192 consecutive patients admitted to Korea University Ansan Hospital with benign eyelid tumor between January 2009 and December 2014 was undertaken, and clinical records including age, sex, involved site, and pathology of tumors were reviewed retrospectively. All eyelid tumors were confirmed histopathologically. RESULTS: The sexual distribution revealed 87 males and 105 females with benign eyelid tumors. The mean age at diagnosis was 42.6 +/- 19.2 years. Molluscum contagiosum (5.5 +/- 3.5 years) and pilomatrixoma (14.0 +/- 15.6 years) were generally found in younger individuals, while seborrheic keratosis (60.2 +/- 15.8 years) and squamous cell papilloma (50.5 +/- 13.4 years) occurred predominantly in elderly patients. Tumors were most common on the upper lid (63.0%). The four most frequent subtypes were melanocytic nevus (37.5%), epidermal cyst (8.3%), squamous cell papilloma (5.7%), and seborrheic keratosis (5.2%). CONCLUSIONS: The most common histopathological diagnosis of benign eyelid tumors was melanocytic nevus. The results of this study provide epidemiological information that will be useful for diagnosis and therapy of such tumors.
Aged ; Diagnosis ; Epidermal Cyst ; Eyelids* ; Female ; Gyeonggi-do ; Humans ; Keratosis, Seborrheic ; Korea ; Male ; Molluscum Contagiosum ; Nevus, Pigmented ; Papilloma ; Pathology ; Pilomatrixoma ; Retrospective Studies

Meningioma is a common primary tumor of central nervous system. However, extracranial extension of the intracranial meningioma is unusual, and mostly accompanied the osteolytic change of the skull. We herein describe an atypical meningioma having extracranial extension with hyperostotic change of the skull. The patient was a 72-year-old woman who presented a large mass in the right frontal scalp and left hemiparesis. Brain magnetic resonance imaging and computed tomography scans revealed an intracranial mass, diffuse meningeal thickening, hyperostotic change of the skull with focal extension into the right frontal scalp. She underwent total removal of extracranial tumor, bifrontal craniectomy, and partial removal of intracranial tumor followed by cranioplasty. Tumor pathology was confirmed as atypical meningioma, and she received adjuvant radiotherapy. In this report, we present and discuss a meningioma en plaque of atypical histopathology having an extracranial extension with diffuse intracranial growth and hyperostotic change of the skull.
Aged ; Brain ; Central Nervous System ; Female ; Humans ; Magnetic Resonance Imaging ; Meningioma* ; Osteoblasts* ; Paresis ; Pathology ; Radiotherapy, Adjuvant ; Scalp ; Skull*