Rett syndrome is progressive neurodegenerative disorder in female patients, characterized by autistic behavior, mental retardation, loss of purposeful hand skills, stereotypic hand movement, breathing dysfunction, severely impaired language, ataxia, and seizure. The diagnosis of Rett syndrome is based on its characteristic clinical manifestation and course. The electroencephalographic (EEG) findings of Rett syndrome are nonspecific, but a progressive deterioration in the EEG, characterized by a slowing of background activity and spike sharp wave discharges, may be observed. We experienced one case of Rett syndrome in a 5 year old girl having mental retardation, loss of purposeful hand skills, stereotypic hand movements (clapping, washing, hand-to-mouth), breathing dysfunction (hyperventilation/apnea). Her EEG findings on Video-EEG monitoring are excessive slowing waves during awake state and frequent spike discharges from left or centrotemporal area during sleeping. We report a case of Rett syndrome with brief review of related literatures.
Ataxia ; Child, Preschool ; Diagnosis ; Electroencephalography ; Female ; Hand ; Humans ; Intellectual Disability ; Neurodegenerative Diseases ; Respiration ; Rett Syndrome* ; Seizures

PURPOSE: This study was attempted to grasp the factors affecting the decision-making conflicts of preoperative cancer patients. METHODS: The subjects of this study were 110 cancer patients scheduled to undergo surgeries at K university hospital. Data were collected utilizing scales for decision-making conflicts and anxiety state, and measurement tools for attitude toward treatment and, shared decision-making. RESULTS: The level of decision-making conflicts over cancer treatment of preoperative cancer patients turned out to be slightly lower than the median. There were significant correlations between shared decision-making between physicians and patients, and quality of life which was a subdomain of attitude toward treatment. In multiple regression analysis, the most powerful predictor was the shared decision-making of physicians and patients. Overall, the explanatory power of the measured variables for decisionmaking conflicts about cancer therapy was 20%. CONCLUSION: The study highlights the importance of shared decision-making and quality of life which involves attitude toward treatment. Thereby, it is deemed to be necessary to develop intervention strategies regarding decision-making conflicts about cancer treatment in consideration of these variables.
Anxiety ; Decision Making* ; Drug Therapy* ; Hand Strength ; Humans ; Quality of Life ; Weights and Measures

No abstract available.
Carney Complex* ; Hyperplasia* ; Myxoma* ; Nevus, Blue* ; Testis*

No abstract available.
Child* ; Fistula* ; Humans

Lipoma is a benign fatty tumor that can arise in any location of the body where fat is present. It is found most commonly in the upper half of the body, particularly the head and neck, shoulders, and back. A mass in the antero-inferior part of the neck may be initially thought to be thyroid masses and then other cervical masses should be considered. Ultrasongraphic examination of benign lipoma demonstrates solid and echogenic mass and may differentiate nonthyroid from thyroid masses. Although the location of tumors, its consistency, and its motion with deglutition, seperation from the thyroid on sonographic examination, all pointed to nonthyroidal origin, did not rule out a possible mass that isolated from the lobes of the thyroid. Fine needle aspiration and biopsy can provide clear answer.We herein report a case of anterior cervical mass in a 48-yr-old male patient presenting a non-tender, slightly hard and nodular mass slowly growing for several years and moved with swallowing, and diagnosed his case as benign lipoma using thyroid scan and ultrasonography. When we encounter patients with anterior neck mass, we should consider benign lipoma mimicking diffuse goiter.
Biopsy ; Biopsy, Fine-Needle ; Deglutition ; Goiter ; Head ; Humans ; Lipoma ; Male ; Neck ; Shoulder ; Thyroid Gland ; Ultrasonography

Influenza virus culture was performed in 149 patients with influenza-like illness who were admitted or visited to the Department of Pediatrics, Asan Medical Center from january, 1991 to March, 1991. The results were as follows; 1) Of the 149 patients, influenza virus were isolated in the 15 cases. 15 isolates were characterized by the WHO Collaborating Center for influenza: 7 cases were very similar to influenza A/Taiwan/1/86 (H1N1), 1 case was A/Beijing/353/89 (H3N2), 7 cases were B/Guangdon-g/55/89. 2) The age of 15 patients who were confirmed by viral isolation was between 11 months to 10 years. 3) The most common clinical symptoms were fever, vomiting, cough, nausea in deceasing order. 4) Of the total 149 patients, Reye syndrome occured in two patients and myositis occured in one: Influenza A/Taiwan/1/86 (H1N1) virus was isolated in one Reye syndrome patient.
Chungcheongnam-do ; Cough ; Fever ; Humans ; Influenza, Human* ; Myositis ; Nausea ; Orthomyxoviridae ; Pediatrics ; Reye Syndrome ; Seoul* ; Vomiting

PURPOSE: To investigate correlations of estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor type 2 (HER2) statuses with magnetic resonance imaging (MRI) features and clinicohistological characteristics in patients with invasive lobular carcinoma (ILC). MATERIALS AND METHODS: Data from 64 histologically confirmed ILCs were analyzed retrospectively. Preoperative breast MRI was reviewed for morphology and dynamic contrast-enhanced kinetics of the tumor. Pathologic reports were reviewed for ER, PR, and HER2 positivity, tumor size, lymph node metastasis, and the number of metastatic lymph nodes. Furthermore, there was an investigation of the MRI features and clinicohistologic characteristics, according to the ER, PR, and HER2 statuses. RESULTS: A significant difference in MRI features and clinicohistological tumor characteristics were observed only in relation to PR status. Of the 64 ILCs, 10 (15.6%) were PR negative. PR negative cancers, compared with PR positive cancers, were more likely to present as non-mass enhancement (P = 0.027); have a significantly larger mean tumor size (5.00 +/- 1.05 cm vs. 2.57 +/- 0.21 cm, P = 0.021); and have significantly more metastatic lymph nodes (P = 0.010). CONCLUSIONS: PR negative ILC presented more frequently as non-mass enhancement on MRI, with larger tumors and increased numbers of metastatic lymph nodes. Therefore, the PR status plays an important role in determining MRI features and clinicohistological characteristics of ILC.
Breast ; Carcinoma, Lobular* ; Estrogens ; Humans ; Kinetics ; Lymph Nodes ; Magnetic Resonance Imaging* ; Neoplasm Metastasis ; Receptor, Epidermal Growth Factor ; Receptors, Progesterone ; Retrospective Studies

Composite pheochromocytoma-ganglioneuroma is one of the mixed neuroendocrine-neural tumors composed of pheochromocytoma and other neural crest derivatives. To date, less than 50 cases of composite pheochromocytoma have been reported, and about 70% of the accompanying tumors were ganglioneuromas. Here, we describe six cases of composite pheochromocytoma-ganglioneuromas in five men and one woman, aged 33 to 64. The size of the tumors ranged from 3.0 to 11.0 cm, and four out of the six presented with intermittent onset of hypertension, palpitation, or dizziness. Microscopically, each tumor was composed of large pleomorphic shaped chromaffin cells arranged in the Zellballen patterns characteristic of pheochromocytoma, and they were mixed with clusters of mature ganglion cells and bundles of spindle-shaped Schwann cells characteristic of ganglioneuroma of variable proportions. All were successfully treated laparoscopically, and none were associated with multiple endocrine neoplasm syndrome or neurofibromatosis. Preoperative diagnosis of a composite pheochromocytoma-ganglioneuroma is impossible because of the low incidence rate, and the radiological findings and symptoms are similar to those of typical pheochromocytomas. Although the significance of microscopic detection of the nonpheochromocytoma component from pheochromocytoma has not yet been clarified, microscopic identification of the composite pheochromocytoma-ganglioneuroma is important because cumulative cases are used in an effort to predict the behavior of this composite tumor.
Adrenal Glands ; Aged ; Chromaffin Cells ; Dizziness ; Female ; Ganglion Cysts ; Ganglioneuroma ; Humans ; Hypertension ; Incidence ; Male ; Neural Crest ; Pheochromocytoma ; Schwann Cells

PURPOSE: The aim of this study was to assess the efficacy and safety of recent-generation patent ductus arteriosus (PDA) closure devices applied by a new selection strategy according to the characteristics of each PDA. METHODS: From February 2003 to January 2006, 138 patients underwent transcatheter closure of PDA (study group). According to the size and morphology of each ductus, a COOK Detachable Coil or "flex" PFM Nit-Occlud was used for a small ductus (group 1, n=43); "medium" PFM Nit-Occlud (group 2, n=49) for a moderate ductus; and an Amplatzer Duct Occluder (group 3, n=46) for a large ductus. The 83 patients who underwent transcatheter closure of PDA from February 2000 to January 2003 were defined as the comparison group. The Qp/Qs ratio, pulmonary/aorta pressure ratio, and MD of the ductus were compared. Immediate and follow-up results including residual shunts and complications were also evaluated and compared among groups. RESULTS: In all 138 patients, complete occlusions were confirmed without major complications, while procedure failure (n=2, 2.2%), device embolization (n=1, 1.1%), and persistent residual shunt (n=4, 4.5%) were documented in the comparison group. Total complication rates were lower in the study group than in the comparison group (study group, 1.4%; comparison group, 9.0%; P<0.05). CONCLUSION: A novel strategy adopting the merits of various recent-generation devices for transcatheter closure of PDA provides excellent clinical results with minimal risk.
Cardiac Catheterization ; Ductus Arteriosus ; Ductus Arteriosus, Patent ; Follow-Up Studies ; Humans

PURPOSE: Submucosal gastric carcinomas metastasize to lymph nodes more often than the intramucosal gastric carcinomas. The objectives of this study are to clarify the characteristics of submucosal gastric carcinomas, especially in reference to the status of lymph node metastasis, and to explore the possibility of a minimally invasive operation. MATENRIALS AND METHODS: The clinicopathologic features of 88 patients with submucosal gastric carcinoma, all of whom were treated with a D2+alpha gastrectomy between January 1994 and December 1999, were examined retrospectively with respect to the status of lymph nodes. The size, depth of submucosal invasion, histologic differentiation, location, and macroscopic finding of the tumor were investigated in association with the presence or the absence of lymph node metastasis. RESULTS: Among the 88 patients, 15 (17.05%) had lymph node metastasis, and the status of metastasis was significantly correlated with tumor size and depth of submucosal invasion. The frequency of metastasis was 0% (0/7) of up to 1.0 cm and 18.5% (15/81) over 1.0 cm in size (p=0.034) and 6.1% (2/33) of up to 1.0mm and 23.6% (13/55) over 1.0 mm in depth of submucosal invasion (p=0.042). CONCLUSION: The tumor size and depth of submucosal invasion are useful indicators of lymph node metastasis in submucosal gastric carcinoma. A minimally invasive op-eration can be applied for submucosal gastric carcinoma up to 1.0 cm in size Further studies are needed to limited surgery for depth of submucosal invasion.
Gastrectomy ; Humans ; Lymph Nodes* ; Neoplasm Metastasis* ; Retrospective Studies