No abstract available.

No abstract available.

A 4-year-old boy has had a solitary sclerotic depressed plaque on the right anterior chest since birth. The histopathologic findings are consistent with morphea profunda: thickening, hyalinization, and homogenization of collagen bundles in the dermis and subcutaneous tissues, admixture with a prominent lymphocytic and plasma cell infiltrate, and sweat glands en-trapped between the thickened collagen bundles. We report a case of congenital solitary morphea profunda.
Child, Preschool ; Collagen ; Dermis ; Humans ; Hyalin ; Male ; Parturition ; Plasma Cells ; Scleroderma, Localized* ; Subcutaneous Tissue ; Sweat Glands ; Thorax

PURPOSE: This study was performed to investigate the epidemiology of enterovirus (EV) infections in children at a secondary hospital during recent 5 years. METHODS: We collected the cerebrospinal fluid, stool and throat swab samples from the pediatric patients with suspected EV infections in KEPCO Medical Center, Seoul, Korea from July 2006 to September 2010. EV detection and genotype identification were performed by RT-PCR at Korea Centers for Disease Control and Prevention. RESULTS: A total of 386 samples were collected from 277 patients during study period. Ninety-eight patients (35.4%) were diagnosed with EV infections. The RT-PCR positive rate was the highest in throat swab samples (48.3%). The median age of patient was 4.7 years (range, 0.1-12.5 years). Aseptic meningitis (50, 51.0%) was the most common clinical manifestation; herpangina (22, 22.4%) and hand-foot-mouth disease (18, 18.4%). One hundred EVs were isolated from 98 patients and 20 genotypes of EV were identified; Echovirus 30 (28 cases, 28%), Enterovirus 71 (12 cases, 12%), Echovirus 25 (10 cases, 10%), Echovirus 9 (9 cases, 9%) and Coxsackievirus A6 (8 cases, 8%). Aseptic meningitis caused by Echovirus 30 was the most common manifestation in 2008. There was no complicated case caused by Enterovirus 71. CONCLUSION: This study showed the epidemiology of confirmed EV infection in children from 2006 to 2010. There is a need for continuous surveillance of EV infections and its clinical manifestations.
Centers for Disease Control and Prevention (U.S.) ; Child ; Echovirus 9 ; Enterovirus ; Enterovirus B, Human ; Enterovirus Infections ; Genotype ; Herpangina ; Humans ; Korea ; Meningitis, Aseptic ; Pharynx

PURPOSE:Mutations of the PROP1 (Prophet of PIT1) gene lead to combined pituitary hormone deficiency (CPHD), which is characterized by a deficiency of growth hormone (GH), thyroid stimulating hormone (TSH), luteinizing hormone (LH)/follicle stimulating hormone (FSH), prolactin (PRL) and adrenocorticotropic hormone (ACTH). We studied the PROP1 mutations in Korean children with hypopituitarism. METHODS:Twelve patients with congenital hypopituitarism were recruited from the Ajou University Hospital, Korea. The pituitary phenotype ranged from isolated growth hormone deficiency (IGHD) to CPHD. Clinical data, including endocrine and neuroradiological data were obtained from the medical records, and the DNA was collected and screened for mutations within PROP1 using polymerase chain reaction (PCR). The PCR products were sequenced directly. RESULTS:Nine patients had abnormal pituitary gland and three patients showed normal pituitary gland in magnetic resonance imaging (MRI). Endocrinologically, seven patients had IGHD and five had CPHD. Three of CPHD had GHD and hypogonadotrophic hypogonadism and the other two had GHD, central hypothyroidism, and ACTH deficiency. We identified no mutations in the PROP1 gene. However, three known polymorphisms were identified: The 27T->C (Ala9Ala) and the associated IVS1+3A->G in exon 1 were found in 25% of patient alleles sequenced. The 424G->A (Ala142Thr) in exon 3 change was identified in 4% of those sequenced. CONCLUSION:This study corresponds to the previous reports that PROP1 mutations are rare in sporadic cases of CPHD. The low mutation frequency in Korean patients may be due to ethnic-specificity or other candidate genes causing this disease. It is necessary to study PROP1 gene and the involved genes in more Korean patients.
Adrenocorticotropic Hormone ; Alleles ; Child ; DNA ; Exons ; Growth Hormone ; Humans ; Hypogonadism ; Hypopituitarism ; Hypothyroidism ; Korea ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Medical Records ; Mutation Rate ; Phenotype ; Pituitary Gland ; Polymerase Chain Reaction ; Prolactin ; Thyrotropin

No abstract available.
Animals ; Brain* ; Cats* ; Magnetic Resonance Spectroscopy*

No abstract available.
Thanatophoric Dysplasia*

BACKGROUND: Unexpected deaths of the patients at ED are surprising to the medical staffs as well as the families. It may also increase the possibilities of medico-legal problems. This study was conducted to review the unexpected death in ED and find the predictive factors leading patients to unexpected death. METHODS: A retrospective study with reviewing available medical records of 183 patients who were expired at ED of Shinchon Severance Hospital from Mar. 1997 to Feb. 1998 and 103 patients of Youngdong Severance Hospital from Mar. 1996 to Feb. 1998 was done. Analyses were 4one on 30 variables of physical findings, laboratory results, procedures and therapies. We used univariate analysis, such as t-test and x2-test and multiple logistic regression analysis. RESULTS: Of the total 286 deaths in ED, 153 cases were enrolled in this analysis. The number of unexpected deaths were 76. Age, mental status, fraction of neutrophil count, BUN level, whether endotracheal incubation was done, use of inotropics were the factors with statistical ,significance on the univariate analysis, but only comatose mentality, endotracheal incubated case, and use of the inotropics were significant on logistic regression analysis. The longer the ED length of stay, the higher the rate of mortality and unexpected death. CONCLUSION: This result suggest that medical attention is needed on the patients with comatose decreased mental stylus, endotracheally intubated case and needing inotropic agents to prevent unexpected deaths. Shortening the ED length of stay may also be a factor for decrease the unexpected death rate.
Coma ; Emergencies* ; Emergency Service, Hospital* ; Humans ; Length of Stay ; Logistic Models ; Medical Records ; Medical Staff ; Mortality ; Neutrophils ; Retrospective Studies

We report a 36-year-old male with the bilateral visual field defect following LASIK was observed. Preoperatively, patient had high myopia(right. -7.25 0.75x18, left. -7.5-0.5x12), normal intraocular pressure(IOP)(right/left: 16/14 mmHg), normal visual field(Humphrey 30-2 visual field)and revealed the tilted discs with symetric optic nerve head cupping(cup-to-disc ratio, 0.70), parapapillary atrophy. Bilateral LASIK keratorefractive surgery was on the same day and intraoperative complication was not noted. After the first operative visit day, examination revealed uncorreted visual acuity right 0.8 and left 0.6 but scotoma was reported. Three months later, Humphrey 30-2 visual field revealed a near-superior altitudinal defect in the right eye and nasal side visual field defect in the left eye. Best corrected visual acuity was 1.0 in the right eye, 0.8 in the left eye. Repeated visual fields over a 11-month period postoperatively showed stability of the defect, with IOP of 12 mmHg to 15 mmHg in each eye. We considered LASIK uses a microkeratome vaccum ring to make a corneal flap, transient elevation of IOP alters the microcirculation of optic nerve head and may have precipitated visual field defect.
Adult ; Atrophy ; Humans ; Intraoperative Complications ; Keratomileusis, Laser In Situ ; Male ; Microcirculation ; Optic Disk ; Scotoma ; Visual Acuity ; Visual Fields*

PURPOSE: Hypertrophic osteoarthropathy(HOA) is a clinical syndrome consisting of clubbing, periostitis and synovitis. Most frequent causes of hypertrophic osteoarthropathy are intrathoracic neoplasms, among which the bronchogenic carcinoma ranks the highest. But computed tomographic evaluation of intrathoracic neoplasm associated with HOA has been seldom reported. The purpose of this study is to evaluate CT findings of intrathoracic neoplasm associated with HOA, and to infer possible mechanism. MATERIALS AND METHODS: Seven cases of intrathoracic neoplasm associated with HOA were included in our study. Diagnoses of HOA were made by Tc99m bone scintigraphy or plain radiography. The findings of chest CT scans were reviewed retrospectively, with main interests on their size, location and internal characteristics, ect. RESULTS: Seven cases of intrathoracic neoplasm consisted of five bronchogenic carcinomas and two thymic tumors. The size of intrathoracic tumors were relativelY, large ranging from 6cm to 13cm(average 8.0cm). All thoracic neoplasms showed wide pleural contact, and one of them invaded thoracic wall. The range of length of pleural contact was 5-18cm(average 9.9cm). All of seven patients had internal necrosis, and one of them showed cavitation in thoracic mass. CONCLUSION: lntrathoracic neoplasms associated with HOA had a tendency to be large, to contain internal necrosis, and to widely abut the thoracic pleura.
Carcinoma, Bronchogenic ; Diagnosis ; Humans ; Necrosis ; Periostitis ; Pleura ; Radiography ; Radionuclide Imaging ; Retrospective Studies ; Synovitis ; Thoracic Neoplasms ; Thoracic Wall ; Thymus Neoplasms ; Tomography, X-Ray Computed