Hallermann-Streiff syndrome is a rare disease; approximately 150 cases have been reported in the world literature. The syndrome consists of proportionate nanism; hypotrichosis; atrophy and extreme thinness of skin, particulary over the facial area; an usual "bird-like" face with mandibular hypoplasia; a prominent thin, pointed nose; congenital cataracts; and severe dental abnormalities. We report a case of Hallermann-Streiff syndrome in premature who showed intrauterine growth retardation with proportionate nanism, brachycephaly, a beaked nose, "bird like" face, hypoplasia of the mandible, microphthalmia, congenital cataract, neonatal teeth, and widening of sagittal suture were all found on our patient.
Animals ; Atrophy ; Beak ; Cataract ; Craniosynostoses ; Dwarfism ; Fetal Growth Retardation ; Hallermann's Syndrome* ; Humans ; Hypotrichosis ; Mandible ; Microphthalmos ; Nose ; Rare Diseases ; Skin ; Sutures ; Thinness ; Tooth

PURPOSE: Our study was undertaken to evaluate the efficacy of gastric aspirate examination (GAE) in the early identification of early onset sepsis. METHODS: A retrospective chart review was done for a total 291 neonates who were admitted with high risk for early onset sepsis to Nowon Eulji Hospital neonatal intensive care unit between Jan. 1999 and Jan. 2003. Contents of gastric aspirate via nasogastric tube were analyzed with wet smear, gram stain and culture. Positive blood culuture was our definitive criterion for sepsis. GAE was considered positive when five or more leukocytes per high power field were counted on wet smear or when any bacteria were present on gram stain or when any organism grew on culture. RESULTS: Positive results in gastric aspirate examination are 57 patients (19.6%) in wet smear, 28 patients (9.6%) in gram stain, 30 patients (10.3%) in culture and positive result in blood is 27 patients (9.3%). There are no statistically correlation between gastric aspirate examinations and early onset sepsis. CONCLUSION: We concluded that gastric aspirate examinations are of limited value in predicting the diagnosis of early onset sepsis in neonate.
Bacteria ; Diagnosis ; Humans ; Infant, Newborn ; Intensive Care, Neonatal ; Leukocytes ; Retrospective Studies ; Sepsis*

PURPOSE: Sensitization to eggs in infancy has been reported to be associated with increasing the risk of respiratory allergic diseases during childhood. So our study was designed to follow up children who have had atopic dermatitis with egg allergy and to evaluate the prognosis according to family history, early egg elimination diet, house dust mite sensitization, egg/histamine wheel ratio of skin prick test, RAST class and age tolerance to find the risk factors which might be helpful for applying some preventable measures. METHODS: Twenty three children diagnosed with egg allergy at Kangnam General Hospital between March 1988 and March 2000 were studied retrospectively. At the time of the first visit, we interviewed an feeding methods, past medical history, atopic family histories and performed skin prick tests, and total IgE and egg-white specific IgE tests (RAST). At the time of the last visit, we evaluated whether the children had allergic diseases or not, as well as prognosis and other allergen sensitization. RESULTS: Four (17.4%) children resolved allergic symptoms, Fifteen (65.2%) children had atopic dermatitis, 11 (47.8%) children had asthma, and 12 children (52.2%) had allergic rhinitis. Prognosis was good in children with early tolerance and no sensitization to house dust mites. There was no relationship between prognosis and atopic family history, early egg elimination diet, infant feeding methods, or RAST class. CONCLUSION: Because 19 (82.6%) of 23 children with egg allergy have persistent allergic diseases and related to sensitization of house dust mites, early environment intervention for house dust mites must be evaluated to prevent other allergic diseases.
Asthma ; Child* ; Comorbidity* ; Dermatitis, Atopic ; Diet ; Egg Hypersensitivity* ; Eggs ; Feeding Methods ; Follow-Up Studies* ; Hospitals, General ; Humans ; Immunoglobulin E ; Infant ; Ovum* ; Prognosis* ; Pyroglyphidae ; Retrospective Studies ; Rhinitis ; Risk Factors ; Skin

PURPOSE: Transglutaminase 4 (TGase 4) belongs to a family of enzymes that catalyzes the post-translational modification of proteins. In an attempt to establish its physiological function(s), the distribution of TGase 4 expression in the human eye was determined. METHODS: Ocular tissues obtained from five human whole eyeball postmortem (40(+1) weeks at gestation age, 2 months, 48, 66, 76 years) were stained with monoclonal antibodies against human TGase 4 using indirect immunohistochemical method. RESULTS: TGase 4 was found in the lacrimal glands, corneal epithelium and endothelium, conjunctival epithelium, lens epithelium, retina (inner segment of photoreceptor, external limiting membrane, outer plexiform layer, inner plexiform layer, retinal nerve fiber layer and internal limiting membrane), iris, ciliary muscle, ciliary nonpigmented epithelium and trabecular meshwork. Endothelium of blood vessels in all ocular tissues was also stained. Conjunctival stroma, choroid, anterior tenon's capsule were faintly stained. No evidence of immunostaining for TGase 4 was found in the corneal stroma, iris stroma, lens nucleus, ciliary process, sclera, extraocular muscle and optic nerve. CONCLUSIONS: The expression pattern of TGase 4 was different from those of other TGase isoforms in the human eye. This result may be helpful in further investigation of the role of TGase 4 in the ocular tissue.
Antibodies, Monoclonal ; Blood Vessels ; Choroid ; Corneal Stroma ; Endothelium ; Epithelium ; Epithelium, Corneal ; Humans* ; Iris ; Lacrimal Apparatus ; Membranes ; Nerve Fibers ; Optic Nerve ; Pregnancy ; Protein Isoforms ; Protein Processing, Post-Translational ; Retina ; Retinaldehyde ; Sclera ; Tenon Capsule ; Trabecular Meshwork

Moyamoya disease is characterized by bilateral stenosis or occlusion of distal internal carotid artery (ICA) bifurcation including its proximal branches and abnormal vascular network (moyamoya vessel, MMV) in the vicinity of the arterial occlusions. It is the most common pediatric cerebrovascular disease in Eastern Asia, particularly in Korea and Japan. The etiology is still unknown, but much about the pathology from autopsies, factors involved in its pathogenesis, and its genetics have been studied and reported. It may cause ischemic attacks or cerebral infarctions in children and cerebral hemorrhage in adults. Because of its aggressive clinical course in very young children, the need for early detection and treatment has been recognized. Magnetic resonance imaging (MRI)/MR angiography (MRA), cerebral hemodynamic studies, and cerebral angiography are used for the diagnosis. The treatment basically focuses on prevention of further ischemia and infarction through revascularization. Technically, direct and indirect bypass methods are used. The treatment strategy needs to be individualized in each patient. Outcomes of revascularization procedures are excellent in preventing transient ischemic attacks (TIAs) in most patients.
Adult ; Angiography ; Autopsy ; Carotid Artery, Internal ; Cerebral Angiography ; Cerebral Hemorrhage ; Cerebral Infarction ; Child ; Constriction, Pathologic ; Diagnosis ; Far East ; Genetics ; Hemodynamics ; Humans ; Infarction ; Ischemia ; Ischemic Attack, Transient ; Japan ; Korea ; Magnetic Resonance Imaging ; Moyamoya Disease* ; Pathology

OBJECTIVE: Surfactant protein (SP)-A and SP-D are involved in host defense mechanism. The author was prompted to perform a study on morphologic change and SP-A, SP-D level of surfactant after pulmonary injury inflicted by intratracheal bleomycin injection. METHODS: Fifteen white adult rats each weighing 250 g (Sprague-Daw ley) were divided into study (receiving bleomycin, n=9) and control groups (n=6). Study group were given a intratracheal injection of belomycin (5 mg/kg). Two groups were grown for five weeks at twenty five degrees Celsius, after which lung tissue were examined for morphologic change and SP-A and SP-D levels were measured using Western blot assay with densitometer. RESULTS: Before the study, the average weight of the study group was 286.69+/-14.54 g, and control was 286.69+/-14.54 g. Five weeks later, the average weight of the study group was 347.31.31+/-60.53 g and control group 352.71+/-16.84 g. However, no statistical significance was noted. On light microscopy, the control group exhibited normal findings while widening of lung interstitium and fibrotic change coupled with more prominent inflammatory cell infiltration were noted in the study group. The SP-A level were 15.34+/-1.52 ODU/microgram in the study group and 7.70+/-2.81 ODU/microgram in the control. SP-D level were 3.53+/-1.46 ODU/microgram and 7.51+/-2.33 ODU/microgram in the study and control groups respectively, there was a statistical significance (p<0.05). CONCLUSION: The morphologic change after pulmonary fibrosis induced by intratracheal bleomycin injection in white rats can be summarized as chronic inflammatory cell infiltration, fibroblast proliferation, deposition of collagen tissues, and lowering of SP-D level were noted. The increase of SP-A level is subject to further study in the future.
Adult ; Animals ; Bleomycin ; Blotting, Western ; Collagen ; Fibroblasts ; Humans ; Lung ; Lung Injury ; Microscopy ; Pulmonary Fibrosis* ; Pulmonary Surfactant-Associated Protein D ; Rats*

OBJECTIVE: The aim of this study was to investigate the prevalence of spinal deformities such as scoliosis, thoracic kyphosis and lumbar lordosis in Korean elementary school students. METHOD: Five hundred forty Korean elementary school students were preliminary screened for spinal deformities by physical examinations and the three dimensional skeletal analysis system. The study time and pain regions were investigated in sitting on chairs that commonly used in Korean elementary school. RESULTS: The prevalence of scoliosis, thoracic kyphosis and lumbar lordosis were 9.8%, 18.7% and 23.1%. There was a significant difference in study time between spinal deformity group and control group (p<0.01). In spinal deformity group, pain regions were low back (45%), posterior neck (23%), buttock (21%) and shoulder and elbow joints (11%). CONCLUSION: The spinal deformities showed high prevalence in elementary school students assessed by the three dimensional skeletal system analyser. The long term follow-up evaluation and confirmative radiographic study will be necessary to assess curve progression in spinal deformity group.
Animals ; Buttocks ; Congenital Abnormalities* ; Elbow Joint ; Humans ; Kyphosis ; Lordosis ; Neck ; Physical Examination ; Prevalence ; Scoliosis ; Shoulder

PURPOSE: This study was designed to compare the amount of polymerization shrinkage of dual-cure resin cements according to different polymerization modes and to determine the effect of light activation on the degree of polymerization. MATERIALS AND METHODS: Four kinds of dual-cure resin cements were investigated: Smartcem 2, Panavia F 2.0, Clearfil SA Luting and Zirconite. Each material was tested in three different polymerization modes: self-polymerization only, immediate light polymerization and 5 minutes-delayed light polymerization. The time-dependent polymerization shrinkage-strain was evaluated for 30 minutes by Bonded-disk method at 37degrees C. Five recordings of each material with three different modes were taken. Data were analyzed using one-way ANOVA and multiple comparison Scheffe'test (alpha=.05). RESULTS: All materials, except Panavia F 2.0, exhibited the highest polymerization shrinkage-strain through delayed light-activated polymerization. No significant difference between light activation modes was found with Panavia F 2.0. All materials exhibited more than 90% of polymerization rate in the immediate or delayed light activated group within 10 minutes. CONCLUSION: As a clinical implication of this study, the application of delayed light activation mode to dual-cure resin cements is advantageous in terms of degree of polymerization.
Polymerization* ; Polymers* ; Resin Cements*

The objective of this study is to differentiate the mild dementia of Alzheimer type from the questionable dementia and non-demented elderly using the neurocognitive assessment. Subjects of 28 women who were registered to kwangju Community Mental Health Center were as follows: 14 non-demented, 9 questionable dementia, 5 mild dementia of Alzheimer type. The diagnosis were made using DSM-IV, Clinical Dementia Rating Scale. The neurocognitive functions were assessed with following test tools 1) attention: Digit span, Visual span, Continuous attention; 2) memory: Logical memroy, Verbal paired associates-easy/hard 3) visual perception and visuospatial ability: Visual recognition test, Construction; and 4) language: Comprehension and Aphasia severity rating scale; 5) higher cortical function: Hypothesis formation, Perseveration, Similarity, Judgment, and Go-No-Go test. Group differences were analyzed with one way ANOVA test in SPSS 8.0 for win and LSD method as post-hoc analysis. The questionable dementia group showed significant difference in Verbal paired associateseasy pair, Construction, Aphasia severity rating scale and Similarity from the non-demented normal control group but showed no difference from the mildly demented group. These results suggest that the questionable dementia is actually very early or very mild stage of dementia of the Alzheimer type.
Aged* ; Aphasia ; Comprehension ; Dementia* ; Diagnosis ; Diagnostic and Statistical Manual of Mental Disorders ; Female ; Gwangju ; Humans ; Judgment ; Logic ; Lysergic Acid Diethylamide ; Memory ; Mental Health ; Visual Perception

Objective: : Filum transection is one of the most commonly performed operative procedure in pediatric neurosurgery. However, the clinical and pathological features as well as the surgical indication are not well-established. This study aimed to analyze the characteristics of patients who underwent transection of the filum during the last 10 years in a single institute. Methods: : A total of 82 patients underwent transection of the filum during the period. As a general rule, we performed the transection in patients who are symptomatic or have abnormality in the urologic or neuromuscular evaluations. There were exceptions as asymptomatic patients who only fit the definition of thickened filum (width greater than 2.0 mm or conus level below L3 vertebral body) were operated by parent’s wish or surgeon’s preference according to radiological findings, etc. Results: : Seventy-six out of 82 patients had fibrous tissue in the pathologic specimen of filum. Interestingly, patients who had glial cells were more correlated with no preoperative syrinx, and no progression of syrinx even for those who did have syrinx initially. Also, larger percentage of symptomatic patients had peripheral nerve twigs than asymptomatic patients. No difference in conus level or thickness of filum was found between patients with or without preoperative syrinx. Significantly more patients with syrinx (56%) were chosen to be operated without any symptom or abnormality in study i.e., solely based on radiological findings than those without syrinx (21%). The surgical outcome for syrinx was favorable, as all but one patient had either improved or static syrinx. The exceptional case had increase in size due to the upward displacement of the proximal end of the cut filum. Conclusion : This study evaluated the pathological, clinical, radiological features of patients who underwent transection of the filum. Interesting correlations between pathological findings and clinical features were found. Excellent outcome regarding preoperative syrinx was also shown.