Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

No abstract available.

PURPOSE: There have been repoty on radiological features of lower respiratory track infection in infants and children caused by RSV(respiratory syncytial virus) in KOREA. The aims of this study were to summarize radiological features of lower respiratory tract infection caused by common viral agents other than RSV and to find any specific radiological features which might provide clue to the etiologic diagnosis. MATERIALS AND METHODS: We retrospectively analyzed radiological features in 51 children with symptoms of lower respiratory tract infection and identification of viral agents(except RSV). They included parainfluenza (n=22), adenovirus(n=16), influenza A(n--11), influenza B(n=2) virus infections. The mean age of the patients was 23 months. RESULTS: Major radiological findings of viral lower respiratory tract infection were bilateral parahilar peribronchial infiltration(62%), bilateral overaeration(60%), atelectasis(59%)(segmental or subsegmental atelectasis(43%), Iobar atelectasis(16%)) and patchy or confluent consolidation(20%). Pleural effusion was seen in only one case and hilar adenopathy was not observed in any of them. In the cares of adeno virus, consolidation was seen in 5 cases(31%) including 3 cases919%) of extensive confluent consolidations and overaerations were less frequent findings(44%) than in other viruses. In 24 patients with radiological follow up for more than 1 week, consolidation improved most rapidly, while was persistent atelectasis. CONCLUSION: The major radiologic features in vital lower respiratory tract infection(except RSV) were overinflation, bilateral peribronchial infiltration and atelectasis. In adenoviral infection, confluent consolidations which are usually seen in bacterial pneumonia were more common findings than in other viral lower respiratory tract infections.
Child ; Diagnosis ; Follow-Up Studies ; Humans ; Infant* ; Influenza, Human ; Korea ; Paramyxoviridae Infections ; Pleural Effusion ; Pneumonia, Bacterial ; Pulmonary Atelectasis ; Respiratory System* ; Respiratory Tract Infections ; Retrospective Studies

One-year-old male Persian cat presented with multiple fractures and no known traumatic history. Marked decrease of bone radiopacity and thin cortices of all long bones were identified on radiography. Tentative diagnosis was osteogenesis imperfecta, a congenital disorder characterized by fragile bone. To determine bone mineral density (BMD), quantitative computed tomography (QCT) was performed. The QCT results revealed a mean trabecular BMD of vertebral bodies of 149.9 ± 86.5 mg/cm³. After bisphosphonate therapy, BMD of the same site increased significantly (218.5 ± 117.1 mg/cm³, p < 0.05). QCT was a useful diagnostic tool to diagnose osteopenia and quantify response to medical treatment.
Animals ; Bone Density ; Bone Diseases, Metabolic ; Cats* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diagnosis ; Fractures, Multiple ; Humans ; Male ; Osteogenesis Imperfecta ; Osteogenesis* ; Radiography

Hyperplasia of the gingivae may occur as a secondary reaction in the treatment of epilepsy with diphenylhydantoin sodium. This study is intended to investigate the incidence and dharacteristics of gingival hyperplasia in 124 epileptic patients taking diphenylhydantoin longstandingly. The results are as follows: 1. The incidence of diphenylhydantoin induced gingival hyperplasia is approximately 69.4%. 2. The individual tolerance or sensitivity may determine the incidence and severity of gingival hyperplasia and are not directly related with the duration of drug use and its dosage. 3. Gingival hyperplasia is more frequent in female patients and younger patients than male and older. 4. There is no direct relationship betwwen occurance of gingival hyperplasia and meticulous oral hygiene. 5. The mechanism involved in a causation of diphenylhydantoin induced gingival hyperplasia has not been elucidated. 6. We recommand that diphenylhydantoin may be prescribed as the drug of the first choice for the control of epileptic seizure in the adolescent female patients and children especially receiving certain dental treatments.
Adolescent ; Child ; Epilepsy ; Female ; Gingiva ; Gingival Hyperplasia* ; Humans ; Hyperplasia ; Incidence ; Male ; Oral Hygiene ; Phenytoin ; Sodium

The objectives of this study were to investigate the efficacy and dose-effect relationship for glycopyrrolate in the suppression of the oculocardiac reflex, and to compare glycopyrrolate with the reported equivalent dose of atropine. Glycopyrrolate 5 and 7.5ug/kg and atropine 10 and 15ug/kg were studied in 40 paediatric patients to assess more fully the dose of glycopyrrolate required for adequate prevention of the oculocardiac reflex. The results obtained were summarized as follows: 1) The increase in the mean heart rate 5 min after the test drug was statistically significant in all of the four groups. 2) Both drugs showed a dose-related response for reduction in slowing of heart rate after rectus muscle traction. 3) There is great individual variation in response to muscle traction within each group, no group being fully protected from marked slowing in heart rate. 4) The frequency of arrhythmias with low doses of both drugs was more than in high doses with both drugs.
Anesthesia* ; Arrhythmias, Cardiac ; Atropine* ; Child* ; Glycopyrrolate* ; Heart Rate* ; Heart* ; Humans ; Reflex, Oculocardiac ; Traction

PURPOSE: The aims of present study were to document the incidence of cytomegalovirus infection in neonatal and infantile periods especially in high-risk patients of associated infection by nested PCR of DNA from leukocytes and to evaluate the effectiveness of nested PCR in the diagnosis of congenital cytomegalovirus infection. METHODS: Genomic DNA was extracted from peripheral leukocytes of 204 neonates and infants during the period from June 1994 to March 1995, and nested PCR was performed. The cases were divided into six groups according to clinical profiles; Group IA-healthy fullterm babies, group IB-fullterm babies with suspected or definite perinatal infection, group IIA-healthy preterm babies, group IIB-preterm babies with suspected or definite perinatal infection, group III-infants with clinical features of viral infection and abnormal liver function tests, group IV-IgM seropositive babies with clinical evidence of cytomegalovirus infection. RESULTS: In group IA(n=70), 2 cases were positive for PCR amplification (2.9%). All of the cases in group IB(n=64), group IIA(n=15), and group IIB(n=20) were negative for cytomegalovirus infection. Only one case(3.3%) among group III(n=30) showed positive reaction, while all of the five cases(100%) in group IV(n=5) were positive. CONCLUSIONS: The incidence of congenital cytomegalovirus infection in our analysis were 1.2% (2/169), and it was similar to that of western countries. Nested PCR was proved to be a both sensitive and specific diagnostic tool in the diagnosis of cytomegalovirus infection and it seems to be useful in the screening of congenital cytomegaloviral infection in the newborn period. Future analysis of samples including non-blood origin from adult women and newborns will be helpful in the epidemiologic analysis of cytomegalovius infection in this period.
Adult ; Cytomegalovirus Infections* ; Cytomegalovirus* ; Diagnosis* ; DNA ; Female ; Humans ; Incidence ; Infant ; Infant, Newborn ; Leukocytes ; Liver Function Tests ; Mass Screening ; Polymerase Chain Reaction*

As the use computed tomographic (CT) scanning spread, the diagnosis of blowout fractures of the medial orbital wall increased. Now, the diagnosis of blowout fracture in the medial wall are not uncommon. Conventionally, the surgery of blowout fractures in medial orbital wall was performed by the various approach with external incision. The conventional method had seveal possible disadvantages, including an external scar, incomplete reduction, increased mobidity rate and general anesthesia. Recently, endoscopic reconstruction of the medial orbital wall has provided good functional and cosmetic results. We performed endoscopic transnasal reduction surgery without external incision in 12 cases of medial blowout fracture under local anesthesia. The fractured bony fragments were removed after the intranasal ethmoidectomy and the entrapped medial rectus was released. And then a sheet of silicone late or uncinate process were placed on the fracture site. For the maintain of the position of fractured wall, Merocel packing or urinary ballon catheter were used in orbital fracture site for 1-3 weeks. There were no specific complications related to this procedure. Result of the surgery in all cases were satisfactory. In this article, we discussed the surgical procedure, the benifit of the transnasal endoscopic approach, the indications for surgery, and possible comlications.
Anesthesia, General ; Anesthesia, Local ; Catheters ; Cicatrix ; Diagnosis ; Orbit* ; Orbital Fractures ; Silicones

The tracheobronchial histopathologic findings in 7 healthy cats used with high frequency oscillation ventilation (HFOV) were compared with those in 6 cats used with conventional mechanical ventilation (CMV). 4-point, 9-variable scoring system was used to evaluate the injury in the trachea, right & left main bronchi and parenchyma. The following results were obtained; 1) The tracheobronchial tree received HFOV had no significant damage compared with CMV (P>0.05). 2) Intraepithelial mucus loss and emphysema were slightly more prominent in CMV groups. As above results; the tracheobronchial histopathologic difference was not prominent between CMV and HFOV groups received with relatively short period, however, the cellular of function and barotrauma may be more prominent in CMV groups. From now on, as causes of tracheobronchial injury in HFV, interaction between humidification and mechanical trauma considers further study.
Animals ; Barotrauma ; Bronchi ; Cats* ; Emphysema ; High-Frequency Ventilation* ; Mucus ; Respiration, Artificial* ; Trachea ; Trees

The purpose of this study were to determine that dexamethasone(Dex) induces differentiation of periodontal ligament(PDL) cells to osteoblastic cells and to investigate expression of matrix Gla protein(MGP), which is one of bone matrix protein. The isolated human PDL cells and gingival fibroblasts were prepared and cultured. The fourth or sixth subpassage cells were used in this experiments. control group, ascorbic acid and beta-glycerophosphate treated group, ascorbic acid, beta-glycerophosphate and 100nM Dex treated group, ascorbic acid, beta-glycerophosphate, and 5micrometer Dex treated group were made for study. The results were as follows: Cellular morphological change of PDL cells according to time was investigated. At first, the cells exhibited confluent monolayer of spindle or polygonal appearance. The multilayer of cells were seen after 7 days of treatment. After 14 days, the cells lost polarity and were densely packed. The mineralized nodule formation was seen at 21 days in the only Dex treated PDL cell groups. In the gingival fibroblast groups and no Dex treated PDL cell groups, the mineralized nodule was not seen. The mineralized nodule formation of 5micrometer Dex treated group was higher than 100 nM Dex treated group. Alkaline phosphatase(ALP) activity was higher in the Dex treated PDL cell groups of 14 and 21 days than 0 and 7 days. MGP was expressed in the control and all experimental groups and the expression was constant at 0, 7, 14, 21 day. The above results confirm that Dex is affected to differentiation of the PDL cells to osteoblastic or cementoblastic cells and has dose-dependent effect for mineralization. And, MGP is expressed in the PDL cells and is not affected to mineralization of PDL cells.
Ascorbic Acid ; Bone Matrix* ; Dental Cementum ; Dexamethasone* ; Fibroblasts ; Gene Expression* ; Humans ; Osteoblasts ; Periodontal Ligament*