Aplasia cutis congenita is a rare disorder characterized by localized or generalized absence of skin at birth. The various hypothesis and classification are proposed. Type V in Frieden's classification, which is associated with fetus papyraceus or placental infarcts, occurs as a large cutaneous defect on the trunk and extremities. We experienced the patient had skin defect affecting the lower abdomen symmetrically, with no family history of the any disorders or chromosomal anomalies and associated anomalies. A twin fetus was died in utero. We report a case of aplasia cutis congenita associated with fetus papyraceus with the review of the associated literatures.
Abdomen ; Classification ; Ectodermal Dysplasia* ; Extremities ; Fetus ; Humans ; Parturition ; Skin ; Twins

This study is comprehend the reality and characteristics of C.V.A. patients and to be helpful to its prevention and cure by resarching C.V.A. outpatient or inpatients who visited some of the oriental hospitals during three months from April 1 to June 30 1996 which are located in Taegu and Kungbuk Province, and concluded as follow: 1. The general characteristics of the subjects were: (1) 52.3% of the subjects were male. (2) In terms of age, 34.7% of them were in the sixties. (3) In terms of job, 28.5% of them were housewives(the highest percentage). (4) 77.6% of them had their spouses. (5) 67.2% of them were middle class. (6) In terms of educational background, 24.6% of them were literate of korean alphabets, and 23.4% were elemantary school gradurates. (7) 51.6% of them were outpatients. 2. 73.3% of the subjects experienced C.V.A. for the first time, and 23.1% were at recurrence, and 3.6% were chronic. 3. In terms of C.V.A. types, 49.8% of the subjects had cerebral hemorrhage, and 41.9% had cerebral infarction, and 8.3% had the others. In cerebral hemorrage, the percentage in "male, forties, job of sales service, unmarried, middle class, high educational background" were higher than the others respectively. And in cerebral infarction, the percentages in female, over seventies, official job, married, upper class were higher than the others respectively. 4. In terms of reasons of C.V.A. 49.5% of them were high blood pressure, and 24.2% were high stress, and 18.8% were overwork, and 4.0% were fatness, and 2.5% were heredity. In high blood pressure, the percentages in "high age, teacher, unmarried, lower class, low educa-tional background" were higher than the others respectively. 5. In terms of family members' C.V.A. , 56.7% of the subjects answered negatively, and 43.3% positively. In terms of the diseases which they had now except for C.V.A., 33.6% of them had hypertention, and 16.2% had diabetes, and 9.4% had neuralgia, and 4.0% had heart disease, and 16.6% had the otehrs, and 20.2% had no other disease. In heart disease, the percentages in "male, teachers, middlelower class, middle school graduates, inpatients" were higher than the others respectively. In neuralgia, the percentages in "fifties and sixties, housewives, spouse alive, upper class, literate of Korean alphabet" were higher than the others respectively. In terms of the diseases which they had now except for C.V.A., 33.6% of them had hypertension, and 16.2% had diabetes, and 9.4% had neuralgia, and 4.0% had heart diseases, the percentages in "fifties and sixties, housewives, spouse alive, upper class, literate of Korean alphabets" were higher than the others respectively. 6. In terms of the diseases which they had before C.V.A., 22.4% of them had hypertension, and 18.8% had diabetes, and 8.1% had heart diseases, and 11.2% had neuralgia and arthritis, and 7.8% had cancer, and 21.7% had the others. 7. In terms of exercise behaviors before C.V.A., 41.2% of them did nothing, and 58.8% did sometimes. 8. In terms of fatness level by self judgement, 36.1% of them thought "proper", and 41.1% thought themselves "fat(the highst percentage), and housewives(45.5%) thought themselves "fat". 9. In terms of favorite food, 50.2% of them liked meat, and 33.2% liked vegetables, and 13.0% liked fish. 10. In terms of fancy things of C.V.A. patients, 57.0% of them were non-smokers, and 53.1% were non-drinkers, and 55.2% disliked coffee. In smoking level, 16.6% of them smoked less than five pieces a day. In drinking, 18.0% of them drank half a glass of soju. In coffee, 25.3% of them drank a cup of coffee a day. 11. The level of satisfaction with C.V.A. patient-healing methods ; In medical therapy, 43.3% of C.V.A. patients thought it "usual", and 44.1% thought it "satisfactory" and 7.9% thought it "unsatis-factory". In acupunture and moxibustion 39.7% of the C.V.A. patients thought it "usual", and 53.0% thought it satisfactory" and 3.3% thought it "unsatisfactory". The level of satisfaction With Physiotherapy was average 61.7% and 4.0% was "unsatisfactory". From above statement, by considering those characteristics we should develop programs and materials to be health to the prevention and cure of C.V.A. and we should help hospitals and medical personnel families concerned to make use of them.
Arthritis ; Cerebral Hemorrhage ; Cerebral Infarction ; Coffee ; Commerce ; Daegu ; Drinking ; Female ; Glass ; Heart Diseases ; Heredity ; Humans ; Hypertension ; Inpatients* ; Male ; Meat ; Moxibustion ; Neuralgia ; Outpatients ; Recurrence ; Single Person ; Smoke ; Smoking ; Spouses ; Stroke* ; Vegetables

PURPOSE: To investigate the clinical manifestations, management, ophthalmologic complications, and prognosis of traumatic optic neuropathy. METHODS: A retrospective survey of 55 patients who visited Chosun Hospital from April 2009 to February 2016 was performed. The sex, age, causes, fracture characteristics, neurologic injury, and combined craniofacial bone fractures of patients who were diagnosed with traumatic optic neuropathy were statistically analyzed. Also, we investigated the rate of visual impairment in the patients with intracranial hemorrhaging and craniofacial fracture on radiologic examination and development of sensory strabismus. RESULTS: Traffic accidents were the most common cause of traumatic optic neuropathy. Among the patients, more than 60% showed severe visual impairment of less than 0.1 that lasted until the final observation. Altitudinal visual defects were the most common visual field defect and presented as marginal atrophy and central scotoma. While intracranial hemorrhaging was showed in 52.4% of the patients, craniofacial fracture was observed in 90.5% of the patients. The initial visual acuity was decreased when the patient presented with orbital fracture located in the retrobulbar area. Intravenous high-dose steroid injection did not affect visual prognosis. Sensory strabismus occurred more commonly under conditions of poor initial vision (p = 0.007) or young age (p < 0.001). CONCLUSIONS: Traumatic optic neuropathy in Korea has a high rate of initial visual impairment with poor prognosis of vision. In addition, high-dose intravenous steroid injection did not result in visual improvement.
Accidents, Traffic ; Atrophy ; Fractures, Bone ; Humans ; Korea ; Optic Nerve Injuries* ; Orbital Fractures ; Prognosis* ; Retrospective Studies ; Scotoma ; Strabismus ; Vision Disorders ; Visual Acuity ; Visual Fields

BACKGROUND: The endothelial expresson and upregulation of ICAM-1 and epidermal keratinocyte expression of ICAM-1 are well documented in psoriasis. ICAM-1 mediates the adhesion and trafficking of circulating activated skin-seeking CD45RO+ memory CD4+ T lymphocytes from the vessel into the dermis and epidermis of psoriatic skin by binding to its ligand LFA-1(lymphocyte function-associated antigen-1) expressed on lymphocyte membranes. OBJECTIVE: The purpose of this study was to investigate the expression of ICAM-1 in vascular endothelium and keratinocyte of psoriatic skin and the difference of ICAM-1 expression between early and fully developed psoriatic lesions. METHODS: We have studied the expression of ICAM-1 in twelve psoriatic patients who had not been treated for psoriatic lesions for 1 month and three normal human skin samples by immunohistochemical staining using monoclonal antibody against ICAM-1. RESULTS: Immunohistochemical staining revealed anti-ICAM-1 antibody positively stained only in the subpapillary endothelial cells of normal skin. But in all psoriatic lesions studied, anti- ICAM-1 antibody was stained positively in the endothelium of papillary and subpapillary plexus, and in fully developed psoriatic lesions, anti-ICAM-1 antibody was stained focally in epidermal keratinocytes. CONCLUSION: The results suggest that ICAM-1 expression on papillary microvessels and keratinocytes may play an important role in the transendothelial and transepidermal migration of lymphocytes from the vessel into the dermis and epidermis of psoriatic skin.
Dermis ; Endothelial Cells ; Endothelium ; Endothelium, Vascular* ; Epidermis ; Humans ; Intercellular Adhesion Molecule-1 ; Keratinocytes* ; Lymphocytes ; Membranes ; Memory ; Microvessels ; Psoriasis ; Skin* ; T-Lymphocytes ; Up-Regulation

PURPOSE: To investigate the effect of steroid treatment on strabismus associated with thyroid ophthalmopathy. METHODS: The present retrospective study was conducted on 22 patients diagnosed with strabismus associated with thyroid ophthalmopathy, who were treated with steroids orally or intravenously and followed up for more than one year. Patients were divided into three groups for analysis: an improved group, with no strabismus at the final follow-up visit; a stable group, with no change in the strabismus angle; and a deteriorated group, in which the strabismus angle had worsened. We investigated the characteristics of each group. RESULTS: In the gender/sex distribution of the 22 patients, 11 of the 15 patients in the improved or stable group were female; six of seven patients in the deteriorated group were male (p = 0.012). Two of the 15 patients in the improved or stable group had a smoking history. However, none smoked after the treatment had started. On the other hand, five of seven patients in the deteriorated group had a smoking history and continued to smoke during and after treatment (p = 0.001). No significant between-group differences were observed with respect to age, diplopia period, strabismus angle, or thyroid function level. CONCLUSIONS: Treatment with steroids may improve or stabilize strabismus associated with thyroid ophthalmopathy. However, the effect of treatment may differ, depending on whether the patient continues to smoke after treatment is initiated.
Diplopia ; Female ; Follow-Up Studies ; Hand ; Humans ; Male ; Retrospective Studies ; Smoke ; Smoking ; Steroids ; Strabismus ; Thyroid Gland

Interferon-gamma has been suggested as a cytokine of connective tissue stabilizer. In addition, it has also been demonstrated that this cytokine inhibited bone remodeling activities of the bone derived cells. In order to illuminate the effects of this cytokine in orthodontic force induced bone remodeling, it was administered to primary cultured periodontal ligament cells which have been known to have some osteoblast like characteristics. Interferon-gamma slightly decreased [3H]thymidine incorporation rate without a significant change in the total cellular DNA content up to 1000 U/ml, which meant these doses were not cytotoxic to the cell. Total protein synthesis was not influenced by various concentration of interferon-gamma whether it was determined by the [3H]proline incorporation rate or by the Lowry smethod. The effect of interferon-gamma on the individual protein was, however, differential, ie, it increased [3H]proline incorporation into the noncollagenous protein marginally, while it decreased [3H]proline incorporation into the collagen, so that it caused dose-dependent suppression of the relative collagen synthesis. On the contrary, the fibronectin synthesis determined by the ELISA was increased by 1000 U/ml of interferon-gamma. The differential effects of the interferon-gamma on the collagen and fibronectin synthesis exhibited not only their protein level but also the steady state mRNA level. Interferon-gamma decreased steady state level of alpha1(I) procollagen mRNA significantly, while showing no significant changes in the fibronectin mRNA level. In addition to this, it was also found that indomethacin did not affect on the interferon-gamma induced collagen decrease in this cell, which meant prostaglandins were not involved in the process of interferon-gamma induced collagen decrease. So it can be concluded that the incubation of periodontal ligament cells with 1000 U/ml of interferon-gamma for 24 hr showed differential effects on the type I collagen and fibronectin gene expression. The decrease in relative collagen synthesis in the protein level was related with decrease in the steady state level of mRNA, while the increase in the fibronectin synthesis in the protein level was not correlated with the mRNA level.
Alkaline Phosphatase ; Bone Remodeling ; Collagen Type I ; Collagen* ; Connective Tissue ; DNA ; Enzyme-Linked Immunosorbent Assay ; Fibronectins* ; Gene Expression ; Indomethacin ; Interferon-gamma* ; Osteoblasts ; Periodontal Ligament* ; Procollagen ; Prostaglandins ; RNA, Messenger

PURPOSE: Neonatal adrenal hemorrhage is not rare disease which can be caused by such risk factors as sepsis, large baby, birth trauma and asphyxia. The clinical manifestations include jaundice, anemia, abdominal mass and differentiation from neuroblastoma, renal vein thrombosis and adrenal abscess is needed. Through the clinical assessment of presenting features, we hope that this study be of any help to early detection and proper management of neonatal adrenal hemorrhage. METHODS: The 16 subjects out of neonates admitted to our hospital from July 1991 to June 1997 were diagnosed as neonatal adrenal hemorrhage. The risk factors, clinical manifestations, diagnostic methods and prognosis of neonatal adrenal hemorrhage were evaluated in these 16 cases. RESULTS: 1) Among 16 cases, males were 10 (62.5%) and females were 6 (37.5%). Mean birth weight was 3.601.08kg and mean gestational age was 39.82+1.08 week, and all were fullterm babies. In modes of delivery, vaginal deliveries were 12 cases (75.0%) and cesarean sections were 4 cases (25.0%). 2) 13 cases (81.2%) were involved in right side, 2 cases (12.5%) in left side and 1 case (6.3%) bilaterally. 3) The risk factors include sepsis in 6 cases (37.5%), large baby in 5 cases (31.3%), birth trauma in 5 cases<31.3%) and asphyxia in 3 cases (18.8%). 4) The clinical manifestations include jaundice in 7 cases (43.8%), anemia in 7 cases (43.8%), fever in 6 cases (37.5%) and abdominal mass in 3 cases (18.8%). 5) In the time of diagnosis, until 7 days of birth were 9 cases (56.2%), 8-14 days were 3 cases (18.8%), 15-21 days were 2 cases (12.5%) and 22-28 days were 2 cases (12.5%). Follow-up studies were done in 13 cases (81.2%), and tha lesions all decreased without any specific complications or sequelae. CONCLUSION: Neonatal adrenal hemorrhage can be diagnosed by abdominal ultrasono- gram in the presence of suggestive manifestations of jaundice, anemia, fever and abdominal mass, with relatively good prognosis. Differentiation from other conditions as well as avoidance of unnecessary explorations can be achieved by serial follow-up examinations of abdominal ultrasonogram.
Abscess ; Anemia ; Asphyxia ; Birth Weight ; Cesarean Section ; Diagnosis ; Female ; Fever ; Follow-Up Studies ; Gestational Age ; Hemorrhage ; Hope ; Humans ; Infant, Newborn ; Jaundice ; Male ; Neuroblastoma ; Parturition ; Pregnancy ; Prognosis ; Rare Diseases ; Renal Veins ; Risk Factors ; Sepsis ; Thrombosis ; Ultrasonography

Bullous pemphigoid(BP) is a bullous disease in elderly people characterized by subepidermal bullae on erythematous and normal skin. Peripheral blood easinophilia have been reported in the patients with BP, and blood eosinophilia may be related to disease activity and severity in BP. We report a 70-year old man BP. He showed peripheral blood eosinophilia, and was treated successfully with a combination of low dose steroids & tetracycline-niacinamide(T-N) therapy. The eosinophil counts fell to normal levels as the skin lesion cleared.
Aged ; Eosinophilia ; Eosinophils ; Humans ; Pemphigoid, Bullous* ; Skin ; Steroids

PURPOSE: To report the first domestic case of choroidal neovascularization in a choroideremia patient treated with intravitreal bevacizumab injection. CASE SUMMARY: A 29-year-old male presented with a sudden decline in vision in the left eye. Fundus examination revealed areas of choriocapillaries and retinal pigment epithelium atrophy with macular hemorrhage. Fluorescein angiogram revealed vascular hyperfluorescence in the juxtafoveal area. Neurosensory detachment around the macula and increased central macular thickness was also observed using optical coherence tomography. Upon the diagnosis of choroideremia with choroidal neovascularization, the patient was treated with 1.25 mg intravitreal bevacizumab. Visual acuity improved after four injections of intravitreal Bevacizumab with improvement in both detachment and fluorescein leakage. CONCLUSIONS: In patients with choroideremia presenting sudden decline in vision, ophthalmologists should detect for possible choroidal neovascularization. The results from the present study show that judicious use of intravitreal Bevacizumab may be effective in such cases. Further studies with a large sample size and sufficiently long follow-up periods are required.
Adult ; Antibodies, Monoclonal, Humanized ; Atrophy ; Choroid ; Choroidal Neovascularization ; Choroideremia ; Eye ; Fluorescein ; Follow-Up Studies ; Hemorrhage ; Humans ; Male ; Retinal Pigment Epithelium ; Sample Size ; Tomography, Optical Coherence ; Vision, Ocular ; Visual Acuity ; Bevacizumab

PURPOSE: To report a case of a macular hole resulting from accidental exposure to tattoo removal by the Q-Switched Nd:YAG laser, which was treated successfully by vitrectomy and silicone oil infusion. CASE SUMMARY: A 33-year-old man presented with decreased visual acuity after accidental exposure to a Q-Switched Nd:YAG laser. According to fundus examination, vitreous hemorrhage and macular edema were observed. After 21 days, a macular hole had developed which was treated by standard pars plana vitrectomy and gas tamponade. Unfortunately, closure was not obtained on the first attempt. Therefore, a second attempt using silicone oil infusion was performed. Four months after the initial visit, BCVA had increased to 20/50, and anatomical occlusion was achieved. CONCLUSIONS: The authors of the present study experienced an unusual case of macular hole developed from the Q-Switched Nd:YAG laser used to remove a tattoo. A satisfactory visual acuity was achieved after silicone oil infusion despite failure in the first surgery.
Adult ; Humans ; Macular Edema ; Retinal Perforations ; Silicone Oils ; Visual Acuity ; Vitrectomy ; Vitreous Hemorrhage