Incontinentia pigmenti is an uncommon genodermatosis. It affects female infants predominantly, described first by Bardach in l925, with the diagnosis of systematized nevus and Bloch in 1926, and Sulzberger in 1928 rnore compIetely. Skin lesions are characterized by 3 stages such as vesicobullous, verucous and finally pigmentary lesions and leave brownish pigmentation on the extremities and trunk. Hesides skin lesions some ectodermaI and mesodermal organs are affected and show developmental abnormalities. We experienced a case of incontinentia pigmenti in a 45-day-old female infant and present it with the review of literature. She showed extensive vesicobullopustular eruption with linear and reticular pigmentation on the extremities and trunk. Clinical and histopathologic findings of these lesions are compatible with Bloch-Sulzberger type of incontinentia pigmenti.
Diagnosis ; Extremities ; Female ; Humans ; Incontinentia Pigmenti* ; Infant ; Mesoderm ; Nevus ; Pigmentation ; Skin

No abstract available.
Mortality*

PURPOSE: To evaluate the findings and the role of CT in plexiform neuro-fibromatosis of the mediastinum. MATERIALS AND METHODS: We retropectively reviewed the CT scans of five patients with plexiform neurofibromatosis of the mediastinum. The CT scans were reviewed with attention to the distribution of the lesions, appearance and attenuation of mediastinal lesions, enhancement pattern after intravenous contrast infusion and associated findingssuch as intercostal neurofibroma. RESULTS: In all five patients CT scans demonstrated fusiform low attenuated masses which were oriented longitudinally and extended over multiple contiguous scans along the distribution of major mediastinal nerves. In four patients, mediastinal lesions appeared infiltrative, obliterating adjacent mediastinal fat plane. One patient had well defined fusiform masses along the major mediastinal nerves. Postcontrast enhanced CT scans revealed slight central enhancement in two patient and no contrast enhancement in three patients. Associated findings such as neurofibromas of intercostal nerves and sympathetic trunk, or subcutaneous neurofibromas were detected on CTscans in all five patients. CONCLUSION: Characteristic CT findings of low attenuation masses along the major mediastinal nerves are helpful to differentiate plexiform neurofibromatosis from mediastinal lymphadenopathy and to prevent from misreading as a malignant disease.
Humans ; Intercostal Nerves ; Lymphatic Diseases ; Mediastinum* ; Neurofibroma ; Neurofibromatoses* ; Tomography, X-Ray Computed

The distribution of pemphigus subclass autoantibodies in a patient with pemphigus vulgaris (PV) has been investigated by semiquantitative indirect immunofluorescence (IIF), using the HP series monoclonal antibodies specific for four human IgG subclasses on human foreskins. IgG1 and IgG4 intercellular substance-specific autoantibodies were detected in the serum of the patient, whereas IgG2 and IgG3 autoantibodies were absent. In addition to foreskins, human tonsillar epithelia were used as substrates of IIF for detecting the PV autoantibodies and it was one of satisfactory substitutes for monkey esophagus.
Antibodies, Monoclonal ; Autoantibodies* ; Esophagus ; Fluorescent Antibody Technique, Indirect ; Foreskin ; Haplorhini ; Humans ; Immunoglobulin G* ; Pemphigus*

The skin defect with sensory impairment of volar aspect of fingers should be reconstructed for the better function of hand. The Neurovascular island graft has been used to provide sensation to volar surface the thumb and to replace pulp tissues avulsed from thumb or index finger. Authors performed neurovascular island graft for defective sensibility of finger tip loss in 14 cases since Jun. 1979 to Mar. 1985. The donor flaps were obtained from the ring finger in 9 cases and middle finger in 5 cases. Among 14 cases, the donor flaps were taken from the ulnar side of 3rd and 4th finger in 11 cases. We followed up the the patients from 7 months to 4 years and 6months and in last follow-up, recovery of protective sensation such as sharp/blunt discrimination, temperature discrimination, and touch/stroke discrimination, was good but two point discrimination and reorientation of sensa-tion were poor. Cold intolerance was developed in 11 cases, and longitudial linear scar band of donor digit was demonstrated in 12 cases.
Cicatrix ; Discrimination (Psychology) ; Fingers ; Follow-Up Studies ; Hand ; Humans ; Sensation ; Skin ; Thumb ; Tissue Donors ; Transplants

Twenty one cases of adult tibial shaft fractures were fixed with flexible intramedullary nails at the Department of Orthopedic Surgery, Kang Nam General Hospital. The results are summerized as follows; l. Average healing time was 17 weeks. No case of nonunion was experienced. 2. As the operation was perfomed by closed method under fluoroscopic control, reduction of operation time is a major advantage. 3. Fixation was usually strong enough to allow immediate weightbearing and mobilization of the adjacent joints without external immobilization. 4. Three cases of delayed union and 2 cases of rotational malunion were experienced, but shortening and angulation were not so severe as to affect overall functional results. 5. In conclusion, Ender nailing is considered to be a valuable method in treating tibial shaft fractures. Even in cases with multiple injuries and in selected open cases, Ender nailing can be a saft procedure.
Adult ; Hospitals, General ; Humans ; Immobilization ; Joints ; Methods ; Multiple Trauma ; Orthopedics ; Tibial Fractures ; Weight-Bearing

Dislocation involving talus is a rare injury comprising less than 1% of all dislocations of the joint. One case of lateral subtalar di location of the talus treated at the Department of Orthopedic Surgery, Kang Nam General Hospital in Decernber 1985. The patient was followed for 6 months and satisfactory healing was obtained.
Dislocations ; Hospitals, General ; Humans ; Joints ; Orthopedics ; Talus

PURPOSE: We evaluated Kawasaki disease(KD) in children in order to evaluate the clinical characteristics and coronary complication of infantile Kawasaki disease. METHODS: A total of 226 medical records of children with KD admitted to The Catholic University of Korea, St. Mary's Hospital, from 1994 to 2003 were retrospectively analyzed. RESULTS: The incidence of lymphadenopathy was statistically lower in infantile Kawasaki disease than in Kawasaki disease of children older than one year. The incidence of atypical Kawasaki disease in infant(32%) was greater than that of total patients(24%), but there was no statistically significant difference in two groups. The incidence of coronary complication in atypical Kawasaki disease in children younger than one year was 50%(6/12), which was greater than that of coronary complication in typical KD of children older than one year. CONCLUSION: Diagnostic difficulties in infantile Kawasaki disease are due to high incidence of atypical characteristics of Kawasaki disease. Yet, early treatment with IVGV and aspirin is necessary because of its high incidence of coronary complication.
Aspirin ; Child ; Humans ; Incidence ; Korea ; Lymphatic Diseases ; Medical Records ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies

PURPOSE: To determine which perinatal risk factors influence neurodevelopmental outcome, we try to investigate which perinatal risk factors are correlated with Baley mental or psychomotor developmental index using BSID-II test (Baley Scales of Infant Development, II) in long term follow up of very low birth weight who received neonatal intensive care. METHODS: This study was performed on 56 very low birth weight infants with corrected age 24 months who were admitted to the neonatal intensive care unit. We analyzed correlation between perinatal risk factors and neurodevelopmental outcome assessed by mental and psychomotor developmental index. RESULTS: Mean birth weight was 1317.8+/-168.5g : Small for gestational age was 11 cases and appropriate for gestational age was 45 cases : Mean mental developmental index was 93.8+/-10.3 and psychomotor developmental index was 99.9+/-13.2. Mean developmental scale did not show any significant difference between perinatal risk factors, such as sex, ventilator care and its duration, hospitalization days, birth weight, small for gestational age. Mental or psychomotor developmental score was significantly delayed in the subgroup with grade III intraventricular hemorrhages and periventricular cyst. On cranial ultrasonogram, sinificantly low mental, psychomotor developmental score was found on the subgroup with grade III intraventricular hemorrhages and periventricular cyst formation larger than 3mm size (P<0.01). CONCLUSIONS: These findings suggest that grade III intraventricular hemorrhage and periventricular cyst larger than 3mm size are associated with poor prognostic risk factors regarding to low neurodevelopmental index score.
Birth Weight ; Child ; Child Development ; Follow-Up Studies ; Gestational Age ; Hemorrhage ; Hospitalization ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Risk Factors* ; Ultrasonography ; Ventilators, Mechanical ; Weights and Measures

Cardiovascular disease (CVD) remains the leading cause of morbidity, mortality, and health care costs in South Korea. The prevalence of preventable and treatable risk factors for CVD such as obesity, hypercholesterolemia, and smoking has continued to increase, despite improvements management of hypertension. Active leadership, participation, and support of professional organizations and medical institutions in national cardiovascular registries and regional treatment network have proven to be effective models to reduce the global burden of CVD in the Europe and North America. Regional treatment network systems for ST-segment elevation myocardial infarction have established to coordinate percutaneous coronary intervention (PCI) treatment centers, non-PCI treatment centers, and emergency centers especially across the Europe. The Act on the Prevention and Management of Cardio-cerebrovascular Disease was enacted in South Korea in 2017 to establish the legal frameworks and a comprehensive plan for the prevention and management CVD and risk factors. To fully achieve the goal of a NationalHealth Plan for Cardiovascular Disease, it is necessary to embark on a nationwide registry project and to promote the regional acute treatment accessibility which can therefore play a key role in achieving the objectives of the 2017 Act. In this regard, the Korean Society of Cardiology advocates a national project for health promotion and cardiovascular prevention to improve cardiovascular outcomes, which includes the expansion and establishment of regional cardiocerebrovascular centers (CCVCs) and new local CCVCs.