A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

In order to clarify the effect of radiation on the mouse jejunal crypt cells by combined administration of adriamycin and radiation and also to evaluate the enhancing effect of adriamycin, the authors performed this study by delivering single irradiation of 1,000 to 1,600 rad to the whole abdomen of mice by cobalt-60 teletherapy unit. In combination with adriyamycin treatment groups, the drug was administered as single dose of 10 mg/kg either 2 hours before or 4 hours after graded single dose, 900 to 1,400 rad, of irradiation. The authors studied the quantitative changes of intestinal crypt cells by microcolony survival assay technique and the morphological changes of small intestinal villi by scanning electron microscope in mice following to combined therapy with adriamycin and irradiation. The average number of jejunal crypts per circumference was 130+/-6 in control group. The mean lethal dose(Do) of each irradiation alone and combined therapy groups 2 hours before and 4 hours after irradiation, were 160, 170, and 170 rad in cell survival curves, respectively. The dose effect factor(DEF) of adriamycin in each groups of pre-irradiation and post-irradiation were 1.19 and 1.26, respectively. The conical shaped villi were noted on 1,200 rad in irradiation alone group and 1,000 rad in combined groups. For the proper clinical application we must be careful of the radiation injury to small bowel when the anticancer chemotherapy and radiation injury to small bowel when the anticancer chemotherapy and radiation therapy to the abdomen and pelvic area are used as combined therapeutic modality.
Abdomen ; Animals ; Cell Survival ; Doxorubicin* ; Drug Therapy ; Mice* ; Microscopy, Electron, Scanning ; Radiation Injuries

Using as assay for jejunal crypt stem cell survival, dose-response curves for the reproductive capacity of crypt stem cells mouse jejunum exposed to multifractionated gamma-ray irradiation(single, 2, 3, 4, 5, 6, 7, 8, 10,12, and 16 fractions) were analyzed and single-dose survival curve of these cells was constructed. The following conclusion were drawn: 1) Survival curves for higher numbers of dose fractions were displaced to higher dose, and characterized by increasingly shallower slopes. 2) The single-dose survival curve had broad shoulder, Dq=460 cGy, remaining near-exponential over initial dose range 0 to 300 cGy, with initial slope 1Do=474 cGy 3) At fractionated dose in the range of 180 to 450 cGy, the average recovered dose per fraction interval was approximately 50% of the dose per fraction. 4) The value of a/b ratio by using of linear regression analysis for the reciprocal dose plots was 8.3Gy which lied in the range of 6-14Gy for early-reacting tissues. 5) The linear-quadratic model for dose-response formula offers valid approximations for all doses to be used in radiotherapy, only two parameters to be determined, and considerable convenience in practical applications.
Animals ; Jejunum ; Linear Models ; Mice* ; Radiotherapy ; Shoulder ; Stem Cells

While the microbiology of cellulitis in the pediatric population and systemic diseases(DM, Cirrhosis, etc) is well known,the causative bacteria and clinical aspect of cellulitis in young adults are less descrihed. this study was undertaken to identify the causative organisms and clinical aspect of adult cellulits. We analysed 121 medical records of 115 young adult patients with cellulitis who had been admitted at the department of orthopaedic surgery, National Police hospital from Jan. 1994. to Dec. 1996. The results were as follows. 1. The age of patient was between 21 yrs and 25 yrs, and the sex of all patients was male. 2. The lower leg and foot were the most frequently involved site of cellulitis as 92 cases(74.2%). 3. The potals were suspected in 65 cases(56.5%),of them, traumas were detected in 43 cases(66.1%), non-traumatic skin lesions in 23 cases(33.9%). 4. In most cases, initial systemic symptoms (fever, chill, headache, etc.) and local symptoms (local heating, tenderness, etc.) were found. 5. The laboratory findings revealed leukocytosis in 19%, elevated ESR in 94%, positive CRP in 80%. 6. Microorganism were detected in 16 of 38 pus cultures. Gram positive microorganisms were detected in 15 of 16 cases culture positive. 7. 6.7% of patients with cellulitis experienced recurrence in same lesions. In conclusion, the retrospective analysis of 115 young adult patients with cellulitis showed that primary cause of young adult cellulit was circulatory disturbance and lymphatic obstruction of the lower extremities most often involved by trauma. therefore, supportive treatments such as rest,elevation,ice packs are considered prior to antibiotic treatment.antibiotic treatment aimed at gram-positive cocci appears to be sufficient.
Adult ; Bacteria ; Cellulitis* ; Fibrosis ; Foot ; Gram-Positive Cocci ; Headache ; Heating ; Hot Temperature ; Humans ; Leg ; Leukocytosis ; Lower Extremity ; Male ; Medical Records ; Police ; Recurrence ; Retrospective Studies ; Skin ; Suppuration ; Young Adult*

Entrapment neuropathy of the suprascapular nerve is rare and frequently overlooked in the differential diagnosis of shoulder pain. There have been few published reports on a ganglion compressing the suprascapular nerve. We experienced a case of entrapment neuropathy of the suprascapular nerve by a ganglion that compressed the inferior branch of suprascapular nerve at the spinoglenoid notch.
Diagnosis, Differential ; Ganglion Cysts ; Shoulder Pain

Early stage osteonecrosis of femoral head is a major therapeutic dilemma in orthopedics. Many joint preserving treatment modalities have been proposed, but none of them is entirely satisfactory. Among them, core decompression has been widely performed. The results of twenty two core decompression procedures that were performed for osteonecrosis of the femoral head in fifteen patients were retrospectively reviewed to evaluate the effectiveness of the procedure. From January 1990 to December 1993, we had treated twenty two cases of the osteonecrosis of femoral head. The average follow-up was 31 months, ranging from 12 months to 58 months and the average age at operation was 39.6 years, ranging from 21 to 58 years. By Ficat-Arlet stage, the stages I were 1 case,IIa 6 cases, IIb 7 cases and III 8 cases. The functional and radiological evaluation was done preoperatively and at final follow up by the Harris Hip score and Ficat stage. The results were as follows. The improved cases were 10 cases and the progressed were 12. Radiologically, none of 1 hip(0%) with stage I, two of 6 hips(33%) with stage IIa, four of 7 hips(57%) with stage IIb, and six of 8 hips(75%) with stage III have progressed. Mean preoperative Harris Hip score was 57 points and it improves to 69 (the improved 13 cases; 85, the progressed 9; cases; 49)at final follow up. In six of the progressed 9 cases, the arthroplasties were performed. Among them, five cases are being observed. In conclusion, the core decompression is not only highly effective in preventing further change in femoral head, but also relieving pain and delaying time for arthroplasty at late stage osteonecrosis of femoral head.
Arthroplasty ; Decompression ; Follow-Up Studies ; Head ; Hip ; Humans ; Joints ; Orthopedics ; Osteonecrosis ; Retrospective Studies

Six different brain tumors in middle cranial fossa are presented which are studied by CT and proved pathologically. The authors experienced rare tumors in middle cranial fossa such as cavernous hemangioma, cysticmeningioma, Schwannoma, Masson's vegetant intravascular hemangioendothelioma and other tumors (arteriovenousmal formation and metastatic adenoid cystic carcinoma) whose CT findings were atypical. The results are as follows; 1. I case of tumors in middle cranial fossa, basal and coronal sections are necessary for further evaluation of the relation with dura and adjacent bone changes. 2. In suspicion of metastasis, bone setting should be done to find out bone involvement. 3. Internal carotid angiography gave little help in the differential diagnosis of tumors in middle cranial fossa.
Adenoids ; Angiography ; Brain Neoplasms ; Cranial Fossa, Middle ; Diagnosis, Differential ; Hemangioendothelioma ; Hemangioma, Cavernous ; Neoplasm Metastasis ; Neurilemmoma

No abstract available.
Burkitt Lymphoma* ; Child* ; Humans

Mitochondrial myopathy is characterized by variable clinical manifestations from mild limb weakness to fatal respiratory failure and central nervous system sequela. But it is a rare event that sleep disordered breathing become a clue of diagnosis for mitochondrial myopathy. We report a case of a 21 year-old man who was diagnosed as mitochondrial myopathy during the investigation for the possible cause of chronic hypoventilation syndrome. Before being admitted to our hospital, he was suspected as having sleep apnea syndrome in another hospital. We re-evaluated the history, physical examination, laboratoy findings and polysomnography in detail. Severe hypoxemia was noted during REM sleep on nocturnal polysomnography and the diagnosis of mitochondrial myopathy was made by muscle biopsy in rectus abdominis muscle. We treated him with bilevel positive airway pressure therapy during sleep and it could reverse the hypoxemia during REM sleep. He could be discharged with improved condition and is being well with the use of this ventilatory assistance.
Anoxia* ; Biopsy ; Central Nervous System ; Diagnosis ; Extremities ; Humans ; Hypoventilation ; Mitochondrial Myopathies* ; Physical Examination ; Polysomnography ; Rectus Abdominis ; Respiratory Insufficiency ; Sleep Apnea Syndromes ; Sleep, REM* ; Young Adult

Primary pulmonary Hodgkin's lymphoma is a rare but distinct entity to be distinguished from nodal Hodgkin's lymphoma and from lymphomas involving lung secondarily. This lymphoma affects women more frequently than men, and typically involves superior portions of the lung. This case is reported to illustrate the clinical, radiographic and anatomic characteristics of the primary pulmonary Hodgkin's lymphoma. A 34-year-old woman presented for the evaluation of hemoptysis. A chest CT revealed a large poorly defined mass in the medial aspect of the right upper lobe, extending to the right mediastinum and trachea. The microscopic examination of the biopsied lesion revealed fibroblastic stroma infiltrated by a mixture of lymphocytes, histiocytes, and eosinophils. The clinical impression was inflammatory pseudotumor, presumably due to slightly favorable response to corticosteroid therapy. Two months later the patient's symptoms worsened despite the steroid therapy and a lobectomy was done. The specimen showed a soft to firm, pale yellow, ill defined mass, 10.0 8.0 cm, involving the visceral pleura. A few satellite nodules around the main mass were noted. The histologic findings were consistent with Hodgkin's lymphoma, nodular sclerosis type.
Adult ; Eosinophils ; Female ; Fibroblasts ; Granuloma, Plasma Cell ; Hemoptysis ; Histiocytes ; Hodgkin Disease* ; Humans ; Lung ; Lymphocytes ; Lymphoma ; Male ; Mediastinum ; Pleura ; Sclerosis ; Tomography, X-Ray Computed ; Trachea