A case of Maffucis syndrome is presented with a brief review of literature because of its rarity. An attempt was made for reconstructive surgery for the deformities involving right hand with rewarding result.
Congenital Abnormalities ; Hand ; Reward

During the past 11 years period from April, 1961 to March, 1972 171 cases of electrical burn patients were treated at Orthopedic Department of Han-Il Hospital. The author reviewed and discussed our clinical experience of electrical thermal injuries. Clinical analysis of these 171 cases of electrical burn patients was carried out and summarized as follows; 1) The majority of the patients ranged two to three decades of life(68.4%); The youngest 1 year old and oldest 53 years of age. Male were involved significantly more than female, the incidence being 163 cases (95.3%) and 8 cases. 2) Seasonal distribution showed more than about two third with 114 cases for rainy summer times and Autumn from May to October. 3) Among 171 cases of electrical burn patients, 131 patients (54.2%) were under 5% of body surface and 15 patients (11.3%) were over 20% of body surface. 4) In the regional distribution of burn wound, the upper extremity were 157(92.3%), 73(42.9%) in lower extremity, and 49 (28.9%) in other region. 5) Occupational distribution; The electrical line man and electrician were seen 136 cases (80%) which is two third of 171 patients, labor 12 cases(7%), 10 cases (5.9%) in painters, 8 cases (4.6%) in company employee and industrialist, 5 cases (2.9%) in house-wife, and 4 cases (2.4%) in motor man. 6) The most frequently early complication at electric burn of 171 cases were gastrointestinal symptom 111 cases (65%), fracture 18 cases (10.5%), head injuries (intracranial bleeding and basal bone fracture of skull) 15 cases (6.4%), spinal cord injury and kidney rupture 1 cases (0.6%), hemorrhage 2 cases (1.2%), and other 8 cases (4%). 7) The predominating organisms cultured out of burn wounds of 26 cases were staphylococcus aureus 9 (34.7%), pseudomonas auerginosa 5 (19.2%), E. coli 3 (11.5%), staphylococcus albus 1(4%), β-hemolytic streptococcus 1 (4%), and no growth were 7 (26.9%). 8) The type of current and the voltage in 102 cases among the 171 cases of electric burn patients was clear. Injury of the 99 cases out of the 102 cases were by indirect current and the 3 was by direct current. In 99 cases by indirect current, electric burn in accordance with high tension current in voltage was 81 cases (79.4%), low tension current injuries 18 cases (17.6%). 9) At the admission, laboratory findings of the electric burn patients showed hemoconcentration and leukocytosis. 10) Early excision of the necrotic tissue and skin grafting was performed in 94 times. The results was excellent in 71 times. 11) Among 171 cases of electric burn patients, later complication were seen 47 cases (27.4%), which is 25 cases (14.3%) in amputation, 8 cases in cicatrical contraction, 4 cases in serum hepatitis, 4 cases in paralysis, 2 cases in osteomyelitis, 2 cases in ankylosis of knee joint, 2 cases in drop foot, 1 case in anal stricture, and 1 case in cataract. 12) The overall motality in this series was 8% (14cases) and the most frerent cause of the death was due to head injury (9 cases) at the time of electrical burn injuries.
Amputation ; Ankylosis ; Burns ; Burns, Electric ; Cataract ; Constriction, Pathologic ; Craniocerebral Trauma ; Female ; Foot ; Fractures, Bone ; Hemorrhage ; Hepatitis ; Humans ; Incidence ; Kidney ; Knee Joint ; Leukocytosis ; Lower Extremity ; Male ; Orthopedics ; Osteomyelitis ; Paralysis ; Pseudomonas ; Rupture ; Seasons ; Skin Transplantation ; Spinal Cord Injuries ; Staphylococcus ; Staphylococcus aureus ; Streptococcus ; Upper Extremity ; Wounds and Injuries

PURPOSE: To evaluate the usefulness of positron emission tomography/computed tomography (PET/CT) for field modification during radiotherapy in esophageal cancer. MATERIALS AND METHODS: We conducted a retrospective study on 33 patients that underwent chemoradiotherapy (CRT). Pathologic findings were squamous cell carcinoma in 32 patients and adenocarcinoma in 1 patient. All patients underwent PET/CT scans before and during CRT (after receiving 40 Gy and before a 20 Gy boost dose). Response evaluation was determined by PET/CT using metabolic tumor volume (MTV), total glycolytic activity (TGA), MTV ratio (rMTV) and TGA ratio (rTGA), or determined by CT. rMTV and rTGA were reduction ratio of MTV and TGA between before and during CRT, respectively. RESULTS: Significant decreases in MTV (MTV2.5: mean 70.09%, p < 0.001) and TGA (TGA2.5: mean 79.08%, p<0.001) were found between before and during CRT. Median rMTV2.5 was 0.299 (range, 0 to 0.98) and median rTGA2.5 was 0.209 (range, 0 to 0.92). During CRT, PET/CT detected newly developed distant metastasis in 1 patient, and this resulted in a treatment strategy change. At a median 4 months (range, 0 to 12 months) after completion of CRT, 8 patients (24.2%) achieved clinically complete response, 11 (33.3%) partial response, 5 (15.2%) stable disease, and 9 (27.3%) disease progression. SUVmax (p = 0.029), rMTV50% (p = 0.016), rMTV75% (p = 0.023) on intra-treatment PET were found to correlate with complete clinical response. CONCLUSION: PET/CT during CRT can provide additional information useful for radiotherapy planning and offer the potential for tumor response evaluation during CRT. rMTV50% during CRT was found to be a useful predictor of clinical response.
Adenocarcinoma ; Carcinoma, Squamous Cell ; Chemoradiotherapy* ; Disease Progression ; Electrons ; Esophageal Neoplasms* ; Humans ; Neoplasm Metastasis ; Positron-Emission Tomography and Computed Tomography* ; Radiotherapy ; Retrospective Studies ; Tumor Burden

BACKGROUND: Metallothioneins(MTs) are ubiquitous low molecular weight cytosolic proteins with high contents of cystein that xhibit a selective binding affinity for zinc, copper and other group II heavy metal ions. In additi~on to the sequestration function and detoxification action, MTs probably participate in the cellirlar protection against. ionizing radiation and alkylating agent cytotoxicity and in cellular pn:liferation. OBJECT: The objective of this study was to evaIuate the expression of MTs and to clarify t.heir role in carcinogenesis and/or biological behaviour in the neoplasms of ectoderrnal origin. METHODS: We performed immunohistochemical studies on the surgical specimens of rnalignant rnelanoma and squamous cell carcinoma and basal cell carcinoma. The results were compared with the normal skin and mu osa and hair t,issues and raf't-on culture tissues of skin and mucosal keratinocytes. RESULTS: 1) MTs were strongly expressed in epidermal basal cells of the normal skin and raft-on cultured artificial skin They were also rnore weakly expressed in epithelial basal cells of the normal mucosa and raft on cultured artificial mucosa and in matrix cells of the normal hair. 2) In rnalignant melanoma, MTs were meaningfully expressed, i.e., a strong expression was seen in the lesions showing a rapid vertical growth phase with fatal out,comes, but a weak expression in the cases whose precursor lesions were thought as dysplastic nevi, compared to the negative finding of norrnal melanocyt,es. The strong expression in a case of congenit.al rnelanocyt.ic nevus is considered as the activation of nevoid melanocytes. 3) MTs were weakly expressed in squarnous cell carcinoma with a diffuse aattern within the tumor mass. St,rong expression of MTs was particularly apparent in the poorl. differentiated squamous cell carcinoma and at the growing border of well differentiated squamous cell carcinoma. 4) In basal cell carcinoma, MTs were rather weakly expressed in solid cireumscribed types with a diffuse pattern and in cystic type with a patchy pattern. However they were not expressed in the solid infiltrative type. CONCLUSION: From these resuilts, we concluded that MTs were ubiquitously present in possible stem cells of various epithelial tissues except melanocytes irrespective of normal or artificial skin and/or mucosa including norrnal hair tissues. In the neoplasms of ectodermal origin, MTs might be involved in the invasive growth of tumor cells in the malignant melanoma and squamous cell carcinoma. It was implied that the role of MTs in basal cell carcinoma might be related to early events of hasal cell carcinogeresis and its down regulating tendency could be possible in its infil trative stage.
Carcinogenesis ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Copper ; Cytosol ; Dysplastic Nevus Syndrome ; Ectoderm* ; Hair ; Ions ; Keratinocytes ; Melanocytes ; Melanoma ; Metallothionein* ; Molecular Weight ; Mucous Membrane ; Nevus ; Radiation, Ionizing ; Skin ; Skin, Artificial ; Stem Cells ; Zinc

Erythema multiforme can be induced by various causes, including infections like virus or fungus, drugs like sulfonamides and penicillins, internal malignences, connective tissue diseases, pregnancy, menstruation, and exoessive sun exposure ramoxone, one of the paraquat dichloride compounds, is the most commonly used weed-kiIler in Korea. It is sometimes used in suicides due to its fatal toxicity, and even weak transutaneous absorption can lead to death. Herein we report a case of erythema multiform in a 46-year-old man induced by accidental contact with the Gramoxone
Absorption ; Connective Tissue Diseases ; Erythema Multiforme* ; Erythema* ; Female ; Fungi ; Humans ; Korea ; Menstruation ; Middle Aged ; Paraquat ; Penicillins ; Pregnancy ; Solar System ; Suicide ; Sulfonamides

Acromelanosis progressiva is a peculiar progressive pigmentary disorder characterized by intensely dark and sharply demarcated hyperpigmentation over the dorsa of fingers and toes, and the pigmentation rapidly spread proximally. A 19-year-old female had sharply demarcated symmetric dark aroarn macules on the dorsa of her hands and feet, forearms, lower legs, face and neck. These pigmentations at first appeared at the age of 5 on the dorsa of her fingers and toes, thereafter these pigmertatons spread progressively to the proximal portion of her body. Histologically, a proliferation of mela biocyte at the epidermal-dermal junction was seen, associated with mild hyperkeratosis. She was othervise normal on physical examination and had no family historv of consanguinity.
Consanguinity ; Female ; Fingers ; Foot ; Forearm ; Hand ; Humans ; Hyperpigmentation ; Leg ; Neck ; Physical Examination ; Pigmentation ; Toes ; Young Adult

No abstract available.
Diagnostic Tests, Routine* ; Hantaan virus*

Statistical analysis of 18,211 cases of electrocardiogram which were recorded in Chung-Ang University Hospital from January 1969 to December 162% of total cases(61% of male and 63% of female) and the incidence of normal electrocardiogram was decrease with aging. 2) Abnormal Q-wave was seen in 0.4% of total cases(0.5% of male and 0.3% of female) and the incidence of abnormal Q-wave was more common over 50 years of age. 3) Left axis deviation was seen in 1.1% of total cases(1.4% of male and 0.9% of female) and the incidence of left axis deviation was more common over 50 years of age. Right axis deviation was seen in 0.3% of total cases(0.3% of male and 0.4% of female) and the incidence of right axis deviation was more common under 30 years of age. 4) Left ventricular hypertrophy was seen in 3.6% of total cases(4.8% of male and 2.5% of female) and the incidence of right ventricular hypertrophy was more common under 30 years of age. 5) ST-segment depression was seen in 2.0% of total cases(2.2% of male and 1.9% of female) and the incidence of ST-segment depression was increased with aging. 6) T-wave inversion was seen in 1.6% of total cases (1.5% of male and 1.7% of female) and the incidence of T-wave inversion was increased with aging. 7) Atrioventricular block was seen in 1.4% of total cases(1.8% of male and 1.0% of female) and the incidence of atrioventricular block was more common over 50 years of age. 8) Incidence of complete left bundle branch block was 0.2% and was more common overs 60 years of age. Incidence of complete right bundle branch block was 1.1% and the incidence was more common over 50 years of age. Incidence of incomplete right bundle branch block was 3.0% and the incidence was more common under 40 years of age. 9) The incidence of premature beat was 2.7% of total cases, atrial fibrillation 1.5%, supraventricular tachycardia 0.2%, sinus tachycardia 7.5%, sinus bradycardia 2.2% and sinus arrhythmia 2.3%. 10) Low voltage was ssen in 3.8% of total cases and the incidence of low voltage was more common over 50 years of age.
Aging ; Arrhythmia, Sinus ; Atrial Fibrillation ; Atrioventricular Block ; Axis, Cervical Vertebra ; Bradycardia ; Bundle-Branch Block ; Cardiac Complexes, Premature ; Depression ; Electrocardiography* ; Humans ; Hypertrophy, Left Ventricular ; Hypertrophy, Right Ventricular ; Incidence ; Male ; Tachycardia, Sinus ; Tachycardia, Supraventricular

We report a case of MALT lymphoma in a 49-year-old woman. Her disease occurred simultaneously in the mucosa of her right upper eyelid conjunctiva and in her left nostril as ulcerating tumors associated with itchy ichthyosiform skin lesions on the trunk, hyperkeratotic palms and soles, and dystrophic nails. Histopathological examinations revealed consistent findings of MALT lymphoma with dissemination; i.e., diffuse infiltrates of lymphoplasmacytoid cells with a few Russel body-like structures, eosinophils, some shoddy granulomas under the irregularly hyperplastic epidermis, and diffuse infiltrates of CCL ( centrocyte-like ) cells and small lymphocytes inside and outside many lymphoid follicle-like structures in the subcutaneous tissue forming florid lymphoepithelial lesions. She died after 27 months duration of her disease with worsening of ichthyosiform skin lesions and dystrophic nails in spite of total excision of the tumors. We discuss the clinical and histopathologic features of MALT lymphoma with dissemination and the various similar diseases to differentiate.
Conjunctiva ; Eosinophils ; Epidermis ; Eyelids* ; Female ; Glycogen Storage Disease Type VI ; Granuloma ; Humans ; Lymphocytes ; Lymphoma, B-Cell, Marginal Zone* ; Middle Aged ; Mucous Membrane ; Skin ; Subcutaneous Tissue ; Ulcer

No abstract available.
Child* ; Humans ; Purpura*