Purpura fulminans (gangrenosa) is a rare clinical syndrome characterized by sudden appearance of rapidly progressive symmetrical subcutaneous ecchymosis durimg the convalescent stage of various infectious disease. A 4 year-old. boy has developed multiple skin defects with necrosis on extrernities and gangrenous changes on the left foot following a meningococcemia. B-K amputation on left leg and. free skin graft for multiple skin defect on extremities were, carried out.
Amputation ; Child, Preschool ; Communicable Diseases ; Ecchymosis ; Extremities ; Foot ; Humans ; Leg ; Male ; Necrosis ; Purpura Fulminans* ; Purpura* ; Skin ; Transplants

Three cases of orbital rhabomyosarcoma are reported here. Histological studies comfirmed the lesion 1:0 be the alveolar type in all cases. This tumor is a rare disease entity in Orient than Europe and America and accordingly, rarely encountered in our ophthalmologic practice, although it is the commonest primary malignant orbital tumor in children. Of the three cases reported here one occurred in adu1t and the other two in children. Case I. This 15 month old male showed a swelling of the left upper lid toward the medial side for 2 months and visited to our hospital on March 10, '69 with complaints of progressive swelling on the region with ptosis. The eye ball was slightly displaced toward the lower temporal side and ocular movement is limitted to upward. On palpation, the childthumb sized tumor was palpable in the upper nasal portion of the orbit. On excision of the tumor, the mass was found to be originated from the superior oblique muscle. The mass round measuring 2.5 X 2.5 X 1.5 cm in size. Histological diagnosis was alveolar rhabdomyosarcoma without cross-striation. No follow-up could be done. Case II. This patient of 15 month old male showed a swelling on the right lower lid existed 6 months and visited to our hospital on September 8, '69. The physical examination revealed that the left eye and the other routine examinations were within normal limits. The right eye showed the swelling of the lower lid, congestion of the palpebral conjunctiva, limitted movement of the eye ball toward the lower side and a palpable bean-sized mass on the region. The excision of tumor was performed under the general anesthesia. This mass was attached with the inferior oblique muscle with no other recognizable adhesion seemingly originating from that muscle. The mass was round and oval in shape, measuring 2 X 2 X 1 cm in size, diagnosis was alveolar rhabdomyosarcoma. The post-operative follow-up studies were unavailable. Case III. This case in one of adult alveolar rhabdomyosarcoma. At the age of 25 years, this man showed a proptosis of the right eye associated with migrain like headache, ocular pain and visual disturbance. This symptoms existed 6 months and gradually increased in intensity. On the physical examination, the left eye was found to be normal. The skull and orbit x-ray and c.b.c. were with in normal limits. The right eye revealed about 5mm proptosis compared with left eye and the adult thumb sized tumor was palpated on the inner side of the lower orbital rim. The visual acuity was in zero. The right optic disc was edematous and elevated about 4.0 D. The pupil showed marked dilatation and the light reflex was abscent and ocular movement was markedly limitted and eye ball is deviated toward the upper side. Under the general anesthesia, the exenteration of the orbit associated with the removal of tumor was carried out with no untoward complications. Origin of the mass was the inferior oblique muscle penetrating to the deeper part of the optic foraman and optic nerve was surrounded by the tumor completely Histology confirmed alveolar rhabdomyosarcoma. Follow-up study was impossible.
Adult ; Americas ; Anesthesia, General ; Child ; Conjunctiva ; Diagnosis ; Dilatation ; Estrogens, Conjugated (USP) ; Europe ; Exophthalmos ; Headache ; Humans ; Infant ; Male ; Optic Nerve ; Orbit* ; Palpation ; Physical Examination ; Pupil ; Rare Diseases ; Reflex ; Rhabdomyosarcoma* ; Rhabdomyosarcoma, Alveolar ; Skull ; Thumb ; Visual Acuity

The increased incidence of the trochanteric fracture of the femur was accompanied by the development of the vehicle and the prolongation of the average life-span. Recently it has been also increased in an active person from thirties to forties. 87 cases of the trochanteric fracture of the femur in 86 patients who have been admitted and treated at the Department of Orthopedic Surgery of Hanyang University Hospital during 8 years and 10 months, from May, 1972 to February, 1981, were reviewed. The results of this study were as follows: 1. The trochanteric fracture of the femur was more common in male and was frequent in a person who was from thirties to forties and who had vigorous social activity. 2. An injury from traffic accident was the most common cause of the fracture from thirties to forties and an injury from slip down was most common in seventies. 3. The unstable fracture which was classified by Evans' classification, Boyd and Griffin Type II Fx. and Tronzo Type III Fx. were most common in the intertrochanteric fracture and the Fielding Type II Fx. was most common in the subtrochanteric fracture of the femur. 4. Other injuries were accompanied by the trochanteric fracture of the femur in 38 patients (44.2%) and the pelvic bone fracture was the most common associated injury in these patients. 5. The weight bearing was allowed earlier in the patient who had been given the surgical Tx. than in the patient who had been given the conservative Tx. 6. In adults, there was no significant differences in the duration to achieve the bony union between the conservative treatment and surgical treatment performed. 7. We considered that the diminution of the probable complications by early weight bearing after accurate open reduction in accordance with the Type of the Fx. followed by secure internal fixation with devices is an ideal method of the Tx.
Accidents, Traffic ; Adult ; Classification ; Clinical Study ; Femur ; Hip Fractures ; Humans ; Incidence ; Male ; Methods ; Orthopedics ; Pelvic Bones ; Weight-Bearing

Pilomattiaoma, aften called calcifying epithelioma of Malherbe, is a benign tumor originating from the outer root sheath cell of the hair follicle and extending into the hair matrix, Pilomatricoma usually occurs as a single, asymptomatic dermal or subcutaneous nodule. Multiple lesions are quite unueuel, comprising only 2-3.5% of cases. We report two patients with multiple pilomatricoma.
Hair ; Hair Follicle ; Humans ; Pilomatrixoma*

Melanosis coli is the brownish pigmentation of the colon associted with the ingestion of anthraquinone compounds as iaxatives. The brownish discoloration of the colon mucosa is due to accumulation of macrophage containing lipofuscin pigment in the lamina propria. This is the one of the complications of laxative abuse, but the pigments disappear by withdrawing the anthraquinone. We report a case of malanosis coli histologically confirmed by fibersigmoidoscopic biopsy in a 70-year-old female patient consuming anthraquinone compound for twelve months with a review of the literature.
Aged ; Biopsy ; Colon ; Eating ; Female ; Humans ; Lipofuscin ; Macrophages ; Melanosis* ; Mucous Membrane ; Pigmentation

PURPOSE: Narcolepsy is a specific neurologic syndrome consisting of excessive daytime drowsiness, cataplexy, and the additional phenomena of sleep paralysis and intense dreamlike hallucinations at sleep-wake transitions. We are reporting a patient with narcolepsy whose repeated self-injury caused traumatic eyeball perforation. METHODS: A 26-year-old male with narcolepsy injured himself, which resulted in eyeball perforation in his both eyes. We diagnosed him as traumatic corneal laceration, traumatic cataract, and vitreous opacity due to lens material in vitreous in both eyes. RESULTS: After improvement of narcoleptic symptom and healing of corneal perforation, we performed cataract extraction and pars plana vitrectomy under local anesthesia. During operation, we found preretinal gliosis at superior portion of optic disc and subretinal hemorrhage in the right eye. We observed successful improvement in visual acuity through intraocular lens implantation.
Adult ; Anesthesia, Local ; Cataplexy ; Cataract ; Cataract Extraction ; Corneal Perforation ; Gliosis ; Hallucinations ; Hemorrhage ; Humans ; Lacerations ; Lens Implantation, Intraocular ; Male ; Narcolepsy ; Sleep Paralysis ; Sleep Stages ; Visual Acuity ; Vitrectomy

STUDY DESIGN: A retrospective analysis of 166 patients was undertaken to observe radiologically the changes of adjacent segments at follow-up more than three years after lumbar fusion. OBJECTIVES: The purpose of this study is to analyse the changes of adjacent segments and to correlate these changes to the length of using level and to the degree of deviation from physiologic lumbar lordosis. The authors also correlate these radiologic changes to the clinical symptoms. MATERIALS AND METHODS: In simple x-ray, authors observed traction spur, disc space narrowing, endplate sclerosis and vacuum phenomenon of adjacent segments. Authors used Gelb's criteria in segmental lordosis angle(SLA) and Katz's classification in clinical results. RESULTS: The average age was 49.6 years old and the average follow-up period was 57.2 months(4 years and 9.2 months). We observed radiologically the traction spurs in 35 cases(21.1%), end-plate sclerosis in 32 cases(19.3%), disc space narrowing in 33 cases(19.9%) and the vacuum phenomenon in 10 cases(6.0%). The numbers of fusion segments and the degree of unphysiologic segmental lordosis angle in fusion segments were related with the frequency and degree of changes of adjacent segments(P<0.05). The clinical results showed satisfaction in 142 cases(85.5%), unsatisfaction in 24 cases(14.5%) and low back pain in 24 cases(14.5%), leg pain in 11 cases(6.6%) and extension of fusion level in 4 cases(2.4%). In low back pain patients more than two kinds of radiological changes were frequently observed than the patients without low back pain(P<0.05). CONCLUSIONS: The radiological changes of adjacent segments were more frequently observed in long fusion and in fusion with unphysiologic lumbar lordosis angle. And these changes are frequently associated with low back pain. Thus long fusion and fusion with unphysiologic lumbar lordosis angle should be avoided if possible.
Animals ; Classification ; Follow-Up Studies* ; Humans ; Leg ; Lordosis ; Low Back Pain ; Retrospective Studies ; Sclerosis ; Spinal Fusion* ; Traction ; Vacuum

PURPOSE: To determine the significance of anomalous position of the gallbladder. MATERIALS AND METHODS: Sixteen patients with anomalous position of the gallbladder were evaluated for analysis. The diagnosis was confirmed by ultrasonography(15 patients) and oral cholecystography(1patient). Among those, six patients underwent CT scan and a patient had 99mTc-DISIDA scan. The images were analysed with respect to the location of the GB and configuration and associated abnormality of the liver and hepatobiliary systems. Medical records of each patient were also reviewed. RESULTS: Among 16 patients having an anomalous position of the gallbladder, nine had retrodisplaced gallbladder, four had left-sided gallbaldder, two had suprahepatic gallbladder, and one had floating gallbladder. Except for one patient, fifteen had abnormality in the liver such as focal atrophic or hypoplastic change and liver cirrhosis. Intrahepatic stones were demonstrated in 6 patients. CONCLUSION: Our results showed that anomalous position of the gallbladder was commonly associated with atrophy or hypoplasia of the liver rather than congenital in origin. The possiblity of an anomalous location of gallbladder should be kept in mind when GB is not in its normal location.
Atrophy ; Diagnosis ; Gallbladder* ; Humans ; Liver ; Liver Cirrhosis ; Medical Records ; Technetium Tc 99m Disofenin ; Tomography, X-Ray Computed

Bullous pemphigoid(BP) and herpes gestationis(HG) are subepidermal bullous diseases which show clinical and immunological similarities. Both diseases show immune deposits along the basement membrane zone and their autoantibodies bind a common antigenic site within the non-collagenous stretch of the 180 kDa BPAG2 ectodomain. Besides its association with pregnancy, HG has some characteristic features that distinguish it from BP. The serum of patients with HG often contains an IgG that avidly fixes complement, and showes IgG1 subclass predominance. We report here two cases of non-pregnant young women presenting clinical and histological features of bullous pemphigoid or herpes gestationis. The immunopathology, IgG subtyping and immunoblotting studies showed that the autoantibodies in the patients were the characteristic ones of herpes gestationis. The patients might be a subtype of BP that have characteristics of autoantibodies of HG patients.
Autoantibodies* ; Basement Membrane ; Complement System Proteins ; Female ; Humans ; Immunoblotting ; Immunoglobulin G ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy

Retinoblastoma has been established as an inheritable disease. To the development of sporadic cases the mutation can be ascrivable. Once the gene has been established, however, it is transmitted as an autosomal dominant characteristics with incomplete penetrance. The cases reported here was established in siblings which are of the extremely rare entities. Two families are reported here, which is one of the rare occurence. 1st. Family: The retinoblastoma affect 4 siblings(male, 2. female, 2) in 8 siblings(male, 3. female, 5). Of this 4 cases, 3 died of progression of retinoblastoma, while the one left was in apparent good health with early enucleation. 2 nd. family: Retinoblastoma existed in two siblings(male, 1. female, 1). The male dead 1 year after diagnosis of retinoblastoma and the other one refused radical treatment and lost in follow up.
Diagnosis ; Female ; Follow-Up Studies ; Humans ; Male ; Penetrance ; Retinoblastoma* ; Siblings