Erythropoietic protoporphyria, sometimes also called erythrohepitic protoporphyria or simple protoporphyria, is a heritable detect of heme synthesis in which the last enzyme of the heme synthetic pathway, ferrochelatase(or heme synthetase), is functioning subopt,imally. A 23-year-old male has experi nced erythema and edema on the fae and hands during or immediately after sun exposure, since 3 years of age. The skin lesions have been accompanied by severe itching, a buring sensation and pain. Severe episodes were followed hy head iche and vomiting. We have seen a case of erythropoietic protoporphyria presenting clinically and histo athologically, wit.h a skin lesion on the sun exposured area, and free erthrocyte protoporphyrin serologically.
Edema ; Erythema ; Hand ; Head ; Heme ; Humans ; Male ; Protoporphyria, Erythropoietic* ; Pruritus ; Sensation ; Skin ; Solar System ; Vomiting ; Young Adult

To evaluate the character, management and clinical results of the posterior capsular rupture during cataract surgery, we retrospectively studied the cataract surgeries that were performed between May 1989 and Decemeber 1991. We discovered 64 eyes in which the posterior capsule was torn. The overall incidence of this complication was 4.4%. Our chart review of these surgical procedure revealed that the tear occurred most frequently during the nucleus removal(44%), occured more frequently at the periphery than the center and the extent was mostly below one-third of the whole posterior capsular surface(75%). We performed vitrectomy(58%) and peripheral iridectomy(14%). Posterior capsular lenses were implallted in 56 cases. The predicted power of lens calculated with SRK-II fomula and manufacturer's A-constant was approximately 0.46 diopter less than actual postoperative refraction. Final visual acuity of 20/40 or better was achieved in 73% of the 64 cases. Our results revealed that when properly managed, a torn posterior capsule is compatible with an excellent visual outcome.
Cataract* ; Incidence ; Retrospective Studies ; Rupture* ; Visual Acuity

No abstract available.
Hemangioma* ; Liver* ; Tomography, Emission-Computed, Single-Photon*

No abstract available.
Hemangioma* ; Liver* ; Tomography, Emission-Computed, Single-Photon*

No abstract available.
Female ; Pregnancy ; Pregnancy, Ectopic*

Rotation-plasty procedure was first done by Borggreve, in 1930 for the congenital short femur. Recently this procedure was used for the malignant bone tumor at the distal femur by Kotz and Salzer in 1982. In spite of its cosmetic problem of the distal stump, this procedure has great functional advantage of converting the above-knee amputation to the below-knee amputation. The authors applied this procedure in case of severe soft tissue crushing and large bone defect from the distal femur to the proximal tibia by the rail-road accident. Conventional below-knee prosthesis with thigh corset gave him normal ambulation. The inverted foot was good to control the prosthesis as a below-knee stump and heel functioned as a patella to support the body weight.
Amputation ; Body Weight ; Femur ; Foot ; Fractures, Comminuted ; Heel ; Knee ; Patella ; Prostheses and Implants ; Thigh ; Tibia ; Walking

Antigens, CD20 ; metabolism ; Breast Neoplasms ; diagnosis ; diagnostic imaging ; metabolism ; Female ; Humans ; Immunohistochemistry ; Lymphoma ; diagnosis ; diagnostic imaging ; metabolism ; Middle Aged ; Positron-Emission Tomography ; Radiography ; Ultrasonography

Goltz syndrome is known as a rare mesoectodermal hereditary disease, characterized by focal dermal atrophies with hernias of adipose tissue and also associated with a multitude of possible skeletal, dental, ophthalmological and other abnormalities. We experienced a case of Goltz syndrome. An one day old female newborn had focal atrophic and telangiectatic skin lesions, microphthalmia, syndactyly and urinary tract abnormality. The finding of skin biopsy was consistent with focal dermal hypoplasia. We report the case with a brief review and related literatures.
Adipose Tissue ; Atrophy ; Biopsy ; Female ; Focal Dermal Hypoplasia* ; Genetic Diseases, Inborn ; Hernia ; Humans ; Infant, Newborn ; Microphthalmos ; Skin ; Syndactyly ; Urinary Tract

PURPOSE: Intraductal papilloma (IDP) is a benign breast disease with malignant potential, for which complete surgical excision is usually recommended. The aim of the present study was to investigate predictive factors for upgrading patients with a benign papillary lesion (BPL). METHODS: This study was an observational study using a prospectively collected cohort. In total, 13,049 patients who underwent a core needle biopsy (CNB) for a breast lesion between January 2009 and May 2015 were enrolled. We reviewed all patients with pathologically confirmed BPL from a CNB. RESULTS: Surgical treatment was performed for 363 out of a total of 592 lesions. According to the pathological differences, the lowest upgrade rate was shown in IDP without atypia (without atypia, 6.0%; with atypia, 26.8%; papillary neoplasm, 31.5%; p<0.001). The univariate analysis showed that, in IDP without atypia, the age at diagnosis, size of BPL on ultrasonography, and density on mammography were associated with upgrading. The multivariate analysis revealed that age >54 years and lesion size >1 cm were significantly associated with upgrade to malignancy (odds ratio [OR]=4.351, p=0.005 and OR=4.236, p=0.001, respectively). CONCLUSION: The indications for surgical treatment can be defined as age >54 years and mass size >1 cm, even in IDP without atypia in the CNB results; this also includes cases of IDP with atypia or papillary neoplasm. Therefore, we suggest that close observation without surgery is sufficient for younger women with a small IDP without atypia.
Biopsy ; Biopsy, Large-Core Needle* ; Breast Diseases ; Breast Neoplasms ; Breast* ; Cohort Studies ; Diagnosis ; Female ; Humans ; Mammography ; Multivariate Analysis ; Observational Study ; Papilloma, Intraductal ; Prospective Studies ; Ultrasonography

No abstract available.
Free Tissue Flaps*