Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

The authors dissected and examined a horseshoe kidney from the cadaver of a 54-year-old Korean female. The results were as follows. Other congenital anomalies or complications were not found, and no renal stones showed on plain radiography of the horseshoe kidney. The horseshoe kidney was located in the area between the 12 th thoracic vertebra and the 4th lumbar vertebra, with the superior extremity of the left kidney 10mm more inferior than that of the right. The isthmus connecting the bilateral kidneys was located at the level of the 3rd lumbar vertebra. The size of the kidney was 102mm × 52mm × 44mm (right) and 108mm × 62mm × 34mm (left), and the superoinferior and anteroposterior lengths of isthmus were 22mm and 10mm, respectively. The abdominal aorta and inferior vena cava passed posteriorly to the isthmus, with the inferior mesenteric artery and lumbar splanchnic nerve passing anteriorly. Some grooves were found on the anterior surface of the bilateral kidney. The hilum of the right kidney faced the anteromedial direction and that of the left kidney faced the anterolateral direction. At the hilar plane, the right renal arteries and veins passed anteriorly and posteriorly to the renal pelvis ; the left renal arteries passed posteriorly to the renal pelvis, with the left renal veins passing anteriorly and posteriorly. Three branches of the right renal arteries passed renal hilum, while two branches did not, and two branches of the left renal arteries passed renal hilum, while six branches did not. The two arteries arising from the aortic bifurcation were distributed to the isthmus. The number of renal veins passing the renal hilum were three in the right, and two in the left. The right and left ovarian veins drained to the renal veins. There were 12 minor calyces distributed normally in the right kidney, 13 minor calyces distributed radially in the left kidney, and 3 minor calyces in the isthmus, composed of parenchyme. The left portion of the horseshoe kidney was concluded to have developed poorly, on the basis of incomplete ascension and abnormal rotation during development, the imperfect configuration of the renal shape, and the abnormal distribution of the renal vessels and renal calyces.
Aorta, Abdominal ; Arteries ; Cadaver ; Extremities ; Female ; Fused Kidney* ; Humans ; Kidney ; Kidney Pelvis ; Mesenteric Artery, Inferior ; Middle Aged ; Radiography ; Renal Artery ; Renal Veins ; Spine ; Splanchnic Nerves ; Veins ; Vena Cava, Inferior

BACKGROUND: The severity of several chronic inflammatory diseases was reported to be associated with polymorphism of the IL-1 receptor antagonist gene(IL-lrn). OBJECTIVE: This study was performed to study the polymorphism of the IL-1rn in vitiligo and in the normal Korean population. METHODS: Thirty one cases of vitiligo and seventy nine normal Koreans as control were studied for the polymorphism of IL-1 rn. RESULTS: The frequency of allele 2 of the IL- I rn in 31 patients with vitiligo was compared with that of the 79 healthy controls. The frequency of allele 2 was 1.6% in vitiligo patients and 3.8% in the normal controls. CONCLUSION: There was no significant difference in the frequency of allele 2 between the vitiligo patients and normal controls.
Alleles ; Humans ; Interleukin-1* ; Vitiligo*

Normal C3WHeN strain mice exposed to topical 8inethoxypsomlen plus long wave ultraviolet (PUVA) showed a reduction in contact hypersensitivity, (CH) which was localized to the skin in the area of PUVA treatment (local suppression), whereas systemic PUVA treatment caused diffuse suppression of CH reaction, regardless of the application site of 2,4-dinitro-1-fluorobenzene (DNFB). There seem to be two different mechanisms responsible for CH reduction by PUVA. Local suppression by topical PUVA treatment was thought to be a result of blocking the afferent phase of immune response, it was associated with a lack of CH effector cells in the peripheral lymph nodes and could not be reversed by indomethacin treatment. Diffuse suppression induced by systemic PUVA treatment seemed to be associated with blocking of egress of effector cells from the regional lymph nodes, this depressed CH response was prevented when indomethacin was administered before PUVA treatment.
Animals ; Dermatitis, Contact* ; Indomethacin ; Lymph Nodes ; Mice ; Skin

Several hair dressing procedures, for example, bleaching and dyeing of the hair, are currently being frequently performed for cosmetic purposes. While allergic contact dermatitis due to hair dressing products has often been described, only a few cases of burns caused by hair dressing chemicals have been reported. A 6-year-old girl presented with an 8x6 cm sized round ulcerative lesion on the occipital area after hair bleaching with ammonium persulfate mixed with hydrogen peroxide. The skin biopsy specimen showed epidermal necrosis and sclerosing change in the dermis. She was referred to another burn center and then she was treated with skin grafting by a plastic surgeon. To the best of our knowledge, this is the first reported case of chemical burn due to a hair bleaching agent in the Korean dermatologic literature.
Ammonium Sulfate ; Bandages ; Biopsy ; Burn Units ; Burns ; Burns, Chemical ; Child ; Cosmetics ; Dermatitis, Allergic Contact ; Dermis ; Hair ; Humans ; Hydrogen Peroxide ; Necrosis ; Quaternary Ammonium Compounds ; Skin ; Skin Transplantation ; Ulcer

The amniotic band syndrome is a collection of congenital deformities presurmably due to rupture of amniotic sac. It appears to cause fetal injury through three basic mechanisms including malformation, disruption, and deformation. The associated anomalies vary firom minor digital defect to major craniofacial and visceral defects. They can be categorized as neural tube-like defects, craniofacial anomalies, limb anomalies, abdominal and thoracic wall defects, visceral anomalies, and constriction bands. We had expericnced a case of severe congenital craniofacial anomaly due to amniotic bands diagnosed by ultrasonogram in the antenatal period is presented with a brief review of the literatures.
Amniotic Band Syndrome* ; Congenital Abnormalities ; Constriction ; Extremities ; Infant, Newborn ; Rupture ; Thoracic Wall ; Ultrasonography

Segmental testicular infarction is a rare cause of an acute scrotum. The etiology can be related to sickle cell anemia, hypersensitivity angiitis and polycythemia in some cases, but the condition is usually an idiopathic phenomenon. Because making the differential diagnosis between segmental testicular infarction and testicular tumor can be difficult, most authors have recommended surgery in the past. We report here on cases of testicular segmental infarction that were treated by conservative management and we describe the radiologic findings.
Anemia, Sickle Cell ; Diagnosis, Differential ; Infarction ; Polycythemia ; Scrotum ; Testis ; Vasculitis, Leukocytoclastic, Cutaneous

No abstract available.
Macrophages, Peritoneal*

BACKGROUND: Leprosy is a chronic granulomatous infectious disease caused by Mycobacterium leprae. It has recently been reported that various clinical manifestations and treatments of leprosy are associated with increased angiogenesis. However, there are few reports on this topic. OBJECTIVE: This study aims to examine the differences in angiogenesis according to clinical forms of leprosy. METHODS: Thirty-three cutaneous lesions that represented the clinical spectrum of leprosy and 7 normal skins were selected for this study. Clinical forms of leprosy included in this study were 7 cases of tuberculoid (TT), 8 cases of borderline tuberculoid (BT), 8 cases of borderline lepromatous (BL), and 10 cases of lepromatous (LL) leprosy. A total of 40 sections of formalin-fixed, paraffin embedded tissues were investigated for Factor VIII-related antigen (FVIIIRA) expression using immunohistochemical staining. The number of FVIIIRA positive blood vessels in hot spot at a power of x40 was observed by 2 independent dermatologists. RESULTS: The number of vessels ranged from 4 to 12 (mean 8.43+/-2.64) in the normal cutaneous tissues, 23 to 38 (mean 28.86+/-5.46) in the TT, 46 to 71 (mean 56.50+/-8.68) in the BT, 49 to 77 (mean 64.75+/-9.82) in the BL, and 74 to 159 (mean 104.40+/-27.71) in the LL. The mean numbers of vessels in the BT, BL, and LL leprosy lesions were significantly higher than the mean number in the normal cutaneous tissues. Also, an overall increase was observed in the mean number of vessels from TT through BT, BL to LL leprosy lesions. CONCLUSION: We suggest that increased angiogenesis is associated with pathogenesis in leprosy. This study should prove helpful to future research into leprosy treatment.

Fibroma of the tendon sheath is a benign soft tissue tumor and this mostly occurs in the distal portion of an extremity. The tumor usually appears as a slow-growing, firm, painless, small nodule in association with tendons and tendon sheaths. Histopathologically, it shows a well-demarcated nodule that consists of haphazardly-arranged, fibroblast-like spindle cells that are embedded in a dense collagenous matrix. A 52-year-old woman presented with a tender, solitary, 2.5x2.0 cm-sized, subcutaneous nodule on the right elbow. The skin biopsy specimen showed that the well-circumscribed tumor contained densely-arranged collagen areas with spindle cells and slit-like vascular channels. We herein report on a rare case of fibroma of a tendon sheath on the right elbow.
Biopsy ; Collagen ; Elbow ; Extremities ; Female ; Fibroma ; Humans ; Middle Aged ; Skin ; Tendons