No abstract available.
Pyloric Stenosis, Hypertrophic*

Solitary cutaneous reticulohistiocytoma is a type of reticulohistiocytosis. The cytoplasm of tumo cells characteristically shows abundant eosinophilic "ground-glass" appearance. We report a case of this rare cutaneous tumor in a 54-year-old woman. It was present as a single nodule on the right index finger of the patient for seven years.
Cytoplasm ; Eosinophils ; Female ; Fingers ; Histiocytosis, Non-Langerhans-Cell* ; Humans ; Middle Aged

Sixteen cases of congenital hemangiomata were treated with intralesional corticosteroid injectinn and followed up for 3 to 48 months. They consisted of 9 cases of strawberry hemangiomata with soft consistency, 4 cases of strawbery hemangiomata with firm consistency and 3 cases of cavernous hemangiomata. The results were as follows : 1) All the soft strawberry hemangiomata showed excellent response with marked shrinkage by single treatment. 2) The firm strawberry hemangiomata showed poor response with visible shrinkage after five times treatment, in average. 3) Among three cases of cavernous hemangiomata, one case with large palpable blood vessels in the lesion showed poor response, and two cases without palpable blood vessels in the lesion showed good response. 4) Temporary atrophy and pallor of the skin color were noticed in about half of the cases. Considering the concern of the patients parents, course of the hemangiomata., and the simplicity and effects of the treatment, this therapeutic method is regarded useful in the management of congenital hemangiomata, especially in soft st r awberry hemangiomata.
Atrophy ; Blood Vessels ; Fragaria ; Humans ; Pallor ; Parents ; Skin

No abstract available.

Severe recurrent attacks of acute febrile neutrophilic dermatosis shortly followed multiple keratoacanthoma in a 43-year-old man with myelodysplastic syndrome. Besides the typical clinicopathological findings of the syndrome(fever, leukocytosis and neutrophilic erythematous plaques of the skin), the patient showed orolingual and conjunctival ulceration and severe cutaneous pathergic reactions. Moreover, to the best of our knowledge, multiple keratoacanthoma has not yet been reported with relation to the acute febrile neutrophilic dermatosis.
Adult ; Humans ; Keratoacanthoma* ; Leukocytosis ; Myelodysplastic Syndromes ; Neutrophils ; Sweet Syndrome* ; Ulcer

Whether immunosuppressive therapy may have beneficial effects in the treatment of IgA nephropathy remains controversial. ACE inhibitor or angiotensin II receptor antagonist(AIIA) are suggested to reduce urinary protein excretion(Up) in patients with renal diseases. We therefore investigated the effects of the angiotensin II receptor antagonist losartan on the proteinuria and renal function in patients with IgA nephropathy. AIIA reduced blood pressure in patients with hypertension, but there were no significant differences statistically before and after therapy. AIIA reduced Up after 1-4 months(2.8+/-1.1 to 1.1+/-1.0g/24h, p=0.001) and 7-13 months(2.8+/-1.1 to 1.7+/-0.6g/24h, p=0.017). There were no significant changes of serum creatinine levels after AIIA treatment. Cough or angioedema were not observed during AIIA treatment. In conclusion, AIIA may be useful in the treatment of patients with IgA nephropathy and proteinuria.
Angioedema ; Angiotensin II* ; Angiotensin Receptor Antagonists* ; Angiotensins* ; Blood Pressure ; Cough ; Creatinine ; Glomerulonephritis, IGA ; Humans ; Hypertension ; Losartan ; Proteinuria ; Receptors, Angiotensin*

Epidermolysis bullosa acquisita (EBA) is an uncommon autoimmune subepidermal blistering disorder and has four clinical subtypes. Among the four types of EBA, the cicatricial pemphigoid-like type is rarer than the other types and clinically the worst one. We experienced a case of cicatricial pemphigoid-like type of EBA in a 69-year-old woman, whose initial symptom was painful erosive lesions of oral mucous membrane before development of ocular and bullous cutaneous lesions. The clinical, histopathological findings and immunoblot assay were all typical of the disease. The course of her disease showed remissions by treatments including corticosteroid and intravenous immunoglobulin, but each time with exacerbations.
Aged ; Blister ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Female ; Glycogen Storage Disease Type VI ; Humans ; Immunoglobulins ; Mucous Membrane

Hereditary pancreatitis is a rare autosomal dominant inherited disease with 80% penetration rate. The disease is characterized by recurrent episodes of pancreatitis often beginning in childhood, positive family history with at least two other affected members and no known precipitating factors. Most forms of hereditary pancreatitis are caused by one of two commoner mutations, R122H in exon 3 and N29I in exon 2 of the cationic trypsinogen (CT) (PRSS1) gene, located on chromosome 7. These genetic defects are speculated to cause excessive trypsin activity or to prevent inactivation of prematurely activated trypsin, resulting in pancreatitis. We performed mutation analysis of a Korean family with two members having clinically suspicious hereditary pancreatitis. We analyzed the CT gene in DNA samples extracted from peripheral blood of five family members. First of all, polymerase chain reaction and restriction enzyme digestion were performed in exon 3 of the CT gene. And then DNA products were purified and sequenced. We found out that three members of the family, the mother and two daughters, had a R122H mutation of the CT gene. We report the first family of hereditary pancreatitis associated with the CT gene mutation, an arginine to histidine amino acid substitution at residue 122, in Korea.
Amino Acid Substitution ; Child ; DNA Mutational Analysis ; Female ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Mutation ; Pancreatic Pseudocyst/*complications ; Pancreatitis/complications/*genetics ; Trypsinogen/*genetics

Acrochordons are commonly found in middle aged rnan. They are mostly a few millimeters in size. The authors report an exceptionally large(10x5cm) one with a 3cm pedicle, which is thought to be the largest one in the Korean literature.
Humans ; Middle Aged

BACKGROUND: Free fatty acids are well known as an energy source. However, theoretically it could be destructive through oxygen free radical chain reactions unless they are bound to albumin in blood. Recently, the toxicity of oxidative agents in several diseases, and additionally the behavior of antioxidants including albumin against this have been suggested. Therefore, we investigated the relationship between free fatty acid/albumin molar ratio and erythrocyte injury in this study. METHODS: Free fatty acid and albumin were analysed in thirty-eight hypoalbuminemia patients and fifty-six healthy controls. To measure the erythrocyte injury, hemoglobin, absolute and relative reticulocyte counts, and lactate dehydrogenase (LD) were also examined. In addition, glutathione peroxidase (GPX) and superoxide dismutase (SOD) were determined in both fifteen patients and fifteen controls, respectively. RESULTS: The albumin levels in study group (3.06+/-0.28 g/dL) were significantly lower than those of control group (4.94+/-0.21 g/dL). The hemoglobin, reticulocyte counts, and LD levels in study group were significantly different from those of control group (P<0.01), but the free fatty acid concentrations showed no difference between two groups. The free fatty acid/albumin molar ratio in study group was significantly higher than control values. In study group, there were significant correlations between the free fatty acid/albumin molar ratio and (a) LD (r=0.43, P< 0.05), (b) relative reticulocyte count (r=0.39, P<0.05), and (c) hemoglobin (r=-0.31, P<0.01), respectively. The GPX and SOD activities in study group were not statistically different from the control values. There was an inverse correlation between albumin and GPX concentrations in study group (r=-0.36, P<0.01). CONCLUSIONS: These results suggest that toxic effect of unbound free fatty acid with decreased albumin activity as antioxidant may be involved in the cellular injury in hypoalbuminemia patients. Further studies for the correlation of free fatty acid/albumin molar ratio with individual antioxidant status are needed.
Antioxidants ; Erythrocytes* ; Fatty Acids, Nonesterified ; Glutathione Peroxidase ; Humans ; Hypoalbuminemia* ; L-Lactate Dehydrogenase ; Molar* ; Oxygen ; Reticulocyte Count ; Superoxide Dismutase