No abstract available.
Pyloric Stenosis, Hypertrophic*

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.

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

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

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

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

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

BACKGROUND: The Rheumatoid Factor (RF) is the only serological marker in the diagnosis of rheumatoid arthritis (RA), but its sensitivity and specificity are not satisfactory for the diagnosis of RA. Therefore, we investigated the diagnostic performance of a new anti-cyclic citrullinated peptide antibodies test (anti-CCP) by the enzyme-linked immunosorbent assay (ELISA) in RA. METHODS: A cyclic peptide variant that contains citrulline was used as an antigenic substrate in ELISA. We performed the RF and anti-CCP in 324 RA patients, 251 non-RA patients (rheumatic diseases other than RA), and 286 normal individuals. Diagnostic performances such as sensitivity and specificity were evaluated by the receiver-operator characteristics (ROC) curve at optimal cut-off values. The optimal cut-off values were determined at the maximal point of the area under the curve. RESULTS: The sensitivity and specificity of anti-CCP were 72.8% and 92% at 3.8 U/mL. The sensitivity and specificity of RF were 80.6% and 78.5% at 9 U/mL. The sensitivity and specificity of anti-CCP and RF were 67%, 95.2% and 63.3%, 90% at 8.4 U/mL, 20 U/mL, respectively. A combination of anti-CCP with RF increased the sensitivity and specificity to 79.3%, 96.4%, respectively. Anti-CCP was positive in 23.8% among 63 sero-negative RA patients. CONCLUSIONS: We considered that the anti-CCP might be useful as another new serological marker for the diagnosis of a RA combination with RF, or not, because the anti-CCP has a higher diagnostic specificity than the RF and was an easy, convenient ELISA method in performance.
Antibodies* ; Arthritis, Rheumatoid* ; Citrulline ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Humans ; Rheumatoid Factor ; Sensitivity and Specificity

Background/Aims: There are inconsistencies in the effects of low to moderate dose alcohol consumption on the development of hypertension in adult men. We hypothesized that a region-specific effect might participate in this heterogeneity. Methods: We conducted a systematic review and meta-analysis to evaluate the effect of alcohol dose on hypertension incidence using contemporary data through December 2017. Subjects were categorized according to their level of alcohol consumption as non-drinkers (reference) and low- (0.01 to 20.0 g/day), moderate- (20.1 to 40.0 g/day), moderate- to high- (40.1 to 60.0 g/day), and high-dose (> 60.0 g/day) drinkers. We defined hypertension as a blood pressure ≥ 140/90 mmHg and/or the use of anti- hypertensive drugs. Results: In total, 11 articles (seven Asian and four Western) were selected for our analysis. Among Asian men, a significantly elevated risk was observed even in the low alcohol dose group in comparison with the group with no alcohol consumption, and the risk increased in a dose-dependent manner (pooled relative risks [95% confidence intervals (CI)]: 1.25 [1.13 to 1.38], 1.48 [1.27 to 1.72], 1.75 [1.43 to 2.15], and 1.78 [1.51 to 2.09]). Among Western men, a similar dose-response relationship was noted in general (p for subgroup difference > 0.1), but a significantly elevated risk was evident only in the high-dose group (pooled relative risks [95% CI]: 1.22 [0.85 to 1.74], 1.57 [0.90 to 2.75], 1.47 [0.44 to 4.91], and 1.49 [1.02 to 2.18]). Conclusions Even low doses of alcohol can lead to the development of hypertension, particularly in Asian men. Our findings could serve as additional evidence for developing an appropriate preventive strategy in each region.