No abstract available.
Carcinoma, Basal Cell ; Osteogenesis

Sclerema neonatorum (SN) is a rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and genitalia. The prognosis of SN is generally very poor, with a high fatality rate. We report the case of a full-term infant with delayed onset of SN showing good prognosis. A 4-month-old Korean male infant presented with diffuse waxy, sclerotic skin lesions on the whole body, sparing the face, nipples, and genital area, which began developing at 2 months of age. Histopathologic findings of the sclerotic skin lesions showed wide, fibrous intersecting bands in the subcutaneous fat tissue. Only sparse infiltration of lymphocytes and histiocytes was observed in the fat lobules and septa. Based on clinical presentation and histopathologic findings, he was diagnosed with SN. The patient survived with conservative care and had mild improvement of the skin lesions on his follow-up visit at 12 months of age.
Follow-Up Studies ; Genitalia ; Histiocytes ; Humans ; Infant* ; Lymphocytes ; Male ; Nipples ; Prognosis* ; Rare Diseases ; Sclerema Neonatorum* ; Skin ; Subcutaneous Fat

No abstract available.
Esophagus* ; Transplants*

From April 1989 to April 1993, 85 patients with orbital fractures were analysed. Among them, 10 patients were repaired surgically. Orbital fractures occured most frequently between the aga of 11 and 40 years. The main causes of injury were violence(41%), traffic accident (27%) and falldown injury (19%). Medial wall fractures were most common, followed by fractures of inferior wall, superior wall in order. In combined wall fracture, medial and inferior wall fracture was most frequent. The associated symptoms and signs of orbital fracture were lid laceration (32%), diplopia (29%). orbital edema(27%). traumatic iridocyclitis(22%). The subciliary approach was used for repair of the fracture. Early surgical intervention was done within 10 days in all cases. The insertion of Lyodura was performed in 9 patients. and calvarian bone graft was fashioned and placed over the defect in 1 patient. Insufficient correction of enophthalmos in 1 patient, lower eye lid retraction in 1 patient, residual diplopia in far upgaze in 2 patients were encountered as postoperative complications.
Accidents, Traffic ; Diplopia ; Enophthalmos ; Humans ; Lacerations ; Orbit* ; Orbital Fractures* ; Postoperative Complications ; Transplants

Segmental neurofibromatosis, a subtype of neurofibromatosis type 1, is characterized by neurofibromas and/or café-au-lait spots limited to an area or segment of the body. Checkerboard pattern is a rare type of cutaneous mosaic manifestation, characterized by squares or broad ribbons of affected skin with sharp demarcation at the midline. Herein, we report the case of a patient with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern. Our patient had multiple hyperpigmented macules on her entire body in a checkerboard pattern since birth. Several café-au-lait patches were observed on the left buttock and right axilla. A neurofibroma was incidentally found beneath the café-au-lait patch by histological examination, which showed ill-defined spindle cells with elongated nuclei at the deep dermis that stained positive for S-100. Based on the clinical presentation and histopathologic results, the patient was diagnosed with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern.
Axilla ; Body Patterning ; Buttocks ; Dermis ; Humans ; Lentigo ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Parturition ; Skin

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by tissue-bound and circulating autoantibodies directed against BP180 and/or BP230 antigens. Various inflammatory cells are involved in the development of blister in BP. OBJECTIVE: The aim of this study was to evaluate the correlation between peripheral leukocyte counts and BP severity. METHODS: We retrospectively included 60 patients with BP, who had not been treated with systemic steroid at the time of blood sampling. The patients were classified into two groups, those with admission history (admission group) and those without admission history (non-admission group). Disease severity was evaluated using three parameters: admission history, initial steroid dosage, and modified version of a pemphigus scoring system. We evaluated the correlation between peripheral leukocyte counts and disease severity measured by the three parameters. RESULTS: The admission group showed a significant increase in disease severity measured by initial steroid dosage and severity score compared with the non-admission group. Additionally, the admission group had increased total leukocyte, eosinophil, and neutrophil counts. In the correlation study, the peripheral eosinophil and neutrophil counts showed positive correlation with BP severity evaluated by both initial steroid dosage and the pemphigus scoring system. CONCLUSION: Peripheral eosinophil and neutrophil counts can be used as a marker in predicting disease severity in patients with BP.
Autoantibodies ; Blister ; Eosinophils* ; Humans ; Leukocyte Count ; Leukocytes ; Neutrophils* ; Pemphigoid, Bullous* ; Pemphigus ; Retrospective Studies ; Statistics as Topic

BACKGROUND/AIMS: Despite several reports on clinical aspects of anemia and malignancy, little is known of male patients with iron-deficiency anemia (IDA) and malignancy in Korea. We examined the cause of anemia, prevalence of and factors associated with malignancy, and treatment response to iron therapy in male IDA patients. METHODS: The results of 202 males with IDA seen from March 2008 to June 2013 were analyzed retrospectively. The patients were divided into two groups based on the causes of anemia: the cancer group included patients with anemia caused by malignancy and the non-cancer group included patients with anemia due to other causes. We compared the clinical characteristics and response to iron therapy between the two groups. RESULTS: The most common cause of IDA was bleeding (42.6%). The prevalence of malignancy was 11.9%, with colorectal cancer (58.3%) being the most common. Among the cancer patients (n = 24), 22 patients (91.7%) were age 50 or older. Independent factors associated with malignancy were old age (OR, 1.05; p = 0.026) and a positive stool occult blood test (OR, 7.48; p = 0.001). The treatment response to iron therapy based on a normalized hemoglobin level was lower in the cancer group (OR, 0.49; p = 0.31), but the difference did not reach statistical significance. The treatment response based on the mean hemoglobin level was significantly lower in the cancer group (12.6 +/- 2.2 vs. 13.8 +/- 1.6 g/dL, p = 0.016). CONCLUSIONS: Old age and a positive stool occult blood test were independent risk factors for malignancy in male IDA patients. We recommend screening for malignancy in patients older than 50 years or with a positive stool occult blood test.
Anemia ; Anemia, Iron-Deficiency* ; Colorectal Neoplasms ; Hemorrhage ; Humans ; Iron ; Korea ; Male ; Mass Screening ; Occult Blood ; Prevalence* ; Retrospective Studies ; Risk Factors

BACKGROUND/AIMS: In the 2-acetylaminofluorene (2-AAF)/70% partial hepatectomy (PHx) model, the mechanism underlying the differentiation of activated hepatic oval cells (HOCs) into hepatocytes and bile ductile cells is unclear. We investigated the role of cyclooxygenase-2 (COX-2) in HOCs and the relationship between COX-2 and extracellular matrix proteins in cellular proliferation. METHODS: Reverse transcription-polymerase chain reaction, immunohistochemical staining, and Western blotting were used to assess COX-2 expression. The co-localization of COX-2 with Thy1, c-Met, epithelial cell adhesion molecule, and alpha-smooth muscle actin was also examined. Additionally, we investigated whether connective tissue growth factor (CTGF), fibronectin (FN), extracellular signal-regulated kinase 1/2 (P-ERK1/2), and AKT were expressed in HOCs. RESULTS: The expression of COX-2, prostaglandin E2 receptors, and c-Met was upregulated in HOCs. However, HOCs treated with the COX-2 inhibitor NS398 showed decreased COX-2, CTGF, FN, and AKT expression, whereas P-ERK1/2 was unaffected. Additionally, NS398 inhibited HOC proliferation, but not the proliferation of HOCs cultured on FN-coated dishes. Furthermore, the proliferative response of HOCs treated with NS398 was reversed by hepatic growth factor treatment. CONCLUSIONS: These results suggest that HOC proliferation is mediated through COX-2, extracellular FN expression, and AKT activation. Thus, COX-2 plays an important role in HOC proliferation following acute injury.
2-Acetylaminofluorene ; Actins ; Animals ; Antigens, Neoplasm ; Bile ; Blotting, Western ; Cell Adhesion Molecules ; Connective Tissue Growth Factor ; Cyclooxygenase 2 ; Dinoprostone ; Epithelial Cells ; Extracellular Matrix ; Extracellular Matrix Proteins ; Fibronectins ; Hepatectomy ; Hepatocytes ; Liver ; Liver Regeneration ; Muscles ; Nitrobenzenes ; Phosphotransferases ; Rats ; Sulfonamides

Purpose: Extremely preterm infants are prone to brain injury and underdevelopment. Intraventricular hemorrhage (IVH) is the most common cause of brain injury and a significant risk factor for neurodevelopmental delay in preterm infants. Severe IVH is known to have a poor outcome; however, the outcomes of low-grade IVH remain controversial. This study aimed to evaluate neurodevelopmental outcomes and brain segmental volumes of preterm infants with low-grade IVH. Methods: This retrospective cohort study included 109 extremely preterm infants who underwent term equivalent age-magnetic resonance imaging and neurodevelopmental evaluation at a corrected age of 18 to 24 months. We compared infants with and without low-grade IVH. Results: Among the 109 extremely preterm infants, 25 had low-grade IVH and 84 had no IVH. There were no significant differences in the neurodevelopmental outcomes between the low-grade and no IVH groups. In multivariate analysis, low-grade IVH was associated with a smaller medullary volume (adjusted odds ratio, 0.575; 95% confidence interval, 0.346 to 0.957; P=0.034). Conclusion We found no significant differences in the neurodevelopmental outcomes of extremely preterm infants at a corrected age of 18 to 24 months between those with low-grade IVH and those without IVH. Low-grade IVH was associated with a smaller medullary volume.