BACKGROUND: The high level of susceptibility of patients with liver cirrhosis (LC) to septicemic infection is thought to be due to decreased reticuloendothelial function and irnpairment of several components of cell mediated and humoral immunity. It is well known that LC may cause many characteristic skin changes including jaundice, spider angioma, palmar erythema, xanthelasrna and nail changes such as clubbing and white nails. OBJECTIVE AND METHODS: During a 7 month period frorn April to October 1995, 100 patients who had been diagnosed as having LC were evaluated for the duration of their LC, the associated diseases they had apart from LC and various skin manifestations. Also, we performed the tuberculin test in 20 patients with LC and evaluated the correlation of the tuberculin test with severity of LC divided into 3 groups according to the Childs classification. RESULTS: The results are summarized as follows ; 1. The most frequent duration of LC was under 5 years (74%). 2. The most common associated disease of patients with LC was upper GI bleeding including esophageal varix (24 cases). Also, diabetes mellitus (13 cases) and hepatic coma (11 cases) were observed. 3. Jaundice (82 cases) was the most frequent skin rnanifestation of patients with LC. In addition, vascular changes (81 cases) and various nail changes (69 cases) were observed. Onychomychosis was the most common nail change of patients with LC. 4. 60% of the patients who were given the tuberculin test showed anergy, and the frequency of anergy increased in proport on to the severity of LC. However, it was not significant statistically (p> 0.05 ). CONCLUSIONS: Some of the skin manifestations help in the diagnosis of LC. Also, increased frequency of tuberculin test anergy in proportion to the severity of LC is thought to be related to decreased cell mediated immunity, although statistically it is not significant.
Child ; Classification ; Diabetes Mellitus ; Diagnosis ; Erythema ; Esophageal and Gastric Varices ; Hemangioma ; Hemorrhage ; Hepatic Encephalopathy ; Humans ; Immunity, Cellular ; Immunity, Humoral ; Jaundice ; Liver Cirrhosis* ; Liver* ; Skin Manifestations* ; Skin* ; Spiders ; Tuberculin Test

No abstract available.
Heart Diseases* ; Heart* ; Humans ; Infant*

BACKGROUND: Generally, there are few problems in the diagnosis of Paget's disease(PD) using the H&E stain. However, the differentiation of PD from the clonal type of Bowens disease and superficial spreading melanoma in situ that shows pagetoid spreading of tumor cells, may present diagnostic difficulties. In addition, the specia1 stains used for demonstrating the presenee of Pagets cells, such as PAS and mucicarmim, are non-specific and not always sensitive. So, inenunohistochemical stains with monoclonal antibodies against various antigens may be helpful for differentiating PD from ather morphologically similar skin lesions. OBJECTIVE: The purpose of this study was to investigate the diagnostic accuracy of immunohisto- chemical staining for diagnostic use in PD. METHODS: Immunohistochemical stains used in the biotin streptavidin amplificxl technique with monoclonal antibodies to several low rnolecular weight cytokeratin(CK)s, EMA and CEA, were performed on formalin-fixed, paraffin-embedded tissue. Twelve cases of PD(10 cases of extranmmmary PD and 2 cases of mammary PD), five cases of superficial spreading melanoma in situ and five cases of Bowens disease were investigated. RESULTS: The results were as follows. 1. Positive reactions with variable intensity using CK7, CKS, CK19 were seen in all cases(100%) of PD and the. staining intensity tor CK7 or CK19 was stronger than that of CKS. 2. Of the 12 cases of PD, both CK18 and CAM5.2 staining showed positivity in 11 cases(92%). 3. EMA and CEA staining showed positivity in 10(83%) and 9(75%) of 12 cases, respectively. 4. Some Pagets cells were negative for CK8, CK18 and EMA, although other positive cells were observed in the same sections. 5. All antigens were consistently negative in all cases of Bowens disease and superficial spreading melanoma in situ. CONCLUSION: The results show that moaoclonal antibodies to low molecular weight CKs are more sensitive than EMA or CEA in the demonstration of Pagets cells. Moreover, among the low molecular weight CK series, CK7 and CK19 are most useful for their high sensitivity and intensity.
Antibodies ; Antibodies, Monoclonal ; Biotin ; Bowen's Disease ; Coloring Agents ; Diagnosis ; Melanoma ; Molecular Weight ; Skin ; Streptavidin

Infantile myofibromatosis is an uncornmon, benign, self-limiting, localized or generalized process, probably of hamartomatous origin, which consists to a large degree of cells having the characteristics of myofibroblasts and sometimes of pericytes. Both solitary and multicentric forms occur. Most lesions are present at birth or in early infancy, and some are familial in origin. A female newborn presented with a firm, round, red colored, 3 x 3 cm sized tumor with central necrosis on the left chest. Histological examination revealed well-circumscribed nodules consisting of short bundles of plump, spindle shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. The tumor was immunoreactive for actin but did not stain for desmin. A Follow-up examination at the age of 3 months revealed a moderate degree of spontaneous regressior of the lesion.
Actins ; Desmin ; Female ; Fibroblasts ; Follow-Up Studies ; Humans ; Infant, Newborn ; Myocytes, Smooth Muscle ; Myofibroblasts ; Myofibromatosis* ; Necrosis ; Parturition ; Pericytes ; Thorax

Edwards syndrome is first introduced by Edwards and characterized by facial anomalies, multiple cardiovascular, gastrointestinal, urogenital, and skeletal malformations. It results from triplication of part or all of chromosome 18 in some or all of the patient's cells. It has an incidence of 1 in 4,500 live births or less and short life expectancy. Recently we experienced a case of 3-day-old female new born infant with this syndrome. Post mortem examination showed progeric face with prominent occiput, large flabby ears, microphthalmia, and micrognathia, bilateral clenched hands with flexion contraction of middle fingers, and bilateral rockerbottom feet. Internal examination revealed horseshoe kidney, esophageal atresia with tracheoesophageal fistula, two accessory spleens, and multiple cardiac anomalies. A trisomy 18 was confirmed by the cytogenetic study.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Incidence

A case of the neurovascalar island pedicle graft for restoration of sensation after amputation of both thumb fingertip pulp in 17 years old male patient is presented at Department of Orthopedic Surgery, College of Medicine, Korea University. Tho island pedicle graft is suggested by Esser firstly and it is very difficult and excitement. At postoperative follow up check within one month, we obtained very good result without other complications.
Amputation ; Follow-Up Studies ; Humans ; Korea ; Male ; Orthopedics ; Sensation ; Thumb ; Transplants

No abstract available.
Blood Pressure* ; Child* ; Humans ; Seoul*

No abstract available.
Child* ; Humans ; Learning Disorders* ; Learning*

MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is one of the mitochondrial encephalomyopathy, A rare disease caused by a disturbance of the mitochondrial chain of respiration. MELAS is confirmed by typical light and electron microscopic findings : "ragged red fibers" by modified Gomori trichrome stain on light microscope and numerous abormal mitochondria on electron microscope. We experienced a boy with the characteristic clinical and pathologic findings of MELAS. Our patient demonstrated bilateral basal ganglia calcifications and infarction at right parieto-occipital and thalamic areas on CT and MR We found that MRI was more sensitive and represented the infarcted lesions better than CT. Detection of cerebral insults of MELAS by MRI is important in making decision on patient treatment and also in predicion of the patient prognosis.
Acidosis, Lactic ; Basal Ganglia ; Brain Diseases ; Child* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; MELAS Syndrome* ; Mitochondria ; Mitochondrial Encephalomyopathies ; Muscular Diseases ; Rare Diseases ; Respiration

No abstract available.
Priapism* ; Prostate* ; Prostatic Neoplasms*