No abstract available.
Hepatorenal Syndrome*

No abstract available.
Humans

No abstract available.
Interleukin-2*

Lowe et al. have described a disease characterized by organic aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation, this disease is now called as Lowe's syndrome or oculo-cerebro-renal syndrome which manifests growth plates, metabolic acidosis, and characteristic eye changes, including bilateral cataract, glaucoma and nystagmus. It is a congenital hereditary affectation. Most of the patients with this syndrome are male sex. We report a new case of Lowe's syndrome in a 5-year old girl.
Acidosis ; Ammonia ; Cataract ; Child ; Female ; Glaucoma ; Growth Plate ; Humans ; Hydrophthalmos ; Intellectual Disability ; Male

Intravenous immunoglobulin (IVIG) infusion is an effective therapy for acute Kawasaki disease (KD). Nonetheless, approximately 10 percent to 20 percent of patients have persistent or recrudescent fever despite IVIG treatment, leading to a higher risk for coronary artery aneurysms (CAA). This unresponsiveness may pose a challenge to the clinicians. Tumor necrosis factor-alpha levels are elevated in the acute phase of the disease, especially in patients who develop CAA. We report a 10-month-old male with KD who failed to respond to multiple doses of IVIG and methylprednisolone and who then was treated with infliximab (5 mg/kg single dose). After infliximab treatment, he became afebrile with normalization of inflammatory markers and no further progression of CAA.
Aneurysm ; Coronary Vessels ; Fever ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Infant ; Male ; Methylprednisolone ; Mucocutaneous Lymph Node Syndrome* ; Tumor Necrosis Factor-alpha ; Infliximab

Proliferating trichilemmal tumor is one of rare benign tumors of the skin appendages, considering as hamartoma of the terminal hair follicle, isthmic segment of the outer root sheath. We report a case of numerous proliferating trichilemmal tumors admixed with ordinary trichilemmal cysts with femilial history in 64-year-old man. He has total 157 lesions, which are chiefly located in scalp (48), back (32), and also face, neck, trunk, extremities. Histologically, the tumor is composed of irregularly arranged and ansatomosed trabeculae, lobules, or sheets of proliferated trichilemmal squamous epithelium with peripheral palisading of the basaloid cells. Several layers of squamoid or ovoid cells have PAS-positive clear cytoplasm. And it also shows abrupt amorphous, trichilemmal keratinization. Some squamous eddies with mild cellular atypism are associated, but definite invasion or other evidence of the malignancy is not found. usual multiple trichilemmal cysts are admixed with tumor and occasionally exhibit connection between them.
Cysts ; Hamartoma

The nm23 gene was originally identified from murine melanoma cell lines of varying metastatic potential. A strong association has been observed between reduced expression of nm23 gene and acquisition of metastatic behavior in some tumor cells including breast cancer and melanoma, but not in others such as colon cancer, neuroblastoma, and cervical cancer. It was proposed that nm23 may function as a suppressor gene for tumor metastasis. It has recently been found that the sequence of nm23 and NDP-kinase(NDP-K) was identical. Mortality associated with human breast carcinoma is almost entirely due to subsequent metastasis, but the molecular basis of this metastasis is not understood. Elucidation of the genetic control of metastatic propensity of a tumor is important in determining prognosis and choice of therapy. The purpose of this study was to investigate the relationship of nm23 protein expression with axillary lymph node metastasis and other prognostic factors. Using an immunohistochemical technique and employing a polyclonal antibody to nm23 protein, we have determined nm23 expression in a series of 72 infiltrating ductal carcinomas of the breast. Immunostaining for the nm23 gene product have heterogenous cytoplasmic and nuclear staining in 61 patients(84.7%). Sections were scored according to relative abundance(1 = less than 25% of the cells, 2 = 26-75%, 3 = 76-100%). In 61 patients with positive immunostaining, the staining was scored as 1 in 41.6%, 2 in 18.0%, and 3 in 40.2%. The staining of tumor cells was greater than that in normal epithelial cells and stromal cells. No relationship was found between nm23 expression and lymph node metastasis, histologic grade, tumor size, estrogen receptors or progesterone receptors. Therefore, nm23 protein is increased in neoplastic tissues but no correlation with metastatic potential could be demonstrated. The biological mechanism of over-expression of nm23 in malignant cells and its role in tumor progression remain to be determined.
Breast Neoplasms ; Breast* ; Carcinoma, Ductal* ; Cell Line ; Colonic Neoplasms ; Cytoplasm ; Epithelial Cells ; Genes, Suppressor ; Humans ; Lymph Nodes ; Melanoma ; Mortality ; Neoplasm Metastasis ; Neuroblastoma ; Prognosis ; Receptors, Estrogen ; Receptors, Progesterone ; Stromal Cells ; Uterine Cervical Neoplasms

The nm23 gene was originally identified from murine melanoma cell lines of varying metastatic potential. A strong association has been observed between reduced expression of nm23 gene and acquisition of metastatic behavior in some tumor cells including breast cancer and melanoma, but not in others such as colon cancer, neuroblastoma, and cervical cancer. It was proposed that nm23 may function as a suppressor gene for tumor metastasis. It has recently been found that the sequence of nm23 and NDP-kinase(NDP-K) was identical. Mortality associated with human breast carcinoma is almost entirely due to subsequent metastasis, but the molecular basis of this metastasis is not understood. Elucidation of the genetic control of metastatic propensity of a tumor is important in determining prognosis and choice of therapy. The purpose of this study was to investigate the relationship of nm23 protein expression with axillary lymph node metastasis and other prognostic factors. Using an immunohistochemical technique and employing a polyclonal antibody to nm23 protein, we have determined nm23 expression in a series of 72 infiltrating ductal carcinomas of the breast. Immunostaining for the nm23 gene product have heterogenous cytoplasmic and nuclear staining in 61 patients(84.7%). Sections were scored according to relative abundance(1 = less than 25% of the cells, 2 = 26-75%, 3 = 76-100%). In 61 patients with positive immunostaining, the staining was scored as 1 in 41.6%, 2 in 18.0%, and 3 in 40.2%. The staining of tumor cells was greater than that in normal epithelial cells and stromal cells. No relationship was found between nm23 expression and lymph node metastasis, histologic grade, tumor size, estrogen receptors or progesterone receptors. Therefore, nm23 protein is increased in neoplastic tissues but no correlation with metastatic potential could be demonstrated. The biological mechanism of over-expression of nm23 in malignant cells and its role in tumor progression remain to be determined.
Breast Neoplasms ; Breast* ; Carcinoma, Ductal* ; Cell Line ; Colonic Neoplasms ; Cytoplasm ; Epithelial Cells ; Genes, Suppressor ; Humans ; Lymph Nodes ; Melanoma ; Mortality ; Neoplasm Metastasis ; Neuroblastoma ; Prognosis ; Receptors, Estrogen ; Receptors, Progesterone ; Stromal Cells ; Uterine Cervical Neoplasms

No abstract available.
Cardiomyopathy, Dilated* ; Child* ; Humans

The thirty eight newborn infants with periventricular leukomalacia who were admitted to the neonatal intensive care unit of Gil General Hospital from March 1, 1988 to June 30, 1991, were investigated for ultrasonographic findings, risk factors and neurological outcome. The results were as follows: 1) There were 38 cases of PVL including 21 echogenic flarings and 17 cystic PVL's. 2) Mean birth weight was 2,250 gm and mean gestational age was 35 week. 3) Mean detection timing was 4th day in echogenic flarings and 18th day in cystic PVL's. 4) PVL's were located in the parietal region in 1 case and fronto-parieto-occipital in 3 cases. 5) Mean cyst size was 6 mm. 6) Multiple logistic regression analysis for the risk factors of PVL showed that low birth weight, apnea and seizure were the most significant contributing factors (p<0.05). 7) In the follow-up study of cystic PVL's, 7 cases showed improvement, 7 cases developed into multicystic encephalomalacia and 3 cases developed into atrophy. 8) Neurodevelopmental outcome of cystic PVL's showed nomal; 6.2%, minor neurodevelopmental defect; 43.8%, major neurodevelopmental defect; 31.2% and death; 18.8%. 9) Neurosonographic predictability for neurodevelopemental sequelae by cystic PVL's showed sensitivity; 63.6%%, specificity; 98.0%, positive predictive value; 92.8% and accuracy; 88.2%. 10) Major neurodevelopmental defect was more frequent, cyst size being larger and location being more extensive (p<0.05).
Apnea ; Atrophy ; Birth Weight ; Encephalomalacia ; Follow-Up Studies ; Gestational Age ; Hospitals, General ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Intensive Care, Neonatal ; Leukomalacia, Periventricular* ; Logistic Models ; Rabeprazole ; Risk Factors* ; Seizures ; Sensitivity and Specificity