No abstract available.
Thanatophoric Dysplasia*

Systemic lupus erythematosus is a multisystemic autoimmune disease in which the kidneys are frequently involved. Clinical diagnosis of SLE is based on the criteria of American Rheumatism Association (ARA). A few cases who were classified as SLE by the ARA criteria but were antinuclear antibody (ANA)-negative have been reported. It was reported that critical factor in ANA positivity is the choice of substrate. It is generally accepted that the cultured cell of human origin, especially HEP-2 cell, is better than tissue section or animal cells. Thus, the ANA test is negative only in approximately 2M of SLE patients when human tissue culture cells are used as substrate. We report a 25-year-old man admitted to our hospital because of generalized edema. He was found to have active lupus nephritis(WHO class IV), photosensitivity and pancytopenia. The result of FANA test which used HEP-2 cell as substrate was repeatedly negative, but anti-ds DNA and anti-Ro antibody were positive.
Adult ; Animals ; Antibodies, Antinuclear ; Autoimmune Diseases ; Cells, Cultured ; Diagnosis ; DNA ; Edema ; Humans ; Kidney ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Pancytopenia ; Rheumatic Diseases

Patients with acromegaly have a reduced life expectancy rnainly due to cardiovascular, respiratory or cerebrovascular diseas-. Malignancy also seems to occur with greater than the expected incidence. In particular, the published retrospective or prospective studies have suggested a strong association of colonic neoplasia with acromegaly. But, there were a few reports of thyroid cancer in acrornegaly. We report a case of thyroid papillary cancer derived from diffuse goiter in acromegaly, sugge- sting the possible carcinogenic role of growth hormone.
Acromegaly ; Bites and Stings ; Colon ; Goiter ; Growth Hormone ; Humans ; Incidence ; Life Expectancy ; Prospective Studies ; Retrospective Studies ; Thyroid Gland ; Thyroid Neoplasms

PURPOSE: Liver transplantation (LT) is regarded as an important management option for fulminant hepatitis (FH), which is associated with considerable mortality under conservative management. The aim of this study was to evaluate the outcome of children with FH according to management. METHODS: We reviewed medical records of patients presented with FH from January 1994 until April 1999. The children were grouped according to the treatment. Group A was classified for supportive treatment only and group B for supportive treatment plus LT. Children were considered as candidates for LT if the level of factor V decreased to below 20% of normal or the patient's condition deteriorated despite intensive care during the initial 48 hours. Underlying disease, duration after jaundice, grade of encephalopathy, laboratory findings, treatment and outcomes were analyzed. RESULTS: The study group comprised 7 females and 8 males aged from 8 months to 15 years old (median age of 4 years). The causes of FH were Wilson disease (4 cases), Epstein-Barr virus infection (1 case), drug (1 case) and idiopathic (9 cases). There were 5 children in group A and 10 in group B, and there were no significant differences in age, sex ratio, underlying diseases, grade of hepatic encephalopathy and laboratory findings between the two groups. One out of 5 in group A and 9 out of 10 in group B survived. But all the children in group A who met the criteria for LT and received only supportive care died. One out of 10 in group B died because of grade IVa hepatic encephalopathy which advanced to brainstem herniation. CONCLUSION: This study showed that patients who were managed with supportive care only, although LT was indicated, died and that 9 out of 10 who received LT survived. Therefore, we suggest LT should be considered in the management of FH.
Adolescent ; Brain Stem ; Child* ; Factor V ; Female ; Hepatic Encephalopathy ; Hepatitis* ; Hepatolenticular Degeneration ; Herpesvirus 4, Human ; Humans ; Critical Care ; Jaundice ; Liver Transplantation* ; Liver* ; Male ; Medical Records ; Mortality ; Sex Ratio

We present a patient with moyamoya disease and a ruptured superior cerebellar artery aneurysm that was managed by endovascular embolization. A 53-year-old man with sudden onset severe headache and altered mental status was referred to our hospital. Computed tomography revealed a subarachnoid hemorrhage. Cerebral angiography showed evidence of moyamoya disease and a 7 mm saccular aneurysm at the origin of the right superior cerebellar artery. Endovascular coil embolization was performed successfully without posterior cerebral artery and superior cerebellar artery obliteration. Endovascular treatment with microcoils appear particularly safe for moyamoya patients with cerebral saccular aneurysms.
Aneurysm ; Arteries ; Cerebral Angiography ; Headache ; Humans ; Middle Aged ; Moyamoya Disease ; Polyenes ; Posterior Cerebral Artery ; Subarachnoid Hemorrhage

We report here on a case of a ruptured left posterior communicating artery (P-com) aneurysm that was treated by clipping in a patient with idiopathic thrombocytopenic purpura (ITP) and steroids were used to control the platelet count during the perioperative period. A 34-year-old female who had been suffering from ITP for four years experienced the sudden onset of a headache and vomiting while showering. She was referred to our hospital as a case of subarachnoid hemorrhage (SAH) due to a ruptured P-com aneurysm. Aneurysmal neck clipping was performed via the left pterional approach 6 hours after the ictus. The aneurysmal clipping was successful without an increased bleeding tendency during the operation. Intravenous steroid injection was given after aneurysmal clipping for 7 days and then it was tapered off. It is important to maintain an adequate platelet count in SAH patients with chronic ITP in order to avoid hemorrhagic diathesis during surgery. Intravenous steroid injection is a helpful method for maintaining an adequate platelet count in these patients during surgery.
Adult ; Aneurysm ; Arteries ; Female ; Headache ; Hemorrhage ; Hemorrhagic Disorders ; Humans ; Intracranial Aneurysm ; Neck ; Perioperative Period ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; Steroids ; Stress, Psychological ; Subarachnoid Hemorrhage ; Vomiting

The major pathogenesis of acute respiratory distress syndrome (ARDS) is an inflammatory process that results from a diversity of injuries to the body. Due to the various cytokines and vasoactive peptides released from the endothelium, the vascular permeability is increased; the migration of inflammatory cells and the leakage of plasma proteins then occur and edema develops in the alveolus. There is a hypothesis that the impairment of alveolar recruitment in ARDS is caused by a defect of the surfactant system and the resultant increase of alveolar surface tension. This has been studied in pediatric patients in ARDS; after the administration of surfactant, hypoxia, respiratory symptoms and survival chances were improved. To alleviate the major pathogenic mechanism in this disease, that is to say, inflammation of the lung, steroids have been used and studied as another treatment modality for ARDS, and it has been concluded that the administration of low dose methylprednisolone may improve patients' symptoms and survival rates. We report here on a case of a young infant admitted with ARDS, who, after the intratracheal administration of 120 mg/kg surfactant, on PaO(2)/FiO(2) was elevated. Subsequent low doses of methylprednisolone were given, and the symptoms did not recur, and no fibrotic change was shown during the follow-up period of 2 months.
Anoxia ; Blood Proteins ; Capillary Permeability ; Cytokines ; Edema ; Endothelium ; Follow-Up Studies ; Humans ; Infant ; Inflammation ; Lung ; Methylprednisolone* ; Peptides ; Respiratory Distress Syndrome, Adult* ; Steroids ; Surface Tension ; Survival Rate

OBJECTIVE: To evaluate the effect of short coasting, by withdrawing both gonadotropins and GnRH agonist (GnRHa), on the prevention in severe ovarian hyperstimulation syndrome (OHSS) without compromising pregnancy outcome. METHOD: Thirty-seven women who had been coasted during COH for IVF were coasted when > or =20 follicles > 15 mm with serum E2 level of 4,000 pg/ml were detected. Coasting was initiated for one or two days depending on the status of follicle on ultrasound and serum E2 level. Both gonadotropin and GnRHa were withheld for coasting. Retrospective study was carried and changes of serum E2 levels, number of oocytes retrieved, fertilization rate, pregnancy rate were compared and analyzed. RESULTS: The mean serum E2 level fell from 6,993 pg/ml on the onset of coasting to 3,396 pg/ml on the day of hCG administration. The mean number of oocytes retrieved and fertilization rate were 15.7 and 70.0%, respectively. Fifteen patients were pregnant (40.6%) and implantation rate was 15.2%. Twenty-six (70.3%) patients were coasted for one day and 11 (29.7%) were coasted for two days. The mean decrease rate of serum E2 level was 43% in one day coasting group and 15% (1st day) and 81% (2nd day) in two day coasting group. The pregnancy outcome was similar between the two groups. After coasting, no severe or moderate OHSS occurred in any patients and mild OHSS occurred in 3 (8.1%) patients. CONCLUSIONS: Coasting for one or two days can be used successfully in the prevention of OHSS without compromising IVF cycle outcome.
Female ; Fertilization ; Gonadotropin-Releasing Hormone* ; Gonadotropins* ; Humans ; Oocytes ; Ovarian Hyperstimulation Syndrome* ; Pregnancy ; Pregnancy Outcome ; Pregnancy Rate ; Retrospective Studies ; Ultrasonography

The present study evaluated the effect of various dosages of soybean isoflavone extract on body weight changes, glucose tolerance and liver function in streptozotocin-induced diabetic rats. One group of normal rats (normal control) was fed an AIN-76-based experimental diet and four groups of diabetic rats were fed the same diet supplemented with four different levels of soybean isoflavone extract for seven weeks. The daily dosages of pure isoflavone for four diabetic groups were set to be 0 mg (diabetic control), 0.5 mg (ISO-I), 3.0 mg (ISO-II) and 30.0 mg (ISO-III) per kilogram of body weight, respectively. The daily consumption of isoflavone at the level of 3.0mg per kilogram of body weight resulted in the suppression of body weight loss and increased the survival rate of diabetic animals one and half times compared to that of the diabetic control group. Blood glucose levels in a fasting state and after the oral administration of glucose were significantly lower in the ISO-II group during the oral glucose tolerance test. The ISO-II group showed a tendency to elongate the gastrointestinal transit time. The activity of serum aminotransferases, indicator of liver function, was not negatively affected by any intake level of isoflavone. The present study demonstrated that the soybean isoflavone extract may be beneficial to diabetic animals by improving their glucose tolerance and suppressing weight loss without incurring hepatotoxicity at the daily dosage of 3.0 mg per kg of body weight.
Administration, Oral ; Animals ; Blood Glucose ; Body Weight ; Body Weight Changes ; Diet ; Fasting ; Gastrointestinal Transit ; Glucose Tolerance Test ; Glucose* ; Liver ; Rats* ; Soybeans* ; Streptozocin ; Survival Rate* ; Transaminases ; Weight Loss

Hematological malignancies rarely affect the breast, and the majority of those that do are lymphomas. In this review, we describe the clinical aspects and multimodal imaging findings of breast lymphoma. We also illustrate the key clinical and radiological findings that allow it to be distinguished from various other malignant and benign diseases of the breast. Breast lymphoma manifests as a breast mass, a change in the subcutaneous tissue or the skin, or enlargement of the associated lymph node on radiological examination. Radiological findings associated with other breast malignancies, such as calcifications, spiculations, or architectural distortions are extremely rare. Skin and subcutaneous changes frequently accompany T-cell lymphoma. Multimodal breast imaging characteristics may aid in the diagnosis of breast lymphoma.
Breast ; Hematologic Neoplasms ; Lymph Nodes ; Lymphoma ; Lymphoma, T-Cell ; Magnetic Resonance Imaging ; Skin ; Subcutaneous Tissue