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

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

OBJECTIVE: The incidence of subarachnoid hemorrhage (SAH) worldwide varies considerably. In spite of many reports about the incidence of SAH, there has been no report about the incidence of SAH on the basis of the Korean population. The purpose of this hospital-based study was to assess the actual incidence rates of aneurysmal SAH in Gwangju city and Jeollanamdo province. METHODS: All cases of SAH confirmed by computerized tomography (CT) between January 2007 and December 2007 were selected for analysis. For the data collection, three major training hospital and ten general hospitals working the CT in Gwangju city and four major general hospitals in Jeollanamdo province participate in this study. RESULTS: According to the official census of Korea, the population was 1,413,444 in Gwangju city and 1,929,836 in Jeollanamdo province in 2007. There were 163 patients in Gwangju city and 266 patients in Jeollanamdo province confirmed SAH by CT in 2007. The crude and the age- and sex-adjusted annual incidence rates per 100,000 population for all ages in Gwangju city were 11.5 and 12.4 for aneurysmal SAH and in Jeollanamdo province were 13.8 and 10.8. The incidence was higher in women and increased with age. The gender distribution varied with age. At young ages, the incidence was higher in men while after the age of 40 years, the incidence was higher in women. CONCLUSION: In the present study, the age- and sex-adjusted annual incidence rates is 11.8 in Gwangju city and Jeollanamdo province. The incidence was higher in women and increased with age.
Aneurysm ; Censuses ; Data Collection ; Female ; Hospitals, General ; Humans ; Incidence ; Korea ; Male ; Risk Factors ; Subarachnoid Hemorrhage

A 32-year-old multiparous woman (gravida 2, para 2) with a history of previous cesarean section had acute abdominal pain and collapsed at 21 weeks of gestation. Exploratory laparotomy was performed because of the patient's worsening condition; ultrasound examination results were suggestive of massive hemoperitoneum, and fetus in vertex presentation with bradycardia. Uterine rupture between the left lower segment and borderline of the cervix in the anterior wall with active bleeding was confirmed. An uncomplicated classical cesarean section was performed, but the fetus was stillborn due to preterm birth. Hysterectomy was performed after the cesarean section. The patient was admitted to intensive care units for 3 days and was discharged in 12 days following delivery. Placenta percreta at the anterior lower segment of the uterus was confirmed in the pathology report.
Abdominal Pain ; Adult ; Bradycardia ; Cervix Uteri ; Cesarean Section ; Female ; Fetus ; Hemoperitoneum ; Hemorrhage ; Humans ; Hysterectomy ; Intensive Care Units ; Laparotomy ; Pathology ; Placenta Accreta* ; Placenta* ; Pregnancy ; Pregnancy Trimester, Second* ; Pregnancy* ; Premature Birth ; Ultrasonography ; Uterine Rupture* ; Uterus

Most of anti-incontinence surgeries bear the risk of postoperative complication such as voiding dysfunction due to urethral obstruction. Fortunately, Tension Free Vaginal Tape(TVT) procedure has much lower incidence of postoperative urethral obstruction than other surgical procedures for stress urinary incontinence. There are many reports about the surgical treatments for short-term urethral obstruction after TVT procedure. However, there are few reports on the effect of surgical releasing of the obstruction lasting for a long period. In our case, the patient had urethral obstruction for 32 months after TVT procedure and she was able to void well after surgical releasing of the tape. We suggest that releasing of the tape will be a treatment of choice for long-term urethral obstruction after TVT procedure.
Humans ; Incidence ; Postoperative Complications ; Suburethral Slings* ; Urethral Obstruction* ; Urinary Incontinence

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