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

Pituitary apoplexy is a clinical syndrome caused by an acute ischemic or hemorrhagic vascular accident involving a pituitary adenoma or an adjacent pituitary gland. Pituitary apoplexy may be associated with a variety of neurological and endocrinological signs and symptoms. However, isolated third cranial nerve palsy with ptosis as the presenting sign of pituitary apoplexy is very rare. We describe two cases of pituitary apoplexy presenting as sudden-onset unilateral ptosis and diplopia. In one case, brain magnetic resonance imaging (MRI) revealed a mass in the pituitary fossa with signs of hemorrhage, upward displacement of the optic chiasm, erosion of the sellar floor and invasion of the right cavernous sinus. In the other case, MRI showed a large area of insufficient enhancement in the anterior pituitary consistent with pituitary infarction or Sheehan's syndrome. We performed neurosurgical decompression via a transsphenoidal approach. Both patients showed an uneventful recovery. Both cases of isolated third cranial nerve palsy with ptosis completely resolved during the early postoperative period. We suggest that pituitary apoplexy should be included in the differential diagnosis of patients presenting with isolated third cranial nerve palsy with ptosis and that prompt neurosurgical decompression should be considered for the preservation of third cranial nerve function.
Brain ; Cavernous Sinus ; Decompression ; Diagnosis, Differential ; Diplopia ; Displacement (Psychology) ; Floors and Floorcoverings ; Hemorrhage ; Humans ; Hypopituitarism ; Infarction ; Magnetic Resonance Imaging ; Oculomotor Nerve ; Optic Chiasm ; Paralysis ; Pituitary Apoplexy ; Pituitary Gland ; Pituitary Neoplasms ; Postoperative Period

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

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

BACKGROUND: Severe bradycardia occurring in strabismus surgery is unpredictable and may lead to cardiac arrest. If we identify patients who have risk of severe bradycardia during strabismus surgery, it is helpful to provide a more meticulous anesthesia. In this study, we investigated the correlation between the degree of bradycardia and indices of power spectral analysis and nonlinear dynamic data analysis of preoperative ECG. METHODS: ECG was collected for twenty minutes from strabismus patients (n = 93). No premedicants were administered. After administration of anesthesia and traction of extraocular muscle, the lowest heart rate was recorded. We calculated the correlation coefficient between the power spectral density, approximate entropy and correlation dimension of preoperative ECG and the difference between preoperative and lowest HR. RESULTS: As the difference between the preoperative heart rate and the intraoperative bradycardia increased, the preoperative ECG showed a low total power (r = 0.27, P = 0.016), low low-frequency power (r = 0.31, P = 0.049), low high-frequency power (r = 0.30, P = 0.007) and high ratio of low to high-frequency power (r = 28, P = 0.03). There was no correlation between bradycardia and approximate entropy and correlation dimension, respectively. CONCLUSIONS: There was a correlation between indices of power spectral analysis of preoperative ECG and degree of bradycardia during strabismus surgery. Therefore we take into consideration preoperative ECG and its analytic indices in order to provide careful preparation for high risk patients who exhibit a tendency to severe bradycardia.
Anesthesia ; Bradycardia* ; Electrocardiography* ; Entropy ; Heart Arrest ; Heart Rate ; Humans ; Nonlinear Dynamics ; Reflex, Oculocardiac ; Statistics as Topic ; Strabismus* ; Traction

No abstract available.
Catheters* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Fetal Therapies* ; Lung*

Microalbuminuria is a marker of generalized endothelial dysfunction resulting from arterial stiffness or insulin resistance, and brachial-ankle pulse wave velocity (baPWV) is a good measure of arterial stiffness. We aimed to investigate whether elevated baPWV is independently associated with microalbuminuria. This study included 1,648 individuals aged over 40 who participated in the baseline Multi-Rural Cohort Study conducted in Korean rural communities between 2005 and 2006. Participants were classified into less than 30 mg/g as normoalbuminuria or 30-300 mg/g as microalbuminuriausing urinary albumin creatinine ratio (UACR). The median and Q1-Q3 baPWV values were significantly higher in the microalbuminuric group both in men (1,538, 1,370-1,777 cm/s vs. 1,776, 1,552-2,027 cm/s, P < 0.001) and women (1,461, 1,271-1,687 cm/s vs. 1,645, 1,473-1,915 cm/s, P < 0.001). BaPWV was independently associated with microalbuminuria in both genders after adjusting for pulse rate; fasting blood glucose; triglyceride; homeostatic model assessment insulin resistance (HOMA(IR)) and, history of hypertension and diabetes. Fasting blood sugar and HOMA(IR) were judged as having nothing to do with multicolinearity (r = 0.532, P < 0.001). Elevated baPWV was independently associated with microalbuminuria regardless of insulin resistance among rural subjects over 40 yr.
Adult ; Aged ; Albuminuria/*diagnosis/etiology/metabolism ; Ankle Brachial Index ; Ankle Joint/*physiopathology ; Blood Chemical Analysis ; Blood Glucose/analysis ; Brachial Artery/*physiopathology ; Cohort Studies ; Diabetes Mellitus, Type 2/complications/diagnosis ; Female ; Humans ; Hypertension/complications/diagnosis ; Male ; Middle Aged ; *Pulse Wave Analysis ; Risk Factors ; *Rural Population ; Serum Albumin/analysis ; Triglycerides/blood ; Vascular Stiffness