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

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 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

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

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

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

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