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

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

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

OBJECTIVE: Distal middle cerebral artery (MCA) aneurysms are the least frequent aneurysms of the MCA, and they represent about 1.1 to 5% of all MCA aneurysms. Patients with ruptured distal MCA aneurysms generally have a poor clinical outcome. The purpose of this article is to review the characteristics of distal MCA aneurysms to avoid the complications of microsurgical dissection and clipping of distal MCA aneurysms. METHODS: A total of 1187 patients with ruptured aneurysms were treated at our hospital between January 1997 and May 2008. All patients underwent surgical procedures. Computed tomography (CT) revealed rupture of distal MCA aneurysms in 15 (1.26%) patients. The location of the aneurysm were the M2 (insular) segment in seven patients, the M2-3 junction in three and the M3 (opercular) segment in five. Brain CT images revealed the presence of both subarachnoid hemorrhage (SAH) and intracranial hemorrhage (ICH) in 11 of 15 (77.3%) patients, with a mean ICH volume of 14.5 cc (range : 5 to 32 cc). Rebleeding occurred in 7 out of the 15 (46.7%) patients. RESULTS: All the patients underwent early surgical procedures, including clipping in seven, trapping in two, bypass surgery in four, Guglielmi detachable coil embolization in one and exploratory craniotomy in one patient. The aneurysm had a fusiform appearance in 9 out of 15 cases (60%), and the mean size of the aneurysm was 10.4 mm (range : 2 to 35 mm). Three patients died due to severe brain swelling (20%). CONCLUSION: In this study, distal MCA aneurysms had a relatively fusiform shape as well as high rates of rebleeding and ICH. A good clinical outcome was associated with early surgery for adequately controlling brain swelling and preventing rebleeding.
Aneurysm ; Aneurysm, Ruptured ; Brain ; Brain Edema ; Cerebral Hemorrhage ; Craniotomy ; Humans ; Intracranial Aneurysm ; Intracranial Hemorrhages ; Middle Cerebral Artery ; Rupture ; Subarachnoid Hemorrhage

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

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