Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involve by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief of the literature.
Adult ; Angiomyolipoma* ; Epilepsy ; Female ; Humans ; Intellectual Disability ; Lung ; Lymphangioleiomyomatosis* ; Muscle, Smooth ; Penetrance ; Rare Diseases ; Tuberous Sclerosis*

No abstract available.
Hemolytic-Uremic Syndrome* ; Postpartum Period*

PURPOSE: The release of histamine from human basophils is controlled by an intrinsic, as yet unidentified, cellular property termed "releasability." We carried out this study to ascertain whether there was any difference in the releasability of basophils from asthmatic children compared to those from normal children. We intended also to clarify the correlation between the releasability and the atopic status of asthma. METHODS: We selected nineteen atopic asthmatic, eighteen nonatopic asthmatic and fourteen normal children for this study. Suspensions of leukocytes were isolated and stimulated with calcium ionophore A23187, anti-IgE and D. pteronyssinus antigen. After incubation, the supernatant was assayed for histamine with an automated fluorometric technique. RESULTS: Basophil histamine release with anti-IgE was different in three groups. Anti-IgE caused significantly more basophil histamine release in asthmatic children than in nomal children. Atopic asthmatic group showed greater basophil histamine release with anti-IgE than nonatopic asthmatic group. D. pteronyssinus antigen caused the significant amount of histamine release only in atopic asthmatic group. CONCLUSIONS: Our data suggests that basophils from asthmatic children are characterized by a specific increase in IgE mediated histamine releasability. The difference of histamine releasability with anti-IgE between atopic and nonatopic asthmatic children may be due to the heterogeneity of IgE bound to cell surface, or may be due to the degree of the basophil activation by cytokines such as IL3. The specific release of histamine with D. pteronyssinus antigen in atopic asthmatic group suggests that the basophil histamine release test can be used to diagnose the causing antigen.
Asthma ; Basophils* ; Calcimycin ; Calcium ; Child* ; Cytokines ; Histamine Release ; Histamine* ; Humans ; Immunoglobulin E ; Leukocytes ; Population Characteristics ; Suspensions

Anthocyanin pigments from varieties of black, red and wild rice were identified and quantified to evaluate their potential as nutritional function, natural colorants or functional food ingredients. Anthocyanin extraction was conducted with acidified methanol with 0.1M HCl (85:15, v/v) and identification of anthocyanin, aglycone and sugar moieties was conducted by comparison with purified standards by HPLC, Ultraviolet-Visible absorption spectrophotometer and paper chromatography. Black and wild rice showed three different types of pigments by HPLC whereas red rice variety did not show any anthocyanins. Out of three pigments detected, one (peak 2) was characterized as cyanidin-3-glucoside (C3G) by comparison of spectroscopic and chromatographic properties with an authentic standard, and another (peak 3) was tentatively identified as cyanidin-fructoside on the basis of spectroscopic properties with lambdamax of aglycone in 1% HCl methanol at 537 nm, electrospray ionization mass spectra with major ions at 449 and 287 m/z and chromatographic properties. But another pigment (peak 1) has not been characterized. The most abundant anthocyanin in black and wild rice was C3G.
Absorption ; Anthocyanins ; Chromatography, High Pressure Liquid ; Chromatography, Paper ; Functional Food ; Glucosides ; Ions ; Methanol

To achieve adequate depth of sedation and assess the severity of pain in mechanically ventilated patients in the intensive care unit, appropriate communication with the patients is necessary. Communication is also important for successful weaning from the mechanical ventilator as well as weaning predictors, such as respiratory muscle capacity. Here, we present a case report of a 39-year-old man with congenial blindness and hearing impairment who successfully weaned off ventilator support using Braille to communicate under an optimal level of sedation and analgesia after septic shock management.
Adult ; Analgesia ; Blindness ; Hearing ; Hearing Loss ; Humans ; Intensive Care Units ; Respiration, Artificial ; Respiratory Muscles ; Shock, Septic ; Ventilators, Mechanical ; Weaning

BACKGROUND: Success of transplantation is critically dependent upon the quality of the donor organ and optimal management. Recently, hormonal replacement therapy has been reported to result in rapid recovery of cardiac function and enable significantly more organs to be transplanted, while some other studies show conflicting results. The aim of this study is to comprehensively evaluate changes in basal circulating hormonal levels of the brain-dead organ donors. METHODS: We reviewed the records of all brain-dead patients between January, 2004, and June, 2007. Hemodynamic variables, plasma hormone levels were recorded at following time points: admission to the ICU (T1, baseline), 30 minutes (min) after first apnea test (T2), 30 min after second apnea test (T3), before operation for harvesting (T4). Hormonal measurements included cortisol, adrenocorticotrophic hormone, triiodothyronine (T(3)), thyroxine, free thyroxine, thyroid-stimulating hormone, growth hormone, and testosterone. RESULTS: Nineteen patients were included in this study. Comparisons of hemodynamic parameters and hormonal levels to baseline values revealed no significant changes throughout the study period. When the patients were divided into 2 groups according to the requirement of norepinephrine (either>0.05 or < or =0.05microgram/kg/min), patients requiring >0.05microgram/kg/min of norepinephrine had T(3) level below the normal range at significantly more time points of measurement (7 vs. 0). CONCLUSION: In this comprehensive assessment of hormonal levels in brain-dead organ donors, we could not observe any significant changes during the ICU stay. Replacement therapy of T(3) may be considered in patients requiring >0.05microgram/kg/min of norepinephrine.
Adrenocorticotropic Hormone ; Apnea ; Growth Hormone ; Hemodynamics ; Humans ; Hydrocortisone ; Norepinephrine ; Plasma ; Reference Values ; Testosterone ; Thyrotropin ; Thyroxine ; Tissue Donors ; Transplants ; Triiodothyronine

Although venous thrombosis is one of the most frequent complications of malignant disease including multiple myeloma, massive pulmonary embolism is an uncommon complication. Free-floating intracardiac thrombus has been rarely reported as a cause of acute pulmonary embolism in patients with multiple myeloma. We report a case presenting acute pulmonary embolism due to free-floating right atrial thrombus in a patient with multiple myeloma.
Humans ; Multiple Myeloma ; Pulmonary Embolism ; Thrombosis ; Venous Thrombosis

PURPOSE: To assess the effectiveness of subsegmental transcatheter arterial chemoembolization(TAE) forhepatocellular carcinomas(HCCs) on the basis of tumor necrosis rate. MATERIAL AND METHODS: Between May 1996 andMarch 1998, ten patients with single HCC after subsegmental TAE underwent surgical resection. Subsegmental TAE wasperformed by injecting a mixture of lipiodol and adriamycin followed by gelatin sponge particles into the distalbranches of the subsegmental arteries. Tumor size and the extent of necrosis were analyzed in ten resectedlesions, and in all patients, complications after subsegmental TAE were assessed. RESULTS: The size of resectedtumors ranged from 1 to 5,5cm. On histological examination, complete necrosis was seen in 6 to 10 resected lesionsand 95% necrosis in three. In the remaining lesion, 85% necrosis had occured. Complete necrosis was noted in 4 of6 small HCCs(less than 3cm in diameter), while in the remaining two the extent of necrosis was 95%. Nocomplications were observed. CONCLUSION: For the treatment of HCC, subsegmental TAE is safe and effective.Curative therapy must, however, involve follow-up and repeated TAE.
Arteries ; Carcinoma, Hepatocellular* ; Doxorubicin ; Ethiodized Oil ; Follow-Up Studies ; Gelatin ; Humans ; Liver Neoplasms ; Necrosis ; Porifera

Anticoagulant ileus is a rare complication that is caused by an intramural hematoma, and the hematoma is due to excessive anticoagulant therapy. The features of anticoagulant ileus vary from slight abdominal pain to vomiting, severe abdominal pain and abdominal rebound tenderness that are due to intestinal obstruction. Anticoagulant ileus is sometimes accompanied by gastrointestinal bleeding, peritoneal bleeding and panperitonitis due to intestinal perforation. The diagnosis of anticoagulant ileus has been made using such radiographic methods as enhanced abdominal computed tomography and small bowel follow-through. We were able to confirm a case of anticoagulant ileus with bleeding by performing double balloon enteroscopy. We report on this case and we review the relevant literature.
Abdominal Pain ; Double-Balloon Enteroscopy ; Hematoma ; Hemorrhage ; Ileus ; Intestinal Obstruction ; Intestinal Perforation ; Vomiting

BACKGROUND AND PURPOSE: Rapid recanalization might improve clinical outcomes after intraarterial thrombolysis (IAT) for acute ischemic stroke patients with collateral circulation. We determined whether rapid recanalization and collateral circulation affect clinical outcomes after IAT. METHODS: We retrospectively evaluated the clinical and radiological data of 134 consecutive patients who underwent IAT for intracranial artery occlusion. The interval from symptom onset to recanalization after IAT (onset-to-recanalization time) as an estimate of the probability of good clinical outcome (modified Rankin scale 0-2) was calculated in patients with collateral circulation in the ischemic hemisphere, which was rated poor (0/1 American Society of Interventional and Therapeutic Neuroradiology criteria) or good (2-4). Changes in National Institute of Health Stroke Scale (NHISS) score before and after IAT and modified Rankins scale scores 3 months after discharge were compared with respect to onset-to-recanalization time. RESULTS: In patients with good collateral circulation, the estimated onset-to-recanalization time for a 0.5 probability of a good clinical outcome was 347 minutes; with poor collateral circulation, it was 172 minutes for a 0.2 probability of good clinical outcome. Outcome analyses according to onset-to-recanalization time showed patients recanalized <6 hours had lower NHISS scores (<4.5, 4.5-6, >6 hours of onset-to-recanalization time, and non-recanalization: 5.1, 6.9, 11.9, and 19.8, respectively) at discharge and higher percentages of good clinical outcome (69%, 66.7%, 21.9%, and 0%, respectively) 3 months after IAT. CONCLUSIONS: The time window to expect a high probability of a good clinical outcome after IAT is highly dependent on the collateral circulation.
Arteries ; Collateral Circulation* ; Humans ; Retrospective Studies ; Stroke