Many patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome(TTP-HUS) satisfactorily respond to plasma exchange. Some patients, however, respond either not at all or only transiently and incompletely. In the refractory case, endothelial cell-derived unusually large von Willebrand factor multimers(ULvWFM) have an important role in the formation of microthrombi. As the ULvWFM may be removed in the cryoprecipitate, we reason the plasma depleted of cryoprecipitate(the plasma cryosupernatant) should be considered for effectiveness in the treatment of refractory TTP- HUS. We experienced a 48 year old woman presented with diarrhea, jaundice and oliguria. She had microangiopathic hemolytic anemia, renal impairment, platelets of 21,000/mm3 and LDH 3,258U/L. She had not improved after plasma exchange with fresh frozen plasma(FFP)(1.5 plasma volumeX7 days). On hospital day 8, her HUS had not responded, platelets of 37,000/mm3 and LDH 1,588U/L. Substitution of cryosupernatant for FFP was associated with prompt increased in the platelet count to normal and complete resolution of HUS. Therefore. the cryosupernatant fraction of plasma should be considered as an alternative to whole FFP for plasma exchange if there is continuing platelet consumption and microvascular thrombosis in spite of intensive conventional plasma therapy.
Anemia, Hemolytic ; Blood Platelets ; Diarrhea ; Female ; Hemolytic-Uremic Syndrome* ; Humans ; Jaundice ; Middle Aged ; Oliguria ; Plasma Exchange* ; Plasma* ; Platelet Count ; Thrombosis ; von Willebrand Factor

Kallmann's syndrome is a rare condition, and this is defined as hypogonadotropic hypogonadism and anosmia or hyposmia. The syndrome may be associated with cleft lip, cleft palate, color blindness, skeletal abnormalities, renal agenesis, sensory neural hearing loss, obesity, etc. About 10 cases of Kallmann's syndrome have been reported in Korea, but there are no reports on cases of Kallmann's syndrome with atrophy of the frontal lobe, severe mental retardation and unilateral renal agenesis. We experienced a case of 17-year-old boy with abnormalities of the olfactory system, as was noted on magnetic resonance imaging (MRI). He had an atrophy of the frontal lobe, mental retardation, a micropenis and unilateral renal agenesis. Hormonal assay documented low levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone and thyroid-stimulating hormone (TSH). So, we report here on an unusual case of Kallmann's syndrome along with briefly reviewing the relevant medical literature.
Adolescent ; Atrophy ; Cleft Lip ; Cleft Palate ; Color Vision Defects ; Congenital Abnormalities ; Follicle Stimulating Hormone ; Frontal Lobe ; Genital Diseases, Male ; Hearing Loss ; Humans ; Hypogonadism ; Intellectual Disability ; Kallmann Syndrome ; Kidney ; Kidney Diseases ; Korea ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Obesity ; Olfaction Disorders ; Penis ; Testosterone ; Thyrotropin

No abstract available.
Peritoneal Dialysis, Continuous Ambulatory*

No abstract available.
Peritoneal Dialysis, Continuous Ambulatory*

Although hemodialysis using heparin bound Hemophan(HBH-HD) has been reported to be a possible modality that can be used in patients at high risk of bleeding, the efficiency of HBH-HD is not certain. To investigate the efficiency of HBH- HD, we compared the total blood compartment volume(TBCV), Kt/V and urea clearance of dialyzer(K) of HBH-HD with those of routine hemodialysis with systemic heparinization(R-HD) in the same patients. HBH-HD was switched to R-HD as soon as the bleeding risk had ceased. Before each HBH-HD, heparin solution(1liter, 20IU/ml saline) was recirculated through the Hemophan(Gambro dialyzer, GFS Plus 11) for 1 hour while removing saline solution(700ml/hr) by applying transmembrane pressure gradient, followed by a single pass rinse with 1 liter of saline solution. Then we performed 10 HBH-HD on 10 patients at risk of bleeding. The dilayzer had to be changed due to severe clotting in one patient during HBH-HD so the comparison of above parameters was possible in 9 patients. The duration of each dialysis was possible in 9 patients. The duration of each dialysis was standardized to 4 hours at blood flow of 200 to 250ml/min. During HBH-HD, there was a slight increase in activated partial thromboplastin time(aPTT)(45.02.6 sec) at 15 min after initiation of dialysis from predialysis level (35.81.3 sec), but no increase in aPTT was observed at 60min, 120min, and the end of dialyses. The loss of TBCV(%) of dialyzers was greater in HBH-HD (174%) than in R-HD(51%). The Kt/V and K of HBH-HD, however, were 1.25+/-0.10 and 143+/-3ml/ min, respectively, which did not differ from those of R-HD which were 1.28+/-0.07 and 145+/-4ml/min, respectively. We conclude that the use of heparin bound Hemophan can be an efficient hemodialysis technique in patients at high risk of bleeding, but clotting of the dialyzer should be observed carefully during hemodialysis(values are mean+/-SE).
Dialysis* ; Hemorrhage ; Heparin* ; Humans ; Renal Dialysis* ; Sodium Chloride ; Thromboplastin ; Urea

BACKGROUND: A Stellate ganglion block (SGB) is a sympathetic nerve block method which has been used most frequently in pain clinics due to its wide range of indications. However, SGB leads to regional sympathetic denervation of the heart and to changes in the hemodynamics. The aim of this study was to compare hemodynamic effects as well as echocardiographic changes after a left SGB (LSGB). METHODS: Fourteen healthy male volunteers were studied. The LSGB was performed with 1% mepicacaine 6 ml. Arterial blood pressure, electrocardiographic and echocardiographic variables were measured before the LSGB, 15 and 30 minutes after the LSGB. RESULTS: Arterial blood pressure, ejection fraction and transmitral inflow velocity variables showed no significant changes compared to pre-LSGB values. P-P interval increased significantly 15 minutes after the LSGB, and the Q-T interval increased significantly 30 minutes after the LSGB. The diastolic pulmonary venous flow velocity decreased significantly 15 minutes after the LSGB. CONCLUSIONS: These results showed that a LSGB decreased the heart rate without detrimental changes of left ventricular relaxation in healthy male volunteers.
Arterial Pressure ; Autonomic Nerve Block ; Echocardiography ; Electrocardiography ; Heart ; Heart Rate ; Hemodynamics ; Humans ; Male ; Pain Clinics ; Relaxation ; Stellate Ganglion* ; Sympathectomy ; Ventricular Function, Left* ; Volunteers

Yerba Mate, derived from the leaves of the tree, Ilex paraguariensis, is widely-used as a tea or as an ingredient in formulated foods. The aim of the present study was to evaluate the effects of Yerba Mate extract on weight loss, obesity-related biochemical parameters, and diabetes in high-fat diet-fed mice. To this end, by using in vivo animal models of dietary-induced obesity, we have made the interesting observations that Yerba Mate has the ability to decrease the differentiation of pre-adipocytes and to reduce the accumulation of lipids in adipocytes, both of which contribute to a lower growth rate of adipose tissue, lower body weight gain, and obesity. Our data from in vivo studies revealed that Yerba Mate treatment affects food intake, resulting in higher energy expenditure, likely as a result of higher basal metabolism in Yerba Mate-treated mice. Furthermore, in vivo effects of Yerba Mate on lipid metabolism included reductions in serum cholesterol, serum triglycerides, and glucose concentrations in mice that were fed a high fat diet. In conclusion, Yerba Mate can potentially be used to treat obesity and diabetes.
Adipocytes ; Adipose Tissue ; Animals ; Basal Metabolism ; Body Weight ; Cholesterol ; Diet, High-Fat ; Eating ; Energy Metabolism ; Food, Formulated ; Glucose ; Ilex paraguariensis ; Lipid Metabolism ; Mice ; Models, Animal ; Obesity ; Tea ; Trees ; Triglycerides ; Weight Loss