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At least 955 records · Page 53Linked to original sources
PubMed2004

Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are now considered to be variants of one single syndrome called thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS). Key features are thrombocytopenia, hemolytic anemia, and subsequently impaired function of different organs, especially the kidneys and the central nervous system (CNS). One possible reason is the deficiency of von Willebrand factor-cleaving protease (vWF-CP) resulting in persistence of uncleaved, ultralarge von Willebrand factor multimers (ULvWFM). METHODS: We report a patient who was initially diagnosed with Evans syndrome (hemolytic anemia and autoimmune thrombocytopenia) as infant. At 10 years of age he developed HUS-like disease with gastrointestinal tract infection, hemolytic anemia, thrombocytopenia,and acute renal failure. However, enteropathogenic Escherichia coli-like or Shiga-like toxins were not detected. RESULTS: Further investigations revealed severe deficiency (<3%; normal >40%) of vWF-CP activity caused by compound heterozygosity of two novel ADAMTS13 gene mutations (1170 G>C [W390C] and 3735 G>A [W1245X]. vWF-CP autoantibodies were not detected. Periodic (every 2 weeks) treatment with fresh frozen plasma (FFP) maintained both platelet level and kidney function within normal range and prevented new episodes of TTP/HUS. CONCLUSION: Enteropathogenic E. coli- and Shiga-like toxin-negative patients who present with hemolytic or thrombocytopenic episodes and HUS like symptoms should be tested for vWF-CP deficiency and other noninfectious reasons for TTP/HUS since plasma substitution possibly provides an efficient therapeutic option for this subgroup of patients.

ADAM Proteins↗
PubMedDate not supplied

An argument for colloid resuscitation for shock.

A focused review of the physiologic mechanisms of colloid and crystalloid fluid resuscitations for acute critical illness is presented. This review suggests that postresuscitation plasma volume, cardiac output, left ventricular mechanical performance, and global and microcirculatory O2 supplies are more favorable with colloid therapy. Conversely, crystalloid may adversely affect microcirculatory blood flow and resultant O2 supply and use by ischemic tissues in shock. Poor relief of global and regional hypoxia may persist in critically ill patients after resuscitation with crystalloid.

Cardiac Output↗
PubMed1977

Dependence on external calcium for the noradrenaline contractility of the resistance vessels in spontaneously hypertensive and renal hypertensive rats, as compared with normotensive controls.

Isolated hindquarters of rats were perfused at constant flow with a plasma substitute so that pressor responses to various concentrations of noradrenaline (NA) could be measured in consective sections of the vascular bed with normal (greater than 1.5 mM) or low (0.2 mM) Ca2+ in the perfusate. The animals used were 6-week-old spontaneously hypertensive rats of the Okamoto strin (SHRy), normotensive controls of the same age NCRy); 6--7-month-old SHR (SHRA) and NCR (NCRA); and rats made hypertensive by clipping of one renal artery (RHR) plus matched normotensive controls (NCRR). Concentration-response curves to NA showed that constrictor responses to NA become more dependent on external calcium as one proceeds peripherally in the vascular system in both SHRA and NCRA, with responses in proximal vessels being least and small pre- and postcapillary vessels being most dependent in both cases. In low calcium SHRA retained their responses better than NCRA, whereas RHR retained their responses to NA poorer than did NCRR. No significant differences in responses in low calcium were observed in SHRY compared to NCRY though a trend in the same direction as in SHRA could be traced. It is concluded that there is no evidence that altered handling of calcium initiates vascular hyperreactivity in SHR, but that the handling of vascular calcium in SHR differs from RHR and both differ from NCR.

Animals↗
PubMed1980

A new technique for maintaining and monitoring conscious, stress-free rabbits in a steady state: its use in the determination of glucose kinetics.

1. A new technique is described for maintaining and monitoring conscious rabbits in a steady state. Solutions were infused and blood samples were withdrawn without major surgery and without causing stress. Cannulation, for infusion and blood sampling, was via the ear veins and arteries. 2. The validity of the technique is discussed with special reference to: the amount of blood removed from the animal; the use of a plasma substitute solution; the use of heparin; and assessing whether the animal was in a steady-state with respect to glucose. 3. The technique was used to determine glucose kinetics in the fasted rabbit after an injection of [2-(3)H]glucose with subsequent blood sampling over 2 h. A two compartment model was used to calculate certain physiological values, the two principal ones being the glucose uptake rate (3.30 +/- 0.24 (13) mg.kg-1.min-1) and the initial volume of distribution (74.8 +/- 7.8 (13) ml.kg-1). 4. The accuracy and precision of the results obtained from this study are discussed and shown to be more reliable than other published values.

Animals↗
PubMed1986

Skin necrosis following Haemaccel.

Haemaccel is a plasma substitute in frequent clinical use with a low incidence of side effects. I report a patient who developed necrotic blisters following two Haemaccel-insulin infusions, a previously undescribed complication. The rationale behind carrier solutions for insulin therapy is discussed and some of the problems associated with Haemaccel use are considered. I conclude by urging caution in the use of Haemaccel as a carrier solution for insulin therapy.

Humans↗
PubMed2004

Plasma volume expansion does not increase maximal cardiac output or VO2 max in lowlanders acclimatized to altitude.

With altitude acclimatization, blood hemoglobin concentration increases while plasma volume (PV) and maximal cardiac output (Qmax) decrease. This investigation aimed to determine whether reduction of Qmax at altitude is due to low circulating blood volume (BV). Eight Danish lowlanders (3 females, 5 males: age 24.0 +/- 0.6 yr; mean +/- SE) performed submaximal and maximal exercise on a cycle ergometer after 9 wk at 5,260 m altitude (Mt. Chacaltaya, Bolivia). This was done first with BV resulting from acclimatization (BV = 5.40 +/- 0.39 liters) and again 2-4 days later, 1 h after PV expansion with 1 liter of 6% dextran 70 (BV = 6.32 +/- 0.34 liters). PV expansion had no effect on Qmax, maximal O2 consumption (VO2), and exercise capacity. Despite maximal systemic O2 transport being reduced 19% due to hemodilution after PV expansion, whole body VO2 was maintained by greater systemic O2 extraction (P < 0.05). Leg blood flow was elevated (P < 0.05) in hypervolemic conditions, which compensated for hemodilution resulting in similar leg O2 delivery and leg VO2 during exercise regardless of PV. Pulmonary ventilation, gas exchange, and acid-base balance were essentially unaffected by PV expansion. Sea level Qmax and exercise capacity were restored with hyperoxia at altitude independently of BV. Low BV is not a primary cause for reduction of Qmax at altitude when acclimatized. Furthermore, hemodilution caused by PV expansion at altitude is compensated for by increased systemic O2 extraction with similar peak muscular O2 delivery, such that maximal exercise capacity is unaffected.

Acclimatization↗
PubMed1981

Bainbridge reflex in conscious, unrestrained, and tranquilized baboons.

The effects of volume loading were examined in conscious, unrestrained, and tranquilized baboons (20-25 kg) 1-3 mo after instrumentation with miniature left ventricular pressure and diameter gauges. The response to volume loading was accompanied by significant increases (P less than 0.01) in cardiac rate (32 +/- 4 beats/min) in tranquilized baboons. When rapid saline infusion was accomplished in conscious unrestrained baboons by means of activating a remote-controlled interrogator, heart rate also rose significantly (41 +/- 8 beats/min, P less than 0.01). After cholinergic blockade with atropine, ganglionic blockade with hexamethonium, bilateral vagotomy, or combined cholinergic and beta-adrenergic blockades, volume loading failed to increase heart rate. In two smaller (12-14 kg) intact conscious baboons with relatively high spontaneous heart rates, volume loading did not alter heart rate. These experiments suggest that the Bainbridge reflex exists in larger primates with relatively low spontaneous heart rates.

Animals↗
PubMed1983

Hormonal and renal response to plasma volume expansion in the primate Macaca mulatta.

The purpose of this study was to investigate the hormonal and renal response to plasma volume expansion in the ketamine-anesthetized rhesus monkey. The blood volume was determined in nine animals and found to be 6% of the body weight. Six monkeys received isoncotic isotonic fluid amounting to 25% of the blood volume. Plasma volume expansion led to significant decrease in the plasma concentrations of antidiuretic hormone (46.7%) and aldosterone (78.4%) as well as plasma renin activity (50.0%). The mean arterial pressure, plasma osmolality, and plasma concentrations of Na+ and K+ were unaffected by plasma volume expansion. However, renal plasma flow, glomerular filtration rate, the excretion of Na+ and K+, and urine flow increased. It was concluded that, in the ketamine-anesthetized rhesus monkey, circulating hormones contribute to blood volume homeostasis presumably through a neural mechanism similar to that observed in dogs and humans.

Aldosterone↗
PubMed1999

Plasma volume expansion with solutions of hemoglobin, albumin, and Ringer lactate in sheep.

We have measured plasma volume expansion (Evans blue and hematocrit changes) and hemodynamic responses in conscious hemorrhaged and normovolemic splenectomized sheep after a 30-min infusion of either 20 ml/kg of diaspirin cross-linked hemoglobin (DCLHb), 20 ml/kg of human albumin (Alb), or 60 ml/kg of a solution of Ringer lactate (RL). All regimens expanded blood volume and increased blood pressure and cardiac output after hemorrhage. However, only 15 +/- 3% of the infused volume of RL was evident as intravascular expansion 10-min postinfusion, compared with 67 +/- 16% and 139 +/- 139% for Alb and DCLHb, respectively. DCLHb infusions were associated with higher blood pressures and lower cardiac outputs compared with RL and Alb infusions, but the increased oxygen content of blood with DCLHb resulted in systemic delivery of oxygen similar to that of the other infusions. These differences in hemodynamics and vascular volume continued for 6 h, and at 24 h vascular volume and all hemodynamics were similar in all three groups. The better volume expansion with DCLHb may be due to greater mobilization of endogenous interstitial protein or reduced transcapillary loss as total intravascular endogenous plasma protein increased after infusion of DCLHb, whereas there was an apparent loss of endogenous intravascular protein after infusions of Alb and RL. Vasoconstriction by DCLHb is one mechanism that could lower blood-to-tissue transport of fluid and protein. In addition to its oxygen-carrying capacity and vasoactivity, DCLHb is associated with volume expansion properties out of proportion to its colloid osmotic pressure.

Animals↗
PubMed2001

Volume expansion potentiates cardiac sympathetic afferent reflex in dogs.

Our previous study (27) showed that the cardiac sympathetic afferent reflex (CSAR) was enhanced in dogs with congestive heart failure. The aim of this study was to test whether blood volume expansion, which is one characteristic of congestive heart failure, potentiates the CSAR in normal dogs. Ten dogs were studied with sino-aortic denervation and bilateral cervical vagotomy. Arterial pressure, left ventricular pressure, left ventricular epicardial diameter, heart rate, and renal sympathetic nerve activity were measured. Coronary blood flow was also measured and, depending on the experimental procedure, controlled. Blood volume expansion was carried out by infusion of isosmotic dextran into a femoral vein at 40 ml/kg at a rate of 50 ml/min. CSAR was elicited by application of bradykinin (5 and 50 microg) and capsaicin (10 and 100 microg) to the epicardial surface of the left ventricle. Volume expansion increased arterial pressure, left ventricular pressure, left ventricular diameter, and coronary blood flow. Volume expansion without controlled coronary blood flow only enhanced the RSNA response to the high dose (50 microg) of epicardial bradykinin (17. 3 +/- 1.9 vs. 10.6 +/- 4.8%, P < 0.05). However, volume expansion significantly enhanced the RSNA responses to all doses of bradykinin and capsaicin when coronary blood flow was held at the prevolume expansion level. The RSNA responses to bradykinin (16. 9 +/- 4.1 vs. 5.0 +/- 1.3% for 5 microg, P < 0.05, and 28.9 +/- 3.7 vs. 10.6 +/- 4.8% for 50 microg, P < 0.05) and capsaicin (29.8 +/- 6.0 vs. 9.3 +/- 3.1% for 10 microg, P < 0.05, and 34.2 +/- 2.7 vs. 15.1 +/- 2.7% for 100 microg, P < 0.05) were significantly augmented. These results indicate that acute volume expansion potentiated the CSAR. These data suggest that enhancement of the CSAR in congestive heart failure may be mediated by the concomitant cardiac dilation, which accompanies this disease state.

Animals↗
PubMed1999

Effects of a three-day head-down tilt on renal and hormonal responses to acute volume expansion.

To clarify whether exposure to 6 degrees head-down tilt (HDT) leads to alterations in body fluid volumes and responses to a saline load similar to those observed during space flight we investigated eight healthy subjects during a 4-day, 6 degrees HDT and during a time-control ambulatory period with cross-over. Compared with the ambulatory period, HDT was associated with greater urinary excretion of water and sodium (UV, U(Na)V) from 0 to 12 h (cumulated UV 1,781 +/- 154 vs. 1,383 +/- 170 ml, P < 0.05; cumulated U(Na)V 156 +/- 14 vs. 117 +/- 9 mmol, P < 0.05), and with higher plasma atrial natriuretic factor (ANF) at 4 h. Hemoglobin and hematocrit increased over the first 24 h, and blood and plasma volumes were decreased after 48 h of HDT (P < 0.05). Plasma renin activity (PRA) and aldosterone did not differ between the two groups. With prolongation of HDT, UV and U(Na)V returned close to baseline values. On the fourth HDT day, a 30-min infusion of 20 ml/kg isotonic saline was performed, while a large oral water load maintained a high urine output. The ambulatory period experiment was done with the subjects in the acute supine posture. Sodium excreted within 4 h of loading was 123 +/- 8 mmol during HDT vs. 168 +/- 16 mmol during the ambulatory period (P < 0.05). The increase in plasma ANF and decrease in PRA were greater during HDT than during the ambulatory period (ANF 30 +/- 5 vs. 13 +/- 4 pg/ml, P < 0.05; PRA -1.4 +/- 0.4 vs. -0.5 +/- 0.2 ng. ml(-1). h(-1), P < 0.05). Our data suggest that after a 3-day HDT period, thoracic volume receptor loading returns to the level seen in the upright position, leading to blunted responses to volume expansion, compared with acute supine control.

Adult↗
PubMed2006

Influence of acute plasma volume expansion on VO2 kinetics, VO2 peak, and performance during high-intensity cycle exercise.

The purpose of this study was to examine the influence of acute plasma volume expansion (APVE) on oxygen uptake (V(O2)) kinetics, V(O2peak), and time to exhaustion during severe-intensity exercise. Eight recreationally active men performed "step" cycle ergometer exercise tests at a work rate requiring 70% of the difference between the gas-exchange threshold and V(O2max) on three occasions: twice as a "control" (Con) and once after intravenous infusion of a plasma volume expander (Gelofusine; 7 ml/kg body mass). Pulmonary gas exchange was measured breath by breath. APVE resulted in a significant reduction in hemoglobin concentration (preinfusion: 16.0 +/- 1.0 vs. postinfusion: 14.7 +/- 0.8 g/dl; P < 0.001) and hematocrit (preinfusion: 44 +/- 2 vs. postinfusion: 41 +/- 3%; P < 0.01). Despite this reduction in arterial O(2) content, APVE had no effect on V(O2) kinetics (phase II time constant, Con: 33 +/- 15 vs. APVE: 34 +/- 12 s; P = 0.74), and actually resulted in an increased V(O2peak) (Con: 3.90 +/- 0.56 vs. APVE: 4.12 +/- 0.55 l/min; P = 0.006) and time to exhaustion (Con: 365 +/- 58 vs. APVE: 424 +/- 64 s; P = 0.04). The maximum O(2) pulse was also enhanced by the treatment (Con: 21.3 +/- 3.4 vs. APVE: 22.7 +/- 3.4 ml/beat; P = 0.04). In conclusion, APVE does not alter V(O2) kinetics but enhances V(O2peak) and exercise tolerance during high-intensity cycle exercise in young recreationally active subjects.

Adult↗
PubMed1975

Studies on the oxygen supply of heterotopic auxiliary liver grafts in rats.

Heterotopic auxiliary liver transplantations were performed in rats using the method of Hess et. al. [6]. Oxygen pressure of liver tissue was measured with a Pt-surface electrode of Kessler and Lübbers [10]. After ligation of the hepatic artery, liver tissue PO2 decreased and remained low during further preparation. Only two of the seven lobes of the rat liver (= 30% of the parenchyma) were used as a graft. After revascularization and application of a gelatine plasma substitute, tissue PO2 of the graft increased to values of normal rat liver, although the graft was supplied with portal blood only. We suggest that one reason for this is the increase of portal perfusion rate, for the whole amount of portal blood perfuses only 30% of liver parenchyma. In the first month after transplantation PO2 of the graft parenchyma was low, but increased continually in the following months, reaching PO2 values of normal rat livers after 1 year.

Animals↗
PubMed1976

Cardiocirculatory and biochemical effects of dextran 1.8% and ringer's lactate after minor surgical procedures.

The effects of dextran 1.8% in Ringer's lactate solution were compared with infusion of Ringer's lactate alone in two groups of 12 patients after minor surgical interventions. Plasma volume increased significantly only in the dextran treatment patients. We observed a marked hemodilution with both infusions, but all changes had disappeared after 24 hours. No adverse effects on the coagulation process, hepatic and renal function were noted. These results suggest that dextran 1.8% in Ringer's lactate is an effective and safe solution in plasma volume expansion for this type of patient.

Adolescent↗