Hyperphosphatasemia in GM1 gangliosidosis.
Explore the source record for details and available documents.
Alkaline Phosphatase↗
Biomedical subjects
R Denis
Publications and source records attributed to R Denis.
Evaluation of a monocyte enrichment procedure using the blood cell separator Fresenius AS 104.
The small number of circulating monocytes in peripheral blood makes it difficult to collect enough cells for studies on the metabolism and analysis of receptor functions of this cell type for diagnostic aims and treatment. Therefore, a new procedure to enrich monocytes from peripheral blood using the cell separator Fresenius AS 104 was developed and evaluated. The separation is done using the tubing set 'P1Y-Gran-collect', which is a closed system with a single-stage separation chamber. The procedure is running continuously, while the cells are harvested cyclically. After processing of 4.9 l whole blood an average yield of (2.47 +/- 2.05) x 10(9) monocytes could be collected with an efficiency of 46 +/- 20%. The monocyte enrichment procedure using the Fresenius AS 104 provides a sufficient number of cells for the next steps: purification and concentration of the cells for drug monitoring, cell culturing, receptor analysis and further in vitro diagnostics.
Blood Cell Count↗
[Bone marrow graft in hereditary diseases].
Since 1968, bone marrow transplantation became the first line therapy for selected metabolic and immunological hereditary disorders. Actually, advances in the supportive care in bone marrow transplantation and a better knowledge of the immunology of BMT complications has been associated with a better disease correction and an increase in long term survival. New approaches are under investigation and include: hematopoietic growth factors, enzymatic replacement and gene therapy. However at the present time BMT is still the only curative treatment for selected hereditary disorders.
Bone Marrow Transplantation↗
[A utilization review program of cefoxitin].
A drug utilization review program of cefoxitin was conducted in a 714-bed teaching hospital. Health records of all 43 in-patients who received cefoxitin during the month of November 1987 were reviewed retrospectively. The use of cefoxitin (47 courses) was evaluated on the basis of "appropriate use" criteria developed from the literature and the physician's clinical experience. Of the 47 courses evaluated, cefoxitin was prescribed for prophylaxis in 47%, and its use was considered inappropriate in 86% of these. Overall, 66% of total cefoxitin usage was deemed inappropriate in this hospital. The cost associated with inappropriate use was estimated at $2672 for the period of the study. Corrective measures were then implemented to rectify the identified problems. A second study was conducted 2 years later to assess the impact of the corrective measures. This utilization review program of cefoxitin showed that optimal use of a drug requires not only close collaboration between pharmacists and physicians but a continuous and not a sporadic process of surveillance of the prescription for the drug being studied.
Bacterial Infections↗
Lysosomal storage diseases presenting as transient or persistent hydrops fetalis.
Two cases of beta-glucuronidase deficiency (mucopolysaccharidosis VII), presented with fetal hydrops at 20 and 26 weeks of gestation. The enzyme deficiency was observed in cultured amniotic fluid cells and in fetal plasma from cord-blood and was confirmed after termination of pregnancy. A third case presented with transient ascites at 6.5 months of gestation. Mild dysmorphic features at birth and gradual neurological deterioration were observed. Deficiency of beta-galactosidase was documented confirming a GM1 gangliosidosis. Evidence has accumulated that fetuses affected by lysosomal diseases, may present with transient or persistent hydrops fetalis. The exact frequency is however not known. Further diagnostic studies in persistent or transient hydrops fetalis, looking for lysosomal and other metabolic diseases, whenever major causes of hydrops fetalis have been excluded, are therefore indicated. Amniocentesis and cordocentesis should always be performed.
Chorionic Villi Sampling↗
The effects of Hespan on serum and lymphatic albumin, globulin, and coagulant protein.
The effects of hydroxyethyl starch (Hespan) resuscitation on serum and lymphatic proteins following hemorrhagic shock were studied in 34 splenectomized dogs. Following shock, five randomly assigned treatment groups received the shed blood plus 50 mL/kg of salt solution (RL) or RL with varying concentrations (0.22-1.5 gm/kg) of Hespan. Each dog received 50 ml/kg/d of the test solution for three days after shock. Prothrombin time, partial thromboplastin time, thrombin time, total serum protein, albumin, globulin, and coagulant protein activity of fibrinogen, prothrombin, and factor VIII were measured before shock, at the end of shock, following resuscitation, and on day 3; thoracic duct lymph values were obtained on day 3. Hespan-supplemented resuscitation lowered all serum proteins including albumin, globulin and coagulant proteins; concomitantly, the lymph protein rose after Hespan resuscitation. This decrease in serum proteins and rise in lymph proteins parallels similar results after albumin resuscitation in man and animals and suggests that Hespan induces an oncotically controlled extravascular protein relocation. Further studies on the significance of these findings need to be conducted.
Animals↗
Relocation of nonalbumin proteins after albumin resuscitation.
Prior work showed that albumin (5% A) resuscitation lowered serum globulins and coagulation protein activity levels compared to balanced electrolyte solution (BES) resuscitation. In this experiment, the effect of 1.25% A in BES (A-BES) on serum and lymphatic levels of nonalbumin proteins was studied in 20 splenectomized dogs subjected to reservoir shock and then treated with (a) BES or A-BES, (b) shed blood, and (c) autogenous bank blood. Serum levels of albumin, globulin, and coagulation protein activity were measured preshock, postshock, and daily for 3 days; skin lymph levels were measured on Day 3. Compared to BES, A-BES resuscitation significantly decreased serum globulins and coagulation protein activity of fibrinogen, Factor VIII, and antithrombin III. In contrast, skin lymph globulins and coagulation protein activity levels increased significantly with A-BES compared to BES resuscitation. These data suggest that the serum protein changes seen with albumin-supplemented resuscitation result from extravascular relocation of nonalbumin proteins, possibly, due to an oncotic homeostatic factor.
Animals↗
Mortality and morbidity related to severe intrapulmonary shunting in multiple trauma patients.
Of 210 multiple trauma patients admitted to our Intensive Care Unit (ICU), 12 (5%) presented with severe hypoxemic respiratory failure needing mechanical ventilation with an FIO2 of 1.0 because of severe intrapulmonary shunting (IS). Five (42%) of these patients survived and two (17%) died because of their underlying respiratory failure. We found a mean of three etiologic factors in each patient to account for their IS. Nonsurvivors had a lower cardiac index than survivors when they first needed FIO2 of 1.0 and ARDS was more frequent among this group. All patients who survived were in severe hypoxemic respiratory failure in the first 5 days post-trauma; all patients who needed FIO2 of 1.0 later than 5 days post-trauma died. Data collected for patients with similar degree of respiratory failure in coronary care ICU (n = 18), in medical ICU (n = 19), and surgical ICU (n = 21) demonstrated that multiple trauma patients with severe hypoxemic respiratory failure were younger and were hospitalized and ventilated for longer periods of time. In multiple trauma patients, as for patients with cardiogenic pulmonary edema, death was seldom related to respiratory failure itself. We concluded that severe hypoxemic respiratory failure in trauma patients is usually of mixed etiologies. It is a serious cause of morbidity in these patients; however, mortality is seldom directly related to this condition. Severe IS occurring shortly after trauma is of better prognosis than late IS.
Adult↗
Selection of aphasic stroke patients for intensive speech therapy.
Selection criteria for intensive speech therapy and the number of patients fulfilling these were investigated in 441 acute strokes coming from a Health District population during one year. Five patients from a total of 71 referred with speech and/or language difficulties were considered suitable for intensive speech therapy at 4 weeks after stroke. Although such therapy was not actually given, by 26 weeks three of the five had recovered and 14 further patients were considered suitable to receive intensive therapy. The most important selection criteria were Functional Communication Profile score of less than 85 and a clinical judgement that the patient was well enough to take part. It is recommended that decisions as to appropriateness of patients for intensive speech therapy be delayed beyond 4 weeks after stroke.
Aged↗
Effects of exercise training on energy balance of ovariectomized rats.
The purpose of the present study was to investigate both the respective and interactive roles of exercise training and ovarian hormones on the regulation of energy balance. Female rats were divided into sedentary and exercise-trained groups. Each group thus formed was further divided into a sham-operated group, an ovariectomized group, or ovariectomized estradiol-treated group. Rats were exercise trained on a rodent motor-driven treadmill. After 33 days of treatment, rats were killed and the energy contents of carcasses, feces, and food were determined. Brown adipose tissue (BAT) thermogenesis was assessed through mitochondrial GDP binding. The results show that ovariectomy led to increases in food intake, body weight, and protein gains, whereas estradiol treatment abolished these effects. The results also show that exercise training reduced fat gain. Exercise training interacted with ovariectomy on energy gain; in sedentary rats ovariectomy enhanced the energy gain, an effect that disappeared in exercise-trained rats. However, exercise training was found to alter neither body weight and protein gains nor energy intake. Ovariectomy did not affect energy expenditure when the results are expressed in relative terms (kJ.kg body wt-0.67.day-1). Similarly, exercise training did not modify energy expenditure (kJ.kg body wt-0.67.day-1) once the cost of the training program was subtracted. BAT mitochondrial GDP binding was not affected by any of the experimental treatments. The present results therefore suggest that neither ovariectomy nor exercise training affect energy expenditure through regulatory forms of BAT-mediated thermogenesis.
Adipose Tissue, Brown↗
The effect of steroids for shock on the immune response to tetanus toxoid.
The effect of corticosteroids on the immune response after severe injury and hemorrhagic shock was studied in 88 patients, who received an average of 13 blood transfusions for injury and hemorrhagic shock, which caused the systolic blood pressure to be below 80 torr for an average of 29 minutes. The immune response to tetanus toxoid was tested in the postoperative period. Besides the administration of blood, crystalloid solution, and plasma for coagulation factor deficiency, 42 patients also received methyl-prednisolone 1 g during additional resuscitation followed by an average of 15 mg/kg given daily for the next 2 days. The total dose of methylprednisolone averaged 3.9 g. The two groups of patients were similar for resuscitation needs and for insult. The immune response to tetanus toxoid was not significantly different between the two groups of patients. These data show that a short-term bolus of massive steroids does not appear to alter, significantly, the immune mechanism following severe hemorrhagic shock.
Adult↗
Spatulated versus end-to-end anastomosis for small vessel injury.
Spatulated anastomosis (SA) was compared to end-to-end anastomosis (EEA) with small arteries (3-4 mm) in nine conditioned dogs (22-30 kg). A 1-cm segment of both common femoral arteries with an average of 3.5-mm external diameter was resected and reconstructed by EEA and by contralateral SA using a running suture of 5/0 proline. Pre- and postoperative flow rates, flow rates at 1 year in three dogs, arteriography at 1 year in six dogs, and gross examination at 1 year were done. The preoperative flow rates averaged 86 ml/min for the SA and 76 ml/min for the EEA; early postoperative flow rates averaged 100 ml/min and 92 ml/min, respectively, whereas 1-year flow rates averaged 63 ml/min and 57 ml/min, respectively. None of these differences is significant. Preoperative and 1-year external diameters averaged 3.54 mm and 3.39 mm in the SA group compared to 3.5 mm and 3.44 mm in the EEA group. Arteriograms showed good flow except for slight narrowing in one SA which, on postmortem exam, was seen to result from a fibrous band which extended from the spatulated segment to the opposite wall. These data show that SA and EEA yield comparable results both acutely and long term. The choice of EEA versus SA for primary repair of injured small vessels should be determined by surgical preference.
Animals↗
Influence of auditory or visual warning on visual reaction time with variations of subjects' alertness.
The purpose of the study was to determine the effect of visual and auditory warning on visual reaction time with variations of subjects' alertness. An experimental group of 30 subjects was tested with an auditory or visual warning signal; foreperiods lasted 3, 2, and 4 sec. Reaction time was shorter as alertness improved and with an auditory warning signal. Comparable measures in a control group showed that visual reaction time was shorter when an auditory warning signal was used.
Adult↗
Review of the immunological aspects of the pathogenesis, diagnosis and prognosis of urinary tract infections.
Explore the source record for details and available documents.
Adult↗
Societal impact on surgical education in trauma.
Societal and demographic changes within Detroit have affected the incidence of trauma treated at the Detroit Medical Center. Using liver injury as an index, this phenomenon was studied by comparing 122 patients treated in 1972 with 38 patients treated in 1981. During 1972 76 patients had major injuries, compared to 13 patients in 1981. Intraoperative hemostasis of an actively bleeding liver in 1972 was achieved by liver suture (43 patients), resectional debridement through a nonanatomic plane (three patients), hepatic artery ligation (three patients), anatomic segmentectomy or lobectomy (four patients), and intrahepatic packing with later removal (one patient). During 1981, 13 patients had persistent bleeding requiring intraoperative hemostasis by liver suture (12 patients) or resectional debridement (one patient). This decline in trauma reflects a reduced city population, the dilutional effect of a four-quadrant emergency medical service begun in the mid 1970's, the implementation of a state gun law, and reduced citizen compliance to violence. These changes likely are not unique to Detroit and highlight the need for a National Trauma Registry which would not only help program directors design a meaningful trauma rotation but also help hospital planners accommodate to changes in patient exposure.
General Surgery↗
The beneficial role of calcium supplementation during resuscitation from shock.
The role of calcium (Ca) in resuscitation from hemorrhagic shock is controversial and in the present report three regimens were compared: supplementation (Ca-S), avoidance (No-Ca), and Ca channel blockade (Ca-B). This was studied in 40 splenectomized dogs subjected to reservoir shock (MAP 60 torr/90 min, then 40 torr/30 min) and treated with: a) 20 ml/kg balanced electrolyte solution (BES); b) shed blood; c) 30 ml/kg BES; and d) 250 ml autologous bank blood. Three groups of six dogs received Ca-S (0.5 mEq/kg), No-Ca, or Ca-B (verapamil 0.15 mg/kg) in BES. Postoperative therapy of 50 ml/kg/d BES with Ca-S, No-Ca, or Ca-B was given for 3 days. The effects of parathyroidectomy (P) via wide thyroidectomy in 22 dogs treated with calphosan (20 ml/d) and L-thyroxin (0.02 mg/kg) preceding shock was also studied as above: Ca-S/P, No-Ca/P, and Ca-B/P; four sham dogs had anesthesia but no shock (Anes/P). Studies done before, during, and after shock and on day three included systemic pressures (MAP), central pressures (CFP), cardiac output (CO), resistance (SVR), heart work (LVW), and outcome. Post-resuscitation Ca was significantly less in all groups (1.6-3.7 mg%) compared to Ca-S (4.8 mg%). Compared to Ca-S dogs, the post-resuscitation studies in the No-Ca and Ca-B dogs showed lower MAP, CO, and LVW in both intact and hypoparathyroid animals. Post-resuscitation CFP was also lower in the Ca-S and Ca-S/P dogs compared to the other euparathyroid and hypoparathyroid dogs. Death after the initiation of resuscitation occurred in two No-Ca/P and three Ca-B/P dogs. These data suggest that calcium supplementation plus an intact calcium-parathyroid axis enhance the resuscitation effort.
Animals↗
[Seat belt syndrome: a new abdominal pathology].
Between January 1976 and December 1980, 43 patients involved in automobile accidents while wearing passive restraints were treated at l'Hôpital du Sacré-Coeur in Montreal. Thirty-seven showed signs of abdominal trauma and were operated upon. There was a strikingly high incidence of gastrointestinal injury. Thirty-two of the 37 had injury to the bowel or its mesentery. This spectrum of injury is most likely related to the rapid deceleration caused by the restraint with the three-point harness.
Abdominal Injuries↗