Allogeneic bone marrow transplantation in a child with severe aplastic anemia and hemophilia A.
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Anemia, Aplastic↗
Biomedical subjects
G Campos
Publications and source records attributed to G Campos.
Plasma fibrinogen level and the risk of major cardiovascular diseases and nonvascular mortality: an individual participant meta-analysis.
CONTEXT: Plasma fibrinogen levels may be associated with the risk of coronary heart disease (CHD) and stroke. OBJECTIVE: To assess the relationships of fibrinogen levels with risk of major vascular and with risk of nonvascular outcomes based on individual participant data. DATA SOURCES: Relevant studies were identified by computer-assisted searches, hand searches of reference lists, and personal communication with relevant investigators. STUDY SELECTION: All identified prospective studies were included with information available on baseline fibrinogen levels and details of subsequent major vascular morbidity and/or cause-specific mortality during at least 1 year of follow-up. Studies were excluded if they recruited participants on the basis of having had a previous history of cardiovascular disease; participants with known preexisting CHD or stroke were excluded. DATA EXTRACTION: Individual records were provided on each of 154,211 participants in 31 prospective studies. During 1.38 million person-years of follow-up, there were 6944 first nonfatal myocardial infarctions or stroke events and 13,210 deaths. Cause-specific mortality was generally available. Analyses involved proportional hazards modeling with adjustment for confounding by known cardiovascular risk factors and for regression dilution bias. DATA SYNTHESIS: Within each age group considered (40-59, 60-69, and > or =70 years), there was an approximately log-linear association with usual fibrinogen level for the risk of any CHD, any stroke, other vascular (eg, non-CHD, nonstroke) mortality, and nonvascular mortality. There was no evidence of a threshold within the range of usual fibrinogen level studied at any age. The age- and sex- adjusted hazard ratio per 1-g/L increase in usual fibrinogen level for CHD was 2.42 (95% confidence interval [CI], 2.24-2.60); stroke, 2.06 (95% CI, 1.83-2.33); other vascular mortality, 2.76 (95% CI, 2.28-3.35); and nonvascular mortality, 2.03 (95% CI, 1.90-2.18). The hazard ratios for CHD and stroke were reduced to about 1.8 after further adjustment for measured values of several established vascular risk factors. In a subset of 7011 participants with available C-reactive protein values, the findings for CHD were essentially unchanged following additional adjustment for C-reactive protein. The associations of fibrinogen level with CHD or stroke did not differ substantially according to sex, smoking, blood pressure, blood lipid levels, or several features of study design. CONCLUSIONS: In this large individual participant meta-analysis, moderately strong associations were found between usual plasma fibrinogen level and the risks of CHD, stroke, other vascular mortality, and nonvascular mortality in a wide range of circumstances in healthy middle-aged adults. Assessment of any causal relevance of elevated fibrinogen levels to disease requires additional research.
Adult↗
Effect of acute sublethal endotoxaemia on in vitro digital vascular reactivity in horses.
Endotoxaemia is a syndrome linked to the development of equine laminitis; however, the relationship between them is uncertain. The aim of this experiment was to evaluate the effect of an experimental acute sublethal endotoxaemia model on in vitro equine palmar digital vascular reactivity. Rings of arteries and veins of each forelimb were obtained from 11 clinically healthy horses submitted to two surgical procedures, 3 weeks apart. Before the second surgery, 0.25 microg/kg of lipopolysaccharide from Escherichia coli O55:B5 in saline, was administered i.v. in 30 min. After 3 h, the vessels were harvested and submitted to in vitro vascular reactivity experiments and histopathology. The response to depolarizing Krebs solution (DKS, 40 mm), phenylephrine (PHE), acetylcholine (ACh) and sodium nitroprusside (SNP) were evaluated. All horses showed colic pain and watery diarrhoea, tachycardia, tachypnea, hyperthermia and leucopenia. Concentration-response curve (CRC) to PHE was shifted to the left in arteries rings from endotoxemic horses without any effect on vein rings. The CRC to ACh was shifted to the right with a reduction in the maximal response. The response to SNP and DKS was similar between groups. There was no evidence of histopathological effects. The increased response to PHE in digital arteries together with a reduction of the endothelium-dependent response to ACh in arteries and veins, confirm the existing reports where endotoxaemia was found to modify the digital vascular reactivity during the acute phase. As the digital endothelial function is impaired, there may be an increased potential to develop a digital prothrombotic state with a reduced vasodilatory capacity.
Acute Disease↗
Successful nonmyeloablative bone marrow transplantation in a corticosteroid-resistant infant with Diamond-Blackfan anemia.
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Adrenal Cortex Hormones↗
Skeletal muscle adaptations in elastic resistance-trained young men and women.
Skeletal muscle adaptations (fiber-type composition, cross-sectional area, myosin heavy chain (MHC) content, and capillarity) were assessed in the vastus lateralis muscle of young men and women after 8 weeks of training with the Sportcord, an elastic resistance device. Ten men [mean (SD) age 20 (1.1) years] and 13 women [20 (1.2) years] performed two sets each to failure of single leg squats and leg extensions at approximately 50 repetitions x min(-1). Biopsy samples were taken from the right vastus lateralis muscle before and after training. Six fiber types (I, IC, IIC, IIA, IIAB, and IIB) were classified using myofibrillar ATPase histochemistry. Training with the Sportcord caused a small, but significant, increase in one-repetition maximum using free weights and a large increase in repetitions to failure. In addition, elastic resistance training caused an increase in the percentage of fibers classified as type IIAB for both men and women, and a decrease in the percentage of type IIB fibers in the men. MHC analysis supported these findings (a significant increase in the percentage of MHCIIa for the men). The cross-sectional areas ofboth the type I and IIAB + IIB fibers increased after training for the men, whereas no area changes were found for the women. The capillary:fiber ratio and capillary contacts per fiber type increased significantly for the men, and similar trends were noted for the women. Capillary density did not change in either the men or the women. These data suggest minor changes in fiber type composition (IIB-->IIAB), fiber size, and capillarization following short-term training with elastic resistance. Although muscular changes did occur using the Sportcord, the extent of these changes was less than those reported previously for short-term resistance-training programs using free weights.
Adaptation, Physiological↗
[Nutritional and metabolic factors as risk factors for cardiovascular diseases in an adult population in the city of Maracibo, Estado Zulia, Venezuela].
To analyze the nutritional and metabolic risk factors for Cardiovascular Diseases (CVD) present in a group of people in the city of Maracaibo a study was performed with 209 volunteers (145 women and 64 men) between 20 and 89 years of age who underwent: a) Anthropometric Evaluation: Body Mass Index (BMI) and Waist-to-Hip Ratio (WHR) and Physical Examination: Systolic (SBP) and Diastolic Blood Pressure (DBP); b) Dietetic Evaluation (24 hours recall), and c) Biochemical Evaluation: Glycemia (GLYC), Triglycerides (TG), Total Cholesterol (CHOL), HDL-C, LDL-C and VLDL-C, applying enzymatic methods. It was also investigated, their Age, Family History of Metabolic Alterations (FHMA), physical activity, smoking habits and alcohol consumption. More than 50% of the individuals showed a BMI > 25; 64% of women showed a WHR value > 0.8; 34 and 28% of men and women respectively had a high fat ingestion (HFI); 36% of men had hypertriglyceridemia and high levels of VLDL-C; 41% of women and 30% of men showed decreased HDL-C. A high frequency of FHMA was found in 85% of women and 78% of men followed by sedentary life in 64% of men and 79% of women. The age significantly (p < 0.05) affected the values for WHR, SBP, DBP, GLYC, CHOL, TG, HDL-C, LDL-C and VLDL-C. The dietetic evaluation showed a diet that was low in calories, high in protein, normal in fat and low in carbohydrates. It is concluded that the population elected for this study might be considered under a high risk for CVD, since both nutritional and metabolic factors, as well as the other risk factors analyzed, were present in a high percentage of the individuals studied.
Adult↗
[Glucose/insulin response to a glucose overload in subjects at risk for type 2 diabetes].
With the purpose of determining how certain risk factors for type 2 diabetes such as family history of diabetes, obesity and dyslipidemia, affect the glucose-insulin response to a glucose challenge, 135 individuals (77 women and 58) men were studied. Their ages ranged from 20-68 years, their basal glycemic values were less than 110 mg/dL but they were considered at risk for diabetes due to the presence of one or more of those factors. We found that the presence of those risk factors did not affect the glycemic response in any case. However, the basal insulin levels as well as the post-challenge values were increased significantly (p < 0.0001) by the presence of obesity in men as well as in women. Dyslipidemia increased the basal and post challenge glucose insulin values only in men (p < 0.002). The coexistence of obesity and family history of diabetes provoked a decrease in the basal insulin levels as well as in the insulin response to glucose. We conclude that, without alteration of the glycemic response, the presence of risk factors as obesity, dyslipidemia or family history of diabetes leads to basal hyperinsulinemia, as well as glucose stimulated hyperinsulinemia, however the coexistence of obesity and family history of diabetes, is responsible for a deficit in the insulin secretion by the pancreas.
Adult↗
Intraabdominal abscess rate after laparoscopic appendectomy.
BACKGROUND: Studies suggest increased intraabdominal abscess (IA) rates following laparoscopic appendectomy (LA), especially for perforated appendicitis. Consequently, an open approach has been advocated. The aim of our study is to compare IA rates following LA performed by a laparoscopic surgery and a general surgical service within the same institution. METHODS: Data of LA patients treated at Los Angeles County-University of Southern California (LAC-USC) Medical Center between March 1992 and June 1997 were reviewed. The main outcome measure was postoperative IA. RESULTS: In all, 645 LA were reviewed. A total of 413 LA (285 acute, 61 gangrenous, 67 perforated appendicitis) were performed by three general surgical services (10 attendings). Ten abscesses occurred postoperatively (2.4%), 6 with perforated appendicitis. After the laparoscopic service was introduced, 232 standardized LA (126 acute, 46 gangrenous, 60 perforated) were performed by two attendings. One IA occurred (gangrenous appendicitis). The IA rate for perforated appendicitis was significantly lower on the laparoscopic service (P = 0.025). There was no difference in IA rates for acute and gangrenous appendicitis. There was no mortality in either group. CONCLUSION: IA rate following LA for perforated appendicitis was significantly reduced on the laparoscopic service. Mastery of the learning curve and addition of specific surgical techniques explained this improved result. Therefore, laparoscopic appendectomy for complicated appendicitis may not be contraindicated, even for perforated appendicitis.
Abdominal Abscess↗
Women relatives of Hispanic patients with type 2 diabetes are more prone to exhibit metabolic disturbances.
Hyperinsulinemia and impaired insulin action are familial and predictive of Type 2 diabetes onset. Since high levels of insulin are characteristic of our general (venezuelan)hispanic population, the purpose of this investigation was to identify early metabolic defects in a group of healthy first degree relatives of Type 2 diabetic patients. We studied 46 (29 women and 17 men; ages ranging 18-66 y) first degree relatives of Type 2 diabetic patients comparing them with 22 (12 women and 10 men; ages ranging 22-60 y) subjects who had no family history of diabetes. All subjects underwent resting blood pressure and anthropometric measurements; a 75 g oral glucose tolerance test with determination of glucose and insulin and a fasting lipid profile. The relatives of Type 2 diabetic patients had higher tricipital (TC) and subscapular (SC) skinfolds, and elevated DBP in relation to the control group. The skinfolds elevation was more evident in women, while in men the elevation in DBP predominates. None of the relatives had glucose intolerance, however, the glucose-stimulated insulin response was elevated at all points in men as well as in women. No difference was observed in the HOMA values for IR and beta cell function, or in the delta I30/delta G30 ratio. The lipid profile showed a marked elevation in TG levels in men as well as in women, with low HDL-C values in men. No other lipid abnormalities were observed. Correlation analysis revealed strong association between BMI and WHR with skinfolds and several parameters of the carbohydrate metabolism in women, but not in men. IR in women was possitively associated with skinfolds, SBP and lipid parameters and beta cell function with VLDL-C. Adult relatives of Type 2 diabetic venezuelan patients from hispanic origin had, early in their lives, several parameters of the metabolic syndrome as hyperinsulinemia, obesity, dyslipidemia and high blood pressure. These alterations were more prominent in women, group in which the association among BMI, WHR and IR were statistically significant respect to SBP, DBP, basal insulin, insulin/glucose ratio, TG and HDL-C.
Adult↗
Improved triglyceride control with low glycaemic index-high carbohydrate modified-lipid diet in a hypertriglyceridaemic child.
This study reports a 7-y-old boy with severe hypertriglyceridaemia who was successfully treated for 6 y with a low glycaemic index-high carbohydrate modified-lipid diet that produced beneficial changes in triglyceride and total cholesterol levels. It is suggested that a selection of a complex digestible carbohydrate and an adequate ratio between polyunsaturated and monounsaturated fat may, in the long term, favourably improve the lipid profile.
Child↗
Isozyme analysis of human polymorphonuclear leukocyte phosphofructokinase from insulin resistant individuals.
Phosphofructokinase (PFK) from human polymorphonuclear leukocytes (PMN) was characterized by immunological titration with subunit specific antibodies and column chromatography on QAE-Sephadex in three different groups: control, type II diabetic, and obese individuals. It was found that PMN phosphofructokinase in the three groups consists mainly of a mixture of L4 and M4 homotetramers with possibly some hybrid forms. The predominant subunit was the L-type. A 24% decrease in the specific activity of the L-type isozyme was observed and an intermediate form (I-isozyme) having 23% of the total activity in diabetic individuals appeared. In obese individuals a 30% decrease was observed in the activity of M-type isozyme and 9% of the total activity corresponded to the intermediate form. Kinetic studies showed different regulatory properties among the isozymes from the three groups. The lower PFK activity found in diabetic and obese individuals can be associated with the decreased activity in the L-type isozyme (for diabetic individuals) and in the M-type isozyme (for obese individuals); the lower activity can also be associated with the four times lower affinity for F-6-P showed by the M-type isozyme, the decreased sensitivity to ATP inhibition (for both isozymes), and the appearance of an intermediate form with a different kinetic behaviour.
Adenosine Triphosphate↗
Effects of hormone replacement modalities on low density lipoprotein composition and distribution in ovariectomized cynomolgus monkeys.
This study was designed to determine the effect of several hormone replacement therapies on LDL size, density, heterogeneity, and composition in surgically postmenopausal cynomolgus monkeys fed an atherogenic diet. Groups (n = 5 each) of ovariectomized cynomolgus monkeys were untreated (control), or treated with conjugated equine estrogens, medroxyprogesterone acetate (progesterone), combined estrogen-progesterone, or tamoxifen for 9 weeks. There were no differences among treatment groups in total plasma, LDL, or HDL cholesterol or triglyceride concentrations. Plasma LDL were isolated by ultracentrifugation and size exclusion chromatography and subfractionated by density gradient centrifugation for subsequent chemical analysis. Estrogen treatment was associated with significantly smaller (measured as LDL molecular weight, 3.9 +/- 0.2 g/mu mol) and denser plasma LDL (1.034 g/ml peak density) compared with control (4.5 +/- 0.1 g/mu mol; 1.030 g/ml peak density) or progesterone-treated animals (4.6 +/- 0.2; 1.029 g/ml peak density). LDL from the estrogen group were relatively enriched in protein and triglyceride and poor in cholesteryl ester and apolipoprotein F (apoE) compared to the control group. Triglyceride enrichment with estrogen treatment occurred predominantly in the lighter, larger LDL subfractions (d = 1.015-1.025 g/ml), which were reduced in concentration (26 +/- 10 mg cholesterol/dl) compared to control (61 +/- 19 mg/dl) or progesterone treated animals (67 +/- 16 mg/dl). Combined estrogen-progesterone or tamoxifen treatment resulted in changes in LDL that followed the same trend as those observed with estrogen treatment. We conclude that short-term estrogen treatment of ovariectomized cynomolgus monkeys results in changes in plasma LDL size, density, and composition while having no apparent effect on overall plasma lipid concentrations.
Administration, Oral↗
[Qualitative and quantitiative differences in the plasma lipoproteins of obese, hyperlipidemic or normolipidemic men and women].
The aim of this study was to evaluate the alterations of the lipoprotein composition and their relation with the insulin-resistance and/or hyperinsulinemia in non diabetic obese patients. Twenty-two no obese(13 women and 9 men) and 30 obese patients (BMI > 30) were studied, who were divided into two groups according to the total lipid levels. The first group was formed by 18 obese patients (10 women and 8 men) with normal serum cholesterol (Chol) concentration < 200 mg/dL and triglycerides (TG) < 150mg/dL (NO), while the second group were formed by 12 obese patients (3 women and 9 men) with elevated Chol level > 200mg/dL and/or TG > 150 mg/dL (HO). A clinical and anthropometric examination was performed to each patient, as well as a glucose tolerance test, including serum glucose and insulin determinations. Likewise, the plasma lipoproteins (VLDL, LDL, HDL2 and HDL3) were isolated by ultracentrifugation and their cholesterol and triglycerides content were determined by enzymatic methods. In this report, we demonstrate the existence of compensatory basal hyperinsulinemia in men and women on both obese patients populations as well as alterations in the lipoprotein composition, mostly a TG overload even on NO. On the other hand, the presence of lipids and lipoproteins modification were obvious in those patients with abdominal obesity, on whom the hyperinsulinemia was more evident, which could be related with the high risk of cardiovascular disease in this kind of patients.
Adult↗
Isozyme analysis of human normal polymorphonuclear leukocyte phosphofructokinase.
Phosphofructokinase (PFK) from human polymorphonuclear leukocytes (PMN) was characterized by immunological titration with subunit specific antibodies, column chromatography on QAE-Sephadex and SDS-polyacrylamide gel electrophoresis. Two different isozymes, M-type and L-type, were found. The M(r) values of the M and L subunits were 79,500 +/- 1,914 and 74,250 +/- 1,258, respectively. The two isozymes presented different kinetic and regulatory properties. The results suggest that PFK from human normal PMN is a mixture of M-type and L-type homotetramers, mainly, with possible minor heterotetrameric forms.
Adenosine Triphosphate↗
[Dyslipidemia and hyperinsulinemia in normoglycemic-obese relatives of patients with non-insulin dependent diabetes mellitus].
To determine the most frequent dyslipidemias among first-degree relatives of NIDDM patients, and its association with their glucose-tolerance status and hyperinsulinemia, we have started to examine members of NIDDM pedigrees, according to American Diabetes Association guidelines for nuclear family studies. In a large family with 2 NIDDM siblings in the 2nd generation, and 4 siblings with NIDDM in the 3rd generation, we have evaluated 14 first degree relatives and also 15 sex and aged matched healthy control subjects without family history of diabetes. The NIDDM relative group presented BMI = 31.8 +/- 3.9 kg/m2, SBP = 128 +/- 18.2 mmHg, DBP = 84 +/- 12.7 mmHg. Both relatives and controls were subjected to a 2h 75g OGTT for glucose and insulin determinations. Although none of NIDDM relatives has IGT, both Glycemic Area (GA) and Insulin Area (IA) were greater (p < 0.01) in the NIDDM relative group. The Insulin/Glucose ratio was also higher (p < 0.01) at 0 and 120 min of OGTT, this might be indirect evidence of Insulin- Resistance. Fasting serum lipids in the NIDDM relatives were TG = 148 +/- 24mg/dl, T-Chol = 244 +/- 10.7mg/dl, HDL-C = 34.2 +/- 2.5mg/dl; lipids in the control group were TG = 84.8 +/- 10.1mg/dl, T-Chol = 167 +/- 10.2mg/dl, HDL-C = 44.4 +/- 2.6mg/dl. Electrophoretic pattern showed type IIa (30.7%) and IIb (61.5%) hyperlipidemias in the NIDDM relatives. In this group, there was a positive and significant association between basal insulin and DBP (r = 0.67; p < 0.01), and between DBP and both TG (r = 0.74; p < 0.01)) and VLDL-C (r = 0.58; p < 0.05). It was also obtained a negative association between basal insulin and HDL-C (r = -0.89; p < 0.001). These data suggest that hyperinsulinemia in association with lipid abnormalities could appear early (before the development of Impaired Glucose Tolerance and Diabetes) in first degree relatives of NIDDM patients.
Adult↗
[Absent or reverse diastolic umbilical flow].
We show our experience in the diagnostic and management of 19 pregnancies with absent or reverse diastolic blood flow velocity (ARFV) in the umbilical artery. The presence of ARFV was a rare condition (2.2% of the high risk patients), and it was associated with a poor prognosis shown by the high percentage of fetuses SGD (small for gestational age) (63.2%), malformations and hydrops fetalis (26%) and asphyxiated fetuses, giving a final perinatal mortality rate of 36.8%. In 8 cases (42.1%) the termination or pregnancy was delayed for at least 48 hours, allowing in some cases the administration of corticosteroid. There were significant differences when comparing the groups of survivors and non survivors in relation to the gestational age at the moment of delivery (33.1 +/- 3.4 vs 28.6 +/- 3.8 weeks), malformations (8.3 vs 57.1%) and C-section (91.7 vs 42.9%). Finally, we conclude that the presence of ARFV in the umbilical artery is associated with a critical fetal condition and termination of pregnancy should be considered. In this decision the gestational age and fetal and maternal well being ought to be taken into account when choosing the best moment and route of delivery.
Adult↗
Insulin and lipogenesis in rat adipocytes. II. Effect of high levels of insulin and glucose on lipid synthesis in isolated rat adipocytes.
Insulin resistant states are characterized by receptor and post-receptor defects in insulin action. When the insulin resistant state progresses, elevated levels of insulin are accompanied by increasing levels of glucose. In a previous paper we demonstrated that treatment of isolated adipocytes with high levels of insulin led to a decrease in insulin binding as well as a decrease in basal and insulin-stimulated lipid synthesis. The results of the present study establish that the addition of high concentrations of glucose in combination with a high level of insulin, does not modify the decrease in binding of insulin to its receptor. However, the decrease in lipid synthesis previously observed in the presence of high concentrations of insulin was completely overcome by the presence of high glucose.
Adipocytes↗
Anterior commissure microwebs associated with vocal nodules: detection, prevalence, and significance.
Vocal fold nodules are a common cause of dysphonia generally attributed to vocal abuse. Anterior commissure microwebs have been reported as an incidental finding in surgical patients with nodules. In a series of 105 nodule patients evaluated at the University of Wisconsin Clinical Science Center voice laboratory (1987-1992), 11 microwebs were identified. Ten of these microweb patients were among the 20 nodule patients who did not respond to voice therapy and underwent microsurgery. In patients with nodules whose hoarseness is refractory to voice therapy, symptoms that occur early in life suggest the presence of occult vocal fold pathology. Microweb detection requires a high index of suspicion, observation during maximal vocal fold abduction, and clearance of secretions from the anterior commissure. Definitive identification is facilitated by gentle separation of the anterior vocal folds during direct microlaryngoscopy. The presence of these tiny shelves of tissue might be coincidental, or they might represent another expression of the tissue response to traumatic factors known to produce vocal nodules. We found little difference in vocal function parameters between two similar groups of nodule patients, one with and one without associated microwebs. Further work is needed to determine the significance of microwebs.
Adolescent↗