Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Using a cation-exchange chromatographic method, we found normal or subnormal values for glycosylated hemoglobin in a few diabetic patients with persistent hyperglycemia. Subsequent investigations revealed that these unexpected results had originated from black patients with diabetes. In view of common occurrence of abnormal hemoglobins in the Negro population, we subjected blood preparations to electrophoresis on cellulose acetate and acrylamide gel. The results have shown the presence of hemoglobin S or hemoglobin C in each patient. When allowance was made for the percentage of the abnormal hemoglobin, the "corrected values" of glycosylated hemoglobin increased to the diabetic range. Furthermore, the corrected values agreed well with the "expected values" calculated from a regression line correlating fasting blood glucose concentrations and proportions of glycosylated hemoglobin in more than 300 diabetics with no evidence of hemoglobinopathy. We conclude that in diabetic patients presenting with hemoglobin S or hemoglobin C, there is a considerable decrease in the values for glycosylated hemoglobin as measured by cation-exchange chromatographic methods, and that this decrease is proportional to the percentage of the abnormal hemoglobin.
 ...
PMID:Low proportions of glycosylated hemoglobin associated with hemoglobin S and hemoglobin C. 45 90

This prospective study demonstrated that among 92 consecutive patients who underwent cholecystectomy for gallstones at an urban university hospital, 27% had pigment stones and 73% had cholesterol stones. Age, sex, and weight, but not race, were significant determinants of stone type. The mean hemoglobin, direct and total serum bilirubin, and fasting glucose concentrations were similar for each group. The presence of alcoholism, diabetes, thyroid disease, or heterozygous hemoglobinopathy did not influence stone type. The average patient with pigment stones is a lean man or woman 63 years old; in contrast the composite patient with cholesterol stones is a modestly overweight woman 43 years old.
 ...
PMID:Pigment vs cholesterol cholelithiasis: clinical and epidemiological aspects. 115 13

GH and FA are useful monitors in the care of diabetic patients. For most situations, GH is the preferred test and should be routinely monitored. FA should be reserved for exceptional situations in which blood glucose control over one to two weeks must be assessed or in patients with a hemoglobinopathy. Patients with diabetes should be advised of their present GH level and the preferred goal.
 ...
PMID:The why and wherefore of fructosamine. 142 70

Venous thrombosis is a common complication of total parenteral nutrition. We report a case of priapism in a 40-year-old man after administration of total parenteral nutrition for chronic idiopathic intestinal pseudo-obstruction. The patient received glucose, amino acids, and 20% fat emulsion; 12 hours after administration, the patient complained of a persistent, painful penile erection lasting 5 hours. Bilateral corpora cavernosa spongiosum shunts achieved immediate and sustained detumescence, but the patient remained impotent. There was no history of penile or pelvic trauma, hemoglobinopathy, coagulopathy, venous thrombosis, or leukemia. The medical literature describes seven other cases of priapism related to total parenteral nutrition. All of the patients received 20% fat emulsion; two patients developed priapism during the weekly infusion of fat emulsion. Among the multiple factors that can favor thrombosis and therefore priapism during total parenteral nutrition, fat infusion appears to be the most important. Three different mechanisms have been postulated: increase in blood coagulability, effects on red blood cells, and fat embolism. In this patient, platelet function was estimated in vivo by the levels of antiheparin platelet factor 4 and beta-thromboglobulin. These two parameters were both elevated before 20% lipid emulsion and were even higher after the 20% fat-emulsion infusion. Therefore, even if a direct thromboplastic effect is possible, 20% fat emulsion increases platelet activity, which was already high in our patient, and thereby favors priapism.
 ...
PMID:Priapism in a patient treated with total parenteral nutrition. 155 16

Technical and maternal factors known to affect urinary estriol level or its measurement are discussed in view of the current use of serial estriol as an indicator of fetoplacental function. Technical artifacts can result from presence of methenamine mandelate, phenolphthalein, glucose, or high urine specific gravity. Aside from normal maternal factors such as inaccurate urine collection or variable fluid intake, estriol levels are depressed by corticosteroids and ampicillin. Pyelone phritis, anemia, hemoglobinopathy, abnormal renal status, malnutrition, and high altitude all depress estriol level or excretion. In some conditions such as pyelonephritis, amniotic estriol level may be a better indicator of fetal status.
 ...
PMID:Factors affecting the yeild of urinary estriol. 433 82

Glycosylated hemoglobin (HbA1) is considered to be representative of prior blood-glucose levels and is being used in pregnant and nonpregnant diabetic patients as a possible index of both long and short-term glucose-control. Factors other than blood-glucose concentration have been reported to affect its value. Variant hemoglobin is one of them. HbA1 and blood-glucose levels were measured in pregnant patients at high risk for diabetes for screening for abnormal carbohydrate metabolism. HbA1 was measured by cation exchange column chromatography and glucose was measured by hexokinase reaction. The mean HbA1 in patients with normal blood sugars was 6.17 +/- 0.6 percent. A value of HbA1 of less than 5 percent as measured by cation exchange column chromatography was highly predictive (P less than 0.001) of hemoglobinopathies (S or C). The mean HbA1 of randomly selected matched patients with "normal" Hb was 5.94 +/- 0.72 percent. In patients with thalassemia, HbA1 values as measured by cation exchange column chromatography were elevated despite normal carbohydrate tolerance. While interpreting the results of HbA1 in the management of pregnant diabetics, the above fact should be kept in mind.
 ...
PMID:Glycosylated hemoglobin (HbA1) and hemoglobinopathies in pregnancy. 650 39

Oxygen transport to and substrate turnover in leg muscle were studied at rest and during light and heavy upright bicycle exercise in two brothers with a hereditary hemoglobinopathy associated with high oxygen affinity (P50 = 13 mmHg). Femoral venous oxygen tension was below normal and femoral venous oxygen saturation above normal at rest and during exercise. Thus, the arterial-femoral venous oxygen saturation difference was decreased. Despite a compensatory increase in hemoglobin concentration, the arterial-femoral venous oxygen content difference tended to be below normal at heavy exercise. Approximately 25% of the oxygen was delivered via the abnormal hemoglobin at relative heavy exercise. Arterial lactate levels, lactate release, and muscle lactate concentration were not increased at any level of exercise. Glucose, alanine, pyruvate, and glycerol turnover were essentially normal, but the glycogen and creatine phosphate stores were abnormally depleted at the termination of heavy exercise. The exercise electrocardiogram (ECG) was normal, indicating that myocardial oxygenation was adequate. Muscle-surface oxygen pressure fields were normal at rest (not investigated during exercise). It is concluded that the high oxygen affinity of the hemoglobin in our two subjects did not lead to heart or skeletal muscle hypoxia during heavy exercise, as judged from the ECG and from the leg lactate turnover. Despite the lack of evidence for muscle hypoxia, the subjects experienced leg muscle fatigue and the creatine phosphate and glycogen stores were depleted more than normally.
 ...
PMID:Tissue oxygenation and muscular substrate turnover in two subjects with high hemoglobin oxygen affinity. 663 May 12

Glycosylated hemoglobin (GHb) was estimated in normal and diabetic human, rat, and dog hemolysate by m-aminophenylboronic acid (PBA) affinity chromatography and the results compared with the values determined using two ion-exchange (ION-E) methods and a colorimetric thiobarbituric acid (TBA) method. There was a good correlation between the values estimated by PBA and both ION-E chromatography methods for the human samples (r = 0.83, P less than 0.0002, r = 0.86, P less than 0.002). In diabetic rat and dog hemolysates, PBA chromatography demonstrated higher glycosylated hemoglobin than in normal hemolysate. In both species, there was an excellent correlation between the PBA-estimated GHb and plasma glucose levels (rat r = 0.79, P less than 0.001; dog r = 0.67, P less than 0.003). The ION-E and TBA methods were not as effective in separating diabetic from normal samples and correlated less well with plasma glucose levels. PBA chromatography relies on the interaction of m-amino phenylboronate with the hydroxyl groups of the glucose residues attached to hemoglobin. It is not affected by intra- or interspecies variations in the hemoglobin moiety and should be adaptable for measurement of GHb in a number of laboratory animals and in patients with hemoglobinopathy. It is not affected significantly by temperature and may offer advantages over the ION-E method in the routine determination of human glycosylated hemoglobin.
 ...
PMID:The measurement of glycosylated hemoglobin in man and animals by aminophenylboronic acid affinity chromatography. 716 May 42

We describe a case of hemoglobinopathy detected on admission for examination for high blood glucose levels and abnormal liver function. In 1991, it was pointed out that he had postprandial hyperglycemia. In 1994, at age 60, he had lassitude and anorexia. He was admitted to our hospital on the suspicion of diabetes mellitus and liver disease. Glycosylated hemoglobin levels was very high, but the 75 gram oral glucose tolerance test result was within the normal range. After abstinence from alcohol, his glutamic oxaloacetic transaminase, glutamic pyruvic transaminase and gamma glutamyl traspeptidase became normal. Diabetes was excluded and abnormal hemoglobinopathy had been suspected. We analyzed his abnormal hemoglobin. In isoelectro-phoresis a fast moving variant was detected suggesting the presence of abnormal hemoglobin at the cathode. We fractionated hemolytic globin by CM-chromatography and detected an abnormal peak before the alpha chain band. Subsequently, we sequenced isolated abnormal alpha chain and detected the substitution of Ariginine for Glutamamine at position 92 (Hb J Cape Town). So far he has not demonstrated any symptoms or signs of HbJ Cape Town. Hemoglobinopathy is not uncommon in aged people.
 ...
PMID:[A case of abnormal hemoglobin (HbJ Cape Town) with high serum levels of HbAlc]. 865 77

The Abbott IMx glycated hemoglobin assay was evaluated in a multicentre study. This method utilizes boronate affinity chromatography, and ion-capture technology. This assay determines both total glycohemoglobin (% GHb) and percentage of hemoglobin Alc (% HbAlc). The precision of the assay was evaluated: the intra-assay and interassay coefficients of variation were judged to be satisfactory (< 6.5%). We determined the accuracy of the assay by comparison with a reference HPLC assay for 603 specimens; coefficients of correlation were between 0.88 and 0.96. We studied the interference of bilirubin and glucose and found no interference at usual concentrations. The presence of abnormal hemoglobins (HbF and some Hb structural variants HbS, HbC) was not detected with the Abbott IMx assay; however, this assay showed no significant interference from the hemoglobin variants tested for heterozygous hemoglobinopathies (percentage of abnormal hemoglobin < 60%). We also determined normal values for HbAlc with this technology (164 specimens): 4.1 to 6.1%.
 ...
PMID:[Multicenter evaluation of thr Abbott glycosylated hemoglobin assay on IMx]. 888 61


1 2 3 4 Next >>