Hemoglobin Providence Asn and Hemoglobin Providence Asp are two abnormal hemoglobins which apparently arise from a single genetic change that substitutes asparagine for lysine at position 82 (EF6) in the j3 chain of human hemoglobin. The second form appears to be the result of a partial in uiuo deamidation of the asparagine situated at position /382. Cellulose acetate and citrate agar electrophoresis of hemolysates from patients with this abnormality shows three bands. Globin chain electrophoresis at acid and alkaline pH shows three /3 chains. These three chains correspond to the normal PA chain and two abnormal fi chains. Sequence analysis indicates that the two abnormal chains differ from PA at only position /382. In the two abnormal chains, the residue which is normally lysine is substituted either by asparagine or by aspartic acid. These substitutions are notable because j382 lysine is one of the residues involved in 2,3-diphosphoglycerate binding. Additionally, p82 lysine is typically invariant in hemoglobin /3 chain sequences. Sequence data on the two forms of Hemoglobin Providence are given in this paper. The functional properties of these two forms are described in the next paper.
This article describes the development of an instrument that measures symptom experience (symptom occurrence and symptom distress). The Adapted Symptom Distress Scale-2 (ASDS-2), adapted from the McCorkle and Young Distress Scale, is a 31-item, 5-point, self-report paper-and-pencil instrument that measures patients' perception of the occurrence and distress of 14 symptoms: nausea, vomiting, pain, eating, sleep, fatigue, bowel elimination, breathing, coughing, concentration, lacrimation, changes in body temperature, appearance, and restlessness. Use of the instrument yields a total score for symptom experience, scores for symptom occurrence, scores for symptom distress, and subscale scores for six symptom categories: gastrointestinal, fatigue/restlessness, concentration, pain/discomfort, respiratory, and appearance. Reliability and validity were determined with well adults (n = 97), medical-surgical patients (n = 82), and oncology patients (n = 175). Findings revealed a Cronbach's alpha of 0.91 for symptom experience, 0.90 for symptom occurrence, and 0.76 for symptom distress. Cronbach's alpha for the subscales ranged from 0.38 for appearance symptoms to 0.83 for gastrointestinal symptoms. Inclusion of symptoms reported by patients with cancer strengthened content validity. A contrasted groups approach was used to demonstrate construct validity.
Verna A. Rhodes, Roxanne W. McDaniel, and Mary H. Johnson T EACHI, NG is an integral part of nursing, yet today s rapidly changing health care arena with shortened hospital stays presents unique challenges for the nurse to provide appropriate patient requirements for self-care. Much care for this chronic multistage disease takes place in the home, which demands knowledge not only of what to do to prevent and/or manage the symptom experience but also what to report to the health care provider. These patient education self-care guides have been developed to assist nurses in designing and implementing individualized teaching. A variety of educational strategies based on individualized assessment for efficient effective teaching of self-care are essential because of the overwhelming emotional and physical obstacles surrounding the patient and family.t Appropriate patient education that provides proactive management of the symptom experience may alleviate fears, enhance quality of life, and be cost effective.
OBJECTIVES:To examine the physiology of nausea, vomiting, and retching (NVR); the impact of NVR on the patient: current measures to control NVR; and selfcare interventions. DATA SOURCES:Research studies, abstracts, and review articles relating to NVR associated with cancer treatment as well as pharmacological and nonpharmacological interventions. CONCLUSIONS:Management of the individual symptoms of NVR require expert, ongoing assessment of the patient's symptom experience that extends beyond the clinic or hospital visit. Although a number of pharmacological antiemetic agents are currently available and additional antiemetic drugs are in phase II or II trials, nonpharmacological interventions are essential to achieve effective management. IMPLICATIONS FOR NURSING PRACTICE:Continual assessment of the individual's symptom experience is imperative. Effective management of the symptom experience depends on the oncology nurses's ability to implement current knowledge of antiemetic, and other drugs; non-pharmacological interventions; and cost-effective and clinically useful patient outcomes.
(1991). HB Luxembourg [α24(B5)TYR→HIS], HB Maputo [β47(CD6)ASP→TYR], and HB Fukuyama [β77(EF1)HIS→TYR] Hemoglobin: Vol. 15, No. 1-2, pp. 97-101.
Hemoglobin Attleboro, a new alpha-chain variant with a substitution of proline for serine at position 138 (H21), was found to be a noncooperative high-affinity hemoglobin (P50 = 0.26 mmHg at pH 7 and 20 degrees C) which lacked an alkaline Bohr effect. Addition of 2,3-diphosphoglycerate (DPG) or inositol hexaphosphate (IHP) led to a decrease in oxygen affinity but to no alteration in either Bohr effect or cooperativity. Ligand binding kinetics studies revealed an overall rate of oxygen dissociation at pH 7.0 and 20 degrees C that was 2.7-fold slower than that for Hb A. At pH 8.5, the kinetic profile was identical with that at pH 7, confirming the absence of a Bohr effect for this variant hemoglobin. Measurement of the rate of oxygen dissociation with carbon monoxide replacement indicated a lack of cooperativity. Sedimentation velocity experiments yielded s20,w values of 2.8 and 4.3 for 65 microM solutions of oxyhemoglobins Attleboro and A, respectively (indicating an enhancement in the oxy dimer population of this variant). Studies of the carbon monoxide combination of this variant revealed an association rate 20-fold faster than that for Hb A; only in the presence of a 1000-fold molar excess of IHP was there a significant reduction in the overall rate. Rapid-scan and traditional stopped-flow experiments conducted in the Soret Soret region demonstrated an alteration in the structure and rate of assembly of the deoxy tetramer of Hb Attleboro relative to that of Hb A. The abnormal properties of this hemoglobin variant can be attributed to major perturbations in the C-terminal region.
Hb Catonsville is an unstable variant in which glutamic acid is inserted into the alpha-globin chain between Pro-37(C2) and Thr-38(C3). The peptide sequence data are consistent with the DNA sequence of the polymerase chain reaction-amplified fragment of the variant globin gene, which shows the insertion of the triplet codon--GAA--into the mutant alpha-globin gene. In the normal alpha-globin gene cluster the codon for glutamic acid is GAG rather than GAA. Thus, there are two features unique to Hb Catonsville, one the insertion of a single residue into the interior of the alpha-globin chain, and two the presence of the alternate codon for glutamic acid. The experimental evidence suggests that Hb Catonsville may be an example of nonhomologous nonallelic gene conversion, an observation not previously reported in this gene family. The mutation occurs in the critical alpha 1 beta 2 interface of the hemoglobin tetramer and leads to a variant with high oxygen affinity, a reduced cooperativity, and Bohr effect.
Hb Hinsdale was detected in two sisters and in a son and daughter of one of them as a band migrating in the Hb F position on cellulose acetate, pH 8.5. On citrate agar (pH 6.3) the variant hemoglobin has a mobility like that of Hb S. Hematologic data from these individuals appear normal except for mild anemia. Oxygen affinity studies show that the variant has low affinity for oxygen and reduced cooperativity. Results of tests for instability were negative. The mutation involves a site that lies in the central cavity close to the 2,3-diphosphoglycerate pocket, so it is not surprising that the variant shows a reduced ability to react with this effector molecule.
Hemoglobin Brockton [beta 138 (H16) Ala----Pro] is an unstable variant associated with a mild anemia. It has the same electrophoretic mobility as and cannot be resolved from Hb A. Oxygen affinity measurements of blood and hemolysate do not indicate biphasic oxygen saturation, showing that the functional properties of the variant are very similar to those of Hb A. This implies that the introduction of proline into the H-helix at position 138 does not disrupt the critical inter- and intrasubunit hydrogen bonds and salt bridges at the beta carboxyl-terminal dipeptide, since these polar interactions are essential for the normal oxygen-binding properties of hemoglobin. X-ray crystallographic data are consistent with these findings and show that the consequences of the beta 138 Ala----Pro substitution are almost entirely confined to the immediate vicinity of the mutation site. Instability probably results from the inability of a buried hydrogen bond to form between Pro 138 beta and Val 134 beta.
p bATTERNS of nausea and vomiting occurrence and distress are not well documented for single antineoplastic chemotherapeutic agents and drug combinations. The purpose of this study, using self-regulation theory, was to describe patterns of nausea and vomiting that occurred during six consecutive cycles of initial selected chemotherapy regimens. A stratified sample of patients (N = 309), 21 to 85 years old, was selected from multiple geographic sites within two Midwestern states. The Rhodes Index of Nausea and Vomiting (INV) Form 2 was used to measure nausea and vomiting every 12 hours for 48 hours. Nonparametric analysis of variance and cluster analysis methods were used to determine the patterns of postchemotherapy nausea and vomiting. Findings revealed that 84% of the sample had their vomiting well controlled during the 48 hours posttherapy, while 71% had little or no nausea (i.e., minimal pattern). In the remaining sample, three distinct antiemetic drug-resistant patterns emerged for each of the dyad symptoms. The drug-resistant patterns of symptom experience, symptom occurrence, and symptom distress were (a) peak, (b) latent, and (c) sustained patterns (Rhodes & Watson, 1987a; Rhodes, Watson, Johnson, Madsen, & Beck, 1987). Additional findings were as follows: 1. Statistically significant relationships between postchemotherapy symptom experience and the antineoplastic drug protocols. The three most emetic chemotherapy drug protocols were cyclo-
Hemoglobin Indianapolis was first described by Adams et al (1,2) as a very unstable variant with a phenotype similar to severe beta-thalassemia. We have also characterized this variant, but there are several differences in the clinical expression of the variant described in our report and those described in the original case. We found Hb Indianapolis to be unstable, but not to the extent that it could not be detected by routine testing. The four family members heterozygous for the variant were not anemic, showed normal hematologic values, and did not exhibit any severe clinical disadvantages, although there was slight reticulocytosis. The variant could not be resolved from Hb A on cellulose acetate (pH 8.4), but isoelectric focusing showed a double band in the region of Hb A that is probably the variant and Hb A. However, the variant chain was clearly evident by globin chain analyses in acid and alkaline buffers. The condition of additional blood samples did not allow us to determine the oxygen dissociation properties of the variant or the rates of globin chain synthesis.