Evidence for multidimensional resilience in adult patients with transfusion-dependent thalassemias: Is it more common than we think?

BACKGROUND: Life expectancy of patients with transfusion-dependent thalassemias has increased with the development of improved treatment over the last few decades. However, ß-thalassemia disorder still has considerable lifetime treatment demands and heightened risk of frequent complications due to transfusion-transmitted infections and iron overload, which may affect thalassemic patients' functioning in different domains. OBJECTIVES: The vast majority of published studies on thalassemic patients have focused on children and adolescent functioning, and little research has examined adults. Hence, the current study was planned to examine the functioning and resilience of adult thalassemic patients in a comprehensive way. METHODS: We examined multidimensional resilience and functioning across different domains (psychological adjustment, treatment adherence, social functioning and occupational functioning). We also examined demographic and medical variables that may relate to resilience and functioning. Participants were adult patients [n = 38; age M = 31·63, standard deviation (SD) = 7·72; 72% female] with transfusion-dependent thalassemia in treatment in a hospital in the northeastern United States. RESULTS: The results suggest that most adult thalassemic patients tend to be resilient, demonstrating good functioning in four main domains: psychological adjustment, treatment adherence, social functioning and occupational functioning. CONCLUSION: Despite the considerable demands of their illness, adult thalassemic patients appeared to be adapting well, demonstrating evidence of multidimensional resilience.

Quality of life in thalassemia: a comparison of SF-36 results from the thalassemia longitudinal cohort to reported literature and the US norms.

Thalassemia is a chronic, inherited blood disorder, which, in its most severe form, causes life-threatening anemia. Advances in treatment have led to increased life expectancy however the need for chronic blood transfusions and chelation therapy remains a significant burden for patients. Our study compared health related quality of life (HRQOL) from the Thalassemia Clinical Research Network's (TCRNs) Thalassemia Longitudinal Cohort (TLC) study to US norms and assessed association with clinical variables. There were 264 patients over age 14 who completed the Medical Outcomes Study 36-Item Short Form Health Survey version 2 (SF36v2) baseline assessment. When compared to US norms, TLC patients had statistically significant (P < 0.05) worse HRQOL on five of the eight subscales (physical functioning, role-physical, general health, social functioning, and role-emotional) and on both summary scales (physical component summary and mental component summary). Women, older patients, and those with more disease complications and side effects from chelation reported lower HRQOL. In general, adolescents and adults with thalassemia report worse HRQOL than the US population, despite contemporary therapy. The SF-36 should become a standard instrument for assessing HRQOL in thalassemia to determine predictors of low HRQOL which may be better addressed by a multidisciplinary team.

The significance of a positive DAT in thalassemia patients.

The DAT is performed for the detection of antibody or complement on the surface of RBCs. Our institution previously performed DATs on all chronically transfused thalassemia patients before each transfusion episode to detect early alloimmunization. The medical records of all thalassemia patients treated at our institution from 2004 to 2007 were reviewed to determine the significance of the high rate of positive DATs (52.5% of 80 patients). The majority of IgG-reactive DATs were associated with a nonreactive eluate (65.4% of 286 eluates performed). A positive DAT was significantly associated with splenectomy (χ² = 15.4; p < 0.001), elevated IgG levels (χ² = 26.8; p < 0.001), HCV (χ² = 20.7; p < 0.001), and warm autoantibody (χ² = 5.87; p = 0.03). Multivariate analysis revealed that only HCV (OR, 5.0; p = 0.037) and elevated IgG levels (OR, 9.0; p = 0.001) were independently associated with a positive DAT. Alloimmunized thalassemic patients were more likely to have a positive DAT than nonalloimmunized patients, but this association was not significant (OR, 2.2; p = 0.11). A positive DAT did not correlate with decreased response to transfusion, RBC survival, hemolysis, or increased transfusion requirements. Only two cases of early alloimmunization were detected by DAT among 288 DAT-positive samples studied during 4 years. This study demonstrated that the routine performance of DATs on pretransfusion specimens in thalassemic patients has limited clinical utility, and the elimination of this test will improve turnaround time and decrease costs.

[A further case of analbuminemia]. / Ein weiterer Fall von Analbuminämie.

At routine follow-up a 23-year-old female presented a high erythrocyte sedimentation rate and handicapping lipodystrophy of the lower limbs. Protein electrophoresis showed absence of an albumin peak and the diagnosis of analbuminaemia was therefore proposed. Investigation of family members disclosed that one brother out of a total of four siblings also had analbuminaemia. The hereditary pathway often appears to be autosomalrecessive. Subsequent review of the literature revealed only 28 other observed cases, although today electrophoresis has become a worldwide routine examination. Surprisingly, the almost complete absence of so important a protein as albumin does not trigger disease. The body is able to compensate for the lack of albumins with other proteins. The absence of abumin is typically associated with hyperlipidaemia and lipodystrophy in the female sex.

Tacrolimus in acute renal failure: does L-arginine-infusion prevent changes in renal hemodynamics?

Nephrotoxicity is one of the main side effects of calcineurin-inhibitors. The influence of tacrolimus on the renal vasculature has not been well described. We have therefore examined the effects of tacrolimus on renal functional parameters as well as the contribution of the NO-system in a model of ischemic acute renal failure (ARF). Induction of ARF was achieved by clamping both renal arteries of female Sprague-Dawley rats. During the experiment, RBF, GFR, MAP, RVR and FENa were determined during infusion of vehicle, TAC, TAC and the NOS-activator L-arginine, and TAC and NOS-inhibition due to L-NMMA. TAC induced a significant rise in RVR with further decrease of RBF and GFR. Simultaneous L-arginine-infusion could reverse these effects during the infusion without complete restoration to preischemic levels. NOS-inhibition increased MAP and RBF without any effect on GFR. FENa did not differ significantly between the groups. Tacrolimus in the situation of ischemic acute renal failure causes vasoconstriction of pre- and postglomerular vessels with a further deterioration of renal function. L-arginine abolishes the functional deterioration, most likely due to increased NO-liberation.

Use and morbidity of venous access devices in patients with hemophilia.

To obtain information on venous access devices (VADs) used for the treatment of patients with hemophilia and infections associated with the use of VADs, a questionnaire was sent to 153 hemophilia treatment centers in the United States and Puerto Rico. Information regarding the treatment of 10,421 patients and 568 VADs was obtained. Infections were reported with 159 (45%) of 353 internal devices and 126 (71%) of 178 external devices. These results indicate that VADs used for the treatment of hemophilia are associated with significantly higher infection rates than are those used in oncology or mixed patient populations.

Parvovirus B19 quiescence during the course of human immunodeficiency virus infection in persons with hemophilia.

To detect and characterize parvovirus B19 infection during the course of progressive immune deficiency from human immunodeficiency virus (HIV), ten subjects enrolled in the Multicenter Hemophilia Cohort Study were followed for 6.4 to 15 years from HIV seroconversion through extreme immune deficiency. Four to five sera or plasma samples from each subject, collected at predetermined CD4+ lymphocyte levels, were tested for immunoglobulin G (IgG) and M (IgM) B19 antibodies and DNA. All 42 samples were positive for B19 IgG antibodies, and three were weakly positive for IgM antibodies. Only one sample, collected coincident with HIV seroconversion, was unequivocally positive for B19 DNA. No persistent hematologic adverse effects of B19 infection were observed. Thus, although B19 IgG antibodies are highly prevalent among HIV-infected persons with hemophilia or related disorders, B19 viremia and its hematologic consequences were not detected, even with severe depletion of CD4+ lymphocytes. If primary B19 infection occurs after immune deficiency, however, the consequences may be more adverse.

Von Willebrand disease: a nursing perspective.

Von Willebrand disease, caused by a deficiency or abnormality of von Willebrand factor, is the most common hereditary bleeding disorder, occurring in approximately 1% of the population. This article is intended to raise the level of awareness in the health care community and define the nurse's role in recognizing the clinical presentation of this underdiagnosed bleeding disorder.

Comparison of activities of fluoroquinolones in murine macrophages infected with Mycobacterium tuberculosis.

In this study the compounds levofloxacin and sparfloxacin, as well as three experimental compounds (AMQ2, AMQ4, and AMQ5), were compared with isoniazid and rifabutin in terms of their capacity to inhibit the intracellular growth of the drug-susceptible Mycobacterium tuberculosis strain Erdman and the isoniazid-resistant katG gene-negative strain 24 within monolayers of mouse bone marrow-derived macrophages. Both levofloxacin and sparfloxacin, as well as compound AMQ4, had substantial activity in this physiologically relevant model, further confirming the potential usefulness of this class of compounds in the therapy of tuberculosis.

Hemophiliac patients in surgery. Implications for perioperative nurses.

The introduction of plasma clotting factor concentrates has changed the treatment of patients with clotting factor deficiencies dramatically. They have enabled hemophiliacs to be independent and have some control over the management of their disease as informed participants. This has played a role in shortening the number of hospitalizations that hemophiliacs may have to endure and to decrease the length of stay when they are admitted. Additionally, staff who care for these patients do not need to be afraid of the disease if they are aware of the disease process and the therapy available for its amelioration.