Book reviews

The School Years. Current Issues in the Socialization of Young People John C. Coleman (Ed.) London, Routledge, 1992 (2nd edn) 208 pp.; £10.99 (paperback)

AIDS and the Hospice Community Madalon O'Rawe Amenta & Clair B. Tehan Binghampton, NY, Harrington Park Press, 1991 186 pp. + index

Social Care & HIV/AIDS The Hull York Research Team London, HMSO, 1993 214 pp., £13.70 (paperback)

Private Risks and Public Dangers Sue Scott, Gareth Williams, Stephen Platt & Hilary Thomas (Eds) Aldershot, Avebury, 1993 205 pp., £34.95 (hb)

AIDS: a problem for sociological research Michael Pollak, Geneviève Paicheler & Janine Pierret London, Sage Publications, 1992 124 pp., £10.95 (paperback)

AIDS: The Winter War Arthur D. Kahn Philadelphia, Temple University Press, 1993 236 pp., U£27.95 (hardback)

AIDS: Facing the Second Decade Peter Aggleton, Peter Davies & Graham Hart (Eds) Taylor & Francis, 1993 215 pp., £12.95 (Pb)

Women and AIDS: Psychological Perspectives Corrine Squire (Ed.) London, Sage Publications, 1993 196 pp., £12.95 (paperback)     

Book Review

Acquainted with the Night Allegra Taylor, 1989 London, Fontana £3.99 (paperback)

The AIDS Handbook Carl Miller 1990 Penguin Books 159pp. £4.99 (paperback)

Caring for Someone with AIDS Edited by David Yelding, 1990 Published for the Research Institute for Consumers Affairs by the Consumers Association and Hodder & Stoughton £6.95 (paperback)

AIDS. A Pocket Book of Diagnosis and Management Edited by A. Mindel, 1990 London, Arnold 244 pp. £12.95

AIDS and its Metaphors Susan Sontag, 1989 Harmondsworth, Penguin 98 pp. £3.99 (paperback)     

HFE gene mutation and iron overload in Egyptian pediatric acute lymphoblastic leukemia survivors: a single-center study

Background: Hereditary hemochromatosis gene (HFE) mutations have a role in iron overload in pediatric acute lymphoblastic leukemia (ALL) survivors. We aimed to evaluate the genotype frequency and allelic distribution of the two HFE gene mutations (C282Y and H63D) in a sample of Egyptian pediatric ALL survivors and to detect the impact of these two mutations on their iron profile.

Patients and methods: This study was performed on 35 ALL survivors during their follow-up visits to the Hematology and Oncology Unit, Pediatric Department, Menoufia University Hospitals. Thirty-five healthy children of matched age and sex were chosen as controls. After completing treatment course, ALL survivors were screened for the prevalence of these two mutations by polymerase chain reaction-restriction fragment length polymorphism. Serum ferritin levels were measured by an enzyme-linked immunosorbent assay technique (ELISA).

Results: C282Y mutation cannot be detected in any of the 35 survivors or the 35 controls. The H63D heterozygous state (CG) was detected in 28.6% of the survivors group and in 20% of controls, while the H63D homozygous (GG) state was detected in 17.1% of survivors. No compound heterozygosity (C282Y/H63D) was detected at both groups with high G allele frequency (31.4%) in survivors more than controls (10%). There were significant higher levels of iron parameters in homozygote survivors than heterozygotes and the controls.

Conclusion: H63D mutation aggravates the iron overload status in pediatric ALL survivors.     

Selective apoptosis of multiple myeloma cells in primary samples induced by arsenic trioxide

Objectives

Currently, multiple myeloma (MM) is an incurable disease. Despite the fact that arsenic trioxide (ATO) shows promising results in vitro, data from treatment of patients with MM are disappointing. Due to these discrepancies, we compared the efficacy and selectivity of ATO at two different concentrations in samples from MM patients.

Methods

The extent of apoptosis induced by 2 and 5 µM ATO was evaluated by flow cytometry using annexin V. 34 diagnostic bone marrow samples obtained from MM patients were analysed.

Results

5 µM ATO efficiently induced apoptosis in primary samples. Besides efficacy, also selectivity of action on MM cells in comparison to remaining haematopoietic cells was demonstrated for 5 µM ATO but not for 2 µM ATO.

Discussion

Our study on primary samples confirmed that ATO has a potential role in therapeutic management of MM. Further controlled studies on MM patients are needed.     

Compliance and satisfaction with deferasirox (Exjade®) compared with deferoxamine in patients with transfusion-dependent beta-thalassemia

Objective

The current standard option for iron chelation therapy (ICT) in transfusion-dependent patients with beta-thalassemia is deferoxamine (DFO). We aimed to compare the compliance with DFO vs. deferasirox (Exjade^®), a novel oral iron chelator, in patients with transfusion-dependent beta-thalassemia.

Methods

In this cross-sectional study, 220 patients from Southern Iran who were receiving DFO or Exjade^® for the last 2 years were investigated in 2012. Satisfaction, compliance, convenience, and life disturbances of the patients with ICT were evaluated. Assessments were performed at four different occasions during 1 year.

Results

According to the results, 114 patients received treatment with DFO and 106 patients were treated with Exjade^®. In comparison with the patients who were treated with DFO, those receiving Exjade^® reported a significantly higher rate of compliance and convenience (P < 0.05). However, no significant difference was observed between the two groups regarding their satisfaction (P > 0.05). In the DFO group, 44.9% of the patients reported irritation and pain at the injection site.

Conclusions

Considering higher rate of compliance and convenience with Exjade^® and the comparable efficacy of these two modalities of ICT documented in previous studies, Exjade^® can be used as a preferable choice of ICT in iron-overload patients with beta-thalassemia.     

Acute physiological responses to physiotherapy applications pre and post autologous stem cell transplantation: an experimental study

Objective

We aimed to investigate the acute physiological responses (APR) to physiotherapy applications in patients undergoing autologous stem cell transplantation (ASCT), the difference between pre- and post-ASCT according to APR.

Methods

Twenty-six patients who were hospitalized for ASCT attended regular physiotherapy program. APR was recorded in the beginning and at the end of each exercise session. The differences in APR were calculated for each session. The mean values of the differences in APR were computed in pre-conditioning, pre-, and post-ASCT. Daily complete blood counts were also recorded during ASCT.

Results

Hemoglobin and platelet counts were significantly lower pre- and post-ASCT. Neutrophil counts were significantly lower post-ASCT. The difference in systolic blood pressure (SBP) in the beginning and at the end of the exercise sessions was significantly higher post-ASCT in comparison to pre-ASCT.

Conclusion

There was no significant change in APR except the SBP which suggests that similar level of exercise intensity could be tolerated in pre- and post-ASCT periods as well as preconditioning.     

Revisiting beta thalassemia intermedia: past,present, and future prospects

Background: The spectrum of thalassemias is wide ranging from thalassemia minor, which consists of mild hypochromic microcytic anemia without obvious clinical manifestations, to thalassemia major (TM), which is characterized by severe anemia since the first years of life and is transfusion dependent. Thalassemia intermedia (TI) describes those patients with mild or moderate anemia.

Objective: To describe the genetic features and major clinical complications of TI, and the therapeutic approaches available in the management of this disease.

Methods: Publications from potentially relevant journals were searched on Medline.

Results and discussion: Over the past decade, the understanding of TI has increased with regard to pathophysiology and molecular studies. It is now clear that clinical presentation and specific complications make TI different from TM. It is associated with greater morbidity, a wider spectrum of organ dysfunction and more complications than previously thought.

Conclusion: TI is not a mild disease. The interplay of three hallmark pathophysiologic factors (ineffective erythropoiesis, chronic anemia, and iron overload) leads to the clinical presentations seen in TI. New treatment modalities are currently being investigated to broaden the options available for TI management.     

The Role of Primary Care Providers in Encouraging Older Patients to Change Their Lifestyle Behaviors

Objectives: This study sought to identify older patients’ perceptions of primary care providers’ influence on their likelihood of improving diet and physical activity.

Methods: 104 adults ages 65 and older were interviewed immediately following a routine primary care visit about their plans and motivations for behavior change and how their clinic visit would influence their likelihood of making lifestyle changes. All interviews were recorded, transcribed and analyzed using a constant comparison approach.

Results: Participants reported that their providers influence their health behaviors by developing strong relationships, addressing concerns and encouraging change, and providing concrete instruction. When providers did not discuss diet or physical activity, or mentioned these topics only briefly, participants often perceived the message that they should continue their current behaviors.

Conclusions: Whether and how diet and physical activity are discussed in primary care influences the likelihood that older adults will make changes in these behaviors.

Clinical Implications: These findings highlight the need for a patient-centered counseling approach and caution providers to think twice before omitting discussion of the need for lifestyle change.     

Elevated levels of T-cell immune response cDNA 7 in patients with immune thrombocytopenia

Objectives

This study aimed to investigate the expression levels of T-cell immune response cDNA 7 (TIRC7) in immune thrombocytopenia (ITP) patients before and after high-dose dexamethasone (HD-DXM) treatment.

Methods

Forty-four patients with ITP were enrolled and received dexamethasone (40 mg/day) for 4 consecutive days. Patients who had platelet counts more than 50 × 10⁹/l or less were defined as responders or non-responders, respectively. Quantitative polymerase chain reaction and enzyme-linked immunosorbent assay were used to measure RNA level and plasma level of TIRC7, respectively.

Results

TIRC7 levels (RNA and plasma level) were significantly higher in ITP than that in control (P < 0.0001). However, after treatment, TIRC7 levels were significantly decreased in responders (P < 0.0001) but not in non-responders (P > 0.05).

Discussions

TIRC7 might be associated with the pathogenesis of ITP, and TIRC7 levels could be used as an indicator to evaluate patients’ response to HD-DXM treatment.     

Human platelet antigen 1, 2 and 5 gene polymorphisms in Egyptians and their potential association with susceptibility to immune thrombocytopenic purpura in Egyptian patients

Objectives: This study determined the incidence of HPA1, HPA2 and HPA5 polymorphisms in 120 Egyptian immune thrombocytopenic purpura (ITP) patients and 120 healthy Egyptian subjects.

Methods: Human platelet antigen (HPA) genotyping was done using the polymerase chain reaction-restriction fragment length polymorphism.

Results: The frequency of HPA1 allele a and b was 78.75 and 21.25% in controls, 80.8 and 19.2% in ITP, respectively. HPA2 allele a and b frequency was 86.25 and 13.75% in controls and of 74.6 and 25.4% in patients, respectively. HPA5 allele a and b frequency was 87.5 and 12.5% in controls, in patients it was 85 and 15%, respectively. With the exception of HPA2, no other significant difference was encountered in HPA allele frequency between controls and ITP patients.

Discussion: Egyptian HPA profile is closely linked to Middle East and neighboring Arabs. The current study noted that in all the studied HPA systems 1, 2 and 5, the ‘a’ allele is more prevalent than the b allele; the most frequent genotype was the homozygous a/a genotype. HPA2b frequency, homo- and hetero-zygous HPA2b genotype frequencies were significantly higher in ITP patients compared to controls.

Conclusion: HPA 2b are 2.37 times more likely to develop ITP compared to those without this allele. The relatively high allele frequency of the HPA-1b in the Egyptian population suggests that this ethnic group has a higher risk of alloimmunization.     

Is there a difference in phenotype between males and females with non-transfusion-dependent thalassemia? A cross-sectional evaluation

Objectives: Non-transfusion-dependent thalassemia includes a variety of phenotypes and genotypes that rarely require regular transfusions. However, these patients can experience a wide range of complications. The objective of this retrospective study was to verify whether there is a significant difference in non-transfusion-dependent thalassemia-related complications and treatment among males and females.

Methods: We performed a re-analysis of samples evaluated in a previously published cross-sectional study, regarding 96 non-transfusion-dependent thalassemia patients followed at the ‘UOSD Malattie Rare del Globulo Rosso’ Centre of the Cardarelli Hospital in Naples, Italy.

Results: We found that females were more anemic than males, but there was no significant difference in prevalence of common complications among genders, except for hypogonadism. Furthermore, the transitory regular transfusions regimen in women who had been pregnant does not seem to have a significant impact on overall prognosis.

Discussion: In non-transfusion-dependent thalassemia patients, the lower levels of hemoglobin found in females do not seem to indicate a higher prevalence of complications.

Conclusion: This data should be considered in studies with experimental treatments aiming to correct anemia in patients with non-transfusion-dependent thalassemia. It should probably also be taken into account in order to set up different transfusion regimens among genders in transfusion-dependent patients.     

Goal-setting to Promote a Healthier Lifestyle in Later Life: Qualitative Evaluation of the AgeWell Trial

Objective: We report a mixed method evaluation of the feasibility and implementation of the AgeWell goal-setting intervention to promote healthy ageing later life.

Method: Researcher field notes, goal-setting interview content, and semi-structured interviews with participants were content analysed to review trial implementation and participants’ perspective on the goal-setting and mentoring intervention.

Results: 75 people were recruited: 21 in the goal-setting and 22 in the goal-setting with mentoring arms of the intervention. Goal-setting was feasible in the main domains of interest. Adherence to the protocol was good and the mentoring schedule was adhered to. Participants reported satisfaction with their goal attainment, but barriers for non-achievement were also identified. Recommendations for small changes to the intervention included reducing the number of goals.

Conclusions: Participants understood the goal-setting process, and were able to set realistic and achievable lifestyle goals. The intervention and the procedures were acceptable but changes in how goal-setting is both introduced and monitored are needed for wider implementation.

Clinical Implications: Goal-setting can be a useful process to help people alter their lifestyle to allow them to age more successfully and reduce risk factors associated with dementia.     

Loss of lean body mass affects low bone mineral density in patients with rheumatoid arthritis – results from the TOMORROW study

Objectives: Osteoporosis is one of the complications for patients with rheumatoid arthritis (RA). Rheumatoid cachexia, the loss of lean body mass, is another. However, the relationship between decreased lean body mass and reduced bone mineral density (BMD) in patients with RA has not been well studied.

Methods: This study included 413 participants, comprising 208 patients with RA and 205 age- and sex-matched healthy volunteers. Clinical data, BMD, bone metabolic markers (BMM) and body composition, such as lean body mass and percent fat, were collected. Risk factors for osteoporosis in patients with RA including the relationship BMD and body composition were analyzed.

Results: Patients with RA showed low BMD and high BMM compared with controls. Moreover, lean body mass was lower and percent fat was higher in patients with RA. Lean body mass correlated positively and percent fat negatively with BMD. Lean body mass was a positive and disease duration was a negative independent factor for BMD in multivariate statistical analysis.

Conclusion: BMD and lean body mass were significantly lower in patients with RA compared to healthy controls. Lean body mass correlated positively with BMD and decreased lean body mass and disease duration affected low BMD in patients with RA.

Trial Registration: [UMIN Clinical Trials Registry, http://www.umin.ac.jp/ctr/, UMIN000003876].     

Optimal length of time of cryopreserved umbilical cord blood infusion after thawing

Objective

Dimethyl sulfoxide (DMSO) can damage hematopoietic progenitor cells (HPCs) at room temperature. To minimize the time of contact between DMSO and the thawed umbilical cord blood (UCB), there is an incentive to infuse the UCB as quickly as possible. However, the infusion of cryopreserved UCB also results in side effects. Currently, it is difficult to determine the optimal length of time for cryopreserved UCB infusion, not only to ensure the maximum effect of engraftment but also to reduce the toxicity of the cord blood infusion.

Methods

Ten units of cord blood were thawed to assess viability, apoptotic events of CD34⁺ cells and CD45⁺ cells, and the number of colony-forming units (CFUs) at four time points: 0, 10, 20, and 30 minutes post-thaw (PT). The infusion time, side effects, and the speed of platelet and neutrophil engraftment of the 10 patients were monitored.

Results

Within 30 minutes, the viability decreased (P < 0.01), the percentage of early apoptotic CD34⁺ cells and CD45⁺ cells was unchanged. At time point PT30, the number of CFUs was decreased compared to PT0 (P < 0.05), but it was unchanged within 20 minutes. All the 10 UCB cells engrafted well in patients.

Conclusion

This study indicates that post-thawed UCB HPCs are preserved for less than 30 minutes at room temperature; thus, the optimal length of time of cryopreserved cord blood infusion should be no more than 20 minutes after thawing.     

Bacterial translocation in patients with liver cirrhosis: physiology,clinical consequences,and practical implications

Introduction: The gut liver axis is an operative unit that works to protect the human body against potentially harmful substances and microorganisms, maintaining the homeostasis of the immune system. Liver cirrhosis profoundly alters this complex system. The intestine becomes more permeable allowing the translocation of bacteria, bacterial products and fragments into the portal circulation, triggering an abnormal local and systemic inflammatory response and a condition of perpetual immunologic alarm. This immune-inflammatory disorder related to dysbiosis is involved in the development of liver damage and liver cirrhosis complications and increases intestinal permeability in a vicious circle.

Areas covered: The most relevant studies on bacterial translocation, the mechanism of intestinal barrier dysfunction and its consequences in patients with liver cirrhosis have been revised through a PubMed search. Data have been discussed with particular regard to their significance in clinical practice.

Expert commentary: The assessment of bacterial translocation and intestinal permeability is not currently used in clinical practice but may be useful to stratify patients’ prognosis.     

Book Reviews

AIDS—Action Now Helen Jackson, 1989 Zimbabwe, AIDS Counselling Trust (Harare) Z$5 (£1.60)

AIDS—Home Care and Hospice Manual AIDS Home Care & Hospice Program San Francisco [first published in 1987]

AIDS and AIDS-Related Infections: current strategies for prevention and therapy Adrian J. Bint, John Oxford & Phillip J. Daly, 1989 London, Academic Press £12.50 (paperback)     

GATA2 null mutation associated with incomplete penetrance in a family with Emberger syndrome

Introduction: GATA2 mutations are associated with several conditions, including Emberger syndrome which is the association of primary lymphedema with hematological anomalies and an increased risk for myelodysplasia and leukemia.

Objective: To describe a family with Emberger syndrome with incomplete penetrance.

Methods: A DNA sequencing of GATA2 gene was performed in the parents and offspring (five individuals in total).

Results: The family consisted of 5 individuals with a GATA2 null mutation (c.130G>T, p.Glu44*); three of them were affected (two of which were deceased) while two remained unaffected at the age of 40 and 13 years old. The three affected siblings (two boys and one girl) presented with lymphedema of the lower limbs, recurrent warts, epistaxis and recurrent infections. Two died due to hematological abnormalities (AML and pancytopenia). In contrast, the two other family members who carry the same mutation (the mother and one brother) have not presented any symptoms and their blood tests remain normal.

Discussion: Incomplete penetrance may indicate that GATA2 haploinsufficiency is not enough to produce the phenotype of Emberger syndrome. It could be useful to perform whole exome or genome sequencing, in cases where incomplete penetrance or high variable expressivity is described, in order to probably identify specific gene interactions that drastically modify the phenotype. In addition, skewed gene expression by an epigenetic mechanism of gene regulation should also be considered.     

Bringing Creative Writing Instruction into Reminiscence Group Treatment

Objectives: This article describes the program evaluation of a group intervention combining reminiscence with literary creative writing instruction.

Method: This 10-week, 1-hour group was completed seven times in an outpatient geriatric mental health clinic. Each session introduced a different reminiscence theme and creative writing practice. Feasibility and acceptability were assessed through group monitoring and qualitative feedback. Effectiveness was assessed with pre- and post-test depression screening (PHQ-9) and qualitative feedback.

Results: A total of 34 veterans participated in the groups, with a mean group size of 4.86 (SD = .69). Participants were 85% male (n = 29) and 15% female (n = 5) with a mean age of 70.89 (SD = 8.30). To increase accessibility, several adaptations were made. There were statistically significant reductions in PHQ-9 depression scores (p = .005). Veterans reported qualitative improvements including increased motivation and self-expression.

Conclusions: This intervention was feasible and acceptable to the veterans being served. Randomized controlled research is needed to better understand efficacy.

Clinical Implications: Clinicians may consider ways to augment or adapt reminiscence interventions to meet the needs of their patient population. Clinicians may find it useful to integrate creative writing instruction into reminiscence interventions.